Malignant peripheral nerve sheath tumor in neurobifromatosis type-1: two case reports
نویسندگان
چکیده
INTRODUCTION Malignant peripheral nerve sheath tumors are rare soft tissue sarcomas. They are considered to carry a poor prognosis with current therapeutic approaches. Successful treatment depends on a multimodal approach. CASE PRESENTATION The authors report two cases with malignant peripheral nerve sheath tumors arising from pre-existing neurofibromas in the grounds of neurofibromatis-type I. Complete surgical removal of all lesions is considered before and after induction chemotherapy. Correlation of the response to chemotherapy in the context of the immuno-histopathological features of the tumors is also discussed with reference to the existing literature. CONCLUSION A need for a multidisciplinary approach with chemotherapy, surgery and radiotherapy is anticipated in the management of malignant peripheral nerve sheath tumors as described in these two reported cases. It is felt that further research on the molecular aspects of malignant peripheral nerve sheath tumors and neurofibromatis-type I will optimize treatment strategies in the future.
منابع مشابه
Malignant Peripheral Nerve Sheath Tumor in the Maxilla: Report of a Rare Case
Malignant peripheral nerve sheath tumor (MPNST) is a rare malignant tumor that develops either from a preexisting neurofibroma or de novo. The cell of origin is believed to be the Schwann cell and possibly other nerve sheath cells. In this report, we describe a rare case of MPNST that arise from the socket of second left maxillary molar that has been already extracted in a young man. He was ref...
متن کاملIntrathoracic Malignant Peripheral Nerve Sheath Tumor: Histopathological and Immunohistochemical Features
Malignant peripheral nerve sheath tumor (MPNST) is a rare nerve sheath tumor derived from Schwann cells or pleuripotent cells of neural crest. Neurogenic tumors make about 10-20% of all mediastinal tumors. Incidence of MPNST is 0.001% in general population and 0.16% in patients with neurofibromatosis I (NF I). We report a case of 60 year female presenting with progressive cough and breathlessne...
متن کاملPrimary Intracranial Malignant Nerve Sheath Tumor in the Cerebellopontine Angle in a Woman with Neurofibromatosis Type 2
Neurofibromatosis type 2 is an inherited disease. The main manifestation of the disease is the development of symmetric, non-malignant brain tumors in the region of the cranial nerve VIII usually as schwannoma. We report here a 20-year-old woman with primary intracranial malignant nerve sheath tumor located in the left cerebellopontine angle. Histologically, the tumor showed malignant spindle c...
متن کاملHuge Intrathoracic Malignant Peripheral Nerve Sheath Tumor in an Adolescent with Neurofibromatosis Type 1
Malignant peripheral nerve sheath tumor (MPNST) is a rare soft tissue malignancy usually found in patients with neurofibromatosis type 1 (NF1) with a poor outcome. Although MPNST can be found in any part of the body including head and neck or extremities, intrathoracic MPNST with or without NF1 is uncommon, especially in children or adolescents. Reported herein is a case of huge intrathoracic M...
متن کاملMalignant Triton Tumors in Sisters with Clinical Neurofibromatosis Type 1
Malignant triton tumors (MTTs) are rare and aggressive sarcomas categorized as a subgroup of malignant peripheral nerve sheath tumors (MPNSTs). MTTs arise from Schwann cells of peripheral nerves or existing neurofibromas and have elements of rhabdomyoblastic differentiation. We report the occurrence of MTTs in two sisters. The first patient is a 36-year-old female who presented with left sided ...
متن کامل