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]

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Synonyms and Keywords: Pineoblastomas; Pinealoblastoma; Pinealoblastomas; PB; Pineal parenchymal 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.

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.[1]

Associated Conditions

Gross Pathology

On gross pathology, pineoblastoma is characterized by solid, large poorly defined masses.[3]

An autopsy specimen showing a rather large pineal tumor. It was a pineoblastoma composed of highly cellular sheets of anaplastic cells with irregular hyperchromatic nuclei and brisk mitotic activity – resembling medulloblastoma and retinoblastoma.[4]

Microscopic Pathology

On microscopic histopathological analysis, pineoblastoma is characterized by:[1][5][6]

  • 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

Immunohistochemistry

Pineoblastoma is demonstrated by positivity to tumor markers such as:[6][7]

Differentiating Pineoblastoma from other Diseases

Pineoblastoma must be differentiated from:[8][9]

Epidemiology and Demographics

Prevalence

Age

Pineoblastoma is a disease that tends to affect children and young adults.[12]

Gender

Pineoblastoma affects men and women equally.[13]

Natural History, Complications and Prognosis

Natural History

Complications

Common complications of pineoblastoma include:[14][15]

Prognosis

Prognosis is generally poor, and the 5-year survival rate of patients with pineoblastoma is approximately 58%.[16]

History and Symptoms

History

The clinical presentation of pineoblastoma is mainly from the obstructive hydrocephalus secondary to compression of the tectum of the midbrain and obstruction of the aqueduct.[14]

Symptoms

Physical Examination

  • Compression of the superior colliculi can lead to a characteristic upward gaze palsy, known as Parinaud syndrome.
  • Common physical examination findings of pineoblastoma include:[10][14]

HEENT

Neurological

Laboratory Diagnosis

There are no specific laboratory findings for pineocytoma. However, the following findings are of significant

CT Scan

  • 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.[17]

MRI

  • Brain MRI may be diagnostic of pineoblastoma.
  • Features on MRI suggestive of pineoblastoma include:[18]
MRI component Findings

T1

  • Isointense to hypointense to adjacent brain

T2

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

Other Imaging Findings

Other imaging studies for pineoblastoma include magnetic resonance spectroscopy (MR spectroscopy), which demonstrates:[19]

Other Diagnostic Studies

Stereotactic biopsy

Treatment

Management Options of Penial Gland tumors
CSF diversion
Surgical resection
Radiation
Stereotactic radiosurgery
  • 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, SRS may develop into an attractive alternative to microsurgery in newly 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
  • Gross total resection has been associated with improved survival, similar to treatment with craniospinal irradiation and multi-agent chemotherapy.[11]
  • 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.
  • Subtotal tumor resection in children under 5 years of age is associated with 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 chemotherapy, it significantly reduces tumor progression.
  • Patients with pineoblastoma will develop hydrocephalus in majority of the cases and they will require CSF diversion. Ventriculo-peritoneal (V-P) shunt placement is associated 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.[11]

References

  1. 1.0 1.1 Pathology of pineoblastoma. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineoblastoma. Accessed on December 1, 2015
  2. Rodjan F, de Graaf P, Brisse HJ, Göricke S, Maeder P, Galluzzi P, Aerts I, Alapetite C, Desjardins L, Wieland R, Popovic MB, Diezi M, Munier FL, Hadjistilianou T, Knol DL, Moll AC, Castelijns JA (September 2012). "Trilateral retinoblastoma: neuroimaging characteristics and value of routine brain screening on admission". J. Neurooncol. 109 (3): 535–44. doi:10.1007/s11060-012-0922-4. PMC 3434888. PMID 22802019.
  3. 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
  4. Image courtesy of Dr. Frank Gaillard. Radiopaedia (original file here). Creative Commons BY-SA-NC
  5. Microscopic features of pineoblastoma. Libre Pathology 2015. http://librepathology.org/wiki/index.php/Pineal_gland#Pineoblastoma. Accessed on December 1, 2015
  6. 6.0 6.1 Histology of pineoblastoma. Dr Frank Gaillard. Radiopaedia 2015. http://radiopaedia.org/cases/pineoblastoma-3. Accessed on December 1, 2015
  7. IHC of pineoblastoma. Libre Pathology 2015. http://librepathology.org/wiki/index.php/Pineal_gland#Pineoblastoma. Accessed on December 1, 2015
  8. 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
  9. DDx of pineoblastoma. Libre Pathology 2015. http://librepathology.org/wiki/index.php/Pineal_gland#Pineoblastoma. Accessed on December 1, 2015
  10. 10.0 10.1 10.2 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. doi:10.1155/2014/135435. PMC 4158562. PMID 25210636.
  11. 11.0 11.1 11.2 11.3 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.
  12. General features of pineoblastoma. Libre Pathology 2015. http://librepathology.org/wiki/index.php/Pineal_gland#Pineoblastoma. Accessed on December 1, 2015
  13. Epidemiology of pineoblastoma. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineoblastoma. Accessed on December 1, 2015
  14. 14.0 14.1 14.2 14.3 14.4 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
  15. 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.
  16. 16.0 16.1 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
  17. 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
  18. 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
  19. MR spectroscopy of pineoblastoma. Dr Mohammad A. ElBeialy. Radiopaedia 2015. http://radiopaedia.org/cases/pineoblastoma-6. Accessed on December 1, 2015

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