Multicentric small cell neuroendocrine neoplasm of the renal pelvis and ureter with concomitant focal high-grade urothelial carcinoma of the ureter: A case report

Banerji, John S.; Korula, Anila; Panicker, Jayalakshmi B

doi:10.4103/0970-1591.44273

Cited by 21 publications

(25 citation statements)

References 7 publications

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“…5 Even though primary ureteral NETs are believed to show a male predominance, several cases have occurred in women, most often after the sixth decade of life. [6][7][8][9][10] Interestingly, ureteral neuroendocrine neoplasms seem to occur more frequently in Asians than in any other ethnic group (Table). Although some of the reported cases include a history of tobacco consumption, a relationship between smoking and urinary tract NETs has not been well established.…”

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confidence: 99%

“…While it is uncertain whether this mechanism operates in vivo, or if it can be extrapolated from biliary to urinary NET pathogenesis, it could potentially explain why many reported ureteral NETs show a mixed histologic profile. 6,15,16 CLINICAL PRESENTATION AND DIFFERENTIAL DIAGNOSIS Clinical presentation is widely variable, from asymptomatic microscopic hematuria to complete ureteral obstruction and hydronephrosis. The most common initial manifestations are flank pain and hematuria.…”

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confidence: 99%

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Primary Neuroendocrine Tumors of the Ureter: A Short Review

Acosta

Kajdacsy-Balla²

2016

Archives of Pathology &Amp; Laboratory Medicine

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Neuroendocrine tumors represent 0.05% of urinary tract malignancies, and most originate in the bladder. Their pathogenesis is debated, since ureters are thought to lack neuroendocrine cells. Morphologically, most ureteral neuroendocrine tumors demonstrate the typical small cell neuroendocrine appearance, are immunohistochemically positive for synaptophysin/chromogranin A/CD56, and show electron-dense granules by electron microscopy. Clinical presentation is similar to that of other more common ureteral neoplasms. Prognosis is usually dismal, but a multimodal treatment approach including platinum-based adjuvant or neoadjuvant chemotherapy seems to be beneficial.

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confidence: 99%

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confidence: 99%

Primary Neuroendocrine Tumors of the Ureter: A Short Review

Acosta

Kajdacsy-Balla²

2016

Archives of Pathology &Amp; Laboratory Medicine

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“…Small cell carcinoma arising from the renal pelvis may be differentiated from that originating in the renal parenchyma by the presence of nonneuroendocrine components, tumor infiltration of the transitional cell epithelium of the renal pelvis and strong positive staining for neuron-specific enolase [7, 10]. As compared to prior reports, the current case had two unique characteristics; the absence of nonneuroendocrine component and the presence of multiple tumors [14]. …”

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confidence: 95%

Small Cell Carcinoma of the Renal Pelvis: A Case Report and Review of the Literature

et al. 2011

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Extrapulmonary small cell carcinoma occurs in nearly all organs except the central nervous system and the liver. We are presenting a case of renal small cell carcinoma (SCC) with two unique characters. A 75-year-old patient was evaluated for back pain with no other complaints. Magnetic Resonance (MR) imaging of the abdomen revealed homogeneous tumor in the left renal pelvis extending beyond the kidney. Metastatic workup was negative. A left nephroureterectomy was performed. Histopathology and immunohistochemistry revealed a small cell carcinoma of the renal pelvis. The patient declined adjuvant therapy and died 2 months after surgery due to unrelated causes. After comprehensive worldwide literature search, we found 13 cases of SCC of the renal pelvis, including the current case. The mean age was 61.6 years (37–83), with a M : F ratio of 1 : 1.8. The average duration of symptoms was 71.4 days (21–168). Gross hematuria was the most common symptom (69.2%) followed by pain (61.5%). Adjuvant chemotherapy was provided to 4 patients (30.7%), and neoadjuvant to 1 patient. The median survival of patients who did and did not receive chemotherapy was 5.5 months (3–8) and 6 months (2–31), respectively, P < .50. In conclusion, renal SCC (both parenchymal and pelvic SCC) is a rapidly fatal disease with a median survival of ≤8 months.

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“…We identified a total of 21 cases of previously published SCC of the renal pelvis and/or ureter spanning a period of 23 years. 5,[9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24] The clinical and pathologic features of these cases are presented in Table 3, and the comparison discussed below reveals some interesting differences between these and the cases in our series.…”

Section: Resultsmentioning

confidence: 99%

Small Cell Carcinoma of the Renal Pelvis and Ureter: Clinicopathologic and Immunohistochemical Features

Miller

Holmäng

Johansson³

et al. 2011

Archives of Pathology &Amp; Laboratory Medicine

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Context.—Small cell carcinoma (SCC) of the renal pelvis and/or ureter is very rare, with only case reports published in the literature. Objective.—To describe the clinicopathologic and immunohistochemical findings in the largest series to date. Design.—A review of a regional cancer registry identified 10 cases diagnosed as SCC from 930 patients with renal pelvic and/or ureteral cancer from 1971 to 1998. The original slides, demographics, treatment, and clinical outcome were reviewed. Representative sections were immunostained for AE1/AE3, cytokeratin 7, cytokeratin 20, CD56, synaptophysin, chromogranin, and thyroid transcription factor 1. Results.—Of the 10 cases, 5 were pure SCC, 2 were mixed (SCC and urothelial carcinoma), 2 were reclassified as poorly differentiated squamous carcinoma, and 1 was reclassified as urothelial carcinoma. The patients with SCC had an age range of 50 to 80 years (median, 72 years) with a female to male ratio of 2.5∶1. All patients had non–organ confined disease. Five of 7 patients died of disease; 4 of those 5 had been clinically followed (median survival, 23 months) and 1 was diagnosed at autopsy. The SCC cases revealed positive staining of the SCC component as follows: AE1/AE3 (7 of 7), CD56 (7 of 7), synaptophysin (6 of 7), thyroid transcription factor 1 (5 of 7), chromogranin (4 of 7), and cytokeratin 7 (1 of 7). None were positive for cytokeratin 20 (0 of 7). Conclusions.—SCC of the renal pelvis/ureter is seen in a predominately female population in Sweden, is clinically aggressive, and has poor survival when presenting at an advanced stage in patients only treated by surgery. An immunostain panel serves as a useful adjunct in classifying these tumors.

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Multicentric small cell neuroendocrine neoplasm of the renal pelvis and ureter with concomitant focal high-grade urothelial carcinoma of the ureter: A case report

Abstract: Malignant small cell neuroendocrine tumors of the pelvi-calyceal system are rare, and even more uncommon is their occurrence with concomitant transitional cell carcinoma, in the same renal unit. We present such a case for its unique presentation.

Cited by 21 publications

References 7 publications

Primary Neuroendocrine Tumors of the Ureter: A Short Review

Primary Neuroendocrine Tumors of the Ureter: A Short Review

Small Cell Carcinoma of the Renal Pelvis: A Case Report and Review of the Literature

Small Cell Carcinoma of the Renal Pelvis and Ureter: Clinicopathologic and Immunohistochemical Features

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