A Case of Malignant Peripheral Sheath Tumor Arising from Neurofibromatosis Type 1.
Min Ju KangHoon KangHyung Ok KimYoung Min ParkPublished in: Annals of dermatology (2008)
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.
Keyphrases
- pet ct
- lymph node metastasis
- lymph node
- radiation therapy
- peripheral nerve
- end stage renal disease
- newly diagnosed
- squamous cell carcinoma
- chronic kidney disease
- ejection fraction
- early stage
- spinal cord injury
- signaling pathway
- peritoneal dialysis
- risk assessment
- preterm infants
- papillary thyroid
- ultrasound guided
- computed tomography
- cell proliferation
- lps induced
- immune response
- pi k akt
- fine needle aspiration