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|LETTERS TO EDITOR
|Year : 2019 | Volume
| Issue : 1 | Page : 308-311
Pontomedullary germinoma with suprasellar and spinal metastasis: A report and comprehensive review of literature
Kanwaljeet Garg, Ravi Sharma, Hitesh K Gurjar, Shashank S Kale
Department of Neurosurgery, All India Institute of Medical Sciences, New Delhi, India
|Date of Web Publication||7-Mar-2019|
Dr. Hitesh K Gurjar
Department of Neurosurgery, All India Institute of Medical Sciences, New Delhi - 110 029
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Garg K, Sharma R, Gurjar HK, Kale SS. Pontomedullary germinoma with suprasellar and spinal metastasis: A report and comprehensive review of literature. Neurol India 2019;67:308-11
|How to cite this URL:|
Garg K, Sharma R, Gurjar HK, Kale SS. Pontomedullary germinoma with suprasellar and spinal metastasis: A report and comprehensive review of literature. Neurol India [serial online] 2019 [cited 2019 Mar 18];67:308-11. Available from: http://www.neurologyindia.com/text.asp?2019/67/1/308/253629
Germinomas are the most common intracranial germ cell tumors and account for approximately 40% of all germ cell tumors. The most common sites for germinomas are the pineal region followed by the suprasellar region. Other rare sites include basal ganglia, thalamus, cerebellopontine angle, and spine., We present an extremely rare case of pontomedullary germinoma along with its suprasellar and spinal metastasis. We also review the available literature.
A 17-year-old male patient presented with a history of gait ataxia, diminution of vision, and lower cranial nerve palsy in the form of difficulty in swallowing and nasal regurgitation of food for 3 months. On examination, the patient had moderate sensorineural hearing loss in the right ear, as well as right IX, X, and XII nerve palsy. Bilateral corticospinal tract involvement was also present. Noncontrast CT scan showed a hyperdense mass lesion in the posterior fossa in the midline with slight effacement of the fourth ventricle, and the suprasellar and pineal regions were free of any lesion [Figure 1]. The patient was advised surgery but was lost to follow-up for 10 months. Noncontrast CT scan after 10 months showed enlargement of the hyperdense mass lesion of the posterior fossa along with the suprasellar hyperdense lesion. However, the pineal region did not have any lesion [Figure 2]. The patient was admitted and his magnetic resonance imaging (MRI) revealed a T2 hyperintense lesion in the midline posterior fossa, extending more towards the right side, with effacement of the fourth ventricle. Post gadolinium scan showed an intensely enhancing mass lesion in the posterior fossa and suprasellar region as well. Pineal region did not show any enhancement [Figure 3] and [Figure 4]. Screening MRI of the spine showed tiny nodular foci of enhancement in the conus at the L1, L2 level [Figure 5]. Based on the clinical presentation and imaging studies, a diagnosis of medulloblastoma was considered. The patient underwent a midline suboccipital craniotomy and subtotal excision of the lesion. Intraoperatively, the cerebellar vermis and roof of the fourth ventricle were free from the lesion. The lesion was arising from the pontomedullary junction and occupying the fourth ventricle. Central decompression was done. The frozen section was suggestive of a medulloblastoma. Subtotal excision was achieved. The patient was doing fine in the postoperative period with no new neurological deficits; however, he could not be weaned off from the ventilator because of poor respiratory efforts. On postoperative day 5, the patient developed wide fluctuation in blood pressure. His systemic work-up was normal. On postoperative day 6, the patient had cardiac arrest from which he could not be revived. The definitive histopathological report was suggestive of a germinoma.
|Figure 1: Noncontrast CT scan showing a hyperdense lesion in the posterior fossa. Suprasellar and pineal regions do not show any lesion|
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|Figure 2: Noncontrast CT after 10 months shows an increased size of the posterior fossa lesion and a new lesion in the suprasellar location. Note the pineal region does not show any lesion|
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|Figure 3: Contrast-enhanced MRI axial view showing the contrast enhancing lesion arising from the dorsal aspect of the pontomedullary junction partly pushing the fourth ventricle and partly occupying it|
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|Figure 4: Contrast-enhanced MRI sagittal view showing the contrast enhancing lesion arising from the dorsal aspect of the pontomedullary junction partly pushing the fourth ventricle and partly occupying it. A well-defined hyperdense lesion can be well seen in the suprasellar region|
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|Figure 5: Screening MRI of the spine showingtiny nodular foci of enhancement in the conus at L1, L2 level|
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Germinomas are the most common germ cell tumors. The classification of germ cell tumors was first proposed by Teilum et al., in 1976 as germinoma and nongerminomatous germ cell tumor (NG-GCT). It was also stated that all germ cell tumors arise from primordial germ cell. This cell then differentiates and gives rise to germinoma or other germ cell tumors (choriocarcinoma, teratoma, endodermal sinus tumor). The pitfall of this theory is that a germinoma, despite being the most undifferentiated tumor, usually has the most favorable prognosis. Sano  proposed a hypothesis for tumorigenesis that a germ cell tumor represent the neoplastic equivalent of an embryonic stage of development.
Primary germinomas of the pontomedullary region are extremely rare, which makes their preoperative diagnosis a challenging task. Till date, only 16 such cases have been reported in the literature that are summarized in [Table 1].,,,,,,,,,,,,,, Only one case has been reported previously from the Indian subcontinent. Pontomedullary germinomas have been reported in female patients mostly and generally present in young adults and adolescents. The possible hypothesis for the female preponderance in a primary pontomedullary germinoma is the delayed closure of the anterior neuropore during female embryonic development.
|Table 1: Summary of cases of pontomedullary germinomas reported in the literature|
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The current case is unique as it is the first reported case of primary pontomedullary germinoma with suprasellar and spinal metastases. This also highlights the significance of cerebrospinal fluid (CSF) dissemination of tumor cells in such pathologies. It is common to find the primary intracranial germinoma being disseminated throughout the ventricular system and subarachnoid space due to the CSF spread. This finding necessitates the radiological screening of the entire spine using contrast-enhanced MRI.
Although CSF and serum markers of germ cell tumors (such as alpha fetoprotein, beta HCG, placental alkaline phosphatase) and imaging help in the diagnosis of a germ cell tumor, the tissue diagnosis is the main modality for its diagnosis. An accurate diagnosis is very important as these tumors are very sensitive to chemotherapy and radiotherapy, limiting the role of surgery only for obtaining a tissue diagnosis or for CSF diversion. Thus, an additional neurological morbidity after surgery in an attempt to completely excise the tumor may be prevented. Radical resection of germinoma offers no benefit over solely its biopsy.
Owing to the rarity of the disease, there is a lack of consensus regarding its treatment. Considering the pathological characteristics, it is plausible to consider that brainstem germinomas must be radiosensitive. Studies support this finding as the 10-year survival rates for those patients treated with radiotherapy approach 90%.,
However, it must be considered that radiotherapy might have significant long-term side effects on the adolescent population that usually suffers from this disease. Physical and mental development may be hindered due to radiation damage to the skeletal and nervous system. This has given way to the trial of a combination of low dose radiation and chemotherapy which has proven to be as effective in the treatment, if not more, as radiotherapy alone.,, Though theoretically surgery may not be needed for the treatment of pontomedullary germinoma, rarely can the diagnosis be clinched preoperatively without a histologic confirmation. Consequently, surgery remains a part of the management protocol. Moreover, surgery does not seem to adversely affect the final outcome with approximately 90% patients having a favorable prognosis.,,,,,,,, Partial resection followed by radiotherapy may be a sufficient treatment for this pathology, however, whole spine radiotherapy may be necessary to ensure a progression-free long-term survival due to the possibility of CSF spread.,
A germinoma must be kept in the list of differential diagnoses in a case of midline pontomedullary lesion. Once confirmed histologically, adjuvant radiotherapy or concurrent chemoradiotherapy must be administered and a relatively good prognosis may be expected. Those with radiological evidence of CSF dissemination must receive whole spine radiotherapy as well.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that name and initial will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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