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腹腔和腹膜后良性脂肪肿瘤——一种极其罕见的脂肪肉瘤模拟物及其诊断挑战。

Intra-Abdominal and Retroperitoneal Benign Lipomatous Tumors-An Extremely Rare Mimic of Liposarcoma and its Diagnostic Challenge.

机构信息

Department of Pathology and Laboratory Medicine, Perelman School of Medicine, Hospital of the University of Pennsylvania, Philadelphia, PA, USA.

出版信息

Int J Surg Pathol. 2024 Feb;32(1):46-57. doi: 10.1177/10668969231167511. Epub 2023 May 2.

Abstract

Lipomas are common superficial soft tissue tumors of mature adipocytes. In contrast, well-differentiated/dedifferentiated liposarcoma typically presents in the retroperitoneum as large masses. We provide clinicopathologic and follow-up details of 9 retroperitoneal/intra-abdominal benign lipomatous tumors (BLT) and discuss the utility of ancillary fluorescence in situ hybridization (FISH) in distinguishing from their malignant counterparts. Clinicopathologic details and histology of 9 intra-abdominal and retroperitoneal lipomas were studied along with ancillary CD10 immunohistochemistry (IHC) and FISH for and amplification. There were 6 females and 3 males. Median age at diagnosis was 52 years (range 36-81 years). Seven were identified incidentally and 2 presented with primary complaints. On imaging, 7 were considered suspicious for liposarcoma. Grossly, the tumors ranged from 3.4 to 41.2 cm (median 16.5 cm). Histologically, all cases showed well-differentiated BLT, further classified as lipoma (n = 7; 1 with metaplastic ossification, 2 with prominent vessels, and 4 ordinary lipomas) and lipoma-like hibernoma (n = 2)-the latter 2 showed intramuscular lesions with interspersed brown fat. CD10 IHC showed strong staining in the 2 hibernomas, whereas the staining was weak in the remaining. and amplification were negative by FISH in all. Follow-up (median 18 months) did not show recurrence on clinical or imaging evaluation. Retroperitoneal/intra-abdominal BLT are extremely rare and are indistinguishable clinically and radiographically from liposarcoma. This necessitates molecular confirmation even when the histology is convincingly benign, for a confident diagnosis. Our cohort shows that conservative excision without removal of abutted organs is sufficient in most cases.

摘要

脂肪瘤是成熟脂肪细胞的常见表浅软组织肿瘤。相比之下,分化良好/去分化脂肪肉瘤通常以大肿块的形式出现在腹膜后。我们提供了 9 例腹膜后/腹腔内良性脂肪性肿瘤(BLT)的临床病理和随访细节,并讨论了辅助荧光原位杂交(FISH)在鉴别其恶性肿瘤方面的应用。研究了 9 例腹腔内和腹膜后脂肪瘤的临床病理细节和组织学,并进行了辅助 CD10 免疫组化(IHC)和 FISH 检测 和 扩增。患者为 6 名女性和 3 名男性。诊断时的中位年龄为 52 岁(范围 36-81 岁)。7 例为偶然发现,2 例有原发性症状。影像学上,7 例被认为有脂肪肉瘤的嫌疑。大体上,肿瘤大小为 3.4-41.2cm(中位数为 16.5cm)。组织学上,所有病例均显示为分化良好的 BLT,进一步分为脂肪瘤(n=7;1 例有骨化性化生,2 例有明显血管,4 例普通脂肪瘤)和脂肪瘤样冬眠瘤(n=2)-后 2 例为肌肉内病变,伴有棕色脂肪穿插。2 例冬眠瘤的 CD10 IHC 染色强烈,而其余病例染色较弱。FISH 检测所有病例均未显示 和 扩增。临床和影像学随访(中位时间 18 个月)未显示复发。腹膜后/腹腔内 BLT 极为罕见,在临床上和影像学上均无法与脂肪肉瘤区分。即使组织学表现明确为良性,为了做出明确的诊断,也需要进行分子学确认。我们的队列表明,在大多数情况下,保守切除而不切除毗邻器官就足够了。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/be19/10901880/08b239f81080/10.1177_10668969231167511-fig1.jpg

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