Leptomeningeal Metastases in Nasopharyngeal Carcinoma: A Rare Case Report and a Review of Literature

El Amin Marnouche^*, Khalid Hadadi, Abdelhak Maghous, Maroua Benlemlih, Mouhcine Hommadi, Mohamed Oukabli, Khalid Saghir Andaloussi, Mohamed Elmarjany and Hassan Sifat
^*Correspondence: El Amin Marnouche, Department of Radiotherapy, Mohamed V Military Hospital, Rabat, Morocco, Tel: +212649945497, Email:

Keywords

Leptomeningeal metastases • Undifferentiated nasopharyngeal carcinoma • Case report

Abbreviations

NPC: Nasopharyngeal carcinoma; LM: Leptomeningeal Metastasis; IC: Intrathecal Chemotherapy; RT: Radiation Therapy; CSF: Cerebrospinal Fluid; UCNT: Undifferentiated Nasopharyngeal Carcinoma; MRI: Magnetic Resonance Imaging, FDG PET-CT: Fluorodeoxyglucose Positron Emission Computed Tomography.

Introduction

Nasopharyngeal carcinoma NPC is an uncommon cancer in most parts of the world. There is a distinct geographical distribution with low incidence in Europe and the United States [1]. Intermediate incidence rates are found in the Mediterranean Basin and the Arctic and in certain places such as southern China and Southeast Asia, the incidence rises significantly and these areas are considered endemic for NPC with 80% of the global burden in Asian countries [2].

The standard of care for these patients consists of concurrent chemoradiotherapy with cisplatin-based regimens, generally followed by adjuvant chemotherapy. This treatment approach results in cure for the vast majority of patients, with 3-year disease-free and overall survival rates of approximately 70% and 80%, respectively [3]. Distant metastases are present in 3% to 6% of the cases and may occur in 18% to 50% of cases during the disease course [4]. The most common sites for distant NPC metastases are the bone, lung, and liver [5]. Brain and skin metastases rarely occur [6,7].

A PubMed database search of existing English literature regarding leptomeningeal metastasis LM metastases of NPC revealed few well-described cases. A case of LM of NPC and related literature is discussed.

Case Description

Case and outcomes

A 36 years old female patient with no history of chronic disease, presented in November 2015 for 18 months history of progressively growing left mass of the neck. Ear, nose and throat evaluation included fiberoptic endoscopy, which indicated a bulb in the right side of nasopharynx. Biopsy was positive for undifferentiated nasopharyngeal carcinoma UCNT. Magnetic resonance imaging MRI revealed an infiltrative mass in the nasopharynx extending into left parapharyngeal space and bilateral lymphadenopathy including level IV. Additional workup revealed no distant metastases. The disease was staged T2N3M0 (AJCC 7^th edition). Thereafter, the patient received three cycles of Induction chemotherapy followed by radiotherapy using intensity-modulated radiation therapy (total dose of 69.96 Gy (2.12 Gy/fraction in 33 fractions) with concomitant weekly cisplatin. Six months after completion of treatment, MRI showed disease resolution.

In March 2018, the patient complained of headache and left temporal swelling. This subcutaneous mass was painless and firm. Physical exam was otherwise unremarkable. Head and Neck MRI revealed a left frontal enhancing lesion measuring 23 mm in greatest dimension with limited leptomeningeal enhancement (Figures 1 and 2). Three other enhancing lesions were found in left subcutaneous temporal area (01 mass) and in left parotid gland (02 masses) (Figures 1-3). Brain and spine MRI revealed no additional lesions. Fluorodeoxyglucose positron emission computed tomography FDG PET-CT scan showed pathological uptakes corresponding to subcutaneous and parotid masses. No distant metastases were found.

Considering the high probability of metastatic disease and with the patient’s consent, the neurosurgeon performed a macroscopic total excision of the frontal mass and a total excision of the subcutaneous lesion with frozen examination. The tumor turned out to be an undifferentiated carcinoma consistent with nasopharyngeal carcinoma metastasis (Figures 4). Epstein- Barr virus staining using in situ hybridization was unavailable in pathology department at this time. The disease was classified stage IV (AJCC 7^th edition).

A small residual enhancement persisted on post-operative brain MRI. Hypo fractionated brain radiotherapy was initiated. A total dose of 50 Gy (2 Gy/fraction) was delivered in preoperative leptomeningeal site with integrated boost of 67.5 Gy (2.7 Gy/fraction) to residual mass. The patient refused parotidectomy due to significant risk of facial paralysis. In order to obtain parotid masses shrinkage, a systemic chemotherapy was initiated. After 03 cures of systemic chemotherapy, head and neck MRI revealed a slight shrinkage of parotid lesions. Whereas Brain MRI showed no active disease. Once again, the patient refused surgery. We decided to continue chemotherapy. After the 6^th cures, she complained lumbar and left thigh pain. FDG PET-CT scan showed a disease progression with pathological uptakes in left parotid gland, left cervical lymphadenopathies and bones (7^th dorsal vertebrae, 4^th lumbar vertebrae, and left femoral shaft). The patient is now being treated with palliative chemotherapy.

Conclusion

Leptomeningeal metastases from NPC are rare. Often, multiple areas of craniospinal axis are involved simultaneously. The diagnosis is based on neuroimaging modality and CSF analysis. The chemotherapy is the mainstay of treatment and radiotherapy is often palliative. The prognosis of patients with LM is poor. However a small subset of patients with low tumor burden and good performance status could be treated more aggressively.

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