Ockner D M, Sayadi H, Swanson P E, Ritter J H, Wick M R
Lauren V. Ackerman Laboratory of Surgical Pathology, Washington University Medical Center, St Louis, Missouri 63110, USA.
Am J Clin Pathol. 1997 Jan;107(1):36-44. doi: 10.1093/ajcp/107.1.36.
Angiomyofibroblastoma (AMFB) of the genital region is a relatively recently described tumor of the superficial soft tissues with a marked preference for female patients. Three cases of AMFB were reviewed, two of which involved adult men. To further elucidate the pathologic features of AMFB, these three cases were compared with 10 cases of aggressive angiomyxoma (AA), a salient diagnostic alternative, and 28 cases of other myxoid tumors that may show morphologic similarities to these neoplasms. Conventional histologic and immunohistochemical features of AMFBs were compared with those of AA, myxoid leiomyoma, myxoid leiomyosarcoma, myxoid liposarcoma, myxoid malignant fibrous histiocytoma, myxoid neurofibroma, and myxoid malignant peripheral nerve sheath tumor. The ultrastructure of two of the three AMFBs also was analyzed. Genital AMFBs were circumscribed, partially myxoid proliferations that demonstrated considerable variation in cellular density. Neoplastic elements were bland cytologically and showed both fusiform and epithelioid profiles, with a tendency to concentrate around intralesional blood vessels. Mitotic activity and necrosis were absent, and the vessels assumed an arborizing configuration and were venule or capillary sized. In contrast, all other tumor types evaluated were infiltrative, cytologically atypical, or both. All AMFBs showed immunoreactivity for vimentin, desmin, actin, and estrogen receptor protein. These results were shared by most examples of AA and smooth muscle tumors as well, but were not seen in any other neoplasms in this study. Electron microscopic findings in cases of AMFB supported the presence of myofibroblastic differentiation in the tumor cells. These results indicate that conventional morphologic analysis is paramount in the recognition of genital AMFB but that immunohistology may be helpful in a limited context in excluding other differential diagnoses. They also support the conclusion that AMFB, AA, and superficial smooth muscle tumors have similar morphotypes and immunohistologic attributes regardless of their origin in men or women.
生殖器区域的血管肌纤维母细胞瘤（AMFB）是一种相对较新描述的浅表软组织肿瘤，明显多见于女性患者。回顾了3例AMFB病例，其中2例累及成年男性。为进一步阐明AMFB的病理特征，将这3例病例与10例侵袭性血管黏液瘤（AA）（一种重要的诊断鉴别对象）以及28例可能与这些肿瘤在形态学上有相似之处的其他黏液样肿瘤进行了比较。将AMFB的传统组织学和免疫组化特征与AA、黏液样平滑肌瘤、黏液样平滑肌肉瘤、黏液样脂肪肉瘤、黏液样恶性纤维组织细胞瘤、黏液样神经纤维瘤和黏液样恶性外周神经鞘瘤的特征进行了比较。还分析了3例AMFB中2例的超微结构。生殖器AMFB边界清晰，部分为黏液样增生，细胞密度差异较大。肿瘤细胞在细胞学上形态温和，呈现梭形和上皮样形态，倾向于聚集在瘤内血管周围。无有丝分裂活性和坏死，血管呈树枝状分布，大小为小静脉或毛细血管。相比之下，所评估的所有其他肿瘤类型均为浸润性、细胞学非典型性或两者兼具。所有AMFB对波形蛋白、结蛋白、肌动蛋白和雌激素受体蛋白均呈免疫反应性。大多数AA和平滑肌肿瘤的例子也有这些结果，但在本研究的任何其他肿瘤中均未见到。AMFB病例的电子显微镜检查结果支持肿瘤细胞存在肌纤维母细胞分化。这些结果表明，传统形态学分析对于识别生殖器AMFB至关重要，但免疫组织学在有限的情况下可能有助于排除其他鉴别诊断。它们还支持这样的结论，即无论起源于男性还是女性，AMFB、AA和浅表平滑肌肿瘤具有相似的形态类型和免疫组化特征。
