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与混合性嗅神经母细胞瘤和颅咽管瘤相关的副肿瘤性边缘叶脑炎:一例报告及文献复习

Paraneoplastic limbic encephalitis associated with mixed olfactory neuroblastoma and craniopharyngioma: A case report and literature review.

作者信息

Nagafuji Hiroshi, Yokoi Hidenori, Fujiwara Masachika, Sato Dai, Saito Koichiro

机构信息

Department of Otolaryngology-Head and Neck Surgery, Kyorin University School of Medicine, Tokyo, Japan Department of Pathology, Kyorin University School of Medicine, Tokyo, Japan.

出版信息

Medicine (Baltimore). 2018 Jun;97(24):e10932. doi: 10.1097/MD.0000000000010932.

Abstract

RATIONALE

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.

PATIENT CONCERNS

The patient presented with generalized seizures, short-term memory loss, and a polypoid mass located high in the nasal cavity.

INTERVENTIONS

He underwent surgical resection of the tumor and postoperative chemoradiotherapy with concurrent intra-arterial cisplatin administration.

DIAGNOSIS

Pathological examination indicated an ONB admixed with craniopharyngioma.

OUTCOMES

The patient's neurological symptoms gradually diminished after surgery. No evidence of recurrence was observed during a 4-year follow-up.

LESSONS

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.

摘要

理论依据

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。

患者情况

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。

干预措施

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。

诊断

病理检查显示为混合颅咽管瘤的ONB。

结果

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。

经验教训

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。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7b94/6024189/bbaa037e0bd9/medi-97-e10932-g001.jpg

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