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American Journal of Public Health Research
DOI: 10.12691/ajphr-1-1-3

The Implementation of a Tele-Homecare System with Service Oriented Architecture and HL7 Message Transmission Standard
Ching-Sung Wang

American Journal of Medical and Biological Research
DOI: 10.12691/ajmbr-1-1-2

The Influence of Ethanol on Pyruvate Kinases Activity in Vivo, in Vitro, in Silico
Lelevich Sergey Vladimirovich, Khrustalev Vladislav Victorovich, Barkovsky Eugene Victorovich, Shedogubova Tatyana Aleksandrovna

American Journal of Medical Sciences and Medicine
DOI: 10.12691/ajmsm-1-1-1

Clinical and Histological Features in Wegener Granulomatosis
Li Cavoli Gioacchino, Passantino Rita, Bono Luisa, Tortorici Calogera, Ferrantelli Angelo, Giammarresi Carlo, Rotolo Ugo

American Journal of Infectious Diseases and Microbiology
DOI: 10.12691/ajidm-1-1-2

The Curse of Lymphatic Filariasis: Would the Continual Use of Diethylcarbamazine Eliminate this Scourge in Papua New Guinea?
Anne Lanham, Lillian Mwanri

We have released issues of some journals on February 2013.
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Dear Label_Name,

Greetings! I hope you are well.

I am writing on behalf of an international publishing house, Science and Education Publishing, USA (http://www.sciepub.com).

Science and Education Publishing (SciEP) is a multidisciplinary open access publisher of Journals covering the fields of Science, Technology and Medicine. All SciEP publications are subject to high-quality peer review, editorial and production processes and are freely available online.

Welcome to the April, 2013 edition of SciEP newsletter, providing monthly summaries of articles on science & education related topics of interest to scientist and professionals. The mission of our journals is to provide an open access forum for publishing new findings on issues pertinent to scientific and academic field. We also invite you to submit original research papers, review articles, short reports, or case studies are considered for publication in our Multidisciplinary Journals.

American Journal of Public Health Research
DOI: 10.12691/ajphr-1-1-3

The Implementation of a Tele-Homecare System with Service Oriented Architecture and HL7 Message Transmission Standard
Ching-Sung Wang

American Journal of Medical and Biological Research
DOI: 10.12691/ajmbr-1-1-2

The Influence of Ethanol on Pyruvate Kinases Activity in Vivo, in Vitro, in Silico
Lelevich Sergey Vladimirovich, Khrustalev Vladislav Victorovich, Barkovsky Eugene Victorovich, Shedogubova Tatyana Aleksandrovna

American Journal of Medical Sciences and Medicine
DOI: 10.12691/ajmsm-1-1-1

Clinical and Histological Features in Wegener Granulomatosis
Li Cavoli Gioacchino, Passantino Rita, Bono Luisa, Tortorici Calogera, Ferrantelli Angelo, Giammarresi Carlo, Rotolo Ugo

American Journal of Infectious Diseases and Microbiology
DOI: 10.12691/ajidm-1-1-2

The Curse of Lymphatic Filariasis: Would the Continual Use of Diethylcarbamazine Eliminate this Scourge in Papua New Guinea?
Anne Lanham, Lillian Mwanri

We have released issues of some journals on February 2013.
American Journal of Public Health Research
American Journal of Medical Sciences and Medicine
American Journal of Medical and Biological Research
American Journal of Pharmacological Sciences
American Journal of Clinical Medicine Research
American Journal of Nursing Research
American Journal of Cancer Prevention
American Journal of Medicine Studies
American Journal of Infectious Diseases and Microbiology
American Journal of Cardiovascular Disease Research
American Journal of Biomedical Research
American Journal of Mechanical Engineering
American Journal of Microbiological Research
Journal of Preventive Medicine
American Journal of Food and Nutrition
American Journal of Hypertension Research
American Journal of Sports Science and Medicine
Journal of Cancer Research and Treatment
American Journal of Zoological Research
Journal of Biomedical Engineering and Technology
American Journal of Microbiological Research
Biomedical Science and Engineering

We are pleased to announce that submissions are now being accepted for peer review for all the journals across all our subject areas in SciEP.

Please submit your manuscript via our online submission system:
http://www.sciepub.com/author/signin

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A Rare Case of Primary CNS Germinoma in the Pineal Region with Long-standing Hearing Loss

Mohammad Alrehaili*, Emad Tashkandi

Department of Internal Medicine, King Abdullah Medical City, Makkah, Saudi Arabia

Abstract: Germinomas in the brain are rare neoplasms which make up less than 1% of all intracranial tumors. Their clinical presentation is usually related to the increased intracranial pressure and/or hydrocephalus. This report describes a case who presented to our hospital for headaches and visual changes, including Parinaud's syndrome, that had started recently. The patient also had been complaining of chronic bilateral hearing loss for several years which seemed not properly evaluated previously. MRI brain showed a markedly enhancing lesion in the pineal region. CSF and serum tumor markers including B-human chorionic gonadotropin (B-HCG) and alfa-fetoprotein (AFP) were normal. Lumbar puncture with cytology was negative for malignancy. Biopsy was taken from the mass and sent to the pathology lab for testing. Histopathological examination showed changes consistent with germinoma and the immunohistochemical staining including that for placental alkaline phosphatase (PLAP) confirmed the diagnosis. There was no evidence of metastasis and the patient was treated with radiation-alone therapy that resulted in marked regression of the mass and significant clinical improvement except for the issue of hearing loss which had persisted, as expected, given its long-standing existance. In addition to highlighting the diagnostic evaluation and treatment approach for such rare tumors, this case suggests considering pineal germinomas among the differential diagnosis when dealing with hearing loss which needs careful evaluation and should never be underestimated.

Keywords: CNS germinoma, pineal region, hearing loss, Parinaud's syndrome, immunohistochemistry

Received December 02, 2019; Revised February 06, 2020; Accepted February 21, 2020

Citation: Mohammad Alrehaili, Emad Tashkandi, A Rare Case of Primary CNS Germinoma in the Pineal Region with Long-standing Hearing Loss. American Journal of Medical Case Reports, 8 (4). 106-109. January 2020. doi:10.12691/ajmcr-8-4-4

1. Introduction

Brain germinomas are rare neoplasms that represent around 1% of all intracranial tumors [1]. However, they are considered the most common type of germ cell tumor affecting the central nervous system. The most common site of involvement is the pineal region, followed by suprasellar region and other locations [2,3]. They usually occur in male children and young adults, with a median age of diagnosis between 10 and 12 years [3]. Here, we present a rare case of pineal region germinoma that was referred to our hospital for headaches and visual changes that had developed during the last few months. Moreover, the patient had chronic hearing loss for several years which is considered an unusual complaint for such tumors. When present, hearing problems secondary to germinoma can be reversed with debulking surgery if performed early enough before irreversible nerve damage occurs [7,8].

2. Description

18 year-old male was referred to our hospital for a 3-month history of headache that was left-sided and progressive in severity. It was associated with blurred vision and difficulty with ocular movements in both sides during the last few weeks. There was no nausea, vomiting, focal weakness or coordination problems. The patient had a past medical history remarkable for chronic hearing loss for several years that was not properly evaluated. He had no surgical history and his family history was unremarkable. He is a student in the high school for the deaf with an average performance. Physical examination revealed sensorineural hearing loss that was confirmed by audiogram. Moreover, he had some abnormalities consistent with Parinaud's syndrome including paralysis of upward gaze in addition to loss of accommodation and pupillary light reflex.

MRI of the brain with contrast showed a heterogeneous, markedly enhanced supratentorial central mass which is expanding the left midbrain and extending to the left cerebral peduncle and the left thalamus (Figure 1). It was lobulated and irregular-shaped and measured 2.8 X 1.6 X 1.7 cm with mass effect on the adjacent third ventricle and ambient cistern. The lateral horns of the lateral ventricle were slightly dilated indicating early hydrocephalus. The patient was admitted and dexamethasone was started due to mass effect and cerebral edema in addition to levetiracetam for seizure prophylaxis. Laboratory investigations including B-HCG and AFP were unremarkable. Cerebrospinal fluid tests did not show significant findings and the cytology was negative for malignant cells. Therefore, a decision was made for neuronavigation-guided tissue biopsy for diagnostic purposes.

Histopathological examination revealed a mixed population of lymphocytes and large cells that have centrally placed nuclei, prominent nucleoli and abundant vacuolated cytoplasm (Figure 2). The tumor cells stained strongly for placental alkaline phosphatase (PLAP), but were negative for CD117, CD30 and alfa-fetoprotein (AFP). With these findings, the patient was diagnosed with pineal germinoma. MRI of the spine and computed tomography (CT) of the chest, abdomen and pelvis showed no evidence of metastasis.

Figure 1. Brain MRI with contrast showed a heterogeneous, irregular-shaped central mass with marked enhancement in the pineal region (left). Repeat imaging following radiotherapy showed significant interval regression (right).

Figure 2. Histopathology of the brain lesion showed a mixed population of lymphocytes and large cells that have centrally placed nuclei, prominent nucleoli and abundant vacuolated cytoplasm(A). The tumor cells stained strongly for placental alkaline phosphatase (B).

The patient's hospital course was complicated by the development of some nausea and vomiting that was associated with gradually worsening mental status requiring intubation and transfer to the neurological intensive care unit. Repeat MRI brain revealed interval progression of the hydrocephalus, with a maximum bifrontal diameter reaching 4.1 cm. Neurosurgery was consulted and ventriculoperitoneal (VP) shunt was inserted with significant improvement in his conscious level. The patient was discharged home in a stable condition with a plan for outpatient radiation therapy that was based on a decision after a multidisciplinary meeting. Upon follow up after radiation, the tumor showed significant interval regression with marked improvement of mass effect leading to near resolution of his clinical manifestations except hearing loss which had persisted. Debulking surgery was not considered as it was felt unlikely to resolve the deafness issue given the chronic nature of the problem besides the excellent sensitivity of the tumor to radiation.

3. Discussion

Malignant germ cell tumors (GCTs) are usually found in the gonads (ovary and testis). Around 2% to 5% of these tumors can occur on the midline body structures and involve any part from the pineal region to the coccyx [4]. Central nervous system is considered the second most common location after the mediastinum for extragonadal GCTs, with the majority being pure germinomas [4,5]. In decreasing order of frequency, germinomas typically occur in the pineal recess (50%-65%), suprasellar region (25%-35%), and basal ganglia/thalamus (5%-10%). Although involvement of the pineal region, as seen in this case, by germinomatous GCTs is common, the tumors in this region are very rare and make up less than 1% of all intracranial neoplasms in children and young adults.

The clinical presentation of pineal germinomas is usually related to increased intracranial pressure and hydrocephalus and can include headaches, visual changes, confusion, nausea and vomiting. Neuro-ophthalmologic findings, classically Parinaud's syndrome, is a common presentation of such tumors due to the compressive effects on the dorsal midbrain [6]. All these manifestations were found in our patient, but he also showed hearing loss which is far less commonly reported in the literature. Moreover, it seemed to be the initial complaint in this case which had been going on for several years with no appropriate work up including brain imaging until late referral to our hospital. This symptom is explained by the proximity of the pineal region to the auditory pathways in midbrain, upper brainstem and diencephalon [7]. It is crucial to consider intracranial imaging when dealing with hearing loss and consider pineal germinoma among the differential diagnosis [7,8] even if the classical symptoms were lacking. Early surgical intervention can reverse hearing loss in some cases.

The best imaging modality for pineal germinoma is brain MRI with contrast which typically shows hyperintense mass with cystic and necrotic changes that markedly enhances following intravenous contrast administration [9,10]. MRI of the whole spine is also recommended in addition to lumbar puncture to evaluate for CSF seeding and drop metastases [3,9]. Measurement of tumor markers, such as B-human chorionic gonadotropin (B-HCG) and alfa-fetoprotein (AFP) can be helpful in the diagnosis and follow up of such tumors as germinomas only secrete B-HCG and the levels of AFP in the serum and CSF are usually normal [11].

There are some tumors that can mimic pineal germinomas and these include pineal parenchymal tumors, such as pilocytic astrocytomas and high grade gliomas, and nongerminomatous GCTs including teratomas and embryonal carcinomas [3]. Tissue biopsy is an important tool that should be done for reaching to a definitive diagnosis of pineal germinoma given the significant differences in treatment options compared with other histological subtypes found in this region. Surgical resection does not have a major role in the management of these highly vascular tumors [11]. However, they are very sensitive to radiation treatment and radiation-alone approach can provide cure with a five-year overall survival greater than 85% [11,12,13]. The main disadvantage associated with this approach is the increased risk of long-term complications associated with high radiation doses and volumes. For this reason, multimodality approach consisting of neoadjuvant chemotherapy followed by lower doses and volumes of radiation might be suggested to minimize radiation toxicity especially for metastatic disease and relapsed cases [11].

4. Conclusion

Pineal germinomas are very rare and represent less than 1% of intrancranial tumors. Similar cases have been rarely reported in the literature. The presence of hearing loss could be the only initial symptom which should alert the physician to the importance of considering pineal tumors in the differential diagnosis. Clinical work up usually requires MRI brain and spine, tumor markers measurement, lumbar puncture and tissue biopsy. The current standard of treatment is radiation treatment, with or without neoadjuvant chemotherapy, which provides long-term control of the disease.

References

[1]   | Goodwin TL, Sainani K, Fisher PG. Incidence patterns of central nervous system germ cell tumors: a SEER Study. Journal of pediatric hematology/oncology. 2009; 31(8): 541-544.
[2]   | Ferla S, Spartà S, Giordano R, Zorat PL, Marin G, Meneghetti G. Pineal germinoma: diagnosis, treatment and tumor response. Ital J Neurol Sci. 1987; 8(3): 267-270.
[3]   | Manoj P. Reddy, MS, Amin F. Saad, MD, Kyle E. Doughty, MD, Danielle Armstrong, DO, Isaac Melguizo-Gavilanes, MD, Brennen S. Cheek, MD, and Michael J. Opatowsky, MD, MBA. Intracranial germinoma. Proc (Bayl Univ Med Cent). 2015 Jan; 28(1): 43-45.
[4]   | Yuping Gao, Jiyao Jiang, Qiang Liu. Extragonadal malignant germ cell tumors: a clinicopathological and immunohistochemical analysis of 48 cases at a single Chinese institution. Int J Clin Exp Pathol 2015; 8(5): 5650-5657.
[5]   | Albany C, Einhorn LH. Extragonadal germ cell tumors: clinical presentation and management. Curr Opin Oncol 2013; 25: 261-265.
[6]   | K Ahmad, S Ansari, K Dhungel, MK Gupta, RK Rauniyar, N Mishra Department of Radiodiagnosis, B.P. Koirala Institute of Health Sciences, Dharan, Nepal. Pineal germinoma presenting with Parinaud’s syndrome. Health Renaissance 2013; 11(3): 276-278.
[7]   | Bader N. Kashlan, Jordan Chick, Osama N. Kashlan, Suresh Ramnath. Hearing Impairment in Pineal Tumors. Journal of Nature and Science, Vol.1, No.3, e48, 2015.
[8]   | Gaspar, N., et al., Reversible hearing loss associated with a malignant pineal germ cell tumor. Case report. J Neurosurg, 2003. 99(3): 587-90.
[9]   | Dumrongpisutikul N, Intrapiromkul J, Yousem DM. Distinguishing between germinomas and pineal cell tumors on MR imaging. AJNR Am J Neuroradiol. 2012; 33(3): 550-555.
[10]   | Smith AB, Rushing EJ, Smirniotopoulos JG. From the archives of the AFIP: lesions of the pineal region: radiologic-pathologic correlation. Radiographics. 2010; 30(7): 2001-2020.
[11]   | Diana S Osorio and Jeffrey C Allen. Management of CNS germinoma. CNS Oncol. 2015 Jul; 4(4): 273-279.
[12]   | Bamberg M, Kortmann RD, Calaminus G, et al. Radiation therapy for intracranial germinoma: results of the German cooperative prospective trials MAKEI 83/86/89. J. Clin. Oncol. 1999; 17(8): 2585-2592.
[13]   | Rogers SJ, Mosleh-Shirazi MA, Saran FH. Radiotherapy of localised intracranial germinoma: time to sever historical ties? Lancet Oncol. 2005; 6(7): 509-519.