Matt Cherry (University of Missouri/Columbia/USA)  

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16 Years  

Male  

16 year old male presented to the Emergency Room complaining of fatigue and increasing nosebleeds over the past month. His hemoglobin was found to be 6.8. Past medical history was significant for chronic epistaxis and chronic headaches.  

Undifferentiated nasopharyngeal carcinoma.  

<- view CT 1

CT 1: Noncontrast axial CT demonstrating an oval soft tissue density mass in the left posterior nasopharyngeal region that slightly crosses midline.


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CT 2: Noncontrast axial CT bone windows show erosion of the medial and lateral left pterygoid plates.


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MRI 1: Axial T1 post contrast image demonstrates the fairly well marginated mass with moderately increased signal and contrast enhancement.


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MRI 2: Coronal T1 post contrast fat suppressed image reveals no intracranial extension.


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MRI 3: Axial T2 precontrast image shows mildly increased signal in the posterior nasopharyngeal mass.


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Nuclearmedicine 1: (PET) Multiplanar images demonstrate intense FDG activity in the posterior nasopharyngeal mass. Questionable small foci of increased uptake noted in the bilateral posterior cervical regions were suspicious for nodal metastasis.

Surgery / Histo  

Juvenile Angiofibroma, Rhabdomyosarcoma, Lymphoma, Chronic rhinosinusitis, Allergic fungal rhinosinusitis, Polyposis  

Further metastatic imaging workup included CT scans of the chest, abdomen, and pelvis which were negative. Bone scan was also negative. Cerebral angiogram was performed to evaluate for possible juvenile angiofibroma. The vascularity associated with the mass was noted to be decreased in comparison to what is typically seen for juvenile angiofibromas. Subsequently a nasal endoscopic biopsy was performed whiched revealed the pathology. Patient started chemoradiation treatments and responded favorably both clinically and on followup imaging.

Nasopharyngeal carcinoma accounts for approximately 1% of all childhood malignancies, and nearly one third of childhood nasopharyngeal neoplasms. It may be related to Ebstein Barr virus infection, and the incidence varies according to geographic location. Approximately 1 in every 100,000 children are affected annually in North America and Europe. There is a bimodal age distribution, peaking in late childhood and adults aged 50-60 years. The World Health Organization (WHO) recognizes three subtypes: squamous cell carcinoma (typically found in adults), non-keratinizing carcinoma, and undifferentiated carcinoma (typically in pediatric patients). This tumor arises from the epithelial cells that cover the surfaces of the nasopharynx. It usually originates in the lateral wall of the nasopharynx, and can spread to the other lateral wall, to the skull base or palate, nasal cavity, and oropharynx. It typically metastasizes to cervical lymph nodes. Distant mets may occur in bone, lung, mediastinum, and rarely the liver. In childhood, the presence of metastatic disease in cervical lymph nodes at diagnosis does not adversely affect the prognosis. Factors associated with a poor prognosis include extent of the primary tumor, skull base involvement, and cranial nerve involvement. Symptoms may include pain, trismus, otitis media, neck mass, headache, nasal congestion/obstruction, nasal bleeding, hearing loss, and cranial nerve palsies. Surgical therapy for these patients is typically limited to a biopsy for tissue diagnosis. Nearly all tumors are unresectable due to the complex anatomical location of the disease. Use of combination radiation therapy and chemotherapy allows long-term survival rates of 55-80%.

 

N/A  

First described by Regaud and Schmincke in 1921.
 

Bernadette Brennan
Nasopharyngeal carcinoma
Orphanet Journal of Rare Diseases, 2006

Inci Ayan
Childhood nasopharyngeal carcinoma: from biology to treatment
The Lancet Oncology, 2003

Arnold Paulino
Nasopharyngeal cancer
eMedicine, 2010
 

Head-Nose  

neoplastic