Pineoblastoma: Difference between revisions
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Image:Microscopic image of pineoblastoma 1.jpg|<sub>Pathology specimen of a pineoblastoma (HE stain, x200 magnification).<ref name=microimage1>Microscopic images of pineoblastoma. Libre Pathology 2015. http://librepathology.org/wiki/index.php/Pineal_gland#Pineoblastoma. Accessed on December 1, 2015</ref></sub> | Image:Microscopic image of pineoblastoma 1.jpg|<sub>Pathology specimen of a pineoblastoma (HE stain, x200 magnification).<ref name=microimage1>Microscopic images of pineoblastoma. Libre Pathology 2015. http://librepathology.org/wiki/index.php/Pineal_gland#Pineoblastoma. Accessed on December 1, 2015</ref></sub> | ||
Image:Microscopic image of pineoblastoma 2.jpg|<sub>Paraffin sections show fragments of a densely hypercellular tumor. Tumor cells have small round, oval, and angulated hyperchromatic nuclei and delicate processes which show strong immunostaining for neurofilament protein (NFP). Tumor cells are arranged in diffuse sheets. Prominent Homer-Wright rosettes are observed in several areas. Scattered mitotic figures are identified. No areas of necrosis are observed.<ref name=micropb2image>Image courtesy of Dr. Frank Gaillard. Radiopaedia (original file [http://radiopaedia.org/cases/pineoblastoma-3 here]). Creative Commons BY-SA-NC</ref></sub> | |||
Image:Microscopic image of pineoblastoma 2.jpg|<sub>Paraffin sections show fragments of a densely hypercellular | |||
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Revision as of 18:51, 1 December 2015
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
-
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.[5]
Microscopic Pathology
On microscopic histopathological analysis, pineoblastoma is characterized by:[3][6][7]
- Hypercellular
- 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
-
Pathology specimen of a pineoblastoma (HE stain, x200 magnification).[8]
-
Paraffin sections show fragments of a densely hypercellular tumor. Tumor cells have small round, oval, and angulated hyperchromatic nuclei and delicate processes which show strong immunostaining for neurofilament protein (NFP). Tumor cells are arranged in diffuse sheets. Prominent Homer-Wright rosettes are observed in several areas. Scattered mitotic figures are identified. No areas of necrosis are observed.[9]
Immunohistochemistry
Pineoblastoma is demonstrated by positivity to tumor markers such as:[7][10]
Gallery
-
Immunohistochemical stain of a pineoblastoma demonstrating positivity to neurofilament.[8]
-
Immunohistochemical stain of a pineoblastoma demonstrating positivity to GFAP.[8]
Differentiating Pineoblastoma from other Diseases
Pineoblastoma must be differentiated from:[11][12]
- Pineocytoma
- Pineal parenchymal tumor with intermediate differentiation
- Papillary tumor of the pineal region
- Pineal germinoma
- Pineal embryonal carcinoma
- Pineal choriocarcinoma
- Pineal yolk sac carcinoma (endodermal sinus tumor)
- Pineal teratoma
- Pineal cyst
- Astrocytoma of the pineal gland
- Meningioma near pineal gland
- Pineal metastasis
- Cavernoma in pineal region
- Aneurysm in pineal region
- Glioblastoma multiforme
- Medulloblastoma
Epidemiology and Demographics
Prevalence
Pineoblastoma constitutes approximately 0.1% of the intracranial neoplasms.[13]
Age
Pineoblastoma is a disease that tends to affect the children and young adult population.[1]
Gender
Pineoblastoma affects men and women equally.[14]
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.[15]
Complications
Common complications of pineoblastoma include:[15][16]
- Obstructive hydrocephalus
- Local recurrence
- CSF metastasis
Prognosis
Prognosis is generally poor, and the 5-year survival rate of patients with pineoblastoma is approximately 58%.[17]
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
- 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.[15]
- Symptoms of pineoblastoma include:[13][15]
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][15]
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.[18]
Gallery
-
Pineal calcification: exploded calcification of tumors of pineal cell origin, whereas engulfed calcification by germinomas.[19]
-
Single head CT image demonstrates a soft tissue mass in the region of the pineal gland with eccentric calcification (anterior) and evidence of hydrocephalus.[20]
MRI
- Brain MRI may be diagnostic of pineoblastoma.
- Features on MRI suggestive of pineoblastoma include:[21]
MRI component | Findings |
---|---|
T1 |
|
T2 |
|
T1 with gadolinium contrast |
|
Diffuse weighted imaging/Apparent diffusion coefficient |
|
Treatment
The predominant therapy for pineoblastoma is surgical resection. Adjunctive chemotherapy and radiation may be required.[17]
References
- ↑ 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
- ↑ 2.0 2.1 Pinealoblastoma. Wikipedia 2015. https://en.wikipedia.org/wiki/Pinealoblastoma. Accessed on December 1, 2015
- ↑ 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
- ↑ 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
- ↑ Image courtesy of Dr. Frank Gaillard. Radiopaedia (original file here). Creative Commons BY-SA-NC
- ↑ Microscopic features of pineoblastoma. Libre Pathology 2015. http://librepathology.org/wiki/index.php/Pineal_gland#Pineoblastoma. Accessed on December 1, 2015
- ↑ 7.0 7.1 Histology of pineoblastoma. Dr Frank Gaillard. Radiopaedia 2015. http://radiopaedia.org/cases/pineoblastoma-3. Accessed on December 1, 2015
- ↑ 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
- ↑ Image courtesy of Dr. Frank Gaillard. Radiopaedia (original file here). Creative Commons BY-SA-NC
- ↑ IHC of pineoblastoma. Libre Pathology 2015. http://librepathology.org/wiki/index.php/Pineal_gland#Pineoblastoma. Accessed on December 1, 2015
- ↑ 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
- ↑ DDx of pineoblastoma. Libre Pathology 2015. http://librepathology.org/wiki/index.php/Pineal_gland#Pineoblastoma. Accessed on December 1, 2015
- ↑ 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. doi:10.1155/2014/135435. PMC 4158562. PMID 25210636.
- ↑ Epidemiology of pineoblastoma. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineoblastoma. Accessed on December 1, 2015
- ↑ 15.0 15.1 15.2 15.3 15.4 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
- ↑ 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.
- ↑ 17.0 17.1 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
- ↑ 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
- ↑ Image courtesy of Dr. Frank Gaillard. Radiopaedia (original file here). Creative Commons BY-SA-NC
- ↑ Image courtesy of Dr. Michael Sargent. Radiopaedia (original file here). Creative Commons BY-SA-NC
- ↑ 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