- Department of Neurosurgery, Hospital São Francisco, Ribeirão Preto, São Paulo, Brazil
- Department of Internal Medicine, Hospital São Francisco, Ribeirão Preto, São Paulo, Brazil
- Department of Documenta Advanced Radiological Center, Hospital São Francisco, Ribeirão Preto, São Paulo, Brazil
- Department of Surgery and Anatomy, Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, Brazil
Department of Surgery and Anatomy, Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, Brazil
DOI:10.4103/sni.sni_387_18Copyright: © 2019 Surgical Neurology International This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.
How to cite this article: Breno Nery, Fred Bernardes Filho, Rodrigo Antônio Fernandes Costa, Leandro César Tângari Pereira, Eduardo Quaggio, Rodolfo Mendes Queiroz, Lucas Giansante Abud, Daniela Pretti da Cunha Tirapelli. Neurocytoma mimicking macroadenoma. 21-Jan-2019;10:8
How to cite this URL: Breno Nery, Fred Bernardes Filho, Rodrigo Antônio Fernandes Costa, Leandro César Tângari Pereira, Eduardo Quaggio, Rodolfo Mendes Queiroz, Lucas Giansante Abud, Daniela Pretti da Cunha Tirapelli. Neurocytoma mimicking macroadenoma. 21-Jan-2019;10:8. Available from: http://surgicalneurologyint.com/surgicalint-articles/9162/
Background:Intraventricular and extraventricular central neurocytomas (CN) are equally frequent among 20–40-year-old men and women. However, sellar and suprasellar extraventricular CN are extremely rare, with only 12 reported cases.
Case Description:The authors report the case of a Brazilian 27-year-old man who presented with progressive vision loss during the last 4 years and serious bilateral keratoconus. We also review the epidemiological, clinical, radiological, pathological, and treatment features of the 12 reported cases. The patient developed left amaurosis and right temporal hemianopsia after undergoing bilateral corneal transplantation, which was detected during campimetry testing, and subsequently underwent magnetic resonance imaging, which revealed a huge hypophyseal tumor. Endocrinological evaluation revealed complete loss of pituitary function. The patient was referred to our department and underwent a two-step surgery (using transsphenoidal approach and cranio-orbital zygomatic approach) based on the diagnosis of an extraventricular central nervous system neurocytoma. Tumor removal was successful, and the patient was discharged at 3 weeks after admission to our department.
Conclusion:Although extraventricular neurocytomas of the brain are rare, careful preoperative consideration of its anatomy, pathophysiological features, and radiological features can enhance the treatment outcomes.
Keywords: Central nervous system, central nervous system neoplasms, central neurocytomas, neurocytoma
Central neurocytomas (CN) are neuronal neoplasms with little aggressive behavior, which were initially described by Hassoun et al. in 1982 as an intraventricular tumor exhibiting histological similarities to oligodendrogliomas.[
A Brazilian 27-year-old man presented with a 4-year history of progressive bilateral vision loss caused by serious keratoconus. A neuro-ophthalmological evaluation revealed near-total left-eye amaurosis and moderate right-eye vision loss. The patient subsequently underwent bilateral corneal transplantation, although his condition progressed to left amaurosis and right temporal hemianopsia during the following 2 months. Thus, he underwent magnetic resonance imaging (MRI), which revealed a 79-cm3 bulky solid expansive lesion that involved the sellar and suprasellar compartments. The lesion had well-defined lobulated margins and a heterogeneous appearance without cystic or calcified components. There was no edema in the adjacent brain parenchyma. The imaging also revealed invasion of the sellar floor, lateral displacement of both internal carotid arteries, medial obliteration of the cavernous sinus, and extrinsic compression of the optic chiasm, hypothalamus, and third ventricle [
We initially selected the endoscopic endonasal approach, which revealed that the tumor was unexpectedly firm. We then sent a specimen for intraoperative pathological analysis. The results were inconclusive for macroadenoma, and we chose to stop the surgery until we received a conclusive result (approximately 60% of the tumor had been removed at that point). The patient did not experience any postoperative deficits during the 8 days until we received conclusive pathological diagnosis of CN without signs of malignancy. Immunohistochemical analysis revealed positive synaptophysin expression, a Ki-67 index of 0.8%, and negative AE1/AE3 expression [
Histological sections show solid neoplastic cell proliferation, as well as noncohesive monomorphic small cells with round nuclei, fine chromatin, and well-distributed micronucleoli. The cytoplasm is poorly defined and a fine smooth granular vascular network is observed in the absence of necrosis or mitotic figures. Left: Hematoxylin and eosin staining (×400). Right: Immunohistochemical analysis revealed positive synaptophysin expression, a Ki-67 index of 0.8%, and negative AE1/AE3 expression (×400). Color versions are available as an online supplementary resource
The patient was successfully treated for postoperative insipidus diabetes during a 1-week stay in the intensive care unit. He was subsequently discharged at 3 weeks after his admission to our department without any new neurological deficits and with improvement of the right-eye hemianopsia. Computed tomography (CT) at 1 week after the surgery revealed gross total resection of the tumor and mild ventricular dilation, without any manifestations of hypertensive hydrocephalus that required further treatment [
Four-month follow-up axial (a) and sagittal results (b) from T1-weighted magnetic resonance imaging revealed a hyperintense sellar mass, which was compatible with an autologous fat graft that was used to prevent nasal fistula. Findings from T1-weighted gadolinium-enhanced magnetic resonance imaging after 4 months (c) and 18 months (d)
Intraventricular and extraventricular CN are equally frequent in 20–40-year-old men and women.[
The CN lesions can be grade III malignant variants (20–27%), which occur more often in older patients and typically exhibit greater infiltrative behavior, metastatic behavior, and unresectability. These lesions also frequently exhibit atypical cells, marked mitosis, vascular proliferation, necrosis, an MIB1 index of >2%, and a Ki-67 index of >2%.[
Sellar and suprasellar extraventricular CN are extremely rare, and only 12 reported cases[
The reported cases were assessed using CT (66.7%) and/or MRI (100%), which revealed that the tumors were generally solid, bulky (a major axis of ≥3.0 cm), homogeneous, well-defined, and with limited or no swelling in the adjacent parenchyma. Two cases involved small-scattered calcifications, and four cases involved cystic components. All 10 patients with published imaging findings had invasion of the cavernous sinus. The CT scans commonly revealed discreetly iso/hyperdense tumors, and discrete iso/hyperintense findings were observed on approximately 58% of the T1- and T2-weighted images. All reported cases exhibited enhancement after the administration of an iodinated or paramagnetic intravenous contrast agent, with one-half of the results being prominent and nine cases exhibiting heterogeneous enhancement. Ten cases had detailed histological and immunohistochemical data, with generally positive results for synaptophysin expression (9/10 cases) and generally negative results for GFAP expression (8/10 cases). Two patients exhibited signs of malignancy (48 years and 64 years old), with MIB1 indexes of 3% and 4–10%, respectively. Only two patients had gross total tumor resection and eight patients had only partial resection, with 75% of those patients undergoing postoperative radiotherapy. Two patients with atypical extraventricular CN developed distant dural metastases or local recurrences. The main differential diagnosis was pituitary adenoma, especially the nonfunctioning type, which is usually isodense during CT, rarely contains calcification (5–7%) or hemorrhagic foci, and usually exhibits variable hypo/isointense signals during T1- and T2-weighted MRI. Furthermore, pituitary adenoma usually exhibits intense enhancement, but this is slightly slower than the rest of the pituitary gland.[
In conclusion, we encountered a rare case of an extraventricular CN in the sellar/suprasellar region. Given the unusual nature of this case, we examined the 12 other reported cases and attempted to identify the features and signs that may help facilitate the diagnosis of these tumors in this region. Furthermore, we reported common histopathological characteristics and surgical approaches from those cases. We strongly believe that total tumor resection must be the primary goal of the surgery, and that combined surgical approaches can help facilitate this outcome.
Availability of data and materials
BN, LCTP, RAFC, RMQ, LGA, EQ, LHC, ISTP, CHM, and FBF contributed to conception and design as well as the acquisition, analysis, and interpretation of data. RMQ, LGA, ISTP, and CHM contributed to acquisition and interpretation of imaging data. BN, LCTP, RAFC, RMQ, LGA, EQ, LHC, ISTP, CHM, and FBF documented the patient's status and contributed to analysis and interpretation of data. All authors read and approved the final manuscript.
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Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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