A malignant peripheral nerve sheath tumor (MPNST) is an extremely rare soft tissue tumor in the general population. On the other hand, there is a higher incidence of MPNST in patients with neurofibromatosis type I (von Recklinghausen's disease). The common sites are the extremities, trunk, head and neck. However, an intraabdominal or intrathoracic manifestation is uncommon. This paper reports two patients, a 31 year-old woman with multiple neurofibromatosis presenting as an intraabdominal malignant peripheral nerve sheath tumor, and a 33 year-old woman with an intrathoracic malignant peripheral nerve sheath tumor. The patients were treated with chemotherapy followed by radiotherapy. However, one patient died as a result of disease progression 21 months after the diagnosis and the other patient is currently being treated with radiotherapy.
Abdominal Neoplasms/*diagnosis ; Adult ; Female ; Humans ; Nerve Sheath Tumors/*diagnosis ; Neurofibromatosis 1/*complications ; Thoracic Neoplasms/*diagnosis

Peripheral nerve sheath tumors rarely occur in the oral cavity and include neurofibroma, schwannoma, and palisaded encapsulated neuroma. We report a rare case of benign peripheral nerve sheath tumor of the tongue. This tumor was a 0.8 x 0.5 cm sized, firm mass on the left lateral surface of the tongue. Histologically, this mass was composed of well- circumscribed variable sized nodules, which consisted of moderately cellular spindle cells with vague nuclear palisading and a small amount of fibrous tissue. Most of the tumor cells were strongly positive for S-100 protein, but negative for epithelial membrane antigen on immunohistochemistry. No axons were found by immunostaining for neurofilament and Bodian stains. In addition, the surrounding, compressed, fibrous tissue showed rare EMA- positive cells. The present case might be a rare case of neurofibroma arising in the tongue, although immunohistochemical and special stains did not support such a diagnosis.
Adult ; Case Report ; Female ; Human ; Immunohistochemistry ; Nerve Sheath Tumors/chemistry/diagnosis/*pathology ; S100 Proteins/analysis ; Tongue Neoplasms/chemistry/diagnosis/*pathology

A 51-year-old woman was presented with a palpable mass in the infraauricular area that had existed for 4-months. The mass from the superficial lobe of the parotid gland was 2.7 cm in the greatest dimension and was ill-defined with rubbery, homogeneous, and fibrotic appearance. Histologically, the lesion was infiltrative into the lobules of the paratid gland, and was composed of a proliferation of spindle or stellate cells with cellular and sclerotic areas. On immunohistochemistry, the cells were only positive for vimentin and focally for smooth muscle actin. Differential diagnosis from nerve sheath tumors and solitary fibrous tumors involving the parotid gland was emphasized.
Actins ; Diagnosis, Differential ; Female ; Fibroma* ; Humans ; Immunohistochemistry ; Middle Aged ; Muscle, Smooth ; Nerve Sheath Neoplasms ; Parotid Gland* ; Solitary Fibrous Tumors ; Vimentin

Malignant peripheral nerve sheath tumor (MPNST) is defined as any malignant tumor arising from or differentiating toward the cells of the peripheral nerve sheath. MPNST accounts for about 5-10% of all soft tissue tumors and is often associated with neurofibromatosis type I (NF-1, von Recklinghausen's disease). It is one of the malignant tumors associated with von Recklinghausen's disease. Its common site is the lower and upper extremities, trunk, head and neck. But intrathoracic manifestations are very rare. We report a case of a 40 year-old man with multiple neurofibromatosis who was presented with an intrathoracic malignant peripheral nerve sheath tumor
Adult ; Case Report ; Diaphragm/pathology ; Human ; Lung/pathology ; Male ; Nerve Sheath Tumors/complications/diagnosis/*pathology ; Neurofibromatosis 1/complications/diagnosis/*pathology ; Prognosis ; Severity of Illness Index ; Thoracic Neoplasms/complications/diagnosis/*pathology

Adult ; Diagnosis, Differential ; Female ; Fibroma ; metabolism ; pathology ; Fingers ; Giant Cell Tumors ; metabolism ; pathology ; Humans ; Mucin-1 ; metabolism ; Nerve Sheath Neoplasms ; metabolism ; pathology ; surgery ; Neurilemmoma ; metabolism ; pathology ; Sclerosis ; metabolism ; pathology ; Soft Tissue Neoplasms ; metabolism ; pathology ; surgery ; Tendons ; Young Adult

Diagnosis, Differential ; Hemangiopericytoma ; metabolism ; pathology ; Hemangiosarcoma ; metabolism ; pathology ; Histiocytoma, Malignant Fibrous ; metabolism ; pathology ; Humans ; Immunohistochemistry ; Leiomyosarcoma ; metabolism ; pathology ; Nerve Sheath Neoplasms ; metabolism ; pathology ; Neuroectodermal Tumors, Primitive ; metabolism ; pathology ; Rhabdoid Tumor ; metabolism ; pathology ; Rhabdomyosarcoma ; metabolism ; pathology ; Soft Tissue Neoplasms ; metabolism ; pathology ; Urinary Bladder ; metabolism ; pathology