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纤维组织细胞性脂肪瘤:12例一种此前未描述过的良性脂肪肿瘤。

Fibrohistiocytic lipoma: twelve cases of a previously undescribed benign fatty tumor.

作者信息

Marshall-Taylor C, Fanburg-Smith J C

机构信息

Department of Pathology, Tufts University, New England Medical Center, Boston, MA, USA.

出版信息

Ann Diagn Pathol. 2000 Dec;4(6):354-60. doi: 10.1053/adpa.2000.19396.

Abstract

A lipoma with a spindled proliferation within it, resembling known (myo)fibroblastic lesions such as fibrous histiocytoma or dermatofibrosarcoma protuberans, (ie, fibrohistiocytic lipoma), has not been previously reported. This tumor varies from other classic lipoma variants, including spindle cell lipoma, myolipoma, angiolipoma, and fibrolipoma. We examine the clinicopathologic findings of this new lipoma variant. The Soft Tissue Pathology Registry of the Armed Forces Institute of Pathology was searched for patients with "lipoma with fibrohistiocytic proliferation." Lesions that were better classified as other entities were excluded. Patient slides and clinical history, including associated lesions, family history, duration of symptoms, history of trauma, natural progression, and treatments, were reviewed. Immunohistochemistry was performed on cases with available material (n = 6). Twelve patients with fibrohistiocytic lipoma were included. All tumors revealed a well-distributed quilt-like proliferation or solid focus of slightly plump to relatively bland spindled cells with collagenous stroma in short fascicular and storiform growth patterns. These spindled cells resembled those seen in either fibrous histiocytoma or dermatofibrosarcoma protuberans. However, the spindled proliferation was all within a well-circumscribed lipoma. The lesions lack the dermal involvement or plump pleomorphism of fibrous histiocytoma and the dermal involvement or infiltrative growth pattern of dermatofibrosarcoma protuberans. The fatty component demonstrated heterogeneously sized adipocytes, as those seen in other lipomas. Inflammation and hemosiderin were minimal. Mast cells were not identified. The tumors were typically found in the subcutis of the trunk of men (10 of 12; one each on the wrist and leg; mean age, 31 years). The average size of the lesions was 3.0 cm, and they were present for a mean duration of 10 months prior to surgical excision. One patient had two concurrent lesions; all others had solitary tumors. Another patient had a intracranial dermoid cyst removed during childhood. Four patients had a personal or family history of hypercholesterolemia, hypertension, or myocardial infarction. There was no history of antecedent trauma. Cases studied were positive for vimentin, calponin (5 of 5), CD34 (3 of 5), and occasionally KP-1 or lysozyme in the spindled component, and all cases studied were negative for the actins, caldesmon, S-100 protein, desmin, cytokeratins, and epithelial membrane antigen. Although the actins were negative in our laboratory, the more sensitive calponin positivity suggests myofibroblastic phenotype of the spindled component of this lesion. CD34-positive fibroblasts were present in three of five cases. Of eight patients with follow-up, there were no recurrences; all patients were alive and free of disease over a mean of 10 years (range, 2 months to 31 years). We have identified a lipoma variant, fibrohistiocytic lipoma, that has not been previously described. In our experience the morphology and calponin positivity suggest myofibroblastic phenotype for the spindled cells, within a lipoma. This entity can be distinguished from fibrous histiocytoma, fibromatosis, dermatofibrosarcoma protuberans, spindle cell lipoma and other lipoma, and liposarcoma variants.

摘要

脂肪瘤内部存在梭形细胞增生,类似已知的(肌)纤维母细胞性病变,如纤维组织细胞瘤或隆突性皮肤纤维肉瘤(即纤维组织细胞性脂肪瘤),此前尚未见报道。该肿瘤与其他经典脂肪瘤变体不同,包括梭形细胞脂肪瘤、肌脂肪瘤、血管脂肪瘤和纤维脂肪瘤。我们研究了这种新的脂肪瘤变体的临床病理特征。检索武装部队病理研究所软组织病理登记处中诊断为“伴有纤维组织细胞增生的脂肪瘤”的患者。排除那些更适合归类为其他疾病的病变。复查患者的切片和临床病史,包括相关病变、家族史、症状持续时间、外伤史、自然病程和治疗情况。对有可用材料的病例(n = 6)进行免疫组织化学检测。纳入12例纤维组织细胞性脂肪瘤患者。所有肿瘤均表现为分布均匀的类似棉被样增生或实性病灶,由轻度丰满至相对温和的梭形细胞构成,伴有胶原性间质,呈短束状和席纹状生长模式。这些梭形细胞类似于纤维组织细胞瘤或隆突性皮肤纤维肉瘤中的细胞。然而,梭形细胞增生均位于边界清楚的脂肪瘤内。这些病变缺乏纤维组织细胞瘤的真皮受累或丰满多形性,以及隆突性皮肤纤维肉瘤的真皮受累或浸润性生长模式。脂肪成分显示出大小不一的脂肪细胞,与其他脂肪瘤所见相同。炎症和含铁血黄素极少。未发现肥大细胞。肿瘤通常见于男性躯干的皮下组织(12例中有10例;手腕和腿部各1例;平均年龄31岁)。病变的平均大小为3.0 cm,手术切除前平均存在10个月。1例患者有2个同时存在的病变;其他患者均为单发肿瘤。另1例患者童年期切除过颅内皮样囊肿。4例患者有高胆固醇血症、高血压或心肌梗死的个人或家族史。无既往外伤史。所研究病例的梭形细胞成分中波形蛋白、钙调蛋白(5/5)、CD34(3/5)呈阳性,偶尔KP-1或溶菌酶呈阳性,所有研究病例的肌动蛋白、钙调蛋白、S-100蛋白、结蛋白、细胞角蛋白和上皮膜抗原均为阴性。虽然我们实验室检测肌动蛋白为阴性,但更敏感的钙调蛋白阳性提示该病变梭形细胞成分具有肌纤维母细胞表型。5例中有3例存在CD34阳性的成纤维细胞。8例有随访的患者均无复发;所有患者均存活,平均10年(范围2个月至31年)无疾病。我们发现了一种此前未描述过的脂肪瘤变体——纤维组织细胞性脂肪瘤。根据我们的经验,其形态和钙调蛋白阳性提示脂肪瘤内梭形细胞具有肌纤维母细胞表型。该实体可与纤维组织细胞瘤、纤维瘤病、隆突性皮肤纤维肉瘤、梭形细胞脂肪瘤和其他脂肪瘤及脂肪肉瘤变体相鉴别。

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