• Journal of Korean Neurosurgical Society
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• 제53권1호
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• pp.46-48
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• 2013

Chondrosarcoma is a very uncommon malignant primary bone tumor, especially, it occurs extremely rare in the spine. A 52-year-old man was admitted to the emergency room with sudden paraplegia. Twelve hours prior to a paraplegic event, he visited an outpatient clinic with discomfort and tenderness around the medial border of the right scapular, and his neurologic status was absolutely intact. Magnetic resonance imaging showed a lobulated soft tissue mass from T3 to T5, which extended to the epidural space. Computed tomography scans showed soft tissue mass on the spinal posterior arch and osteolytic change of the adjacent bony structures. Emergent surgery was performed and the lesion was removed. Dark reddish blood and gel-like material were encountered around the dura and posterior arch during the operation. Multiple pulmonary nodules were found on a chest CT scan and a biopsy of one of them had been proven to be a metastasis of chondrosarcoma. The histologic examination showed dedifferentiated chondrosarcoma. The patient's neurologic deficit was improved slowly from ASIA A to ASIA D. Chondrosarcoma in the spine is extremely rare, even more with acute hemorrhage and sudden expansion into the epidural space. We named it chondrosarcoma apoplexy. We should consider the possibility of a hemorrhagic event when the patient's neurologic deficit worsens suddenly with spinal bone tumor.

• Investigative Magnetic Resonance Imaging
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• 제20권1호
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• pp.66-70
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• 2016

Introduction: Distant metastases of mucoepidermoid carcinoma (MEC) are reported with the most common sites being the soft tissue of skin, lung, liver, and bone. We report here a very rare case of MEC with multiple metastases to the kidney, adrenal gland, skull and gluteus maximus muscle. Case report: A 63-year-old male patient presented with left-sided headache. Radiologic evaluations including CT and MRI showed ill-defined soft tissue lesion involving the left infratemporal fossa and left sphenoid sinus, and multiple enlarged lymph nodes in neck and mediastinum. PET-CT demonstrated multiple hypermetabolic lesions in and around the left kidney, left adrenal gland, right ischium, right gluteus maximus and skull base. These lesions were confirmed as MEC with multiple metastases through biopsy. Discussion: Only one case of metastasis to the skull has been previously reported, and moreover, there has not been a case of metastatic MEC to the kidney, adrenal gland and gluteus maximus muscle so far in the medical literature. It is important to acknowledge the possibility of every unusual MEC metastases, since the presence of metastasis has statistically significant influence on the survival of MEC.

• 대한골관절종양학회지
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• 제3권2호
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• pp.89-97
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• 1997

Twenty hundred and five out of 266 patients who were registered in Korea Cancer Center Hospital from Mar. 1985 to Jan. 1994, were analyzed in the aspect of survival and local recurrence. Fifty one patients were excluded due to inadequate data and follow up. Prognostic factors for survival were evaluated statistically. One hundred and four cases were male, 101 female. Average age was 39.7(range 1 to 77) year with a peak incidence around 4th decade. The most frequent diagnosis was malignant fibrous histiocytoma(MFH)(24.1%). Liposarcoma, synovial sarcoma, rhabdomyosarcoma, malignant peripheral nerve sheath tumor and fibrosarcoma were relatively common diagnostic entities, in decreasing order. In location, extremity was 179(87.3%) and trunk 26(12.7%). Average follow up period was 7.5 years(6 months to 10 years). Actuarial 5 years and 10 years survival rate were 64.0% and 40.8% respectively. In univariate analysis with log-lank test, significant differences in survival rate were noted in histopathological diagnosis, size(10 cm), stage and metastasis. Age, sex, tumor location, tumor depth and local recurrence didn't affect the survival rate. Adjuvant chemotherapy and/or radiotherapy did not affect overall survival rate, but lowered the local recurrence rate when compared with surgery only. Surgical margin did not affect the survival rate, but local recurrence rate was different according to each margin; 5.7% in more than wide; 39.5% in marginal; and 60.0% in intralesional excision. In multivariate analysis for results of univariate analysis with Cox's propotional model, metastasis was a meaningful factor for survival of soft tissue sarcoma.

• Archives of Plastic Surgery
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• 제34권1호
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• pp.119-122
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• 2007

Purpose: Although liposarcoma is the second most common soft tissue sarcoma in adults, the incidence of liposarcoma of the head and neck is rare. There is only one reported case in Korea and moreover, only in adolescence. We report a case of liposarcoma on the neck in a 32-year-old male in adult. Methods: The patient had a slow growing, none tender mass on the posterior neck without lymphadenopathy, which has been present for 3 years and recurred twice during that time. MRI showed a 1.5 cm sized ovoid, well demarcated mass that was located in the subcutaneous layer of the posterior neck. Results: The mass was surgically removed. The resection margin was free of tumor on frozen biopsy and histopathologic examination indicated myxoid and round cell liposarcoma. The whole body F-18 FDG PET-CT applied on the fourteenth day postoperatively, revealed a moderate FDG-uptaking soft tissue lesion showing postoperative wound healing process on the posterior neck region and there was no distant metastasis. Conclusion: Liposarcoma is the second most common soft tissue sarcoma in adults. But, it rarely involves the head and neck region. Prognosis is principally dependent on histologic subtype and grade. Low grade liposarcoma such as well differentiated and myxoid liposarcoma tend to recur locally, rarely metastasize. On the other hand, high grade liposarcoma such as round cell and pleomorphic liposarcoma have higher rates of local recurrence and distant metastasis. Complete surgical excision provides the most effective means of treatment. Radiotherapy or chemotherapy can be used as an asjunctive treatment modality.

• 대한정형외과학회지
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• 제55권5호
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• pp.397-404
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• 2020

목적: 본 연구의 목적은 연부조직에 발생한 평활 근육종 환자의 치료 결과를 후향적으로 분석하여 생존율과 재발 및 전이의 양상 등 치료 결과를 알아보고 예후에 영향을 미치는 인자를 분석하고자 한다. 대상 및 방법: 2001년 1월부터 2017년 12월까지 본원에서 연부조직에 발생한(사지 및 체간) 평활 근육종으로 치료 받은 72명의 환자를 대상으로 하였다. 치료 후 재발, 전이 발생, 생존율 및 생존의 예후 인자를 조사하였다. 생존자 중 1년 이상 추시되지 않은 증례는 제외하였다. 평균 추시 기간은 41개월(범위, 7-163개월)이었다. 결과: 전체 환자의 5년 생존율은 73.6%, 10년 생존율은 66.2%였다. 생존율에 영향을 미치는 인자는 종양의 크기(p=0.018), 병리학적 등급(p=0.017), 국소재발(p=0.04)이었다. 전이성 병변은 72명의 환자 중 23명(31.9%)의 환자에서 발생하였으며 전이 후 사망까지의 중간값은 15.6개월(범위, 3-78개월)이었다. 결론: 연부조직에 발생하는 평활 근육종은 주로 하지에 발생하며 종양의 크기, 병리학적 등급, 국소재발이 생존율에 영향을 미치고, 전이 후 생존율은 치료에도 불구하고 불량하였다.

• Journal of Chest Surgery
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• 제35권8호
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• pp.638-641
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• 2002

저등급의 섬유점액성 육종은 최근에야 진단적 분류가 이루어진, 드문 연부 조직 종양이다. 젊은 성인의 심부연부 조직에서 발생하는 경향이 있으며, 국소 재발이나 원격 전이가 가능하다. 진단적 범주가 확실히 정립되어 있지 않고 특정한 질환명으로 구분되고 있지 않다. 조직학적으로, 주로 소용돌이 양상으로 자라는 방추상세포와 점액성이나 섬유성 기질이 번갈아 관찰되는 것이 특징이다. 형태학적이나 면역조직학적 특징에 대한 세심한 고려가 상기 질환의 진단에 도움이 되며, 다른 양성이나 악성 연부 조직 종양과의 감별에 도움이 된다. 흉백에 발생한 저등급의 섬유점액성 육종을 경험하였기에, 문헌 고찰과 함께 보고하는 바이다.

• Investigative Magnetic Resonance Imaging
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• 제23권4호
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• pp.361-366
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• 2019

Desmoplastic small round cell tumor (DSRCT) is a rare and aggressive malignancy common in young male patient. Typical imaging features of DSRCT include multiple soft tissue masses in the peritoneal cavity, omentum, or mesentery without an organ of origin. This report presents a rare manifestation of DSRCT revealing a solitary large retroperitoneal mass with hepatic metastasis and malignant portal vein thrombosis in 70-year-old women together with the review of literature. The tumor showed a hemorrhagic and necrotic mass with peripheral portion of T2 hypo-intensity and delayed enhancement that indicated desmoplastic stroma with dense cellularity.

• 대한골관절종양학회지
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• 제5권4호
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• pp.201-207
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• 1999

Malignant tumors of the hand are very rare. Between 1989 and 1998, 17 patients with malignant tumors of the hand were evaluated for clinical features, prevalence, treatment and results. The mean age was 45 years and median duration of follow-up was 38 months. A painless mass was the most common symptom and the most common lesion was the digit. Fifteen cases of soft tissue and 2 cases of bone tumor were diagnosed. On pathology, soft tissue tumors were comprised of 8 cases of malignant melanoma, 2 cases of angiosarcoma and 1 case each of malignant fibrous histiocytoma, malignant peripheral nerve sheath tumor, extraskeletal chondrosarcoma, and squamous cell carcinoma. Bone tumors were metastatic tumors and each originated from the rectal cancer or lung cancer. Lymph node involvements were noted in 4 cases of malignant melanoma and 1 case of squamous cell carcinoma. Six cases of metacarpo-phalangeal joint disarticulation and 4 cases of phalanx amputation were performed. Wide excision, ray amputation and below-elbow amputation were also performed. Three cases expired due to metastasis and progression of the original lesion. Among the surviving 14 cases, a malignant melanoma had metastasis on the axillary lymph node and 13 cases showed no local recurrence or metastasis during the follow-up.

• Tuberculosis and Respiratory Diseases
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• 제70권5호
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• pp.423-427
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• 2011

Epithelioid sarcomas are rare soft tissue sarcomas with a high tumor grade and high local recurrence and metastasis rates. Although the lung is the most common site of metastasis, endobronchial metastasis hasn't been reported yet. We now report a case of epithelioid sarcoma with endobronchial metastasis. A 28-year-old man had recurrent pneumothorax and underwent wedge resection. He presented at our hospital with hemoptysis, dyspnea, and chest pain. Chest computed tomography revealed left pneumothorax, multiple lung nodules and endobronchial lesions at the right lower basal lobe. Bronchoscopy showed a hemorrhagic mass obstructing the bronchus of the right lower basal lobe. Magnetic resonance imaging revealed multiple nodular lesions in the left thigh muscles. The bronchoscopic biopsy of the endobronchial lesion and the muscle biopsy of the thigh showed the same feature epithelioid sarcoma. This is the first case report of an epithelioid sarcoma with endobronchial metastasis that was diagnosed by bronchoscopic biopsy.

• Yonsei Medical Journal
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• 제59권9호
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• pp.1049-1056
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• 2018

Purpose: Local recurrence is the most common cause of failure in retroperitoneal soft tissue sarcoma patients after surgical resection. Postoperative radiotherapy (PORT) is infrequently used due to its high complication risk. We investigated the efficacy of PORT using modern techniques in patients with retroperitoneal soft tissue sarcoma. Materials and Methods: Eighty patients, who underwent surgical resection for non-metastatic primary retroperitoneal soft tissue sarcoma at the Yonsei Cancer Center between 1994 and 2015, were retrospectively reviewed. Thirty-eight (47.5%) patients received PORT: three-dimensional conformal radiotherapy in 29 and intensity-modulated radiotherapy in nine patients. Local failure-free survival (LFFS), overall survival (OS), and RT-related toxicities were investigated. Results: Median follow-up was 37.1 months (range, 5.8-207.9). Treatment failure occurred in 47 (58.8%) patients including local recurrence in 33 (41.3%), distant metastasis in eight (10%), and both occurred in six (7.5%) patients. The 2-year and 5-year LFFS rates were 63.9% and 47.9%, respectively. The 2-year and 5-year OS rates were 87.5% and 71.1%. The 5-year LFFS rate was significantly higher in PORT group than in no-PORT group (74.2% vs. 24.3%, p<0.001). In multivariate analysis, PORT was the only independent prognostic factor for LFFS. However, there was no significant correlation between RT dose and LFFS. OS showed no significant difference between the two groups. Grade ${\leq}2$ acute toxicities were observed in 63% of patients, but no acute toxicity ${\geq}$ grade 3 was observed. Conclusion: PORT using modern technique markedly reduced local recurrence in retroperitoneal sarcoma patients, with low toxicity. The optimal RT technique, in terms of RT dose and target volume, should be further investigated.