Costa M J, Weiss S W
Department of Pathology, Grady Memorial Hospital, Emory University School of Medicine, Atlanta, Georgia.
Am J Surg Pathol. 1990 Dec;14(12):1126-32.
Described by Enzinger in 1979, angiomatoid malignant fibrous histiocytoma is a distinctive tumor of adolescence and early adult life characterized by sheets of relatively bland rounded or spindled cells separated by areas of cystic hemorrhage and surrounded by a prominent inflammatory infiltrate and often a fibrous pseudocapsule. On the basis of the original 41 cases, the tumor has been considered a low-grade malignancy. We are reporting the clinicopathologic findings and follow-up information of 108 new cases of angiomatoid malignant fibrous histiocytoma to determine the long-term behavior of this tumor and whether various histologic features (atypia, mitoses, infiltrative borders, and inflammatory infiltrate) are useful in predicting outcome. Follow-up information was obtained in 94 (87%) cases. Local recurrences developed in 11 patients (12%), all of whom were cured by re-excision. The initial excision in all patients developing local recurrence appeared to be incomplete. Local recurrence was statistically associated with irregular tumor border and head and neck location. Five patients developed metastases. Four had only local metastases, which responded to surgery, whereas the fifth patient developed presumed pulmonary and cerebral metastases and died. The development of both local and distant metastases was correlated with invasion into the deep fascia or muscle but not to various histologic parameters such as mitotic rate and pleomorphism. We conclude that angiomatoid malignant fibrous histiocytoma is intrinsically a low-grade tumor, and assessment of various histologic parameters or grading provides little or no additional prognostic information. Because death from disease occurred in only one patient (1%) in our series, it seems reasonable to reclassify angiomatoid malignant fibrous histiocytoma with fibrohistiocytic tumors of intermediate malignancy rather than with the conventional malignant fibrous histocytoma, the majority of which are high-grade sarcomas.
血管样恶性纤维组织细胞瘤由恩津格于1979年首次描述，是一种好发于青少年及青年早期的独特肿瘤，其特征为成片相对温和的圆形或梭形细胞，其间有囊性出血区域分隔，周围有显著的炎症浸润，且常被纤维性假包膜包裹。基于最初的41例病例，该肿瘤被认为是低度恶性肿瘤。我们报告了108例新的血管样恶性纤维组织细胞瘤的临床病理特征及随访信息，以确定该肿瘤的长期行为，以及各种组织学特征（异型性、核分裂象、浸润性边界和炎症浸润）是否有助于预测预后。94例（87%）获得了随访信息。11例患者（12%）出现局部复发，均通过再次切除治愈。所有发生局部复发的患者初次切除似乎均不完整。局部复发与肿瘤边界不规则及头颈部位置在统计学上相关。5例患者发生转移。4例仅有局部转移，手术有效，而第5例患者出现可能的肺和脑转移并死亡。局部和远处转移的发生与侵犯深筋膜或肌肉相关，但与有丝分裂率和多形性等各种组织学参数无关。我们得出结论，血管样恶性纤维组织细胞瘤本质上是一种低度恶性肿瘤，评估各种组织学参数或分级几乎不能提供额外的预后信息。由于在我们的系列研究中仅有1例患者（1%）死于该疾病，因此将血管样恶性纤维组织细胞瘤重新归类为中度恶性的纤维组织细胞肿瘤似乎是合理的，而不是归类于传统的恶性纤维组织细胞瘤，后者大多数是高度恶性肉瘤。
