Unilateral pulmonary agenesis with AM. Philadelphia, W.B. Sunders Com- esophageal atressia and distal pany, 1996, pp 1199. tracheoesophageal fistula Report of two 6. Herbst JJ. The esophagus In Nelson Text cases J Pediatr Surg 1989; 24: 1084-1086. Book of Pediatrics, 15th edn. Eds. Behrman RE, Khegman RM, Arvind AM. 4. Sarin YK. Esophageal atresia and Philadelphia, W.B. Sounders Company, tracheoesophageal fistula with right pul- 1996, pp 1052-1053. monary agenesis Indian Pediatr 1996; 33: 595-597. 7. Mackinlay GA. Neonatal surgery In: ForFar and Arneils Text Book of Pediatrics, 5. Stern R, Congenital anomalies. In: 4th edn. Eds. Campbell AGM, Nelson Text Book of Pediatrics, 15th edn. Mcintosh N. Edinburgh, Churchill Eds. Behrman RE, Kliegman RM, Arvin Livingstone, 1992, pp 1850-1852. Paravertebral Extraosseous have been extremely rare To the best of Ewing's Sarcoma our knowledge EES located in paraverte- bral area has not been reported in Indian T.P. Yadav literature We report one such case R.P.Singh V.K. Gupta Case Report N.K. Chaturvedi* C. Vittal Prasad+ A 12-year-old male child was admitted with the complaints of progressively in- creasing dull aching pain in the upper back Extra Osseous Ewing's Sarcoma (EES) and right shoulder, radiating to the right has been considered a distinct clinico- hand since last two months and progres- pathological entity despite its striking ul- sive weakness of his right upper limb since trastructural similarity to Ewing's Sarcoma last one month Around the same time he of Bone (ESB) and same translocation in- noticed a painless swelling in the right side volving band ql2 of chromosome 22(1,2) of neck which was slowly increasing in Today more than 150 cases of EES in differ- size There was history of loss of appetite ent locations have been described, for and weight, however, he did not suffer example, larynx, scalp, nasal fossa, neck, from headache, vomiting, visual distur- chest wall, lung, perineum, finger, arm, lip bance, dysphagia, dyspnea or disturbance and toe(3) Paravertebral location of EES of gait He was a thin built pale child Examination of central nervous system re- From the Departments of Pediatiics, Pathology* and vealed weakness (power 3/5) and hypoto- Radiology*, Dr. Ram Manohai Lohia Hospital nia of right upper limb The biceps, the tri- New Delhi ceps, and supinator reflexes in right upper Reprint Requests: Dr. T.P. Yadav, 16 LF limb were elicitable with difficulty The Tansen Marg, Bengali Market, New Delhi 110 001. thenar and hypothenar eminances were Manuscript Received July 19,1997 significantly wasted and there was no sen- Initial review completed October 13,1997, sory loss Rest of the systemic examination Revision Accepted November 22,1997 did not reveal any abnormality There was 557 CASE REPORTS restriction of anterior flexion and lateral bending of neck. A soft noncystic, nontender swelling was palpable in the right supraclavicular region measuring 2 x 2 cm. It was fixed to the underlying structures. The skin over the swelling was freely mobile. There were no signs of in- flammation. No bruit was heard over the swelling. Investigation revealed a haemoglobin of 12 g/dl, total leukocyte count of 9500 cells/ cu mm with 63% polymorphs and 32% lymphocytes and ESR of 65 mm in the first hour. The urine examination for albumin, sugar, VMA and HVA did not reveal any abnormality. There was no reaction to 5TU of PPD. The skiagram of chest and cervical spine revealed a soft tissue shadow on right side in the apical area without any area of bone destruction. The ultrasonographic examination of abdomen did not reveal any abnormality. The examination of bone marrow aspirate clear cytoplasm with occasional mitosis at also did not show any abnormal cells. The places (Fig. 2). Fibrous band was seen CT scan of neck and upper thorax showed a dividing tumor tissue in irregular planes. large soft tissue mass with sharp margin and Vascularization was prominent and occa- some degree of contrast enhancement inside sional pseudorossettes were seen. Reaction the mass, anterolateral to the seventh to PAS was highly positive. The picture cervical vertebral body, encroaching was highly suggestive of Ewing's sarcoma. through the thoracic inlet and extending A diagnosis of Extra Osseous upto the body of second thoracic vertebra. Paravertebral Ewing's Sarcoma with There was destruction of right transverse , intraspinal extension with no lymph node process of seventh cervical and second tho- involvement or distant metastasis (G3 T3 racic vertebrae. The mass was extending No Mo), i.e., stage IV of TNMG classifica- into the intraspinal area pressing the spinal tion was made. cord at the level of seventh cervical vertebra. Some amount of tracheal shift toward the left The patient was given chemotherapy was also seen (Fig. 1). MRI of cervical and followed by radiotherapy The chemo- upper thoracic area substantiated the therapy consisted of VACA regimen which finding of CT scan. comprised of injection Vincristine (1 mg/ dose), Injection Cyclophosphamide (200 Fine needle aspiration cytology and mg/dose) given on day 1 of every week histopathology of the mass in the neck re- and injection Adriamycin (30 mg/dose) vealed tissue composed of densely packed and Injection Actinomycin-D (0.5 mg/ small cells with round nuclei, scanty to dose) given on day one of alternate week. 558 INDIAN PEDIATRICS VOLUME 35-JUNE 1998 The total duration of this regimen was 30 was started again. After receiving two weeks after which 20 sittings of radio- cycles, he did not report back. therapy were given in dose of 20 cGY per sitting (total dose 400 cGY). The patient tol- Discussion erated chemotherapy and radiotherapy well without any untoward happening. EES like ESB is a poorly differentiated, The patient became asymptomatic, the highly malignant round cell tumor without swelling was not visible and features of cellular structural differentiation, with cord compression disappeared completely. early and high degree of metastasis and has Repeat CT showed a decrease in the soft an aggressive clinical behavior with high tissue mass. After a five month symptom rate of local recurrence and distant free period, he developed fullness in right metastasis(l-4). It has been reported to supraclavicular region again. CT scan and originate from different sites, namely, MRI repeated, revealed a lobulated soft tis- trunk (32%), extremity (26%), head and sue mass extending from seventh cervical neck (18%), retroperitonium and pelvis vertebra to the third thoracic vertebra and (16%) and others (10%)(4,5). Paravertebral extending intraspinally at level of sixth and origin of EES has been extremely rare. To seventh cervical vertebrae. The transverse the best of our knowledge no such case has process and upper half of lamina of C 6, 7 been reported from Indian literature and and T1 were destroyed partially by mass only few cases have been reported world lesion. The chemotherapy (VACA regimen) wide(6,7). 559 CASE REPORTS The clinical features in such cases are Paravertebral EES carries a poor prog- mainly local swelling, radicular pain and nosis and may require more extensive field features of cord compression. In such pa- radiation and more intensive chemo- tients other causes of compression of spinal therapy to achieve better local and systemic cord/nerve root need to be ruled out, for tumor control(8). example, benign extra and intradural tu- REFERENCES mor like neurentric cyst, lipoma, angioma, meningioma, neurofibroma, dermoid cyst, 1. Tefft M, Vawter GF, Mitus A. abscess, acute leukemia, lymphoma, Paravertebral "round cell" tumors in chil- metastasis, malignant tumor of vertebrae dren. 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Raney RB, Asmar L, Newton WA, Gehan gest the possibility of these belonging to the EA, Maurer HM. Extraosseous Ewing's same group of peripheral primitive Sarcoma (EOE) in childhood: The Inter- neuroectodermal tumors (6,7). group Rhabdomyosarcoma study experi- ence. Proc Ann Meet Am Soc Clin Oncol The aim of treatment in such patients is 1994; 13:1418. complete removal/destruction of tumor 6. Ambros IM, Ambros PF, Strehl S, Kovar tissue, complete restoration of function of H, Gadner H, Salzn-Kuntoschil M. MIC-2 part without recurrence. Multi-modal treat- is a specific marker for Ewing's Sarcoma ment strategies including surgery, radio- and Peripheral Primitive Neuroectoder- therapy and chemotherapy have been the mal tumors. Cancer 1991; 67:1886-1893. main-stay of treatment depending on the 7. Ladany M, Heinemann FS, Huvos AG, stage and site of tumor with variable Hanumantha Rao P, Chen Q and Jhanwar response and relapse rates(3,4,5,8). Our SC. Neural differentiation in small patient was subjected to chemotherapy and round cell tumor of bone and soft tissue with the translocation t(ll;22) (q 24; ql2): radiotherapy. He had complete neurologi- An immunohistochemical study of 11 cal recovery but local recurrence occurred cases.