Question
A 3-year-old boy presents with a 7 cm, solitary abdominal mass which is surgically removed. Grossly, the mass is well-circumscribed, soft, and tan. A representative section is shown here.What is the diagnosis?
A. Follicular lymphoma.
B. Burkitt lymphoma.
C. Wilms tumor.
D. Neuroblastoma.
Show Answer
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Correct Answer » C
Explanation
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Ans:C.)Wilm’s Tumor.
Wilm’s Tumor
- Description: 5-10% are bilateral, synchronous or metachronous, earlier age of onset, germline mutation
- Clinical presentation: large abdominal mass, hematuria, pain abdomen after trauma, intestinal obstruction, and hypertention, lung metastases at the time of primary diagnosis
- Adverse Prognosis: anaplastic histology, loss of genetic material on chromosomes 11q and 16q, and gain of chromosome 1q
- Associated with ↑risk of second primary tumors (bone and soft-tissue sarcomas, leukemia, lymphomas and breast cancers)
- Prognosis: most patients can expect to be cured of their malignancy; 5% anaplastic, TP53 mutations and resistance to chemotherapy
- Syndromes at an Increase Risk for Development WAGR, Denys-Drash and Beckwith-Wiedemann
- Morphology: large, solitary, well-circumscribed mass; C/S (soft, homogenous, tan to grey with occasional foci of hemorrhage, cyst formation, and necrosis; 10% bilateral or multicentric
- Microscopic Appearance :classic triphasic combination of blastemal, stromal, and epithelial cell types- blastemal → sheets of small blue cells epithelial → abortive tubules or glomeruli stromal → fibrocytic or myxoid
