• The Journal of the Korean bone and joint tumor society
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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

Malignant small round cell tumor (SRCT) of the thoracopulmonary region appears to originate in the soft tissues of the chest wall or the peripheral lung. A differential diagnosis of poorly differentiated small round cell tumors which include Ewing's sarcoma of bone and soft tissue, embryonal rhabdomyosarcoma, Askin tumor, neuroblastoma, peripheral neuroectodermal tumor, small cell osteogenic sarcoma and Iymphoma are often difficult by light microscopy alone. In recent, by the extensive studies electron microscopic examination, histochemical study, immune-chemical study, cytogenetics and gene analysis, these tumors may be derived from the primitive and pluripotential cells, differentiating into mesenchymal, epithelial and neural features in variable proportions. Treatment for SRCT of thoracopulmonary region is not determined because of massive involvement of the lung, pleura or soft tissues of the chest wall resulted in a dismal outcome despite aggressive surgery, irradiation and chemotherapy.

• 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

Primary cardiac tumors are known to be rare. We studied the surgical results for primary cardiac tumors. Material and Method: Between August 1980 and December 2003, we classified 86 patients who had operation for primary cardiac tumors in our center into 3 groups; myxoma, nonmyxoma benign tumors, and malignant tumors. The mean age was $44.3\pm20.8$ years and 59 patients $(66.3\%)$ were female. In postoperative pathologic diagnosis, there were 81 cases $(94.2\%)$ of benign tumors in which myxoma was the most common tumor $(70\;cases,\;78.7\%);$ 5 fibroma $(5.6\%)$, 3 rhabdomyoma $(3.4\%)$, and 5 malignant tumors $(5.8\%)$. Result: $86.4\%$ of benign tumor was myxoma and the mean age was $50.4\pm15.4\;(range\;7\~80)$ years. Tumor was more common in females (49 cases) and most common preoperative symptom was dyspnea $(62.9\%)$. 57 cases were located at left atrial septum and only one case, which was located at right ventricular septum, was resected incompletely. There were no hospital deaths and one patient had mitral valve replacement on the first operative day due to newly developed postoperative mitral regurgitation. The mean follow up period was $109.3\pm71.8$ months and there was no evidence of recurrence in this period. 11 cases $(12.8\%)$ were non myxoma benign tumors; 5 fibromas, 3 rhabdo-myomas, etc. There were two hospital deaths and the causes of death were fungal endocarditis and hypoxia. There were no reoperations in nonmyxoma benign tumors. Malignant tumors were in 5 cases $(5.8\%);$ undifferentiated sarcoma in 2, rhabdomyosarcoma in 1, etc. Although there were no hospital mortalities, 3 patients who were followed up died from complications of tumors. Conclusion: Myxomas showed very excellent prognosis after complete resection and nonmyxoma benign tumors showed relatively good results for relief of symptoms. Surgery helped to relieve symptoms for malignant tumors, but the prognosis was poor.

• Journal of the korean academy of Pediatric Dentistry
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• 제26권2호
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• pp.331-338
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• 1999

Chemoradiation therapy used on pediatric oncology patients often causes dental developmental anomalies that affect future dental care. Defects noted include tooth and root agenesis, root thining and shortening, and localized enamel defects. The effect of radiotherapy usually are confined to the radiation site, but the effects of chemotherapy may be more wide spread because of its systemic distribution and structures and organs unrelated to the primary tumor may be affected. Many pediatric cancers are treated with a combination of radiation and multiagent chemotherapy to create synergic and additive effects. Dental treatment affected by chemoradiation damage to developing teeth includes orthodontic tooth movement, prosthetic abutment considerations, periodontal health, space maintenance, requirements for home fluoride regimens to protect hypomineralized areas, restoration options for hypoplastic/hypomineralized teeth, and endodontic procedures. The following case demonstrate chemoradiation therapy effects on the dental development.

• Journal of the korean academy of Pediatric Dentistry
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• 제31권1호
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• pp.79-84
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• 2004

Pilomatricoma is a benign soft tissue tumor of hair follicle origin. They occur most commonly in the head and neck region and are usually found in girls during the first two decades of life. Although malignant transformation has been described, it is exceedingly rare. The clinical presentation is typically that of an asymptomatic, superfical, solitary, firm mass that is often accompanied by a reddish-blue discoloration of the overlying skin. Histopathologically pilomatricoma are seen as epithelial islands embedded in a cellular stroma. The epithelial component consist of two main cell types : basaloid cells and ghost cells. Intracellular and stromal calcification is reported in many cases. Diagnosis is usually suspected based on palpation of a superficial, rock-hard mass and confirmed by histopathologic examination. Surgical excision is both curative and the treatment of choice. Recurrence is rare. In this case, the radiographs showed a dense calcification, measuring about 1cm diameter. So simple excision was peformed intraorally, and resultantly the lesion was proved to be a pilomatricoma by histological examination.

• Korean Journal of Head & Neck Oncology
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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.

• Korean Journal of Bronchoesophagology
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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.