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原发性输尿管小细胞癌:病例报告及文献复习

Primary small cell carcinoma of the ureter: Case report and review of the literature.

作者信息

Farci Fabiola, Manassero Francesca, Baldesi Ramona, Bartolucci Annamaria, Boldrini Laura, Selli Cesare, Faviana Pinuccia

机构信息

Department of Surgical, Medical, Molecular Pathology and Critical Area, University of Pisa Unit of Pathology, Department of Translational Research and New Technologies in Medicine and Surgery, University of Pisa, Via Roma, Pisa, Italy.

出版信息

Medicine (Baltimore). 2018 Jun;97(24):e11113. doi: 10.1097/MD.0000000000011113.

Abstract

RATIONALE

Primitive small cell carcinoma of the ureter is extremely rare, in this case report is meticulously described its aggressive clinical course and the pathological clues that help with the diagnosis. Also, a detailed table with the clinico-pathological features of analogous case reports in literature is provided.

PATIENT CONCERNS

A 79-year-old female presented with gross hematuria and flank pain.

DIAGNOSES

Small cell carcinoma of the ureter. The surgical specimen showed a mixed histology of small cell carcinoma and transitional cell carcinoma; the common neuroendocrine markers (chromogranin A, synaptophysin, CD56) were positive, and vimentin and thyroid transcription factor 1 were negative. The patient had an advanced stage at presentation with regional nodes involvement (pT3N1).

INTERVENTIONS

Segmental ureterectomy was performed but it was only possible to administer 1 cycle of platinum-based adjuvant chemotherapy due to the rapid decline of her clinical parameters.

OUTCOMES

The disease rapidly spread locally and metastasized.

LESSON

The clinicians must be aware of this aggressive tumor with silent clinical course and advanced stages at presentation.

摘要

理论依据

输尿管原发性小细胞癌极为罕见,本病例报告详细描述了其侵袭性临床病程及有助于诊断的病理线索。此外,还提供了一份文献中类似病例报告临床病理特征的详细表格。

患者情况

一名79岁女性,表现为肉眼血尿和侧腹疼痛。

诊断

输尿管小细胞癌。手术标本显示为小细胞癌和移行细胞癌的混合组织学;常见神经内分泌标志物(嗜铬粒蛋白A、突触素、CD56)呈阳性,波形蛋白和甲状腺转录因子1呈阴性。患者就诊时已处于晚期,伴有区域淋巴结受累(pT3N1)。

干预措施

进行了输尿管节段切除术,但由于其临床参数迅速下降,仅能给予1周期铂类辅助化疗。

结果

疾病迅速在局部扩散并发生转移。

经验教训

临床医生必须意识到这种具有隐匿临床病程且就诊时处于晚期的侵袭性肿瘤。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/521e/6023684/2938df694d0d/medi-97-e11113-g002.jpg

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