No abstract available.
Leiomyosarcoma* ; Myosarcoma* ; Rhabdomyosarcoma*

Rhabdomyosarcoma is the most common soft tissue sarcoma occurring in children, though retroperitoneal rhabdomyosarcoma is rare. We experienced a case of embryonal rhabdomyosarcoma of the retroperitoneum in a 43-month-old child, and describe the CT , MRI and pathologic findings.
Child* ; Child, Preschool ; Humans ; Magnetic Resonance Imaging ; Myosarcoma ; Rhabdomyosarcoma ; Rhabdomyosarcoma, Embryonal* ; Sarcoma

PURPOSE: To evaluate radiologic findnings of rhabodmyosarcoma of children in the head and neck concerning theorigin, morphologic characteristics, extent, and the route of intracranial extension on CT and MR. MATERIALS AND METHODS: Twenty cases of pathologically proven rhabdomyosarcoma were analyzed. Fifteen CT scans (postcontrast CT(n=13), precontrast CT (n=2)) and eleven MR scans were obtained. Postcontrast MR scans were performed in the tencases. Six cases had CT and MR scans. Nine cases had only CT scan and five had only MR scans. We retrospectivelyanalyzed the origin, morphologic characteristics (attenuation, signal intensity, margin), extent, intracranialextension, route and clinical staging of rhabdomyosarcoma on CT and MR scans. RESULTS: Out of the twentyrhabdomyosarcomas, sixteen cases were of parameningeal group. Ten cases of parameningeal group located in theinfratemporal fossa which was the most common site of origin. The mass showed iso-density in the 8 cases (62%) andhigh density in the 5 cases (38%) on the postcontrast CT scans compared with the muscle. All cases on the CT scanshowed homogenous attenuation of the tumor except one which showed internal necrosis. Twelve cases (80%) on preandpostcontrast CT scans (n=15) showed moderately-defined margin and the rest showed ill-defined margin. On MR scans,all the cases (n=11) showed iso- and high signal intensity on T1WI and T2WI, respectively. Strong enhancement ofthe mass was seen in all cases (n=10). Nine cases showed homogenous signal intensity of tumor on pre-andpostcontrast MR scan while 2 cases showed inhomogenous signanl intenisty because of hemorrhage(n=1) andnecrosis(n=1). Therefore the margin of rhabdomysarcoma on all pre-and postcontrast MR scans was well-defined. Theextent of rhabdomyosarcoma was various depending on corresponding origin and parameningeal group showed widerextent than other head and neck group. Infratemporal rhabdomyosarcoma showed expansile growth and intracranialextension via foramen ovale. Those in the middle ear invaded intracranium by directly destoying petrous bone andvia internal auditory canal. Those in the nasal cavity extended into intracranium by directly destroying skullbase of frontal lobe. Nasopharyngeal one extended into intracranium mainly by directly destroying base of skull orpartially via foramen ovale. Clinical Stage 1,2 and 3 were one, four and fifteen cases, respectively. CONCLUSION: Rhabdomyosarcoma of children in the head and neck tends to show relatively severe bony destruction of skull baseand various intracranial extension routes can be helpful radiologic findings on the CT or MR scan although its CTdensity or signal intensity of MR was not specific. And it is peculiar that infratempral fossa was the most commonsite of origin of rhabdomyosarcoma.
Child* ; Ear, Middle ; Foramen Ovale ; Frontal Lobe ; Head* ; Humans ; Myosarcoma ; Nasal Cavity ; Neck* ; Necrosis ; Petrous Bone ; Rhabdomyosarcoma* ; Skull ; Skull Base ; Tomography, X-Ray Computed

No abstract available.
Adult* ; Humans ; Lip* ; Rhabdomyosarcoma, Alveolar*

Cell Line, Tumor ; Chromosome Aberrations ; Comparative Genomic Hybridization ; methods ; Gene Amplification ; Gene Dosage ; Gene Expression ; Humans ; Receptor, Fibroblast Growth Factor, Type 1 ; genetics ; metabolism ; Rhabdomyosarcoma ; genetics ; metabolism ; pathology ; Rhabdomyosarcoma, Alveolar ; genetics ; metabolism ; pathology ; Rhabdomyosarcoma, Embryonal ; genetics ; metabolism ; pathology

Uterine rhadomyosarcoma (RMS) is a rare tumor accounting for 2~6% of the patients with uterine malignant neoplasm and it happens under 1% of the patients with genitourinary malignancies. RMS can be classified in four types; embryonal, alveolar, pleomorphic, and undifferenciated. About twenty percent of RMS occurs in the genitourinary tract, with sligtly more than half being embryonal rhadomyosarcoma. Alveolar rhabdomyosarcoma (ARMS) of the uterine cervix is very rare. Emerich et al. reported the first case of ARMS of the cervix in 1996. So, little information is available regarding its prognosis and therapy. We report a case of a 56 year-old woman with ARMS of the uterine cervix with a review of the literature. She was received the operation and radiotherapy. She has since been disease-free. We herein report a case of very rare ARMS of the cervix.
Accounting ; Arm ; Cervix Uteri ; Female ; Humans ; Prognosis ; Rhabdomyosarcoma ; Rhabdomyosarcoma, Alveolar

Endoscopy ; Humans ; Myosarcoma ; diagnosis ; surgery ; Nasal Cavity ; Nose Neoplasms ; diagnosis ; surgery ; Paranasal Sinuses ; Skull Base ; Skull Base Neoplasms ; diagnosis ; surgery

OBJECTIVETo study the clinicopathologic characteristics of sclerosing rhabdomyosarcoma (SRMS) and its distinction from embryonal rhabdomyosarcoma (ERMS) and alveolar rhabdomyosarcoma (ARMS).

METHODSThe clinical, histologic and immunohistochemical features of 4 cases of SRMS were studied. The literature was reviewed.

RESULTSAll the 4 cases occurred in adults. The age of patients ranged from 20 to 54 years (mean = 41.5 years). The male-to-female ratio was 1:1. The tumor was located in the left wrist, right thigh, right face and right cheek respectively and the tumor size varied from 2.5 cm to 10 cm in dimension (mean = 5.7 cm). Histologically, SRMS was characterized by the presence of large amounts of heavily hyalinized matrix, mimicking osteoid or chondroid tissue. The tumor cells were composed predominantly of primitive small round cells which were arranged in diverse growth patterns, including fascicular, cord-like, single-file, trabecular, microalveolar and pseudovascular structures. A few rhabdomyoblasts were identified in 1 case. A second spindle cell component was focally found in 2 cases, resembling spindle cell rhabdomyosarcoma or peripheral nerve sheath tumor. Immunohistochemically, all cases showed diffuse staining for Myo D1 and focal staining for desmin. The staining for myogenin was often negative. Three of the cases also expressed muscle-specific actin and 2 cases were positive for alpha-smooth muscle actin. They were all negative for h-caldesmon, S-100 protein, CD31, CD34, AE1/AE3 and anaplastic lymphoma kinase protein.

CONCLUSIONSSRMS differs from ERMS and ARMS morphologically. Recent cytogenetic studies however suggest a histogenetic relationship with ERMS. Familiarity with its morphologic features and immunophenotype may help to distinguish this peculiar variant of rhabdomyosarcoma from a variety of lesions with abundant sclerosing matrix.

Actins ; metabolism ; Adult ; Chondrosarcoma ; pathology ; Combined Modality Therapy ; Desmin ; metabolism ; Diagnosis, Differential ; Facial Neoplasms ; metabolism ; pathology ; therapy ; Female ; Follow-Up Studies ; Hemangiosarcoma ; pathology ; Humans ; Male ; Middle Aged ; MyoD Protein ; metabolism ; Osteosarcoma ; pathology ; Rhabdomyosarcoma ; classification ; metabolism ; pathology ; therapy ; Rhabdomyosarcoma, Alveolar ; classification ; pathology ; Rhabdomyosarcoma, Embryonal ; classification ; pathology ; Sclerosis ; pathology ; Soft Tissue Neoplasms ; metabolism ; pathology ; therapy ; Vimentin ; metabolism ; Young Adult

OBJETIVES: Gastric leiomyosarcomas are uncommon, represent 1 to 3% of primary malignant neoplasms of the stomach and variable in clinical outcomes. This paper is a retrospective study of the clinical behaviour, histopa thologic features and prognostic factors. METHODS: Between January 1990 and August 1996, the records of nine patients with primary gastric leio myosarcoma treated at Chung-Nam National University Hospital were reviewed for clinical presentations and histopathologic features. The statiscal analysis with SAS system was employed for evaluation of prognostic factors. RESULTS: 1) The median age of the patients was 53.4 years with even age distribution from 31 to 76 years. The male and female ratio was 1.25 to 1. 2) The common symptoms were abdominal pain(67%), bleeding(22%) and indigestion(11%). 3) The locations of the tumor was body(56%) and fundus(44%). The growth pattern of the tumor was mostly submucosal(67%). 4) The average size of tumors was 11cm (6-18cm). 5) The central ulcer on the tumor showed 100%. The endoscopically and preoperative diagnosed leiomyosar coma with endoscopic biopsy was 33% . 6) The low grade tumors were 7 cases and high grade was 1 in eight resected primary gastric leiomyosarcomas. 7) The overall resectability was 89%. The resected cases had no lymph node metastasis. Chemotherapy and radiotherapy was done 5 cases and 1 case, respectively in patients with high mitotic number, large size and distant metastases. 8) The follow-up period was 4 to 49 months and 3 patients were died. Two of 3 died patients were initially diagnosed stage IVA and 1 patient was stage II with very large size (18cm). The average survival time of 3 died patients was 37 months. 9) The two patients from surgically resected eight cases were recurred at liver and peritoneum and died 11 months and 31 months after recurrence, respectively. CONCLUSION: The advanced stage and recurred case has short survival time but the more many cases and longer follow-up periods should be need to identify for prognostic factors and the effective postoperative adju vant therapy should be studied for high risk patients.
Age Distribution ; Biopsy ; Coma ; Drug Therapy ; Female ; Follow-Up Studies ; Humans ; Leiomyosarcoma* ; Liver ; Lymph Nodes ; Male ; Myosarcoma ; Neoplasm Metastasis ; Peritoneum ; Radiotherapy ; Recurrence ; Retrospective Studies ; Stomach ; Ulcer

BACKGROUNDMyofibroblastic sarcoma was used to be a controversial neoplasm. This study investigated the clinicopathological features of 20 cases of myofibroblastic sarcoma arising in different locations.

METHODSThe paraffin-embedded tissue samples from 20 cases of patients with myofibroblastic sarcoma were stained immunohistochemically, and 5 cases examined by electron microscopy. Student's t test was used to analyze the difference of Ki-67 labeling index between grade 1 and grade 2 myofibroblastic sarcomas.

RESULTSHistologically, the tumors were composed of slender spindle cells with eosinophilic cytoplasm, and fusiform, tapering, wavy, or plump ovoid; vesicular nuclei and a small central eosinophilic nucleoli. Immunohistochemically, the tumor cells expressed smooth muscle actin (18/20), muscle specific actin (16/20), fibronectin (20/20) and desmin (2/20). Ultrastructurally, the tumor cells revealed abundant rough endoplasmic reticulum and longitudinally arranged fine filaments with focal densities in the cytoplasm. A clinical follow-up of 19 patients showed that 2 cases experienced local recurrence and distant metastasis 6 months to 4 years after the initial operation. Nine cases recurred locally 17 to 46 months after the initial excision, and 9 cases were alive with no evidence of disease.

CONCLUSIONSMyofibroblastic sarcomas, which exhibit diverse histological appearance, can easily be misdiagnosed as benign tumors. Myofibroblastic sarcomas are local destructive lesions with frequent recurrence, and may metastase distantly.

Adolescent ; Adult ; Child ; Desmin ; analysis ; Female ; Fibrosarcoma ; chemistry ; pathology ; Humans ; Immunohistochemistry ; Male ; Middle Aged ; Myosarcoma ; chemistry ; pathology ; Recurrence ; Soft Tissue Neoplasms ; chemistry ; pathology