The pineal gland is a neuroendocrine gland responsible for melatonin production and release into the bloodstream [11]. Tumors in the pineal region are fairly rare, accounting for less than 1% of primary intracranial neoplasms of the central nervous system (CNS). They are often diagnosed at early age, between 10 and 21 years in 68% of cases. Also, they affect males more than females [2, 5].
Intracranial germ cell tumors (GCTs) can be divided into germinomas and nongerminomatous germ cell tumors (NGGCTs), which include five major groups of teratomas (mature, immature, and teratoma with malignant differentiation), choriocarcinomas, yolk sac or endodermal sinus tumors, embryonal carcinomas, and mixed GCTs (intermediate- and poor-prognosis groups) [2,3,4,5,6,7,8,9,10].
A series of 370 patients aged 3–73 years with pineal tumors found that the most common type was germinoma (27%), followed by astrocytoma (26%) then pineoblastoma (12%), pineocytoma (12%), ependymoma (4.3%), teratoma (4.3%,), metastasis, ganglioglioneuroma, lymphoma, meningioma, and pineal cyst (2.7%), mixed embryonal cell tumor (embryonal carcinoma) (1.6%), choriocarcinoma (1.1%), and oligodendroglioma (0.54%) [12].
In our case, the patient had pineal teratoma with age of 34 years at time of diagnosis, and recurrent teratoma with spinal and vertebrae metastasis when he came to our hospital. Just one report of pineal region metastasis to spinal cord was found in literature.
The most frequent location of GCTs is the pineal and neurohypophyseal (suprasellar) region, while they occur less commonly in basal ganglia or other brain localities [6].
Clinical signs depend on the tumor location and size and patient age. Pineal region tumors usually cause obstructive hydrocephalus leading to increased intracranial pressure (ICP), revealed by headache, nausea, vomitus, and somnolence.
Other possible symptoms of a tumor in the pineal region are visual disorders, endocrine abnormalities, sexual dysfunction, growth failure, puberty delay, ataxia, seizures, and behavioral changes.
Many patients develop diabetes insipidus (DI) because of dysfunction of neurohypophysis.
Parinaud’s syndrome is present in half of such patients, causing paralysis of upward gaze, while paralysis of downward gaze is less frequent, similarly to convergence disorder and/or convergence–retraction nystagmus [1, 3].
Computed tomography (CT) and magnetic resonance imaging (MRI) are the radiological examination modalities of choice in the diagnostic strategy, in addition to the presence of specific markers produced by GCTs such as alpha-fetoprotein (AFP), beta-human chorionic gonadotropin (beta-hCG), and placental alkaline phosphatase (PLAP). However, the final diagnosis depends on histological examination by biopsy, and it is important to choose the correct management [6,7,8].
Chemotherapy and radiation efficiently treat germinomas. On the other hand, surgical resection alone can treat teratomas. Meanwhile, mixed GCTs may require a combination of all the treatment choices mentioned before [10].
Germinomas and mature teratomas have the best recovery and life expectancy [9].
Based on the discussion above and due to the sudden death of the patient, the main diagnosis is recurrent pineal teratoma with spinal and vertebrae metastases. Because the tumor is at an advanced stage and neurological symptoms are caused by metastases, such patients with teratoma should be observed with frequent examination of both brain and spinal cord to detect any potential metastases.