Pineal germinoma

Jump to navigation Jump to 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 gland germinoma; Pineal germinomas; Germinoma of the pineal gland; Pineal dysgerminoma; Pineal dysgerminomas; Pineal gland tumor; Brain tumor

Overview

Pineal germinoma is the most common tumor of the pineal gland accounting for 50% of all tumors and the majority (80%) of intracranial germ cell tumors.[1] Pineal germinoma is a type of germ cell tumor. It refers to a tumor in the pineal gland that has a histology identical to two other tumors: dysgerminoma in the ovary and seminoma in the testis.[2]

Pathophysiology

Pathogenesis

  • Pineal germinoma is a malignant neoplasm of the germinal tissue of the pineal region.

Differentiating Pineal Germinoma from other Diseases

Pineal germinoma must be differentiated from:[3][4]

Epidemiology and Demographics

Prevalence

Pineal germinoma is the most common tumor of the pineal gland but accounts for less than 1% of all intracranial tumors. Pineal germinoma accounts for 50% of all the pineal gland tumors and the majority (80%) of intracranial germ cell tumors.[1][5]

Age

Pineal germinoma is a disease that tends to affect the children and young adult population.[6] Most patients are 20 years or younger at the time of diagnosis.

Gender

Males are more commonly affected with pineal germinoma than females. The male to female ratio is approximately 13 to 1.[6]

Natural History, Complications and Prognosis

Natural History

Pineoblastoma is the most agressive pineal parenchymal tumor. If left untreated, patients with pineoblastoma may progress to develop seizures, obstructive hydrocephalus, local recurrence, and CSF metastasis.[7]

Complications

Common complications of pineoblastoma include:[7][8]

Prognosis

Prognosis is generally poor, and the 5-year survival rate of patients with pineoblastoma is approximately 58%.[9]

History and Symptoms

History

When evaluating a patient for pineoblastoma, you should take a detailed history of the presenting symptom (onset, duration, and progression), other associated symptoms, and a thorough family and past medical history review.

Symptoms

Physical Examination

Compression of the superior colliculi can lead to a characteristic gaze palsy, known as Parinaud syndrome. Common physical examination findings of pineoblastoma include:[10][7]

HEENT

  • Bulging soft spots (fontanelles)
  • Eyes that are constantly looking down (sunsetting sign)
  • Deficiency in upward-gaze
  • Pupillary light-near dissociation (pupils respond to near stimuli but not light)
  • Convergence-retraction nystagmus
  • Papilledema

Neurological

CT

  • Head CT scan may be diagnostic of pineoblastoma.
  • Findings on CT scan suggestive of pineoblastoma include a mass with a solid component that tends to be slightly hyperdense compared to adjacent brain due to high cellularity. Calcification is present that is peripherally disperse or "exploded", similar to pineocytoma.[11]

Gallery

  • Pineal calcification: exploded calcification of tumors of pineal cell origin, whereas engulfed calcification by germinomas.[12]

    Pineal calcification: exploded calcification of tumors of pineal cell origin, whereas engulfed calcification by germinomas.[12]

  • Single head CT image demonstrates a soft tissue mass in the region of the pineal gland with eccentric calcification (anterior) and evidence of hydrocephalus.[13]

    Single head CT image demonstrates a soft tissue mass in the region of the pineal gland with eccentric calcification (anterior) and evidence of hydrocephalus.[13]

  • Pre-contrast CT scan demonstrating moderate internal obstructive hydrocephalus, due to a large, partially calcified, dense mass which appears to be centered upon the pineal gland. The quadrigeminal plate appears anteriorly displaced and the aqueduct obliterated. It does not appear to have an intimate relationship with the tentorium.[14]

    Pre-contrast CT scan demonstrating moderate internal obstructive hydrocephalus, due to a large, partially calcified, dense mass which appears to be centered upon the pineal gland. The quadrigeminal plate appears anteriorly displaced and the aqueduct obliterated. It does not appear to have an intimate relationship with the tentorium.[14]

  • Axial brain CT image demonstrating a large hyperdense lobulated mass in the pineal region with peripheral foci of calcification and associated hydrocephalus. A VP shunt has recently been inserted (note the small amount of pneumocephalus). Hyperdense material is observed coating the frontal horns of the lateral ventricle and filling the floor of the third ventricle.[15]

    Axial brain CT image demonstrating a large hyperdense lobulated mass in the pineal region with peripheral foci of calcification and associated hydrocephalus. A VP shunt has recently been inserted (note the small amount of pneumocephalus). Hyperdense material is observed coating the frontal horns of the lateral ventricle and filling the floor of the third ventricle.[15]

MRI

  • Brain MRI may be diagnostic of pineoblastoma.
  • Features on MRI suggestive of pineoblastoma include:[16]
MRI component Findings

T1

  • Isointense to hypointense to adjacent brain

T2

  • Isointense to adjacent brain
  • Areas of cyst formation or necrosis may be present

T1 with gadolinium contrast [T1 C+ (Gd)]

  • Vivid heterogenous enhancement

Diffuse weighted imaging/Apparent diffusion coefficient [DWI/ADC]

  • Restricted diffusion due to dense cellular packing
  • ADC values are typically 400-800 mm2/s

Gallery

  • MRI image of pineoblastoma demonstrating restricted diffusion on DWI.[17]

    MRI image of pineoblastoma demonstrating restricted diffusion on DWI.[17]

  • Sagittal T1 with contrast CT image of pineoblastoma demonstrating an enhancing mass in the region of the pineal gland is present. A tongue of tissue is observed extending inferiorly through the aqueduct, obstructing it, and resulting in hydrocephalus with transependymal edema.[17]

    Sagittal T1 with contrast CT image of pineoblastoma demonstrating an enhancing mass in the region of the pineal gland is present. A tongue of tissue is observed extending inferiorly through the aqueduct, obstructing it, and resulting in hydrocephalus with transependymal edema.[17]

  • Axial T1 with contrast demonstrating an irregular heterogenous enhancing pineal mass with several tiny cystic foci and eccentric coarse calcifications. There is moderate mass effect on the adjacent tectum and vermis, with loss of definition and possible parenchymal invasion on the left. There is associated aqueduct compression with moderate hydrocephalus.[18]

    Axial T1 with contrast demonstrating an irregular heterogenous enhancing pineal mass with several tiny cystic foci and eccentric coarse calcifications. There is moderate mass effect on the adjacent tectum and vermis, with loss of definition and possible parenchymal invasion on the left. There is associated aqueduct compression with moderate hydrocephalus.[18]

  • Axial T2-weighted MRI image demonstrating a lesion that is hyperintense.[19]

    Axial T2-weighted MRI image demonstrating a lesion that is hyperintense.[19]

Other Imaging Findings

Other imaging studies for pineoblastoma include magnetic resonance spectroscopy (MR spectroscopy), which demonstrates:[20]

Gallery

  • MR spectroscopy of the lesion demonstrating elevation of the choline and lipid lactate peaks and depression of the neural markers; N-acetyl aspartate (NAA) and creatine (Cr).[21]

    MR spectroscopy of the lesion demonstrating elevation of the choline and lipid lactate peaks and depression of the neural markers; N-acetyl aspartate (NAA) and creatine (Cr).[21]

Treatment

  • The predominant therapy for pineoblastoma is surgical resection. Adjunctive chemotherapy and radiation may be required.[22][9]
  • The main goal of open surgery on pineoblastoma is the complete tumor removal with minimal morbidity, whenever possible. However, even if gross total excision cannot be achieved, establishment of an accurate diagnosis, maximal cytoreduction, and restoration of the CSF pathway may be achieved.
  • Gross total resection has been associated with improved survival, similar to treatment with craniospinal irradiation and multi-agent chemotherapy.[22]
  • Children under the age of 36 months with pineoblastoma should be treated with multi-agent chemotherapy for 12 to 24 months with the goal of delaying radiation past the age of 36 months. Craniospinal irradiation before this age of 3 has been associated with significant cognitive and neuroendocrine sequelae.
  • Tate et al. summarized the existing literature on patients with pineoblastoma and found that children under 5 years of age and subtotal tumor resection markedly worsened patient survival. According to the Children's Oncology Group trials, these tumors require craniospinal irradiation (with local tumor doses of at least 50 Gy) and adjuvant chemotherapy. When carboplatin and vincristine were administered during craniospinal irradiation followed by 6 months of non-intensive non-cisplatin containing adjuvant chemotherapy, an 84% 2-years progression free survival was reported in pineoblastomas without evidence of dissemination at presentation.
  • Patients with pineoblastoma will develop hydrocephalus in majority of the cases and they will require CSF diversion. Ventriculo-peritoneal (V-P) shunt placement is a viable option with low morbidity and mortality rate. However, shunt malfunction in this population is as high as 20%. In addition, tumor metastasis through a CSF shunt has been reported. Endoscopic third ventriculostomy (ETVC) is an alternative option, which also permits a biopsy of the tumor in the same procedure. Ahn et al. reported that the biopsy samples, obtained in the lateral ventricle or pineal region, were more favorable towards a successful diagnosis than those in the thalamus or tectal region. Neuroendoscopic biopsy procedures have been proven safe with low complication rates.[22]

References

  1. ↑ 1.0 1.1 Pineal germinoma. Dr Henry Knipe et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineal-germinoma. Accessed on December 2, 2015
  2. ↑ Classification of germinoma. Wikipedia 2015. https://en.wikipedia.org/wiki/Germinoma. Accessed on December 2, 2015
  3. ↑ Differential diagnoses of pineoblastoma. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineoblastoma. Accessed on December 1, 2015
  4. ↑ DDx of pineoblastoma. Libre Pathology 2015. http://librepathology.org/wiki/index.php/Pineal_gland#Pineoblastoma. Accessed on December 1, 2015
  5. ↑ Epidemiology of pineal germinoma. Operative Neurosurgery 2015. http://operativeneurosurgery.com/doku.php?id=pineal_germinoma. Accessed on Dcember 2, 2015
  6. ↑ 6.0 6.1 Epidemiology of pineal germinoma. Dr Henry Knipe et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineal-germinoma. Accessed on December 2, 2015
  7. ↑ 7.0 7.1 7.2 7.3 7.4 Clinical presentation of pineoblastoma. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineoblastoma. Accessed on December 1, 2015
  8. ↑ Stoiber EM, Schaible B, Herfarth K, Schulz-Ertner D, Huber PE, Debus J; et al. (2010). "Long term outcome of adolescent and adult patients with pineal parenchymal tumors treated with fractionated radiotherapy between 1982 and 2003--a single institution's experience". Radiat Oncol. 5: 122. doi:10.1186/1748-717X-5-122. PMC 3019157. PMID 21184689.
  9. ↑ 9.0 9.1 Treatment and prognosis of pineoblastoma. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineoblastoma. Accessed on December 1, 2015
  10. ↑ 10.0 10.1 Palled S, Kalavagunta S, Beerappa Gowda J, Umesh K, Aal M, Abdul Razack TP; et al. (2014). "Tackling a recurrent pinealoblastoma". Case Rep Oncol Med. 2014: 135435. doi:10.1155/2014/135435. PMC 4158562. PMID 25210636.
  11. ↑ CT findings of pineoblastoma. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineoblastoma. Accessed on December 1, 2015
  12. ↑ Image courtesy of Dr. Frank Gaillard. Radiopaedia (original file here). Creative Commons BY-SA-NC
  13. ↑ Image courtesy of Dr. Michael Sargent. Radiopaedia (original file here). Creative Commons BY-SA-NC
  14. ↑ Image courtesy of Dr. Frank Gaillard. Radiopaedia (original file here). Creative Commons BY-SA-NC
  15. ↑ Image courtesy of Dr. Bita Abbasi. Radiopaedia (original file here). Creative Commons BY-SA-NC
  16. ↑ Radiographic features MRI of pineoblastoma. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineoblastoma. Accessed on December 1, 2015
  17. ↑ 17.0 17.1 Image courtesy of Dr. Michael Sargent. Radiopaedia (original file here). Creative Commons BY-SA-NC
  18. ↑ Image courtesy of Dr. Frank Gaillard. Radiopaedia (original file here). Creative Commons BY-SA-NC
  19. ↑ Image courtesy of Dr. Mohammad A. ElBeialy. Radiopaedia (original file here). Creative Commons BY-SA-NC
  20. ↑ MR spectroscopy of pineoblastoma. Dr Mohammad A. ElBeialy. Radiopaedia 2015. http://radiopaedia.org/cases/pineoblastoma-6. Accessed on December 1, 2015
  21. ↑ Image courtesy of Dr. Mohammad A. ElBeialy. Radiopaedia (original file here). Creative Commons BY-SA-NC
  22. ↑ 22.0 22.1 22.2 Alexiou, George A (2012). "Management of pineal region tumours in children". Journal of Solid Tumors. 2 (2). doi:10.5430/jst.v2n2p15. ISSN 1925-4075.


Template:WikiDoc Sources

Retrieved from "https://www.wikidoc.org/index.php?title=Pineal_germinoma&oldid=1191772"