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Surgical Neurology International 2023Intracranial germinomas are rare tumors, accounting for 0.5-2% of primary intracranial neoplasms. While they typically occur in the pineal gland, suprasellar region,...
BACKGROUND
Intracranial germinomas are rare tumors, accounting for 0.5-2% of primary intracranial neoplasms. While they typically occur in the pineal gland, suprasellar region, basal ganglia, and thalamus, germinomas arising in the medulla oblongata are exceptionally rare. Diagnosis of medulla oblongata germinoma is challenging, potentially leading to misdiagnosis and poor prognosis.
CASE DESCRIPTION
We present a case of a 29-year-old man complaining of left leg numbness. Radiological findings revealed a contrast-enhanced lesion in the medulla oblongata. The patient underwent tumor biopsy, and intraoperative pathological diagnosis (IOD) suspected the diagnosis of medulla oblongata germinoma. He underwent chemoradiotherapy after confirming the diagnosis of germinoma. Intracranial germinoma arising in the medulla oblongata differs from germinomas in other locations due to its higher incidence in individuals in their 20s and a slight female predominance.
CONCLUSION
When encountering lesions in the medulla oblongata, germinoma should be considered as one of the differential diagnoses, and surgical strategies including IOD should be planned accordingly.
PubMed: 37941640
DOI: 10.25259/SNI_682_2023 -
Medicine Aug 2019Tumors of the pineal region are rare, and metastatic carcinoma occurring in the pineal region is extremely rare. No previous reports have described pineal region...
INTRODUCTION
Tumors of the pineal region are rare, and metastatic carcinoma occurring in the pineal region is extremely rare. No previous reports have described pineal region metastasis with intraventricular seeding.
PATIENT CONCERNS
We report a case of a 51-year-old woman presented with a 1-week history of severe headache, nausea, and vomiting. Imaging examination revealed 2 lesions in the pineal region and the right lateral ventricle.
DIAGNOSIS
Pinealocytoma or germinoma was considered as the preoperative diagnosis. The postoperative pathological diagnosis was small cell neuroendocrine carcinoma. After bronchoscopic biopsy, small cell lung cancer was confirmed.
INTERVENTIONS
A right frontal craniotomy and a translateral ventricle approach were performed to remove 2 lesions completely. And regular radiotherapy and chemotherapy were initiated after surgery.
OUTCOMES
The patient was discharged from the hospital 2 weeks after operation and went to another cancer hospital for bronchoscopic biopsy, radiotherapy, and chemotherapy. Finally, the patient died 2 years after surgical treatment.
CONCLUSION
Metastatic tumors of the pineal region are very rare. For patients with pineal lesions, a diagnosis of a metastatic tumor should be considered. Retrograde cerebrospinal fluid circulation might be the reason for a secondary metastasis.
Topics: Diagnosis, Differential; Female; Humans; Lung Neoplasms; Middle Aged; Neoplasm Metastasis; Pinealoma; Small Cell Lung Carcinoma
PubMed: 31441839
DOI: 10.1097/MD.0000000000016652 -
Journal of Neurosurgery. Case Lessons Apr 2021Germinoma is the most common type of germ cell tumor that develops intracranially. Germinomas usually grow in the midline structures, such as the pineal and suprasellar...
BACKGROUND
Germinoma is the most common type of germ cell tumor that develops intracranially. Germinomas usually grow in the midline structures, such as the pineal and suprasellar regions, and are rarely found in other locations. To the best of the authors' knowledge, no previous research has reported on growth of this tumor in the cranial vault.
OBSERVATIONS
The authors reported an unusual case of primary cranial vault germinoma in a young adult. Macroscopically, the tumor had a solid-soft consistency and grayish color with brownish spots on the surface. The histological examination revealed anaplastic cells with round, hyperchromatic, pleomorphic nuclei; prominent nucleoli; and abundant, clear cytoplasm, arranged in lobules and sheets that were infiltrated by lymphocytes and separated by fibrous connective tissue. These findings were consistent with the histopathological characteristics of germinoma.
LESSONS
Primary cranial vault germinoma is a unique tumor because no previous research has reported any growth in that location. It should be considered one of the differential diagnoses of lesions located over the cranial vault. Histopathological examination is still the primary modality for diagnosing these tumors and excluding other differential diagnoses.
PubMed: 35855389
DOI: 10.3171/CASE2082 -
American Journal of Ophthalmology Case... Sep 2018To report a rare case of bilateral periphlebitis associated with a pineal germinoma.
PURPOSE
To report a rare case of bilateral periphlebitis associated with a pineal germinoma.
OBSERVATIONS
A 17-year-old male teenager presented at a local clinic complaining of blurred vision in both eyes. The treating physician identified bilateral uveitis, and prescribed the patient with a local steroid treatment. However, the inflammatory findings did not improve with the treatment, and the patient was referred to our hospital for further examination. At the first visit, his best-corrected visual acuities were 0.3 for the right eye and 0.06 for the left eye; we found no inflammation in the anterior ocular segment, but observed bilateral retinal periphlebitis and a proliferative membrane from the papilla to the macula in the ocular fundus. In addition, we found a tractional serous retinal detachment in the macula. We suspected tuberculous uveitis clinically and initiated treatment with an antituberculous drug. However, the condition of the patient did not improve. Two months after our initial examination, left optic neuritis appeared, and we initiated a steroid pulse therapy. Although the periphlebitis remained, the left optic nerve findings and the visual acuity of both eyes improved. Thus, we reduced the oral steroid dose gradually. However, two months after initiating the dose reduction, the patient suffered a consciousness disturbance, and we detected a pineal tumor by magnetic resonance imaging (MRI). The patient was diagnosed as having a germ cell tumor by pathological examination and underwent radiation and chemotherapy. We noted marked improvements in both the periphlebitis findings and in the visual acuity following the treatment for the pineal tumor.
CONCLUSIONS AND IMPORTANCE
Cases of pineal tumor accompanied with retinal periphlebitis have been reported rarely. Because juvenile retinal vasculitis cases of unknown cause can be associated with pineal germinomas, we recommend brain MRI examinations for such cases.
PubMed: 30094391
DOI: 10.1016/j.ajoc.2018.07.003 -
Chinese Neurosurgical Journal Jan 2023The goal of this study is to show the feasibility and benefits of using the simultaneous biportal endoscopic procedure to treat pineal tumors in patients with...
BACKGROUND
The goal of this study is to show the feasibility and benefits of using the simultaneous biportal endoscopic procedure to treat pineal tumors in patients with obstructive hydrocephalus.
METHODS
We retrospectively reviewed three patients with pineal tumors and acute obstructive hydrocephalus who were treated in one session with a frameless stereotactic guided simultaneous biportal endoscopic third ventriculostomy and endoscopic tumor biopsy performed through two separate ports using one rigid ventriculoscope.
RESULTS
In the three patients, ventriculostomy and endoscopic biopsies were conducted. There was no death or morbidity throughout the 45-min procedure. All of the patients' histological findings were confirmed. Germinoma was diagnosed in two patients who recieved postoperative radiotherapy, and the third patient diagnosed with a pineocytoma. Magnetic resonance imaging with flow-sensitive sequences was used to confirm ventriculostomy patency in all patients 6 months after the surgery.
CONCLUSION
Biportal endoscopic approach enables better visual control of both procedures. Furthermore, it allows the surgeon to safely pass the ventriculoscope via the foramen of monro, even if it is narrow. Moreover, during endoscopic tumor biopsy and third ventriculostomy, the intracranial pressure can be smoothly managed using the outlet tubes accessible. This treatment may be an alternative to traditional uniportal endoscopic operations in certain patients.
PubMed: 36624512
DOI: 10.1186/s41016-022-00313-0 -
Cureus Apr 2016Primary intracranial germ cell tumors are rare, representing less than 5% of all central nervous system tumors. Overall, the majority of germ cell tumors are germinomas... (Review)
Review
Primary intracranial germ cell tumors are rare, representing less than 5% of all central nervous system tumors. Overall, the majority of germ cell tumors are germinomas and approximately one-third are non-germinomatous germ cell tumors (NGGCT), which include teratoma, embryonal carcinoma, yolk sac tumorĀ (endodermal sinus tumor), choriocarcinoma, or mixed malignant germ cell tumor. Germ cell tumors may secrete detectable levels of proteins into the blood and/or cerebrospinal fluid, and these proteins can be used for diagnostic purposes or to monitor tumor recurrence. Germinomas have long been known to be highly curable with radiation therapy alone. However, many late effects of whole brain or craniospinal irradiation have been well documented. Strategies have been developed to reduce the dose and volume of radiation therapy, often in combination with chemotherapy. In contrast, patients with NGGCT have a poorer prognosis, with about 60% cured with multimodality chemoradiation. There are no standard approaches for relapsed germ cell tumors. Options may be limited by prior treatment. Radiation therapy has been utilized alone or in combination with chemotherapy or high-dose chemotherapy and transplant. We discuss two cases and review options for frameless radiosurgery or fractionated radiotherapy.
PubMed: 27239400
DOI: 10.7759/cureus.585 -
Caspian Journal of Internal Medicine 2021Germinoma is a rare lesion found commonly in the pineal and suprasellar regions of the brain. Clinical presentation mainly involves the location and size of the tumor...
BACKGROUND
Germinoma is a rare lesion found commonly in the pineal and suprasellar regions of the brain. Clinical presentation mainly involves the location and size of the tumor and the patient age. Endocrine abnormalities are the most common symptom.
CASE PRESENTATION
The patient was a 26-year-old Iranian female who suffered from germinoma for a long time and was referred to Loghman Hakim Hospital for amenorrhea, polyuria, and polydipsia. Despite diagnostic challenges, she was finally diagnosed with suprasellar germinoma after endoscopic transsphenoidal surgery, followed by radiotherapy and medical interventions to complement the surgery.
CONCLUSION
It is important to be able to diagnose the patient's problem at an early stage based on their history, hormonal profile, laboratory results and radiological view.
PubMed: 34760091
DOI: 10.22088/cjim.12.0.397 -
Case Reports in Oncology 2024Intracranial germinomas mainly arise in the pineal gland or neurohypophyseal region. The basal ganglia have been reported as the site of occurrence for ectopic...
INTRODUCTION
Intracranial germinomas mainly arise in the pineal gland or neurohypophyseal region. The basal ganglia have been reported as the site of occurrence for ectopic germinomas, whereas other sites have been rarely described. We experienced a case of multifocal ectopic germinoma that arose in the septum pellucidum and the dorsal brain stem, not including the pineal gland, neurohypophysis, and basal ganglia of ectopic germinoma in a pregnant woman.
CASE PRESENTATION
The patient initially presented to our institution with complaints of diplopia in the past 14 weeks of gestation, and imaging later revealed two intracranial neoplastic lesions, with one lesion involving the septum pellucidum and the other involving the dorsal brainstem. Both tumors were partially excised via a transcortical approach. Based on the results of pathology and immunohistochemistry, the patient was diagnosed with germinoma, and the intraoperative spinal fluid cytology was class V in Papanicolaou classification. The patient received three courses of ifosfamide, carboplatin, and etoposide together with whole-brain irradiation, which resulted in complete elimination of the tumors. No evidence of recurrence was identified after 18 months.
CONCLUSION
The results illustrated the need to consider germinoma in the differential diagnosis if the lesions involve midline structures such as the septum pellucidum or dorsal brainstem. Given the rarity of germinoma arising outside the pineal gland and neurohypophyseal region, these findings provide key insights into the diagnosis and treatment of this disease.
PubMed: 38549623
DOI: 10.1159/000538088 -
Neuro-oncology Dec 2019We integrated clinical, histopathological, and molecular data of central nervous system germ cell tumors to provide insights into their management.
BACKGROUND
We integrated clinical, histopathological, and molecular data of central nervous system germ cell tumors to provide insights into their management.
METHODS
Data from the Intracranial Germ Cell Tumor Genome Analysis (iGCT) Consortium were reviewed. A total of 190 cases were classified as primary germ cell tumors (GCTs) based on central pathological reviews.
RESULTS
All but one of the cases that were bifocal (neurohypophysis and pineal glands) and cases with multiple lesions including neurohypophysis or pineal gland were germinomas (34 of 35). Age was significantly higher in patients with germinoma than other histologies. Comparison between tumor marker and histopathological diagnoses showed that 18.2% of histopathologically diagnosed germinomas were marker positive and 6.1% of non-germinomatous GCTs were marker negative, suggesting a limitation in the utility of markers or histopathology alone using small specimens for diagnosis. Comparison between local and central histopathological diagnoses revealed a discordance of 12.7%. Discordance was significantly less frequent in biopsy cases, implying difficulty in detecting all histopathological components of heterogeneous GCTs. Germinomas at the typical sites (neurohypophysis or pineal gland) showed a better progression-free survival than those at atypical sites (P = 0.03). A molecular clinical association study revealed frequent mitogen-activated protein kinase (MAPK) pathway mutations in males (51.4% vs 14.3%, P = 0.007), and phosphatidylinositol-3 kinase/mammalian target of rapamycin (PI3K/mTOR) pathway mutations in basal ganglia cases (P = 0.004). Basal ganglia cases also had frequent chromosomal losses. Some chromosomal aberrations (2q, 8q gain, 5q, 9p/q, 13q, 15q loss) showed potential prognostic significance.
CONCLUSIONS
The in-depth findings of this study regarding clinical and molecular heterogeneity will increase our understanding of the pathogenesis of this enigmatic tumor.
Topics: Adolescent; Adult; Biomarkers, Tumor; Central Nervous System Neoplasms; Child; Combined Modality Therapy; Data Analysis; Female; Follow-Up Studies; Humans; Male; Middle Aged; Neoplasms, Germ Cell and Embryonal; Prognosis; Retrospective Studies; Survival Rate; Young Adult
PubMed: 31420671
DOI: 10.1093/neuonc/noz139 -
Oncology Letters Aug 2015Intracranial germinomas (IG) are rare tumors that typically occur in the pineal gland and suprasellar region of the brain. IG can be successfully treated with radiation...
Intracranial germinomas (IG) are rare tumors that typically occur in the pineal gland and suprasellar region of the brain. IG can be successfully treated with radiation therapy and its recurrence is rare. The present study reports the case of a patient with IG involving the pineal and suprasellar regions. The patient was treated with radiotherapy, achieving significant remission. However, recurrence was discovered 10 months later. The patient was subsequently treated with chemotherapy and radiotherapy, resulting in gradual remission. The patient had recovered 2 months later and was in good health. The entire evolution process, including magnetic resonance (MR) imaging and MR spectroscopy findings, are described herein. The present case suggests that MR spectroscopy may be useful for monitoring the effects of treatment.
PubMed: 26622651
DOI: 10.3892/ol.2015.3351