腹股沟区脂肪母细胞瘤样肿瘤:黏液样脂肪肉瘤的近似模拟物。

IF 4.4 Q1 PATHOLOGY
PATHOLOGICA Pub Date : 2025-02-01 DOI:10.32074/1591-951X-964
Manuel Mazzucchelli, Giuseppe Angelico, Lucia Salvatorelli, Giada Maria Vecchio, Chiara Romano, Paolo Vigneri, Maria Paola Mariani, Gerardo Ferrara, Gaetano Giuseppe Magro
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引用次数: 0

摘要

脂肪母细胞瘤样肿瘤是一种罕见的间质肿瘤,通常发生在年轻女性的外阴区域。虽然它被认为是一种良性肿瘤,但罕见的局部复发和异常远处转移已被报道。组织学检查显示一个边界清晰的分叶型肿瘤,由成熟脂肪细胞、梭形细胞和脂肪母细胞组成,分布在丰富的粘液样基质中,有许多薄壁毛细血管样血管。由于这种肿瘤的罕见性和其形态与其他良恶性脂肪瘤肿瘤相似,脂肪母细胞瘤样肿瘤的诊断往往具有挑战性。在此,我们提出一个病例发生在腹股沟区域的一个28岁的妇女。组织学检查显示成熟脂肪细胞、无色梭形细胞和纤维细胞质的混合物,以及大量单液泡形成的脂肪母细胞,这些脂肪母细胞位于含有大量薄壁分支血管的突出粘液样基质中。免疫组化结果显示,肿瘤细胞CD34呈弥漫性免疫染色,α-平滑肌肌动蛋白、desmin、Rb1、MDM2、STAT6呈阴性。主要鉴别诊断为黏液样脂肪肉瘤、梭形细胞脂肪瘤和细胞血管纤维瘤。FISH检测DDIT3为阴性;此外,FISH没有发现区域RB1增加或减少的证据。基于形态学,免疫组织化学和细胞遗传学/分子检查结果,最终诊断为腹股沟区域的“脂肪母细胞瘤样肿瘤”。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Lipoblastoma-like tumor of the inguinal region: a close mimicker of myxoid liposarcoma.

Lipoblastoma-like tumor is a rare mesenchymal neoplasm, typically arising in the vulvar region of young women. Although it is considered a benign tumor, rare local recurrences and exceptionally distant metastases have been reported. Histological examination reveals a well-circumscribed tumor with lobulated pattern, composed of a mixture of mature adipocytes, spindle cells and lipoblasts set in abundant myxoid stroma with numerous thin-walled capillary-like vessels. Due to the rarity of this neoplasm and its morphological resemblance with other benign and malignant lipomatous tumors, the diagnosis of lipoblastoma-like tumor is often challenging. Herein, we present a case occurring in the inguinal region of a 28-year-old woman. Histological examination showed a mixture of mature adipocytes, bland-looking spindle cells with fibrillary cytoplasm, and numerous univacuolated lipoblasts set in a prominent myxoid matrix containing numerous thin-walled branching vessels. Immunohistochemically, neoplastic cells showed diffuse immunostaining for CD34 and negativity for α-smooth muscle actin, desmin, Rb1, MDM2 and STAT6. The main differential diagnoses included myxoid liposarcoma, spindle cell lipoma and cellular angiofibroma. FISH was negative for DDIT3; moreover, no evidence of regional gain or loss of RB1 was identified by FISH. Based on morphological, immunohistochemical and cytogenetic/molecular findings, a final diagnosis of "lipoblastoma-like tumor" of the inguinal region was rendered.

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来源期刊
PATHOLOGICA
PATHOLOGICA PATHOLOGY-
CiteScore
5.90
自引率
5.70%
发文量
108
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