Ossifying fibromyxoid tumor of soft parts (OFMT) is a recently named soft tissue tumor of uncertain nature. A case is described that presented in a 13-year-old boy as a discrete mass in the muscles of the lower abdominal wall. Light microscopy showed, in addition to the typical features of this entity, microcysts formed by accumulations of the myxoid stroma. Bone formation was lacking. Tumor cells were strongly immunoreactive for vimentin and glial fibrillary acidic protein and weakly so for S-100 protein. A few cells stained for desmin and alpha-smooth muscle actin. Ultrastructurally, there were abundant, patternless cytoplasmic intermediate filaments; short, poorly interdigitating processes; and discontinuous segments of thick external lamina. In addition, several cells contained typical ribosome-lamella complexes in small groups. Ribosome-lamella complexes occur in neoplastic hematopoietic cells but are uncommon in solid tumors, particularly those affecting the soft tissues. These findings extend the range of appearances described for OFMT, which is added to the list of tumors in which ribosome-lamella complexes have been demonstrated. The balance of evidence suggests that OFMT may represent a peripheral nerve sheath tumor of low-grade malignancy, although the picture is incomplete.
Poorly differentiated synovial sarcoma is a variant of synovial sarcoma in which the tumor cells lack the bland spindle cell appearance of the usual type monophasic synovial sarcoma. Although poorly differentiated synovial sarcoma has been recognized as an entity for many years, no series addressing the clinicopathologic features of this variant have appeared. We describe the histologic, immunohistologic, and molecular findings of a series of 20 poorly differentiated synovial sarcomas. Three types of poorly differentiated synovial sarcoma can be recognized: a large cell epithelioid variant, a small cell variant, and a high-grade spindle cell variant. Epithelial membrane antigen reactivity was seen in 95% of cases, and reactivity for cytokeratin was seen in 42%. The S100 antigen was expressed in 63% of cases. Electron microscopic findings in poorly differentiated synovial sarcoma parallel those found in usual type synovial sarcoma. In 10 cases, material was available for molecular studies; 9 of 10 cases showed the presence of t(X;18) or the associated fusion gene product. These data indicate that poorly differentiated synovial sarcoma is a lesion that shares immunologic, ultrastructural, and molecular characteristics with the usual synovial sarcoma. Follow-up data were available in 16 patients with a mean follow-up of 39 months. Eight patients died with a mean survival time of 33 months. Poorly differentiated synovial sarcoma is a variant of synovial sarcoma that may be associated with a poor prognosis.
Summary. Sezary cell leukaemia (SCL) is a mature T-cell leukaemia with characteristic cerebriform nuclei, whereas Sezary syndrome (SS) involves a mature T-cell lymphoma with a similar nuclear morphology. We have examined these diseases by cytogenetics, chromosome painting and fluorescence in situ hybridization (FISH). Both diseases had complex cytogenetic abnormalities. All three cases of SCL investigated had inv(14)(q11;q32) and two had iso(8q). No case of SS had these abnormalities but, instead, iso(17q) or 17pþ was present in the three cases of SS investigated and FISH indicated loss of heterozygosity due to deletion of a region at 17p13 that included the tumour suppressor gene P53, implicating it in this malignancy. One case of SCL had iso(17q). The abnormalities of chromosomes 8 and 14 in SCL are commonly observed in T-prolymphocytic leukaemia (T-PLL) and suggest that SCL may be a variant of T-PLL rather than of SS.
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