title: Epithelioid hemangioendothelioma and multiple thoraco-lumbar lateral meningoceles: two rare pathological entities in a patient with NF-1.
abstract: Epithelioid hemangioendothelioma (EHE) is a rare vascular soft-tissue tumour of intermediate malignancy. Neurofibromatosis type I (NF-1) is a genetic syndrome associated with soft tissue sarcoma and higher risk of developing neoplasia. Lateral meningoceles are uncommon entities, being mostly associated with NF-1. We report a case of a 31-year-old woman, with NF-1 and past history of right thalamic/peduncular astrocytoma WHO grade II, admitted to the Neurosurgery Department in December 2003 due to severe low back pain, irradiating to the left leg without a radicular pattern. Thoraco-lumbar magnetic resonance imaging (MRI) showed a large left posterior paravertebral expansive lesion, bilateral and multiple thoraco-lumbar lateral meningoceles and dural ectasias with scalloping of the vertebral bodies. Biopsy of the paravertebral mass lesion disclosed EHE. We present this case because of the novel association between NF-1 and EHE, and the unusual aggressiveness of the neoplasia. Additionally, we highlight the co-existence of bilateral and multiple lateral meningoceles.
authors.affiliation: Departments of Neuroradiology, Hospital S. João, Alameda Prof. Hernâni Monteiro, 4202-451 Porto, Portugal. carinacoutoreis@hotmail.com
authors.forename: C
authors.initials: C
authors.lastname: Reis
authors.forename: E
authors.initials: E
authors.lastname: Carneiro
authors.forename: J
authors.initials: J
authors.lastname: Fonseca
authors.forename: P
authors.initials: P
authors.lastname: Pereira
authors.forename: R
authors.initials: R
authors.lastname: Vaz
authors.forename: R
authors.initials: R
authors.lastname: Pinto
authors.forename: A F
authors.initials: AF
authors.lastname: Capelinha
authors.forename: J M
authors.initials: JM
authors.lastname: Lopes
authors.forename: A
authors.initials: A
authors.lastname: Salgado
mesh_terms: D000328:Adult; D017809:Fatal Outcome; D005260:Female; D018323:Hemangioendothelioma, Epithelioid / Q000000981:diagnostic imaging / Q000209:etiology* / Q000473:pathology; D006801:Humans; D008159:Lumbar Vertebrae; D008588:Meningocele / Q000000981:diagnostic imaging / Q000209:etiology* / Q000473:pathology; D009456:Neurofibromatosis 1 / Q000150:complications*; D011859:Radiography; D013120:Spinal Cord Neoplasms / Q000000981:diagnostic imaging / Q000209:etiology* / Q000473:pathology; D013904:Thoracic Vertebrae