A case of malignant atrophic papulosis with cranial nerve and peripheral nerve impairment*
نویسندگان
چکیده
Malignant atrophic papulosisis is a rare, multisystem obliterative vasculopathy of unknown etiology, occasionally involving the cranial nerve. We describe the first case of malignant atrophic papulosisis with cranial nerve and peripheral nerve involvement in China. A 47-year-old woman presented to our hospital with atrophic porcelain white papules over the trunk and extremities, numbness in the right calf, vision decrease and impaired movement of the right eye. She was diagnosed with malignant atrophic papulosisis, based on characteristic symptoms and histopathologic examination. The patient was treated with dipyridamole and aspirin for 9 months, but later died of gastrointestinal hemorrhage. We reviewed currently available case reports on cranial nerve involvement in malignant atrophic papulosisis and emphasized the importance of skin biopsy in diagnosing this disease.
منابع مشابه
18F-FDG PET/CT in Neurolymphomatosis: Report of 3 Cases
Neurolymphomatosis is a rare manifestation of non-Hodgkin lymphoma characterized by infiltration of peripheral nerves, nerve roots, plexus and cranial nerves by malignant lymphocytes. This report presents positron emission tomography/computed tomography (PET/CT)imaging with 2-deoxy-2-18F-fluoro-D-glucose (18F-FDG) in 3 cases of non-Hodgkin lymphoma with nerve infiltration, including one newly d...
متن کاملMalignant 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 کاملHead and Neck Nerve Sheath Tumors: A 10-Year Evaluation in Iran
Background and Objective: Approximately 45% of benign peripheral nerve sheath tumors (PNST) occur in head and neck regions. Malignant peripheral nerve sheath tumors (MPNST) are less common and only 8-15% of these tumors occur in head and neck region. In this study, we aimed at evaluating the demographic characteristics, pathologic findings, locations, main clinical presentations and family h...
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 کامل