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Case Reports
. 2023 Dec 15;19(3):2294579.
doi: 10.1080/21645515.2023.2294579. Epub 2023 Dec 18.

Case report: Leptomeningeal metastasis of advanced nasopharyngeal carcinoma treated with chemoimmunotherapy

Mengting Shi  1   2   3   4 , Dongchen Sun  1   2   3   4 , Xilun Ma  5 , Jiaqing Liu  2   3   6 , Yaxiong Zhang  1   2   3   4 , Tingting Liu  1   2   3   4 , Xueyuan Chen  1   2   3   4 , Silang Mo  1   2   3   4 , Yuanyuan Zhao  1   2   3 , Li Zhang  1   2   3
Affiliations
Case Reports

Case report: Leptomeningeal metastasis of advanced nasopharyngeal carcinoma treated with chemoimmunotherapy

Mengting Shi et al. Hum Vaccin Immunother. .

Abstract

Leptomeningeal metastasis (LM) of nasopharyngeal carcinoma (NPC) is rare and associated with a poor prognosis. Immune checkpoint inhibitors (ICIs) have been the standard first-line treatment for metastatic NPC, but their effect on meningeal metastasis of NPC needs further investigation. A 38-year-old man complained of bilateral neck masses and sought medical care. He was diagnosed with nasopharyngeal undifferentiated non-keratinizing carcinoma with bilateral cervical lymph node metastasis and multiple bone metastasis, stage cT4N2M1 IVb. Then, the patient received first-line anti-PD-1 antibody tislelizumab combined with gemcitabine and cisplatin and achieved partial response. After seven cycles of first-line chemoimmunotherapy, the patient subsequently developed neurological symptoms, including unsteady walking, slurred speech, coughing on drinking, and unconsciousness. MRI showed leptomeningeal linear enhancement, and cerebrospinal fluid (CSF) analysis indicated Epstein-Barr virus (EBV) infection and squamous cell carcinoma cytology, suggesting the diagnosis of leptomeningeal metastasis. After the definite diagnosis of LM, the patient's condition deteriorated rapidly, leading to his death from brain herniation. We reported the first case of advanced NPC with pathologically confirmed leptomeningeal metastasis after receiving first-line chemoimmunotherapy. Considering the poor prognosis of LM, it is suggested to perform MRI and CSF examination when patients have neurological symptoms. Although immunotherapy significantly improved survival outcomes of advanced NPC patients, it seemed not effective in the setting of LM. The effect of other treatment options, such as radiation therapy and intrathecal therapy, requires further verification.

Keywords: EBV; Nasopharyngeal carcinoma; cerebrospinal fluid; immunotherapy; leptomeningeal metastasis.

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Conflict of interest statement

No potential conflict of interest was reported by the author(s).

Figures

Figure 1.
Figure 1.
(a). Magnetic resonance imaging (MRI) showed the nasopharyngeal mass occupies the right pharyngeal recess, bilateral pterygopalatine fossa, posterior nostrils, left posterior nasal cavity, ethmoid sinus, sphenoid sinus, bilateral cavernous sinus, and involves the left Merkle cavity and Gasser ganglion. (b). Baseline cranial MRI of this patient showed no signs of meningeal metastasis.
Figure 2.
Figure 2.
(a) Changes in target lesions by nasopharyngeal MRI every 2 cycles during first line chemoimmunotherapy; (b) Leptomeninges strengthening on T1WI enhancement; (c) Blurred sulcus on T2FLAIR. (d) Linear reinforcement in the sulci on CTA. (e) Cerebrospinal fluid cytology: cerebrospinal fluid smear and sediment paraffin-embedded sections showed large nuclei, deep staining, and a high nucleoplasm ratio, considering the possibility of cancer cells. Immunohistochemical results: heteromorphic cells CK (+), P63(+), Ki-67 (about 40%), CD20(-), CD3(-), consistent with squamous cell carcinoma.
Figure 3.
Figure 3.
Timeline of the diagnosis and treatment of this NPC patients with leptomeningeal metastasis.
See this image and copyright information in PMC

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