1/30. Low-grade fibrosarcoma with palisaded granulomalike bodies (giant rosettes): report of a case that metastasized."Hyalinizing spindle cell tumor with giant rosettes" is a tumor recently described by Lane et al. and thought by them possibly to represent a form of low-grade fibromyxoid sarcoma. Proof of a metastatic potential was lacking. We report an example of this tumor on the arm of a 28-year-old woman. The ultrastructural study of the tumor confirmed the fibroblastic nature of the lesion, which subsequently metastasized to the lung. Histologically, the giant rosettes simulated palisaded granulomas. Our findings warrant classifying the tumor as a sarcoma, and we suggest the designation low-grade fibrosarcoma with palisaded granulomalike bodies (giant rosettes).- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
2/30. Low grade fibromyxoid sarcoma. a further low-grade soft tissue malignancy characterized by a ring chromosome.Supernumerary rings in the context of a simple karyotype characterize several low-grade malignant tumors of soft tissue and bone. Low-grade fibromyxoid sarcoma is an uncommon low-grade sarcoma, the cytogenetics of which has not yet been reported. Here we describe the first molecular-cytogenetic characterization of a pulmonary metastasis of low-grade fibromyxoid sarcoma. The histology of the primary and recurrent tumors was consistent with the diagnosis of low-grade fibromyxoid sarcoma of the usual type, whereas the pulmonary metastasis was of the "giant rosettes" variant. cytogenetic analysis revealed a ring chromosome. Because gain of material of chromosomes 7 and 16 was detected by CGH, the ring chromosome is assumed to be composed of material from these respective chromosomes.- - - - - - - - - - ranking = 0.14285714285714keywords = giant (Clic here for more details about this article) |
3/30. Hyalinising spindle cell tumour with giant rosettes: report of a case with unusual features including original histological and ultrastructural observations.Hyalinising spindle cell tumour with giant rosettes (HSCTGR) is an uncommon, recently described low-grade sarcoma which shares many histological features with low-grade fibromyxoid sarcoma (LGFMS). We report a case of HSCTGR occurring in the deep soft tissues of the thigh of a 46-year-old woman, that presented as a slowly growing, painless mass. Microscopically the tumour was composed of spindled stromal cells amongst which were scattered so-called collagen rosettes. The distinctive feature of this case was the previously unreported finding of lymphoid cells of T-cell phenotype admixed with fibrohistioctyic cells in the cellular cuff surrounding the collagenous core of the rosettes. The case was further unusual in that it included focal areas of increased cellularity with a mitotic count of up to three per 10 high-power fields. While the latter feature has been associated with increased recurrences and metastases in LGFMS, it is not known whether the significance is similar in HSCTGR. The spindled stromal cells showed ultrastructural features of poorly differentiated fibroblasts, while those at the edges of the rosettes showed features of altered fibroblasts, some with a fibrohistiocytic appearance. These findings support the interpretation that HSCTGR forms part of the spectrum of sarcomas showing fibroblastic differentiation.- - - - - - - - - - ranking = 0.71428571428571keywords = giant (Clic here for more details about this article) |
4/30. Long-term survival after multiple resections of a fibrosarcoma involving the lung and chest wall.We report on the case of a 61-year-old male patient who developed a giant fibrosarcoma involving both the lung and chest wall. This patient underwent three extended resections including the chest wall in each case. radiotherapy was administered after the last resection, when the tumor was obviously not completely removed. The patient lives a normal life with no signs of recurrence 5 years after his last resection. Multiple extended resections of large and aggressive sarcomas can result in long-term survival, with good quality of life, in adequately selected patients.- - - - - - - - - - ranking = 0.14285714285714keywords = giant (Clic here for more details about this article) |
5/30. Hyalinizing spindle cell tumor with giant rosettes: report of a case showing remarkable myofibroblastic differentiation.We examined the proliferative activity and the differentiation line of tumor cells in a case of "hyalinizing spindle cell tumor with giant rosettes" (HSCGR). A 6 cm tumor within the right deltoid muscle of a 58-year-old female was found by physical and radiographical examinations. A biopsy revealed the histological features of a spindle cell tumor with rosette-like structures. Wide excision was done under the diagnosis of HSCGR. The tumor presented as a gray-whitish, solid mass with focal pseudocystic degeneration. Immunohistochemically, the tumor cells were diffusely positive for vimentin and were also focally positive for S-100, but negative for desmin and alpha-smooth muscle actin. The cells stained positively for Ki-67 with even distribution, there being a correlation with the cellularity of the areas, with a labeling index ranging from 0.3 to 0.5%. In addition, flow cytometry revealed an almost normal diploid dna pattern and 5.8% S-phase fraction, indicating low proliferative activity. Ultrastructurally, many tumor cells displayed discontinuous basal lamina, pinocytotic vesicles, dilated rough endoplasmic reticulum, and microfilaments with focal dense bodies. The main component of the rosette was collagenous fibrils with normal diameter and normal periodic banding. We interpreted this case of HSCGR as a low grade fibrosarcoma with remarkable differentiation of myofibroblastic lineage, and with focally accumulated, morphologically normal collagenous fibrils.- - - - - - - - - - ranking = 0.71428571428571keywords = giant (Clic here for more details about this article) |
6/30. Fibronexus in "malignant fibrous histiocytoma" of the bone: a case report of pleomorphic myofibrosarcoma.The fibronexus (fibronexus junction) has been thought to be a characteristic ultrastructural feature of myofibroblasts, but it is controversial whether the fibronexus is also a characteristic of various myofibroblastic tumors. We report here a case of pleomorphic bone sarcoma (pleomorphic/storiform malignant fibrous histiocytoma) with fibronexus junctions arising in the head of the left humerus of a 70-year-old woman. By light microscopy the tumor was composed of large spindle or polygonal cells occasionally arranged in fascicles. Foamy giant cells with bizarre nuclei were not uncommon. Immunohistochemically, the tumor cells were positive diffusely for vimentin and focally for muscle actin (HHF35) and alpha-smooth muscle actin but were negative for desmin, high-molecular weight caldesmon, and S-100 protein. Ultrastructurally, the tumor cells had indented nuclei and spindle or polygonal cytoplasm, with little rough endoplasmic reticulum and small vesicles, and rather numerous mitochondria. The tumor cells had myofilaments with focal densities in the periphery, fibronectin fibrils adjacent to intracellular myofilaments, and by definition, therefore, fibronexus junctions. These findings suggest myofibroblastic differentiation, and a diagnosis of pleomorphic myofibrosarcoma is thought to be appropriate. We believe that the fibronexus is a characteristic and useful ultrastructural feature for differentiating myofibroblastic tumors from other pleomorphic myogenic sarcomas.- - - - - - - - - - ranking = 0.14285714285714keywords = giant (Clic here for more details about this article) |
7/30. Low-grade fibrosarcoma (hyalinizing spindle cell tumor with giant rosettes) with pulmonary metastases at presentation: case report and review of the literature.Hyalinizing spindle cell tumor with giant rosettes (HSCT) is presently considered a low-grade fibrosarcoma and is also considered a variant of low-grade fibromyxoid sarcoma by some. None of the HSCTs in the original series had manifested malignant behavior in the form of metastasis, but since that initial report, 2 patients have been reported with pulmonary metastases and another patient with multiple pulmonary nodules in the absence of an identifiable primary tumor. Our patient is the second recorded case of HSCT with pulmonary metastases at the time of diagnosis. A needle biopsy of the axillary mass in the present case consisted mainly of densely hyalinized collagen, whereas the lung nodules had the characteristic giant collagen rosettes surrounded by a bland spindle cell stroma. This case, in addition to at least 2 others in the literature, has demonstrated that the HSCT is a malignant neoplasm with the capacity to metastasize; however, the presence and even persistence of metastatic lesions in the lung has not altered to date an otherwise indolent clinical course. The fact should not be overlooked that the HSCT is a recently reported entity whose natural history and nosology are subjects of continuing observation, study, and discussion.- - - - - - - - - - ranking = 0.85714285714286keywords = giant (Clic here for more details about this article) |
8/30. Giant fibrosarcoma arising in dermatofibrosarcoma protuberans on the scalp during pregnancy.BACKGROUND: dermatofibrosarcoma protuberans (DFSP) is a low-grade malignant soft tissue tumor of subcutaneous tissues that has a propensity for local recurrence; however, it seldom metastasizes. Most lesions occur over the trunk or proximal extremities, and there is slight male preponderance. DFSP is rare on the head and neck. Less than 5% tumors are located on the scalp. OBJECTIVE: To describe a case of giant fibrosarcoma arising in DFSP on the scalp during pregnancy. METHOD: In our patient, the tumor was on scalp and measured 15 x 11 x 7 cm. The recurrent tumor that developed in the same region was measured 33 x 30 x 15 cm. Histopathologic examination of this mass revealed fibrosarcoma. The mass was excised from the subperiosteal plane with a 3-cm free margin. Because of the high possibility of recurrence, the defect site was reconstructed with a split-thickness skin graft. RESULTS: After 1 year of follow-up, no recurrence or metastasis was detected. CONCLUSION: We present a unique case in which the person has a giant fibrosarcoma on her scalp arising from DFSP with fibrosarcomatous change during pregnancy.- - - - - - - - - - ranking = 0.28571428571429keywords = giant (Clic here for more details about this article) |
9/30. Low-grade fibromyxoid sarcoma and hyalinizing spindle cell tumor with giant rosettes share a common t(7;16)(q34;p11) translocation.Low-grade fibromyxoid sarcoma (LGFMS) is a rare metastasizing soft tissue tumor with deceptively bland histologic features. The hyalinizing spindle cell tumor with giant rosettes (HSCT) is thought to be a closely related tumor differing only by the presence of collagen rosettes. We report the occurrence of a common t(7;16)(q34;p11) translocation in 2 cases of HSCT and 2 cases of LGFMS, thereby providing the first cytogenetic proof that LGFMS and HSCT are variants of the same entity. The tumors occurred in the thighs of 2 females and in the buttock and supraclavicular fossa of 2 males. One HSCT had a spectrum of unusual histologic features, including the presence of plump epithelioid cells with abundant cytoplasm and strands and nests of clear epithelioid cells separated by eosinophilic hyalinized stroma. Two cases showed a hitherto unreported, focal staining with epithelial membrane antigen, thus adding to the immunohistochemical profile of these tumors. LGFMS and HSCT probably have a wider spectrum of morphologic features than previously thought, the awareness of which will help pathologists to avoid diagnostic pitfalls. Demonstration of the t(7;16)(q34;p11) translocation will help to diagnose difficult cases with unusual histologic features.- - - - - - - - - - ranking = 0.71428571428571keywords = giant (Clic here for more details about this article) |
10/30. Giant cell fibroblastoma with a dermatofibrosarcoma protuberans component.We describe a unique case of giant cell fibroblastoma that contained a component of dermatofibrosarcoma protuberans in the left inguinal region of a 37-year-old man. Our case adds further proof of the close histogenetic relationship between these two tumors.- - - - - - - - - - ranking = 0.14285714285714keywords = giant (Clic here for more details about this article) |
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