1/237. Sarcoma botryoides arising from the biliary tract of children. A case report with review of the literature.Sarcoma botryoides (embryonal rhabdomyosarcoma) originating from the common bile duct is reported in a 4-year-old girl. Grossly, the tumor is characterized by multiple polypoid and grape-like projections into the lumen of the common bile duct and shows plate-like thickening of the common bile duct wall. On multiple cut-sections these polypoid masses are soft and light yellow white except for multifocal hemorrhage. Histologically the tumor consists of undifferentiated mesenchymal cells with a myxomatous appearance; rhabdomyoblasts with varing degree of maturation are scattered here and there within the matrix. These cells are characterized by a strap and racket shape with abundant eosinophilic cytoplasm and elongated cytoplasm with cross striations. The histogenesis of the tumor may be related possibly to an abnormal differentiation of the primitive mesenchymal cells toward striated muscle cells.- - - - - - - - - - ranking = 1keywords = soft (Clic here for more details about this article) |
2/237. 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 = 6.026168048944keywords = neoplasm (Clic here for more details about this article) |
3/237. Pancreatic metastasis of cardiac rhabdomyosarcoma diagnosed by fine needle aspiration. A case report.BACKGROUND: Fine needle aspiration (FNA) is a valuable technique in the diagnosis of soft tissue tumors or their metastases. CASE REPORT: A rhabdomyosarcoma of the left atrium with metastasis to the pancreas was diagnosed by FNA in a 74-year-old female. The patient presented with dyspnea, weight loss and generalized weakness and was found to have a cardiac arrhythmia. magnetic resonance imaging showed a 9-cm mass in the left atrium and anterior mediastinum. Computed tomography (CT) of the abdomen revealed a 2.8-cm nodule within the head of the pancreas. The patient underwent CT-guided percutaneous aspiration biopsy of the pancreatic mass on the first hospital day and, on the second day, transvenous FNA biopsy of the intracardiac mass. The cytologic morphology and immunocytochemistry of the aspirated material from both sites established a diagnosis of cardiac rhabdomysarcoma with metastasis to the pancreas. CONCLUSION: This is the fifth reported case of rhabdomysarcoma metastatic to the pancreas and the first in which the diagnosis was made by FNA, thereby eliminating the need for open biopsy.- - - - - - - - - - ranking = 1keywords = soft (Clic here for more details about this article) |
4/237. Microtubular aggregates within the rough endoplasmic reticulum of embryonal rhabdomyosarcoma cells: a case report.This study presents a case of embryonal rhabdomyosarcoma (ERMS) of the forearm soft tissue in a 12-year-old female, in which microtubular aggregates in rough endoplasmic reticulum (rER) were noted ultrastructurally. Histologically, tumor cells consisted of typical rhabdomyoblastoid cells with abundant eosinophilic cytoplasm and relatively immature, small tumor cells. Ultrastructurally, two different types of tumor cells were also identified by light microscopy. More than half the tumor cells possessed the characteristic features of rhabdomyoblastic differentiation, such as abundant thick and thin filaments with Z-bands. The other tumor cells were less differentiated cells in which microtubular aggregates (MA) in rER were observed. MA in rER have been described in several nonepithelial tumors, including malignant melanoma, osteosarcoma, extraskeletal myxoid chondrosarcoma, and chordoma. ERMS is another example of mesenchymal tumor in which MA in rER are observed by electron microscopy. Considering the differential diagnosis among mesenchymal tumors, it is important to know that MA can be also observed in ERMS.- - - - - - - - - - ranking = 1keywords = soft (Clic here for more details about this article) |
5/237. 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 = 6.026168048944keywords = neoplasm (Clic here for more details about this article) |
6/237. 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 = 7.026168048944keywords = neoplasm, soft (Clic here for more details about this article) |
7/237. 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 = 6.026168048944keywords = neoplasm (Clic here for more details about this article) |
8/237. Embryonal "Botryoid" rhabdomyosarcoma of the larynx: a clinicopathologic and immunohistochemical study of two cases.Two cases of embryonal rhabdomyosarcoma of the larynx are reported. The tumors occurred in a 16-year-old boy and in a 66-year-old man. They manifested clinically with nonspecific symptoms, including voice hoarseness and sense of throat fullness. Treatment consisted of total and partial laryngectomy, respectively. Grossly, both lesions had an exophytic growth pattern and microscopically featured a proliferation of small round to oval cells. Cell cytoplasms were occasionally stainable and fibrillary. Quite often, tumor cellularity was denser beneath the covering mucosa, recalling a "cambium layer" pattern. Tumor cells immunoreacted for desmin, actins, myoglobin, and sarcomeric actin; no immunostaining was noted for epithelial markers. No further antitumoral treatment was administered after surgery. There has been no recurrence of tumor at 2 and 10 years, respectively. Based on our series and the available literature, it seems that rhabdomyosarcoma of the larynx pursues a less-aggressive course than that seen in the homonimic juvenile or adult soft tissue lesion. Surgery alone appears to be a valid treatment option, especially when a polypoid, or "botryoid" gross pattern, coupled with the embryonal small cell histotype is encountered. In light of these findings, it is suggested that botryoid rhabdomyosarcoma of the larynx may deserve a specific consideration among the various laryngeal mesenchymal malignancies.- - - - - - - - - - ranking = 1keywords = soft (Clic here for more details about this article) |
9/237. 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 = 6.026168048944keywords = neoplasm (Clic here for more details about this article) |
10/237. 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 = 6.026168048944keywords = neoplasm (Clic here for more details about this article) |
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