• 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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• 제9권1호
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• pp.117-122
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• 1991

흉폐부 또는 흉벽에서 발생하는 악성소세포 종양군인 Ewings sarcoma, 횡문근육종, Askin tumor, 신경아세포종, PNET, 임파종 등은 현미경학적 소견만으로는 감별하기 어렵다. 그러나 최근에는 조직세포화학적검사, 면역세포화학적검사, 세포배양, 세포유전학적 검사등의 도움으로 상기한 악성소세포 종양군들이 모두가 같은 계통의 primitive pluripotent cells로 부터 분화되어 발병되는 것으로 확인되었다. 치료는 외과적 절제술, 방사선치료, 항암요법 등이 시도되고 있으나 예후는 재발 과원격전이로 인하여 불량한 것으로 보고되고 있다. 본 저자들은 본원에서 치료한 예를 보고하면서 흉폐부에서 발생하는 악성소세포 종양군의 조직발생과 감별진단에 대하여 논하고자 한다.

• Journal of Korean Neurosurgical Society
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• 제30권5호
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• pp.642-646
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• 2001

A 12-years-old female admitted to the hospital with the complaint of pain on the right upper chest area which persisted about 1 month prior to admission. $Caf{\acute{e}}$-au-lait spots of various size laying on a whole body and freckling on the axilla were found on physical examination. A huge mass was found on the plain chest X-ray and on chest MRI. The mass encroached thoracic spine, posterior rib, back muscles, and then into the neural canal and compressed thoracic spinal cord. On the 5th day of hospitalization, the patient complained tingling on the both legs and 2 days later, monoparesis on the right leg. Open thoracotomy and decompressive laminectomy was done to remove mass. Pathologic reports confirmed rhabdomyosarcoma, embryonal type.

• Journal of Yeungnam Medical Science
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• 제28권1호
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• pp.70-76
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• 2011

Rhabdomyosarcomas are soft tissue sarcomas; while extremely rare in adults, they are one of the most common neoplasms in children and adolescents. Histologically, they can be classified into embryonal (ERMS), alveolar (ARMS), pleomorphic, and undifferentiated types. The ARMS type is very rare, and is associated with a poor prognosis. Common primary sites of ARMS are the trunk and extremities. We report on a case of paraaortic, supraclavicular, and axillary lymph node metastasis from paratesticular ARMS treated with VAC (vincristine, dactinomycin, cyclophosphamide)/ IE(ifosfamide, etoposide) chemotherapy in a young adult. Administration of six cycles of chemotherapy with VAC/IE resulted in complete remission. The patient has maintained complete remission over the past 27 months.

• Advances in pediatric surgery
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• 제14권2호
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• pp.164-172
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• 2008

The survival rate for rhabdomyosarcoma (RMS) has significantly improved after the introduction of combined multimodality treatment. We report the 20-year treatment outcome of pediatric rhabdomyosarcoma in a single institution. The medical records of 16 patients treated for rhabdomyosarcoma between December 1986 and August 2007 at the Department of Pediatric Surgery, Seoul National University Children's Hospital, were retrospectively reviewed. Mean age at diagnosis was 7.1 years (range: 1.3 -14.2 years). Retroperitoneum was the most common primary site (n=7, 43.8 %), and embryonal type was predominant (n=11, 6 %). Before the treatment, most patients were in advanced TNM stage (stage III 50 %, IV; 25 %). The patient distribution according to the Intergroup Rhabdomyosarcoma Study Clinical Grouping System (IRS-CGS) was as follows; Group I 31.3 %, Group II 12.5 %, Group III 31.3 % and Group IV 25 %. Patients were classified into three groups according to the extent of resection of the primary tumor; complete resection (CR, n=5; 31.3 %), gross total resection (GTR, n=7; 43.8 %) and incomplete resection (IR, n=4; 25 %). Recurrence was observed in 9 patients (56.3 %) while there was no recurrence in CR patients. All patients with recurrence were identified as moderate or high-risk according to the IRS-V Risk Group. Pre-treatment TNM stage of RMS in our institution was advanced with aggressive clinical feature, however postsurgical conditions according to IRS-CGS were similar to the previous reports by IRS. This suggests that down-staging of IRS-CGS was achieved with multimodality treatment with CR or GTR. It also suggests that complete resection is the most important prognostic factor in the treatment of RMS in children. Patients classified as moderate or high-risk need close follow-up due to high recurrence rate. In case of localized recurrence, better outcome may be achieved with multimodality treatment including limited surgery.

• Journal of Chest Surgery
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• 제38권4호
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• pp.301-307
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• 2005

원발성 심장 종양은 비교적 드문 질환으로 알려져 있다. 저자들은 수술을 시행받은 원발성 심장 종양의 결과에 대하여 살펴보았다. 대상 및 방법: 1980년 8월부터 2003년 12월까지 연세심장혈관 병원에서 원발성 심장 종양으로 수술을 시행 받은 86명의 환자들을 대상으로 병리적 진단에 따라 점액종, 점액종외의 양성 종양 및 악성 종양으로 나누어 고찰하였다. 환자들의 평균 연령은 $44.3\pm20.8$세였으며 남자가 29명$(33.7\%)$ 여자가 59명$(66.3\%)$이었다. 수술 후 병리적 진단으로는 양성 종양이 81예$(94.2\%)$였고 이 중 점액종이 70예$(78.7\%)$로 가장 많았으며 섬유종이 5예$(5.6\%)$, 횡문근종이 3예$(3.4\%)$였고, 악성 종양은 5예$(5.8\%)$였다 결과: 점액종은 양성 종양의 $86.4\%$였고 환자들의 평균 나이는 $50.4\pm15.4\;(7\~80)$세였다. 여자가 49명$(70\%)$이었으며 가장 흔한 증상은 호흡 곤란$(62.9\%)$이었다. 종양은 57예$(81.4\%)$에서 좌심방 중격에 위치하였고, 우심실 중격에 위치한 1예를 제외하고는 모두 완전 절제가 가능하였다. 수술 사망은 얼었으며 1예에서 수술 후 승모판막 부전이 발생하여 승모판막 대치술을 시행 받았다. 평균 추적 관찰 기간은 $109.3\pm71.8$개월이었고 이 기간 중 종양의 재발 소견을 보인 환자는 없었다. 점액종외의 양성 종양은 11예$(12.8\%)$로 섬유종이 5예, 횡문근종이 3예였다. 2예에서 진균성 심내막염 및 저산소증으로 인한 수술 사망이 있었으며 재수술은 없었다. 악성 종양은 5예$(5.8\%)$로 미분화성 육종이 2예, 횡문근육종 1예 등이었고, 수술 사망은 없었으나 추적이 가능했던 3예에서 합병증에 의한 사망이 있었다. 걸론: 점액종은 완전 절제 후 예후가 매우 좋았으며, 점액종외의 양성 종양은 증상 완화에 도움이 되었다. 악성종양은 종양과 관련된 증상을 완화시키기 위하여 수술이 필요하였으나 예후는 불량하였다.

• 대한소아치과학회지
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• 제26권2호
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• pp.331-338
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• 1999

하악 좌측부위의 횡문근육종으로 2년동안의 화학요법과 6주간의 방사선요법으로 치료를 받았고, 이에 따른 후유증으로 치아의 발육장애를 동반한 8세 환아에 대해, 임상적, 방사선학적으로 관찰을 시행하고, 치료한 결과 다음을 보고하는 바이다. 1. 구강내 소견으로서 맹출한 영구치의 경미한 법랑질 형성부전과 치아우식이 관찰되었으며, 하악 좌측 부위의 미맹출된 제 1 대구치로 인해 수직고경이 상실되어 과개교합이 형성되었다. 2. 방사선학적으로 맹출한 영구치의 치근은 짧은 치근에서부터 무형성증에 이르기까지 다양한 치근으 발육장애를 보였으며, 미맹출 상태인 제 2 소구치와 제 2 대구치는 왜소치나 형성부전의 소견을 보였다. 3. 치료는 보존치료 및 저석회화 소견을 보이는 영구치를 위해 불소도포를 시행하였고, 향후 적절한 수직고경의 회복과 심미적 개선을 위한 포괄적인 보철치료가 수행되어야 할 것으로 사료된다.

• 대한소아치과학회지
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• 제31권1호
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• pp.79-84
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• 2004

Pilomatricoma(Calcifying epithelioma of Malherbe)는 모낭 세포 기원의 양성 연조직 종양으로, 보통 머리와 목 부위에 나타나며, 20세 이전의 여성들에게서 호발하고, 악성으로의 전이가 보고되기도 하나, 이는 극히 드물다. 임상적으로, 무통성, 표재성, 고형성이며 종종 적청색을 나타내고, 크기는 보통 $0.5{\sim}3.0cm$ 정도로 다양하게 나타난다. 조직학적으로, 세포기질 내에 상피조직이 존재하는 형태이며 상피조직에서는 기저세포(basaloid cell)와 유령세포(ghost cell)가 특징적으로 나타나고, 세포내와 기질내 석회화가 종종 관찰되기도 한다. 진단은, 초진시 고형 물질의 촉진으로 병소를 의심해볼 수 있고, 조직병리학적 검사로 확진할 수 있다. 감별진단으로는 아가미낭(branchial cyst), 혈관종(hemangioma), 횡문근육종(rhabdomyosarcoma), 표피포함낭(epidermal inclusion cyst), 모낭(pilar cyst), 이하선 종양(parotid gland tumor), 피부의 골종(osteomas of the skin), 림프절의 석회화(calcified lymph nodes), 죽종(atheromas) 등이 있다. 치료로는 외과적 절제술이 추천되며 재발은 드물다. 본 증례는 우측 하악각 부위에 결절이 촉진되는 6세 여자 환아에게서 임상 검사와 방사선 검사 후 병소의 외과적 절제술을 시행하였으며, 조직병리학적 검사를 통해 pilomatricoma로 확진되었고, 양호한 치료 결과를 얻었기에 이에 보고하는 바이다.

• 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.

• 대한기관식도과학회지
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• 제13권2호
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• pp.61-64
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• 2007

Treatment choice for primary pulmonary sarcoma is complete surgical resection. A 69 year old man developed dyspnea due to left lung atelectasis. There was endobronchial tumor completely obstructing the left main bronchus. The tumor was resected completely by main bronchial resection via a left thoracotomy incision, and diagnosed as leiomyosarcoma. Bronchoscopy and computed tomography in 6 months after operation, there was no evidence of recurrence.