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伴有神经内分泌分化的腹膜后黏液性囊腺瘤:1例罕见病例及诊断与治疗的综合方法

Retroperitoneal mucinous cystadenoma with neuroendocrine differentiation: a rare case and comprehensive approach to diagnosis and management.

作者信息

Ho Yen, Chen Wei-Yu, Lai Chung-Howe, Syu Syuan-Hao

机构信息

Department of Urology, Wan Fang Hospital, Taipei Medical University, Taipei, Taiwan.

Department of Pathology, Wan Fang Hospital, Taipei Medical University, Taipei, Taiwan.

出版信息

Diagn Pathol. 2025 May 3;20(1):58. doi: 10.1186/s13000-025-01658-7.

DOI:10.1186/s13000-025-01658-7
PMID:40319316
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12049035/
Abstract

BACKGROUND

Retroperitoneal mucinous cystadenomas are exceptionally rare neoplasms, with limited cases reported in the literature. The occurrence of neuroendocrine differentiation in such tumors is even more uncommon, posing unique diagnostic and management challenges.

CASE PRESENTATION

We report a case of a 32-year-old woman who was incidentally diagnosed with a right retroperitoneal cyst during routine prenatal ultrasonography. The patient remained asymptomatic until postpartum, prompting further evaluation of the cyst. Imaging studies identified a large cystic mass, ultimately leading to diagnostic laparoscopy and surgical excision. Histopathological analysis confirmed the diagnosis of a mucinous cystadenoma with neuroendocrine cell proliferation.

DISCUSSION

This case highlights the complexity of diagnosing and managing retroperitoneal mucinous cystadenomas, particularly those with neuroendocrine features. Given the rarity of these tumors, thorough histopathological examination is crucial to differentiate them from other cystic lesions. Surgical excision remains the definitive treatment, with long-term follow-up essential to ensure complete resolution and monitor for recurrence or malignant transformation.

CONCLUSION

Retroperitoneal mucinous cystadenomas with neuroendocrine differentiation represent a rare clinical entity requiring careful evaluation. This report underscores the importance of considering neuroendocrine differentiation in retroperitoneal cystic lesions and emphasizes the role of complete surgical excision followed by close monitoring to ensure favorable outcomes.

摘要

背景

腹膜后黏液性囊腺瘤是极为罕见的肿瘤,文献报道的病例有限。此类肿瘤发生神经内分泌分化更为罕见,带来了独特的诊断和管理挑战。

病例报告

我们报告一例32岁女性,在常规产前超声检查时偶然诊断为右腹膜后囊肿。该患者产后一直无症状,促使对囊肿进行进一步评估。影像学检查发现一个大的囊性肿块,最终进行了诊断性腹腔镜检查和手术切除。组织病理学分析确诊为伴有神经内分泌细胞增殖的黏液性囊腺瘤。

讨论

该病例突出了腹膜后黏液性囊腺瘤诊断和管理的复杂性,尤其是那些具有神经内分泌特征的肿瘤。鉴于这些肿瘤的罕见性,彻底的组织病理学检查对于将它们与其他囊性病变区分开来至关重要。手术切除仍然是确定性治疗,长期随访对于确保完全消退以及监测复发或恶性转化至关重要。

结论

具有神经内分泌分化的腹膜后黏液性囊腺瘤是一种罕见的临床实体,需要仔细评估。本报告强调了在腹膜后囊性病变中考虑神经内分泌分化的重要性,并强调了完全手术切除后密切监测以确保良好预后的作用。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3253/12049035/9c8f6d0509e7/13000_2025_1658_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3253/12049035/fc29fce96136/13000_2025_1658_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3253/12049035/9c8f6d0509e7/13000_2025_1658_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3253/12049035/fc29fce96136/13000_2025_1658_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3253/12049035/9c8f6d0509e7/13000_2025_1658_Fig2_HTML.jpg

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