Esthesioneuroblastoma

The Ästhesioneuroblastom or olfactory neuroblastoma (English Esthesioneuroblastoma, olfactory neuroblastoma ) is a rare cancer of the nasal cavity. The incidence is estimated at one case per 1 million population per year. Age -, race - or gender clusters are not known.

The tumor was described in 1924 by Berger and Luc. He starts from the neuroepithelium of the olfactory mucosa, which is mainly located on the cribriform plate, partially also in the upper nasal turbinates and on the nasal septum.

According to recent molecular biological research, the malignancy is probably from a degenerate olfactory stem cell. From other malignancies of this region such as carcinomas, sarcomas and lymphomas, the Ästhesioneuroblastom already different in the light- microscopic observation; the proof succeeds with immunohistochemistry displayable cell structures ( S-100, NSE positive, cytokeratin, vimentin negative).

Pathology and diagnosis

Symptoms of the disease are constipation of the nasal passages, discharge, nosebleeds, pain, and loss of smell. Frequent misdiagnosis is benign nasal polyposis. It is recommended to be carried ear, nose and ears medical examination including biopsy, if the symptoms persist for longer than two months. The extent of the tumor can be assessed by means of computed tomography or magnetic resonance imaging.

Classification

The degree of degeneracy of the tumor cells by Hyams in the grade G1 -4 ( G4: completely undifferentiated ) classified.

The standardized classification of tumor spread (staging) is divided as follows in the TNM system of UICC:

• T1 - tumor confined to the nasal cavity and paranasal sinuses
• T2 - erosion of the cribriform plate [ cribrosa ]
• T3 - Breakthrough in the anterior cranial fossa or in an orbital
• T4 - Burglar in the brain
• N0 - No lymph node metastases
• N1 - lymph node metastasis ( only 5 percent at initial diagnosis )
• M0 - No distant metastasis
• M1 - distant metastases (lung, liver, brain, etc.)

After Kadish, the disease is divided into the following stages:

• Stage A: Tumor extends from within the nasal cavity.
• Stage B: The tumor spreads within the nasal cavity and into one or more sinuses.
• Stage C: spread beyond the sinuses, including the orbit, skull base or intracerebral growth, involvement of cervical lymph nodes or presence of distant metastases.

Treatment and prognosis

The treatment is based generally on the fullest possible surgical removal of the tumor, followed by irradiation. The inverse procedure is possible. There are various chemotherapeutic pretreatments usually with platinum-based regimens similar to those for locally advanced carcinomas. Standardized guidelines are not available for this rare tumor. The cure rate is the few studies available indicates that range from 45 to 80 percent. Metastases are not common, but feared the local recurrence at the original tumor site.

In Erstoperierten about 50 % healed. The medium-term survival ( 2-5 years) is 73% -88% according to the most advanced combination treatment surgery / radiation; significantly better than for older combinations or for one of the methods alone.

Swell

• Anonymous: Ästhesioneuroblastom: a rare tumor that should tackle multimodal. Imfocus Oncology 7-8/2003 (PDF file, 44 kB)
• Guideline radiotherapy of primary tumors of the central nervous system and CNS metastases in adulthood (1999, not updated ) AWMF