Primary Renal Large Cell Neuroendocrine Carcinoma in a Young Man

Rationale: The incidence of primary large-cell neuroendocrine carcinoma (LCNEC) is extremely rare in the urinary tract. In the present study, we investigated a case of primary LCNEC associated with the upper ureter. Patient concern: A 58-year-old Korean female patient with right ureter mass, which was accidentally detected. An enhancing mass measuring 3.3 cm in size was found in the computed tomography (CT) scan. No definitive evidence of lymph node and distant metastasis was observed. Diagnosis: Histopathological analysis revealed large atypical epithelial cells in upper ureter mass, based on neuroendocrine morphology. Immunohistochemistry was positive for synaptophysin, CD 56, and cytokeratin. Accordingly, the upper ureter mass was finally diagnosed as LCNEC stage III, pT3 cN0 cM0. Intervention: Right nephroureterectomy was conducted. Outcomes: Multiple metastatic lesions were detected in the right adrenal, paracaval, and right pararenal space of the patient in a CT scan 3 months post-surgery. The patient chemotherapy and radiation therapy were proceeded for metastatic and recurred mass. But patient died by multiorgan failure Lessons: In summary, this case study demonstrated that LCNEC can develop even in the upper ureter for the first time, despite the absence of neuroendocrine cells in the normal urothelium. The occurrence of LCNEC in the ureter is still very rare but is possible. Therefore, further studies are needed to establish appropriate treatment strategies.

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“…SCC and LCNEC represent malignant tumors, which are characterized by increased mitosis, vascular emboli, and tumor necrosis. [5,6]…”

Section: Discussionmentioning

confidence: 99%

Primary large-cell neuroendocrine carcinoma of the upper ureter

et al. 2019

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“…LCNEC of the kidney is extremely rare [2–6]. While various treatments have been attempted, no standard treatment for this disease exists.…”

Section: Discussionmentioning

confidence: 99%

“…Primary LCNEC of the urinary tract is an extremely rare and aggressive disease, and reports of LCNEC of the kidney are exceedingly scarce [2–6]. To the best of our knowledge, there are no reports available on LCNEC with cardiac metastasis.…”

Section: Introductionmentioning

confidence: 99%

Large cell neuroendocrine carcinoma of the kidney with cardiac metastasis: a case report

et al. 2017

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BackgroundPrimary large cell neuroendocrine carcinoma of the kidney is a rare and generally very aggressive disease. We present a case of a patient with primary large cell neuroendocrine carcinoma of the kidney with cardiac metastasis.Case presentationA 59-year-old Japanese man presented to his previous physician with hematuria. Computed tomography revealed masses in the heart and right kidney, and fluorodeoxyglucose-positron emission tomography showed abnormal uptake in the heart. A cardiac biopsy under transesophageal echocardiographic guidance revealed a metastatic tumor. Subsequently, multiple lung lesions were detected, and a right nephrectomy was performed after these metastases were suspected to have originated from renal carcinoma. Large cell neuroendocrine carcinoma of the kidney was ultimately diagnosed. Pancreatic metastasis was detected on computed tomography postoperatively. Three courses of chemotherapy with carboplatin and irinotecan were administered, and were temporarily effective against the metastatic lesions in the lungs and pancreas. However, our patient’s general condition deteriorated with the progression of the lesions, and he died 9 months after his initial examination.ConclusionsMulti-agent chemotherapy, including platinum-based drugs was effective against large cell neuroendocrine carcinoma metastases, albeit only temporarily. This is the first reported case of large cell neuroendocrine carcinoma with cardiac metastasis.

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“…Крупноклеточная нейроэндокринная карцинома (LCNEC) обладает высоким злокачественным потенциалом и является крайне редким заболеванием. В настоящее время в литературе насчитывается лишь 7 подобных случаев [8][9][10][11][12][13]. Микроскопически опухоль имеет то же строение, что и при других локализациях: легко различимые ядрышки, массивный везикулярный хроматин и / или крупный размер клетки, обильная цитоплазма, обширные зоны некроза, высокая митотическая активность >2 на 10 HPF, а в отдельных случаях -32 и 50 на 10 HPF.…”

Section: нейроэндокринная карцинома почкиunclassified

Neuroendocrine tumors of the urinary system: literature review

et al. 2019

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Most often, neuroendocrine carcinoma (NEC) is found in the gastrointestinal tract, broncho-pulmonary system, but they can also occur in other organs, such as the kidney, bladder, which is of most interest because of the rarity of this pathology. Until recently, there was not even a proper morphological classification for kidney NEC, and among some histological types, such as large cell neuroendocrine carcinoma of the kidney, only about 7 cases were recorded, and independent bladder carcinoids, about 15 cases. Currently, there are no clinical and morphological features of the NEC of the kidney and bladder, fundamentally distinguishing them from other neuroendocrine tumors and “classic” cancer of the same localization. This article also provides the data of the N.N. Blokhin National Medical Research Centre of Oncology on the incidence of all malignant neoplasms of the urinary system.

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Primary Renal Large Cell Neuroendocrine Carcinoma in a Young Man

Cited by 7 publications

References 9 publications

Primary large-cell neuroendocrine carcinoma of the upper ureter

Primary large-cell neuroendocrine carcinoma of the upper ureter

Large cell neuroendocrine carcinoma of the kidney with cardiac metastasis: a case report

Neuroendocrine tumors of the urinary system: literature review

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