• Journal of Chest Surgery
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• v.12 no.3
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• pp.165-169
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• 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
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• v.54 no.5
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• pp.422-424
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• 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
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• v.36 no.1
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• pp.63-66
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• 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
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• v.45 no.3
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• pp.604-608
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• 1998

Von Recklinghausen's disease is an autosomal dominant hereditary disease associated with characteristic cafe-au-lait spots of skin and multiple neurofibromatosis. It is complicated by malignancies, which in most cases is neurofibrosarcoma. The development of lung cancer in von Recklinghausen's disease is rare. A 61-year-old male was admitted for cough and sputum for 20 days. He had multiple cafe-au-lait spots and subcutaneous neurofibromas in whole body area and Lisch nodules in both iris and he had been diagnosed von Recklinghausen's disease 35 years ago. Chest radiography showed emphysematous bullae in both upper lung field and mass in right upper lung field. Chest CT scan revealed subcarinal lymph node enlargement. Bronchoscopic biopsy was done in mass in superior segment of right lower lobe and the results showed squamous cell carcinoma. The presence of von Recklinghausen's disease and lung cancer are noteworthy.

• Archives of Plastic Surgery
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• v.34 no.4
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• pp.495-497
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• 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
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• v.15 no.11
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• pp.4535-4538
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• 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
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• v.55 no.3
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• pp.181-184
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• 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.

• Korean Journal of Veterinary Research
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• v.15 no.1
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• pp.27-38
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• 1975

The following tumors occurring naturally in the dog were studied pathologically and discussed briefly. Tumors of the skin and subcutis: Fibroma, Lipoma, Epidermal cyst, Melanosarcoma, Sweat gland adenoma, Mastocytoma (2 cases), Mastosarcoma, and Sebaceous gland carcinoma. Tumors of the spleen and lymph node: Fibrosarcoma of the capsule of spleen, Leiomysarcoma of the spleen, and Lymphosarcoma of the lymph node (2 cases). Tumors of the lung: Bronchogenic carcinoma (3 cases), Adenocarcinoma type, Squamous carcinoma type, and Undifferentiated (round cell) carcinoma type respectively. Tumors of the alimentary tract and liver: Fibroma of the stomach, Hemangioma of the liver, Bile duct carcinoma, Liver cell carcinoma, and Myelogenous leukemia manifested in the liver. Tumor of the peritoneum: Fibrosarcoma. Tumors of the urogenital system: Fibroma of the uterus, Fibroma of the prepuce, Follicular cyst of the ovary, Transmissible venereal tumor of the vagina (6 cases), Carcinoma of the kidney, Adenoma of the prostate (2 cases), and Seminoma of the testis. Tumors of the mammary gland: Mixed tumor (2 cases), and Myoepithelioma. Tumor of the nervous system: Neurofibrosarcoma of the thigh.