25443Centre Hospitalier de l'Université de Montréal, Montreal, Quebec, Canada.
103387Anna-Laberge Hospital, Châteauguay, Quebec, Canada.
Int J Surg Pathol. 2022 Feb;30(1):33-38. doi: 10.1177/10668969211020504. Epub 2021 May 24.
We present the case of a 31-year-old woman who underwent surgical excision for a polypoid, vulvar lesion. Histopathological analysis showed a diffuse myxoid stroma admixed with scant collagen fibrils. Thin-walled and branching blood vessels were prominent, with a mild perivascular lymphocytic infiltrate. Cytologically bland spindle cells with inconspicuous nucleoli were immersed in a loose myxoid stroma. This combination of histopathological features along with multinodularity in the subcutaneous fat raised concern for deep angiomyxoma, a locally destructive neoplasm. Among our differential of myxoid lesions of the vulva, we ultimately favored the diagnosis of vulvar cutaneous myxoma. Upon further investigation, we learned that our patient was indeed known for the Carney complex. We highlight that vulvar cutaneous myxomas arising in the context of the Carney complex pose a significant diagnostic challenge for pathologists and should not be overdiagnosed as aggressive lesions such as deep angiomyxoma or other malignant stromal neoplasms.
我们报告了一例 31 岁女性，因息肉样外阴病变接受了手术切除。组织病理学分析显示弥漫性黏液样基质伴有稀少的胶原纤维。薄壁和分支状的血管明显，伴有轻度血管周围淋巴细胞浸润。细胞学上，胞质淡染的梭形细胞有不明显的核仁，浸泡在疏松的黏液样基质中。这种组织病理学特征的组合以及皮下脂肪的多结节性引起了对深部血管黏液瘤的关注，深部血管黏液瘤是一种局部破坏性肿瘤。在我们对外阴黏液样病变的鉴别诊断中，我们最终倾向于外阴皮肤黏液瘤的诊断。进一步调查后，我们了解到我们的患者确实患有卡尼综合征。我们强调，卡尼综合征背景下发生的外阴皮肤黏液瘤对病理学家来说是一个重大的诊断挑战，不应误诊为深部血管黏液瘤或其他恶性间质肿瘤等侵袭性病变。
