Limits...
Dyspnea and choking as presenting symptoms in primary medulla oblongata germinoma.

Yip CM, Tseng HH, Hsu SS, Liao WC, Chen JY, Chen CH, Chang CY - Surg Neurol Int (2014)

Bottom Line: The latest brain MRI and whole spine MRI done 1 year after surgery showed neither residual nor recurrent tumor in the whole axis.Intracranial germ cell tumors originate from extragonadal seminal cells and have been found in 0.4-3.4% of patients with primary central nervous system (CNS) tumors in Western countries, while the incidence is reported to be 5-8 times greater in Japan and the Far East.Although germinoma of medulla oblongata is rare and difficult to diagnose preoperatively, it should be included in the differential diagnosis of medulla masses with fourth ventricle extension, especially in Asian population.

View Article: PubMed Central - PubMed

Affiliation: Division of Neurosurgery, Kaohsiung Veterans General Hospital, Kaohsiung, Taiwan, R. O. C.

ABSTRACT

Background: The medulla oblongata is the lower half of the brainstem. It contains the cardiac, respiratory, vomiting, and vasomotor centers and deals with autonomic functions such as breathing, heartbeat, and blood pressure. Primary medulla oblongata germinoma is very rare and less than 20 cases have been reported in the English literature.

Case description: A 22-year-old female without any particular past medical history presented to us in October 2012 with the chief complaint of dyspnea and frequent choking for 1 month. Neurological examination revealed lower cranial nerve palsies and nystagmus. Her brain computed tomography (CT) and brain magnetic resonance imaging (MRI) demonstrated a mass lesion at the dorsal surface of medulla oblongata with extension into the inferior fourth ventricle and foramen magnum. She underwent bilateral suboccipital craniotomy and C1 laminoplasty with the grossly total resection of the tumor. The histological examination of the tumor proved germinoma. Postoperative adjuvant radiotherapy was arranged. The latest brain MRI and whole spine MRI done 1 year after surgery showed neither residual nor recurrent tumor in the whole axis. She is regularly followed-up at our outpatient department and is doing well except having left vocal cord palsy, which occurred before surgery.

Conclusion: Medulloblastoma, ependymoma, glioma, hemangioblastoma, and cavernous angioma are common intraaxial tumors in the medulla oblongata and fourth ventricle. Intracranial germ cell tumors originate from extragonadal seminal cells and have been found in 0.4-3.4% of patients with primary central nervous system (CNS) tumors in Western countries, while the incidence is reported to be 5-8 times greater in Japan and the Far East. Although germinoma of medulla oblongata is rare and difficult to diagnose preoperatively, it should be included in the differential diagnosis of medulla masses with fourth ventricle extension, especially in Asian population.

No MeSH data available.


Related in: MedlinePlus

Immediate postoperative brain MRI. T1-weighted postcontrast image, sagittal (a), axial (b), coronal (c) views showed the completed removal of the tumor in the inferior portion of fourth ventricle and foramen magnum
© Copyright Policy - open-access
Related In: Results  -  Collection

License
getmorefigures.php?uid=PMC4109171&req=5

Figure 4: Immediate postoperative brain MRI. T1-weighted postcontrast image, sagittal (a), axial (b), coronal (c) views showed the completed removal of the tumor in the inferior portion of fourth ventricle and foramen magnum

Mentions: Under general anesthesia, she was put in prone position and underwent a bilateral suboccipital craniotomy and C1 laminoplasty with the removal of the tumor. A midline longitudinal incision was made from external occipital proturbance to the spinal process of C2, then a Y shape incision was made on the exposed dura of cerebellum and upper cervical spinal cord. Reflected the dural flaps to expose the cerebellar hemisphere and upper cervical cord. After the arachnoid membrane was opened and the cerebrospinal fluid (CSF) was drained, bilateral tonsils and the superficial part of the tumor was exposed. Grossly, the tumor was soft and gray-red [Figure 2], quite circumscribed but the margin between the tumor and the dorsal side of medulla was not very clear. Internal decompression of the tumor was done first to gain more working space, then dissected the tumor from the neighboring structures with extreme caution and finally this tumor was removed in piece-meal fashion with the aid of Cavitron Ultrasonic Surgical Aspirator (CUSA). Intraoperative frozen section of the specimen reported “malignant tumor”, therefore, we tried to achieve maximum tumor resection. Histology examination showed that the specimen composed of sheets of large anaplastic cells divided by delicate fibrovascular septa with small lymphocytes [Figure 3a]. Mitosis and necrosis were present. The immunohistochemistry results of neoplastic cells revealed positive for CD117 and placenta alkaline phosphatase [Figure 3b], but negative for CD3, CD20, and synaptophysin immunostains. Germinoma was diagnosed based on the morphology of the tumor cells and the result of immunohistochemical stains. Based on the histological diagnosis of the tumor, whole spine MRI was checked, which disclosed no evidence of abnormal enhancing mass lesion. Although her immediate postoperative brain MRI showed no evidence of abnormal enhancing mass lesion, or abnormal leptomeningeal enhancement in the brain [Figure 4], we administered postoperative adjuvant radiotherapy as following dosage: Total 45 Gy on the tumor bed and total 30.6 Gy on the ventricle system. Chemotherapy was not recommended by the oncologist because the patient's α-fetoprotein (AFP) and human chorionic gonadotropin (β-HCG) levels were within normal limits, and there was no dissemination of the tumor cells.


Dyspnea and choking as presenting symptoms in primary medulla oblongata germinoma.

Yip CM, Tseng HH, Hsu SS, Liao WC, Chen JY, Chen CH, Chang CY - Surg Neurol Int (2014)

Immediate postoperative brain MRI. T1-weighted postcontrast image, sagittal (a), axial (b), coronal (c) views showed the completed removal of the tumor in the inferior portion of fourth ventricle and foramen magnum
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4109171&req=5

Figure 4: Immediate postoperative brain MRI. T1-weighted postcontrast image, sagittal (a), axial (b), coronal (c) views showed the completed removal of the tumor in the inferior portion of fourth ventricle and foramen magnum
Mentions: Under general anesthesia, she was put in prone position and underwent a bilateral suboccipital craniotomy and C1 laminoplasty with the removal of the tumor. A midline longitudinal incision was made from external occipital proturbance to the spinal process of C2, then a Y shape incision was made on the exposed dura of cerebellum and upper cervical spinal cord. Reflected the dural flaps to expose the cerebellar hemisphere and upper cervical cord. After the arachnoid membrane was opened and the cerebrospinal fluid (CSF) was drained, bilateral tonsils and the superficial part of the tumor was exposed. Grossly, the tumor was soft and gray-red [Figure 2], quite circumscribed but the margin between the tumor and the dorsal side of medulla was not very clear. Internal decompression of the tumor was done first to gain more working space, then dissected the tumor from the neighboring structures with extreme caution and finally this tumor was removed in piece-meal fashion with the aid of Cavitron Ultrasonic Surgical Aspirator (CUSA). Intraoperative frozen section of the specimen reported “malignant tumor”, therefore, we tried to achieve maximum tumor resection. Histology examination showed that the specimen composed of sheets of large anaplastic cells divided by delicate fibrovascular septa with small lymphocytes [Figure 3a]. Mitosis and necrosis were present. The immunohistochemistry results of neoplastic cells revealed positive for CD117 and placenta alkaline phosphatase [Figure 3b], but negative for CD3, CD20, and synaptophysin immunostains. Germinoma was diagnosed based on the morphology of the tumor cells and the result of immunohistochemical stains. Based on the histological diagnosis of the tumor, whole spine MRI was checked, which disclosed no evidence of abnormal enhancing mass lesion. Although her immediate postoperative brain MRI showed no evidence of abnormal enhancing mass lesion, or abnormal leptomeningeal enhancement in the brain [Figure 4], we administered postoperative adjuvant radiotherapy as following dosage: Total 45 Gy on the tumor bed and total 30.6 Gy on the ventricle system. Chemotherapy was not recommended by the oncologist because the patient's α-fetoprotein (AFP) and human chorionic gonadotropin (β-HCG) levels were within normal limits, and there was no dissemination of the tumor cells.

Bottom Line: The latest brain MRI and whole spine MRI done 1 year after surgery showed neither residual nor recurrent tumor in the whole axis.Intracranial germ cell tumors originate from extragonadal seminal cells and have been found in 0.4-3.4% of patients with primary central nervous system (CNS) tumors in Western countries, while the incidence is reported to be 5-8 times greater in Japan and the Far East.Although germinoma of medulla oblongata is rare and difficult to diagnose preoperatively, it should be included in the differential diagnosis of medulla masses with fourth ventricle extension, especially in Asian population.

View Article: PubMed Central - PubMed

Affiliation: Division of Neurosurgery, Kaohsiung Veterans General Hospital, Kaohsiung, Taiwan, R. O. C.

ABSTRACT

Background: The medulla oblongata is the lower half of the brainstem. It contains the cardiac, respiratory, vomiting, and vasomotor centers and deals with autonomic functions such as breathing, heartbeat, and blood pressure. Primary medulla oblongata germinoma is very rare and less than 20 cases have been reported in the English literature.

Case description: A 22-year-old female without any particular past medical history presented to us in October 2012 with the chief complaint of dyspnea and frequent choking for 1 month. Neurological examination revealed lower cranial nerve palsies and nystagmus. Her brain computed tomography (CT) and brain magnetic resonance imaging (MRI) demonstrated a mass lesion at the dorsal surface of medulla oblongata with extension into the inferior fourth ventricle and foramen magnum. She underwent bilateral suboccipital craniotomy and C1 laminoplasty with the grossly total resection of the tumor. The histological examination of the tumor proved germinoma. Postoperative adjuvant radiotherapy was arranged. The latest brain MRI and whole spine MRI done 1 year after surgery showed neither residual nor recurrent tumor in the whole axis. She is regularly followed-up at our outpatient department and is doing well except having left vocal cord palsy, which occurred before surgery.

Conclusion: Medulloblastoma, ependymoma, glioma, hemangioblastoma, and cavernous angioma are common intraaxial tumors in the medulla oblongata and fourth ventricle. Intracranial germ cell tumors originate from extragonadal seminal cells and have been found in 0.4-3.4% of patients with primary central nervous system (CNS) tumors in Western countries, while the incidence is reported to be 5-8 times greater in Japan and the Far East. Although germinoma of medulla oblongata is rare and difficult to diagnose preoperatively, it should be included in the differential diagnosis of medulla masses with fourth ventricle extension, especially in Asian population.

No MeSH data available.


Related in: MedlinePlus