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罕见的卵巢浸润性黏液性腺癌、单侧肾发育不全和双角子宫同时存在 1 例报告:是否构成一个新的三联征?

A case report on rare co-occurrence of invasive ovarian mucinous adenocarcinoma, unilateral renal agenesis, and bicornuate uterus: is it a new triad?

机构信息

Department of Urology, Kilimanjaro Christian Medical University College (KCMUCO), P. O Box 2240, Moshi, Tanzania.

Department of Urology, Kilimanjaro Christian Medical Centre (KCMC), P. O Box 3010, Moshi, Tanzania.

出版信息

BMC Womens Health. 2024 May 20;24(1):300. doi: 10.1186/s12905-024-03130-y.

DOI:10.1186/s12905-024-03130-y
PMID:38769573
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11103989/
Abstract

BACKGROUND

Concomitant invasive ovarian mucinous adenocarcinoma, unilateral renal agenesis and bicornuate uterus is a rare combination. Unilateral renal agenesis has been associated with genital anomalies, such as unicornuate and bicornuate uterus. Furthermore, a wealth of studies has reported the association between unicornuate uterus and ovarian anomalies, such as the absence of an ovary or ectopic ovaries, but rarely has there been a combination of the three to the best of our knowledge. The present case report is the first case presentation with a combination of the three syndromes: ovarian mucinous tumor, unilateral renal agenesis, and bicornuate uterus.

CASE PRESENTATION

We report the case of a 17-year-old who presented with abdominal distension. On examination, a CT scan revealed a large multicystic abdominal mass on the right side, with an absence of the right kidney while the left kidney was normal in size, appearance, and position. Intraoperatively, massive blood-stained ascitic fluid was evacuated. Additionally, a large whitish polycystic intra-abdominal mass with mucus-like materials and solid areas was attached to the midpoint of the colon and the right ovary, with visible metastasis to the omentum. The uterus was bicornuate. The mass and omentum were taken for histopathology and a diagnosis of invasive ovarian mucinous cystadenocarcinoma with metastasis to the colon and omentum was made after a pathological report.

CONCLUSIONS

The presence of these conditions in the same individual could potentially complicate medical management and fertility considerations. Thus, a need for a multidisciplinary medical team, including gynecologists, urologists, and oncologists, to address their unique needs and provide appropriate treatment and guidance. Further research and case studies are needed to better understand the possible association and implications of these rare co-occurring conditions.

摘要

背景

同时患有侵袭性卵巢黏液性腺癌、单侧肾发育不全和双角子宫是一种罕见的组合。单侧肾发育不全与生殖器异常有关,如单角子宫和双角子宫。此外,大量研究报告了单角子宫与卵巢异常之间的关联,如卵巢缺失或异位卵巢,但据我们所知,很少有这三种情况同时存在的报道。本病例报告是首例同时存在这三种综合征的病例:卵巢黏液性肿瘤、单侧肾发育不全和双角子宫。

病例介绍

我们报告了一例 17 岁女性患者,因腹胀就诊。检查时,CT 扫描显示右侧有一个大的多囊性腹部肿块,右侧肾脏缺失,而左侧肾脏大小、形态和位置正常。术中,大量血性腹水被排出。此外,大量灰白色多囊性腹腔内肿块,附有黏液样物质和实性区域,附着在结肠中段和右侧卵巢,可见转移至大网膜。子宫呈双角状。肿块和大网膜被送检进行组织病理学检查,病理报告诊断为侵袭性卵巢黏液性囊腺癌,伴结肠和大网膜转移。

结论

这些情况同时存在于同一患者中,可能会使医疗管理和生育考虑变得复杂。因此,需要一个多学科医疗团队,包括妇科医生、泌尿科医生和肿瘤科医生,来满足他们的独特需求,并提供适当的治疗和指导。需要进一步的研究和病例研究,以更好地理解这些罕见的共存情况之间的可能关联和影响。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7b4a/11103989/13bd79c36322/12905_2024_3130_Fig4_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7b4a/11103989/6acd7c2c9aa6/12905_2024_3130_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7b4a/11103989/c653d694252c/12905_2024_3130_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7b4a/11103989/43e2e0b60a51/12905_2024_3130_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7b4a/11103989/13bd79c36322/12905_2024_3130_Fig4_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7b4a/11103989/6acd7c2c9aa6/12905_2024_3130_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7b4a/11103989/c653d694252c/12905_2024_3130_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7b4a/11103989/43e2e0b60a51/12905_2024_3130_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7b4a/11103989/13bd79c36322/12905_2024_3130_Fig4_HTML.jpg

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本文引用的文献

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Congenital unilateral renal agenesis: Prevalence, prenatal diagnosis, associated anomalies. Data from two birth-defect registries.先天性单侧肾发育不全:患病率、产前诊断、相关畸形。两个出生缺陷登记处的数据。
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