Pal L, Bansal K, Behari S, Sagar B Chandrashekhar, Gupta R K, Gupta R K, Shankar S K
Department of Pathology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India.
Clin Neuropathol. 2002 Mar-Apr;21(2):47-51.
Neurothekeomas are rare, benign nerve sheath tumors, usually arising from cutaneous nerves in the head and neck region. So far, only 4 cases of the intracranial counterpart have been reported and these were located in the posterior fossa, sellar and parasellar region. We report here the first case of neurothekeoma located deep inside the brain parenchyma. Histologically, the tumor had lobular appearance with bland morphology in spite of cellular pleomorphism, myxoid background and variable S-100 positivity, characteristics of neurothekeomas. The schwannian origin was further confirmed ultrastructurally by demonstrating basal lamina and Luse bodies. Because of its benign nature, the lesion does not need postoperative radiotherapy. Histogenesis of neurothekeoma arising in the parenchyma remains enigmatic. The probable cell of origin could be the Schwann cell or perineurial cell of the nerve twigs around the blood vessels or by extreme differentiation of the precursor cell resting in a suitable microenvironment to the schwannian phenotype.
神经鞘黏液瘤是一种罕见的良性神经鞘瘤，通常起源于头颈部皮肤神经。迄今为止，仅报道过4例颅内神经鞘黏液瘤，且均位于后颅窝、鞍区和鞍旁区域。我们在此报告首例位于脑实质深部的神经鞘黏液瘤。组织学上，尽管肿瘤具有细胞多形性、黏液样背景和S-100阳性程度不一等神经鞘黏液瘤的特征，但呈小叶状外观且形态温和。通过显示基底膜和Luse小体，超微结构进一步证实了其雪旺氏起源。由于其良性性质，该病变无需术后放疗。脑实质内神经鞘黏液瘤的组织发生仍然不明。可能的起源细胞可能是血管周围神经小枝的雪旺细胞或神经束膜细胞，或者是处于合适微环境中的前体细胞向雪旺氏表型的极端分化。
