Pineoblastoma

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

Synonyms and Keywords: Pineoblastomas; Pinealoblastoma; Pinealoblastomas; PB; Pineal parenchymal tumor; Pineal gland tumor; Brain tumor

Overview

Pineoblastoma is a rare, malignant pineal parenchymal tumor. It is a supratentorial midline primitive neuroectodermal tumor. It is considered as a WHO grade IV tumor according to the WHO classification of tumors of the central nervous system.[1][2]

Pathophysiology

Pathogenesis

Pineoblastoma originates from the neuroectodermal cells. It is the least differentiated pineal gland tumors, with pineocytoma and pineal parenchymal tumour with intermediate differentiation representing better differentiated tumors along the same spectrum.[3]

Associated Conditions

Pineoblastoma may occur in patients with hereditary uni- or bilateral retinoblastoma. When retinoblastoma patients present with pineoblastoma, this is characterized as "trilateral retinoblastoma".[2]

Gross Pathology

On gross pathology, pineoblastoma is characterized by solid, large poorly defined masses.[4]

Gallery

Microscopic Pathology

On microscopic histopathological analysis, pineoblastoma is characterized by:[3][6][7]

  • Hypercellular appearance
  • Tightly packed small round blue cells (high nuclear to cytoplasmic ratio)
  • Oval and angulated hyperchromatic nuclei with atypia
  • Mitoses
  • Homer-wright & Flexner-Winterstein rosettes
  • Fleurettes

Gallery

Immunohistochemistry

Pineoblastoma is demonstrated by positivity to tumor markers such as:[7][10]

Gallery

Differentiating Pineoblastoma from other Diseases

Pineoblastoma must be differentiated from:[11][12]

Epidemiology and Demographics

Prevalence

Pineoblastoma constitutes approximately 0.1% of the intracranial neoplasms.[13] Pineoblastoma together with germ cell tumors are the most common pineal tumors in children.[14]

Age

Pineoblastoma is a disease that tends to affect children and young adults.[1]

Gender

Pineoblastoma affects men and women equally.[15]

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.[16]

Complications

Common complications of pineoblastoma include:[16][17]

Prognosis

Prognosis is generally poor, and the 5-year survival rate of patients with pineoblastoma is approximately 58%.[18]

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:[13][16]

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.[19]

Gallery

MRI

  • Brain MRI may be diagnostic of pineoblastoma.
  • Features on MRI suggestive of pineoblastoma include:[24]
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

Other Imaging Findings

Other imaging studies for pineoblastoma include magnetic resonance spectroscopy (MR spectroscopy), which demonstrates:[28]

Gallery

Treatment

  • The predominant therapy for pineoblastoma is surgical resection. Adjunctive chemotherapy and radiation may be required.[14][18]
  • 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.[14]
  • 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.[14]

References

  1. 1.0 1.1 General features of pineoblastoma. Libre Pathology 2015. http://librepathology.org/wiki/index.php/Pineal_gland#Pineoblastoma. Accessed on December 1, 2015
  2. 2.0 2.1 Pinealoblastoma. Wikipedia 2015. https://en.wikipedia.org/wiki/Pinealoblastoma. Accessed on December 1, 2015
  3. 3.0 3.1 Pathology of pineoblastoma. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineoblastoma. Accessed on December 1, 2015
  4. Radiographic features of pineoblastoma. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineoblastoma. Accessed on December 1, 2015
  5. Image courtesy of Dr. Frank Gaillard. Radiopaedia (original file here). Creative Commons BY-SA-NC
  6. Microscopic features of pineoblastoma. Libre Pathology 2015. http://librepathology.org/wiki/index.php/Pineal_gland#Pineoblastoma. Accessed on December 1, 2015
  7. 7.0 7.1 Histology of pineoblastoma. Dr Frank Gaillard. Radiopaedia 2015. http://radiopaedia.org/cases/pineoblastoma-3. Accessed on December 1, 2015
  8. 8.0 8.1 8.2 Microscopic images of pineoblastoma. Libre Pathology 2015. http://librepathology.org/wiki/index.php/Pineal_gland#Pineoblastoma. Accessed on December 1, 2015
  9. Image courtesy of Dr. Frank Gaillard. Radiopaedia (original file here). Creative Commons BY-SA-NC
  10. IHC of pineoblastoma. Libre Pathology 2015. http://librepathology.org/wiki/index.php/Pineal_gland#Pineoblastoma. Accessed on December 1, 2015
  11. 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
  12. DDx of pineoblastoma. Libre Pathology 2015. http://librepathology.org/wiki/index.php/Pineal_gland#Pineoblastoma. Accessed on December 1, 2015
  13. 13.0 13.1 13.2 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. PMC 4158562Freely accessible. PMID 25210636. doi:10.1155/2014/135435. 
  14. 14.0 14.1 14.2 14.3 Alexiou, George A (2012). "Management of pineal region tumours in children". Journal of Solid Tumors. 2 (2). ISSN 1925-4075. doi:10.5430/jst.v2n2p15. 
  15. Epidemiology of pineoblastoma. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineoblastoma. Accessed on December 1, 2015
  16. 16.0 16.1 16.2 16.3 16.4 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
  17. 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. PMC 3019157Freely accessible. PMID 21184689. doi:10.1186/1748-717X-5-122. 
  18. 18.0 18.1 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
  19. 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
  20. Image courtesy of Dr. Frank Gaillard. Radiopaedia (original file here). Creative Commons BY-SA-NC
  21. Image courtesy of Dr. Michael Sargent. Radiopaedia (original file here). Creative Commons BY-SA-NC
  22. Image courtesy of Dr. Frank Gaillard. Radiopaedia (original file here). Creative Commons BY-SA-NC
  23. Image courtesy of Dr. Bita Abbasi. Radiopaedia (original file here). Creative Commons BY-SA-NC
  24. 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
  25. 25.0 25.1 Image courtesy of Dr. Michael Sargent. Radiopaedia (original file here). Creative Commons BY-SA-NC
  26. Image courtesy of Dr. Frank Gaillard. Radiopaedia (original file here). Creative Commons BY-SA-NC
  27. Image courtesy of Dr. Mohammad A. ElBeialy. Radiopaedia (original file here). Creative Commons BY-SA-NC
  28. MR spectroscopy of pineoblastoma. Dr Mohammad A. ElBeialy. Radiopaedia 2015. http://radiopaedia.org/cases/pineoblastoma-6. Accessed on December 1, 2015
  29. Image courtesy of Dr. Mohammad A. ElBeialy. Radiopaedia (original file here). Creative Commons BY-SA-NC

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