Pineal yolk sac tumor

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Niloofarsadaat Eshaghhosseiny, MD[2]Sujit Routray, M.D. [3]

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.Pure pineal yolk sac tumors secrete AFP.On microscopic histopathological analysis, pineal yolk sac tumor is characterized by poorly differentiated endothelial-like, cuboidal, or columnar cells with prominent nucleoli and significant mitotic activity.Pineal yolk sac tumor is demonstrated by positivity to tumor markers such as AFP, cytokeratin, and AAT.In upto 50% of cases, these tumors co-exist with other germ cell tumors Pineal yolk sac tumor may be associated with Down syndrome.Common complication of pineal yolk sac tumor includes obstructive hydrocephalus.Prognosis of pineal yolk sac tumor is generally poor.Symptoms of pineal yolk sac tumor include headache, nausea, vomiting, weakness, confusion, and somnolence.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.Biopsy is generally done to confirm the diagnosis of pineal yolk sac tumor.

Historical Perspective

  • Tumors of brainstaim as well as pineal gland,were unoperable until late of 20th century.[1]
  • Dr Cushing reported one of the first case in 1904.[1]
  • Dr Horsley in 1905 was the first who did direct surgical intervention,and in 1913 Dr Oppenhein and Krause resected pineal tumor sucssesfully.[1]
  • There are only two case Reports of pineal yolk sac tumor that are associated with Down's syndrome in English literature.[2]

Classification

Adapted from WHO:

TUMOR FREQUENCY ORIGIN
GERMCELL TOMURS 60% Rest of germ cells
Germinoma MATURE TERATOMAMATURE TERATOMATERATOMA with Malignant Transformstion Yolk sac tomur

(endodermal sinus tumor) Embryonal carcinoma Choriocarcinoma

PINEAL PARANCHIMAL TUMORS 30% pineal glandular tissue
pineocytoma (WHO grade ɪ ) pineal paranchymal tomur of intermediate diffrentiation(WHO grade ɪɪ or ɪɪɪ)

pineoblastoma(WHO grade ɪv) papillary tumor of pineal region

TOMURS OF SUPPORTIVE AND ADJUCENT STRUCTURES 10%
ASTROCYTOMAGlioma (glioblastoma or oligodendroglioma)Medulloepithelioma Glial cells
Ependymomachoroid plexus papilloma Ependymal lining
MENINGIOMA Arachnoid cells
HemangiomaHemangiopericytoma or

blastomaChemodectomaCraniopharyngioma

vascular cells
NON-NEOPLASTIC TUMOR LIKE CONDITIONS < 1%
Arachnoid cysts Arachnoid cells
Degenerative cysts(pineal cysts) Glial cells
Cysticercosis parasites
Arteriovenous malformations vascularization
Cavernomas Aneurysms of the vein Galen
METASTASES <.,1% Absence of blood -

brain barrier

Lung (most common),breast,stomach,kidney,melanoma

Pathophysiology

Causes

Differentiating Intracranial Germ cell Tumors from Other Diseases

Epidemiology and Demographics

  • The incidence of pineal tumor is approximately [1%] of all intracranial tumors.[3]
  • The incidence of pineal tumor is 0,06 -0,07 per 100,000 persons per year.[3]
  • Patients of all age groups may develop pineal yolk sac tumor,But is more common in children(3-8%)and also in Japenes population.[3]
  • There is no racial predilection to pineal tumors.[3]
  • pineal tumors affect men more than women.[8]
  • The majority of pineal tumor cases are reported in Japenes population.[3]

Risk Factors

  • There are no established risk factors for pineal yolk sac tumor.

Screening

  • There is insufficient evidence to recommend routine screening for pineal yolk sac tumors.

=Natural History, Complications, and Prognosis

Diagnosis

Diagnostic Study of Choice

  • In 54% of cases diagnosis is delayed because of non-specific symptoms.[9]

=History and Symptoms


Physical Examination

facial nerve parasia.[6]

Laboratory Findings

Electrocardiogram

X-ray

  • There are no x-ray findings associated with pineal yolk sac tumors.

Echocardiography or Ultrasound

CT scan

.

=MRI

Other Imaging Findings

  • There are no other imaging findings associated with cranial GCTs.

=Other Diagnostic Studies

Treatment

Management Options of Penial Gland tumors
CSF diversion
  • The optimal surgical strategy to treat acute hydrocephalus in patients with pineal tumors is uncertain.
Surgical resection
  • Some series report long-term survival with surgery alone, even in patients with pineoblastomas.
  • Indeed, for pineoblastomas, gross total surgical resection appears to correlate with improved survival.
  • Patients with symptomatic recurrent pineocytomas should also be considered for surgical resection of the lesion
Radiation
Stereotactic radiosurgery
  • Stereotactic radiosurgery (SRS) is emerging as a useful treatment alternative for pineocytomas, although experience is limited.
  • The precise radiation fields that are defined by MRI or CT-computerized treatment planning minimize damage to the surrounding brain, and the risks of general anesthesia and craniotomy are avoided.
  • SRS is increasingly being used to treat pineal region tumors, either as an additional therapy after conventional treatments or as a primary treatment.
  • Due to the low rate of side effects, IRS may develop into an attractive alternative to microsurgery in de novo diagnosed pineocytomas. In malignant PPTs, IRS may be routinely applied in a multimodality treatment schedule supplementary to conventional irradiation.
Chemotherapy as part of multimodality therapy

References

  1. 1.0 1.1 1.2 Shahinian H, Ra Y (2013). "Fully endoscopic resection of pineal region tumors". J Neurol Surg B Skull Base. 74 (3): 114–7. doi:10.1055/s-0033-1338165. PMC 3712663. PMID 24436899.
  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. doi:10.1136/jcp.2004.016659. PMC 1770394. PMID 15280413.
  3. 3.0 3.1 3.2 3.3 3.4 3.5 3.6 3.7 Ji J, Gu C, Zhang M, Zhang H, Wang H, Qu Y; et al. (2019). "Pineal region metastasis with intraventricular seeding: A case report and literature review". Medicine (Baltimore). 98 (34): e16652. doi:10.1097/MD.0000000000016652. PMC 6716749 Check |pmc= value (help). PMID 31441839.
  4. 4.0 4.1 4.2 4.3 Ronchi A, Cozzolino I, Montella M, Panarese I, Zito Marino F, Rossetti S; et al. (2019). "Extragonadal germ cell tumors: Not just a matter of location. A review about clinical, molecular and pathological features". Cancer Med. doi:10.1002/cam4.2195. PMID 31568647.
  5. Takami H, Fukuoka K, Fukushima S, Nakamura T, Mukasa A, Saito N; et al. (2019). "Integrated Clinical, Histopathological, and Molecular Data Analysis of 190 Central Nervous System Germ Cell Tumors from the iGCT Consortium". Neuro Oncol. doi:10.1093/neuonc/noz139. PMID 31420671.
  6. 6.0 6.1 6.2 6.3 Fang AS, Meyers SP (2013). "Magnetic resonance imaging of pineal region tumours". Insights Imaging. 4 (3): 369–82. doi:10.1007/s13244-013-0248-6. PMC 3675249. PMID 23640020.
  7. Mufti ST, Jamal A (2012). "Primary intracranial germ cell tumors". Asian J Neurosurg. 7 (4): 197–202. doi:10.4103/1793-5482.106652. PMC 3613642. PMID 23559987.
  8. Al-Hussaini M, Sultan I, Abuirmileh N, Jaradat I, Qaddoumi I (2009). "Pineal gland tumors: experience from the SEER database". J Neurooncol. 94 (3): 351–8. doi:10.1007/s11060-009-9881-9. PMC 2804886. PMID 19373436.
  9. 9.0 9.1 9.2 9.3 9.4 9.5 Fetcko K, Dey M (2018). "Primary Central Nervous System Germ Cell Tumors: A Review and Update". Med Res Arch. 6 (3). doi:10.18103/mra.v6i3.1719. PMC 6157918. PMID 30271875.
  10. 10.0 10.1 Uda H, Uda T, Nakajo K, Tanoue Y, Okuno T, Koh S; et al. (2019). "Adult-Onset Mixed Germ Cell Tumor Composed Mainly of Yolk Sac Tumor Around the Pineal Gland: A Case Report and Review of the Literature". World Neurosurg. 132: 87–92. doi:10.1016/j.wneu.2019.08.079. PMID 31470154.
  11. Fujimaki T, Matsutani M, Funada N, Kirino T, Takakura K, Nakamura O; et al. (1994). "CT and MRI features of intracranial germ cell tumors". J Neurooncol. 19 (3): 217–26. doi:10.1007/bf01053275. PMID 7807172.
  12. Morana G, Alves CA, Tortora D, Finlay JL, Severino M, Nozza P; et al. (2018). "T2*-based MR imaging (gradient echo or susceptibility-weighted imaging) in midline and off-midline intracranial germ cell tumors: a pilot study". Neuroradiology. 60 (1): 89–99. doi:10.1007/s00234-017-1947-3. PMID 29128947.
  13. 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.