Pineal Germinoma

Nandakumar Srinivasan, MD; Aneesh Pakala, MD; Chandana Mukkamalla, MD; Alok Oswal, MD

Disclosures

South Med J. 2010;103(10):1031-1037. 

In This Article

Case Report

A 29-year-old African American gentleman with no significant past medical history presented with two months, history of left side facial numbness, polyuria, polydipsia, and a ten pound weight loss. He complained of intermittent nausea and vomiting for six weeks, dizziness, intolerance to cold, left-sided hearing loss, decreased taste sensation, decreased libido, and erectile dysfunction. He denied headache, visual disturbances, seizures, or syncope. His vital signs were unremarkable but for an elevated blood pressure of 152/118 and a heart rate of 124. His neurological exam showed abnormalities in the sensory distribution of the left sided trigeminal, facial, and vestibulocochlear nerves. The rest of his neurological exam including the remaining cranial nerves, motor and sensory nerves, reflexes, cerebellar signs, and gait were unaffected. Genital exam revealed bilateral atrophied testicles. The chest, heart, and abdominal exams were unremarkable.

Routine Chemistries

Complete blood count and comprehensive metabolic panel (Na = 138, K = 4.3, Cl = 99, blood urea nitrogen = 7, Cr = 0.8, Ca+ = 9.3, T-protein = 6.8, and albumin = 4.2) were unremarkable except for a slightly elevated CO2 of 32. His urine and serum osmolarity were 89 mOsm/kg and 298 mOsm/kg, respectively.

Imaging

Computed tomography (CT) of the head showed a 2.5 cm hyperdense pineal gland mass with an eccentric calcification, suggesting pineal germinoma. Subsequent magnetic resonance imaging (MRI) of the brain (Figs. 1 and 2) showed a 2.8 cm metastatic isointense pineal gland lesion on T1 and T2 sequences with heterogeneous enhancement on contrast studies. These lesions were bilaterally encasing the trigeminal and vestibulocochlear nerves. Metastasis was seen over the superior sella, and the neurohypophysis was not visualized. MRI of the spine (Fig. 3) showed a subarachnoid spread of metastatic disease extending from the level of the conus medullaris inferiorly to involve the cauda equina nerve roots with moderate mass effect on the conus. Positron emission tomography (PET) scan showed a metabolically active primary pineal mass with a standard uptake value of 7.2.

Figure 1.

Magnetic resonance imaging (MRI) of brain: T1 axial + contrast.

Figure 2.

MRI of brain: T1 sagittal + contrast.

Figure 3.

MRI of L-spine: T1 sagittal + contrast.

Endocrine Studies

Decreased lactate hydrogenase (0.1 IU/mL), follicle stimulating hormone (0.3 IU/mL), and free testosterone (<20 ng/dL) levels were detected. Prolactin was elevated (27.5 ng/mL), while thyroid stimulating hormone (0.5 micro IU/mL), free T4 (0.8 ng/dL), adrenocorticotropic hormone (15 pg/mL), AM cortisol (6.6 mcg/dL), and insulin-like growth factor 1 (128 ng/mL) levels were within normal range. His tumor marker studies, including alpha-fetoprotein (1.5 ng/mL), carcinoembryonic antigen (4.12 ng/mL), and human chorionic gonadotrophin (2.7 mIU/L), were unremarkable.

Cerebrospinal Fluid (CSF) Studies

Xanthochromic, hazy CSF with a white blood count of 430/mm3 (L-82%, M-17%, and others-1%), red blood count of 890, protein 102 mg/dL, and glucose 50 mg/dL was found. The CSF cytology (Fig. 4) revealed placental alkaline phosphatase positive, high-grade malignant cells in a background of small lymphocytes consistent with germinoma (Fig. 5).

Figure 4.

Photomicrographs of cerebrospinal fluid showing tumor cells with large nuclei and prominent nucleolus admixed with lymphocytes.

Figure 5.

Immunohistochemistry highlights placental alkaline phosphatase-positive germinoma cells.

Management

His symptoms of diabetes insipidus resolved with desmopressin. The development of infertility, in the future, was raised with this patient before the treatment was started. Considering the metastatic nature of his disease and the lack of time for inducing spermatogenesis, treatment was initiated with radiation and chemotherapy, with carboplatin and etoposide.

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