Patrinou Alexandra, Malindretos Pavlos, Koutroubas Georgios, Anagnostou Nikolaos, Argiraki Elefteria, Syrganis Christos
Department of Nephrology and Hypertension, Achillopouleio General Hospital of Volos, Volos, Greece.
NDT Plus. 2010 Jun;3(3):288-290. doi: 10.1093/ndtplus/sfq039. Epub 2010 Mar 24.
Neurofibromatosis Type 2 (NF2) is a dominantly inherited tumour-prone disorder, characterized by the development of multiple schwannomas, meningiomas and ependymomas. Its prevalence is around 1:60 000. Vestibular schwannoma (VS) is the hallmark of NF2. Retroperitoneal schwannomas are expected to occur in only 3% of cases. We present the case of a large retroperitoneal schwannoma in a patient with NF2. A well-circumscribed heterogenic mass (9.5 × 4 × 4 cm) behind and under the left kidney and extending into the left retroperitoneal space was revealed during a lumbar and retroperitoneal space magnetic resonance imaging (MRI). Brain, orbits, cervical, thoracic and lumbar MRI revealed bilateral VS, multiple meningiomas as well as multiple schwannomas and ependymomas in the cervical, thoracic and lumbar spine. The retroperitoneal mass represents a schwannoma probably derived from an intercostal nerve. The patient underwent neurosurgical excision of the VS, and 3 months later, the patient's condition remained stable.
2型神经纤维瘤病（NF2）是一种显性遗传的肿瘤易感性疾病，其特征是多发施万细胞瘤、脑膜瘤和室管膜瘤。其患病率约为1:60000。前庭神经鞘瘤（VS）是NF2的标志。腹膜后施万细胞瘤预计仅在3%的病例中出现。我们报告一例NF2患者发生的巨大腹膜后施万细胞瘤。腰椎和腹膜后间隙磁共振成像（MRI）显示左肾后方及下方有一个边界清晰的异质性肿块（9.5×4×4cm），并延伸至左腹膜后间隙。脑部、眼眶、颈椎、胸椎和腰椎MRI显示双侧VS、多发脑膜瘤以及颈椎、胸椎和腰椎的多发施万细胞瘤和室管膜瘤。腹膜后肿块为可能源自肋间神经的施万细胞瘤。患者接受了VS的神经外科切除，3个月后，患者病情保持稳定。
