Younus R, Khan A, Syed S.
Giant Askin Tumor: A diagnosis and therapeutic Challenge.
J Pak Med Assoc Jan ;52(2):82-5.

A 17 year old female presented to the surgical department, Jinnah Postgraduate Medical Center (JPMC), Karachi with an eight month history of right anterior chest wall mass associated with fever. There was no remarkable past history. Physical examination revealed a 10x10 cm rounded, fixed mass on the right anterior chest wall extending into the supraclavicular region. No other abnormality was detected. A chest x--ray showed opacity in the right apical region. A computed tomographic scan of the chest revealed large lobulated soft tissue density masses in the right upper chest and surrounding the medial end of the right clavicle extending into the root of the neck and the superior mediastinum. No bony erosion or lung metastasis was seen. Bone scan and other studies revealed no evidence of distant metastasis. Ultrasound guided FNAC revealed highly cellular monomorphic population of small, round to oval blue cells with vesicular nucleus, scant cytoplasm and indistinct cell outlines. For further distinct diagnosis, excision biopsy was performed, which showed a malignant, lobular tumor with characteristic Homer Wright rosettes. No glycogen was seen (Figure 1). For further confirmation, immunocytochemistry and electron microscopy were performed. Immunocytochemistry showed positive staining for cytokeratin, chromogranin, neuron specific enolase, MIC 2, S 100. Whereas Pan-B, Pan-T, leukocyte common antigen and desmin were negative. Electron microscopy revealed dense core neurosecretory granules. Diagnosis of Askin tumor was made on these results. Multi drug chemotherapy (four courses of vincristine, doxorubicin, cyclophosphamide and decarbazine) were administered at the oncology department, JPMC, Karachi, in the hope to down grade the tumor but the patient had a progressive disease and the tumor continued to grow rapidly and achieved an enormous size of 25x20x15cm with fluctuating areas, with pointing, threatening imminent rupture. Therefore, local control, partial excision of the mass was done followed by radiation therapy. 50 Gy of irradiation in divided doses was given over a month at the Karachi Institute of Radiotherapy and Nuclear Medicine (KIRAN). Repeat bone scan showed multiple bone metastasis. Brain and liver scans were negative for metastasis. Repeat CT scan showed tumor extension into the axilla but no metastasis to the lung was found. Palliative chemotherapy (two courses of cisplatin, etoposide and bleomycine) were administered, but the tumor progressed and invaded the skin causing multiple skin ulceration and bleeding. The patient died of her disease after 18 months of initial diagnosis.

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