Humans ; Neurofibrosarcoma ; Adrenal Glands

Neurofibrosarcoma is the most common malignant change in patients with neurofibromatosis. But the incidence of neurofibrosarcoma arising from cutaneous neurofibroma is very low. We report two cases of neurofibrosarcoma arising from cutaneous neurofibroma in patients with neurofibromatosis showing typical clinical manifestations.
Humans ; Incidence ; Neurofibroma* ; Neurofibromatoses* ; Neurofibrosarcoma*

Two cases of neurofibrossrcoma involving cutaneous tissues of the patients with multiple neurofibromatosis were presented. One case, 49-year-old man, showed highly malignant histopathological feature with lethal outcome and the other case, 56-year-old man, showed less malignant histopathology with good recovery from local excision.
Humans ; Middle Aged ; Neurofibromatoses* ; Neurofibrosarcoma*

Malignant peripheral nerve sheath tumor (MPNST) is a rare neurogenic malignant tumor. MPNST has aty-pical clinical symptoms and imaging presentations, difficult diagnosis, a high degree of malignancy, and poor prognosis. It usually occurs in the trunk, approximately 20% in the head and neck, and rarely in the mouth. This paper reports a case of MPNST of the tongue. A summary of the clinical features, diagnosis, and treatment of MPNST is presented in combination with a literature review to provide a reference for the diagnosis and treatment of this disease.
Humans ; Nerve Sheath Neoplasms/pathology* ; Neurofibrosarcoma ; Tongue/pathology*

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.
Abdominal Wall ; Humans ; Nerve Sheath Neoplasms ; Neurilemmoma ; Neurofibroma ; Neurofibromatoses ; Neurofibromatosis 1 ; Neurofibrosarcoma ; Peripheral Nerves ; Prognosis ; Sarcoma

Malignant peripheral nerve sheath tumor (MPNST) is a term encompassing tumors previously diagnosed as malignant schwannoma, malignant neurilemmoma, neurogenic sarcoma, and neurofibrosarcoma The occurrence rate of MPNST in neurofibromatosis type 1 patients is known to be about 4.6%. Tumors occurring in this particular group have a worse prognosis in that they occur at an earlier age, are more centrally located, tend to be of a larger size and show more metastases and recurrences. We present a typical case of MPNST in a 36-year-old man with NF type 1, which occurred on the left buttock. A PET-CT showed findings of possible inguinal lymph node metastasis and a lymph node biopsy confirmed the diagnosis. The patient was treated with wide surgical resection and is undergoing adjuvant radiation therapy.
Adult ; Biopsy ; Buttocks ; Humans ; Lymph Nodes ; Neoplasm Metastasis ; Nerve Sheath Neoplasms ; Neurilemmoma ; Neurofibromatoses ; Neurofibromatosis 1 ; Neurofibrosarcoma ; Peripheral Nerves ; Prognosis ; Recurrence

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.
Chemotherapy, Adjuvant ; Diagnosis ; Female ; Humans ; Immunohistochemistry ; Middle Aged ; Neurilemmoma ; Neurofibroma ; Neurofibrosarcoma ; Periosteum ; Peripheral Nerves* ; Radiotherapy ; Sarcoma ; Scalp* ; Skin ; Skull

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.
Deglutition Disorders ; Extremities ; Head ; Headache ; Human Body ; Humans ; Incidence ; Middle Aged ; Neck ; Neurilemmoma ; Neurofibromatosis 1 ; Neurofibrosarcoma ; Peripheral Nerves ; Recurrence ; Sarcoma

OBJECTIVETo investigate the clinical characteristics, diagnosis and treatment of urinary neurofibrosarcoma.

METHODSClinical data of 1 case of urinary neurofibrosarcoma was analyzed retrospectively and related literature reviewed. The 61-year old male patient had undergone TURP for benign prostate hyperplasia ten months before.

RESULTSTotal urethral cavernostomy, total cystectomy and ileal cystostomy were performed on the patient and pathological diagnosis pointed to urinary neurofibrosarcoma. Another operation was done for metastatic rectal mass, which revealed abdominal metastasis. With progressing consumptive constitution, the patient died 40 days after the second operation.

CONCLUSIONIt is difficult to make a definite diagnosis in urinary neurofibrosarcoma, and the effective treatment for this highly malignant disease needs to be further studied.

Humans ; Male ; Middle Aged ; Neurofibrosarcoma ; diagnosis ; pathology ; surgery ; Retrospective Studies ; Urethral Neoplasms ; diagnosis ; pathology ; surgery ; Urologic Surgical Procedures ; methods

The CT can image both bone and soft tissue structures of paranasal sinuses and face and so CT has added animportant new dimension to radiological evaluation of disease of paranasal sinuses and face. CT is more accuratemethod of staging of tumors and essential for therapeutic planning. The author studied 25 cases of proven tumorsof paranasal sinuses and face during the period from Oct. 1977 to Aug.1980 in Kyung Hee University Hospital. Theresult were as follows; 1. Among 14 females and 11 males, their age range was form 14 years to 65 year. 2. Thedistribution of tumors were mucocele, squamous cell carcinoma, metastatic carcinoma, meningioma, angiofibroma,Masson's hemangiosarcoma, fibrous dysplasia, neurogenic sarcoma, Schwannoma, hemangioma, epidermoid, transitionalcell carcinoma and unknown. 3. Determination of location and extent of mucocele was easily done by CT. Thus in allcases of esthmoid mucocele, chief complaint of exophthalmos could be easily explained by identification of itsextension into peripheral fat space of orbit. 4. It is our belief that CT was useful method to determine stagingof tumors of paranasal sinuses and was essential in choosing appropriate treatment modality. 5. The contrastenhancement is generally not helpful in pathologic diagnosis of tumors but intracranial extension of tumors areclearly defined by contrast enhancement.
Carcinoma, Squamous Cell ; Diagnosis ; Exophthalmos ; Female ; Hemangioma ; Hemangiosarcoma ; Humans ; Male ; Meningioma ; Methods ; Mucocele ; Neurilemmoma ; Neurofibrosarcoma ; Orbit ; Paranasal Sinuses