Nagafuji Hiroshi, Yokoi Hidenori, Fujiwara Masachika, Sato Dai, Saito Koichiro
Department of Otolaryngology-Head and Neck Surgery, Kyorin University School of Medicine, Tokyo, Japan Department of Pathology, Kyorin University School of Medicine, Tokyo, Japan.
Medicine (Baltimore). 2018 Jun;97(24):e10932. doi: 10.1097/MD.0000000000010932.
Paraneoplastic limbic encephalitis (PLE) is a rare disorder of the nervous system associated with malignant disease. It has a subacute onset with the following symptoms: cognitive dysfunction, seizures, irritability, hallucinations, and short-term memory loss. Herein, we report the case of a 35-year-old man with PLE, an olfactory neuroblastoma (ONB) admixed with craniopharyngioma, and serum anti-Hu antibodies.
The patient presented with generalized seizures, short-term memory loss, and a polypoid mass located high in the nasal cavity.
He underwent surgical resection of the tumor and postoperative chemoradiotherapy with concurrent intra-arterial cisplatin administration.
Pathological examination indicated an ONB admixed with craniopharyngioma.
The patient's neurological symptoms gradually diminished after surgery. No evidence of recurrence was observed during a 4-year follow-up.
We reported a histologically unusual heterogeneous tumor that comprised ONB and craniopharyngioma. This is the first reported case of PLE with anti-Hu antibodies possibly associated with ONB admixed with craniopharyngioma.
副肿瘤性边缘叶脑炎（PLE）是一种与恶性疾病相关的罕见神经系统疾病。其起病亚急性，有以下症状：认知功能障碍、癫痫发作、易怒、幻觉和短期记忆丧失。在此，我们报告一例35岁患有PLE的男性病例，其患有混合颅咽管瘤的嗅神经母细胞瘤（ONB）以及血清抗Hu抗体。
该患者出现全身性癫痫发作、短期记忆丧失以及鼻腔高处的息肉样肿物。
他接受了肿瘤手术切除以及术后同步动脉内给予顺铂的放化疗。
病理检查显示为混合颅咽管瘤的ONB。
患者术后神经症状逐渐减轻。在4年随访期间未观察到复发迹象。
我们报告了一例组织学上不寻常的异质性肿瘤，其由ONB和颅咽管瘤组成。这是首例报告的可能与混合颅咽管瘤的ONB相关的伴有抗Hu抗体的PLE病例。
