1/158. bone marrow metastases in disseminated alveolar rhabdomyosarcoma: case report with ultrastructural study and review.A case of desseminated alveolar rhabdomyosarcoma in an 18-year-old male with leuco-erythroblastic anaemia is described. Numerous bizarre malignant cells, including frequent multinucleated giant cells, were seen in bone marrow aspirates, and osteolytic lesions appeared late in the clinical course. The primary site of the neoplasm remained undertermined during life and also at necropsy, which revealed minute pulmonary metastases and extensive lymph nodal, pleural and skeletal deposits. The diagnosis was confirmed on necropsy tissue by ultrastructural examination which demonstrated numerous thin (5 nm) and thick (15 nm) intracytoplasmic filaments in tumour cells, sometimes organized in bundles; scattered dense Z-band-like bodies, and rod-shaped structures were also seen. The fine structure of the rhabdomyosarcoma in the present case is compared with previous ultrastructural studies. Elongated, thick intracytoplasmic filaments whose diameter corresponds to that of myosin myofilaments are strong evidence for rhabdomyoblastic differentiation and are considered to be the sine qua non of a positive electron microscopic diagnosis of rhabdomyosarcoma. Orgaized bundles of filaments and Z-band-like dense bodies are usually present, and rod-shaped structures are found infrequently, but none of these are necessary for the ultrastructural diagnosis.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
2/158. Pleomorphic rhabdomyosarcoma of the uterus in a postmenopausal woman with elevated serum CA125.BACKGROUND: Pure rhabdomyosarcoma of the uterus is an exceedingly rare tumor. While there are no commonly associated tumor markers, a previously reported case confirmed CA125 production by a pure uterine rhabdomyosarcoma. CASE: We describe a postmenopausal woman who presented with vaginal bleeding and was diagnosed with pleomorphic rhabdomyosarcoma of the uterus. Of particular interest was an elevated serum CA125 level at presentation. Immunohistochemical staining with CA125 antibody, however, showed no activity within the tumor. Despite aggressive therapy, the patient experienced distant nodal metastases and succumbed to the disease within 25 months of diagnosis. CONCLUSION: Our patient illustrates the typical presentation and aggressive behavior of this rare neoplasm. While serum CA125 may be a useful tumor marker in selected patients, elevated levels do not directly reflect tumor burden when associated with negative immunohistochemical staining.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
3/158. rhabdomyosarcoma in a patient with cardio-facio-cutaneous syndrome.A boy with characteristic facial features, pulmonary valvular stenosis, ectodermal abnormalities, growth failure, and mental retardation was admitted for intestinal occlusion at 20 months of age. Clinical findings were consistent with a diagnosis of cardio-facio-cutaneous syndrome (CFC-s), and a huge abdominal mass was evident on computed tomography scan. A biopsy was performed, and embryonal rhabdomyosarcoma was diagnosed. Molecular analysis was performed by reverse transcription (RT) polymerase chain reaction (PCR) on tumor rna to seek the chimerical transcript of the most common soft tissue sarcoma translocations and analyze neurofibromatosis 1 (NF1) gene expression. Translocations involving 1;13, 2;13, and 11;22 were not found, and the specific transcripts of the NF1 gene were present. Chemotherapy was implemented, but the child died 7 months later of tumor progression. Few patients with CFC-s have been described, and their follow-up is not well known. The association of CFC-s with rhabdomyosarcoma has not been reported previously, but other neoplasms have been reported in patients with noonan syndrome, a condition similar to CFC-s. More observations are needed, but this and other reports suggest there could be a higher risk of malignancy in patients with syndromes in the Noonan phenotype category.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
4/158. 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 = neoplasm (Clic here for more details about this article) |
5/158. A case of infantile rhabdomyofibrosarcoma with immunohistochemical, electronmicroscopical, and genetic analyses.A case of infantile rhabdomyofibrosarcoma arising on the buttocks of a 15-month-old boy is reported with histological, immunohistochemical, electronmicroscopical, and cytogenetic findings. Histological examination showed a proliferation of spindle-shaped cells in a fasciculated pattern, with occasional rounded rhabdomyoblastic cells with abundant eosinophilic cytoplasm. Immunohistochemically, the tumor cells expressed desmin and MyoD1 but were only weakly positive for myoglobin. No clear rhabdomyoblastic features were observed by electronmicroscopic examination. Chromosome analysis showed a clone of 46, XY, der(2)t(2;11)(q37;q13), different from any karyotypic abnormality in the original report of this neoplasm. loss of heterozygosity at 11p15.5, the most frequent genetic alteration in embryonal rhabdomyosarcoma, was not detected. The low degree of striated muscle differentiation and tumor localization supported the diagnosis of infantile rhabdomyofibrosarcoma rather than spindle-cell rhabdomyosarcoma in this case. The present case has been uneventful as of 25 months after surgery. The rather long recurrence-free period, which has not been reported in previous cases, may be attributable to chemotherapy-induced rhabdoid differentiation of the tumor cells.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
6/158. Primary meningeal rhabdomyosarcoma in a child with hypomelanosis of Ito.Intracranial rhabdomyosarcomas are rare neoplasms, and those thought to be primary meningeal tumors are even more rare. Hypomelanosis of Ito is a neurocutaneous disorder believed to involve a defect in cells of neural crest origin. We report the case of a 15-month-old boy with hypomelanosis of Ito who developed a primary meningeal rhabdomyosarcoma. The patient initially presented with hydrocephalus and 2 months later developed neurologic signs localizing to the spinal cord. Radiologic studies revealed widespread leptomeningeal enhancement with compression of the spinal cord at C5-C7. A brain biopsy revealed a tumor diffusely involving the meninges. Microscopically, the tumor was composed of rhabdomyoblasts, many of which showed prominent cross-striations on routine hematoxylin-eosin staining. To the best of our knowledge, this is the first reported case of meningeal rhabdomyosarcoma in a patient with hypomelanosis of Ito and the fourth reported case of a primary meningeal rhabdomyosarcoma reported in the world literature.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
7/158. Sclerosing, pseudovascular rhabdomyosarcoma in adults. Clinicopathological and immunohistochemical analysis of three cases.rhabdomyosarcoma in adults represents a rare soft tissue neoplasm which is seen most frequently in its pleomorphic subtype in this age group. Very rarely, clear cell and spindle-cell variants have been reported. In this study we describe three cases of rhabdomyosarcoma in adult patients, characterised by prominent hyaline sclerosis and a pseudovascular growth pattern. All cases were identified in the consultation files of one of the authors and routinely processed. Immunohistochemical studies were performed on paraffin sections with the alkaline phosphatase-antialkaline phosphatase method. The patients, two women and one man, were 40, 41, and 56 years old. One developed a deep-seated soft tissue mass in the left lower leg, and one, a tumour of the left upper jaw. In one patient a bone tumour in the proximal body of the sacrum without extension into soft tissues was seen. The patients were treated by wide excision, piecemeal excision and incomplete excision in one case each; additional radiotherapy was performed in all three cases, and chemotherapy in two patients. In one patient multiple pulmonary metastases were noted, which showed progression despite systemic chemotherapy. Histologically, the neoplasms were composed of round/polygonal and spindle-shaped tumour cells including typical rhabdomyoblasts. In all cases a pseudovascular pattern and prominent hyaline sclerosis of the intercellular matrix was seen. Immunohistochemically, tumour cells stained positively for desmin and muscle actin (HHF35) and also for markers of striated muscle differentiation (myogenin, MyoD1, fast myosin). In this paper an unusual morphological variant of rhabdomyosarcoma arising in adult patients is described, which should be added to the morphological spectrum of these neoplasms.- - - - - - - - - - ranking = 3keywords = neoplasm (Clic here for more details about this article) |
8/158. A new human pleomorphic rhabdomyosarcoma cell-line, HS-RMS-1, exhibiting MyoD1 and myogenin.A number of human cell lines derived from alveolar or embryonal rhabdomyosarcoma (RMS) have been described. To our knowledge, however, no cell line established from pleomorphic RMS has been reported. We describe here the establishment and characterization of a new human cell line, HS-RMS-1, which originated from a typical pleomorphic RMS arising in the gluteal muscle of a 26-year-old man. HS-RMS-1 cells had pseudotetraploid complex karyotypes with no specific abnormalities. Both in vitro and in vivo the cells on light microscopic examination exhibited pleomorphic features with immunopositive reaction for myogenic antigens including MyoD1 and myogenin, although no Z band-like structures were detected electron-microscopically. RT-PCR demonstrated the expression of MyoD1 and myogenin in HS-RMS-1 cells at the mRNA level, and direct sequencing analysis revealed cDNAs of MyoD1 and myogenin identical to those previously reported. This cell line, HS-RMS-1, established from pleomophic RMS will be useful for further studies including the molecular aspects of human RMS.- - - - - - - - - - ranking = 0.0029289327519674keywords = complex (Clic here for more details about this article) |
9/158. Primary cardiac rhabdomyosarcoma of the left atrium: an unusual presentation.rhabdomyosarcoma accounts for almost 20% of all primary malignant neoplasms of the heart. These tumors usually arise from the ventricular walls. In adult patients, they sometimes arise from the atrial walls and mimic atrioventricular valve stenosis. We describe a case of left atrial rhabdomyosarcoma that presented as severe mitral stenosis and required emergency surgery. The atrial mass was detected by transthoracic and transesophageal echocardiography, but only histopathology confirmed the nature of the lesion. Although rhabdomyosarcomas of the heart are highly lethal, operation is indicated for emergency cases, in order to clarify the diagnosis, relieve symptoms, and improve short-term survival.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
10/158. Unusual second malignancies following radiation therapy: subcutaneous pleomorphic rhabdomyosarcoma and cutaneous melanoma. Two case reports.BACKGROUND: Among nonepithelial second neoplasms which are known to be induced by irradiation, rhabdomyosarcomas are extremely rare, and melanomas are infrequent. We report a high-grade sarcoma with rhabdomyoblastic differentiation, which appeared 30 years after megavoltage irradiation for an endometrial adenocarcinoma, and a malignant melanoma which arose after 6 years in the irradiation field of a fibrosarcoma. methods: histology and immunohistochemistry were performed in both cases. In the first case, electron microscopy was also performed. In the second, the previous tumor was re-evaluated. RESULTS: The first case showed histological, immunohistochemical and ultrastructural features of a rhabdomyosarcoma. In the second case, a lentigoid malignant melanoma was histologically and immunohistochemically demonstrated, whereas the previously resected tumor was a fibrosarcoma negative to melanoma markers. CONCLUSIONS: Rare cases of rhabdomyosarcomas and melanomas are induced by irradiation, although in some cases other factors (i.e., genetic risk, chemotherapy) may have a prominent etiopathogenetic role in their development. A close follow-up and a careful examination of the irradiated area should facilitate an early diagnosis of these aggressive postradiation second neoplasms.- - - - - - - - - - ranking = 2keywords = neoplasm (Clic here for more details about this article) |
| | Next -> |