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《类似于非典型性脂肪肉瘤的冬眠瘤:64 例形态学独特亚群病例》

Hibernoma Mimicking Atypical Lipomatous Tumor: 64 Cases of a Morphologically Distinct Subset.

机构信息

Department of Pathology, Brigham and Women's Hospital, Harvard Medical School, Boston, MA.

出版信息

Am J Surg Pathol. 2018 Jul;42(7):951-957. doi: 10.1097/PAS.0000000000001061.

DOI:10.1097/PAS.0000000000001061
PMID:29629919
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6438389/
Abstract

Hibernoma is a benign adipocytic tumor with predilection for subcutaneous tissue of the thigh, upper trunk, and neck of middle-aged adults. 11q13 rearrangement resulting in MEN1/AIP codeletion is characteristic. Hibernomas are composed, in varying proportions, of brown fat cells, mature adipocytes, and microvacuolated lipoblast-like cells. Examples containing predominantly multivacuolated lipoblast-like cells are uncommon and distinction from atypical lipomatous tumor (ALT) is important for clinical management. We herein present the clinicopathologic features of 64 hibernomas histologically mimicking ALT. MDM2 and CDK4 immunohistochemistry as well as MDM2 fluorescence in situ hybridization were performed in a subset of cases. Clinical and follow-up information were obtained from referring pathologists. Thirty-four patients were male and 30 female, with a median age of 43 years (range, 24 to 78 y). The tumors were well circumscribed and mostly deeply located (53/64 cases, 83%) with a median tumor size of 12.9 cm (range, 3.5 to 23 cm) and predilection for the thigh (42/64 cases, 66%). Histologically, large cells with prominent lipoblast-like cytoplasmic fatty vacuoles and small central nuclei were present to a prominent degree in all cases, along with mature univacuolated adipocytes and smaller numbers of large, finely vacuolated cells with eosinophilic granular cytoplasm. Nuclear atypia and mitoses were absent. None of the 39 cases tested showed CDK4 and MDM2 overexpression or MDM2 amplification. Follow-up, available for 16/64 cases (median, 47 mo; range, 1 to 165 mo), revealed no recurrences or metastases. Hibernoma mimicking ALT shows predilection for deep soft tissue, especially in the thigh. These tumors behave in a benign manner and MDM2/CDK4 negativity may be useful in excluding ALT.

摘要

褐色脂肪瘤是一种良性脂肪细胞肿瘤,好发于中老年人的大腿、上半身和颈部的皮下组织。其特征为 11q13 重排导致 MEN1/AIP 缺失。褐色脂肪瘤由不同比例的棕色脂肪细胞、成熟脂肪细胞和微泡状脂肪母细胞样细胞组成。含有主要为多泡状脂肪母细胞样细胞的病例较为少见,因此与非典型脂肪肉瘤(ALT)进行鉴别对于临床管理非常重要。本文报道了 64 例组织学上类似于 ALT 的褐色脂肪瘤的临床病理特征。对部分病例进行了 MDM2 和 CDK4 免疫组化以及 MDM2 荧光原位杂交。临床和随访信息由转诊病理医生提供。34 例患者为男性,30 例为女性,中位年龄为 43 岁(范围,24 岁至 78 岁)。肿瘤边界清楚,大多位于深部(64 例中的 53 例,83%),肿瘤大小中位数为 12.9cm(范围,3.5cm 至 23cm),大腿部位最常见(64 例中的 42 例,66%)。所有病例均表现为大细胞,细胞质内有明显的脂肪母细胞样空泡,细胞核小而居中,同时存在成熟的单泡状脂肪细胞和数量较少的大而有空泡的细胞,胞质呈嗜酸性颗粒状。核异型性和有丝分裂均不存在。39 例检测的病例均未显示 CDK4 和 MDM2 过表达或 MDM2 扩增。16/64 例(中位随访时间 47 个月,范围 1 至 165 个月)的病例中有 16 例获得随访,均未见复发或转移。表现为 ALT 样的褐色脂肪瘤偏爱深部软组织,尤其是大腿。这些肿瘤表现为良性行为,MDM2/CDK4 阴性可能有助于排除 ALT。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4172/6438389/d8672520a3a0/nihms-1014858-f0004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4172/6438389/d5798d4512ed/nihms-1014858-f0001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4172/6438389/57d574c1813c/nihms-1014858-f0002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4172/6438389/f46252d80785/nihms-1014858-f0003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4172/6438389/d8672520a3a0/nihms-1014858-f0004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4172/6438389/d5798d4512ed/nihms-1014858-f0001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4172/6438389/57d574c1813c/nihms-1014858-f0002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4172/6438389/f46252d80785/nihms-1014858-f0003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4172/6438389/d8672520a3a0/nihms-1014858-f0004.jpg

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