Rare case of blastemal predominant adult Wilms’ tumor with skeletal metastasis case report and brief review of literature
A 19-year-old female presented with severe low backache and colicky left loin pain of 3 months and progressive weakness of 15 days duration. The pain was radiating to the groin and left leg. There was neither any history of prior abdominal surgery nor any history of trauma. Further there was no complaint of hematuria, pyuria, or calculuria. Menstrual cycle was regular.\nPer abdomen examination revealed tenderness in left loin and spine.\nRoutine laboratory investigations including a full blood count, chest X-ray, and renal function tests were normal. Magnetic resonance image (MRI) of lumbosacral spine was reported as spinal metastasis with right renal mass [Figure and ]. Computed tomography (CT) guided aspiration of spinal lesion was performed. The cytology smears were cellular and showed mildly pleomorphic round to oval cells attempting microacinar and papillary structures. Cytologically possibilities of blastemal predominant adult Wilms’ and Ewing's sarcoma/primitive neuroectodermal tumor (PNET) were considered.\nThe patient underwent right radical nephrectomy. The nephrectomy specimen showed a large tumor of 15 × 10 × 6 cm with areas of hemorrhage and necrosis almost replacing the normal kidney.\nMicroscopically, the tumor comprised of monomorphous cells present as nests, islands, and sheets with intervening tumor necrosis and lymphoid collections. At areas, the tumor cells were seen around the blood vessels []. Ureter was free of tumor. The tumor cell on treatment with periodic acid schiff (PAS) agent did not show significant positivity, which favored the diagnosis of Wilms’ tumor rather than Ewing's/PNET. Immunohistochemistry was performed with a panel of antibodies like vimentin, cytokeratin, CD45, synaptophysin, chromogranin, s-100, neuron-specific enolase (NSE), CD117, CD99. The tumor exhibited positivity for s-100, CD117, and NSE focally []. Rest of the markers was negative. Along with kidney lymph nodes around renal hilum and inferior vena cava were sent for histopathological examination. There was no evidence of metastasis in all 10 retrieved lymph nodes.\nFurther she received radiotherapy and six cycles of chemotherapy. Twelfth day after surgery, she was put on combined chemoradiotherapy. She received a total of 3060 cGy to spine over 17 fractions (180 cGy/day) and additional boost of 1080 cGy to tumor bed. She was offered pulsed intensive regimen with three drugs over 24 weeks. The chemotherapy schedule was dactinomycin 45 μg/kg/day at weeks 0, 6, 12, 18, and 24, doxorubicin 45 mg/m2/day at third and ninth week and then 30 mg/m2/day at weeks 15 and 21.Vincristine was given 1.5 mg/m2/day at weeks 12, 15, 18, 21, and 24.\nCT of the chest and abdomen at 3 months following surgery has not shown any evidence of recurrence of tumor or metastasis in other viscera. After completion of chemotherapy schedule, patient was lost to follow-up and presented with weakness of lower limbs and backache of 15 days duration one-and-half-year after surgical therapy. MRI of lumbosacral spine showed D11 and D12 extradural metastasis. There was no evidence of tumor bed recurrence. The patient underwent D10, D11, D12 laminectomy and excision of mass lesion histopathologically which exhibited similar morphology as that of the renal lesion.\nThere was no evidence of neurological improvement in the postoperative period and she was started on four drug regimen with inclusion of cyclophosphamide (J regimen of NWTSG-4), but patient refused to continue treatment after 4 weeks.