| ||Welche DD wären mit dem bildgebenden Befund auch vereinbar:|
Juveniles Angiofibrom, Rhabdomyosarkoma Lymphom, Chronische Rhinosinusitis, Allergische fungale Rhinosinusitis, Polyposis
| ||Verlauf / Prognose / Häufigkeit / Sonstiges :|
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%.
Orphanet Journal of Rare Diseases, 2006
Childhood nasopharyngeal carcinoma: from biology to treatment
The Lancet Oncology, 2003