University of Michigan Medical School, Ann Arbor, MI, USA.
BMC Ophthalmol. 2012 Aug 31;12:44. doi: 10.1186/1471-2415-12-44.
Schwannomas are slow-growing typically encapsulated tumors composed of differentiated Schwann cells, the primary class of peripheral glial cells. Complete excision is the treatment of choice for orbital schwannomas that cause pain, disfigurement, diplopia, or optic neuropathy. The presence of multiple schwannomas in a single patient suggests possible association with neurofibromatosis type 2 (NF2) or schwannomatosis.
We present 2 patients who experienced recurrent orbital schwannoma without evidence for neurofibromatosis. The recurrence in one patient, a 59-year old man, occurred 6 years after complete excision of the initial tumor. This recurrence consisted of 2 independent tumors in the same orbit. The recurrence in the second patient, a 5 year-old girl, occurred multiple times within days to weeks of partial excisions until eventually a complete excision was performed.
The clinical history, histopathologic features and particularly the intraoperative findings suggest that the 59 year old man suffers from orbital schwannomatosis, while the rapid recurrence in the second patient correlated with the cellular features of her plexiform schwannoma. Hence, the recurrence in each patient is linked to a different etiology, with implications for treatment and patient counseling given the difficulty in treating orbital schwannomatosis. To our knowledge, this is the first description of isolated orbital schwannomatosis.
神经鞘瘤是一种生长缓慢的肿瘤，通常被包膜包裹，由分化的施万细胞组成，施万细胞是主要的周围神经胶质细胞。对于引起疼痛、毁容、复视或视神经病变的眶内神经鞘瘤，完全切除是首选治疗方法。单个患者中存在多个神经鞘瘤提示可能与神经纤维瘤病 2 型（NF2）或神经鞘瘤病有关。
我们介绍了 2 名患有复发性眶内神经鞘瘤但无神经纤维瘤病证据的患者。其中 1 名 59 岁男性患者，在初次肿瘤完全切除 6 年后复发。该复发病例发生在同一眼眶内的 2 个独立肿瘤中。第 2 名患者是一名 5 岁女孩，在部分切除后数天至数周内多次复发，最终进行了完全切除。
患者的临床病史、组织病理学特征，特别是术中发现，提示 59 岁男性患者患有眶内神经鞘瘤病，而第二位患者的快速复发与她的丛状神经鞘瘤的细胞特征相关。因此，每位患者的复发都与不同的病因有关，这对治疗和患者咨询具有重要意义，因为治疗眶内神经鞘瘤病存在困难。据我们所知，这是首例孤立性眶内神经鞘瘤病的描述。
