Pineal yolk sac tumor

Jump to: navigation, search

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]

Synonyms and Keywords: Pineal yolk sac tumors; Pineal yolk sac tumour; Pineal yolk sac tumours; Pineal endodermal sinus tumor; Pineal endodermal sinus tumors; Pineal endodermal sinus tumour; Pineal endodermal sinus tumours; Pineal yolk sac carcinoma; Pineal yolk sac carcinomas; Pineal gland tumor; Germ cell tumor; Brain tumor

Overview

  • Pineal yolk sac tumor is a rare type of extra gonadal yolk sac tumor. They make up a small fraction of all intracranial germ cell tumors and an even small fraction of pineal masses overall.[1]
  • Pure pineal yolk sac tumors secrete AFP.
  • On microscopic histopathological analysis, pineal yolk sac tumor is characterized by poorly differentiated endothelium-like, cuboidal, or columnar cells with prominent nucleoli and significant mitotic activity.[2]
  • Pineal yolk sac tumor is demonstrated by positivity to tumor markers such as AFP, cytokeratin, and AAT.[3] PAS-diastase positive hyaline globules and Schiller-Duval bodies are the characteristic features of pineal yolk sac tumors.[2]
  • In upto 50% of cases, these tumors co-exist with other germ cell tumors.[4]
  • Pineal yolk sac tumor may be associated with Down syndrome.[5]
  • Common complication of pineal yolk sac tumor includes obstructive hydrocephalus.
  • Prognosis of pineal yolk sac tumor is generally poor.[3]
  • Symptoms of pineal yolk sac tumor include headache, nausea, vomiting, weakness, confusion, and somnolence.[2][3]
  • Head CT scan and brain MRI may be helpful in the diagnosis of pineal yolk sac tumor.
  • On head CT scan, pineal yolk sac tumor is characterized by a hypodense, heterogenous mass in the pineal region with signs of obstructive hydrocephalus.
  • On brain MRI, pineal yolk sac tumor is characterized by hypointensity on T1-weighted images and hyperintensity on T2-weighted images. There may be enhancement after contrast administration.[2]
  • Biopsy is generally done to confirm the diagnosis of pineal yolk sac tumor.[2]
  • The mainstay of therapy for pineal yolk sac tumor is radiotherapy and/or chemotherapy. Sometimes, surgical resection may be done.[2]

References

  1. Pineal yolk sac tumour. Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineal-yolk-sac-tumour. Accessed on December 8, 2015
  2. 2.0 2.1 2.2 2.3 2.4 2.5 Davaus T, Gasparetto EL, Carvalho Neto Ad, Jung JE, Bleggi-Torres LF (2007). "Pineal yolk sac tumor: correlation between neuroimaging and pathological findings.". Arq Neuropsiquiatr. 65 (2A): 283–5. PMID 17607429. 
  3. 3.0 3.1 3.2 Tan HW, Ty A, Goh SG, Wong MC, Hong A, Chuah KL (2004). "Pineal yolk sac tumour with a solid pattern: a case report in a Chinese adult man with Down's syndrome.". J Clin Pathol. 57 (8): 882–4. PMC 1770394Freely accessible. PMID 15280413. doi:10.1136/jcp.2004.016659. 
  4. Pathology of pineal yolk sac tumour. Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineal-yolk-sac-tumour. Accessed on December 9, 2015
  5. Associations of pineal yolk sac tumor. Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineal-yolk-sac-tumour. Accessed on December 8, 2015

Linked-in.jpg