Malignant Peripheral Nerve Sheath Tumor in Neurofibromatosis Type I : Unusual Presentation of Intraabdominal or Intrathoracic Mass
Case II : A 33 year-old woman with a known neurofibromatosis was admitted due to left upper chest pain. A chest PA and CT scan demonstrated a homogenous mass measuring 3.5×2.5 cm at the left upper lung field with a well-demarcated margin (). A wide excision was performed. The microscopic findings showed a MPNST with focal neurofibroma area and positive immunohistochemical staining for the S-100 protein on the cytoplasm of the tumor cells. She was rehospitalized 6 months later due to persistent coughing and dyspnea. A chest CT scan demonstrated an inhomogeneous mass and a large amount of a left pleural effusion (). A needle biopsy of the mass showed a tumor recurrence. After 6 cycles of MAID combination chemotherapy, a CT scan showed that the mass was markedly reduced (). She is currently being treated with radiotherapy.