Esthesioneuroblastoma

Classification according to ICD-10
C30.0	Malignant neoplasm of the nasal cavity
ICD-10 online (WHO version 2019)

The Esthesioneuroblastoma or olfactory neuroblastoma (engl. 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 or gender clusters are not known.

The tumor was described by Berger and Luc in 1924. It starts from the neuroepithelium of the olfactory mucous membrane, which is mainly located on the lamina cribrosa , partly also in the upper turbinates and on the nasal septum.

According to recent molecular biological research, the malignancy probably develops from a degenerate olfactory stem cell . The aesthetic neuroblastoma already differs from other malignancies in this region such as carcinomas , sarcomas and lymphomas in the light microscopic examination; the detection succeeds with immunohistochemical display of cell structures (S-100, NSE positive; cytokeratin, vimentin negative).

Fine tissue image of an esthesioneuroblastoma (HE)

Symptoms of illness and diagnosis

Symptoms of the disorder include nasal congestion, discharge, nosebleeds, pain, and loss of smell. A common misdiagnosis is benign polyposis nasi . It is recommended that an ear, nose and throat examination, including a biopsy , be performed if symptoms persist for more than two months. The extent of the tumor can be assessed using computed tomography or magnetic resonance imaging .

Classification

The degree of degeneracy of the tumor cells after Hyams in the degrees G1-4 (G4: completely dedifferentiated) classified.

The standardized classification of tumor spread ( staging ) is based on the TNM system as follows:

• T1 - tumor confined to the nasal cavity and paranasal sinuses
• T2 - erosion of the lamina cribriformis [cribrosa]
• T3 - breakthrough in the anterior fossa or in an eye socket
• T4 - invasion of the brain
• N0 - No lymph node metastases
• N1 - lymph node metastases (only in 5 percent at the initial diagnosis)
• M0 - No distant metastases
• M1 - distant metastases (lungs, liver, brain, etc.)

According to Kadish, the disease is divided into the following stages:

• Stage A: tumor spreads within the nasal cavity.
• Stage B: The tumor spreads within the nasal cavity and into one or more paranasal sinuses .
• Stage C: Spread beyond the paranasal sinuses to include the orbit , the base of the skull or intracerebral growth, involvement of cervical lymph nodes or the presence of distant metastases.

Therapy and prognosis

Treatment is usually based on the surgical removal of the tumor as completely as possible, followed by radiation . The reverse procedure is also possible. There are various chemotherapeutic pre-treatments mostly with platinum-based regimens similar to those for locally advanced carcinomas. Standardized guidelines are not available for this very rare tumor. According to the few available studies, the cure rate is said to be between 45 and 80 percent. Metastases are not common, but local recurrence at the original tumor site is feared.

In the case of first-time operations, around 50% are cured. The medium-term survival rate (2–5 years) is 73% -88% after the most modern combination treatment , surgery / radiation ; significantly better than for older combinations or for one of the procedures alone.

swell

• Anonymous: Esthesioneuroblastoma: a rare tumor that should be addressed multimodally. ImFocus Oncology 7-8 / 2003 (PDF file; 44 kB)
• Guideline for radiotherapy of primary tumors of the central nervous system and CNS metastases in adulthood (1999, not updated) of the AWMF

Individual evidence

1. ^ L. Berger, G. Luc, D. Richard: L'esthésioneuroépithéliome olfactif. In: Bull Assoc Franç Étude Cancer. 1924; 13, pp. 410-421.
2. ↑ ME Platek, M. Merzianu u. a .: Improved survival following surgery and radiation therapy for olfactory neuroblastoma: analysis of the SEER database. In: Radiation oncology. Volume 6, 2011, p. 41, ISSN  1748-717X . doi: 10.1186 / 1748-717X-6-41 . PMID 21518449 . PMC 3098784 (free full text).
3. ↑ CA Spaulding, MS Kranyak, WC Constable, FM Stewart: Esthesioneuroblastoma: a comparison of two treatment eras. In: Int J Radiat Oncol Biol Phys . 1988; 15, pp. 381-390. PMID 3138210

This article is about a health issue. It is not used for self-diagnosis and does not replace a diagnosis by a doctor. Please note the information on health issues !