Lal Shubha, Pant Ishita, Chaturvedi Sujata, Sarma Pragyan
Institute of Human Behaviour and Allied Sciences, Department of Pathology, New Delhi, India.
Guru Teg Bahadur Hospital, Department of Neurosurgery, New Delhi, India.
Autops Case Rep. 2021 May 6;11:e2021277. doi: 10.4322/acr.2021.277. eCollection 2021.
Paragangliomas are rare, encapsulated, benign neuroendocrine tumors that can arise from the adrenal medulla or extra-adrenal paraganglia. Extra-adrenal paragangliomas may develop a gangliocytic component with ganglion cells (Gangliocytic paragangliomas). Nearly 25%of cauda equina paragangliomas are gangliocytic paragangliomas. Here, we describe the case of a 35-year-old male who presented with weakness of both lower limbs over the last two months. Radiological findings were suggestive of myxopapillary ependymoma. However, the histopathological examination revealed a tumor with cells arranged in sheets, papillae, lobules, and around vessels forming pseudo rosettes. Ganglion cells were seen in small groups and, also singly. Tumor cells were immunopositive for chromogranin, synaptophysin, and S-100. Ganglion cells were immunopositive for synaptophysin, NSE, and NFP. A final histological diagnosis of Gangliocytic paraganglioma (WHO grade I) was made. To date, only nine gangliocytic paraganglioma cases have been previously reported, and to the best of our knowledge, this is the largest gangliocytic paraganglioma.
副神经节瘤是一种罕见的、有包膜的良性神经内分泌肿瘤，可起源于肾上腺髓质或肾上腺外副神经节。肾上腺外副神经节瘤可能会发展出带有神经节细胞的神经节细胞成分（神经节细胞性副神经节瘤）。近25%的马尾副神经节瘤是神经节细胞性副神经节瘤。在此，我们描述一例35岁男性病例，该患者在过去两个月出现双下肢无力。影像学检查结果提示为黏液乳头型室管膜瘤。然而，组织病理学检查显示肿瘤细胞呈片状、乳头状、小叶状排列，并围绕血管形成假菊形团。可见小群及单个的神经节细胞。肿瘤细胞嗜铬粒蛋白、突触素和S-100免疫阳性。神经节细胞突触素、神经元特异性烯醇化酶和神经丝蛋白免疫阳性。最终组织学诊断为神经节细胞性副神经节瘤（世界卫生组织I级）。迄今为止，此前仅报道过9例神经节细胞性副神经节瘤病例，据我们所知，这是最大的神经节细胞性副神经节瘤。
