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

The authors report on a case of primary malignant melanoma of the 7th cervical spinal nerve root in a 45-year-old woman. Neuro-radiological features of this extra-dural mass were suggestive of a nerve sheath tumor. The lesion underwent total gross resection through the anterolateral approach. The patient's postoperative course was uneventful. Histopathological investigation confirmed malignant melanoma. There was no evidence of tumor recurrence or other melanotic lesions on regular follow-up examinations until the postoperative eighth month. When treating a common, benign-looking lesion of the cervical spinal nerve root, surgeons should be aware of the potential to encounter such a malignant tumor.
Cervical Vertebrae ; Female ; Human ; Melanoma/*pathology/surgery ; Middle Aged ; Nerve Sheath Tumors/*pathology/surgery ; Peripheral Nervous System Neoplasms/*pathology/surgery ; Spinal Nerve Roots/*pathology

We describe a rare case of malignant transformation in a vestibular schwannoma in a 33-yr-old woman. She presented herself with headache, tinnitus, and hearing loss and underwent posterior fossa explorations three times during the short period of 3 months. The clinicopathological features of the original tumor were typical of benign vestibular schwannoma. Despite a comlpete microsurgical excision, two months later, the tumor recurred locally with a rapid increase in size causing a progressive worsening of neurological symptoms. A diagnosis of malignant schwannoma was made for the recurrent tumor on the basis of the microscopic findings of high cellularity, moderate pleomorphism, and the presence of mitotic cells. Repeat magnetic resonance imaging performed a month after the second surgery unexpectedly showed definite tumor enlargement. She remained clinically stable following the third debulking of the tumor and adjuvant radiotherapy. We propose that this recurrent tumor represent malignant transformation from a benign vestibular schwannoma which was an unusual occurrence in a patient without neurofibromatosis.
Adult ; Case Report ; Cell Transformation, Neoplastic ; Cranial Nerve Neoplasms/*pathology ; Female ; Human ; Magnetic Resonance Imaging ; Neoplasm Recurrence, Local ; Nerve Sheath Tumors/*pathology ; Neuroma, Acoustic/*pathology

We present a congenital plexiform cellular tumor with high mitotic activities arising in the right thigh of a 3-days-old infant. This subcutaneous tumor measured 6.5 x 4.5 cm in diameter with multinodular, whitish, elastic cut surface. Microscopically, the tumor was composed of fascicles of closely packed uniform spindle cells with frequent nuclear palisadings. The most remarkable finding was frequent mitoses(4-5/10 HPF). The tumor cells were strongly positive for S-100 protein, myelin basic protein and vimentin, and weakly positive for Leu 7. On electron microscopic examination, the spindle cells were found to be surrounded by continuous basal lamina and had interlocking long cytoplasmic processes. Although the prognosis of this tumor is difficult to predict, aggressive behavior such as recurrences may be suggested, but it is less likely to be metastatic. Further accumulation of similar unusual cases may be helpful in evaluation of its biologic behavior.
Case Report ; Human ; Infant, Newborn ; Male ; Neoplasm Proteins/analysis ; Nerve Sheath Tumors/chemistry/*congenital/pathology/surgery ; Soft Tissue Neoplasms/chemistry/*congenital/pathology/surgery ; Thigh ; Tumor Markers, Biological/analysis

Biomarkers, Tumor ; metabolism ; Hemangioendothelioma, Epithelioid ; metabolism ; pathology ; Hemangiosarcoma ; metabolism ; pathology ; Humans ; Immunohistochemistry ; Leiomyosarcoma ; metabolism ; pathology ; Lung Neoplasms ; metabolism ; pathology ; Nerve Sheath Neoplasms ; metabolism ; pathology ; Pulmonary Blastoma ; metabolism ; pathology ; Sarcoma ; metabolism ; pathology ; Sarcoma, Synovial ; metabolism ; pathology ; Solitary Fibrous Tumors ; metabolism ; 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

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

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