Steeper T A, Rosai J
Am J Surg Pathol. 1983 Jul;7(5):463-75. doi: 10.1097/00000478-198307000-00009.
Nine case of a distinctive soft tissue tumor of the female pelvis and perineum are described. They were characterized by their occurrence in young women (ages 21-38), large size (up to 60 X 20 cm), locally infiltrative nature, and grossly gelatinous appearance. The initial clinical impression was usually that of a Bartholin gland cyst. The microscopic appearance was that of spindle or stellate cells widely separated by a loose myxoid stroma focally rich in collagen fibrils, a prominent vascular component, including many large thick-walled vessels without an arborizing pattern, and foci of proliferating glandular elements in two cases. Mitotic activity was exceedingly low. Ultrastructural study of the spindle cells showed features consistent with myofibroblastic differentiation. Four patients developed large local recurrences; one tumor recurred twice, 14 and 15 years after initial excision. No distant metastases have been documented to date, and all patients are alive and well. The differential diagnosis of this unusual tumor includes myxoma, myxoid liposarcoma, sarcoma botryoides, myxoid variant of malignant fibrous histiocytoma, nerve sheath myxoma, and other soft tissue tumors with secondary myxoid changes. We have chosen the term "aggressive angiomyxoma" for this neoplasm to emphasize the neoplastic nature of the blood vessels and its locally infiltrative and recurrent nature.
本文描述了9例女性盆腔和会阴区一种独特的软组织肿瘤。其特点为发生于年轻女性（21 - 38岁），体积较大（可达60×20 cm），具有局部浸润性，大体呈胶冻样外观。最初的临床印象通常为巴氏腺囊肿。显微镜下表现为梭形或星状细胞，被富含胶原纤维的疏松黏液样间质广泛分隔，有显著的血管成分，包括许多无分支模式的大的厚壁血管，2例有增生的腺性成分灶。有丝分裂活性极低。梭形细胞的超微结构研究显示其特征与肌成纤维细胞分化一致。4例患者出现大的局部复发；1例肿瘤在初次切除后14年和15年复发两次。迄今为止未记录到远处转移，所有患者均存活且状况良好。这种不寻常肿瘤的鉴别诊断包括黏液瘤、黏液样脂肪肉瘤、葡萄状肉瘤、恶性纤维组织细胞瘤的黏液样变型、神经鞘黏液瘤以及其他有继发性黏液样改变的软组织肿瘤。我们为这种肿瘤选择了“侵袭性血管黏液瘤”这一术语，以强调血管的肿瘤性质及其局部浸润和复发的特性。
