Department of Pathology, The University of Texas M.D. Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX, USA.
Am J Surg Pathol. 2011 Oct;35(10):1450-62. doi: 10.1097/PAS.0b013e31822b3687.
Cases listed in the U.T.M.D Anderson Cancer Center files as low-grade fibromyxoid sarcoma and originally diagnosed before 2004 were reviewed. They were included in the study if the diagnosis was confirmed and if there was adequate histologic material and clinical information with at least 5 years of follow-up. Thirty-three cases met the study criteria. The patients were 6 to 52 years old at the time of diagnosis (median, 29 y); 19 were male and 14 were female. The most common tumor locations were the shoulder area (5), thigh (5), and inguinal area (4). Tumor size varied from 1.5 to 16 cm (median, 9.4 cm) in those cases in which it was known. The typical histologic findings were contrasting fibrous and myxoid areas, moderate to low cellularity, bland-appearing spindle cells with no or slight nuclear pleomorphism and rare mitotic figures, and a swirling, whorled growth pattern. Prominent vascularity in myxoid areas and perivascular hypercellularity were fairly common, whereas larger hypercellular zones were sometimes seen in primary tumors but were more frequent in recurrences (local) and metastases. Hypercellular regions sometimes had round rather than spindle cells, a diffuse sheet-like cell arrangement, and/or a somewhat increased mitotic rate. Very hypocellular fibrotic areas were also observed and sometimes had thick collagen bundles. Pericollagenous rosettes were present in 6 cases but not in all specimens from these. Other growth pattern variations included storiform, fascicular-herringbone, and patternless areas; uncommonly noted were cell clusters, strands, palisades, and a retiform network. Additional unusual features were moderate nuclear pleomorphism (seen mostly in recurrent and metastatic tumors), cysts, osseous metaplasia, and a tigroid pattern with alternating fibrous and myxoid strips. One patient had a recurrence with features of sclerosing epithelioid fibrosarcoma, whereas 2 had dedifferentiated recurrences with anaplastic predominantly round cells and numerous mitotic figures. Fourteen patients died of tumor after 3 (this patient's tumor became dedifferentiated) to 42 years (median, 15 y). Nineteen patients were alive at last follow-up of 5½ to 70 years (median, 13 y), 6 with tumor and 13 without. Twenty-one patients had recurrence after intervals of up to 15 years (median, 3½ y), and 15 had metastases (mostly in the lungs and pleura) after periods of up to 45 years (median, 5 y). Except for dedifferentiation, which led to short survival after it occurred, histologic differences were not related to tumor behavior or patient survival. The 4 patients whose neoplasms measured <3.5 cm were all tumor free at last follow-up.
在 U.T.M.D.安德森癌症中心的档案中被列为低度纤维黏液样肉瘤的病例，并且在 2004 年前被诊断为低度纤维黏液样肉瘤的病例进行了回顾。如果诊断得到确认，并且有足够的组织学材料和至少 5 年的随访临床信息，则将其纳入研究。符合研究标准的有 33 例。这些患者在诊断时的年龄为 6 至 52 岁（中位数为 29 岁）；19 名男性，14 名女性。最常见的肿瘤部位为肩部（5 例）、大腿（5 例）和腹股沟区（4 例）。在已知肿瘤大小的情况下，肿瘤大小从 1.5 至 16 厘米不等（中位数为 9.4 厘米）。典型的组织学表现为纤维和黏液样区域对比鲜明，细胞数量中等至低，外观温和的梭形细胞核无或轻度多形性，罕见有丝分裂象，呈漩涡状、盘旋状生长模式。黏液样区域的明显血管生成和血管周围细胞增生相当常见，而在原发性肿瘤中有时会出现较大的细胞增生区，但在复发（局部）和转移中更为常见。有时，细胞增生区的细胞呈圆形而非梭形，细胞排列呈弥漫片状，并且/或者有较高的有丝分裂率。也观察到非常少细胞的纤维性区域，有时有厚的胶原束。在 6 例中存在围绕胶原的玫瑰花结，但并非所有这些标本都存在。其他生长模式的变化包括束状、鱼骨状和模式不明区域；偶尔出现细胞簇、条索、栅栏状和网状结构。其他不常见的特征包括中度核多形性（主要见于复发和转移肿瘤）、囊肿、骨化生和交替的纤维性和黏液性条带的波纹状图案。1 例患者的复发病灶具有硬化性上皮样纤维肉瘤的特征，而 2 例患者的复发病灶具有去分化，主要为圆形细胞和大量有丝分裂象。14 例患者在诊断后 3（该患者的肿瘤发生去分化）至 42 年（中位数为 15 年）因肿瘤死亡。19 例患者在最后随访时存活，随访时间为 5 年半至 70 年（中位数为 13 年），其中 6 例有肿瘤，13 例无肿瘤。21 例患者在间隔长达 15 年的时间后复发，15 例患者在间隔长达 45 年的时间后发生转移（主要在肺部和胸膜）。除去分化外，其发生后导致生存时间缩短，组织学差异与肿瘤行为或患者生存无关。肿瘤直径<3.5cm 的 4 例患者在最后随访时均无肿瘤。
