Pineoblastoma: Difference between revisions
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*The predominant therapy for pineoblastoma is [[surgical resection]]. Adjunctive [[chemotherapy]] and [[radiation]] may be required.<ref name="Alexiou2012">{{cite journal|last1=Alexiou|first1=George A|title=Management of pineal region tumours in children|journal=Journal of Solid Tumors|volume=2|issue=2|year=2012|issn=1925-4075|doi=10.5430/jst.v2n2p15}}</ref><ref name="prognosispb1">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</ref> | *The predominant therapy for pineoblastoma is [[surgical resection]]. Adjunctive [[chemotherapy]] and [[radiation]] may be required.<ref name="Alexiou2012">{{cite journal|last1=Alexiou|first1=George A|title=Management of pineal region tumours in children|journal=Journal of Solid Tumors|volume=2|issue=2|year=2012|issn=1925-4075|doi=10.5430/jst.v2n2p15}}</ref><ref name="prognosispb1">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</ref> | ||
*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. | *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. | ||
{| class="wikitable" | |||
|+ | |||
! colspan="2" |Management Options of Penial Gland tumors | |||
|- | |||
|'''CSF diversion''' | |||
| | |||
* The optimal surgical strategy to treat acute hydrocephalus in patients with pineal tumors is uncertain. | |||
* CSF diversion (ventriculoperitoneal [VP] shunt or third ventriculostomy may be necessary in symptomatic patients, although debulking surgery may obviate the need for this procedure | |||
* When CSF diversion is necessary, endoscopic third ventriculostomy can be carried out at the same time as the biopsy and is preferred over VP shunts, which can be complicated by infection, shunt malfunction, subdural hematoma, and rarely, tumor seeding | |||
|- | |||
|'''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''' | |||
| | |||
* Postoperative adjuvant RT is frequently (but not universally) recommended, and local control is dose-dependent. | |||
* The incidence of leptomeningeal recurrence was significantly lower among patients receiving CSI compared with those who did not. | |||
* The five-year survival rates were 86 and 49 percent for pineocytomas and non-pineocytoma PPTs, respectively. | |||
* Adjuvant RT is not universally recommended after gross total resection of a pineocytoma | |||
|- | |||
|'''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''' | |||
| | |||
* The similarity of pineoblastomas to medulloblastomas in terms of their clinical behavior and tendency for leptomeningeal seeding has led to the use of similar chemotherapy regimens in patients with pineoblastoma as part of a multimodality approach. | |||
* Chemotherapy has been used to delay radiation therapy in very young children, for whom the long-term neurocognitive and developmental side effects of craniospinal irradiation (CSI) are a major concern. | |||
* The importance of radiation therapy as a component of the initial treatment of supratentorial primitive neuroectodermal tumors (PNETs) is also supported by the German HIT-SKK87 and HIT-SKK92 protocols, as well as the Canadian pediatric brain tumor protocol | |||
|} | |||
*Gross total resection has been associated with improved survival, similar to treatment with craniospinal irradiation and multi-agent chemotherapy.<ref name="Alexiou2012">{{cite journal|last1=Alexiou|first1=George A|title=Management of pineal region tumours in children|journal=Journal of Solid Tumors|volume=2|issue=2|year=2012|issn=1925-4075|doi=10.5430/jst.v2n2p15}}</ref> | *Gross total resection has been associated with improved survival, similar to treatment with craniospinal irradiation and multi-agent chemotherapy.<ref name="Alexiou2012">{{cite journal|last1=Alexiou|first1=George A|title=Management of pineal region tumours in children|journal=Journal of Solid Tumors|volume=2|issue=2|year=2012|issn=1925-4075|doi=10.5430/jst.v2n2p15}}</ref> | ||
*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. | *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. |
Revision as of 04:03, 2 May 2019
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; 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]
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.[2]
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".[3]
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:[2][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
-
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
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]
- Obstructive hydrocephalus
- Local recurrence
- CSF metastasis
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
- 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.[16]
- Symptoms of pineoblastoma include:[13][16]
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
-
Pineal calcification: exploded calcification of tumors of pineal cell origin, whereas engulfed calcification by germinomas.[20]
-
Single head CT image demonstrates a soft tissue mass in the region of the pineal gland with eccentric calcification (anterior) and evidence of hydrocephalus.[21]
-
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.[22]
-
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.[23]
MRI
- Brain MRI may be diagnostic of pineoblastoma.
- Features on MRI suggestive of pineoblastoma include:[24]
MRI component | Findings |
---|---|
T1 |
|
T2 |
|
T1 with gadolinium contrast [T1 C+ (Gd)] |
|
Diffuse weighted imaging/Apparent diffusion coefficient [DWI/ADC] |
|
Gallery
-
MRI image of pineoblastoma demonstrating restricted diffusion on DWI.[25]
-
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.[25]
-
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.[26]
-
Axial T2-weighted MRI image demonstrating a lesion that is hyperintense.[27]
Other Imaging Findings
Other imaging studies for pineoblastoma include magnetic resonance spectroscopy (MR spectroscopy), which demonstrates:[28]
- Elevation of the choline and lipid lactate peaks
- Depression of the neural markers (N-acetyl aspartate (NAA) and creatine (Cr)
- Prominent glutamate and taurine peaks at 3.4 ppm with shot TE signal voxel MR spectroscopy
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).[29]
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.
Management Options of Penial Gland tumors | |
---|---|
CSF diversion |
|
Surgical resection |
|
Radiation |
|
Stereotactic radiosurgery |
|
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.[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
- ↑ 1.0 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 Pathology of pineoblastoma. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineoblastoma. Accessed on December 1, 2015
- ↑ Pinealoblastoma. Wikipedia 2015. https://en.wikipedia.org/wiki/Pinealoblastoma. 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.
- ↑ 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). doi:10.5430/jst.v2n2p15. ISSN 1925-4075.
- ↑ Epidemiology of pineoblastoma. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineoblastoma. Accessed on December 1, 2015
- ↑ 16.0 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
- ↑ 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.
- ↑ 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
- ↑ 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
- ↑ Image courtesy of Dr. Frank Gaillard. Radiopaedia (original file here). Creative Commons BY-SA-NC
- ↑ Image courtesy of Dr. Bita Abbasi. 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
- ↑ 25.0 25.1 Image courtesy of Dr. Michael Sargent. Radiopaedia (original file here). Creative Commons BY-SA-NC
- ↑ Image courtesy of Dr. Frank Gaillard. Radiopaedia (original file here). Creative Commons BY-SA-NC
- ↑ Image courtesy of Dr. Mohammad A. ElBeialy. Radiopaedia (original file here). Creative Commons BY-SA-NC
- ↑ MR spectroscopy of pineoblastoma. Dr Mohammad A. ElBeialy. Radiopaedia 2015. http://radiopaedia.org/cases/pineoblastoma-6. Accessed on December 1, 2015
- ↑ Image courtesy of Dr. Mohammad A. ElBeialy. Radiopaedia (original file here). Creative Commons BY-SA-NC