• Imaging Science in Dentistry
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• 제34권2호
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• pp.111-116
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• 2004

Rhabdomyosarcoma, when it occurs in the head and neck, is primarily found in children. Alveolar rhabdomyosarcoma is rarely seen in the oral lesion, comparing to the embryonal and the pleomorphic variants. This is a report of a case of alveolar rhabdomyosarcoma in the mandible in a ten-year old girl who complained of a non-painful swelling on the right cheek. The right lower 1st molar was mobile. Her radiographs revealed an extensive radiolucency with somewhat irregular border on the right mandibular ramus. The right mandibular 1st and 2nd molars lost their lamina dura and were floating. CT images revealed smooth-outlined soft tissue mass occupying the pterygomandibular space, the infratemporal space, and the masseteric muscle with thinning and perforation of the right mandibular angle and ramus. Histopathological and immunohistochemical findings established the final diagnosis of alveolar rhabdomyosarcoma.

• 대한세포병리학회지
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• 제16권1호
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• pp.52-56
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• 2005

Fine-needle aspiration cytology (FNAC) has been used extensively in the diagnosis of metastatic malignancies. However, metastatic soft tissue sarcomas are often overlooked, primarily due to the low frequency with/ which they occur. Here, we report a rare case of metastatic rhabdomyosarcoma in both cervical lymph nodes, which was detected by FNAC. A 45-year-old woman presented with anosmia, postnasal drip, and sneezing, symptoms which had persisted for 1 month. The patient was found to have a tumorous lesion at the upper portion of the mid-turbinate, with multiple enlarged cervical lymph nodes, and this lesion was examined closely at our facility. FNA cytology smears obtained from both cervical lymph nodes revealed a high degree of cellularity, and displayed cohesive clusters with gland-like spaces, as well as single isolated cells with abundant karyorrhectic debris. The tumor cells exhibited round to oval nuclei containing fine chromatin, occasional small nucleoli, and scanty cytoplasm, or a total lack of cytoplasm. Some of the tumor cells were arranged in multinucleated forms and abundant dense eosinophilic cytoplasms, reminiscent of a rhabdomyoblast. The histological findings on the lymph nodes revealed an outstanding sinusoidal infiltration and a prominent alveolar growth pattern, interspersed with occasional typical rhabdomyoblasts. The immunohistochemical results [desmin (+), myoglobin (+), myogenin (+), pan CK (-), synaptophysin (-), neuron specific enolase (-)] supported a confirmative diagnosis of alveolar rhabdomyosarcoma. Alveolar rhabdomyosarcoma is a representative sarcoma, which typically manifests with nodal metastasis and carcinoma-like clustering. The cytopathologist should remain alert upon encountering unusual morphology, so that the possibility of this condition, although somewhat remote, should not be dismissed or overlooked.

• Journal of Chest Surgery
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• 제25권6호
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• pp.598-604
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• 1992

Rhabdomyosarcoma is the most common soft tissue sarcoma in childhood and acounts for 6% to 15% of all cases of childhood cancer, Rhabdomyosarcoma in seventh most common form of childhood neoplasms, following acute leukemia, tumors of the central nervous system, lymphoma neuroblastoma, Wilm`s tumor, bone tumor. Rhabdomyosarcoma can arise anywhere in the body, but primary site in the thorax is relatively rare. We experienced a case of aveolar rhabdomyosarcoma arising from intercostal muscle, A 12 year-old woman was suffered from the intermittent left chest pain radiating to the scapular area and dyspnea, On physical examination, pulmonary friction rub was heard on the left upper lobe area. Qn adimission, the chest simple radiography revealed a 7 x 6, 5cm sized radio-opaque mass with pleural effusion in the superior mediastinum and the CT showed a well difined radio-opaque mass including the destructed 2nd rib and pleural effusion. The percutaneous tra-nsthoracic needle aspiration biopsy was likely to show blastoma. After the chemotherapy[vincristine, actinomycin-D, cyclophosphamde] was done to treat blastoma, the pleural effussion was subsided and the mass was slightly decreased by 4.5x 4. 5cm. For treatment and diagnosis, we performed en-bloc resection and the defected chest was reconstucted with Gortex patch. Grossly, the specimen was colored graysh-white and arised in between two ribs The microscopic findings showed that the tumor cells were small round with scant pinkish cytoplasm on the H-E stain and the tumor cell nests were grouped by reticulum fibers and showed alveolar pattern on the silver stain The electromicroscopic finding presented that the cytoplasm contained tangled fibrillar and flocculent materials. The histopathologic findings were compatable with laveolar rhabdomyosarcoma. She was discharged without any complication. After discharge, she has been treated with radiation theraphy and chemotheraphy, and not recurred untill last follow-up We report a case of alveolar rhabdomyosarcoma arising to intercostal muscle, developed in 12 year-old waman, with brief review of literatures.

• Clinical and Experimental Pediatrics
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• 제58권12호
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• pp.505-508
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• 2015

It is uncommon for pediatric patients with rhabdomyosarcoma to present with clinical and/or laboratory features of disseminated intravascular coagulation (DIC). We report a case of metastatic alveolar rhabdomyosarcoma with severe bleeding because of DIC in a 13-year-old boy. He experienced persistent oozing at the site of a previous operation, gross hematuria, and massive epistaxis. Two weeks after initiating combination chemotherapy consisting of vincristine, doxorubicin, and cyclophosphamide, the patients' laboratory indications of DIC began to resolve. During this period, the patient received massive blood transfusion of a total of 311 units (26 units of red blood cells, 26 units of fresh frozen plasma, 74 units of platelet concentrates, 17 units of single donor platelets, and 168 units of cryoprecipitate), antithrombin-III and a synthetic protease inhibitor. Despite chemotherapy and radiation therapy, he died 1 year later because of disease progression. In children with metastatic rhabdomyosarcoma and massive DIC, prompt chemotherapy and aggressive supportive care is important to decrease malignancy-triggered procoagulant activities.

• Journal of Yeungnam Medical Science
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• 제1권1호
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• pp.207-212
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• 1984

횡문근육종은 소아에서 발생하는 악성종양중 비교적 드문 것으로 과거에는 생존율이 낮았으나 최근 수술, 약물 및 방사선 치료를 병합한 적극적인 치료로 생존율이 많이 향상되었다. 저자들은 본원 소아과에 흉벽에 발생한 stage III의 alveolar형 횡문근육종으로 입원하여, 약물요법과 방사선 치료를 하여 현재까지 (>15개월) disease free상태로 생존하고 있는 예를 문헌고찰과 함께 보고한다.

• Asian Pacific Journal of Cancer Prevention
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• 제16권2호
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• pp.757-760
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• 2015

Aim: To present the epidemiologic data (age, gender, size etc) and histopathologic and immunohistochemical features of cases of rhabdomyosarcoma (RMS) reported in our department. Settings: Section of Histopathology, Department of Pathology and Microbiology, Aga Khan University Hospital, Karachi, Pakistan. Duration: All cases of RMS diagnosed between January 1, 2003 and December 31, 2012 were included in the study. Results: A total of 277 cases were included. Embryonal rhabdomyosarcoma (ERMS) was by far the dominant histologic type (87.4%) followed by alveolar type (ARMS)(9.4%). ERMS was much more common in males (64.0%)and over 65% of cases occurred in the first decade of life (over 90% in the first two decades). Head and neck region was commonest site for ERMS (46.7%), followed by the genitourinary system (16.1%). Over 65% cases of ARMS occurred in the extremities. Over 80% cases of ARMS occurred in the first 3 decades of life. Immunohistochemical staining for Desmin and MyoD1 was positive in 96.7% and 85.4% respectively. Conclusions: The epidemiologic data and microscopic findings in our patients are similar to international published data on rhabdomyosarcoma.

• Advances in pediatric surgery
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• 제5권1호
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• pp.45-52
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• 1999

Pediatric solid tumors have many histologic similarity. These tumors contained small round cell types, and cause frequent diagnostic problems in pediatric pathology. An important advance in the differentiation of these small round cell tumors has been the identification of consistent chromosomal translocations associated with several types of tumors. Eighteen patients with soft tissue sarcoma were available for review. Seventeen cell lines were also included in this study. The RNA from the specimens were analyzed by reverse transcriptase-polymerase chain reaction (RT-PCR). PAX3-FKHR fusion was present in four of five alveolar rhabdomyosarcoma and PAX7-FKHR fusion was detected in one of five alveolar rhabdomyosarcoma. None of the specimens expressed more than one chimeric transcript. EWS-FLI1 or EWS-ERG fusions were detected in all seven Ewings' sarcoma. No specimens showed EWS-WT1 fusion. These results corresponded well to the histopathologic diagnosis. There were no differences in the histologic appearances of tumors with the more frequent PAX3-FKHR or EWS-FLI1 fusions compared with those containing the variant PAX7-FKHR or EWS-ERG fusions. RT-PCR assay for chimeric transcript is a useful tool for rapid and objective diagnosis of pediatric solid tumors. Through these tools, we can approach genetically to the differential diagnosis of undifferentiated small round tumors.

• Archives of Plastic Surgery
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• 제32권3호
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• pp.381-384
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• 2005

Rhabdomyosarcoma is a rare malignancy of head and neck region. When rhabdomyosarcoma occurs in maxillary area, total maxillectomy is necessary. Total maxillectomy causes defects of orbital floor, palate, gingiva, and alveolar bone, causing severe facial deformity and functional impairment. Immediate maxillary reconstruction has to cover both bone and soft tissue to minimize cosmetic and functional problems. The fibular osteocutaneous free flap can provide paranasal, gingiva, oral mucosal lining and foundation for dental prosthesis, thus ensuring good cosmetic results and mastication, phonation function. We have experienced a reconstruction case of a 19-year-old man with rhabdomyosarcoma of the left maxillary sinus. The patient underwent total maxillectomy and neck dissection. We designed a fibular free flap that had a vascularized bone segment and a double skin paddle. Surgical outcomes were excellent in cosmetic and functional aspects.

• 대한두경부종양학회지
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• 제30권2호
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• pp.119-122
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• 2014

Rhabdomyosarcoma is the most common sarcoma in children less than 15 years of age. Two major histological subtypes are embryonal and alveolar. Embryonal rhabdomyosarcoma is diagnosed by immunopathology and treatments require coordinated management plans that include surgery, chemotherapy, and usually radiotherapy. 8-month-old male infant visited with swelling in left parotid area. Computed tomography scan showed a heterogeneous mass in the left parotid area and the result of fine-needle aspiration cytology was suspicious malignancy. Left total parotidectomy was performed and CSF leakage was noted and repaired. Confirmed by positive reactions to desmin and myogenin, the diagnosis was embryonal rhabdomyosarcoma. On postoperative brain MRI, extension along the meninges was noted and for treatment, chemotherapy and gamma knife radiosurgery were done. Five years after initial surgical resection(3 years and 10 months after completion of chemotherapy and gamma knife radiosurgery), the child did not show any evidence of local recurrence or distant metastasis.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• 제49권2호
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• pp.61-67
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• 2023

This systematic review aimed to analyze the clinicopathological profile and relevant prognostic factors of head and neck rhabdomyosarcoma in pediatric patients. The search was carried out in the electronic search portals PubMed, Lilacs, Embase, Scopus, and Web of Science. The search yielded studies that were then analyzed regarding study topic, data extraction, and risk of bias using the STROBE (Strengthening the Reporting of Observational Studies) guidelines. Finally, three studies were included for qualitative analysis. Most of the cases involved embryonic and alveolar rhabdomyosarcoma. Expression of MYOD1 was highly correlated with diagnosis of spindle cell/sclerosing rhabdomyosarcoma, which appears to have a poor prognosis in children. Furthermore, tumor size <5 cm and absence of metastasis accompanied by complete resection and administration of adjuvant therapies such as chemotherapy and radiotherapy favored a better prognosis.