1/11. Malignant fibrothecomatous tumour of the ovary: diagnostic value of anti-inhibin immunostaining.Malignant ovarian tumours of the fibrothecoma group are rare. The clinicopathological features of a case of ovarian malignant fibrothecoma in which there was metastatic disease in the small intestine and peritoneum at presentation are described. A number of differential diagnoses were considered but positive immunohistochemical staining of the resected ovarian and small intestinal neoplasms with anti-inhibin was of value in confirming a sex cord-stromal tumour and in excluding other lesions. The two tumours were also ultrastructurally identical. Classical malignant fibrothecomas are said to show four or more mitotic figures per 10 high power fields (HPF). Although the intestinal secondary was mitotically active, the primary ovarian tumour contained only one to two mitoses per 10 HPF, showing that formal mitotic counts are not an absolute indicator of malignant behaviour in this group of tumours.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
2/11. Myofibroblastic tumours: neoplasias with divergent behaviour. Ultrastructural and flow cytometric analysis.myofibroblasts are spindle cells having ultrastructural features in common with smooth muscle cells and fibroblasts. In the last few years, tumours have been described in which myofibroblasts represent not only a reactive mechanism but also a true neoplastic component. They constitute new nosologic entities which might be termed "myofibroblastic tumours". Tumours with benign and, rarely, malignant behaviour are reported to belong to this group of lesions. Recently, a third tumour type with borderline biological course, named "inflammatory myofibroblastic tumour" (IMT), has been identified, a condition that has been regarded as a benign and reactive disorder for a long time. Only in recent reports has been demonstrated that, in spite of an apparently benign morphological pattern, some cases of IMT have a malignant course. In this connection, dna analysis by flow cytometry is a valuable diagnostic tool, because it allows identification of the ploidy status, a procedure that is often useful for predicting the nature and the biological behaviour of the lesion. In this study, 11 cases of myofibroblastic tumours were examined retrospectively by evaluating clinicopathological features and dna ploidy status by flow cytometry. The diagnosis of myofibroblastic tumour was confirmed by performing histology, immunohistochemistry, and electron microscopy in all patients. In detail, these 11 cases were composed of 1 benign myofibroblastoma, 1 myofibrosarcoma and 9 IMTs. Among these myofibroblastic tumours, all those with local recurrence or distant metastases (one myofibrosarcoma and three IMT) showed an aneuploid cell population demonstrable by flow cytometric analysis, whereas the other cases with benign course (one benign myofibroblastoma and six IMT) exhibited an euploid dna content. These data suggest the following: a) Besides the rare myofibroblastomas and myofibrosarcomas, IMTs represent a larger group of lesions with potentially different biological and clinical course. b) dna flow cytometric analysis is a reliable tool that support histopathological examination in characterizing those cases of IMT that, though being malignant, mimic benign lesions. Consequently, it establishes the basis for a different therapeutic approach according to the euploid or aneuploid dna content.- - - - - - - - - - ranking = 6keywords = behaviour (Clic here for more details about this article) |
3/11. Congenital infantile fibrosarcoma of the upper extremity.Congenital-infantile fibrosarcoma is an unusual childhood tumor that occurs mainly in children under 5 years of age. More than 300 cases have been reported in the literature so far, very few of them at birth. A distinction must be made between it and its adult counterpart because of differences in their clinical behaviour. We report here a case of congenital fibrosarcoma in a 4-day-old female infant.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
4/11. dermatofibrosarcoma protuberans of the scalp.dermatofibrosarcoma protuberans is an uncommon tumour of the dermis and rarely occurs on the scalp; there are few known predisposing factors. A case is described of such a tumour that occurred at the site of radiotherapy for a basal cell carcinoma. Histological characteristics, behaviour and management of dermatofibrosarcoma protuberans are discussed.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
5/11. Primary sclerosing epithelioid fibrosarcoma of the sacrum: a case report and review of the literature.Sclerosing epithelioid fibrosarcoma is a rare tumour characterised histologically by a predominant population of epithelioid cells arranged in strands and nests, embedded in a fibrotic and hyalinised stroma. It is a low grade tumour with an indolent course. A 48 year old woman presented with a painful swelling over her back for six months. Investigation and biopsy revealed features of sclerosing epithelioid fibrosarcoma involving the left half of the sacrum, left sacro-iliac joint, and posterior part of the left ilium. Preoperative radiotherapy and wide location excision of the tumour were followed by metastatic recurrence of the tumour in the lung and scalp six years after initial presentation. The tumour showed typical histology of sclerosing epithelioid fibrosarcoma. The radiological features confirmed its primary location in the sacrum. The patient declined chemotherapy and died of disseminated disease eight years after initial presentation. review of the literature confirms the fact that sclerosing epithelioid fibrosarcoma, despite its low grade, is a clinicopathologically distinct tumour with full malignant potential, the recurrence, metastasis, and mortality rate being 48%, 60%, and 35%, respectively. Sclerosing epithelioid fibrosarcoma can occur as a primary bone tumour, the clinical behaviour of which is probably similar to its soft tissue counterpart.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
6/11. Infantile rhabdomyofibrosarcoma: a distinct variant or a missing link between fibrosarcoma and rhabdomyosarcoma?Infantile rhabdomyofibrosarcoma (IRMFS) is a rare soft tissue tumour affecting infants and young children. It occupies an intermediate position between infantile fibrosarcoma and spindle cell rhabdomyosarcoma in its clinical presentation, behaviour, morphology, immunohistochemical and ultrastructural features. This case is reported here to reiterate its occurrence as tumour with distinct morphological immunohistochemical and clinical behavioral patterns.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
7/11. Congenital fibrosarcoma: presence of a histiocytic component.We describe the histologic and ultrastructural appearance of a massive soft-tissue tumor in the left lower extremity of a newborn girl. In its clinical presentation and morphologic features, this tumor corresponded to the entity currently known as congenital fibrosarcoma. The variability of histologic conformation of these lesions is emphasized. Ultrastructural study disclosed many cells with the subcellular morphology of elements of the histiocytic-macrophagic series. Whether these cells are viewed as an integral part of the tumor or as a secondary feature, their presence in a congenital fibrosarcoma is of interest for understanding of biologic behaviour of these uncommon tumors.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
8/11. Juvenile fibrosarcoma of the temporal bone.A case of juvenile fibrosarcoma arising from the head and neck region is described. This type of tumour should be considered as a separate entity different from the fibrosarcoma in adults because of the different clinical behaviour. The symptomatology, the radiographic features and the literature data are reviewed.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
9/11. Multiple inflammatory fibrosarcoma of the abdominal cavity in a child.Inflammatory fibrosarcoma is a rare condition in childhood. In the abdominal location, its behaviour is often aggressive and potentially metastasizing. We report a case of a 3-year-old female with abdominal inflammatory fibrosarcoma who relapsed after 1 month from radical surgery. Chemotherapy was ineffective, and we registered a brief stabilisation of disease only with alpha-IFN. Our case confirms the potential malignancy of this tumour and its resistance to treatment. It is noteworthy that the therapy with alpha-IFN improved the quality of life in this child for 4 months.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
10/11. Congenital fibrosarcoma. Report of three cases.Congenital/Infantile Fibrosarcomas are relatively rare soft tissue tumours. Only 238 cases have been reported till 1986 in the world literature of which 60 were truly congenital. A distinction must be made between them and their adult counterparts because of differences in their clinical behaviour. The authors report three cases and highlight their biological behaviour. Despite having an aggressive histological appearance they have a relatively benign course.- - - - - - - - - - ranking = 2keywords = behaviour (Clic here for more details about this article) |
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