Papillary tumor of the pineal region
| Classification according to ICD-10 | |
|---|---|
| D43.0 | Reformation of uncertain or unknown brain behavior, supratentorial |
| ICD-10 online (WHO version 2019) | |
The papillary tumor of the pineal region , or PTPR for short , is a rare brain tumor that presumably originates from specialized ependymal cells of the subcommissural organ . Papillary tumors of the pineal region are located on the back wall of the third ventricle and are in close spatial relation to the pineal gland (glandula pinealis). After the first description in 2003, over 119 cases have been published. In the vast majority of cases, children and young adults are affected. There is no known connection with a hereditary disease.
Clinical manifestations
Due to their typical location, papillary tumors in the pineal region often obstruct the flow of cerebrospinal fluid , which can lead to an increase in intracranial pressure . Headache, nausea, and vomiting are non-specific symptoms. Oculomotor disorders with double vision often occur when the tumor presses the four-hill plate ( Parinaud's syndrome ). In magnetic resonance imaging, papillary tumors of the pineal region appear as masses that enrich the contrast medium.
pathology
Histologically, it is a neuroectodermal tumor with a papillary structure and an epithelial-like appearance. The tumor cells often sit on the blood vessels with wide appendages, giving the impression of clumsy pseudorosettes. Ependymal rosettes as in ependymomas can also occur. The mitotic activity of the tumor cells is usually moderate. Necrosis is common and can lead to a misdiagnosis of plexus carcinoma . In the past, papillary tumors of the pineal region were often misinterpreted as plexus papillomas or papillary ependymomas . The immunohistochemical expression profile (strong, often punctiform expression of cytokeratin ) with mostly missing expression of the epithelial membrane antigen (EMA, positive in ependymomas) and the potassium channel Kir7.1 (positive in plexus papillomas and plexus carcinomas) allows a diagnostic differentiation in most cases .
Pathogenesis
There is currently no knowledge of the mechanisms involved in the pathogenesis of papillary tumors of the pineal region.
therapy
As a rule, a complete surgical removal is sought. If this is not possible, radiation therapy can be considered. As with the treatment of ependymomas, adjuvant chemotherapy plays a minor role. Treatment should, if possible, take place in the context of clinical studies, which are currently (2012) still only in the planning stage.
forecast
Even after complete surgical removal, the prognosis of papillary tumors in the pineal region is characterized by frequent recurrences. The biological value of papillary tumors of the pineal region must therefore be assessed analogously to grade II-III of the WHO classification of tumors of the central nervous system .
literature
Individual evidence
- ↑ Jouvet A: Papillary tumor of the pineal region. In: Am J Surg Pathol . 2003; 27: 505-12. PMID 12657936 (first description)
- ↑ a b c Fevre-Montange M et al .: Prognosis and histopathologic features in papillary tumors of the pineal region: a retrospective multicenter study of 31 cases. In: J Neuropathol Exp Neurol , 2006; 65: 1004-11. PMID 17021405
- ↑ Hasselblatt M et al. Immunohistochemical profile and chromosomal imbalances in papillary tumors of the pineal region. In: Neuropathol Appl Neurobiol . 2006; 32: 278-83. PMID 16640646
