Med Pediatr Oncol 2002;38:455–456

Letter to the Editor: Tumor Thrombus in a Child With PrimitiveNeuroectodermal Tumor

To the Editor: Up to 15% of adult patients withclinically overt cancer experience venous thromboembo-lism (VTE), but it is rare in children [1–10]. The highestincidence is in Wilms tumor [3,4] and it is seen rarely inpatients with hepatoblastoma, hepatocellular carcinoma,neuroblastoma, rhabdomyosarcoma, malignant periphe-ral nerve sheath tumors, and chondrosarcomas [2–9]. Ourexperience with a child with peripheral primitive neuro-ectodermal tumor (pPNET) who developed a tumorthrombus is therefore reported here.

She was a 12-year-old girl who was admitted to a statehospital with dyspnea and swelling of her left leg. Imag-ing investigations revealed intravascular thrombosis inthe vena cava inferior and left femoral and iliac veins.The procoagulant activity was investigated. AntithrombinIII, protein C, protein S, factor V levels were evaluated asnormal and anticardiolipin antibodies were also negative.

Her clinical findings progressed and abdominal distentiondeveloped despite low molecular weight heparin for1.5 years. A biopsy of the thrombus in the femoral veinthen showed a primitive neuroectodermal tumor. She wasthen referred to our division for further treatment.

Her disease progressed, hepatomegaly, ascites, andedema of the left leg (Fig. 1), thrombosis of the iliac andfemoral veins, inferior vena cava inferior, right atrium,and ascites were identified. There was a soft tissue mass,presumably the primary tumor enveloping the proximalportion of the left femoral vein. CT of the thorax revealedmetastatic nodules in both lungs and a right pleuraleffusion. Her course thereafter was stormy and complexand she died of progressive disease and heart failure afteronly temporary improvement with polychemotherapy.This consisted of vincristine (2 mg/m2), doxorobicin(75 mg/m2), cyclophosphamide (1.2 gr/m2) alternatingwith ifosfamide (1.8 gr/m2� 5 day), and etoposide(100 mg/m2� 5 day) every 3 weeks.

The occurrence of VTE in patients with pPNETs,Ewing sarcoma family tumors (ESFT), has not been re-ported in the literature previously. This case is alsoparticular with its long history of VTE until the diagno-sis of a malignancy was made. We can’t be sure if thethrombus was composed of tumor from the beginning orwhether it was superimposed on a bland thrombus as partof a paraneoplastic syndrome.

ACKNOWLEDGMENT

We thank Prof. Dr. Misten Demiryont and Assoc. Prof.Dr. Bilge Bilgic from the Department of Pathology,Istanbul School of Medicine, University of Istanbul forpathological evaluation.

Omer Gorgun, MD*Rejin Kebudi, MD

Inci Ayan, MD

H. Haldun Emiroglu, MD

Division of Pediatric OncologyOncology Institute

University of IstanbulIstanbul, Turkey

Fig. 1. Edema of the left leg and large venous collaterals in theabdominal wall are evident.

——————*Correspondence to: Omer Gorgun, Division of Pediatric Oncology,Oncology Institute, University of Istanbul, Capa, 34390, Istanbul,Turkey. E-mail: omergorgun@hotmail.com

� 2002 Wiley-Liss, Inc.DOI 10.1002/mpo.10065

REFERENCES

1. Sgarabotto D, Prandoni P, Stefani PM, et al. Prevalence andpatterns of symptomatic thromboembolism in oncohematology.Hematologica 1998;83:442–446.

2. Akyuz C, Hazar V, Sevinir B, et al. Successful treatment ofintracaval and atrial extension of rhabdomyosarcoma withoutsurgical excision: an unusual presentation. Med Pediatr Oncol1999;32:313–314.

3. Martinez-Ibanez V, Sanchez de Toledo J, De Diego M, et al.Wilms’ tumours with intracaval involvement. Med Pediatr Oncol1996;26:268–271.

4. Mushtaq I, Carachi R, Roy G, et al. Childhood renaltumours with intravascular extension. Br J Urol 1996;78:772–776.

5. Gurgey A, Buyukpamukcu M, Baskut C, et al. Portal veinthrombosis in association with factor V Leiden mutation in a

patient with hepatocellular carcinoma. Med Pediatr Oncol 1997;29:224–225.

6. Hoekstra HJ, Pras B, Mooyaart EL, et al. Pelvic girdle chon-drosarcoma and inferior vena cava thrombosis. Eur J Surg Oncol1997;23:577.

7. Rodriguez-Rubio FI, Abad JI, Rabago G, et al. Long survival of apatient with paratesticular rhabdomyosarcoma with inferior venacava involvement. Urology 1997;50:978–979.

8. Ramanathan RC, Shore DF, Thomas JM. Intracardiac extension oftumour thrombus from a soft tissue sarcoma. Eur J Oncol 1998;24:145–146.

9. Ohtsuka Y, Takahashi H, Ohnuma N, et al. Detection of tumorthrombus in children using color Doppler ultrasonography.J Pediatr Surg 1997;32(10):1507–1510.

10. Ritchey ML, Kelalis PP, Breslow N, et al. Intracaval and atrialinvolved with nephroblastoma: review of National Wilms’ TumorStudy 3. J Urol 1988;140:1113–1118.

456 Gorgun et al.