Atypical plexus papilloma

from Wikipedia, the free encyclopedia
Classification according to ICD-10
D33.0 Benign neoplasm: brain, supratentorial
D33.1 Benign neoplasm: brain, infratentorial (IV ventricle)
ICD-10 online (WHO version 2019)

An atypical plexus papilloma is a rare brain tumor that originates from the epithelium of the choroid plexus and is prone to recurrence . The tumor, which occurs mainly in infants and young children, is classified as grade II according to the WHO classification of tumors of the central nervous system . Atypical plexus papillomas are to be distinguished from benign plexus papillomas and plexus carcinoma , a malignant tumor.

Symptoms

Due to their location within the ventricular system , atypical plexus papillomas often obstruct the flow of nerve water , which can lead to an increase in intracranial pressure . Headache , nausea and vomiting are typical but unspecific symptoms.

pathology

Atypical plexus papilloma with increased proliferative activity on immunohistochemical staining for Ki-67 .
Proliferating tumor cell nuclei are colored brown. Counterstain with hematoxylin. Magnification 100 ×.

The histological picture is similar to that of plexus papillomas, but atypical plexus papillomas show increased mitotic activity, while malignancy criteria for plexus carcinoma are not met.

therapy

The tumor cannot always be completely removed surgically . Due to the rarity of atypical plexus papillomas, further treatment will be carried out in the context of clinical studies. An international therapy study that is being conducted under the auspices of the International Society for Pediatric Oncology (SIOP) is the CPT-SIOP-2000 study.

forecast

After complete surgical removal, the prognosis of atypical plexus papillomas (especially in children under three years of age) is relatively favorable. Postoperative chemotherapy is usually only performed if the atypical plexus papillomas have not been completely surgically removed. Malignant degeneration of atypical plexus papillomas has only been described in isolated cases.

literature

  • W. Paulus, S. Brandner: Choroid plexus tumors. In: DN Louis, H. Ohgaki, OD Wiestler, WK Cavenee (Eds.): World Health Organization classification of tumors. Pathology and genetics of tumors of the nervous system. IARC Press, Lyon 2007, pp. 81-85.
Individual evidence
  1. ^ A. Jeibmann et al .: Prognostic implications of atypical histologic features in choroid plexus papilloma. In: J Neuropathol Exp Neurol . Volume 65, 2006, pp. 1069-1073. PMID 17086103
  2. ↑ C. Thomas et al .: Pediatric atypical choroid plexus papilloma reconsidered: increased mitotic activity is prognostic only in older children. In: Acta Neuropathol . Volume 129, 2015, pp. 925-927. PMID 25935663
  3. ^ A. Jeibmann et al.: Malignant progression in choroid plexus papillomas. In: J Neurosurg . Volume 107, No. 3, 2007, pp. 199-202. PMID 17918524

Web links