• 대한영상의학회지
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• 제82권1호
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• pp.225-230
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• 2021

고립성 외안근 전이는 매우 드물게 나타나며, 그중에서도 양측 다발성 외안근 전이는 더욱 드물다. 횡문근육종은 주로 소아에게 생기는 드문 연조직 악성 종양으로, 고립성 외안근 전이를 일으키는 원발암 중 하나로 알려져 있다. 이에 저자는 부비동에서 발생한 횡문근육종 환자에서 발견된 양측 다발성 외안근 전이의 한 증례를 보고하고, 이와 더불어 이전에 보고된 횡문근육종의 고립성 외안근 전이 10예에 대해 간략히 고찰하고자 한다.

• 대한치과의사협회지
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• 제18권5호통권134호
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• pp.343-347
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• 1980

A case of 35-year-man with a rhabdomyosarcoma in the mandible has been presented. Chief complaints are dull sensation and growing mass at the area of the right mandible. In order to make diagnosis, roentgenograms were taken which revealed some sort of radiopaque and punched appearane at the area from right body to angle of the mandible, and biopsy was made, by which rhabdomyosarcoma was observed. The author treated this patient in terms of hemimandiblectomy, neck dissection and resin-made mandible implantation, and has observed this patient for two and half years after operation. The author satisfy that this patient has been recovered.

• 대한골관절종양학회지
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• 제1권2호
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• pp.200-204
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• 1995

Twenty three rhabdomyosarcoma patients who were registered in Korea Cancer Center Hospital from Mar. 1985 to Apr. 1994 were analysed in the aspect of treatment and survival. Thirteen cases were male and 10 female. Average age was 29.5 years(range 1 to 66). Locations of the tumor were as follows: 13 in lower extremity, 6 in upper extremity and 4 in trunk. According to the UICC classification, stage II b was 1 case, stage III a 4, stage III b 10, stage IV a 3 and stage IV b 5. In histological categories, embryonal rhabdomyosarcoma was 7 cases, alveolar 7, pleomorphic 7 and undetermined 2. Average follow up period was 35.3 months(1 tk 7.5 years). Ten cases were continuous disease free, 3 no evidence of disease, 3 alive with disease and 7 died of disease at final follow up. Kaplan-Meier's actuarial 5-year survival rate was 60.3% and 5-year continuous disease free survival rate was 31.4%, Surgical margin was an important factor in local tumor control. Although there was no definite statistical significance, our results suggest chemotherapy and radiation therapy have meaningful roles in reducing local recurrence and improving survival.

• Radiation Oncology Journal
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• 제7권2호
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• pp.235-245
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• 1989

Rhabdomyosarcoma is a highly malignant soft tissue sarcoma that can arise in any site of the body containing striated muscle or its mesenchymal analgae. It is the most common childhood sarcoma with two peak age frequencies, one at ages 2 to 6, and one in the adolescence. The site, stage and extent of disease, and pathologic characteristics of the tumor contribute to prognostic factors that influence therapeutic decisions. The results of treatment of 52 patients with rhabdomyosarcoma, who were treated at Department of Radiation Oncology, Yonsei University College of Medicine, Yosei Cancer Center from 1976 to 1987 were retrospectively analyzed. The most frequent clinical group and primary sites were IRS group III (57.7%) and head and neck (42.3%) including orbit (11.5%) and parameningeal region(13.5%). The overall and disease free 5 year survival rate of eligible 41 patients were 31.7%, 29.3%, respectively. The complete remission (CR) rate was 50% in clinical group III and 0% in IV. Primary tumors of the orbit, clinical group 1 and embryonal subtype had the best prognosis. The Survival rate was improved by additiion of chemotherapy to operation and radiation therapy.

• 대한정형외과학회지
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• 제55권3호
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• pp.271-275
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• 2020

외상은 횡문근육종을 포함한 일부 육종의 발생에 영향을 미친다고 알려져 있고 수술 후 반흔이나 화상 흉터, 골절 부위, 금속 내고정물 주위에서 수년 간의 잠복기를 거쳐 육종을 일으킨 사례들이 드물게 보고되고 있다. 저자들은 컨베이어 벨트에 의해 압궤상을 입은 전완부에 11년 뒤 발생한 횡문근육종의 사례를 경험하였다. 조직 손상으로 인한 염증 반응, 많은 방사선 촬영에 대한 노출, 금속 내고정물, 감각 및 운동 마비로 인한 무의식적이고 반복적인 손상이 육종의 발생에 영향을 미쳤으리라고 생각된다. 이러한 광범위한 조직 손상 후에는 육종 발생 가능성을 염두에 두고 육종 유발 요인에 대한 노출에 주의를 기울여야 하겠다.

• Radiation Oncology Journal
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• 제34권4호
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• pp.290-296
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• 2016

Purpose: Radiotherapy (RT) is considered a mainstay of treatment in parameningeal rhabdomyosarcoma (PM-RMS). We aim to determine the treatment outcomes and prognostic factors for PM-RMS patients who treated with RT. In addition, we tried to evaluate the adequate dose and timing of RT. Materials and Methods: Twenty-two patients with PM-RMS from 1995 to 2013 were evaluated. Seven patients had intracranial extension (ICE) and 17 patients had skull base bony erosion (SBBE). Five patients showed distant metastases at the time of diagnosis. All patients underwent chemotherapy and RT. The median radiation dose was 50.4 Gy (range, 40.0 to 56.0 Gy). Results: The median follow-up was 28.7 months. Twelve patients (54.5%) experienced failure after treatment; 4 local, 2 regional, and 6 distant failures. The 5-year local control (LC) and overall survival (OS) were 77.7% and 38.5%, respectively. The 5-year OS rate was 50.8% for patients without distant metastases and 0% for patients with metastases (p < 0.001). Radiation dose (<50 Gy vs. ${\geq}50Gy$) did not compromise the LC (p = 0.645). However, LC was affected by ICE (p = 0.031). Delayed administration (>22 weeks) of RT was related to a higher rate of local failure (40.0%). Conclusion: RT resulted in a higher rate of local control in PM-RMS. However, it was not extended to survival outcome. A more effective treatment for PM-RMS is warranted.

• 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.

• 대한두경부종양학회지
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• 제10권2호
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• pp.218-224
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• 1994

Soft tissue sarcoma of the head and neck is not frequent neoplasm, accounting for less than 1% of all malignant neoplasm in the region. The histological varieties include osteogenic sarcoma, malignant fibrous histiocytoma, rhabdomyosarcoma, fibrosarcoma, tenosynovial sarcoma, angiosarcoma and chondrosarcoma. Rhabdomyosarcomas of the head and neck usually occur in children under the age of 10 years (over 70%) and rarely develop in adults over the age of 20 years. The prevalent sites of involvement include the orbit, nasal cavity, external ear, paranasal sinus and soft tissue of mouth and the primary location of tumor is considered to be one of the important prognostic factors. Before the 1960s, when surgical resection was the only method of treatment, the 5-year survival rate was less than 20%, but recently it has been greatly improved by the multimodality treatment, combining surgery with chemotherapy and radiation therapy. Here we treated a rhabdomyosarcoma woman with three cycles of high dose chemotherapy followed by radiation therapy. After the, completion of preoperative treatments, successful result of more than partial response was achieved. Three months later total maxillectomy and radical neck dissection was performed. There was no evidence of tumor infiltration in the resected tumor and regional lymphnodes but metastasized tumor cells in cervical lymphnodes were detected. Tumor cell infiltration was also found on the bone marrow biopsy to evaluate the pancytopenia which occurred during postoperative recovery. Two months later she died of secondary bone marrow failure. We think that this multimodality treatment combining pre-operative chemotherapy, radiotherapy and surgery might play an important role in curative resection and eyeball preservation in patients with rhabdomyosarcoma involving the eyeball.

• Journal of Chest Surgery
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• 제28권4호
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• pp.412-415
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• 1995

Primary cardiac tumors are extremely rare and about 20-25% of primary cardiac tumors are malignant. Recently we have experienced a case of primary cardiac rhabdomyosarcoma originated on the right atrial wall. Patient was 41 years-old women and chief complaints were pitting edema and exertional dyspnea. Emergency operation was performed to relieve symptoms by pericardial effusion that hac been seen on the echocardiogram and large soft mass in right atrial cavity on the MRI. About 700ml amounts of bloody pericardial effusion was collected into the pericardial cavity. 4X5X5 cm sized ingrowing soft mass in the right atrial cavity and two small sized outgrowing masses were inspected. A tumor was invaded into the SA node and superior limbic portion of fossa ovalis. And then mass & right atrial free wall was resected but areas of SA node and fossa ovalis were not resected. Defect of right atrial wall was reconstruced with bovine patch. She was discharged after 2 weeks without any problems and she has been treated with chemotherapy.

• 대한치과의사협회지
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• 제12권12호
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• pp.913-918
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• 1974

The authors have observed clinically and histopathologically on two cases of rhabdomyosarcomas occurred in the oral cavity. The results were as follow: 1. A case of adult pleomorphic rhabdomyosarcoma, involving the right mandibular third molar area of 52 year old male, revealed pathological fracture of the right mandibular angle area. Microscopically, sections of this tumor showed cross striations by staining of phosphotungstic acid hematoxylin. 2. A case of embryonal alveolar rhabdomyosarcoma, involving the right buccal area of one year and ten months old female, revealed severe bone destruction on the right mandibular body area. Microscopically, sections of this tumor did not show cross striations.