Abstract：
BACKGROUND: Paragangliomas (PGLs) are rare neoplasms that may be associated with hereditary PGL syndromes and variable risk of metastasis. Middle ear adenomas are extremely rare tumors with no known hereditary predisposition and extremely low risk of metastasis. Although often easily differentiated, they may share clinical and pathological features that misdirect and confuse the diagnosis.
METHODS: The authors discussed a 35-year-old woman with left-sided hearing loss and bleeding from the external ear canal who presented to an outside hospital. She underwent resection of a middle ear and mastoid mass, initially diagnosed as a middle ear adenoma with neuroendocrine features, with later mastoidectomy and ligation of the sigmoid sinus with microsurgical excision of persistent tumor in the jugular foramen and temporal bone. Histopathologically, her tumor was vascular, composed of benign-appearing epithelioid cells with \"salt and pepper\" neuroendocrine chromatin arranged in vague nests. Lesional cells were GATA3-immunopositive, glucagon-negative, and succinate dehydrogenase-immunonegative, consistent with PGL rather than middle ear adenoma, and required further workup for hereditary PGL syndromes.
CONCLUSIONS: This case demonstrates potential challenges in differentiating a PGL from a middle ear adenoma. The authors offer clinical, histopathological, and imaging principles to aid in diagnosis and workup.
METHODS: The authors discussed a 35-year-old woman with left-sided hearing loss and bleeding from the external ear canal who presented to an outside hospital. She underwent resection of a middle ear and mastoid mass, initially diagnosed as a middle ear adenoma with neuroendocrine features, with later mastoidectomy and ligation of the sigmoid sinus with microsurgical excision of persistent tumor in the jugular foramen and temporal bone. Histopathologically, her tumor was vascular, composed of benign-appearing epithelioid cells with \"salt and pepper\" neuroendocrine chromatin arranged in vague nests. Lesional cells were GATA3-immunopositive, glucagon-negative, and succinate dehydrogenase-immunonegative, consistent with PGL rather than middle ear adenoma, and required further workup for hereditary PGL syndromes.
CONCLUSIONS: This case demonstrates potential challenges in differentiating a PGL from a middle ear adenoma. The authors offer clinical, histopathological, and imaging principles to aid in diagnosis and workup.
摘要：
背景：副神经节瘤（PGL）是罕见的肿瘤，可能与遗传性PGL综合征和不同的转移风险有关。中耳腺瘤是极其罕见的肿瘤，没有已知的遗传易感性和极低的转移风险。虽然通常很容易区分，他们可能有共同的临床和病理特征，误导和混淆诊断。
方法：作者讨论了一名35岁的女性，患有左侧听力损失和外耳道出血，她出现在医院外面。她接受了中耳和乳突肿块切除术，最初诊断为具有神经内分泌特征的中耳腺瘤，随后进行乳突切除术和乙状窦结扎，并显微手术切除颈静脉孔和颞骨的持续性肿瘤。组织病理学,她的肿瘤是血管性的,由良性上皮样细胞组成，带有“盐和胡椒”神经内分泌染色质，排列在模糊的巢中。病变细胞为GATA3免疫阳性,胰高血糖素阴性,和琥珀酸脱氢酶-免疫阴性，符合PGL而不是中耳腺瘤，并需要进一步检查遗传性PGL综合征。
结论：该病例显示了鉴别PGL与中耳腺瘤的潜在挑战。作者提供了临床，组织病理学,和成像原理，以帮助诊断和检查。
方法：作者讨论了一名35岁的女性，患有左侧听力损失和外耳道出血，她出现在医院外面。她接受了中耳和乳突肿块切除术，最初诊断为具有神经内分泌特征的中耳腺瘤，随后进行乳突切除术和乙状窦结扎，并显微手术切除颈静脉孔和颞骨的持续性肿瘤。组织病理学,她的肿瘤是血管性的,由良性上皮样细胞组成，带有“盐和胡椒”神经内分泌染色质，排列在模糊的巢中。病变细胞为GATA3免疫阳性,胰高血糖素阴性,和琥珀酸脱氢酶-免疫阴性，符合PGL而不是中耳腺瘤，并需要进一步检查遗传性PGL综合征。
结论：该病例显示了鉴别PGL与中耳腺瘤的潜在挑战。作者提供了临床，组织病理学,和成像原理，以帮助诊断和检查。
