|Year : 2017 | Volume
| Issue : 4 | Page : 103-105
Case of hemangioblastoma and central neurocytoma presenting as synchronous primary brain tumors in a young man
Veronica De Los Santos, Federico Salle, Ramiro Lima, Edgardo Spagnuolo
Department of Neurosurgery, Maciel Hospital-ASSE, School of Medicine, University of the Republic, Montevideo, Uruguay
|Date of Submission||17-Apr-2017|
|Date of Acceptance||22-Aug-2017|
|Date of Web Publication||28-Dec-2017|
Rene 1581, Montevideo 11400
Source of Support: None, Conflict of Interest: None
This study presents a very infrequent case of a young man with two different brain tumors. The peculiarity of this case is that both tumors appeared at the same time. The patient was admitted with headaches and confusion. Computed tomography scan and magnetic resonance imaging confirmed two tumors, one in the posterior fossa and another in the intraventricular supratentorial space. These findings were initially interpreted as the same tumor with two different localizations, a very infrequent situation. A first surgery was performed to remove the posterior fossa tumor. The pathology confirmed an hemangioblastoma. Six months later, a second surgery was performed for the supratentorial tumor. The surgical aspect was totally different compared to the tumor of the posterior fossa, and the pathology confirmed a central neurocytoma. The authors made a bibliographic review and did not find references of this association.
Keywords: Brain tumors, central neurocytoma, hemangioblastoma, synchronous primary brain tumors
|How to cite this article:|
De Los Santos V, Salle F, Lima R, Spagnuolo E. Case of hemangioblastoma and central neurocytoma presenting as synchronous primary brain tumors in a young man. Transl Surg 2017;2:103-5
|How to cite this URL:|
De Los Santos V, Salle F, Lima R, Spagnuolo E. Case of hemangioblastoma and central neurocytoma presenting as synchronous primary brain tumors in a young man. Transl Surg [serial online] 2017 [cited 2019 May 25];2:103-5. Available from: http://www.translsurg.com/text.asp?2017/2/4/103/219375
| Introduction|| |
Multiple brain tumors are frequently found in metastatic disease. Multiple primitive and synchronous brain tumors are unusual. They can sometimes be seen as part of genetic systemic diseases, such as neurofibromatosis or von Hippel–Lindau (VHL) syndrome. The diagnosis of two different sporadic brain tumors at the same time is very infrequent, even more so in a healthy young man with no medical records or relevant family history, like in this case. The aim of this article is to present a rare case of two synchronous primary brain tumors: hemangioblastoma and central neurocytoma.
| Case Report|| |
A 26-year-old male, Caucasian, employee, with no medical records or family history of genetic or neoplastic diseases, was admitted to the emergency department because of persistent headaches and gait disturbances within the past 2 months. Mental confusion was noted some days before the admission. The magnetic resonance imaging (MRI) showed two tumors [Figure 1]. One of them was located in the midline of the posterior fossa, near the cerebellar vermis and the foramen magnum. The tumor had intense enhancement with contrast and clear limits. The second tumor was supratentorial, intraventricular, with less clear limits and less enhancement with contrast. A thoraco-abdominal computed tomography scan was performed searching for systemic disease, but there were no pathological findings. Twenty four hours after admission, the posterior fossa tumor was operated on through a median suboccipital approach (Ramiro Lima RL, who operated the patient the first time). It turned out to be an bleeding lesion, but easy to aspirate with an ultrasonic aspirator. There was severe bleeding during surgery and postoperative dysfunction of the lower cranial nerves with dysphagia, dysphonia, and ventilatory impairment. The patient needed a prolonged stay in critical care. Six months after that and once recovered, he was submitted to surgery of the intraventricular tumor (Edgardo Spagnuolo ES, who operated the patient the second time). A new MRI was performed before this surgery and it showed significant growth of the supratentorial tumor, with no remnant of the tumor in the posterior fossa [Figure 2]. An interhemispheric approach was chosen for the second surgery. An avascular, totally intraventricular, easy-to-aspirate tumor was found. Immediate postoperative MRI [Figure 3] showed total removal of the lesion with some bleeding in the surgical bed.
|Figure 1: Preoperative magnetic resonance imaging with posterior fossa and supratentorial tumors|
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|Figure 2: (a) Magnetic resonance imaging 5 months after initial surgery. Total removal of the posterior fossa tumor, growing supratentorial tumor. (b) Preoperative axial magnetic resonance imaging before the second surgery|
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|Figure 3: Postoperative magnetic resonance imaging after the second surgery, removal of the supratentorial lesion|
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The pathology report of the first surgery corresponded to a hemangioblastoma [Figure 4], while the report of the second surgery was concordant with a central neurocytoma [Figure 5]a. Positive synaptophysin immunolabeling [Figure 5]b and negative glial fibrillary acidic protein confirmed the diagnosis. The patient was finally submitted to chromosomal studies which ruled out VHL syndrome. Thus, the tumors were interpreted as sporadic.
|Figure 4: Histology of the posterior fossa tumor (H and E, ×25). More vascularized lesion, constituted by median caliber vessels with hyaline walls, associated with an important capillary network mixed with polyhedral cells with abundant cytoplasm, compatible with hemangioblastoma|
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|Figure 5: (a) Histology of the supratentorial tumor (H and E, ×40). A cellular proliferation with a solid pattern forming perivascular rosettes; cells with rounded nuclei and thin chromatin lumps, without mitoses, capillary vascular network and microcalcifications, probable central neurocytoma. (b) Histochemical study with positive antibody immunolabeling for synaptophysin, confirming neuronal differentiation as typically seen in central neurocytoma. Glial fibrillary acidic protein and Ki67 also studied (not shown)|
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The long-term outcome of the patient was good; he is conscious, without spinal deficit, although some signs of lower cranial nerve dysfunction persist at 1-year follow-up. He is under physical rehabilitation.
Informed consent of the patient as well as approval of the institutional bioethics committee was obtained before submission of this article.
| Discussion|| |
Multiple synchronous brain tumors are more common as a form of presentation of brain metastases., Multiple primary brain tumors have a low incidence. Multicentric glioma and tumors related to phakomatoses are the ones that usually can be seen., The simultaneous association of two different primary brain tumors is extremely rare, especially if there is no family history or genetic diseases., The patient we present here was diagnosed with a cerebellar hemangioblastoma and a supratentorial central neurocytoma in the lateral ventricle. Hemangioblastomas are infrequent tumors that account for 2% of all intracranial tumors. However, if we exclude the supratentorial compartment, they can reach up to 10% of all posterior fossa tumors. These are benign lesions, highly vascularized, that generally originate from stromal cells in the cerebellum. About 20%-25% of hemangioblastomas occur in VHL patients. In these cases, a characteristic chromosomal alteration can be detected and lesions in other organs, such as retinal angiomas, are generally associated. Although it is not frequent, other lesions in the central nervous system (CNS) can also be found. Hence, when a hemangioblastoma is diagnosed, the entire CNS must be studied. Intra- and extra-cranial metastases are rare but should be ruled out., In this particular case, once the posterior fossa hemangioblastoma was confirmed, we hypothesized that the supratentorial lesion was a secondary location of the same tumor. In the meantime between the first and the second surgeries, genome analysis of the patient was performed and VHL was discarded. The second pathology report (central neurocytoma) also discarded a possible VHL syndrome, as associations of hemangioblastomas with other histologically different tumors have not been described.
Central neurocytomas are even rarer than hemangioblastomas and represent only 0.5%–0.8% of all CNS tumors. They are benign and grow slowly, presenting mostly in young adults. They are usually intraventricular; extension outside the ventricular wall is exceptional. Their origin is in subependymal neuronal cells of the ventricular wall.
In the case of our patient, the symptomatic lesion was the one in the posterior fossa. The other tumor was incidentally found. Interestingly, it grew in 6 months, even outside the ventricular wall, which is rare for such a benign tumor. It is of note that both tumors turned out to be benign, so complete surgical excision can achieve a cure, which we think has been accomplished in this case.
This patient, therefore, had two synchronous tumors of different cellular origin. This association is very rare, except in patients suffering from phakomatoses such as von Recklinghausen's disease. This type of neurofibromatosis associates meningiomas, schwannomas, and sometimes gliomas.
The association of two low incidence tumors such as hemangioblastomas and central neurocytomas has not been described so far. The authors could not find any bibliographic references.
Even though this is a case report and lacks statistical value, we think it might help clinical practitioners keep in mind that synchronous primary tumors of the CNS can be sporadic and not always related to genetic diseases. In addition, when two tumors are diagnosed at the same time, they can correspond to different histological types and different therapeutic approaches might be needed.
In conclusion, the association of two pathologically different CNS tumors is very rare, except in some systemic diseases such as phakomatoses. The finding of two synchronous tumors, a posterior fossa hemangioblastoma and a supratentorial intraventricular central neurocytoma, has not been notified in literature so far. What is more, these are individually infrequent tumors that originate from different cellular types, arising in a patient with no genetic diseases. Therefore, when primary synchronous tumors are diagnosed in the CNS, one should not take for granted that they correspond to the same histological type.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]