Dr.E.ThirulogachandarPROF.S.TITO’S UNIT

Karthik a 15 yr male admitted withc/o swelling of Rt.chest-1 week breathlessness -1 week dry cough- 1week fever -4 days

HOPI;H/o Swelling Rt.upper chest -1 week Onset was spontaneous, progressively

increased to face over a period of 3 weeks after admission.

No h/o trauma.H/o breathlessness -1 week grade 3 on onset

progressed to grade 4 after 3 weeks. H/o dry cough -1 week.H/o fever -4 days low grade.Noh/o chestpain, syncope, haemoptysis, hoarseness of voice

Past history:nil relevant

Personal history :mixed diet

Family history : nil significant illness

General examn: pt conscious, oriented, febrile comfortable, pallor+, not jaundiced, no cyanosis, no clubbing, no lymphadenopathy.Pulse:82/mt,BP 96/72 mmHg,RR-17/mt

Inspection&palpation: Rt. Upper chest is swollen with fullness

supraclavicular region, Trachea in midline, AI in normal place, movements diminished Rt.side, vocal fremitus increased in Rt.infraclavicular,axillary&suprascapular regions Percussion :impaired resonance present in

Rt.infraclavicular,axillary and suprascapular regions.

Auscultation :Bilateral air entry prtesent NVBS+bilaterally Br.breath sounds present in

Rt.infraclavicular,axillary suprascapular and areas.

VR increased in same regionsCVS: S1,S2 heard,no murmursAbd:soft,no organomegalyCNS:no FND.

MASS lesion Rt. Upper lobe-?cause

CBC-Hb-8.6 BLOOD-SUGAR-96

TC-7400 UREA-18

DC-P72L27E3 CREATININE-0.7

ESR-12/24 Na-134,K-3.9

PCV-24% LFT-WNL

PLT-1.OLAC

MCV-72.5

MCH-27.4

MCHC-30.1

RBC 3.0 MILLIONS

URINE-ALB-nil

sug-nil

dep-1-4pc/hpf

Pleural fluid Analysis: colour-milky white Gram’s stain&AFB- neg culture-neg Cholesterol-214mg/dl TGL-2800mg/dlUSG Abd;NORMAL WITH Lt.pleural effusion

CT guided FNAC-Necrotic mass with inflammation

Biopsy-skeletal muscle bundles with blood clot-non diagnostic

β HCG-neg α-FP-neg LDH-neg Ab to Ach receptor-not done

CTS OPINION:As he had massive pleural effusion-chylothorax with respiratory distress Lt.ICD was placed chyle was drained.

about 200 ml of chyle was draining daily.

In view of diagnostic dilemma -5 weeks and GC deterioration of the pt, he was sent to CTS Dept,GGH,Chennai .

CT-guided biopsy repeated-nondiagnostic Planned open biopsy and deferred. Finally median sternotomy and whole

tumour was excised and sent for HPE.

Thymoma –stage B 1

CHYLOTHORAX: 1.Malignancy (50%) lymphoma(3/4),Br.car 2.trauma,surgery-25% 3.Miscellaneous- sarcoidosis,ccf lymphangiomatosis, yellow nail syndrome, kaposi’s sarcoma, post RT, Tb,filariasis,histoplasmosis,cirrhosis 4.Idiopathic-neonatal

Conservative 50% resolve Octreotide Cessation of oral intake Total parenteral nutritionSURGICAL TREATMENT: Thoracic duct ligation Pleurodesis Pleuro peritoneal shunt pleurectomySURGICAL INDICATION:>1litre/day for 2 days or persistent leak for

2 weeks, nutritional,metabolic complications, post oesophagectomy status

Treatment of chylothoraxMEDICAL

anterioranterior

Anterior

Posterior-Des.aorta,azygosvein,nodes,ganglia

Middle-Heart,tachea,bronchus,nodes,vessels

THYMUS,FAT,NODES

the functional unit of the thymus gland is the Lobule◦ composed of a cortex & medulla◦ epithelial cells◦ lymphocytes

thymomas are, by definition, primary tumors of benign-appearing thymic epithelial cells

Separated into three histologic categories— 1. Thymoma. 2. Thymic carcinoma. 3.Neuroendocrine tumor.

Given this definition, most thymomas are “benign”◦ the majority behave in an indolent fashion

◦ however, they can invade and metastasize (uncommon)

◦ when a thymoma is contained within the thymic capsule, it is referred to as “benign” or “noninvasive”

◦ when it penetrates through the capsule, it is referred to as “malignant” or “invasive”

Represent 20% of all mediastinal neoplasms in adults

Most common anterior mediastinal neoplasm in adults (less common in children)

incidence of thymoma is 0.15 cases per 100,000

Age range is generally 40-60 Equal prevalence on gender. No known risk factors (+/- myasthenia gravis) Thymic carcinoma represents less than 1% of

thymic malignancies.

95% in anterior mediastinum. Neck. Left hilar region. Within lung parenchynma. Anterior cardiophrenic angle.

Most important gross feature — The presence or absence of encapsulation of tumor and the gross invasion into adjacent structure.

The invasion present — The thymoma must be considered malignant lesion regardless the microscopic appearance.

Extensive spread. Incidence of distal metastasis is 3%.

Stage I: Intact capsule or growth within the capsule Stage II: Invasion into surrounding fatty tissue or

mediastinal plura◦ Stage IIA: microscopic invasion through the capsule◦ Stage IIB: macroscopic and microscopic invasion through

the capsule Stage III: Macroscopic invasion into neighboring organs

(pericardium, great vessels, lung) Stage IVA: Pleural or pericardial dissemination Stage IVB: Lymphogenous or hematogenous metastases

Masaoka et al. Cancer 1981;48:2484-2492

40-60 yrs. Sex distribution — Equal. - asymptomatic in 1/3 of pts - chest pain, breathlessness, cough, SVC syndrome, paralysis of hemidiaphragm -hoarseness, weight loss, fatigue, fever,If ass. With MG-Mc in women

Fatiguablity,ptosis,diplopia,dysphagia, weakness

Standard posteroanterior and lateral chest radiographies.

CXR—Smooth or lobulated mas, right side the silhouette sign present, left side the sign abscent.

Calcification — 10%. CT—Delineate the extent of mass,

cannot not differentiating benign and malignant, assessing intrathoracic spread of an invasive thymoma.

Ach receptor Ab

Unnecessary for a suspected locally symptomatic thymoma, because the capsule of tumor may be violated by invasive procedure.

Only distinguish the tumor from the other malignant tumour. It is to establish the diagnosis before making decision of therapy.

Fine needle biopsy by CT or sono-guide. Extend substernal mediastinoscopy. VATS.

Stage-Directed◦ Stage I: Surgical resection only◦ Stage II: Surgical resection +/- adjuvant

radiotherapy◦ Stage III: Surgery with adjuvant radiotherapy or

neoadjuvant chemotherapy, surgery, and adjuvant radiotherapy

◦ Stage IV: Chemoradiotherapy without surgical resection

Cisplatin remains cornerstone of therapy “CODE” regimen: neoadjuvant cisplatin,

epirubicin/doxorubicin, etoposide. Improved survival in pts with stage III and IV dz when compared with historic controls.

Somatostatin or somatostatin analogs with prednisolone have been reported to induce both PR and CR in pts with refractory disease

Thymoma is associated with a wide variety of paraneoplastic syndromes

Mechanism is unknown Theory:

◦ medullary/dendritic cell areas are required for induction of tolerance.

◦ If these areas are abnormal, positively selected autoreactive CD4+ T-cells may escape surveillance and generate autoantibody-producing B-cells

Most commonly associated disease. 30-50%of patient with thymoma

associate with MG. Only 10-15% patient with MG have

thymoma. Little effect on local presention, clinical

behavior, prognosis.

In the Johns Hopkins series 10%of pts with thymoma had PRCA, 2nd most common paraneoplastic syndrome

Antigen has not been determined Usually responds to rx with corticosteroids

Def: thymoma, hypogammaglobulinemia, defects in cell-mediated immunity (low or absent B cells, CD4 lymphopenia, and inverted CD4:CD8 ratio

Develops in 3-6% of pts with thymoma Most develop syndrome within six years of

dx of thymoma Immunologic abnormalities do not correct

with corticosteroids or thymectomy

Tarr et al. Medicine. Vol 80 No 2 March 2001

Recurrent sinopulmonary infections Mucocutaneous candidiasis Encapsulated organisms CMV, HSV, VZV PCP MTB

Tarr et al. Medicine. Vol 80 No 2 March 2001

THANK YOU

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