Meis J M, Enzinger F M
Department of Soft Tissue Pathology, Armed Forces Institute of Pathology, Washington, D.C. 20306-6000.
Am J Surg Pathol. 1993 Nov;17(11):1103-12.
We report 20 cases of a peculiar fatty tumor that occurred in 16 female and four male patients who were 14-70 years old (median, 36 years). Most lesions were situated in the subcutis, superficial muscular fascia, or skeletal muscle of the limbs and limb girdles (15), trunk (3), and the head and neck (2). They were 1.5-11 cm in size (median, 4 cm) and usually described as yellow (13 of 15) and encapsulated (13 of 15). Microscopically they were well circumscribed and consisted of nests, strands, and sheets of eosinophilic and vacuolated cells, which contained glycogen and fat droplets, resembling brown fat cells, lipoblasts and chondroblasts. In all cases there was a variable background of mature adipose tissue associated with a prominent, partially fibrinous to hyalinized myxoid matrix that contained acid mucopolysaccharides usually resistant to hyaluronidase digestion. Several cases had foci of serous atrophy, perivascular fibrosis, and small thrombi; two were focally calcified. The lesions stained for S100 protein (11 of 12), vimentin (10 of 11), and CD68 antigen with KP1 (9 of 11); focal staining for keratin was also seen (4 of 11), but none stained for epithelial membrane antigen or actin or with HMB45. Follow-up in 12 cases (median, 9.5 years) revealed no local recurrences or metastases. Despite its deep location and atypical cellular features, the lesion's nonaggressive behavior suggests it is benign and neither a myxoid liposarcoma nor a myxoid chondrosarcoma, with which it is most frequently confused. The presence of glycogen in vacuolated fat cells is similar to brown fat, and the presence of sulfated stromal mucins supports focal chondroid differentiation. Although the pathogenesis remains uncertain, a lipoma with hibernomatous features, myxoid change, chondroid metaplasia, and secondary degenerative features is favored over a lipogranulomatous process.
我们报告了20例特殊的脂肪性肿瘤,发生于16例女性和4例男性患者,年龄在14至70岁之间(中位年龄36岁)。大多数病变位于四肢及肢带的皮下组织、浅肌筋膜或骨骼肌(15例)、躯干(3例)以及头颈部(2例)。肿瘤大小为1.5至11厘米(中位大小4厘米),通常呈黄色(15例中的13例)且有包膜(15例中的13例)。显微镜下,肿瘤边界清晰,由嗜酸性和空泡状细胞巢、条索及片状结构组成,这些细胞含有糖原和脂肪滴,类似棕色脂肪细胞、成脂细胞和软骨母细胞。所有病例中均存在不同程度的成熟脂肪组织背景,伴有显著的、部分呈纤维状至玻璃样变的黏液样基质,该基质含有通常对透明质酸酶消化有抗性的酸性黏多糖。部分病例有浆液性萎缩、血管周围纤维化及小血栓形成;2例有局灶性钙化。病变对S100蛋白染色阳性(12例中的11例)、波形蛋白染色阳性(11例中的10例)、CD6抗原KP1染色阳性(11例中的9例);也可见角蛋白局灶性染色阳性(11例中的4例),但均无上皮膜抗原或肌动蛋白染色阳性,也无HMB45染色阳性。12例患者随访(中位随访时间9.5年)未发现局部复发或转移。尽管该病变位置较深且细胞特征不典型,但其非侵袭性行为提示为良性,既不是黏液样脂肪肉瘤也不是黏液样软骨肉瘤,这两种肿瘤是其最常被混淆的对象。空泡状脂肪细胞中糖原的存在类似于棕色脂肪,硫酸化基质黏蛋白的存在支持局灶性软骨样分化。尽管发病机制仍不确定,但相较于脂肪肉芽肿过程,更倾向于诊断为具有冬眠瘤特征、黏液样改变、软骨样化生及继发性退行性变特征的脂肪瘤。