eMedicine Specialties > Radiology > Brain/Spine

Pineal Germinoma

Author: Chi-Shing Zee, MD, Chief of Neuroradiology, Professor, Departments of Radiology and Neurosurgery, University of Southern California School of Medicine
Coauthor(s): Zhenwei Yao, MD, Attending Radiologist, Department of Radiology, Huashan Hospital, Fudan University. Shanghai, China; John L Go, MD, Assistant Professor of Clinical Radiology, Director, Spinal Imaging and Intervention, Division of Neuroradiology, University of Southern California Keck School of Medicine; Paul E Kim, MD, Assistant Professor of Clinical Radiology, Director, Spinal Imaging and Intervention, Division of Neuroradiology, University of Southern California Keck School of Medicine
Contributor Information and Disclosures

Updated: Mar 8, 2010

Introduction

Background

Although pineal region neoplasms constitute only 0.3-2.7% of intracranial tumors, they are considered an important clinical entity because of their strategic location. Pineal region neoplasms can be classified into 3 major groups according to their cellular origin: (1) tumors of germ cell origin, (2) tumors of pineal cell origin, and (3) tumors of other cell origin. Tumors of germ cell origin include germinoma, mature teratoma, malignant teratoma, embryonal cell carcinoma, endodermal sinus tumor, choriocarcinoma, and mixed germ cell tumors. In addition, synchronous pineal and suprasellar germinomas are found in 5-10% of cases; see the images below. Pineal germinomas are seen predominantly in males from infancy to 20 years of age.

Coronal, post-contrast T1-weighted image shows a ...

Coronal, post-contrast T1-weighted image shows a small enhancing mass in the region of pituitary infundibulum in the suprasellar cistern.

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Coronal, post-contrast T1-weighted image shows a ...

Coronal, post-contrast T1-weighted image shows a small enhancing mass in the region of pituitary infundibulum in the suprasellar cistern.


Axial, post-contrast T1 weighted images demonstra...

Axial, post-contrast T1 weighted images demonstrates an enhancing mass in the pineal region.

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Axial, post-contrast T1 weighted images demonstra...

Axial, post-contrast T1 weighted images demonstrates an enhancing mass in the pineal region.


Recent studies

Radovanovic et al reported that of 51 pineal region tumors that were treated surgically, only 17 of them were neoplasms originating from the pineal body (pineal tumors). The authors noted that beta-HCG (human chorionic gonadotropin) is mainly positive in choriocarcinomas, embryonal carcinomas, and mixed germ cell tumors; alpha-fetoprotein (AFP) is expressed by yolk sac tumors, embryonic carcinomas, immature teratomas, and mixed germ cell tumors; and beta-HCG is usually low in germinomas, which are often positive for placental alkaline phosphatase (PLAP) on immunohistochemistry.1

Mori et al reported their experience with gamma knife radiosurgery (GKRS) for pineal and related tumors in 49 patients. The diagnosis was germ cell tumors (GCT) in 38 patients (53 tumors), pineal parenchymal tumors (PPT) in 9 (19 tumors), and unknown in 2 (2 tumors). Mean treatment volume was 3.3 ml in GCT cases and 3.7 ml in PPT. Survival rates at 5 years and 10 years after GKRS in GCT cases were both 68%. In PPT cases, the 5-year rate was 100% and 10-year, 67%. The authors concluded that GKRS is an effective and safe adjuvant treatment for pineal and related tumors.2

Santagata et al identified that CRX, a homeobox transcription factor, is a sensitive and specific clinical marker and a potential lineage-dependent therapeutic target in pineoblastoma, as well as for retinoblastoma. In their study, the investigators found that in over 95% of pineal parenchymal tumors and retinoblastomas, immunohistochemical analysis demonstrated strong expression of CRX. CRX was not detected, however, in the majority of tumors considered in the differential diagnosis of pineal region tumors, except for medulloblastoma, 40% of which exhibited CRX expression in a heterogeneous pattern that was readily distinguished from that seen in retinopineal tumors.3

Frequency

United States

Pineal region neoplasms constitute 0.3-2.7% of all intracranial tumors. Germinoma is the most common pineal region neoplasm, constituting approximately 40% of cases. Pineal germinomas occur more frequently in males and are more common than suprasellar germinomas.

International

A high incidence of pineal germinoma is found in Japan. Increasing evidence suggests a high incidence of pineal and suprasellar germinoma in the Chinese population, as well.

Mortality/Morbidity

  • Overall 5-year survival rate for patients with a pineal region or suprasellar germinoma treated with radiation is 59%.
  • The 5-year survival rate for patients aged 25 years or younger who do not undergo biopsy for a tumor in the pineal region is 81% after radiation treatment. Most of these tumors are probably germinomas.

Race

The incidence of pineal region neoplasms is higher in Asians than in other races.

Sex

  • Pineal germinomas are seen predominantly in males, with a 9:1 male-to-female ratio.
  • Suprasellar germinomas affect males and females equally.

Age

Germinomas appear in persons from newborn to 20 years of age.

Presentation

Anatomy and physiology

 Herophilus discovered the pineal gland in 300 BC, and Galen labeled the gland konareion, because of its resemblance to the nuts found in the cones of the stone pine (Greek: kônos, Latin: pinus pinea). The pineal gland is located in the most posterior portion of the roof of the third ventricle, in the pineal recess. The posterior commissure is posterior and inferior to the pineal gland. Anterior to the pineal gland are the habenular trigone and the striae medullaris thalami. The splenium of the corpus callosum is situated above the pineal gland.

The pineal gland is surrounded by a pial layer. Through numerous perforators, the medial posterior choroidal arteries provide the principal blood supply of the gland. A large venous outflow proceeds through the internal cerebral veins and the vein of Galen and contributes to the vascular distribution of substances synthesized in the pineal gland.

The pineal gland serves as the center of a system of input, feedback, and regulation among diverse areas of the brain. The pineal gland integrates the signals it receives from the environment regarding time of day and time of year and then strategically releases numerous neurohormonal substances that regulate the diurnal and seasonal cycles of several physiological systems. Of the various substances produced by the pineal gland, melatonin is the most important. The synthesis of melatonin is governed by exposure to light.

Pathophysiology

Many types of neoplasms can arise from the pineal region, owing to the presence of various histologically unrelated tissues. Neoplasms include those of the pineal gland (eg, germ cell tumors, pineal cell tumors, pineal cyst); thalamic quadrigeminal plate and tectum (eg, glioma); ependyma (eg, ependymoma); and velum interpositum and tentorium (eg, meningioma). Metastasis and vascular lesions have also been reported.

The most common pineal tumors are germinomas. Germinomas are most commonly seen in the pineal region, followed by the suprasellar region. Rarely, simultaneous pineal and suprasellar germinomas may occur.4,5 Occasionally, germinomas may be seen in the thalamus or basal ganglia. The cellular origin of germinoma usually is ascribed to primitive germ cells that are believed to migrate over wide areas of the embryo during early fetal life. Other germ cell tumors include embryonal cell carcinoma, endodermal sinus carcinoma, choriocarcinoma, and mixed germ cell tumor; however, these are much less common than germinomas.

The marker profile in cerebrospinal fluid or serum may be helpful in the differential diagnosis of germ cell tumors in the pineal region. Choriocarcinoma produces human chorionic gonadotropin, endodermal sinus tumor produces alpha fetoprotein, and embryonal cell carcinoma produces alpha fetoprotein and human chorionic gonadotropin. Mixed germ cell tumors may produce a combination of these tumor markers.

Clinical presentation
The location of the pineal gland (deep in the brain, near critical neural and vascular structures) accounts for the neurologic symptoms that may occur when a mass lesion arises in the gland. Parinaud syndrome, a condition secondary to compression of the tectum, is the most important clinical presentation of pineal germinomas. The triad of Parinaud syndrome includes palsy of the upward gaze, dissociation of light and accommodation, and failure of convergence. In addition, findings secondary to hydrocephalus resulting from aqueductal compression are seen. Pineal germinomas may be associated with precocious pseudopuberty in boys.6

Preferred Examination

MRI is the preferred imaging modality. MRI enables the accurate delineation of pineal masses before surgery. MRI allows true pineal masses to be distinguished from parapineal masses that impinge on the pineal gland.

Limitations of Techniques

MRI is not perfect in the detection of calcifications. CT may be needed to evaluate a calcified pineal gland that is associated with a pineal germinoma or tumor calcification associated with other neoplasms in the pineal region.

Differential Diagnoses

Astrocytoma, Brain
Ganglioglioma
Meningioma, Brain
Pineal Tumors

Other Problems to Be Considered

Other pineal germ cell tumors
Pineocytoma
Pineoblastoma
Metastasis

More on Pineal Germinoma

Overview: Pineal Germinoma
Imaging: Pineal Germinoma
Multimedia: Pineal Germinoma
References
Further Reading
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References

  1. Radovanovic I, Dizdarevic K, de Tribolet N, Masic T, Muminagic S. Pineal region tumors--neurosurgical review. Med Arh. 2009;63(3):171-3. [Medline].

  2. Mori Y, Kobayashi T, Hasegawa T, Yoshida K, Kida Y. Stereotactic radiosurgery for pineal and related tumors. Prog Neurol Surg. 2009;23:106-18. [Medline].

  3. Santagata S, Maire CL, Idbaih A, Geffers L, Correll M, Holton K, et al. CRX is a diagnostic marker of retinal and pineal lineage tumors. PLoS One. Nov 20 2009;4(11):e7932. [Medline].

  4. Guerrero-Vázquez S, Armesto-Pérez V, Macía-Suárez D, Branas-Fernández FM. [Simultaneous suprasellar and pineal germinoma: a case report]. Rev Neurol. Apr 1-15 2008;46(7):411-5. [Medline].

  5. Lee L, Saran F, Hargrave D, Bódi I, Bassi S, Hortobágyi T. Germinoma with synchronous lesions in the pineal and suprasellar regions. Childs Nerv Syst. Dec 2006;22(12):1513-8. [Medline].

  6. Rivarola, Belgorosky A, Mendilaharzu H. Precocious puberty in children with tumours of the suprasellar and pineal areas: organic central precocious puberty. Acta Paediatr. Jul 2001;90(7):751-6. [Medline].

  7. Zhang QB, Feng XY, He HJ, Jiang BD. [Multi-slice helical CT perfusion imaging in evaluating intracranial neoplasms and tumor-like lesions]. Zhonghua Zhong Liu Za Zhi. Feb 2007;29(2):131-5. [Medline].

  8. Pu Y, Mahankali S, Hou J, Li J, Lancaster JL, Gao JH, et al. High prevalence of pineal cysts in healthy adults demonstrated by high-resolution, noncontrast brain MR imaging. AJNR Am J Neuroradiol. Oct 2007;28(9):1706-9. [Medline]. [Full Text].

  9. Costa F, Fornari M, Valla P, Servello D. Symptomatic pineal cyst: case report and review of the literature. Minim Invasive Neurosurg. Aug 2008;51(4):231-3. [Medline].

  10. Moon WK, Chang KH, Han MH, Kim IO. Intracranial germinomas: correlation of imaging findings with tumor response to radiation therapy. AJR Am J Roentgenol. Mar 1999;172(3):713-6. [Medline]. [Full Text].

  11. Chang AH, Fuller GN, Debnam JM, Karis JP, Coons SW, Ross JS, et al. MR imaging of papillary tumor of the pineal region. AJNR Am J Neuroradiol. Jan 2008;29(1):187-9. [Medline]. [Full Text].

  12. Allen JC, Nisselbaum J, Epstein F, et al. Alphafetoprotein and human chorionic gonadotropin determination in cerebrospinal fluid. An aid to the diagnosis and management of intracranial germ-cell tumors. J Neurosurg. Sep 1979;51(3):368-74. [Medline].

  13. Chang CG, Kageyama N, Kobayashi T, et al. Pineal Tumors: clinical diagnosis, with special emphasis on the significance of pineal calcification. Neurosurgery. Jun 1981;8(6):656-68. [Medline].

  14. Edwards MS, Hudgins RJ, Wilson CB, et al. Pineal region tumors in children. J Neurosurg. May 1988;68(5):689-97. [Medline].

  15. Farng KT, Chang KP, Wong TT. Pediatric intracranial germinoma treated with chemotherapy alone. Chung Hua I Hsueh Tsa Chih (Taipei). Dec 1999;62(12):859-66. [Medline].

  16. Ganti SR, Hilal SK, Stein BM, et al. CT of pineal region tumors. AJR Am J Roentgenol. Mar 1986;146(3):451-8. [Medline].

  17. Hoffman HJ, Otsubo H, Hendrick EB, et al. Intracranial germ-cell tumors in children. J Neurosurg. Apr 1991;74(4):545-51. [Medline].

  18. Jenkin D, Berry M, Chan H, et al. Pineal region germinomas in childhood treatment considerations. Int J Radiat Oncol Biol Phys. Mar 1990;18(3):541-5. [Medline].

  19. Jenkin RD, Simpson WJ, Keen CW. Pineal and suprasellar germinomas. Results of radiation treatment. J Neurosurg. Jan 1978;48(1):99-107. [Medline].

  20. Kilgore DP, Strother CM, Starshak RJ, Haughton VM. Pineal germinoma: MR imaging. Radiology. Feb 1986;158(2):435-8. [Medline].

  21. Kluczewska E, Staniek-Sadowska J, Malecka-Tendera E. [Radiological evaluation of pineal pathology and its regions]. Neurol Neurochir Pol. Sep-Oct 1999;33(5):1129-38. [Medline].

  22. Koide O, Watanabe Y, Sato K. Pathological survey of intracranial germinoma and pinealoma in Japan. Cancer. Apr 15 1980;45(8):2119-30. [Medline].

  23. Lin SR, Crane MD, Lin ZS, et al. Characteristics of calcification in tumors of the pineal gland. Radiology. Mar 1978;126(3):721-6. [Medline].

  24. Smirniotopoulos JG, Rushing EJ, Mena H. Pineal region masses: differential diagnosis. Radiographics. May 1992;12(3):577-96. [Medline].

  25. Tamaki N, Yin D. Therapeutic strategies and surgical results for pineal region tumours. J Clin Neurosci. Mar 2000;7(2):125-8. [Medline].

  26. Teo HE, Tan AM. Clinics in diagnostic imaging (37). Germinoma of the pineal gland. Singapore Med J. May 1999;40(5):375-8. [Medline].

  27. Zee CS, Segall H, Apuzzo M, et al. MR imaging of pineal region neoplasms. J Comput Assist Tomogr. Jan-Feb 1991;15(1):56-63. [Medline].

  28. Zimmerman RA, Bilaniuk LT. Age-related incidence of pineal calcification detected by computed tomography. Radiology. Mar 1982;142(3):659-62. [Medline].

  29. Zimmerman RA, Bilaniuk LT, Wood JH, et al. Computed tomography of pineal, parapineal, and histologically related tumors. Radiology. Dec 1980;137(3):669-77. [Medline].

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Keywords

pineal gland, pineal region neoplasms, germ cell tumors, pineal cell tumors, germinoma, mature teratoma, malignant teratoma, embryonal cell carcinoma, endodermal sinus tumor, choriocarcinoma, mixed germ cell tumor, parapineal masses, thalamic quadrigeminal plate and tectum, glioma, ependyma, ependymoma, velum interpositum and tentorium, meningioma, Parinaud syndrome

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Contributor Information and Disclosures

Author

Chi-Shing Zee, MD, Chief of Neuroradiology, Professor, Departments of Radiology and Neurosurgery, University of Southern California School of Medicine
Chi-Shing Zee, MD is a member of the following medical societies: American Society of Neuroradiology
Disclosure: Nothing to disclose.

Coauthor(s)

Zhenwei Yao, MD, Attending Radiologist, Department of Radiology, Huashan Hospital, Fudan University. Shanghai, China
Disclosure: Nothing to disclose.

John L Go, MD, Assistant Professor of Clinical Radiology, Director, Spinal Imaging and Intervention, Division of Neuroradiology, University of Southern California Keck School of Medicine
Disclosure: Nothing to disclose.

Paul E Kim, MD, Assistant Professor of Clinical Radiology, Director, Spinal Imaging and Intervention, Division of Neuroradiology, University of Southern California Keck School of Medicine
Paul E Kim, MD is a member of the following medical societies: American College of Radiology, American Medical Association, American Society of Neuroradiology, American Society of Spine Radiology, and Radiological Society of North America
Disclosure: Nothing to disclose.

Medical Editor

Pamela W Schaefer, MD, Assistant Professor of Radiology, Harvard Medical School; Associate Director of Neuroradiology, Clinical Director of Magnetic Resonance Imaging, Department of Radiology, Massachusetts General Hospital
Disclosure: Nothing to disclose.

Pharmacy Editor

Bernard D Coombs, MB, ChB, PhD, Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand
Disclosure: Nothing to disclose.

CME Editor

Robert M Krasny, MD, Resolution Imaging Medical Corporation
Robert M Krasny, MD is a member of the following medical societies: American Roentgen Ray Society and Radiological Society of North America
Disclosure: Nothing to disclose.

Chief Editor

James G Smirniotopoulos, MD, Professor of Radiology, Neurology, and Biomedical Informatics; Program Director, Diagnostic Imaging Program, Center for Neuroscience and Regenerative Medicine (CNRM), Uniformed Services University of the Health Sciences
James G Smirniotopoulos, MD is a member of the following medical societies: American College of Radiology, American Roentgen Ray Society, American Society of Head and Neck Radiology, American Society of Neuroradiology, American Society of Pediatric Neuroradiology, Association of University Radiologists, and Radiological Society of North America
Disclosure: Nothing to disclose.

 
 
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