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Presented by Dr. Jonathan Epstein and prepared by Dr. J. Judd Fite
Case 2. A 33 year old man was noted to have a renal mass with lung metastases at the time of presentation.
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Question 1 of 1
1. Question
Case 2. A 33 year old man was noted to have a renal mass with lung metastases at the time of presentation.
Correct
Answer: A. Medullary renal cell carcinoma
Histology: The tumor consists of tubulopapillary structures, cords, glandular and solid sheets with extensive necrosis. It also has microcystic and reticular patterns somewhat similar testicular yolk sac tumors. Cell have a moderate amount of basophilic or eosinophilic cytoplasm. Rhabdoid morphology is focally present. Nuclei are vesicular and pleomorphic with prominent nucleoli associated with frequent mitotic activity. There is a diffuse infiltration by neutrophils within tumor epithelial sheets. Associated with the tumor is an inflamed abundant dense desmoplastic stroma. The tumor is infiltrative into adjacent renal tissue.
Discussion: The differential diagnosis of renal medullary cancer is predominantly collecting duct carcinoma as both can share similar morphology and involve the renal medulla. However, the microcystic and reticular patterns are more typical of medullary carcinoma. Most experts feel that a definitive diagnosis of medullary carcinoma can only be made in association with sickle cell trait/disease, and almost exclusively in black patients. Inactivation of tumor suppressor gene hSNF5/INI1 on long arm of chromosome 22 is frequently detected, and can be demonstrated immunohistochemically, although it can also be lost in collecting duct carcinoma. Occasionally sickled erythrocytes can be identified which if present would help to establish the diagnosis of medullary carcinoma. There are only rare reports of survival beyond 1st year post-diagnosis. The majority of patients have lymph node metastases at time of diagnosis often with metastases to liver, lung and bone.
Incorrect
Answer: A. Medullary renal cell carcinoma
Histology: The tumor consists of tubulopapillary structures, cords, glandular and solid sheets with extensive necrosis. It also has microcystic and reticular patterns somewhat similar testicular yolk sac tumors. Cell have a moderate amount of basophilic or eosinophilic cytoplasm. Rhabdoid morphology is focally present. Nuclei are vesicular and pleomorphic with prominent nucleoli associated with frequent mitotic activity. There is a diffuse infiltration by neutrophils within tumor epithelial sheets. Associated with the tumor is an inflamed abundant dense desmoplastic stroma. The tumor is infiltrative into adjacent renal tissue.
Discussion: The differential diagnosis of renal medullary cancer is predominantly collecting duct carcinoma as both can share similar morphology and involve the renal medulla. However, the microcystic and reticular patterns are more typical of medullary carcinoma. Most experts feel that a definitive diagnosis of medullary carcinoma can only be made in association with sickle cell trait/disease, and almost exclusively in black patients. Inactivation of tumor suppressor gene hSNF5/INI1 on long arm of chromosome 22 is frequently detected, and can be demonstrated immunohistochemically, although it can also be lost in collecting duct carcinoma. Occasionally sickled erythrocytes can be identified which if present would help to establish the diagnosis of medullary carcinoma. There are only rare reports of survival beyond 1st year post-diagnosis. The majority of patients have lymph node metastases at time of diagnosis often with metastases to liver, lung and bone.
