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DOI:10.2214/AJR.05.0519
AJR 2006; 186:S233-S235
© American Roentgen Ray Society

AJR Teaching File: Solid Masses of the Pineal Region

Kevin P. Banks1 and Stephen J. Brown1

1 Both authors: United States Army, MCHE-DR, 3851 Roger Brooke Dr., Fort Sam Houston, TX 78234.

Received April 19, 2005; accepted after revision August 18, 2005.

 
Address correspondence to K. P. Banks (Kevin.Banks{at}amedd.army.mil).

CONTINUING MEDICAL EDUCATION

The AJR Teaching File articles are available for .25 CME credit for completing all three articles. They are free to ARRS members and may be purchased by nonmembers for $10.00 each. Detailed information including objectives, disclosure information, and how to obtain CME credit can be found at www.arrs.org by selecting AJR Integrative Imaging.

Keywords: germ cell tumors • germinoma • MRI • neuroradiology • pineal gland


Clinical History
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Clinical History
Radiologic Description
Differential Diagnosis for Solid...
Diagnosis
Commentary
Objective
Conclusion
References
 
A 19-year-old man presents with headaches and visual disturbances. Physical examination reveals double vision, vertical gaze paralysis, and light-near dissociation.


Radiologic Description
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Clinical History
Radiologic Description
Differential Diagnosis for Solid...
Diagnosis
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Unenhanced head CT reveals a well-defined homogeneously hyperdense solid lesion arising in the pineal region (Fig. 1A). There is a large solid calcification with two adjacent smaller calcifications seen within the mass rather than calcifications "exploding" to the periphery. The surrounding brain parenchyma shows mild edema. Obstructive hydrocephalus is present.


Figure 1
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Fig. 1A —19-year-old man presents with headaches and visual disturbances. Physical examination reveals double vision, vertical gaze paralysis, and light-near dissociation. Unenhanced CT images of head show well-defined hyperdense mass arising in pineal region. Large dense calcification is seen in inferior portion with two adjacent smaller calcifications. Surrounding brain parenchyma shows mild edema. Obstructive hydrocephalus is present.

 
Sagittal T1-weighted and axial T2-weighted MR images show the solid mass to be isointense to gray matter (Figs. 1B and 1C). A coronal T1-weighted image obtained after gadolinium administration shows uniform enhancement (Fig. 1D). No other lesions are present.


Figure 2
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Fig. 1B —19-year-old man presents with headaches and visual disturbances. Physical examination reveals double vision, vertical gaze paralysis, and light-near dissociation. Sagittal T1-weighted MR image shows mass to be isointense to gray matter.

 

Figure 3
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Fig. 1C —19-year-old man presents with headaches and visual disturbances. Physical examination reveals double vision, vertical gaze paralysis, and light-near dissociation. Axial T2-weighted MR image shows isointense signal intensity to gray matter. Surrounding increased signal intensity verifies presence of localized edema.

 

Figure 4
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Fig. 1D —19-year-old man presents with headaches and visual disturbances. Physical examination reveals double vision, vertical gaze paralysis, and light-near dissociation. Postcontrast T1-weighted coronal MR image shows avid uniform enhancement of mass.

 

Differential Diagnosis for Solid Pineal Region Mass
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Clinical History
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Differential Diagnosis for Solid...
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The differential diagnosis in this case is a pineal germ cell tumor, including germinoma, teratoma, choriocarcinoma, endodermal sinus tumor, and embryonal carcinoma; pineoblastoma; pineocytoma; tectal plate astrocytoma; and meningioma.


Diagnosis
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Differential Diagnosis for Solid...
Diagnosis
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The correct diagnosis is pineal germinoma.


Commentary
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Clinical History
Radiologic Description
Differential Diagnosis for Solid...
Diagnosis
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Objective
Conclusion
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Recognition of the unique location and solid composition of the tumor in this case is essential to the development of an accurate, focused differential diagnosis. Solid lesions of the pineal region include germ cell tumors, pineal parenchymal tumors, astrocytomas, and meningiomas. Pineal germinomas are uncommon lesions, accounting for only 3-8% of brain tumors in pediatric patients and fewer than 4% of intracranial neoplasms [1, 2].

Lesions in the pineal region frequently obstruct the aqueduct of Sylvius, which leads to hydrocephalus and its associated symptoms of headaches and nausea. A classic clinical finding is the combination of vertical gaze paralysis; pupillary dilation; lid retraction; pupillary light-near dissociation; and paralysis of convergence, which is known as Parinaud's syndrome. Parinaud's syndrome is seen if the tumor exerts significant mass effect on the tectum of the midbrain [3].

As part of their evaluation, individuals with a pineal region mass should have levels of serum or CSF {alpha}-fetoprotein, ß-HCG, and placental alkaline phosphatase measured. If these markers are elevated, the presence of a germ cell tumor can be presumed [1, 2].

Germ cell tumors are the most frequently encountered type of tumor in the pineal region, constituting one half of such lesions in females and three quarters of those encountered in males. "Germ cell tumor" is a broad term that encompasses germinomas, teratomas, endodermal sinus tumors, and embryonal carcinomas. The mixed-variety germ cell tumor, which is composed of a combination of two or more of these subtypes, constitutes a quarter of intracranial germ cell tumors [3]. These neoplasms tend to disseminate along CSF pathways with drop metastases to the spine, and metastases are also found often in the suprasellar cistern, although synchronous tumors may also arise independently in this region [1]. The incidence of germ cell tumors peaks near puberty, with nonseminomatous germ cell tumors frequently diagnosed by age 10 and germinomas usually diagnosed in the second decade of life.

The most common pure germ cell tumor subtype is a germinoma. This is seen on CT as a homogeneous hyperdense mass, which is a characteristic feature of germinoma, primitive neuroectodermal tumor, ependymoma, and lymphoma. When seen in a pineal region mass, this CT finding is fairly specific for germinoma. On MR evaluation, signal intensity is likewise homogeneous and similar to that of gray matter on both T1- and T2-weighted sequences. The mass frequently engulfs a densely calcified pineal gland. In contrast, teratomas are multilocular heterogeneous masses containing lipid areas [3]. Pineal calcification on skull radiographs, uncommon in children younger than 10 years, is a useful clue to the diagnosis of a germ cell tumor because approximately 70% of patients with pineal region tumors have calcifications [2].

Specific clinical findings include diabetes insipidus, which is noted in many patients and is highly suggestive for a germinoma with spread to the suprasellar region. In the absence of a pineal mass, other causes that should be considered are eosinophilic granuloma involving the pituitary stalk or suprasellar subarachnoid cistern. Likewise, precocious puberty in the setting of a pineal region tumor suggests a diagnosis of choriocarcinoma instead of other common entities such as a hypothalamic hamartoma or sellar craniopharyngioma [4].

Pineal parenchymal neoplasms are less common and usually are either pineocytomas or pineoblastomas. These entities classically result in preexisting pineal calcifications dispersing to the periphery of the lesion [3].

Astrocytomas in the pineal region typically arise from the neighboring tectal plate and have a similar appearance on CT and MRI as when encountered elsewhere in the brain. Given their location of origin, pineal calcifications are displaced superiorly.

Meningiomas have a similar appearance in the pineal region as elsewhere; a dural tail attached to the tentorium is the distinctive feature.

Although the correct histologic diagnosis of a solid tumor of the pineal region may be suggested with careful evaluation of morphologic features, attenuation, and signal intensity characteristics, few of these tumors have a truly pathognomonic imaging pattern. In the end, biopsy is almost always necessary to accurately guide therapy. If biopsy yields germinoma, common practice is to treat with radiation therapy or chemotherapy, followed by a second-look resection of any residual soft-tissue or calcifications, which are presumed to represent a nongerminoma component of the original tumor.


Objective
Top
Clinical History
Radiologic Description
Differential Diagnosis for Solid...
Diagnosis
Commentary
Objective
Conclusion
References
 
The objective of this article is to describe the imaging findings associated with solid tumors in the pineal region. In addition, it reviews the clinical and radiologic features of these lesions, which should be considered in development of an ordered and accurate differential diagnosis.


Conclusion
Top
Clinical History
Radiologic Description
Differential Diagnosis for Solid...
Diagnosis
Commentary
Objective
Conclusion
References
 
Recognition of the unique location and solid composition of a tumor in the pineal region is essential to the development of an accurate, focused differential diagnosis. Solid lesions of the pineal region include germ cell tumors, pineal parenchymal tumors, astrocytomas, and meningiomas.


References
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Clinical History
Radiologic Description
Differential Diagnosis for Solid...
Diagnosis
Commentary
Objective
Conclusion
References
 

  1. Maher CO, Raffel C. Neurosurgical treatment of brain tumors in children. Pediatr Clin N Am 2004;51 : 327-357[Medline]
  2. Packer RJ, Cohen BH, Coney K. Intracranial germ cell tumors. Oncologist 2000;5 : 312-320[Abstract/Free Full Text]
  3. Smirniotopoulos JG, Rushing EJ, Mena H. Pineal region masses: differential diagnosis. RadioGraphics1992; 12:577 -596[Abstract]
  4. Hoffman H, Yoshida M, Becker LE, et al. Pineal region tumors in childhood. Pediatr Neurosurg 1994;21 : 91-104[Medline]

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Am. J. Roentgenol.Home page
C. C. Roberts, K. P. Banks, J. R. Hesselink, and F. S. Chew
Imaging of the pineal region and spine: self-assessment module.
Am. J. Roentgenol., March 1, 2006; 186(3 Suppl): S224 - S226.
[Abstract] [Full Text] [PDF]


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