1/57. Intimal-type primary sarcoma of the aorta. Report of a case with evidence of rhabdomyosarcomatous differentiation.We report an intimal sarcoma presenting as an aortic aneurysm. A 68-year-old man suffered from chest pain and speech disturbance. Computed tomography showed a sacciform aneurysm of the aorta, which was resected, revealing a polypoid tumour measuring 1.5x2x2.5 cm projecting into the lumen. This proved to be a poorly differentiated high-grade sarcoma having morphological, immunophenotypic and ultrastructural features consistent with rhabdomyosarcomatous differentiation. Primary sarcomas of the aorta are extremely rare. Many cases have been diagnosed as "intimal" on the basis of their site of origin, and they are not easy to classify from their histological pattern. Electron microscopy and the use of a more comprehensive panel of immunohistochemical markers should be applied in the histological classification of"intimal" sarcoma.- - - - - - - - - - ranking = 1keywords = rhabdomyosarcoma (Clic here for more details about this article) |
2/57. Pleomorphic large cell sarcoma of the spleen with rhabdomyosarcomatous differentiation.An unusual case is reported of pleomorphic large cell sarcoma of the spleen with rhabdomyosarcomatous differentiation in a 34-year old male. According to our knowledge, such a neoplasm has never been reported in the literature.- - - - - - - - - - ranking = 1keywords = rhabdomyosarcoma (Clic here for more details about this article) |
3/57. Sarcomatoid renal cell carcinoma with scant carcinomatous components.A 30-year-old male underwent radical nephrectomy for a right renal tumor 15 cm in diameter. On microscopic examination of initial 17 sections, the tumor consisted of pleomorphic giant cells and spindle neoplastic cells. There was no carcinomatous component. Immunohistochemically, the neoplastic cells were negative for keratin and epithelial membrane antigen but positive for vimentin. The giant cells were also scatteringly, weakly positive for myoglobin. At that time a diagnosis of rhabdomyosarcoma of the kidney was made. However, further microscopic examination of another eight sections revealed small areas of clear cell-type renal cell carcinoma (RCC) which transited to sarcomatous components and led to a diagnosis of sarcomatoid RCC. The patient underwent three cycles of adjuvant chemotherapy. He has been free of the disease for 14 months after nephrectomy.- - - - - - - - - - ranking = 0.2keywords = rhabdomyosarcoma (Clic here for more details about this article) |
4/57. Detection of isolated distant metastasis in soft tissue sarcoma by fluorodeoxyglucose positron emission tomography: case report.Fluorodeoxyglucose (FDG), labeled with F-18, is a glucose analog that accumulates in cells in proportion to the rate of glucose metabolism, and increased carbohydrate metabolism has been recognized as a feature of malignant cells versus normal cells. In addition, it permits the detection of metastases not discovered by bone scan. Although detection of the primary site of disease is usually accomplished well with conventional techniques, the performance of FDG positron emission tomography (PET) may be useful to determine metastases that are not clinically evident. The authors describe a case of early detection of distant metastases by FDG-PET in a young patient diagnosed with rhabdomyosarcoma of the hand.- - - - - - - - - - ranking = 0.2keywords = rhabdomyosarcoma (Clic here for more details about this article) |
5/57. A highly aggressive primitive mesenchymal tumor with a translocation (1;19)(q12;q13.2).Soft tissue sarcomas constitute a heterogeneous group of malignant tumors of mesenchymal origin, the classification of which may present a diagnostic challenge. We present here the cytological, histopathological, immunohistochemical, and cytogenetic findings of an unusual case of a highly aggressive sarcoma. Based on the morphology and the immunohistochemical profile, this primitive tumor and its metastases could not be conclusively classified as any of the defined subtypes of sarcomas, although the findings were suggestive of a variant of rhabdomyosarcoma. Cytogenetic characterization using G-banding, SKY, FISH, and CGH revealed almost identical chromosomal compositions of the primary tumor and the metastasis. The hypertetraploid karyotype was characterized by numerical imbalances as well as by an unbalanced translocation t(1;19)(q12;q13.2), which has not been previously reported.- - - - - - - - - - ranking = 0.2keywords = rhabdomyosarcoma (Clic here for more details about this article) |
6/57. Malignant islet cell tumor with sarcomatous differentiation.Malignant mesenchymal neoplasms of the pancreas are rare and malignant islet cell tumors with sarcomatous dedifferentiation are rarer still. We present a case of malignant islet cell tumor with sarcomatous differentiation, which to our knowledge is only the second reported case showing such a combination of morphologic features. Clinically, the neoplasm was not hormonally active and immunohistochemical staining was negative for gastrin, glucagon, insulin and somatostatin. The sarcomatous component strongly reacted with an antibody directed against vimentin, and a minority of cells stained strongly with antisera directed against desmin and smooth muscle actin. The spindle cell component was nonreactive with antibodies directed against factor viii. The myogenous direction of differentiation in the present tumor is similar to that seen in the prior case report of malignant islet cell tumor with rhabdomyosarcomatous differentiation.- - - - - - - - - - ranking = 0.2keywords = rhabdomyosarcoma (Clic here for more details about this article) |
7/57. rhabdomyosarcoma of the uterus: report of two cases, including one of the spindle cell variant.Most uterine sarcomas fall into the category of leiomyosarcoma, endometrial stromal sarcoma, or undifferentiated sarcoma. Pure rhabdomyosarcomas are extremely rare, although a rhabdomyosarcomatous element may be present as a component of an adenosarcoma or carcinosarcoma (malignant mixed mullerian tumor). This report describes two uterine rhabdomyosarcomas in 28- and 67-year-old women. These were of spindle cell and pleomorphic types, respectively. At presentation the pleomorphic rhabdomyosaroma was stage IV, exhibiting massive pelvic and abdominal dissemination that mimicked an ovarian neoplasm. The spindle cell rhabdomyosarcoma was stage I, being confined to the uterus. Grossly, both uterine tumors had a polypoid appearance. Immunohistochemically, tumor cells were positive with the skeletal muscle markers sarcomeric actin, myoglobin, and myoD1. The patient with stage IV disease died within a short time of diagnosis and the other patient is alive and well at 2 years' follow-up. This report adds to the published literature on uterine rhabdomyosarcomas. This is the first reported uterine case of the spindle cell variant of embryonal rhabdomyosarcoma. Based on these cases and the published literature, rhabdomyosarcoma, especially the pleomorphic variant, appears to be a very aggressive neoplasm with an extremely poor prognosis. Immunohistochemical demonstration of skeletal muscle differentiation is necessary for a definitive diagnosis.- - - - - - - - - - ranking = 1.4keywords = rhabdomyosarcoma (Clic here for more details about this article) |
8/57. Spindle cell lesions of the head and neck mimicking rhabdomyosarcoma in children.Malignancies of the head and neck are uncommon among children. The most common solid tumors to occur in this region are rhabdomyosarcoma, hodgkin disease, and non-Hodgkin lymphoma. Two children are described who presented with signs and symptoms consistent with rhabdomyosarcoma, but who were found to have benign spindle cell lesions. These rare lesions should be part of the differential of children with a head and neck mass.- - - - - - - - - - ranking = 1.2keywords = rhabdomyosarcoma (Clic here for more details about this article) |
9/57. dna ploidy and karyotype in recurrent and metastatic soft tissue sarcomas.To study mechanisms involved in evolution of soft tissue sarcomas, we compared dna ploidy and karyotypes at different stages of their disease in two patients with myxoid liposarcomas (MLS), one with a fibrosarcoma (FS), and two with rhabdomyosarcomas (RMS). None of the MLS samples revealed clearcut histologic changes in later samples as compared to their primaries, and the dna ploidy in all samples was diploid. In one patient karyotypes at four different times during the 19 yr of his disease all revealed a t(11;12) (p15;q13), but additional clonal chromosomal abnormalities occurred only in later recurrences. In another patient the karyotypes obtained in the 26th and 28th yr of his disease were similar and included the t(12;16) (q13;p11), characteristic of MLS. A comparison with karyotypes of six other MLS patients at different disease stages suggests that the presence of a t(12;16) may correlate with less aggressive clinical behavior. The histology of the FS remained low-grade and the dna ploidy diploid. The karyotype, however, showed evolution. In both MLS and FS, chromosomal changes thus seem to be a more sensitive marker for tumor progression than histologic changes or dna ploidy. In one embryonal RMS, karyotypes obtained 7 and 11 yr after the primary diagnosis were different but clearly had a common "progenitor." In one alveolar RMS, the primary and the synchronous lung and lymph node metastases all revealed a t(2;13). The findings in RMS suggest that polyploidization is an early event in tumor evolution, especially in the alveolar subtype, which may be followed by additional chromosomal changes. In addition, dna ploidy was measured in eight other RMSs. Among the RMSs the embryonal subtype was characterized by dna aneuploidy, whereas three of the alveolar cases were in the tetraploid range and one was peridiploid. In local recurrences and in metastases changes in dna index were observed in half the cases.- - - - - - - - - - ranking = 0.2keywords = rhabdomyosarcoma (Clic here for more details about this article) |
10/57. Pleomorphic sarcoma of the adult urinary bladder: sonographic findings.Sarcomas with muscular differentiation (eg, rhabdomyosarcoma, leiomyosarcoma, pleomorphic sarcoma) are uncommon in the adult urinary bladder. We report the sonographic findings in a case of pleomorphic sarcoma with muscular differentiation arising from the bladder dome that mimicked urachal carcinoma; findings of correlative imaging are also discussed.- - - - - - - - - - ranking = 0.2keywords = rhabdomyosarcoma (Clic here for more details about this article) |
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