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Presented by Dr. Andres Matoso and prepared by Dr. Sintawat Wangsiricharoen
Adult female with renal mass
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Answer: C. Renal carcinoid
Histology: Well demarcated neoplasm composed of tightly packed cords and trabeculae of tumor cells with a ribbonlike appearance. The nuclei of tumor cells are elongated with their long axis perpendicular to the long axis of the cord.
Discussion: Primary renal carcinoid tumors are extremely rare and for an unknown reason, they have an association with horseshow kidney. They occur evenly divided between males and females, and most patients are diagnosed while asymptomatic and discovered to have metastasis at the time of diagnosis. The morphologic features of renal carcinoids are the same as carcinoid in other places and the most common architectural pattern is the one seen in this case with tightly packed cords of tumor cells. Mitoses range from 0/10HPFs to 3-4/10HPFs. By immunohistochemistry, renal carcinoids are positive for neuroendocrine markers synaptophysin and chromogranin and focally positive for CK7 or CK20 and negative for TTF-1 and WT1. The main differential diagnoses include other tumors with neuroendocrine differentiation such as small cell carcinoma, PNET, paraganglioma and neuroblastoma. Small cell carcinoma has high grade features that are not seen in carcinoid tumors including high mitotic activity, nuclear molding, and necrosis. Glandular structures seen in carcinoid tumors could simulate pseudorosettes seen in PNET but CD99 immunolabeling in PNET should help in that differential diagnosis. Neuroblastomas in the kidney are rare, especially in adults while primary carcinoids of the kidney have not been described yet in pediatric patients. Paragangliomas are negative for cytokeratin and contain a nested arrangement of tumor cells that are positive for GATA3.
References
Am J Surg Pathol. 2007 Oct;31(10):1539-44
J Surg Oncol. 1998;68:113–119.
Urology. 1976;8:146–148.
Incorrect
Answer: C. Renal carcinoid
Histology: Well demarcated neoplasm composed of tightly packed cords and trabeculae of tumor cells with a ribbonlike appearance. The nuclei of tumor cells are elongated with their long axis perpendicular to the long axis of the cord.
Discussion: Primary renal carcinoid tumors are extremely rare and for an unknown reason, they have an association with horseshow kidney. They occur evenly divided between males and females, and most patients are diagnosed while asymptomatic and discovered to have metastasis at the time of diagnosis. The morphologic features of renal carcinoids are the same as carcinoid in other places and the most common architectural pattern is the one seen in this case with tightly packed cords of tumor cells. Mitoses range from 0/10HPFs to 3-4/10HPFs. By immunohistochemistry, renal carcinoids are positive for neuroendocrine markers synaptophysin and chromogranin and focally positive for CK7 or CK20 and negative for TTF-1 and WT1. The main differential diagnoses include other tumors with neuroendocrine differentiation such as small cell carcinoma, PNET, paraganglioma and neuroblastoma. Small cell carcinoma has high grade features that are not seen in carcinoid tumors including high mitotic activity, nuclear molding, and necrosis. Glandular structures seen in carcinoid tumors could simulate pseudorosettes seen in PNET but CD99 immunolabeling in PNET should help in that differential diagnosis. Neuroblastomas in the kidney are rare, especially in adults while primary carcinoids of the kidney have not been described yet in pediatric patients. Paragangliomas are negative for cytokeratin and contain a nested arrangement of tumor cells that are positive for GATA3.
References
Am J Surg Pathol. 2007 Oct;31(10):1539-44
J Surg Oncol. 1998;68:113–119.
Urology. 1976;8:146–148.