Plexus papilloma

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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)
Plexus papilloma. Extensive tumor in the area of ​​the right lateral ventricle with a midline shift.

The plexus papilloma is a rare benign brain tumor that originates from the covering layer ( epithelium ) of the venous plexus ( plexus choroidus ). Plexus papillomas are rare tumors that only make up about 0.4 to 0.6% of all brain tumors. Mostly children and young people are affected. The plexus papilloma leads to an increase in intracranial pressure with headache, nausea and vomiting. The diagnosis is made by magnetic resonance imaging. After surgical removal, the prospect of healing is very good, which is why the tumor is classified as grade I according to the WHO classification of tumors of the central nervous system .

The atypical plexus papilloma and malignant plexus carcinoma must be differentiated from benign plexus papillomas .

Clinical picture and diagnostics

Due to their location within the internal fluid spaces of the brain, 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. In advanced cases, hydrocephalus (head of water) can develop .

In magnetic resonance imaging , plexus papillomas are very characteristically represented as a mass with a cauliflower-like shape and an intensely homogeneous contrast medium absorbing mass in the cerebral ventricles .

pathology

Histology of the plexus papilloma with typical papillary structures. Hematoxylin-eosin stained section. Original enlargement 1:40
At higher magnification, the relatively uniformly shaped (monomorphic) cell nuclei and the lack of mitotic activity are clearly visible. Hematoxylin-eosin stained section. Original magnification 1: 400

The histological appearance with the formation of papillary structures largely resembles that of the normal choroid plexus. Tumors that have atypical histological features (especially increased mitotic activity ) are called atypical plexus papilloma .

The immunohistochemical detection of the expression of cytokeratin and transthyretin is diagnostically helpful . Differentiation from brain metastases can cause difficulties in adults . The detection of the expression of plexus tumor-specific markers can help to substantiate the suspected diagnosis of a plexus papilloma.

Disease emergence

The vast majority of these are sporadic cases, i.e. cases in which no connection with a hereditary disease can be identified. Plexus papillomas can rarely occur as part of syndromes such as Aicardi syndrome or Li-Fraumeni syndrome . Over on the development of disease (pathogenesis) sporadic choroid plexus papilloma mechanisms involved, little is known. In particular, there is no convincing evidence of a viral pathogenesis. Although genetic material of the Simian virus 40 (SV40), which can generate tumors in animal models, was detected in plexus papilloma tissue , this detection was only possible in population groups that had received vaccine against polio from 1955 to 1963 contaminated with the SV40 virus ( e.g. in the USA). Since the incidence of plexus papillomas was not increased in comparison to other countries during this period, the SV40 detection is probably an epiphenomenon (favoring virus replication in patients with latent infection) and not a causal relationship .

Treatment and prognosis

The tumor can almost always be completely removed surgically . Due to the rarity of tumors of the choroid plexus, further treatment will be carried out in the context of clinical studies. The only clinical study currently in progress is the CPT-SIOP-2000 study, a therapeutic study carried out under the auspices of the International Society for Pediatric Oncology (SIOP).

After complete surgical removal, the prognosis of plexus papillomas is favorable. In contrast to atypical plexus papillomas and plexus carcinomas, postoperative radiation or chemotherapy is therefore usually not necessary if the plexus papillomas have been completely surgically removed (grade I WHO). Metastatic spread along the CSF pathway or malignant degeneration has only been described in isolated cases.

Veterinary medicine

In dogs, plexus papillomas account for up to 14 percent of all tumors in the skull. They are rarely found in other animals such as goats and cattle.

literature

  • W. Paulus, S. Brandner: Choroid plexus tumors. In: DD Louis, H. Ohgaki, O. Wiestler, Cavenee (Eds.): World Health Organization classification of tumors. Pathology and genetics of tumors of the nervous system. IARC Press, Lyon 2007.

Individual evidence

  1. ^ CH Rickert, W. Paulus: Tumors of the choroid plexus. In: Microsc Res Tech ., 52, 2001, pp. 104-111. PMID 11135453 (review article)
  2. ^ AW McEvoy et al: Management of choroid plexus tumors in children: 20 years experience at a single neurosurgical center. In: Pediatr Neurosurg. 32 (4), Apr 2000, pp. 192-199. PMID 10940770
  3. ^ A. Jeibmann et al .: Prognostic implications of atypical histologic features in choroid plexus papilloma. In: J Neuropathol Exp Neurol . 65, 2006, pp. 1069-1073. PMID 17086103
  4. ↑ R. Beschorner et al .: Choroid plexus tumors differ from metastatic carcinomas by expression of the excitatory amino acid transporter-1. In: Hum Pathol . 37 (7), Jul 2006, pp. 854-860. PMID 16784985
  5. ↑ M. Hasselblatt et al .: Identification of novel diagnostic markers for choroid plexus tumors: a microarray-based approach. In: Am J Surg Pathol . 30, 2006, pp. 66-74. PMID 16330944
  6. ↑ J. Aicardi: Aicardi syndrome In: Brain Dev. 27 (3), Apr 2005, pp. 164-171. PMID 15737696
  7. ↑ H. Ohgaki et al.: More about: cell and molecular biology of simian virus 40: implications for human infections and disease. In: J Natl Cancer Inst . 92 (6), Mar 15, 2000, pp. 495-497. PMID 10716971
  8. ^ JE Wolff: Choroid plexus tumors. In: Br J Cancer . 87 (10), Nov 4, 2002, pp. 1086-1091. PMID 12402146 (meta-analysis)
  9. ^ AW McEvoy et al.: Metastatic choroid plexus papilloma: a case report. In: J Neurooncol. 56 (3), Feb 2002, pp. 241-246. PMID 12061730 .
  10. ^ Jeibmann et al.: Malignant progression in choroid plexus papillomas. In: J Neurosurg . 107 (3 Suppl), 2007, pp. 199-202. PMID 17918524
  11. ^ SL Kraft et al.: Retrospective review of 50 canine intracranial tumors evaluated by magnetic resonance imaging. In: J Vet Intern Med. 11 (4), 1997, pp. 218-225. PMID 9298476
  12. ^ R. Klopfleisch, D. Beier, JP Teifke: Choroid plexus carcinoma in a goat. In: J Comp Pathol . tape 135 , no. 1 , 2006, p. 42-46 , PMID 16820166 .
  13. ↑ MJ Hoenerhoff include: choroid plexus papilloma in a Scottish highland cow. In: J Comp Pathol. 135 (2-3), Aug-Oct 2006, pp. 146-149. Epub 2006 Sep 6. PMID 16952368

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This version was added to the list of articles worth reading on November 3, 2008 .