Isomorphic response in eruptive xanthomas.
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Biomedical subjects
Publications and source records attributed to A Gurrera.
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AIMS: The ultrastructural detection of leiomyomatous rather than myofibroblastic features in some cases of myofibroblastoma of the breast led some electron microscopically orientated pathologists to doubt the commonly accepted myofibroblastic nature of such a tumour, so the alternative terms 'myogenic stromal tumour' or 'variant of leiomyoma' have been proposed. The aim of this study was to analyse the immunohistochemical expression of h-caldesmon, a reliable marker in distinguishing smooth muscle versus myofibroblastic cellular differentiation, in a large series of myofibroblastomas of the breast to clarify whether these tumours are basically leiomyomatous. Moreover, cases from primary myofibroblastic lesions of the breast, such as fibromatosis and inflammatory myofibroblastic tumour, were analysed to assess whether h-caldesmon expression parallels that observed in their soft tissue counterparts. METHODS AND RESULTS: Paraffin-embedded sections from 12 cases of myofibroblastoma, seven cases of fibromatosis, and one case of inflammatory myofibroblastic tumour were evaluated immunocytochemically for the expression of h-caldesmon. As expected, all myofibroblastic lesions failed to express h-caldesmon. Conversely, focal staining, ranging from 2% to 10% of neoplastic cells, was detected in myofibroblastomas, even though it was restricted to 50% of analysed cases. CONCLUSIONS: Our results, indicating that smooth muscle differentiation occurs in a minority of the myofibroblastoma cells exclusively in half of the analysed cases, support the separation of myofibroblastoma from leiomyoma. The detection of smooth muscle cells in breast myofibroblastoma is easily explained if we postulate its histogenesis from the CD34+ fibroblasts of mammary stroma capable of multidirectional mesenchymal differentiation, including smooth muscle. We recommend retention of the term myofibroblastoma for all the desmin-positive and/or alpha-smooth muscle actin-positive spindle cell tumours of the breast consistent with the previously well-established morphological criteria for such neoplasms, unless one is dealing with a typical leiomyoma easily recognizable at light microscopy.
Hepatic biliary cystadenoma is a well-delineated neoplasm in some domestic animals, especially in cats, but it has not been reported in equines. We report on a case of hepatic biliary tumor, incidentally found in a 10-year-old horse, with gross and microscopic features similar to those observed in biliary adenofibroma of humans. The tumor presented as a solid mass measuring 16 cm in diameter and histologically was composed of complex tubulocystic biliary components embedded in an abundant fibrotic stroma. We regarded this tumor as a morphological variant of biliary cystadenoma of domestic animals. Differential diagnoses from other hepatic biliary tumor-like and tumor lesions are provided.
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.
We describe a case of persistent mullerian duct syndrome incidentally found in a 79-year-old man. It was characterized by the presence of a uterus-like retroperitoneal mass composed of an ovoidal corpus and rudimental portio with a central lumen. Histologically the mass was leiomyomatous in nature, closely resembling myometrium, while the central lumen was lined by a monolayer of ciliated cuboidal to columnar epithelium similar to embryo-fetal celomatic epithelium. Although this is a dysembryogenetic syndrome due to the lack of regression of the mullerian duct, it may be incidentally discovered in elderly patients, as in the present case during hernioplastic surgery. We also briefly discuss the regression of mullerian duct, focusing on morphological features observed in a large collection of human embryos and fetuses, in order to better understand the pathogenesis of a "uterus' in a male without evidence of sexual abnormalities.
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