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1
Question:

A 55-year-old man comes to the office due to left-sided facial numbness.  His symptoms began a month ago with tingling over his left mid-face and have progressively worsened to complete numbness on that side.  The patient has also had 2 months of headaches, nasal congestion with intermittent epistaxis, and left ear fullness.  He has had no fever, rhinorrhea, or purulent nasal discharge.  The patient has taken over-the-counter allergy medications, but these have provided no relief.  He has no other medical problems and does not use tobacco, alcohol, or illicit drugs.  He recently immigrated to the United States from southern China to be closer to his son.  Temperature is 37 C (98.6 F), blood pressure is 126/80 mm Hg, and pulse is 84/min.  Examination reveals sensory loss to touch and pain on the left side of the face.  No facial muscle weakness or other neurological findings are present.  Enlarged, nontender, and mobile cervical lymph nodes are palpable bilaterally.  No oropharyngeal or nasal lesions are visualized.  Otoscopy of the left ear demonstrates clear fluid behind the retracted tympanic membrane, but the right ear is normal.  Nasopharyngoscopy reveals a soft-tissue mass in the nasopharynx.  Which of the following is the most likely diagnosis?

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Nasopharyngeal carcinoma

Epidemiology

  • Endemic to Asia
  • Linked with Epstein-Barr virus reactivation
  • Risk factors: Diet (salty fish), smoking, genetics

Manifestations

  • Obstruction: Nasal congestion, epistaxis, headache
  • Mass effect: Cranial nerve palsy, otitis media
  • Spread: Neck mass (cervical lymphadenopathy)

Diagnosis

  • Endoscope-guided biopsy

Treatment

  • Radiation therapy
  • Chemotherapy

Nasopharyngeal carcinoma (NPC) is a tumor associated with Epstein-Barr virus reactivation that is rare in the United States but endemic in southern China.  Risk is likely increased in this region due to a nitrosamine-rich diet (eg, salt-cured food, early exposure to salted fish) and genetic predisposition.

The nasopharynx links the nasal cavity and the oropharynx.  NPC tumors obstruct this passage and invade adjacent tissues, often resulting in nasal congestion with epistaxis, headache, facial numbness (para-cavernous sinus tumor invasion), and/or serous otitis media (eustachian tube obstruction).  Early metastasis to the bilateral cervical lymph nodes is common.

Diagnosis is made with endoscope-guided biopsy of the primary tumor, and treatment usually involves a combination of radiation therapy and chemotherapy.  Survival is excellent if the neoplasm is discovered early, but most patients present with advanced disease.

(Choice A)  Granulomatosis with polyangiitis is a vasculitis that commonly causes ear (otitis) and nasal (rhinorrhea, bloody discharge) symptoms.  However, patients usually have systemic manifestations (eg, fever, malaise, weight loss) and ulcerative lesions, not a soft-tissue mass.

(Choice B)  Mucormycosis is a fungal infection primarily seen in patients with diabetes mellitus or significant immunocompromise.  Manifestations typically include a pan-sinusitis with fever and purulent rhinorrhea (unlike in this patient).  Necrotic destruction of bone may occur, and examination often reveals black eschars.

(Choice C)  Nasal polyps are usually seen in patients with chronic sinusitis, asthma, or aspirin sensitivity and may obstruct the nasal cavity, resulting in congestion and thick rhinorrhea.  However, cranial nerve involvement is unlikely and examination would reveal a grey mass with inflammatory material.

(Choice E)  Tertiary syphilis may cause meningitis or meningovasculitis with headache and cranial nerve deficits; however, nasal congestion and a soft-tissue mass are unlikely.

Educational objective:
Nasopharyngeal carcinoma is a tumor associated with Epstein-Barr virus reactivation that most commonly affects individuals living in southern China.  Manifestations are due to nasopharyngeal obstruction or invasion of adjacent tissues and include nasal congestion with epistaxis, headache, diplopia, cranial nerve deficits (eg, facial numbness), and otitis media.  Early metastasis to the cervical lymph nodes is common.