Diagnosis and Management of Pineal Germinoma: From Eye to Brain

Figures & data

Figure 1 Nearly midline sagittal T1 MRI with gadolinium of pineal region germinoma in a 20-year-old male patient. The patient had signs and symptoms of double vision, light-near dissociation, and decreased upgaze. Cerebrospinal fluid had no markers of germinoma. Diagnosis was made with surgical resection via a infratentorial-supracerebellar approach to minimize risk of uncontrollable hemorrhage given the small size of bulky disease and proximity to major venous structures. (A) – splenium of corpus callosum; (B) – Vein of Galen; (C) – quadrigeminal cistern; (D) – inferior and superior colliculi; (E) – 4th ventricle; (F) – cerebral aqueduct (nearly collapsed); (G) – pineal germinoma, homogenously enhancing; (H) – subependymal spread of germinoma enhancing into mildly enlarged lateral ventricle; (I) – small sellar component of germinoma enhancing.

Figure 2 Midline sagittal T1 MRI with gadolinium of bifocal pineal region/suprasellar germinoma in a 15-year-old female patient. The patients had signs and symptoms of headache, bitemporal hemianopia, and polydipsia. Diagnosis was made via transsphenoidal resection of the suprasellar component of the mass to relieve the visual compromise. Arrows point out key structures as follows: (A) – splenium of corpus callosum; (B) – Vein of Galen; (C) – quadrigeminal cistern; (D) – inferior and superior colliculi; (E) – 4th ventricle; (F) – cerebral aqueduct; (G) – very small pineal germinoma, homogeneously enhancing; (H) - large sellar/suprasellar component of germinoma enhancing; (I) – 3rd ventricle; (J) – location of elevated and compressed optic chiasm (not well visualized).

Figure 3 Axial CT without contrast of a 14-year-old boy presenting with headache and Parinaud’s syndrome. The tumor (A) is hyperdense, likely due to its cellularity. The third ventricle (B) is rounded, and the temporal horn of the lateral ventricle (C) is slightly dilated, consistent with early hydrocephalus.

Table 1 Components in Diagnosis of Pineal Germinoma

Component	Findings	Frequencies
History	Headache	Very common
	Blurry vision	Common
	Double vision	Common
	Weakness	Uncommon
	Low back pain	Uncommon
Physical examination	Papilledema	Common
	Parinaud’s syndrome	Common
	Decreased up-gaze	Common
	Diplopia	Common
	Visual field deficits	Less common
	Other ocular findings	Uncommon to rare
	Hemiparesis	Uncommon
	Paraparesis	Uncommon
Imaging	CT Homogenous hyperdense	Very common
	Cysts, calcifications, hemorrhage	Very common
	Enhances with contrast	Very common
	Hydrocephalus	Common
	MRI
	T1 and T2 -isointense	Very common
	DWC – hyperintense	Very common
	Enhances with gadolinium	Very common
	Hydrocephalus	Common
	MRI spectroscopy
	High lipid profile	Very common
	Spinal MRI
	Enhancing lesions	Less common
Cerebrospinal fluid	β-HCG < 50 mIU/mL	Very common
	PLAP – elevated	Very common
	α-FP – negative	Very common
	Cytology - positive	Less common
Endocrine findings	Diabetes insipidus	Less common
	Hyperprolactinemia	Common
Tissue examination	Endoscopic biopsy	Almost always
	Stereotactic biopsy	Almost always

Notes: Almost always = > 95%; very common = 80% - 95%; common = 50% - < 90%; less common = 20% - < 50%; uncommon = 5% - < 20%; rare = < 5%.

Abbreviations: CT, computed tomography; MRI, magnetic resonance imaging; DWC, diffusion-weighted imaging; β-HCG, beta human chorionic gonadotropin; PLAP, placental alkaline phosphatase; α-FP, alpha fetoprotein.

Figure 4 Diagram of general management plan. Exceptions may occur. Option 1: four cycles of carboplatin on day 1 at 600 mg/m² and etoposide at 150 mg/m²/day on days 1–3. Option 2: four cycles of carboplatin 600 mg/m² on day 1 and VP16 90mg/m² on days 1–3 alternating with ifosfamide 1800 mg/m² on days 1–5 and VP16 90 mg/m² on days 1–5.

Abbreviations: VP16, etoposide; WVI, whole-ventricular irradiation; CSI, cranio-spinal irradiation; Gy, Gray.

Figure 5 (A) Axial MRI with gadolinium of the same 14-year-old from Figure 3. The enhancing tumor (A) is extending towards the thalamus. (B). Sagittal MRI with gadolinium of the same 14-year-old showing the enhancing tumor (B) and a cyst within the tumor (C). The patient had an endoscopic third ventriculostomy with biopsy to confirm the diagnosis of germinoma and treat his hydrocephalus. He was then treated with chemotherapy, followed by local radiation therapy.