Syncope due to nasopharyngeal carcinoma - The Lancet

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May 6, 2005 - Consultations were requested with the cardiology and neurology services, and consultation with the ear-nose-and-throat services and a CT ...
Case Report

Syncope due to nasopharyngeal carcinoma Carol Chen-Scarabelli, Asha R Kaza, Tiziano Scarabelli

A 62-year-old white male, with a history of paroxysmal supraventricular tachycardia and atrial fibrillation and who was taking warfarin presented to his general practitioner with complaints of new-onset left-sided headaches and near-syncopal episodes. Initial assessment included a CT scan of the brain (to rule out an intracranial lesion, bleed, or both), which was negative. However, over the next few months, the patient started to complain of decreased hearing in the left ear, otorrhoea (which resolved with antibiotic treatment), hoarseness, almost nightly presyncopal episodes associated with tongue paresthaesias, and diaphoresis. The patient was subsequently admitted to hospital for further assessment, during which time he had recurrent syncopal episodes associated with hypotension, bradycardia, and long sinus pauses up to 2 s in duration. Consultations were requested with the cardiology and neurology services, and consultation with the ear-nose-and-throat services and a CT scan of the sinuses, were requested for outpatient assessment. He was diagnosed with vasodepressor syncope and discharged, but was readmitted 1 week later with recurrent syncope. During the second admission, he was assessed by the ear-nose-and-throat service, and a maxillofacial and sinus CT scan was requested. Past medical history was remarkable for chronic obstructive pulmonary disease, hypertension, chronic sinusitis, headaches, and the patient had a substantial history of smoking (ie, 100 packs a year), heavy alcohol use, and poor dental health. Extensive work-up consisted of an electroencephalogram and a brain magnetic resonance angiography, both of which were negative. Carotid-sinus hypersensitivity (assessed by carotid massage, which did not produce any substantial

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slowing of heart rate or pauses), orthostatic hypotension, and structural heart disease (assessed by echocardiogram) were also ruled out as possible causes of syncope. During a routine dental examination, a panorex film incidentally showed general cloudiness of the right sinus and a domed radio-opacity in the floor of the left sinus. Maxillofacial CT suggested a softtissue lesion, and subsequent brain MRI showed a large parapharyngeal-space tumour with encasement of both the left maxillary nerve and left carotid sheath (which encloses the carotid sinus and arteries, jugular veins, and hypoglossal and vagus nerves; figure 1). Because of recurrent syncope, the patient underwent a pacemaker implantation, with initial abatement of the syncope. A biopsy sample of the nasopharyngeal lesion identified a keratinising squamous-cell carcinoma; contrast-enhanced CT of the soft tissue of the neck also identified invasion of the skull base and intracranial extension. The tumour, staged as T4N2M0, IVa by the American Joint Committee on Cancer, was not amenable to surgery. Consequently, the patient had chemotherapy (with cisplatin and fluorouracil) combined with radiotherapy. Treatment was complicated by dehydration, mucositis, and deep-vein thrombosis of the right upper arm and right lower leg. Furthermore, after a few months without any syncope recurrence the patient developed intermittent vasodepressor syncopal episodes. However, 6 months after diagnosis and chemoradiotherapy, the patient had no recurrence of syncope, and repeat CT scans showed improvement in the lesion and no progression of the skull-base lesion. A repeat nasal endoscopy and biopsy sample showed no evidence of malignant disease.

Division of Cardiology (C Chen-Scarabelli MSN) and Department of Radiology (A R Kaza MD), VA Ann Arbor Healthcare System, Ann Arbor, MI, USA and Division of Cardiology, St John Hospital and Medical Center/Wayne State University, Detroit, MI, USA (T Scarabelli MD) Correspondence to: Ms Carol Chen-Scarabelli, VA Ann Arbor Healthcare System, 2215 Fuller Road, Division of Cardiology (111A), Ann Arbor, MI 48105, USA [email protected]. gov

Figure 1: Work-up of the patient (A) MRI of the brain: red arrows=bilateral internal carotid arteries. Green arrows=margins of the mass. (B) Neck CT focusing on base of skull: red dashed line=tumour mass. Red arrow=left internal carotid artery. Green arrow=right internal carotid artery. (C) Head CT: dashed line=tumour margins. Red arrows=left internal carotid artery.

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Case Report

Carotid-sinus syncope

Glossopharyngeal neuralgia

Tumour compression on carotid sinus

Tumour-induced irritation of glossopharyngeal nerve

Stimulation of carotid sinus

Stimulation of glossopharyngeal nerve

Stimulation of medullary vasodepressor region Increase in vagal tone (bradycardia) and decrease in sympathetic tone (vasodilation) Reduced venous return and decreased cardiac output Cerebral hypoperfusion SYNCOPE

Figure 2: Syncope in head and neck cancer

Nasopharyngeal carcinoma is rare in most areas of the world, accounting for 0·25% of all cancers in the USA (2% of all head and neck cancers) compared with 18% of all cancers in southeast China. People aged 30–50 years are most commonly affected, and incidence in men is twice that of women.1 Parapharyngeal infiltration, invasion of the skull base, and involvement of the cranial nerve are the main risk factors for developing syncope in nasopharyngeal carcinoma.2 In the setting of head and neck cancer, syncope usually arises because of carotid-sinus hypersensitivity (secondary to mechanical compression of the carotid sinus) and glossopharyngeal neuralgia (from tumour-induced irritation of the glossopharyngeal nerve.3,4 Acute unilateral head or neck pain

Carotid-sinus hypersensitivity

Cardioinhibitory component

Vasodepressor component

Increased parasympathetic tone

Decreased sympathetic activity

Slowing of sinus rate or increase in PR interval and advanced block

Loss of vascular tone and hypotension, independent of heart-rate change

Reduced venous return and reduced cardiac output, with resultant cerebral hypoperfusion SYNCOPE

Figure 3: Carotid-sinus hypersensitivity

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usually precedes the syncopal episode (figures 2 and 3).5,6 Pacemaker therapy might alleviate syncope resulting from a cardioinhibitory reflex with bradycardia. However, pacing might become ineffective if pure vasodepressor syncope (with hypotension as the main feature) develops.3 WHO classifies nasopharyngeal carcinoma into three histopathological categories: keratinising squamous-cell carcinoma (type I); non-keratinising epidermoid carcinoma (type II); and undifferentiated carcinomas (type III).7 Genetic susceptibility, carcinogens, diets high in salt-cured fish and meat, and infection with Epstein-Barr virus (EBV) have all been implicated in the aetiology of nasopharyngeal carcinoma.1,8 Furthermore, cigarette smoking and alcohol consumption have been associated with type I disease,9 and EBV has been associated with type II disease.7 75% of patients with nasopharyngeal carcinoma in the USA have type I disease, which is most common in US-born, nonHispanic whites; 25% of patients have types II and III, which commonly arises in Asian people.10 More than two thirds of type I disease are associated with cigarette smoking, alcohol consumption, or both.9 Diagnosis of nasopharyngeal carcinoma is usually made after a patient presents with a seemingly unrelated complaint. Common symptoms include hearing loss (especially unilateral), neck mass, and nasal stuffiness.1 Symptom recognition, awareness of risk factors, and timely referral to the ear-nose-andthroat service are essential for early diagnosis and intervention. Imaging usually include CT or MRI, with studies favouring MRI for both staging and follow-up.11 Outlook for patients with nasopharyngeal carcinoma varies with stage (5-year survival ranges from 80% for stage I to less than 50% for stage IV) and with subgroup (keratinising has a worse prognosis than does non-keratinising).1 Treatment usually involves both chemoradiotherapy followed by chemotherapy (commonly cisplatin and fluorouracil).1 This patient received the Al-Sarraf/Intergroup regimen12,13 of: 100mg/m2 cisplatin on days 1, 22, and 43 during 35 fractions of radiotherapy, given as 2 Gy per day on Monday–Friday to a total dose of 70 Gy, and postradiotherapy chemotherapy with 80mg/m2 cisplatin on day 1 and 1000 mg/m2 fluorouracil on days 1–4 given every 4 weeks for three courses. In our patient, cisplatin was replaced with 300 mg/m2 carboplatin13 because of rising creatinine. Furthermore, our patient completed only two courses of chemotherapy after irradiation because of nausea, vomiting, and rising creatinine. A repeat CT of the head showed improvement in the softtissue bulk in the left nasopharyngeal cavity in the parapharyngeal space and in the base of the skull, with no progression of the skull base lesion. Nasal endoscopy and biopsy sample showed no evidence of malignant disease. http://oncology.thelancet.com Vol 6 May 2005

Case Report

Conflict of interest We declare no conflicts of interest. References 1 American Cancer Society. Nasopharyngeal Cancer. http://www.cancer.org/docroot/CRI/content/CRI_2_4_1X_What_ is_nasopharyngeal_cancer_17.asp?rnav=cri (accessed March 24, 2005). 2 Tang Y, Wang JM, Huang CH. Syncope in nasopharyngeal carcinoma: report of three cases and review of the literature. Changgeng Yi Xue Za Zhi 1993; 16: 59–65. 3 Macdonald DR, Strong E, Nielsen S, Posner JB. Syncope from head and neck cancer. J Neurooncol 1983; 1: 257–67. 4 Lin RH, Teng MM, Wang SJ, et al. Syncope as the presenting symptom of nasopharyngeal carcinoma. Clin Neurol Neurosurg 1994; 96: 152–55. 5 Papay FA, Roberts JK, Wegryn TL, et al. Evaluation of syncope from head and neck cancer. Laryngoscope 1989; 99: 382–88. 6 Chen-Scarabelli C, Scarabelli TM. Neurocardiogenic syncope. BMJ 2004; 329: 336–41. 7 Gius D, Coleman CN. Treatment of nasopharyngeal cancer: raising the “Barr”. J Natl Cancer Inst 2002; 94: 1594–95.

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Lo KW, To KF, Huang DP. Focus on nasopharyngeal carcinoma. Cancer Cell 2004; 5: 423–28. Vaughan TL, Shapiro JA, Burt RD, et al. Nasopharyngeal cancer in a low-risk population: defining risk factors by histological type. Cancer Epidemiol Biomarkers Prev 1996; 5: 587–93. Marks JE, Phillips JL, Menck HR. The National Cancer Data Base report on the relationship of race and national origin to the histology of nasopharyngeal carcinoma. Cancer 1998; 83: 582–88. Olmi P, Fallai C, Colagrande S, Giannardi G. Staging and followup of nasopharyngeal carcinoma: magnetic resonance imaging versus computerized tomography. Int J Radiat Oncol Biol Phys 1995; 32: 795–800. Al-Sarraf M, LeBlanc M, Giri PG, et al. Chemoradiotherapy versus radiotherapy in patients with advanced nasopharyngeal cancer: phase III randomized Intergroup study 0099. J Clin Oncol 1998; 16: 1310–17. Forastiere AA, Metch B, Schuller DE, et al. Randomized comparison of cisplatin plus fluorouracil and carboplatin plus fluorouracil versus methotrexate in advanced squamous-cell carcinoma of the head and neck: a Southwest Oncology Group study. J Clin Oncol 1992; 10: 1245–51.

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