Answer Choices 1 Wilm's Tumor 2 Neuroblastoma 3 Nephroblastomatosis 4 Renal Cell Carcinoma 5 Mesoblastic Nephroma Wilm's tumor
Explanation The clinical picture is suggestive of Wilm's Tumor. It accounts for most renal tumors in childhood during the first 5 years of life. It affects both sexes equally. It is a solitary growth that affects either part of the kidneys. There are congenital anomalies associated with it, most commonly the GUT anomalies, hemihypertrophy, sporadic aniridia, and mental retardation. It is commonly manifested by an abdominal mass that is described as generally smooth, firm and rarely crosses the midline, and it causes abdominal pain and vomiting. Hypertension is seen in 60% of the patients either due to elaboration of renin by the tumor cells or due to compression of the renal vasculature by the tumor. Hematuria is also uncommon and mostly microscopic. CT scan confirms the diagnosis which will show an intrarenal tumor, therefore ruling out Neuroblastoma. Treatment is by surgical removal. Chemotherapy is indicated post- operatively for the residual tumor. Neuroblastoma is a malignancy of the neural crest. It is the most common solid tumor in children outside the CNS. It is slightly more common in males and whites and median age of diagnosis is 2 years old. It arises mostly in the abdomen either in the adrenal gland or retroperitoneal sympathetic ganglia followed by the thoracic area mostly seen in the posterior mediastinum. Other sites are the head, neck, and epidural area. Tumors in the head and neck region are sometimes associated with Horner's Syndrome (Mioisis, Ptosis, Anhidrosis, and Enophthalmos). Diagnosis is by CT scan or MRI but pathologic diagnosis is made by biopsy. Tumor markers such as VMA and HVA (Homovanillic Acid) help confirm the diagnosis. Treatment is surgery, chemotherapy, and radiation depending on the stage of the tumor. Nephroblastomatosis are immature renal elements called Nephrogenic rest. It is a Wilm's tumor precursor lesion that is both unifocal and deep within the Renal parenchyma (intralobar rest) or multi- focal (perilobar rest). Subsequent development of Wilm's tumor in the other kidney is more likely in patients with this feature; therefore prompt inspection of the contralateral kidney is necessary during surgery of the neprhogenic rest. CT scan follow-up should also be done. Renal Cell Carcinoma is rare during the first decade of life but can occur occasionally in teenagers. Initial presentations are abdominal mass and hematuria. Surgical resection may offer cure, but prognosis is poor with post-operative residual disease. Mesoblastic Nephroma is a massive, firm, solitary renal mass and is generally thought to be benign. It resembles Leiomyoma or low-grade leiomyosarcoma grossly and microscopically. It also accounts for the majority of congenital renal tumors. It is more often seen in males and noted to produce renin.
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- Spring '15