Neurofibromatosis type 1 can be severe and associated with malignant transformation. Proper follow-up and monitoring are very important in preventing the malignant transformation of neurofibromatosis. We encountered a case of malignant transformation of plexiform neurofibroma into neurofibrosarcoma (also known as malignant peripheral nerve sheath tumor). She had been presenting with a large mass on her back for a few years, which was also associated with an ulcer. She underwent a wide-excision biopsy of her back, and the histopathology examination (HPE) came back with a malignant peripheral nerve sheath tumor. This case concludes that any patient with a known case of neurofibromatosis should undergo follow-up to detect any malignant transformation of the disease. Early detection of the malignant transformation of neurofibromatosis can help prevent the disease\'s progression. The main treatment is surgical resection; however, the risk of local recurrence is higher, especially in patients with neurofibromatosis type 1.

UNASSIGNED: Malignant triton tumors (MTT) are subtype of malignant peripheral nerve sheath tumor (MPNST) which develop from Schwan cells of peripheral nerves or within neurofibromas, and shows rhabdomyoblastic differentiation. It is a rare soft tissue tumor with poor prognosis.
UNASSIGNED: We report a case of Malignant Triton Tumor (MTT) arising in the right shoulder in a 46 year old male patient presented to our Musculoskeletal Oncology Clinic at Royal Rehabilitation center at King Hussein Medical Center during June 2018.
UNASSIGNED: The patient was complaining of an 8 months long progressive right shoulder pain and swelling at the posterior lateral area of the shoulder. As accurate diagnosis is crucial in such case, investigations that included x-rays and magnetic resonance imaging (MRI) demonstrated an soft tissue tumor involving the right shoulder area leading to the differential diagnosis of aggressive soft tissue tumor which laid down the plan of an open incisional biopsy to be reported histopathological as a case of Malignant Triton Tumor which is a very rare and aggressive sarcoma originates from the peripheral nerve sheaths as it is subtype of malignant peripheral nerve sheath tumors after which excision of the entire tumor with safety margin was performed and referred for adjuvant chemotherapy.
UNASSIGNED: The treatment of choice is radical tumor excision with wide margins followed by chemotherapy and /or radiotherapy to improve the 5 years survival rates.

The cytomorphology of MPNST in effusion specimens is rarely described. In this paper, the detailed cytopathological and immunohistochemical characteristics of metastatic MPNST has been described in pleural effusion. Patients\' medical history and the judicious utilization of ancillary studies contribute to ensure precise cytological diagnoses. The cytomorphology of malignant peripheral nerve sheath tumour (MPNST) in effusion specimens can be diagnostically challenging. The author presents detailed cytopathological and immunohistochemical characteristics of a case of metastatic MPNST in pleural effusion.

UNASSIGNED: Malignant peripheral nerve sheath tumors of the scalp are rare neoplasms of the peripheral nervous system. Here, we describe an unusual malignant peripheral nerve sheath tumor of the scalp in an 84-year-old Asian man. The tumor was associated with bony destruction, intracranial, and extracranial extension. Trans-arterial embolization was done twice preoperatively. En block excision was performed and the dura and soft tissue defect were reconstructed by anterolateral thigh free fasciocutaneous flap. There is no recurrence and the wound healed well during follow-up.

Malignant triton tumor (MTT) is a highly aggressive malignant neoplasm, classified as a variant of malignant peripheral nerve sheath tumor with rhabdomyoblastic differentiation. There are few reports that MTT occurred in urogenital system. In the present study, we report the first MTT occurring in the uterus. A 57-year-old woman came to the emergency department due to persistent vaginal bleeding for 2 months. The gynecological palpation found that a club-shaped excrescence existed in the vagina about 7 cm × 3 cm × 3 cm. The mass located in the lower segment of the uterus and the cervix was confirmed by gynecological vaginal ultrasound and magnetic resonance imaging, which was preliminarily diagnosed as cervical carcinoma. After neoplasm punch biopsy, the pathological diagnosis was malignant triton tumor. The patient finally lost follow-up. This is the first report about MTT in the uterus and suggests that pathological biopsy combined with imaging examination is necessary for the diagnosis of rarely MTT.

BACKGROUND: Neurofibromatosis type 1 (NF1) is an autosomal dominant neurocutaneous syndrome that causes multiple central and peripheral nerve sheath tumors. People with NF1 have a 10% chance of developing malignant peripheral nerve sheath tumors (MPNSTs). Here we report a unique instance of a malignant schwannoma that has remained free of metastasis since its initial removal a decade ago. The malign schwannoma has been infrequently documented in the literature, and remarkably, no instances of such an extensive postoperative time without metastases have ever been described.
METHODS: A 46-year-old male patient with NF had multiple neurofibromas in different parts of his body, underwent surgery about 10 years ago (2013), and was diagnosed histopathologically as MPNST.
METHODS: He was admitted to our institution with a recurrent mass in the posterior third of the proximal thigh and severe pain radiating to the left lower extremity, which presented as sciatic pain (2021). A magnetic resonance imaging and fluorodeoxyglucose-positron emission tomography examination revealed that the tumor was likely malignant.
METHODS: Surgical excision was performed.
RESULTS: A 10-year follow-up revealed no metastases or neurologic impairment.
CONCLUSIONS: When articles about benign schwannomas are placed in a separate category, little is written about NF-1-related malignant schwannomas of the sciatic nerve. MPNSTs are high-grade, aggressive sarcomas with a high risk of local recurrence (40%-65%) and metastasis to other body parts. Therefore, among the various benign peripheral nerve sheath tumors in NF-1 patients, the diagnosis of MPNST is crucial.Orthopedic surgeons should be aware that neurofibromas in NF-1 have a significant risk of developing MPNSTs. This study reports the successful treatment of a giant malignant sciatic nerve schwannoma with a long follow-up period without metastasis.

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BACKGROUND: Malignant peripheral nerve-sheath tumor (MPNST) is an extremely rare sarcoma of the nasal cavity and paranasal sinuses. Non-specific clinical and radiological presentation and challenging histological diagnosis make it little known by physicians. We describe a case of maxillary sinus MPNST, following CARE guidelines.
METHODS: A 62-year-old woman consulted for swelling of the right cheek and hard palate with several months\' progression. CT and MRI revealed a tissue mass in the right maxillary sinus with osteolysis of the orbital and maxillary floors, hard palate and lateral and medial walls of the maxillary sinus. Biopsy confirmed diagnosis of low-grade MPNST. After total resection and 60Gy adjuvant radiotherapy, 2-year follow-up showed no signs of recurrence.
CONCLUSIONS: MPNST in the nasal cavity and paranasal sinuses is very rare. Because of a high risk of recurrence, wide resection should be implemented, possibly completed by radiotherapy if resection cannot be complete because of proximity to at-risk structures.

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