Magro G, Gurrera A, Scavo N, Lanzafame S, Bisceglia M
Dipartimento F.G. Ingrassia, Sezione di Anatomia Patologica, Università di Catania, Via S. Sofia 87, I-95123 Catania, Italia.
Pathologica. 2002 Oct;94(5):238-46. doi: 10.1007/s102420200039.
We describe the clinical, radiological and pathological features of a series of six cases of primary fibromatosis of the breast. Most patients (5 of 6) were women of 22-58 years of age; one case occurred in a 47-year-old man. The fibromatosis of the breast in all cases presented as a monolateral solid nodule, clinically suspicious for malignancy and in three cases was also associated with cutaneous and/or nipple retraction. None of the patients was affected by any genetic disorder characterized by fibromatoses involving multiple sites, including breast. Both echographic and mammographic examinations revealed solid masses with irregular margins but without calcifications, mimicking scirrhous carcinoma. All cases were surgically treated by lumpectomy (4 of 6) or quadrantectomy (2 of 6), and after a follow-up period ranging from 5 months to 13 years all patients are well and disease-free. Histological examination revealed finger-like infiltrating margins entrapping adjacent breast parenchyma and adipose tissue in all cases. The hallmark of the lesion was the presence of bland-looking spindle cells, with a low mitotic index, organized in long sweeping and intersecting fascicles. Three different morphological phases (i.e. proliferative, involutional and residual), identical to those observed in Dupuytren's superficial fibromatosis, variably coexisted in each case. At immunohistochemistry the cells which comprised the lesion exhibited a diffuse expression of vimentin and a heterogeneous immunoreactivity to a-smooth muscle actin, thus confirming their fibro-myofibroblastic nature. No expression of estrogen, progesterone or androgen receptors or of pS2 correlated-estrogen protein was observed. We discuss both the pathogenesis of the mammary fibromatosis, pointing to the fibroblastic cells of the mammary stroma as the putative precursors, and the differential diagnosis versus the bland-looking, monomorphic spindle cell tumor-like or tumorous lesions of the breast.
我们描述了一系列6例乳腺原发性纤维瘤病的临床、放射学及病理学特征。多数患者（6例中的5例）为22至58岁的女性；1例发生于一名47岁男性。所有病例的乳腺纤维瘤病均表现为单侧实性结节，临床上怀疑为恶性，3例还伴有皮肤和/或乳头回缩。所有患者均未患任何以包括乳腺在内的多部位纤维瘤病为特征的遗传性疾病。超声和乳腺X线检查均显示边界不规则的实性肿块，但无钙化，类似硬癌。所有病例均接受了保乳手术（6例中的4例）或象限切除术（6例中的2例），经过5个月至13年的随访，所有患者情况良好，无疾病复发。组织学检查显示所有病例均有指状浸润边缘，包绕相邻的乳腺实质和脂肪组织。病变的特征是存在外观温和的梭形细胞，有丝分裂指数低，排列成细长的、相互交叉的束状。三种不同的形态学阶段（即增殖期、退化期和残留期），与在掌腱膜浅部纤维瘤病中观察到的相同，在每个病例中不同程度地共存。免疫组织化学检查显示，构成病变的细胞波形蛋白呈弥漫性表达，对α-平滑肌肌动蛋白呈异质性免疫反应，从而证实其纤维-肌成纤维细胞性质。未观察到雌激素、孕激素或雄激素受体或pS2相关雌激素蛋白的表达。我们讨论了乳腺纤维瘤病的发病机制，指出乳腺间质的成纤维细胞为假定的前体细胞，以及与外观温和的、单形性梭形细胞瘤样或肿瘤性乳腺病变的鉴别诊断。
