Levy Michael T, Braun John T, Pennant Marjorie, Thompson Lester D R
Department of Pathology, Southern California Permanente Medical Group, Woodland Hills Medical Center, 5601 De Soto Avenue, Woodland Hills, CA 91365, USA.
Head Neck Pathol. 2010 Mar;4(1):37-43. doi: 10.1007/s12105-009-0157-7. Epub 2009 Dec 24.
Paragangliomas are relatively uncommon neoplasms that arise in adrenal and extra-adrenal paraganglia of the autonomic nervous system. Parasympathetic paraganglioma develop predominantly in the head and neck. It is exceedingly uncommon to develop a primary intraparathyroid paraganglioma. There is only a single case report in the English literature. The information from the single previous case report (Medline 1960-2009) was combined with this case report. Our patient was a 69 year old woman who presented with a thyroid gland mass, with extension into the substernal space. The patient had a history of renal cell carcinoma removed 18 months before. At surgery, a thyroid lobectomy and a parathyroidectomy were performed. The parathyroid tissue showed a very well defined zellballen arrangement of paraganglion cells within the parenchyma of the parathyroid gland. The cells had ample basophilic, granular cytoplasm. The nuclei were generally round to oval with 'salt-and-pepper' nuclear chromatin distribution. There was a richly vascularized stroma. Mitotic figures, necrosis, invasive growth, and profound nuclear pleomorphism were absent. The neoplastic cells were strongly and diffusely immunoreactive with chromogranin, synaptophysin, CD56, and focally with cyclin-D1. The paraganglioma showed a delicate S-100 protein positive supporting sustentacular framework. Keratin, CD10, PTH, calcitonin and RCC markers were negative. The patient showed no stigmata of Multiple Endocrine Neoplasia (MEN) and has no paraganglioma in any other anatomic site. She is alive without any additional findings 12 months after surgery. Isolated paraganglioma within the parathyroid is rare, and should be separated from parathyroid adenoma, hyperplasia or metastatic disease to assure appropriate management.
副神经节瘤是相对罕见的肿瘤，起源于自主神经系统的肾上腺和肾上腺外副神经节。副交感神经副神经节瘤主要发生在头颈部。原发性甲状旁腺内副神经节瘤极为罕见。英文文献中仅有一例病例报告。将之前这例病例报告（Medline 1960 - 2009）的信息与本病例报告相结合。我们的患者是一位69岁女性，表现为甲状腺肿物，并延伸至胸骨后间隙。该患者有18个月前肾细胞癌切除史。手术中进行了甲状腺叶切除术和甲状旁腺切除术。甲状旁腺组织显示在甲状旁腺实质内副神经节细胞呈界限非常清晰的巢状排列。细胞具有丰富的嗜碱性颗粒状细胞质。细胞核通常呈圆形至椭圆形，核染色质呈“椒盐”样分布。有丰富血管化的间质。未见有丝分裂象、坏死、浸润性生长及明显的核多形性。肿瘤细胞对嗜铬粒蛋白、突触素、CD56呈强弥漫性免疫反应，对细胞周期蛋白D1呈局灶性免疫反应。副神经节瘤显示出纤细的S - 100蛋白阳性支持性支持细胞框架。角蛋白、CD10、甲状旁腺激素、降钙素和肾细胞癌标志物均为阴性。该患者无多发性内分泌肿瘤（MEN）体征，其他任何解剖部位均无副神经节瘤。术后12个月她存活且无任何其他异常发现。甲状旁腺内孤立性副神经节瘤罕见，应与甲状旁腺腺瘤、增生或转移性疾病相鉴别，以确保恰当的处理。
