Angiomatoid "malignant" fibrous histiocytoma: A clinicopathologic study of 158 cases and further exploration of the myoid phenotype
Angiomatoid "malignant" fibrous histiocytoma: A clinicopathologic study of 158 cases and further exploration of the myoid phenotype
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DOI：
10.1016/s0046-8177(99)90065-5
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发表时间：
1999-11-01
期刊：
影响因子：
3.3
通讯作者：
Miettinen, M
中科院分区：
文献类型：
--
作者：
Fanburg-Smith, JC;Miettinen, M
血管瘤样“恶性”纤维组织细胞瘤（AMFH）一直被认为是儿童时期的一种低级别肉瘤，由于其纤维性假包膜、血管瘤样改变、密集的淋巴浆细胞反应以及纺锤状或圆形细胞的增殖，已被归类为纤维组织细胞肿瘤。我们希望研究大量此类肿瘤的临床病理和免疫表型特征，特别是进一步探讨其肌样分化。从AFIP的软组织登记处检索1979年至1995年编码为AMFH的病例。仅纳入光镜下符合AMFH标准的病例，共158例。98例进行免疫组化。92%的病例临床病史显示男女比例为1.8，年龄范围2 ~ 71岁，中位体长2.0 cm，四肢b>躯干b>头颈部分布，66%病变发生在正常淋巴组织区域。所有可切除的肿瘤边界均清晰。80%的病例有不同程度的淋巴浆细胞浸润；50X例假血管间隙充血，52%以圆形细胞形态为主；48%以梭形细胞为主。51%的病例Desmin阳性，主要发生在圆细胞和梭形细胞肿瘤中。多数desmin阳性伴邻近淋巴样浸润的病例（67%）表现为散在性相似，肿瘤周围淋巴样浸润可见desmin阳性细胞，肿瘤附近有14%可见肌肉特异性和平滑肌肌动蛋白。3%的病例Heavy-caldesmon阳性，73%的病例calponin局部阳性，12%的病例calponin广泛阳性。MyoD1、肌红蛋白和myf4在所有肿瘤中均为阴性。45%的病例CD99阳性；其中52%为圆细胞形态。15%的病例kp - 1呈阳性。所有肿瘤均呈波形蛋白阳性，CD21、CD35、S100蛋白、CD34、角蛋白8/18和溶菌酶阴性。86例患者的临床随访显示，1年内仅有1例患者存活并伴有局部淋巴结转移（转移率为1%），2例局部复发，平均随访时间为6年。这些病变的肌样细胞，主要是肌成纤维细胞，表型由desmin、钙钙蛋白和偶尔的肌动蛋白阳性支持。偶尔出现的重caldesmon和平滑肌肌动蛋白也提示少见的平滑肌表型；然而，骨骼肌标志物的缺乏表明AMFH与骨骼肌肿瘤没有关系。这些病变在临床和形态学上与淋巴结相似，在邻近淋巴样浸润中发现类似的desmin阳性细胞，并且66%的病例在正常淋巴样组织中发现，这一事实提高了这些病变中的一些可能是由淋巴样组织的肌样细胞引起的或与之有关。AMFH几乎都是良性的，但1%的转移率使其被归类为低级别的“恶性”。以圆形细胞为主、cd99阳性和desmin阳性的AMFH病例不应分别与尤文氏肉瘤/PNET或横纹肌肉瘤混淆。版权所有(C) 1999由W.B. Saunders公司。
Angiomatoid "malignant" fibrous histiocytoma (AMFH) has been considered to be a low-grade sarcoma of childhood, and, with its fibrous pseudocapsule, angiomatoid change, dense lymphoplasmacytic response, and proliferation of spindled or round cells, has been classified as a fibrohistiocytic neoplasm. We canted to study the clinicopathologic and immunophenotypic features of a large number of these tumors and to especially further explore their myoid differentiation. Cases coded as AMFH from 1979 to 1995 were retrieved from the Soft Tissue Registry of the AFIP. Only cases that met the criteria for AMFH by light microscopy were included, a total of 158 cases. Immunohistochemistry was obtained on 98 cases. Clinical history on 92% of ail cases revealed a gender ratio of 1.8 females: males, age range of 2 to 71 years, median size of 2.0 cm, and a distribution of extremities > trunk > head and neck, with 66%, lesions occurring in areas of normal lymphoid tissue. All tumors with available margins were well-circumscribed. Eighty percent of cases had some degree of lymphoplasmacytic infiltration; 50X cases had pseudovascular spaces filled with blood Fifty-two percent had predominantly round cell morphology; 48% had a predominantly spindle cell pattern. Desmin positivity was noted in 51% cases and occurred in both predominantly round cell and spindle cell tumors. Most of the desmin-positive cases with adjacent lymphoid infiltrate (67%) showed scattered similar, desmin-positive cells in the surrounding lymphoid infiltrate, adjacent to the tumor, Muscle-specific and smooth-muscle actins were seen in 14% cases. Heavy-caldesmon was strongly positive in 3%, and calponin was focally positive in 73% and extensively positive in 12% cases. MyoD1, myoglobin, and myogenin (myf4) were negative in all tumors studied. Forty-five percent of cases were positive for CD99; 52% of these had round cell morphology. Fifteen percent of cases were positive for KP-I. All tumors were positive for vimentin and negative for CD21, CD35, S100 protein, CD34, keratins 8/18, and lysozyme. Clinical follow-up on 86 patients indicated that only 1 patient was alive with a local nodal metastasis (1% frequency of metastasis) within 1 year, and 2 others had local recurrence, all over a mean follow-up period of 6 years. The myoid, primarily myofibroblastic, phenotype of these lesions is supported by desmin, calponin, and occasional actin positivity. The occasional heavy-caldesmon and smooth muscle actin additionally suggest rare smooth muscle phenotype; however, lack of skeletal muscle markers indicate no relationship of AMFH to skeletal muscle tumors. The resemblance of these lesions to lymph nodes, clinically and morphologically the finding of similar desmin positive cells in the adjacent lymphoid infiltrate, and the fact that 66% cases were found in sites of normal lymphoid tissue raise the possibility that some of these lesions may arise from or be related to myoid cells of lymphoid tissue. AMFH has an almost invariably benign behavior, but the 1% metastatic rate warrants its classification as low-grade "malignant." The predominantly round cell, CD99-positive and desmin positive AMFH cases, respectively, should trot be confused with Ewing's sarcoma/PNET or rhabdomyosarcoma, respectively. Copyright (C) 1999 by W.B. Saunders Company.