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Practical GuidelinesFor the Management of Children with Cancer‘SOBO’ Paediatric Oncology Ward, Queen Elizabeth CentralHospital College of Medicine, Blantyre, Malawi
December 2017 | Trijn Israels | George Chagaluka | Simon Bailey | Liz Molyneux
© 2017 T. Israels. No part of this manual may be reproduced or transmitted in any form or by any means without permission of the authors.
ISBN 978-90-9024383-2
Layout: Freestyle Print
Printed by: Freestyle Print
Photographs by T. Israels
Financial support for the production and printing of this manual was kindly provided by World Child Cancer.
We would like to acknowledge financial support over the years from the following partners for the department of paediatric oncology: The KiMa foundation, World Child Cancer, Children with Cancer in Malawi, the St Baldrick’s Foundation and VOKK.
We would like to thank Sister in Charge and all the nursing staff, Mr Banda and Mrs Eunice Chimata for their help and care for the patients.
Contents
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3
1. General Approach to Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 52. When is Treatment with Curative Intent (not) Feasible? – Some Considerations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 63. Routine Investigations at Admission . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 74. Side Effects of Chemotherapeutic Drugs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 85. Supportive Care . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 96. SIOP PODC Adapted Treatment Guidelines . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 117. Burkitt Lymphoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 12
Clinical Presentations of Patients with Burkitt Lymphoma (BL) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 138. Non Hodgkin’s Lymphoma (other than BL) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 149. Wilms Tumour . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1510. Hodgkin’s Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1611. Acute Lymphoblastic Leukaemia (ALL) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1712. Neuroblastoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1813. Rhabdomyosarcoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1914. Retinoblastoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2015. Brain Tumours . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2116. Hepatocellular Carcinoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2217. Osteosarcoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2318. Germ Cell Tumour . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2419. Kaposi’s Sarcoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 25
Appendix: Treatment Flow Sheets . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 26
eBL 1st Presentation – Stage I – Localized Tumours Only . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 27eBL and Other NHL Protocol 1st Presentation – All Other Stages . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 28eBL and NHL Relapse Protocol . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 29Wilms Tumor – 1A – Preoperative Chemotherapy for Localized Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 30Wilms Tumor – 1B – Preoperative Chemotherapy for Metastatic Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 31Wilms Tumor – Optional – Prolonged and Intensified Preoperative Chemotherapy for Localized Diseaseto Improve Resectability . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 32Wilms Tumor – Post-operative Chemotherapy – AV4 – Localized Disease at Diagnosis –Pathology: Stage I, Intermediate Risk (IR) – Not for Surgical Staging Only . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 33Wilms Tumor – Post-operative Chemotherapy – AV14 Localized Disease at Diagnosis – Pathology: Stage I, High Risk (HR) and Stage II, IR and Stage II, III LR or Surgical Stage I or II / Easy Surgery . . . . . . . . . . . . . . . . . . . . . . 34Wilms Tumor – Post-operative Chemotherapy – AVD 14 – Metastatic Disease at Diagnosis – Localized Diseaseat Diagnosis – Pathology: Stage II High Risk, Stage III IR and HR or Surgical Stage III / Difficult Surgery . . . . . . . . . . . . . . . . . . . . . . . . . . 35OEPPA – Hodgkin’s Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 36Flow Sheet: Prednisolone Pre-Phase ALL . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 37Flow Sheet: Induction ALL . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 38Flow Sheet: Continuation ALL . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 39Flow Sheet: Intensification ALL . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 40Doxorubicin / Cisplatin – Osteosarcoma, Hepatocellular Carcinoma (Neuroblastoma) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 41Etoposide / Cisplatin / Bleomycin – Germ Cell Protocol . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 42Kaposi Sarcoma Vincristine, Bleomycin and Etoposide . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 43Low Grade Glioma – Vincristine and Carboplatin . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 44Retinoblastoma – Chemotherapy – JOE . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 45
Practical Guidelines for the Management of Children with Cancer Practical Guidelines for the Management of Children with Cancer
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What Is It? (The Diagnosis)
This question is usually answered by a careful and thorough history and examination followed by a Fine Needle Aspirate (FNA) or an open biopsy.
When dealing with a malignancy, or the suspicion of a malignancy, one would like to make a definitive diagnosis. In malignancies, this is most commonly done through obtaining tissue and looking at it through a microscope.
This can be done by fine needle aspirate and looking at the cells with a microscope (cytology).
At times one will need to do a tissue biopsy to be able to see the structure of the tumour tissue (histology) to make a definite diagnosis.
Where Is It? (The Extent of Disease/Staging)
After a careful history and examination this question is usually answered by X-rays, ultrasound, CSF sampling, bone marrow aspiration or some other specific investigations. For almost all tumours it is useful to know where it is: what is the primary tumour and has it metastasized to other places. Some additional investigations (e.g. imaging, bone marrow aspirate, CSF) are aimed at assessing involvement of preferential sites for metastases. The presence of distant metastases will often worsen the prognosis of the patient and will sometimes change the management. Is It Safe to Treat?
This question may be answered by looking for evidence of anaemia (FBC and differential) malaria (Rapid Diagnostic Test (RDT) for malaria or thick blood films), stool and urine microscopy. An HIV test is also helpful in anticipating problems and it is important to assess for malnutrition.
In general, there are three treatment modalities in paediatric oncology; chemotherapy, surgery and radiotherapy. Radiotherapy is not available yet in Malawi. Surgery of the primary tumour is often needed in solid tumours to achieve complete cure. Chemotherapy kills malignant (cancer) cells, but will also kill normal cells of the body. This especially affects all rapidly dividing cells; i.e. bone marrow, mucosa (mouth and gastrointestinal tract) and hair follicle cells.
One needs to consider the intensity of the chemotherapy, level of supportive care and tolerance of the patient (malnutrition) in deciding which treatment is safe for a particular patient.
Quality of Life
Palliative care becomes the major focus of treatment when cure is no longer feasible. Adequate pain control, control of other symptoms such as vomiting, and giving psychological, religious and spiritual support are important to improve quality of life. Often the child and parents will prefer to go home with the best available symptomatic control.
Different tumours have differing presenting symptoms and sites, preferential sites for primary tumours and metastases, degrees of aggression of tumour cells and sensitivity to chemotherapy. To know what the diagnosis is and how best to treat we need to ask some simple questions:
This booklet with treatment guidelines is written for the paediatric oncology ward (‘SOBO’) of the Queen Elizabeth Central Hospital (QECH) in Blantyre, Malawi.
It is meant to be a practical manual for the physicians working on the ward (and those covering the ward when on call), nurses, visiting nurses, clinical officers, physicians and students.
The information is not extensive, for more information on the different diseases please refer to a paediatric oncology textbook. The treatment strategies are the optimal treatments currently available to us in QECH.
We hope this manual will help us to further improve the treatment for children with cancer in Malawi.
Dr. Trijn IsraelsProf. Simon BaileyDr. George ChagalukaProf. Liz Molyneux
December 2017
Introduction 1.General Approach to Management
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2.When is Treatment with Curative Intent (not) Feasible? – Some Considerations
3.Routine Investigations at Admission
Weigh the balance between toxicity of treatment, burden for the patient and parents and the chance of survival. At times deciding on treatment with palliative intent is a fair choice and in the best interest of the child and his / her family if chances of cure are not realistic.
Patients with a relatively good prognosis include: endemic Burkitt lymphoma (especially stage I – III), Wilms tumour (especially localized disease), Hodgkin’s disease (especially lower stage), retinoblastoma – intra ocular disease only, acute lymphoblastic leukaemia (especially if very chemo sensitive disease) and germ cell tumour.
Patients with a poor prognosis include those with: neuroblastoma (especially metastatic disease), rhabdomyosarcoma (especially if a large (> 5 cm) primary tumour or metastatic disease), acute myeloid leukaemia and most brain tumours.
Multidisciplinary Teams (MDTs)
Clinicians caring for children with cancer in low-income countries often do so in isolation. Cancer diagnosis and care requires the expertise of several disciplines such as pathology, surgery, radiology, oncology and palliative care. It is very helpful to have the input of all these teams in decisions about diagnosis and in planning treatment. The best way of bringing these groups together is to hold regular (e.g. every fortnight or every month) meetings together. These are called Multidisciplinary Teams Meetings (MDTs) and are routine in high income settings.
MDTs are held every month in the QECH and attended by members of SOBO, by surgeons, pathologists and ophthalmologists.
Fine Needle Aspirate (FNA)The pathologist will examine the cells and attempt to make a diagnosis. Only cytology (examination of cells) can be done on a fine needle aspirate.
Remote Pathology ServiceWhen a Fine Needle Aspirate (FNA) or Bone Marrow sample is taken on the ward, it is put on to microscope slides and stained. When viewed with a microscope this shows the type and number of cells on the slide and is helpful in diagnosing several common tumours such as Burkitt lymphoma. It is important to make a diagnosis as soon as possible and our pathology department are overwhelmed with the amount of work they have; and so the slides in the paediatric annexe are photographed with a microscope camera and immediately uploaded by computer and sent to Newcastle where an expert reviews them and gives an opinion as to what the diagnosis is likely to be. This takes about two days. This is called a remote pathology service.
HistopathologyHistopathology is when a surgical sample (i.e. biopsy) is taken and looked at in the lab. This is necessary for some diagnoses and to be able to tell between some cancer subtypes. This is done in the QECH histopathology lab and results of a biopsy have often delayed in Malawi. Recently, because of an improvement in staffing, the reports have been coming back to the ward more quickly.
Ultrasound Abdomen (USS)An abdominal USS is important:
• In solid abdominal tumours as a scan helps to define a location of the tumour and its relation to organs. The structure of the tumour aids in diagnosis.
• To assess abdominal involvement, especially in patients with Burkitt lymphoma.
• To assess the kidneys and anticipate problems if they are involved.
• To rule out bilateral renal involvement in Wilms tumour.
Elisa HIVIt is important to assess the HIV status of the patient. A newly diagnosed child with HIV will need to commence antiretroviral therapy as soon as possible.
Full Blood Count (FBC)• The FBC helps to assess involvement of
bone marrow in the disease process, to look for anaemia, the possible need for a blood transfusion and the tolerance of this patient for chemotherapy.
• To review the platelet count for likelihood of bleeding.
Urine and Stool MicrobiologyLook for schistosoma eggs in urine and parasites / worms in the stool. These infections / infestations need to be treated before a child receives chemotherapy.
Malaria Parasitaemia (MPs)Malaria is a common condition in Malawi and needs to be treated before a child receives chemotherapy.
OtherIn certain circumstances it may be necessary to exclude TB (CXR, sputum if child old enough to produce some etc.)
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4.Side Effects of Chemotherapeutic Drugs
5.Supportive Care
Vincristine: Neuropathy (constipation, neuropathic pain, areflexia, foot drop), jaw pain, severe tissue necrosis with extravasation.
Precautions to prevent extravasation: Always place a fresh cannula, make sure it is well positioned in the vein! Flush cannula with water for injection first to see that there are no leaks, then follow the vincristine bolus injection with another flush of water for injection.
Cyclophosphamide: Bone marrow suppression, nausea and vomiting, haemorrhagic cystitis.Precaution: Patient must be well hydrated with lots of fluids before and after the dose.
Cisplatin: Bone marrow suppression (especially platelets, delayed), nausea and vomiting, kidney damage, deafness.
Actinomcyin: Nausea and vomiting, bone marrow suppression (mild), veno-occlusive disease (VOD) characterized by fluid retention, ascites, jaundice, painful liver. (This can be life threatening).Precaution: Give enough fluids. Reduce dose in children <12kg body weight.
Doxorubicin (=Adriamycin): Bone marrow suppression, nausea and vomiting, oral sores. Late effect: Cardiomyopathy (dependant on cumulative dose).Precaution: Review the total cumulative dose received regularly. Avoid going above 150 mg/m2.
Prednisolone: Weight gain, Cushing’s syndrome, hypertension, osteoporosis, adrenal suppression (stress hormones), increased susceptibility to and severity of infections.Precaution: Use short courses.
Procarbazine: Bone marrow depression, nausea.
Etoposide: Bone marrow depression, nausea and vomiting (both relatively mild when given orally), mucositis.
Bleomycin: Nausea and vomiting, bone marrow suppression, mucositis, pulmonary fibrosis (dependent on cumulative dose).
Methotrexate: Bone marrow suppression, mucositis and diarrhoea. Leucovorin (folinic acid) is given after MTX infusions to prevent severe side effects.
Fever Protocol
Background: Chemotherapy often causes bone marrow suppression. This can cause the white blood cells to drop with a lowered resistance to, especially bacterial, infections. Neutropenia is defined as a neutrophil count of less than 0.5 x 109 per litre. Children who are neutropenic have an increased risk of bacterial infections which can develop very rapidly.
Protocol: If a child on the ward has a fever (axillary temperature above 38° Celsius):
X Check Malaria parasites (MPs)
If MPs are negative:
X Take a blood culture (important to get information as to which bacteria are causing the infection and sensitivity / resistance to antibiotics).
X Start antibiotics: Benzyl penicillin (50,000 i.u. per kg/day divided over three doses IV). Gentamicin (6mg per kg once daily IV).
• If fever persist for more than 48 hours, 2nd line antibiotics need to be considered.
• Usual choice: ceftriaxone (50mg per kg once daily IV)
• If fevers still persist add amikacin (and stop gentamicin).
• Change antibiotics when needed based on the results of a positive blood culture.
• Antifungal therapy (fluconazole) and antiviral therapy (acyclovir) are added early to the antibiotics if the child has had prolonged or ‘heavy’ chemotherapy (e.g. in ALL) or if the child is malnourished.
• G-CSF (Granulocyte cell stimulating factor) promotes the production of granulocytes (neutrophils) and is useful in preventing febrile neutropenia. If G-CSF is available it is sometimes prescribed in this circumstance.
Mouth Care
• Children may develop a painful mouth (mucositis) from the chemotherapy. Adequate pain control is important to enable the child to eat and drink.
• 1% gentian-violet paint (“GV paint”) three times a day can be used when there are oral sores and/or thrush.
• Thrush (candida infection) can also be treated with nystatin oral drops three times daily.
• If fungal infection is severe fluconazole needs to be added.
• When herpetic lesions (pain, blisters) are suspected clinically, add oral acyclovir (older than 2 years: 200mg 5 times daily x 5/7; younger than 2 years: 100mg 5 times daily x 5/7).
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Nutrition
Children with cancer are often malnourished. • Malnutrition will reduce their immunity to infections,
tolerance to chemotherapy and increase the risk of surgery.
• Try to encourage children to eat.• Encourage the children to eat one sachet of
chiponde every day.• If a child cannot eat, but can drink; milk (F100) is a
nutritious alternative.• If a child cannot or will not eat or drink adequately a
nasogastric tube (NGT) should be inserted and used for feeds.
Pain control: ‘Pain is what the patients says hurts.’Do not wait for pain, anticipate it and try to prevent it.
Non-pharmacological interventions:Distraction (e.g. for procedures), play therapyPhysical comfort (positioning or massage).
Pharmacological interventions:WHO analgesic ladder for children.
Step 1: Non-opioid +/- adjuvante.g. paracetamol 10-15mg/kg every 4-6 hours oral or ibuprofen 5-10mg/kg every 6-8 hours oral.
Step 2: Strong opioid (morphine) +/- adjuvant+/- step 1
e.g. starting dose: oral morphine 0.15-0.3mg/kg every 4 hours.
Titrate against pain and side effects (nausea, vomiting, and constipation).
Do not use codeine and morphine concurrently.
Use of Adjuvants
1. For raised intracranial pressure: Dexamethasone 10mg/m2 per day orally Usually in daily practice we would give 5mg once daily. (For a maximum of 3 days to limit side effects.)
2. For neuropathic pain (burning, stinging in extremities): Amitriptyline (0.2 – 0.5mg/kg) orally Age 1 – 5 years 6.25mg. Age 6 – 12 years 12.5mg.
Anti-Emetics
• Anti-emetics are given to reduce chemotherapy associated vomiting.
• Give oral metoclopramide (10 mg or 100 – 400 μg/kg) 30 minutes before chemotherapy.
• If needed add oral (0,15mg/kg, max 8 mg) or IV (0,01mg/kg, max 4 mg) ondansetron 8 hourly. If needed add dexamethasone IV ( 5mg/m2 – max 4 mg stat 30 minutes before chemotherapy, followed by 5mg/m2 divided in two to three doses).
Reference for Further Reading
Israels T, Renner L, Hendricks M, Hesseling P, Howard S, Molyneux E. SIOP PODC: Recommendations for Supportive Care of Children with Cancer in a Low-Income Setting. Pediatr Blood Cancer 2013, Jun;60(6):899-904.
6.SIOP PODC Adapted Treatment Guidelines
The PODC (Pediatric Oncology in Developing Countries) Committee of the International Society of Pediatric Oncology (SIOP) has a working group on adapted treatment guidelines. These are guidelines adapted to the local realities in low income countries which means that they are often of reduced intensity to avoid undue treatment-related toxicity and mortality.
Some of these guidelines (e.g Wilms tumour and KS) are very similar to the treatment guidelines currently used in Malawi. Others are different, but may still provide useful further reading with reference to the principles and considerations taken into account.
So far SIOP PODC has published guidelines on acute lymphoblastic leukaemia (ALL), endemic Burkitt lymphoma (eBL), Wilms tumour, supportive care, retinoblastoma, Kaposi sarcoma (KS), neuroblastoma and medulloblastoma. All guidelines are published in Pediatric Blood & Cancer.
References for Further Reading
Parikh NS et al. SIOP-PODC adapted risk stratification and treatment guidelines: Recommendations for neuroblastoma in low- and middle-income settings. Pediatr Blood Cancer. 2015 Mar 21. doi: 10.1002/pbc.25501.
Hunger SP et al. Treatment strategies and regimens of graduated intensity for childhood acute lymphoblastic leukemia in low-income countries: A proposal. Pediatr Blood Cancer 2009, May;52(5):559-65.
Parkes J et al. SIOP PODC adapted treatment recommendations for standard-risk medulloblastoma in low and middle income settings. Pediatr Blood Cancer 2015, Apr;62(4):553-64.
Chantada G et al. SIOP-PODC recommendations for graduated-intensity treatment of retinoblastoma in developing countries. Pediatr Blood Cancer 2013, May;60(5):719-27.
Hesseling P et al. Practical recommendations for the management of children with endemic Burkitt lymphoma (BL) in a resource limited setting. Pediatr Blood Cancer 2013 March; 60(3):357-62.
Israels T et al. SIOP PODC: clinical guidelines for the management of children with Wilms tumour in a low-income setting. Pediatr Blood Cancer 2013 January; 60(1):5-11.
Israels T et al. SIOP PODC: Recommendations for Supportive Care of Children with Cancer in a Low-Income Setting. Pediatr Blood Cancer 2013 Jun;60(6):899-904.
Molyneux E et al. The management of children with Kaposi sarcoma in resource limited settings. Pediatr Blood Cancer 2013 April;60(4):538-42.
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Girl (8) with jaw tumour
Girl (10) with abdominal BL
Boy (6) with upper jaw tumour
Girl (10) with displaced teeth in BL
7.Burkitt Lymphoma
Clinical Presentations of Patients with Burkitt Lymphoma (BL)
Clinical Presentation• Rapidly growing tumour, peak age 4 – 7 years, boys
more often affected than girls.• Site of presentation: Jaw, retro-orbital, abdomen
(including kidneys), paraspinal (can cause paraplegia, urinary/stool incontinence) and in CNS. Relatively common childhood cancer in Malawi (~ 40% of patients).
• Treatment is needed urgently when children present with neurological symptoms or orbital disease to try to prevent irreversible damage.
Differential Diagnosis• Other lymphoma. • Face/jaw: Abscess, dental cyst.• Eye: Retinoblastoma, rhabdomyosarcoma. • Kidney: Wilms tumour.• Extremity (arm/leg): Osteosarcoma, osteomyelitis.
Investigations at Admission• For routine investigations please see chapter 13.• For final diagnosis: Fine needle aspirate (FNA).• For staging: Bone marrow aspiration (BMA),
cerebrospinal fluid (CSF), ultrasound abdomen (USS). (Treatment is currently no different for different stages of disease, but a higher stage of disease does affect outcome; stage IV (with bone marrow and/or CSF involvement) has a poorer prognosis).
Supportive CareTumour Lysis Syndrome:The rapid destruction of tumour cells (breakdown products) may cause a tumour lysis syndrome, characterized by renal failure (‘blockage of kidney’) and laboratory abnormalities (especially potassium�, phosphate�, calcium�).
To prevent/manage tumour lysis syndrome: • Allopurinol 5mg/kg tds orally x 5/7; starting the day
before chemotherapy.• Hyperhydration according to age / weight.• Watch for decreased urine output and/or fluid
overload as signs of tumour lysis syndrome. • Close monitoring (sometimes daily) of the
electrolytes in important and can be measured quickly on the blood gas analyser in the paediatric annexe. Samples for analysis by the blood gas machine need to be taken in a heparinised syringe that is available in the annexe lab.
Nausea and vomiting. Prevent with metoclopramide (maxolon) 10mg po; half an hour before and ~ 5 hours after chemotherapy. If remain nauseated and vomiting or develops despite metoclopramide, add ondansetron 4mg IV or PO stat (<5 years – 2 mg).
Neutropenia do weekly full blood counts (FBC). If neutrophils are lower than 1.0 x 109/L it is necessary to delay chemotherapy until recovery of the neutrophil count (above 1.0 x 109/L).
Treatment• Chemotherapy.• See flow sheets: eBL localized stage I, eBL and NHL
all other stages, eBL and NHL relapse.
Reference for Further Reading:
Hesseling P, Israels T, Harif M, Chantada G, Molyneux E. Practical recommendations for the management of children with endemic Burkitt lymphoma (BL) in a resource limited setting. Pediatr Blood Cancer 2013 March; 60(3):357-62.
Practical Guidelines for the Management of Children with Cancer Practical Guidelines for the Management of Children with Cancer
1312
Boy (3) with Wilms tumour Boy (3) with Wilms tumour (and malnutrition)
Boy (16) with NHL before treatment Boy (16) after treatment
8.Non-Hodgkin Lymphoma Other Than BL
9.Wilms Tumour
Burkitt lymphoma is one of the non-Hodgkin lymphomas. Others include lymphoblastic lymphoma, large B-cell lymphoma and anaplastic large cell lymphoma.
Lymphoblastic leukaemia is best treated on the leukaemia protocol.
Clinical Presentation • Clinical presentation is varied and depends on
primary site, histological subtype and extent of disease. Non-Hodgkin lymphomas often present in the abdomen, mediastinum and head and neck, less commonly in superficial lymph nodes and bone.
Differential Diagnosis• Other causes of lymph node swelling (e.g.
tuberculosis), HIV, Kaposi Sarcoma, Burkitt lymphoma, Hodgkin’s disease.
Investigations at Admission:• Routine investigations (Chapter 13).• Mantoux if possible, CXR and FNA for AFBs when
TB is a possible diagnosis.• For final diagnosis: Fine needle aspirate (FNA) If this
fails to provide a diagnosis a biopsy will be needed.
Treatment• Chemotherapy.• See flow sheets: eBL localized stage I, eBL and NHL
all other stages, eBL and NHL relapse.
Clinical Presentation• Slow growing painless mass in the flank. Usually
clinically relatively well. Peak age 3 years. Relatively common tumour.
• Patients often found to have hypertension and (microscopic) haematuria.
• Metastasizes to lungs (Chest X-ray), less frequently to the liver (USS).
Differential Diagnosis• Burkitt lymphoma (more rapidly growing, more
weight loss), Neuroblastoma (adrenal mass, patients usually more severely ill and in more pain and often anaemic).
Investigations at Admission• Routine investigations (chapter 13).• Ultrasound abdomen (renal tissue visible? cystic
tumour? IVC involved? local spread to lymph node or liver? other kidney normal?).
• Blood pressure, urine dipstick for blood.• For cytology: Fine needle aspirate.• For staging / metastases: Chest X-ray (lungs),
Ultrasound abdomen (liver).
Treatment • Chemotherapy and surgery.• See flow sheets:
Preoperative chemotherapy – 2 different protocols* Postoperative chemotherapy – 3 different protocols**
* Preoperative chemotherapy depends on the absence / presence of distant metastatic disease i.e. chest X-ray (metastases lungs) and USS (metastases liver).** Postoperative chemotherapy depends on stage and risk group of the tumour at surgery (pathologist’s report) – or, if no pathology report is available on surgical staging and difficulty of the surgery.
References for Further Reading:
Israels T, Moreira C, Scanlan T, Molyneux L, Kampondeni S, Hesseling P et al. SIOP PODC: clinical guidelines for the management of children with Wilms tumour in a low-income setting. Pediatr Blood Cancer 2013 January; 60(1):5-11.
Israels T, Chagaluka G, Pidini D, Caron H, de Kraker J, Kamiza S et al. The efficacy and toxicity of SIOP preoperative chemotherapy in Malawian children with a Wilms tumour. Pediatr Blood Cancer 2012 October; 59(4):636-41.
Practical Guidelines for the Management of Children with Cancer Practical Guidelines for the Management of Children with Cancer
1514
Boy (13) with Hodgkin’s disease Boy (13) with Hodgkin’s disease
10.Hodgkin’s Disease
11.Acute Lymphoblastic Leukaemia (ALL)
Clinical Presentation • Usually adolescents, more common in boys;
painless enlarged lymph nodes in the neck (80%), often with a widened mediastinum on chest X ray.
• Can also involve other lymph nodes, lymphatic structures (abdomen, LN in the groin or axilla, spleen).
• B symptoms (systemic disease): fever, night sweats, pruritus (itch), weight loss – especially with advanced disease.
Differential Diagnosis• Other causes of lymph node swelling (e.g.
tuberculosis), Burkitt lymphoma (usually younger children), Kaposi’s sarcoma (usually HIV positive) and other non-Hodgkin’s lymphoma.
Investigations at Admission• Routine investigations (Chapter 13).• Mantoux skin test if possible or FNA for AFB
(acid fast bacilli) when TB is a possible diagnosis. GenExpert on body fluid sample.
• For final diagnosis: Fine needle aspirate (FNA) – histology with a tissue diagnosis is much preferred in Hodgkin’s disease.
• For staging: Chest X-ray, USS abdomen.
Treatment• See flow sheets for ABVD, OEPPA or COPP (last p =
procarbazine) depending on availability of drugs.
Acute leukaemia can be classified as acute myeloid and acute lymphoblastic leukaemia. Acute lymphoblastic leukaemia is the most common.
Clinical PresentationPatients present with signs and symptoms caused by invasion of the bone marrow by leukaemic cells.Anaemia, bleeding tendency (low platelets), fever (reduced immunity) and bone pain are common symptoms. The history will usually be short (a few weeks).
Differential DiagnosisMalaria, acute viral infection such as EBV, aplastic anaemia.
Investigations at Admission• Routine investigations (Chapter 13).• Blood film for morphology (send to haematology).• Bone marrow aspirate if platelets are adequate.
Treatment• Please see the ALL flow sheets.• Start with a prednisone and allopurinol pre-phase
treatment. Start with a low dose and gradually increase the dose if the child is in a poor condition or at risk for tumour lysis syndrome (high white cell count, massive hepatosplenomegaly).
References for Further Reading:
Chagaluka G, Carey P, Banda K, Schwab C, Chilton L, Schwalbe E et al. Treating childhood acute lymphoblastic leukemia in Malawi. Haematologica 2013 January;98(1):e1-e3.
Hunger SP et al. Treatment strategies and regimens of graduated intensity for childhood acute lymphoblastic leukemia in low-income countries: A proposal. Pediatr Blood Cancer 2009 May;52(5):559-65.
Practical Guidelines for the Management of Children with Cancer Practical Guidelines for the Management of Children with Cancer
1716
12.Neuroblastoma
13.Rhabdomyosarcoma
Clinical Presentation • Solid tumour, clinical manifestations vary, at times
with hypertension, peak age 0-4 years. Patients often ill at presentation, especially with metastatic disease. Pain and anaemia are common.
• Site of presentation: Abdomen (adrenal), sympathetic chain, 25% primaries are in neck or thorax, 70% abdomen, 5% pelvis.
• Metastases: Bone, lymph nodes, bone marrow, (skin), often present with metastatic lumps on the head, or racoon eyes.
• Prognosis is usually poor if metastatic disease is present.
• Patients who are below one year often have less aggressive disease and a better prognosis.
Differential Diagnosis• Burkitt lymphoma (in widespread disease). • Other solid tumours (abdomen).
Investigations at Admission• Routine investigations (Chapter 13).• Ultrasound abdomen (? adrenal tumour). Plain X-ray
may identify calcification.• MRI if there is doubt about the origin of the mass.• For final diagnosis: Fine needle aspirate (FNA) and
Bone Marrow Aspirate.• Urine for tumour markers if available.• Tumour markers: Metabolites of catecholamines in
urine (VMA and HVA).
Treatment• Chemotherapy and surgery.• See flow sheet VAC (good empirical treatment to
start with, if neuroblastoma confirmed, consider carboplatin instead of cyclophosphamide).
• Surgery of primary tumour with curative intent if no residual metastases post chemotherapy.
Rhabdomyosarcoma is the most common soft tissue sarcoma in childhood.
Clinical Presentation• Solid tumour. Most common primary sites: Head
and neck; orbit of eye, nose and throat (40 %), bladder, vagina (20 %), extremities (20 %).
• Prognosis is very poor in metastatic disease.• Prognosis is better in small primary tumours
(smaller than 5 cm).
Investigations at Admission• Routine investigations (please see chapter 13).• Measure the tumour (important for prognosis in
localized tumours).
Treatment• Chemotherapy and surgery.• See flow sheet VAC, with A = Actinomcyin.• Surgery of primary tumour if completely resectable
and no metastases.
Practical Guidelines for the Management of Children with Cancer Practical Guidelines for the Management of Children with Cancer
1918
Patient (1 year) with a rhabdomyosarcoma of the lower arm
Patient with advanced,Metastatic retinoblastoma
Patient after enucleation of the left eye. Relapse (metastases) on the right side
14.Retinoblastoma
15.Brain Tumours
Clinical Presentation• Early: Leukocoria (white pupil reflex in the eye),
strabismus (squint).• Late: Proptosis (eye pushed forward), orbital mass,
and often destruction of the eye ball.• Can be inherited (then more often bilateral disease).• Average age at diagnosis 2 years (unilateral
disease), 1 year (bilateral disease), but often older in Malawi.
• Metastases: Intracerebral, bone, peri-auricular (around the ear).
Differential Diagnosis • When advanced disease: Burkitt lymphoma
(BL usually develops more rapidly + ask about white pupil reflex in eye to differentiate), orbital rhabdomyosarcoma, metastatic neuroblastoma, other lymphoma.
Investigations at Admission• Routine investigations (chapter 13).• Bone marrow and CSF examination to assess
tumour spread.• Usually referred from ophthalmologist. Need both
eyes examined by ophthalmologist.
Treatment• Chemotherapy with JOE.• Amount of treatment depends on the stage of
disease.• If intraocular only: 2 courses of JOE then surgery
(enucleation of the eye).• If extra ocular disease: 2 - 4 courses of JOE and
then exenteration. • If distant metastases: (palliative) chemotherapy.
Post op needs further chemotherapy to complete 6 courses in total.
Palliative Chemotherapy:• 1st choice: cyclophosphamide orally 40mg/kg, once
weekly (tablets are 50mg).• Alternative: cisplatin IV or oral etoposide 100mg/
m2 weekly.
Inform the guardian about the risk of another child in the family having retinoblastoma (especially if the patient is below 1 year or bilateral disease) and early signs (white pupil). If possible, refer mother with any further children to ophthalmologist for early screening.
Reference for Further Reading:
Chantada G, Luna-Fineman S, Sitorus RS, Kruger M, Israels T, Leal-Leal C et al. SIOP-PODC recommendations for graduated-intensity treatment of retinoblastoma in developing countries. Pediatr Blood Cancer 2013 May;60(5):719-27.
Clinical Presentation • Often signs and symptoms of raised intracranial
pressure: Headache, vomiting, disturbed vision.• Differential diagnosis• Important to exclude curable conditions• Burkitt lymphoma, infectious causes
Investigations at Admission• Routine investigations (Chapter 13)• MRI head
Treatment• As radiotherapy is not available treatment is mainly
symptomatic.• Dexamethasone for raised ICP; VP shunt for
hydrocephalus.
Some low grade gliomas can be managed with surgery and/or chemotherapy and depending on the site of the tumour.
Reference for Further Reading:
Parkes J, Hendricks M, Ssenyonga P, Mugamba J, Molyneux E, Schouten-van Meeteren A, Qaddoumi I, Fieggen G, Luna-Fineman S, Howard S, Mitra D, Bouffet E, Davidson A, Bailey S. SIOP PODC adapted treatment recommendations for standard-risk medulloblastoma in low and middle income settings. Pediatr Blood Cancer. 2015 Apr;62(4):553-64.
Practical Guidelines for the Management of Children with Cancer Practical Guidelines for the Management of Children with Cancer
2120
Boy (12) with hepatocellular carcinoma
Osteosarcoma Patient with Burkitt lymphoma. Essential to differentiate from osteosarcoma.
16.Hepatocellular Carcinoma
17.Osteosarcoma
Clinical Presentation• Abdominal pain, enlarged hard, lumpy, not tender
liver (and spleen), weight loss.• Occasionally: Fever, jaundice.• More common with endemic hepatitis B infection
(as in Malawi before Hep B vaccination was introduced). Usually children older than 5 years; boys more than girls. A relatively rare tumour.
Differential Diagnosis• In young children (usually < 2 years)
hepatoblastoma.• Hepatitis (usually shorter history, often low grade
fever, liver smoothly enlarged, more painful).
Investigations• Routine investigations (chapter 13).• Serum alpha feto protein (AFP), CXR.• For final diagnosis: FNA.
Treatment• Chemotherapy and surgery.• Curative only if resection is possible (this depends
on the site and extent of the liver infiltrates).• Chemotherapy is used in an attempt to shrink the
tumour and reduce pain.• Please see flow sheet cisplatin / doxorubicin.
Clinical Presentation• Painful swelling arising from affected bone. More
common in teenagers.• Common sites: distal femur (upper leg) ≈ 30 %,
proximal tibia (lower leg) ≈ 15%, proximal humerus (upper arm) ≈ 10 %.
• Metastases: lungs, always check for regional lymphadenopathy.
• Extent of the disease at presentation is the most important prognostic factor.
Differential Diagnosis• Burkitt lymphoma has to be excluded before
treatment.• Ewing sarcoma: Usually affects the diaphysis (mid
bone) and flat bones.
Investigations at Admission• X-ray of the affected bone may show bone
destruction, periosteal elevation and new bone formation.
• For final diagnosis: Fine needle aspiration.• For metastases: chest X-ray; check groin and lower
abdomen for LNs.
Treatment• Chemotherapy and surgery.• First choice: Please see flow sheet cisplatin /
doxorubicin. • (Alternative is VAC (A = Adriamycin)).• Surgical treatment may improve quality of life in
metastatic disease.
Practical Guidelines for the Management of Children with Cancer Practical Guidelines for the Management of Children with Cancer
2322
Oste Skin lesions foot (painful) osarcoma A 1 year old patient on oxygen (pulmonary disease) and with axillary lymphadenopathy.
18.Germ Cell Tumour
19.Kaposi’s Sarcoma
Often in children below 3 years or above 12 years.Most common germ cell tumours: teratoma (often sacrococcygeal) and yolk sac tumour.
Yolk sac tumour
Clinical Presentation• Usually a painless mass.• In infancy usually sacrococcygeal region. In older
children usually testes or ovaries affected.• Tumour markers; serum α-fetoprotein and serum β–HCG are often raised.
Investigations at Admission• Routine investigations: please see chapter 13.• Ultrasound abdomen.• Tumour markers can be done in the college lab• For final diagnosis: Fine needle aspirate, but can
often go straight for surgery.
Treatment• Complete resection is often curative.• Preoperative chemotherapy may be needed to
shrink the tumour and make surgery possible. Please see flow sheet cisplatin, etoposide and bleomycin.
Clinical Presentation• Usually in HIV infected patients but can also occur
in HIV negative patients.• Varied clinical presentation. • Enlarged, often generalized, hard, lymph nodes.• Dark raised patches and nodules in the skin
and subcutaneous tissue of feet, legs, face and genitalia.
• Often painful brawny, lymphoedema especially of legs (difficulty to walk).
• Oral cavity, dark, pigmented lesions on hard palate.• Gastrointestinal disease is common and associated
withbloody rectal discharge, abdominal pain and sometimes obstruction.
• Pulmonary disease with bloody pleural effusion is often life threatening (differential diagnosis TB).
Differential Diagnosis• TB, lymphoma if LNs involved.
Investigations at Admission• Routine investigations please see chapter 13.• Consider TB when appropriate, beware of false
negative Mantoux result in HIV infected children.• Fine needle aspirate only if diagnosis in doubt.
(Aspirate often bloody).
• Treatment is symptomatic.• If HIV positive:
1. ARVs (anti retrovirals) are first line treatment. 2. Chemotherapy includes vincristine, bleomycin and etoposide (VBE) see flow charts.
• If HIV negative: Give chemotherapy (VBE).
Thalidomide is often helpful in palliative care.Thalidomide 3 mg/kg nocte for 2 months can be used but it is expensive.
Be sure to warn the guardian never to give thalidomide to anyone else as it is teratogenic (can goes congenital abnormalities if taken in early pregnancy).
References for Further Reading Molyneux E, Davidson A, Orem J, Hesseling P, Balagadde-Kambugu J, Githanga J et al. The management of children with Kaposi sarcoma in resource limited settings. Pediatr Blood Cancer 2013 April;60(4):538-42.Chagaluka G., Stanley C., Banda K., Depani S. Katangwe K., Israels T et al Kaposi’s sarcoma in children: an open randomised trial of vincristine, oral etoposide and a combination of vincristine and bleomycin. Eur. J. Cancer.
Practical Guidelines for the Management of Children with Cancer Practical Guidelines for the Management of Children with Cancer
2524
eBL
1st P
rese
ntat
ion
– St
age
I – L
ocal
ized
Tum
ours
Onl
y
Patie
nt N
ame:
Max
olon
(met
oclo
pram
ide)
10
mg
befo
re a
nd a
fter (
afte
rnoo
n) c
hem
othe
rapy
to re
duce
vom
iting
.Al
lopu
rinol
5 m
g/kg
tds
for 5
day
s to
pre
vent
tum
or ly
sis
synd
rom
e.
At A
dmis
sion
:
Cycl
opho
spha
mid
e
IT M
TX/H
C
Wei
ght
Heig
htBo
dy S
urfa
ce A
rea
Cycl
opho
spha
mid
e da
y 1:
---
----
mg
(40
mg/
kg, m
ax 1
.6 g
r)Cy
clop
hosp
ham
ide
day
8, 1
8, 2
8: -
----
mg
(60
mg/
kg, m
ax 2
.4 g
r)In
trath
ecal
Met
hotre
xate
(MTX
) 12.
5 m
g / H
ydro
corti
sone
(HC)
12.
5 m
g
......
....
......
....
......
....
Age:
Date
of A
dmis
sion
:
1DA
YS
Date
Give
n:
Size
Tum
our:
Pres
entin
g Co
mpl
aint
s:
USS:
(Pre
sum
ed) D
iagn
osis
:
FNA
Date
:FN
A Re
sult:
......
......
......
.....
......
......
.....
......
......
......
.....
......
......
.....
818
28
Appendix:Treatment Flow Sheets
27
Practical Guidelines for the Management of Children with Cancer
26
eBL
and
Oth
er N
HL
Prot
ocol
1st
Pre
sent
atio
n –
All
Oth
er S
tage
s
Patie
nt N
ame:
Max
olon
(met
oclo
pram
ide)
10
mg
befo
re a
nd a
fter (
afte
rnoo
n) c
hem
othe
rapy
to re
duce
vom
iting
.Al
lopu
rinol
5 m
g/kg
tds
for 5
day
s to
pre
vent
tum
or ly
sis
synd
rom
e.
At A
dmis
sion
:
Cycl
opho
spha
mid
eDo
xoru
bici
n(S
tage
2, 3
and
4)
Pred
niso
lone
Vinc
ristin
e
IT M
TX/H
C
Wei
ght
Heig
ht
Body
Sur
face
Are
a
Cycl
opho
spha
mid
e da
y 1,
15,
28:
40
mg/
kg (m
ax 1
.6 g
r)Cy
clop
hosp
ham
ide
day
8: 6
0 m
g/kg
(max
2.4
gr)
Doxo
rubi
cin
day
15: 6
0 m
g/m
2 ove
r 4 h
ours
Pred
niso
lone
day
1-5
: 60
mg/
m2 p
er d
ay in
2 d
ivide
d do
ses
Dose
Vin
cris
tine
day
1, 8
, 15,
28,
42:
1.5
mg/
m2 (
max
2 m
g))
Intra
thec
al M
etho
trexa
te: 1
2.5
mg
/ Hyd
roco
rtiso
ne 1
2.5
mg
if >
3 yr
s
......
....
......
....
......
....
Age:
Date
of A
dmis
sion
:
1DA
YS
Date
Give
n:
Size
Tum
our:
USS:
Pres
entin
g Co
mpl
aint
s:
FNA
Date
:FN
A Re
sult:
(Pre
sum
ed) D
iagn
osis
:
......
......
......
.....
......
......
.....
......
......
......
.....
......
......
.....
815
28
eBL
and
NH
L Re
laps
e Pr
otoc
ol
Patie
nt N
ame:
Max
olon
(met
oclo
pram
ide)
10
mg
befo
re a
nd a
fter (
afte
rnoo
n) c
hem
othe
rapy
to re
duce
vom
iting
.Al
lopu
rinol
5 m
g/kg
tds
for 5
day
s to
pre
vent
tum
our l
ysis
syn
drom
e.
At A
dmis
sion
:
Cycl
o
Doxo
rubi
cin
Pred
niso
lone
Vinc
ristin
e
Met
hotr
exat
e
Etop
osid
e
MTX
/HC
Wei
ght
Heig
ht
Body
Sur
face
Are
a
Met
hotre
xate
day
8 1
g /m
2 – s
ee p
roto
col f
or m
ore
deta
iled
dire
ctio
ns a
nd fo
lic a
cid
resc
ue.
Cycl
opho
spha
mid
e da
y 1,
8, 2
2 (6
0 m
g/kg
, max
2.4
gr)
Doxo
rubi
cin
day
8 (6
0 m
g/m
2 ove
r 4 h
ours
)Pr
edni
solo
ne d
ay 1
-5 (6
0 m
g/m
2 per
day
in 2
divi
ded
dose
s)Vi
ncris
tine
day
1, 8
, 18,
28
(1.5
mg/
m2 (
max
2 m
g))
Etop
osid
e da
y 22
and
23
150/
mg/
m2 d
ose
– da
ily x
2 d
ays)
Ritu
ximab
(if a
vaila
ble)
D1,
22
– (3
75m
g/m
2 ) –
don’
t give
if in
fect
ed.
Intra
thec
al M
etho
trexa
te 1
2.5
mg
/ Hyd
roco
rtiso
n 12
.5 m
g
......
....
......
....
......
....
Age:
Date
of A
dmis
sion
:
1DA
YS
Date
Give
n:
Size
Tum
our:
USS:
Pres
entin
g Co
mpl
aint
s:
FNA
Date
:FN
A Re
sult:
(Pre
sum
ed) D
iagn
osis
:
......
......
......
.....
......
......
.....
......
......
......
.....
......
......
.....
815
22
2928
Wilm
s Tum
our
1A –
Pre
oper
ativ
e Ch
emot
hera
py fo
r Loc
alize
d D
iseas
e
Nam
e of
Pat
ient
:
At a
dmis
sion
:
Wei
ght (
kg)
Heig
ht (c
m):
Body
sur
face
area
(m2 ):
Both
dru
gs a
re g
iven
i.v. p
ush.
Date
of B
irth:
Dose
Vin
cris
tine
(1.5
mg/
m2 (
max
2 m
g))
Dose
Act
inom
ycin
(45μ
g/kg
(max
2 m
g))
Body
wei
ght <
12kg
?(If
‘yes
’ red
uce
dose
of b
oth
drug
s to
2/3
)
Date
of A
dmis
sion
:
Actin
omyc
in D
Vinc
ristin
e
Surg
ery
1W
EEKS
Date
Give
n:...
......
......
.....
......
......
......
.....
......
......
.....
......
......
......
..
23
4
Befo
re C
hem
o:•
USS
(+ tu
mou
r size
)•
FBC
• Ch
est X
-Ray
• Bl
ood
pres
sure
• Ur
ine
dips
tick
(blo
od, p
rot)
• FN
A•
MPs
/ El
isa
• Si
ze tu
mou
r (ta
pe m
easu
re)
• M
UAC
Befo
re S
urge
ry:
• Tu
mou
r res
pons
e•
Size
tum
our (
tape
mea
sure
)•
Repe
at U
SS•
Clin
icia
n’s
asse
ssm
ent
Wilm
s Tum
our
1B –
Pre
oper
ativ
e Ch
emot
hera
py fo
r Met
asta
tic D
iseas
e
Nam
e of
Pat
ient
:
At a
dmis
sion
:
Wei
ght (
kg)
Heig
ht (c
m):
Body
sur
face
area
(m2 ):
Vinc
ristin
e an
d ac
tinom
ycin
are
give
n i.v
. pus
h.Do
xoru
bici
n is
give
n in
a 6
hou
r inf
usio
n.
Date
of B
irth:
Dose
Vin
cris
tine
(1.5
mg/
m2 (
max
2 m
g))
Dose
Act
inom
ycin
(45μ
g/kg
(max
2 m
g))
Dose
Dox
orub
icin
(30m
g/m
2 )
Body
wei
ght <
12kg
?(If
‘yes
’ red
uce
dose
of b
oth
drug
s to
2/3
)
Date
of A
dmis
sion
:
Doxo
rubi
cin
Actin
omyc
in D
Vinc
ristin
e
Surg
ery
12
34
56
78
910
WEE
KS
Befo
re C
hem
o:•
USS
(+ tu
mou
r size
)•
Inve
stig
atio
ns**
• Si
ze tu
mou
r (ta
pe m
easu
re)
• M
UAC
Befo
re S
urge
ry:
• Tu
mou
r res
pons
e•
Size
tum
our (
tape
mea
sure
)•
Repe
at U
SS•
Clin
icia
n’s
asse
ssm
ent
** In
vest
igat
ions
: FBC
, BP,
Ches
t X-r
ay, F
NA, d
ipst
ick
urin
e (p
rot/b
lood
), El
isa,
MPs
® C
hest
X-r
ay a
nd /
or u
ltras
ound
abd
omen
to d
eter
min
e re
gres
sion
and
rese
ctab
ility
of m
etas
tase
s.®®
3130
Wilm
s Tum
our
Opt
iona
l – P
rolo
nged
and
Inte
nsifi
ed P
reop
erat
ive
Chem
othe
rapy
for L
ocal
ized
Dise
ase
to Im
prov
e Re
sect
abili
ty
Nam
e of
Pat
ient
:
At a
dmis
sion
:
Wei
ght (
kg)
Heig
ht (c
m):
Body
sur
face
area
(m2 ):
Vinc
ristin
e an
d ac
tinom
ycin
are
give
n i.v
. pus
h. D
oxor
ubic
in is
give
n in
a 6
hou
r inf
usio
n.
Date
of B
irth:
Dose
Vin
cris
tine
(1.5
mg/
m2 (
max
2 m
g))
Dose
Act
inom
ycin
(45μ
g/kg
(max
2 m
g))
Body
wei
ght <
12kg
?(If
‘yes
’ red
uce
dose
of b
oth
drug
s to
2/3
)
Date
of A
dmis
sion
:
Doxo
rubi
cin
Actin
omyc
in D
Vinc
ristin
e
See
prev
ious
pre
op c
hem
o 1A
Surg
ery
1 ......
......
23 ...
......
...
45 ...
......
...
67 ...
......
...
8W
EEKS
Date
Give
n:
Befo
re C
hem
o:•
USS
(+ s
ize tu
mou
r)•
FBC
• Ch
est X
-Ray
• Bl
ood
pres
sure
• Ur
ine
dips
tick
(blo
od, p
rot)
• FN
A, M
Ps /
Elis
a, S
ize
tum
our (
tape
mea
sure
)•
MUA
C
Befo
re P
rolo
ngat
ion:
• Tu
mou
r res
pons
e•
Size
tum
our (
tape
mea
sure
)•
Repe
at U
SS•
Clin
icia
n’s
asse
ssm
ent
Befo
re S
urge
ry:
• Tu
mou
r res
pons
e•
Size
tum
our (
tape
mea
sure
)•
Repe
at U
SS•
Clin
icia
n’s
asse
ssm
ent
*
Wilm
s Tu
mou
rPo
st-o
pera
tive
Chem
othe
rapy
– A
V4 –
Loc
alize
d D
iseas
e at
Dia
gnos
isPa
thol
ogy:
Sta
ge I,
Inte
rmed
iate
Risk
(IR)
– N
ot fo
r Sur
gica
l Sta
ging
Onl
y
Nam
e of
Pat
ient
:
At a
dmis
sion
:
Wei
ght (
kg)
Heig
ht (c
m):
Body
sur
face
area
(m2 ):
Both
dru
gs a
re g
iven
i.v. p
ush.
Date
of B
irth:
Dose
Vin
cris
tine
(1.5
mg/
m2 (
max
2 m
g))
Dose
Act
inom
ycin
(45μ
g/kg
(max
2 m
g))
Body
wei
ght <
12kg
?(If
‘yes
’ red
uce
dose
of b
oth
drug
s to
2/3
)
Date
of A
dmis
sion
:
Actin
omyc
in D
Vinc
ristin
e
1W
EEKS
Date
Give
n:...
......
......
.....
......
......
......
.....
......
......
.....
......
......
......
..
23
4
3332
Wilm
s Tum
our
Post
-ope
rativ
e Ch
emot
hera
py –
AV1
4 Lo
caliz
ed D
iseas
e at
Dia
gnos
isPa
thol
ogy:
Sta
ge I,
Hig
h Ri
sk (H
R) a
nd S
tage
II, I
R an
d St
age
II, II
I LR
or S
urgi
cal S
tage
I or
II /
Easy
Sur
gery
Nam
e of
Pat
ient
:
At a
dmis
sion
:
Wei
ght (
kg)
Heig
ht (c
m):
Body
sur
face
area
(m2 ):
Vinc
ristin
e an
d ac
tinom
ycin
are
give
n i.v
. pus
h.
Date
of B
irth:
Dose
Vin
cris
tine
(2.0
mg/
m2 (
max
2 m
g))
Dose
Act
inom
ycin
(45
μg/k
g (m
ax 2
mg)
)
Body
wei
ght <
12kg
?(If
‘yes
’ red
uce
dose
of b
oth
drug
s to
2/3
)
Date
of A
dmis
sion
:
Actin
omyc
in D
Vinc
ristin
e
WEE
KS
Date
Give
n:
Give
1st
dos
e Vi
ncris
tine
whe
n pe
rista
lsis
is re
-est
ablis
hed
and
no o
bvio
us s
urgi
cal p
robl
ems
1 ......
......
2 ......
......
34
5 ......
......
67
8 ......
......
910
11 ......
......
1213
14 ......
......
Wilm
s Tum
our
Post
-ope
rativ
e Ch
emot
hera
py –
AVD
14 –
Met
asta
tic D
iseas
e at
Dia
gnos
isLo
caliz
ed D
iseas
e at
Dia
gnos
is –
Path
olog
y: S
tage
II H
igh
Risk
, Sta
ge II
I IR
and
HR
or S
urgi
cal S
tage
III /
Diffi
cult
Surg
ery
Nam
e of
Pat
ient
:
At a
dmis
sion
:
Wei
ght (
kg)
Heig
ht (c
m):
Body
sur
face
area
(m2 ):
Vinc
ristin
e an
d ac
tinom
ycin
are
give
n i.v
. pus
h. D
oxor
ubic
in is
give
n in
2-
6 ho
urs.
Date
of B
irth:
Dose
Vin
cris
tine
(2.0
mg/
m2 (
max
2 m
g))
Dose
Act
inom
ycin
(45
μg/k
g (m
ax 2
mg)
)
Dose
Dox
orub
icin
(30
mg/
m2 )
Body
wei
ght <
12kg
?(If
‘yes
’ red
uce
dose
of b
oth
drug
s to
2/3
)
Date
of A
dmis
sion
:
Doxo
rubi
cin
Actin
omyc
in D
Vinc
ristin
e
WEE
K
Date
Give
n:
Give
1st
dos
e Vi
ncris
tine
whe
n pe
rista
lsis
is re
-est
ablis
hed
and
no o
bvio
us s
urgi
cal p
robl
ems.
1 ......
......
2 ......
......
34
5 ......
......
67
8 ......
......
910
11 ......
......
1213
14 ......
......
3534
OEP
PAH
odgk
in’s
Dise
ase
Nam
e of
Pat
ient
:
At a
dmis
sion
:
Wei
ght (
kg)
Heig
ht (c
m):
Body
sur
face
area
(m2 ):
Max
olon
(met
oclo
pram
ide)
10
mg
befo
re a
nd a
fter (
afte
rnoo
n) c
hem
othe
rapy
to re
duce
vom
iting
.Al
lopu
rinol
5 m
g/kg
tds
for 5
day
s to
pre
vent
tum
or ly
sis
synd
rom
e.
Date
of B
irth:
Vinc
ristin
e IV
day
1 -
-- (1
.5m
g/m
2 max
dos
e 2m
g)
Etop
osid
e IV
day
s 1,
2, 3
---
(100
mg/
m2 )
Pred
niso
lone
day
s 1-
5 --
- (4
0mg/
m2 )
Proc
arba
zine
days
1-1
4 --
- (1
00m
g/m
2 )
Doxo
rubi
cin
day
1 --
- (5
0mg/
m2 )
Date
of A
dmis
sion
:
Doxo
rubi
cin
Vinc
ristin
e
Etop
osid
e
Proc
arba
zine
(14d
ays)
Pred
niso
lone
(5 d
ays)
1W
EEKS
Date
Give
n:...
......
......
.....
......
......
......
.....
......
......
.....
......
......
......
..
1428
38
Size
Tum
our:
USS:
Pres
entin
g Co
mpl
aint
s:FN
A Da
te:
FNA
Resu
lt:
(Pre
sum
ed) D
iagn
osis
:
Flow
She
et: P
redn
isol
one
Pre-
Phas
e A
LL
Nam
e of
Pat
ient
:
Date
of B
irth:
Hosp
ital N
umbe
r:
Full
Bloo
d Co
unt:
Pred
niso
lone
dos
e gi
ven:
Bone
Mar
row
:
Intr
athe
cal
Met
hotr
ixat
e:
Pred
niso
lone
:
1-2
Year
s Ol
d -
8mg
2-3
Year
s Ol
d -
10m
g3+
Yea
rs O
ld -
12m
g
40m
g/m
2 /da
y in
2 d
ivide
d do
ses
Pred
niso
lone
– 4
0mg/
m2 /
x7da
ys
Wei
ght:
Surf
ace
Area
:
Haem
oglo
bin
Whi
te B
lood
Cou
nt
Neut
roph
ils
Plat
elet
s
Day
Wee
k
Date
Dosi
ng m
ay b
e st
arte
d gr
adua
lly fo
r hig
h pr
esen
ting
WBC
med
iast
inal
dis
ease
and
if v
ery
unw
ell
Mal
awi A
LL 3
Shee
t 1
12
34
56
7
PRED
NISO
LONE
PRE
-PHA
SE
3736
Flow
She
et: I
nduc
tion
ALL
Nam
e of
Pat
ient
:
Date
of B
irth:
Hosp
ital N
umbe
r:
Full
Bloo
d Co
unt:
Trea
tmen
t giv
en:
Bone
Mar
row
:
Intr
athe
cal
Met
hotr
exat
e:
Aspa
ragi
nase
:
Vinc
ristin
e:
Pred
niso
lone
:
Dose
Dose
Dose
Dose
1-2
Year
s Ol
d -
8mg
2-3
Year
s Ol
d -
10m
g3+
Yea
rs O
ld -
12m
g
6000
IU/m
2 per
dos
e (in
tram
uscu
lar)
Day
4 –
1st d
ose
1.5m
g/m
2 per
dos
e
40m
g/m
2 /d
ay in
2 d
ivide
d do
ses
then
wea
n ov
er 5
day
s
Wei
ght:
Surf
ace
Area
:
Haem
oglo
bin
Whi
te B
lood
Cou
nt
Neut
roph
ils
Plat
elet
s
Day
Wee
k
Date
Mal
awi A
LL 3
Shee
t 2
Indu
ctio
n
18
1522
23
45
Flow
She
et: C
ontin
uatio
n A
LL
Nam
e of
Pat
ient
:
Date
of B
irth:
Hosp
ital N
umbe
r:
Full
Bloo
d Co
unt:
Trea
tmen
t giv
en:
Intr
athe
cal
Met
hotr
exat
e:
6 M
erca
ptop
urin
e:
Vinc
ristin
e:
Pred
niso
lone
:
Cotr
imox
azol
e:
Dose
Dose
Dose
Dose
1-2
Year
s Ol
d -
8mg
2-3
Year
s Ol
d -
10m
g3+
Yea
rs O
ld -
12m
g
60m
g/m
2 /da
y -
in th
e ev
enin
g
1.5m
g/m
2 per
dos
e
40m
g/m
2 /d
ay in
2 d
ivide
d do
ses
then
wea
n ov
er 5
day
s
Daily
Wei
ght:
Surf
ace
Area
:
Haem
oglo
bin
Whi
te B
lood
Cou
nt
Neut
roph
ils
Plat
elet
s
Day
Wee
k
Mal
awi A
LL 3
Shee
t 4
5764
7178
1011
1213
Give
hou
r afte
r mea
l – n
ot w
ith m
ilk
May
nee
d to
adj
ust d
ose
afte
r blo
od c
ount
3938
Flow
She
et: I
nten
sific
atio
n A
LL
Nam
e of
Pat
ient
:
Date
of B
irth:
Hosp
ital N
umbe
r:
Full
Bloo
d Co
unt:
Trea
tmen
t giv
en:
Intr
athe
cal
Met
hotr
exat
e:
Doxo
rubi
cin:
Etop
osid
e:
Cyta
rabi
ne:
6 M
erca
ptop
urin
e:
Vinc
ristin
e:
Pred
niso
lone
:
Dose
Dose
Dose
Dose
Dose
Dose
Dose
1-2
Year
s Ol
d -
8mg
2-3
Year
s Ol
d -
10m
g3+
Yea
rs O
ld -
12m
g
45m
g/m
2 per
dos
e
100m
g/m
2 per
dos
e
100m
g/m
2 per
dos
e
60m
g/m
2 in
the
even
ing
1.5m
g/m
2 per
dos
e
40m
g/m
2 /d
ay in
2 d
ivide
d do
ses
for 5
day
s
Wei
ght:
Surf
ace
Area
:
Haem
oglo
bin
Whi
te B
lood
Cou
nt
Neut
roph
ils
Plat
elet
s
Dox
orub
icin
/ C
ispl
atin
Patie
nt N
ame:
At A
dmis
sion
:
Cisp
latin
Doxo
rubi
cin
Wei
ght
Heig
htBo
dy S
urfa
ce A
rea
Doxo
rubi
cin
......
... (5
0mg/
m2 ,
max
70m
g)Ci
spla
tin, d
ay 1
+2
(50m
g/m
2 , m
ax 7
0mg)
......
....
......
....
......
....
Age:
Date
of A
dmis
sion
:
1W
EEKS
Date
Give
n:
Size
Tum
our:
USS:
FNA
Date
:
Pres
entin
g Co
mpl
aint
s:
......
......
......
..
Ches
t X-R
ay:
FNA
Resu
lt:
(Pre
sum
ed) D
iagn
osis
:
......
......
......
..
2 ......
......
......
..
3 ......
......
......
.
......
......
......
..
4 ......
......
......
..
......
......
......
..
5 ......
......
......
..
6 ......
......
......
..
7 ......
......
......
..
......
......
......
..
Ost
eosa
rcom
a, H
epat
ocel
lula
r Car
cino
ma
(Neu
robl
asto
ma)
4140
Etop
osid
e / C
ispl
atin
/ Bl
eom
ycin
Patie
nt N
ame:
At A
dmis
sion
:
Cisp
latin
Bleo
myc
in
Etop
osid
e
Wei
ght
Heig
htBo
dy S
urfa
ce A
rea
Etop
osid
e da
y 1-
3 IV
; OR
1-5
po ..
... (1
00m
g/m
2 )Ci
spla
tin, d
ay 1
+2
.....
(50m
g/m
2 )Bl
eom
ycin
day
1 ..
... (1
5 IU
/m2 )
......
....
......
....
......
....
Age:
Date
of A
dmis
sion
:
0W
EEKS
Date
Give
n:
Size
Tum
our:
Alph
a Fe
topr
otei
n:
USS:
FNA
Date
:
Pres
entin
g Co
mpl
aint
s:
......
......
......
..
Ches
t X-R
ay:
FNA
Resu
lt:
(Pre
sum
ed) D
iagn
osis
:
......
......
......
..
12
3 ......
......
......
..
......
......
......
..
45
6 ......
......
......
..
......
......
......
..
Ger
m C
ell P
roto
col
Kapo
si S
arco
ma
Vin
cris
tine,
Ble
omyc
in a
nd E
topo
side
Patie
nt N
ame:
At A
dmis
sion
:
Etop
osid
e
Vinc
ristin
e
Bleo
myc
in
Wei
ght
Heig
htBo
dy S
urfa
ce A
rea
Etop
osid
e 1-
3 IV
1 h
r inf
usio
n, o
r 1-5
day
s po
.....
(100
mg/
m2 )
Vinc
ristin
e da
y 1,
IV b
olus
(1.5
mg/
m2 ,
max
2 mg)
Bleo
myc
in d
ay 1
IV 1
5 m
in in
fusi
on 1
5 IU
/m2
......
....
......
....
......
....
Age:
Date
of A
dmis
sion
:
12
34
56
78
910
WEE
KS
Date
Give
n:
Size
Sen
tinel
Nod
e:
Lans
ky S
core
:
Pres
entin
g Co
mpl
aint
s:
USS:
FNA:
......
.....
......
.....
Pres
umed
) Dia
gnos
is:
Ches
t X-R
ay:
Date
:
......
.....
......
.....
......
.....
......
.....
Resu
lt:
......
.....
......
.....
......
.....
......
.....
......
.....
......
.....
......
.....
......
.....
......
.....
......
.....
......
.....
......
.....
HIV
pos
Y/N
CD4
coun
t...
On A
RTs
Y/N
If ‘Y
es’ s
ince
whe
n?
4342
Low
Gra
de G
liom
a –
Vin
cris
tine
and
Car
bopl
atin
Freq
uenc
y.
The
Initi
al p
hase
con
sist
of w
eekl
y ch
emot
hera
py fo
r 10
wee
ks w
ith V
incr
istin
e an
d 3
wee
kly
with
Car
bopl
atin
.Th
e ne
xt p
hase
last
s fo
r 10
cour
ses
of V
incr
istin
e an
d Ca
rbop
latin
at a
ppro
ximat
ely
28 d
ay in
terv
als,
whe
n th
e ne
utro
phils
are
>1x
109
/l an
d pl
atel
ets
> 1
00 x
109
/l.
Drug
: Vi
ncris
tine
Dosa
ge:
1.5
mg/
m2 f
or c
hild
ren
>10
kg
(max
tota
l dos
e 2m
g)
Drug
: Ca
rbop
latin
Dosa
ge:
600
mg/
m2 f
or c
hild
ren
>10
kg
Both
: Re
com
men
datio
ns fo
r chi
ldre
n un
der 1
0kg
in w
eigh
t:
Less
than
6 m
onth
s of
age
: 50%
of c
alcu
late
d do
se b
y bo
dy s
urfa
ce a
rea
6
mon
ths
to 1
yea
r of a
ge: 7
5% o
f cal
cula
ted
dose
by
body
sur
face
are
a
Over
1 y
ear o
f age
: 100
% o
f cal
cula
ted
dose
by
body
sur
face
are
a
VCR/
Carb
o
Wee
k 1
VCR
Wee
k 2
VCR
Wee
k 3
VCR/
Carb
o
Wee
k 4
VCR
Wee
k 5
VCR
Wee
k 6
VCR/
Carb
o
Wee
k 7
VCR
Wee
k 8
VCR
Wee
k 9
VCR/
Carb
o
Wee
k 10
VCR/
Carb
o
Wee
k 14
VCR/
Carb
o
Wee
k 18
VCR/
Carb
o
Wee
k 22
VCR/
Carb
o
Wee
k 26
VCR/
Carb
o
Wee
k 30
VCR/
Carb
o
Wee
k 34
VCR/
Carb
o
Wee
k 38
VCR/
Carb
o
Wee
k 42
VCR/
Carb
o
Wee
k 46
VCR/
Carb
o
Wee
k 50
Retin
obla
stom
a –
Che
mot
hera
py –
JOE
Eye
exam
inat
ion
Date
Che
mot
hera
py G
iven
Cour
se n
umbe
r
MPS
/PCV
Seru
m c
reat
inin
e
HB WBC
/Neu
t
Plts
Any
toxic
ity
Carb
opla
tin D
ose
600
mg/
m2 f
or c
hild
ren
>10
kg
Reco
mm
enda
tions
for c
hild
ren
unde
r 10k
g in
wei
ght:
Less
than
6 m
onth
s of
age
: 50%
of c
alcu
late
d do
se b
y BS
A6
mon
ths
to 1
yea
r of a
ge: 7
5% o
f cal
cula
ted
dose
by
BSA
Over
1 y
ear o
f age
: 100
% o
f cal
cula
ted
dose
by
BSA
Etop
osid
e Do
se30
0 m
g/m
2 for
chi
ldre
n >
10 k
gRe
com
men
datio
ns fo
r chi
ldre
n un
der 1
0kg
in w
eigh
t:Le
ss th
an 6
mon
ths
of a
ge: 5
0% o
f cal
cula
ted
dose
by
BSA
6 m
onth
s to
1 y
ear o
f age
: 75%
of c
alcu
late
d do
se b
y BS
AOv
er 1
yea
r of a
ge: 1
00%
of c
alcu
late
d do
se b
y BS
A
Vinc
ristin
e Do
se1.
5 m
g/m
2 for
chi
ldre
n >
10 k
gRe
com
men
datio
ns fo
r chi
ldre
n un
der 1
0kg
in w
eigh
t:Le
ss th
an 6
mon
ths
of a
ge: 5
0% o
f cal
cula
ted
dose
by
BSA
6 m
onth
s to
1 y
ear o
f age
: 75%
of c
alcu
late
d do
se b
y BS
AOv
er 1
yea
r of a
ge: 1
00%
of c
alcu
late
d do
se b
y BS
A
PRE
12
34
56
4544
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