Figure - available from: Rare Tumors
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Sections from renal mass show hyper cellular tumor with trabecular and nesting pattern. Individual tumor cells show salt and pepper nuclei (H&EX 250).
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Primary renal carcinoid tumors are quite rare. The pathogenesis of these tumors is unknown due to lack of enterochromaffin cells in the kidney. Because of nonspecific clinical manifestations and radiologic features, they are commonly misdiagnosed. Hence, Primary renal carcinoid tumors should be considered in differential diagnosis of any renal mass...
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... Well-differentiated NET of renal is a relatively low-grade malignancy, but it often occurs local invasion, lymph node and distant metastasis. In our study, seven cases (24.1%, 7/29) had local renal sinus, 16,20,21 perirenal fat and nerve invasion; eight cases (27.6%, 8/29) had lymph nodes metastasis; 6,16,17,21,27 and nine cases (31%, 9/29) had distant metastasis, 6,7,13,14,16,21,22,25,26 including five (17.2%, 5/29) cases of liver metastasis 6,7,14,16,26 and three cases (10.3%, 3/ 29) of lung metastasis. 6,21,25 In addition, the tumor can metastasize to bone, 6 contralateral kidney and adrenal, 13 eye, 14 pancreas, 21,25 thyroid, skin, 13 renal vein and inferior vena cava. ...
... Well-differentiated NET of renal is a relatively low-grade malignancy, but it often occurs local invasion, lymph node and distant metastasis. In our study, seven cases (24.1%, 7/29) had local renal sinus, 16,20,21 perirenal fat and nerve invasion; eight cases (27.6%, 8/29) had lymph nodes metastasis; 6,16,17,21,27 and nine cases (31%, 9/29) had distant metastasis, 6,7,13,14,16,21,22,25,26 including five (17.2%, 5/29) cases of liver metastasis 6,7,14,16,26 and three cases (10.3%, 3/ 29) of lung metastasis. 6,21,25 In addition, the tumor can metastasize to bone, 6 contralateral kidney and adrenal, 13 eye, 14 pancreas, 21,25 thyroid, skin, 13 renal vein and inferior vena cava. ...
Hua Jiang, He Zhang Department of Urology, The Fifth Affiliated Hospital of Zunyi Medical University (Zhuhai Sixth People’s Hospital), Zhuhai, People’s Republic of ChinaCorrespondence: Hua Jiang, The Fifth Affiliated Hospital of Zunyi Medical University (Zhuhai Sixth People’s Hospital), No. 1439, Zhufeng Road, Zhuhai, 519100, People’s Republic of China, Tel +8613726205880, Email jianghua426@zmu.edu.cnAbstract: Primary carcinoid tumor of the kidney is an extremely rare well-differentiated neuroendocrine tumor, which is generally a low-grade malignant cancer with a good prognosis. Carcinoid tumors are rarely found in the urinary system. Here, we report a 34-year-old woman with primary renal well-differentiated neuroendocrine tumor who underwent nephron sparing surgery and no evidence of recurrence or distant metastasis was found during routine follow-up. We searched the case of renal carcinoid with the search phrase “carcinoid [title] and kidney [title]” and “carcinoid [title] and renal [title]” using the PubMed and restricted the search to articles published in English since 2013. The clinical manifestations, age, sex, tumor size, location, gross pathology, light microscopy and immunohistochemistry were analyzed. A total of 28 cases of renal carcinoid were retrieved from PubMed. Higher proportion of positive labeling of CgA, Syn, NSE and CD56 are most valuable in the diagnosis of primary renal well-differentiated neuroendocrine tumor. At present, radical nephrectomy remains the gold standard in the curative-intent therapy for well-differentiated neuroendocrine carcinoma of kidney, in metastatic renal carcinoid, long-term use of octreotide may be an effective adjuvant therapy.Keywords: renal, well-differentiated neuroendocrine tumor, pathological features
Well-differentiated renal neuroendocrine tumors are rare tumors. As their biologic behavior is not fully known, there is a need to know more about these cases. We performed a retrospective chart review of all the cases diagnosed with renal neuroendocrine tumors from January 2016 to December 2020 (five years) in order to understand their clinical features, morphological characteristics and outcome. We included six cases with mean age of 46.2 years (4 males) in our study. All patients underwent radical nephrectomy. Histologically all showed tumor disposed in nests and trabeculae and majority of the tumors belonged to well-differentiated neuroendocrine tumor Grade 1 (WHO criteria of gastoroenteropancreatic neuroendocrine neoplasms). Lymph node metastasis was seen in two cases at the time of clinical presentation. All the tumors were diffusely positive for neuroendocrine tumor markers (synaptophysin, chromogranin, NSE, CD56). Follow-up data was available in all cases with an average follow-up of two years and neither has shown evidence of metastasis or relapse till last follow-up. Role of morphological patterns and immunohistochemical markers is highlighted with the importance of including Ki-67 index in grading them to better understand their outcome.
