CLINICAL CASE REVIEWSPONSORED BY GUERBET

Pediatric Pineoblastoma
Jad El Bulbul, MD and Ari Goldberg, MD, PhD
Dept of Radiology, Loyola University Medical Center

Case Summary
An 8-year-old boy with no significant past medical
history presented for neurosurgery consultation aftera brain lesion was discovered on computed tomography (CT). He presented with 3 days of headache, emesis, lethargy, and right eye pain. The headaches began
6 weeks earlier and initially occurred once a week, andsince increased in frequency. He showed no changes ingait or speech, no numbness or tingling, and no abnormal gaze or nystagmus. He denied photophobia anddizziness. After initial work-up, including CT, the patientunderwent magnetic resonance imaging (MRI) of thebrain with and without gadolinium-based contrast.

Imaging Findings
MRI with and without contrast demonstrated a
2.4 x 2.0 x 1.9 cm heterogeneously enhancing mass
within the pineal recess. There was mass effect fromthis lesion at the level of the cerebral aqueduct, resulting in non-communicating hydrocephalus (Figure 1).
A punctate focus of hyperintense T1 signal within themass, which demonstrated signal loss on postcontrast
fat-suppressed images, indicated partial fatty composition (Figure 2).

Diagnosis
Pineoblastoma

Discussion
Microscopic examination demonstrated a densely
cellular proliferation of undifferentiated neoplastic cells,n©

APPLIED RADIOLOGYwith sheet-like growth and small, round blue cells withhyperchromatic nuclei, high nuclear-cytoplasmic ratio,
and indistinct cell borders. Tumor cells were positive for synaptophysin and showed high Ki-67 labeling index (>50%). The findings were consistent withpineoblastoma, WHO grade IV.
Pineal gland tumors are uncommon, accountingfor ~1% of all central nervous system (CNS) tumorsin adults and 3% in children.1-2 The tumors can be classified into three major categories: germ cell tumors(GCTs), the most common pineal tumors; pineal parenchymal tumors (PPTs), and gliomas. The WHO classification of CNS tumors divides pineal tumors into fourgroups: pineocytomas (Grade I), pineal parenchymal
tumors of intermediate differentiation (Grade II or III),
papillary tumor of the pineal region (Grade II or III), andpineoblastoma (Grade IV).3
Highly malignant and aggressive, pineoblastomas are considered primary neuroectodermal tumors(PNETs) of the pineal region.4 They hold a poor prognosis and can metastasize locally and throughout the neuroaxis.1
Contrast-enhanced magnetic resonance imaging (CEMRI) is the modality of choice for diagnosing
pineoblastomas; computed tomography (CT) cannot
differentiate between (PPT) and (GCT).1 On MRI, pineoblastomas are isointense on T1- weighted images andhave vivid heterogeneous enhancement comparedto GCTs and PPTs, which enhance homogenously.
Given the disease’s predilection to spread along theneuroaxis, MRI of the brain and spine with and without

www.appliedradiology.com

SPONSORED BY GUERBETA

CLINICAL CASE REVIEWBC

FIGURE 1. (A) Sagittal T1 pre-contrast sequence; (B) Sagittal T1 post-contrast and (C) Coronal T1 post-contrast sequences revealing a 2.4x2x1.9 cm
lesion in the pineal region (red arrow) that is heterogeneously enhancing, consistent with pineoblastoma.

A

B

FIGURE 2. (A) Axial T1 pre-contrast and (B) axial T1 post contrast sequences demonstrating a pineal mass that heterogeneously enhances and also
has partial fatty composition.

contrast is important for disease recognition and quantification, and is therefore recommended1,5-6.
Clinical manifestations of pineoblastomas are similar to those of other pineal tumors. They are commonly
associated with obstructive hydrocephalus, resulting
in elevation of intracranial pressure. This elevation typically causes headaches, nausea/vomiting, vision abnormalities, and reduced levels of consciousness. 1 In
addition, compression of the tectal plate may manifest
as Parinaud syndrome, which includes upward gazepalsy, convergence nystagmus, and pupillary light-neardissociation. Focal neurological deficits (FNDs) may
also be prominent.7

www.appliedradiology.com

Treatment generally begins with the managementof hydrocephalus, including cerebrospinal fluid (CSF)
diversion, commonly achieved by either ventriculoperitoneal (VP) shunt or endoscopic third ventriculostomy (EVP).7,8 Gross total resection of pineoblastoma
has been associated with prolonged survival and better outcomes. The addition of postoperative adjuvant
radiotherapy is generally recommended while theinclusion of chemotherapy as part of multimodality
therapy is controversial8.
This patient underwent gross total resection andadjuvant radiotherapy; MRI surveillance demonstrated
no appreciable disease for two years. The patient later©

APPLIED RADIOLOGYnCLINICAL CASE REVIEW

showed signs of recurrence and further CEMRI studies detected metastatic lesions in the frontal lobes tohelp guide therapy.

Conclusion
We report a case of pineoblastoma, a highly
malignant form of pineal gland tumors. Because
pineoblastoma is extremely aggressive it warrants acomprehensive work-up and therapy. Contrast-enhanced MRI is essential to diagnosing this neoplasmand helps to guide disease management.

References
1. Dhall G, Khatua S, Finlay JL. Pineal region tumors in children. Curr Opin Neurol.
2010;23(6):576-582. doi:10.1097/WCO.0b013e3283404ef1
2. Ramasubramanian A, Kytasty C, Meadows AT, Shields JA, Leahey A, Shields
CL. Incidence of pineal gland cyst and pineoblastoma in children with retinoblastoma during the chemoreduction era. Am J Ophthalmol. 2013;156(4):825-829.
doi:10.1016/j.ajo.2013.05.023n©

APPLIED RADIOLOGYSPONSORED BY GUERBET

3. Brat DJ, Parisi JE, Kleinschmidt-DeMasters BK, et al. Surgical neuropathology
update: a review of changes introduced by the WHO classification of tumours of
the central nervous system, 4th edition. Arch Pathol Lab Med. 2008;132(6):9931007. doi:10.1043/1543-2165(2008)132[993:SNUARO]2.0.CO;2
4. Borit A, Blackwood W, Mair WG. The separation of pineocytoma from pineoblastoma. Cancer. 1980;45(6):1408-1418. doi:10.1002/1097-0142(19800315)45:
6<1408::aid-cncr2820450619>3.0.co;2-0
5. Pham TT, Siebert E, Asbach P, Willerding G, Erb-Eigner K. Magnetic resonanceimaging based morphologic evaluation of the pineal gland for suspected pineoblastoma in retinoblastoma patients and age-matched controls. J Neurol Sci.
2015 Dec 15;359(1-2):185-92. doi: 10.1016/j.jns.2015.10.046. Epub 2015 Oct 28.
PMID: 26671110.
6. Galluzzi P, de Jong MC, Sirin S, et al. MRI-based assessment of the pineal
gland in a large population of children aged 0-5 years and comparison withpineoblastoma: part I, the solid gland. Neuroradiology. 2016;58(7):705-712.
doi:10.1007/s00234-016-1684-z
7. Tate MC, Rutkowski MJ, Parsa AT. Contemporary management of pineoblastoma. Neurosurg Clin N Am. 2011;22(3):409-ix. doi: 10.1016/j.nec.2011.05.001
8. Tate M, Sughrue ME, Rutkowski MJ, Kane AJ, Aranda D, McClinton L, McClinton L, Barani IJ, Parsa AT. The long-term postsurgical prognosis of patients withpineoblastoma. Cancer. 2012 Jan 1;118(1):173-9. doi: 10.1002/cncr.26300. Epub
2011 Jun 29. PMID: 21717450.

www.appliedradiology.com