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

Plexiform schwannoma is a rare benign tumor arising from the peripheral nerve sheath and characterized by a multinodular and plexiform growth pattern. This tumor usually arises sporadically. In rare cases, plexiform schwannomas have been associated with neurofibromatosis type 2. Plexiform schwannoma should be differentiated from plexiform neurofibroma, because the latter is pathognomonic tumor of neurofibromatosis type 1 and has a potential of malignant transformation. We report a case of multiple plexiform schwannomas associated with bilateral acoustic neuromas and meningioma.
Meningioma ; Neurilemmoma* ; Neurofibroma, Plexiform ; Neurofibromatoses* ; Neurofibromatosis 1 ; Neurofibromatosis 2* ; Neuroma, Acoustic ; Peripheral Nerves

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

Neurofibromatosis is an autosomal dominant hereditary disorder characterized by skin abnormalities such as cafe au-lait spots, and soft tissue legion such as generalized subcutaneous neurofibroma. Malignant peripheral nerve sheath tumor is a spindle cell sarcoma that mostly arises in the soft tissue but rarely arises in the head and neck region. Malignant peripheral nerve sheath tumor can develop from the pre-existing neurofibromas or schwannomas, and from the peripheral nerves. They can also occur after radiation therapy. Malignant peripheral nerve sheath tumor is usually associated with neurofibromatosis type I. The authors recently experienced a case of malignant peripheral nerve sheath tumor associated with neurofibromatosis type I in a 20-year-old female patient who presented with a well marginated oval shape mass in the left neck.
Female ; Head ; Humans ; Neck ; Nerve Sheath Neoplasms ; Neurilemmoma ; Neurofibroma ; Neurofibromatoses ; Neurofibromatosis 1 ; Peripheral Nerves ; Sarcoma ; Skin Abnormalities ; Young Adult

Nerve sheath tumors of genitourinary tract are a rare and sometimes have challenging problems. Neurofibroma is a benign tumor characterized by diffuse proliferation of peripheral nerve elements. It rarely occurs in the retroperitoneal region. Although neurofibroma may occur as a solitary lesion, its common manifestation is in the syndrome of neurofibromatosis with a variable clinical Herein we report a case of giant retroperitoneal neurofibroma and review the subject briefly.
Nerve Sheath Neoplasms ; Neurofibroma* ; Neurofibromatoses ; Peripheral Nerves

Nerve sheath tumors are not frequent in the head and neck area. They might arise from large or small units of cranial, cervical and brachial plexuses as well as the cervical symphatic nerves. Their clinical course and findings are not similar, and preoperative diagnosis are extremely difficult in some cases. We experiencd eighteen cases of extracranial nerve sheath tumors in the head and neck regions during the past six years Twelve of them were schwannomas, and six of them were neurofibromas which did not associate with Von Recklinghausen's diseases. This analysis of eighteen cases and two representative cases are reported with literature review.
Brachial Plexus ; Diagnosis ; Head* ; Neck* ; Nerve Sheath Neoplasms* ; Neurilemmoma ; Neurofibroma

Schwannomas are benign nerve sheath tumors that can originate at any anatomic sites. These tumors are usually soitary, benign, and slow-growing masses attached to large nerve trunks. These tumors have been described as arising in the pelvic retroperitoneum. We present two cases of pelvic retroperitoneal Schwannomas with a brief review of literatures.
Nerve Sheath Neoplasms ; Neurilemmoma*

Schwannomas are the most common type of benign peripheral nerve sheath tumors. They typically present as a solitary lesion, but multiple schwannomas rarely occur in patients with neurofibromatosis type 2 (NF2), or patients without the other hallmarks of NF2. The latter is termed schwannomatosis. They most commonly occur in the head and neck involving the brachial plexus and spinal nerves. Although rarely found in the extremities, when these masses occur peripherally, they most commonly affect the sciatic, ulnar, and tibial nerve. It is reported that 2.4% to 5% of all patients undergoing schwannoma excision present as schwannomatosis. One-third of patients with schwannomatosis show tumors limited to a single extremity or segment of the spine and it is referred to as segmental schwannomatosis. We report a case of recurred segmental schwannomatosis of the posterior tibial nerve without features of NF2 after schwannoma excision.
Brachial Plexus ; Extremities ; Head ; Humans ; Neck ; Nerve Sheath Neoplasms ; Neurilemmoma ; Neurofibromatoses* ; Neurofibromatosis 2* ; Spinal Nerves ; Spine ; Tibial Nerve

Ancient schwannoma is a rare variant of schwannoma and a slow growing benign tumor associated with degeneration that may be diagnosed as a malignant tumor, because it presents with a large size and an inhomogeneous signal intensity. The main differential diagnosis of plexiform soft tissue tumor includes plexiform neurofibroma, malignant peripheral nerve sheath tumor (MPNST). In this case, we describe the MRI findings in a case of ancient schwannoma involving left thigh of a 63-year-old woman mimicking a plexiform MPNST. The tumor appeared as an inhomogeneous signal intensity and multinodular appearance, causing misdiagnosis as a plexiform MPNST.
Diagnosis, Differential ; Diagnostic Errors ; Female ; Humans ; Middle Aged ; Nerve Sheath Neoplasms ; Neurilemmoma ; Neurofibroma, Plexiform ; Peripheral Nerves ; Thigh

OBJECTIVE: This is a retrospective review of 22 surgically treated benign and malignant tumors of brachial plexus region to describe clinical presentation, the characteristics of brachial plexus tumor and clinical outcomes with a literature review. METHODS: Twenty-one patients with consecutive 22 surgeries for primary brachial plexus tumors were enrolled between February 2002 and November 2011 were included in this study. The medical records of all patients were reviewed. RESULTS: Eleven male and 10 female patients were enrolled. Mean age was 39 years. Three patients had brachial plexus tumor associated with neurofibromatosis (13.6%). Presenting signs and symptoms included parenthesis and numbness (54.5%), radiating pain (22.7%), direct tenderness and pain (27.2%), palpable mass (77.3%). Twelve patients presented preoperative sensory deficit (54.5%) and 9 patients presented preoperative motor deficit (40.9%). Twenty tumors (90.9%) were benign and 2 tumors (9.1%) were malignant. Benign tumors included 15 schwannomas (68.2%), 4 neurofibromas (18.2%) and 1 granular cell tumor (4.5%). There were 1 malignant peripheral nerve sheath tumor (MPNST) and 1 malignant granular cell tumor. Gross total resection was achieved in 16 patients (72.7%), including all schwannomas, 1 neurofibroma. Subtotal resection was performed in 6 tumors (27.3%), including 3 neurofibromatosis associated with brachial plexus neurofibromas, 1 MPNST and 2 granular cell tumor in one patient. CONCLUSION: Resection of tumor is the choice of tumor in the most of benign and malignant brachial plexus tumors. Postoperative outcomes are related to grade of resection at surgery and pathological features of tumor.
Brachial Plexus ; Female ; Granular Cell Tumor ; Humans ; Hypesthesia ; Male ; Medical Records ; Nerve Sheath Neoplasms ; Neurilemmoma ; Neurofibroma ; Neurofibromatoses ; Peripheral Nerves ; Retrospective Studies