Plexus carcinoma

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Classification according to ICD-10
C71.5 Malignant neoplasm of the brain ventricle
ICD-10 online (WHO version 2019)

The Plexuskarzinom is a malignant brain tumor , probably from the epithelium of the choroid plexus runs out. It is classified as grade III according to the WHO classification of tumors of the central nervous system and makes up less than 0.4% of all brain tumors. Mostly children and young people are affected; In the first year of life, plexus carcinomas are among the most common brain tumors. Mostly these are sporadic cases in which no connection with a hereditary disease can be identified. In Li-Fraumeni syndrome , plexus carcinomas occur more frequently. A benign tumor of the choroid plexus is known as a plexus papilloma .

Symptoms

Because of their location within the ventricular system , plexus carcinomas often obstruct the flow of cerebral fluid , which can lead to an increase in intracranial pressure . Headache , nausea and vomiting are typical but unspecific symptoms . In advanced cases, hydrocephalus (water head) can develop .

pathology

Plexus carcinoma with largely abolished papillary growth pattern, increased cell density and nuclear polymorphism.
Hematoxylin-eosin stained section. Original magnification 1: 400

The histological picture is only slightly similar to that of the normal choroid plexus: The papillary growth pattern, which is still clearly recognizable in benign plexus papillomas , is largely eliminated, mitotic activity , cell density and nucleus polymorphism (diversity) are increased. Tumor necrosis often occurs or diffuse infiltration of the brain occurs. Difficulties can be presented in the diagnostic differentiation from carcinoma metastases in adults because the immunohistochemical expression profile of conventional diagnostic markers overlaps. The immunohistochemical detection of expression of the amino acid transporter Excitatory Amino Acid Transporter-1 and the inwardly rectifying potassium channel Kir7.1 can corroborate the suspected diagnosis of a Plexuskarzinoms.

Pathogenesis

Little is known about the factors involved in the pathogenesis of sporadic plexus carcinoma. Although mutations in the SMARCB1 gene (INI1), which typically occur in atypical teratoid / rhabdoid tumors (WHO grade IV), have also been described in plexus carcinomas, the majority of cases, in retrospect, evidently were misdiagnosed atypical teratoid / rhabdoid tumors Tumors acted.

therapy

Complete surgical removal can be difficult due to the infiltrative growth and abundance of blood vessels of plexus carcinomas. As part of the further treatment, which is mandatory in the context of clinical studies, an accompanying chemotherapy usually follows . 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. Also a radiotherapy of the tumor may play a role in an individual therapy concept.

forecast

Even after complete surgical removal, the further course of plexus carcinoma is characterized by frequent recurrences .

Veterinary medicine

Plexus carcinomas are also rarely found in people with horns .

literature

  • A. Aguzzi, S. Brandner, W. Paulus: Choroid plexus tumours. In: P. Kleihues, WK Cavenee (Ed.): World Health Organization classification of tumors. Pathology and genetics of tumors of the nervous system. IARC Press, Lyon 2000.

Individual evidence

  1. ^ CH Rickert, W. Paulus: Tumors of the choroid plexus. In: Microsc Res Tech . 2001; 52, 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. 2000 Apr; 32 (4), pp. 192-199. PMID 10940770
  3. ^ R. Beschorner et al .: Choroid plexus tumors differ from metastatic carcinomas by expression of the excitatory amino acid transporter-1. In: Hum Pathol . 2006 Jul; 37 (7), pp. 854-860. PMID 16784985
  4. ↑ M. Hasselblatt et al .: Identification of novel diagnostic markers for choroid plexus tumors: a microarray-based approach In: Am J Surg Pathol . 2006; 30, pp. 66-74. PMID 16330944
  5. ^ AR Judkins et al .: INI1 protein expression distinguishes atypical teratoid / rhabdoid tumor from choroid plexus carcinoma. In: J Neuropathol Exp Neurol . 2005 May; 64 (5), pp. 391-397. PMID 15892296 .
  6. ↑ B. Wrede et al .: Chemotherapy improves the survival of patients with choroid plexus carcinoma: a meta-analysis of individual cases with choroid plexus tumors. In: J Neurooncol. 2007; 85 (3), pp. 345-351. PMID 17576522
  7. ↑ JA Wolff et al .: Radiation therapy and survival in choroid plexus carcinoma. In: The Lancet . 1999 Jun 19; 353 (9170), p. 2126. PMID 10382700 .
  8. ^ JE Wolff: Choroid plexus tumors. In: Br J Cancer . 2002 Nov 4; 87 (10), pp. 1086-1091. PMID 12402146 (meta-analysis)
  9. ^ R. Klopfleisch et al .: Choroid plexus carcinoma in a goat. In: J Comp Pathol. 2006 Jul; 135 (1), pp. 42-46. PMID 16820166

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