1/21. Fibrous tumors in children - a morphologic and interphase cytogenetic analysis of problematic cases.We describe and discuss the findings by fluorescent in situ hybridization (FISH) for detection of non-random chromosomal gains, in a group of unusual fibrous lesions in children. Nuclear disaggregation was used to prepare slides from eight cases which were hybridized using alpha-satellite enumeration probes for chromosomes 8, 11 and 17. trisomy 8 and 11 were detected in a high percentage of nuclei in cases of congenital/infantile fibrosarcomas (ranging from 45 to 80%), and in a low grade fibrosarcoma in an older child (23%). Only gains of chromosome 17 were detected in a case of infantile fibromatosis (22%). In this study we have found that given the unconventional histopathologic features, the detection of more than one non-random chromosomal gains by FISH, may aid in further defining fibrous tumors in children, and may be useful as an ancillary diagnostic test in the future.- - - - - - - - - - ranking = 1keywords = fibromatosis (Clic here for more details about this article) |
2/21. Desmoplastic malignant melanoma.A 68-year-old man sought dermatologic attention for a tumor of the arm. biopsy specimen showed abnormal, essentially amelanotic, spindle-shaped cells in the cutis, greatly fibrotic stroma, and focal epidermal invasion. Desmoplastic malignant melanoma was diagnosed. The lesion was widely excised and axillary lymphadenectomy performed; one node showed metastasis. Nine months later, he died with widespread metastatic disease. To our knowledge, this is the first report of this entity since its delineation in 1971 and the only case in which diagnosis was established on initial biopsy and followed by definitive therapy. Desmoplastic melanoma has been confused with benign fibrosis, invasive fibromatosis, and fibrosarcoma, and is another example, with morpheaform basal cell carcinoma and sclerodermoid metastatic lesions from breast carcinoma, in which desmoplastic stroma may obscure the epithelial nature of cutaneous neoplasm.- - - - - - - - - - ranking = 1keywords = fibromatosis (Clic here for more details about this article) |
3/21. Retroperitoneal neurofibrosarcoma in a patient with neurofibromatosis. 2: A case report and review of the literature.Neurofibromatosis (NF) type I (NF1) is the most common familial cancer-predisposing syndrome in humans, while type 2 (NF2) accounts for an extremely small percentage of the total cases of NF. Tumors occurring in patients with NF1 are primarily peripheral neurofibromas, while NF2 patients present with central schwannomas. Malignant transformation has been described in NF1 patients; however, in NF2 the risk of malignant transformation is extremely rare. In this case report, the authors document a retroperitoneal neurogenic sarcoma occurring in a 20-year-old woman with NF2 (bilateral acoustic schwannomas, meningioma, and multiple intraspinal tumors).- - - - - - - - - - ranking = 5keywords = fibromatosis (Clic here for more details about this article) |
4/21. Primitive myxoid mesenchymal tumor of infancy: a clinicopathologic report of 6 cases.Soft tissue sarcomas in the first year of life are rare, and the most common sarcomas in infancy are embryonal rhabdomyosarcoma, Ewing sarcoma/primitive neuroectodermal tumor, congenital infantile fibrosarcoma, and primitive sarcomas such as undifferentiated sarcoma. In this study, we report 6 cases of a primitive myxoid mesenchymal tumor of infancy (PMMTI), which previously may have been included under the diagnostic categories of congenital-infantile fibrosarcoma or infantile fibromatosis. PMMTI occurred in 6 infants, 3 of whom had a congenital presentation of a soft tissue mass. All patients were otherwise healthy. The tumors occurred on the trunk, extremities, and head and neck. Grossly, the tumors were nonencapsulated and had a multinodular appearance with focal infiltrative growth, a white fleshy cut surface, and a tumor diameter ranging from 2 to 15 cm. Histologically, a diffuse growth of primitive spindle, polygonal, and round cells occurred in a myxoid background. The tumor cells were arranged in a vaguely nodular pattern with peripheral collagenized stroma, higher cellularity at the periphery, and a delicate vascular network in the background. Immunohistochemically, the tumors displayed diffuse reactivity for vimentin and no reactivity for smooth muscle actin, muscle specific actin, desmin, S-100 protein, or myogenin. Electron microscopy documented a poorly differentiated fibroblastic proliferation. Four cases tested negative for the ETV6-NTRK3 gene fusion by RT-PCR. One tumor had a complex karyotypic abnormality with rearrangements involving chromosomes Y, 9, and 3. Three patients had recurrences or metastasis treated with a combination of surgery and chemotherapy. One patient is alive with persistent locally aggressive disease, 2 are alive with no evidence of recurrence, 1 had a recurrence treated surgically without further follow-up information, 1 patient died with persistent tumor and sepsis 6 weeks after diagnosis, and 1 patient was lost to follow-up. The morphologic appearance combined with the ultrastructural features and absence of the typical gene rearrangement of congenital-infantile fibrosarcoma are unique, and we propose that PMMTI represents a new category of pediatric fibroblastic-myofibroblastic tumor.- - - - - - - - - - ranking = 1keywords = fibromatosis (Clic here for more details about this article) |
5/21. Aggressive infantile fibromatosis. Pulmonary metastases documented by plain film and computed tomography.Aggressive infantile fibromatosis, one of the juvenile fibromatoses, is generally considered a benign lesion, but it does tend to recur locally because of its invasive nature. Distant metastases are considered rare. This case report documents pulmonary metastases arising 2 years after resection of the primary lesion from the thigh of an infant. This is the seventh reported case of distant metastasis from aggressive infantile fibromatosis. Computed tomography of the original lesion as well as the metastases are presented.- - - - - - - - - - ranking = 6keywords = fibromatosis (Clic here for more details about this article) |
6/21. The ubiquitous fibroblast. Multiple oncogenic potentials with illustrative cases.The wide range of oncogenic proliferative potentials of the fibroblast is demonstrated with a series of eight patients. Diagnoses included infantile digital fibromatosis, "aggressive fibromatosis," aggressive fibromatosis progressing to poorly differentiated sarcoma, infantile myofibromatosis, recurrent desmoid tumor, fibrosarcoma arising in a keloid, dermatofibrosarcoma protuberans, and malignant fibrous histiocytoma of left atrium. Still other types of fibroblastic tumefactions might have been included. Oncogenic factors that may have been operative in the causation of the lesions presented include: genetic factors, sex-linked factors, hormonal factors, numerous growth factors, and certain viruses, especially retroviruses. Certain fibromatoses in children are commonly self-limited and need only be monitored carefully as the process regresses. Aggressive fibromatosis, on the other hand, can prove fatal if the lesion is not completely resected with a wide margin and, occasionally, the process may become frankly malignant, with metastases. The standard triad of excisional surgery, radiotherapy, and chemotherapy has been used to treat frankly malignant fibrous tumors with variable results.- - - - - - - - - - ranking = 5keywords = fibromatosis (Clic here for more details about this article) |
7/21. Fibrosarcoma mimicking breast fibromatosis.Fewer than 1% of malignant breast tumors are sarcomas. Lack of correlation between the biologic behavior and histopathologic appearance of these tumors has been reported. A well-differentiated fibrosarcoma could be mistaken for breast fibromatosis, which may be a familial trait and may occur in gardner syndrome. Forty-three cases of mammary fibromatosis have been reported. Most authors are concerned with misdiagnosis of breast fibromatosis as a malignant lesion leading to unnecessary mastectomy. However, the result can be disastrous when a well-differentiated fibrosarcoma is diagnosed as fibromatosis and the patient develops lung metastases.- - - - - - - - - - ranking = 8keywords = fibromatosis (Clic here for more details about this article) |
8/21. Primary intraspinal fibrosarcoma.Fibrosarcomas of dura mater are very rare tumors representing only 0.5% of all central nervous system sarcomas. A higher incidence of dural fibrosarcomas has been reported in patients with neurofibromatosis. We present a case of spinal dura mater fibrosarcoma in a patient without neurofibromatosis. The diagnostic and therapeutic strategy in the clinical management is discussed.- - - - - - - - - - ranking = 2keywords = fibromatosis (Clic here for more details about this article) |
9/21. Aggressive fibromatosis. Report of two cases with rare clinicopathological manifestations.Two cases of aggressive fibromatosis are described, one with tricentric origin and with the first tumor originating in the hand, the other with malignant transformation into a myxoid fibrosarcoma, grade III. The primary treatment of aggressive fibromatosis should be wide excision, and the follow-up should be long, taking into account the possibility of multicentricity and the risk of malignant transformation.- - - - - - - - - - ranking = 6keywords = fibromatosis (Clic here for more details about this article) |
10/21. Fibromatosis and fibrosarcoma of the larynx and pharynx in an infant.A 14-month-old infant had congenital solitary fibromatosis of the larynx that showed fibrosarcomatous change and that spread to the pharynx and the anterior part of the neck. The histologic condition is discussed, as is the surgical treatment with total laryngopharyngectomy. A five-year postoperative follow-up is included.- - - - - - - - - - ranking = 1keywords = fibromatosis (Clic here for more details about this article) |
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