• Journal of Chest Surgery
• /
• 제12권3호
• /
• pp.165-169
• /
• 1979

Neurofibromatosis [Von Recklinghausen`s disease] is a rare Mendelian dominant disease, which shows multiple generalized symptoms and signs at various sites [Ex Skin, Bone, Nerve, Endocrine, Mediastinum rarely Lung, etc.]. We experienced one case of neurofibromatosis which has typical skin lesions [cafe-au-lait, multiple nodules, axillary freckling] with neurofibrosarcoma [malignant change from mediastinal lesion]. Patient was admitted our department because of recently developed severe dyspnea which was probably due to main tracheal compression by mediastinal neurofibrosarcoma. After successful removal of mediastinal mass dyspnea disappeared completely. Patient`s postoperative course was uneventful, and the patient was discharged 14 days after operation.

• Journal of Chest Surgery
• /
• 제54권5호
• /
• pp.422-424
• /
• 2021

Malignant peripheral nerve sheath tumors are rare sarcomas of the heart. Herein, we report the case of a 24-year-old man who complained of dyspnea, cough, and upper left back pain. He was found to have multiple primary heart tumors obstructing the right superior pulmonary vein in the left atrium, which were diagnosed as malignant peripheral nerve sheath tumors. The patient underwent successful resection of the tumors and immunohistochemistry was utilized for diagnosis.

• Journal of Yeungnam Medical Science
• /
• 제36권1호
• /
• pp.63-66
• /
• 2019

Malignant peripheral nerve sheath tumor (MPNST) is rare, accounting for 5-10% of all soft tissue sarcomas. MPNST is characteristically aggressive and has a poor prognosis. Fifty percent of patients with MPNST have neurofibromatosis type 1 (NF1). NF-associated MPNST occurs more often at younger ages than sporadic MPNST, but the survival difference is controversial. Superficial MPNST from a recurrent neurofibroma is extremely rare and only a limited number of cases have been reported in the literature. Herein, we report an unusual case of superficial MPNST from a recurrent neurofibroma in a patient without NF1.

• Tuberculosis and Respiratory Diseases
• /
• 제45권3호
• /
• pp.604-608
• /
• 1998

Von Recklinghausen병에 동반되는 악성종양은 주로 신경섬유육종이지만 드물게는 폐암이 동반되는 경우가 보고되고 있고 ras 유전자가 양 질환의 발생에 영향이 있음이 알려져 있어 앞으로 이에 관한 연구가 필요하리라 생각된다.

• Archives of Plastic Surgery
• /
• 제34권4호
• /
• pp.495-497
• /
• 2007

Purpose: Malignant peripheral nerve sheath tumors (MPNSTs) are rare neoplasms, usually arising from somatic soft tissues or peripheral nerves. Primary MPNST of the scalp is extremely rare. The case is being reported for its rarity. Methods: A 53-year-old female was presented with a scalp mass on vertex area. The tumor was localized in scalp skin and did not invade underlying periosteum or skull and treated with complete surgical excision followed by adjuvant chemotherapy and radiotherapy. Results: Histologically, the tumor showed malignant spindle cells with focal S-100 positivity on immunohistochemistry and a diagnosis of MPNST was made. Conclusion: Authors experienced a rare case of primary scalp MPNST and report the case.

• Asian Pacific Journal of Cancer Prevention
• /
• 제15권11호
• /
• pp.4535-4538
• /
• 2014

Objective: To explore the value of porous titanium alloy plates for chest wall reconstruction after resection of chest wall tumors. Materials and Methods: A total of 8 patients with chest wall tumors admitted in our hospital from Jan. 2006 to Jan. 2009 were selected and underwent tumor resection, then chest wall repair and reconstruction with porous titanium alloy plates for massive chest wall defects. Results: All patients completed surgery successfully with tumor resection-induced chest wall defects being $6.5{\times}7cm{\sim}12{\times}15.5$ cm in size. Two weeks after chest wall reconstruction, only 1 patient had subcutaneous fluidify which healed itself after pressure bandaging following fluid drainage. Postoperative pathological reports showed 2 patients with costicartilage tumors, 1 with squamous cell carcinoma of lung, 1 with lung adeno-carcinoma, 1 with malignant lymphoma of chest wall, 2 with chest wall metastasis of breast cancers and 1 with chest wall neurofibrosarcoma. All patients had more than 2~5 years of follow-up, during which time 1 patient with breast cancer had surgical treatment due to local recurrence after 7 months and none had chest wall reconstruction associated complications. The mean survival time of patients with malignant tumors was ($37.3{\pm}5.67$) months. Conclusions: Porous titanium alloy plates are safe and effective in the chest wall reconstruction after resection of chest tumors.

• Korean Journal of Otorhinolaryngology-Head and Neck Surgery
• /
• 제55권3호
• /
• pp.181-184
• /
• 2012

The malignant peripheral nerve sheath tumor is an extremely rare soft tissue sarcoma. It is a highly malignant sarcoma, which is locally invasive, frequently leading to multiple recurrences and eventual metastatic spread. The peak incidence of disease is known to occur sporadically between the age of 20s and 50s, and is usually associated with the neurofibromatosis type I. In human body, the trunk and extremities are the most commonly involved sites, with only 8-14% of all lesions appearing in the head and neck region. We present a case of malignant peripheral nerve sheath tumor involving the right parapharynx in a 48-year-old patient who complained of headaches in the right parietal area and of dysphagia that aggravated over a month. After surgery, tumor was finally diagnosed as malignant peripheral nerve sheath tumor by histopathologic examinations. The authors report a case of malignant peripheral nerve sheath tumor in the right parapharynx with a review of the literature.

• 대한수의학회지
• /
• 제15권1호
• /
• pp.27-38
• /
• 1975

개에 자연발생(自然發生)한 다음과 같은 종양(腫瘍)을 수집(蒐集)하여, 그 육안적(肉眼的) 및 조직학적(組織學的) 소견(所見)을 검토(檢討)하고, 이에 관(關)한 간단한 고찰(考察)을 가(加)했다. 피부(皮膚) 및 피하직(皮下織)의 종양(腫瘍) : 섬유종(纖維腫), 지방종(脂肪腫), 표피성(表皮性) 낭종(囊腫), 흑색육종(黑色肉腫), 한선종(汗腺腫), 비만세포종(肥滿細胞腫)(2예(例)), 비만세포육종(肥滿細胞肉腫), 피지선암종(皮脂腺癌腫). 비장(脾臟) 및 임파절(淋巴節)의 종양(腫瘍) : 비피막(脾被膜)의 섬유육종(纖維肉腫), 비(脾)의 평활근육종(平滑筋肉腫), 임파절(淋巴節)의 임파육종(淋巴肉腫)(2예(例)). 폐장(肺臟)의 종양(腫瘍) : 기관지성암종(氣管枝性癌腫)(3예(例)), 각각(各各) 선암종형(腺癌腫型), 편평세포암종형(扁平細胞癌腫型) 및 미분화세포(未分化細胞)(원형(圓形))암종형(癌腫型). 소화관(消化管) 및 간장(肝臟)의 종양(腫瘍) : 위(胃)의 섬유종(纖維腫), 간(肝)의 혈관종(血管腫), 담관암종(膽管癌腫), 간세포암종(肝細胞癌腫), 간(肝)에 출현(出現)한 골수성백혈세포세포(骨髓性白血細胞細胞). 복막(腹膜)의 종양(腫瘍) : 섬유육종(纖維肉腫). 비뇨생식계(泌尿生殖系)의 종양(腫瘍) : 자궁(子宮)의 섬유종(纖維腫), 난소(卵巢)의 난포낭종(卵胞囊腫), 질(膣)의 전염성(傳染性) 성기종(性器腫)(6예(例)), 신암종(腎癌腫), 섭호선종(攝護腺腫)(2예(例)), 포피(包皮)의 섬유종(纖維腫), 고환(睾丸)의 정충종(精蟲腫). 유선(乳腺)의 종양(腫瘍) : 혼합종(混合腫)(2예(例)), 근상피종(筋上皮腫). 신경계(神經系)의 종양(腫瘍) : 대퇴부(大腿部)의 신경섬유육종(神經纖維肉腫).