|Year : 2018 | Volume
| Issue : 1 | Page : 34-39
Multiple primary intracranial tumors with diverse histological origin
Harish Naik, Vernon Velho
Department of Neurosurgery, JJ Group of Hospitals and Grant Medical College, Mumbai, Maharashtra, India
|Date of Submission||06-Jun-2018|
|Date of Acceptance||03-Sep-2018|
|Date of Web Publication||14-Nov-2018|
Dr. Harish Naik
Department of Neurosurgery, JJ Group of Hospitals and Grant Medical College, Mumbai - 400 008, Maharashtra
Source of Support: None, Conflict of Interest: None
Context: Most primary intracranial neoplasms occur as solitary lesions. Presence of multiple intracranial tumours with different histological origin in same individual or even collision tumours is exceedingly rare.
Aims: With the advancement in neuroimaging techniques, more such synchronous tumours are being identified. The most common combination identified till date is meningioma associated with glioma. Our understanding of multiple, dissimilar intracranial tumours will gradually improve as more cases are identified, reported and studied. The explanations for the simultaneous occurrence of different primary intracranial tumours in the absence of phacomatoses or prior radiation exposure are at present hypothetical, and these tumours could be simply coincidental.
Material and Methods: We present here a series of four cases who had multiple intracranial tumours of different histological origin and were managed successfully. None of our patients had received radiation in the past or had any family history of phakomatoses.
Conclusions: Simultaneous occurrence of histologically different primary braintumours is rare, and its preoperative diagnosis is still challenging. Syndromic association should be ruled out in patients of histologically different multiple brain tumours. Most common primary brain tumour associated with other histologically different brain tumours is meningioma. Thorough clinical and imaging studies are mandatory in these cases to provide optimal treatment.
Keywords: Collison tumours, different histological origin, multiple intracranial tumours
|How to cite this article:|
Naik H, Velho V. Multiple primary intracranial tumors with diverse histological origin. Int J Neurooncol 2018;1:34-9
| Introduction|| |
Simultaneous existence of primary brain tumors with different pathologies was described in not more than 0.9% of all primary brain tumors, but the majority of those cases were seen after radiotherapy or are associated with familial tumor syndromes., Different brain tumors of distinct histology can co-exist in the setting of phakomatoses or as a complication of radiotherapy. In the absence of these predisposing factors, this phenomenon is uncommon. When the lesions are in proximity, they are described as collision tumors and are extremely rare.
| Case Reports|| |
A 36-year-old patient was admitted with complaints of headache and diplopia. He also had a history of two episodes of focal convulsion involving the left upper limb and lower limbs. On admission, his general examination was found to be normal. No neurocutaneous markers were seen. Neurological examination revealed left temporal hemianopia and ptosis of the right eye. Gadolinium-enhanced cranial magnetic resonance imaging (MRI) showed two different lesions in two different locations. An ill-defined mass lesion measuring 4 cm × 2.5 cm × 2.3 cm was identified in the subcortical region of the right frontal lobe with perifocal edema. It was heterogeneously hypointense on T1-weighted images (WIs) and hyperintense on T2WIs and fluid-attenuated inversion recovery (FLAIR) images [Figure 1]a. It showed mild patchy enhancement on postcontrast study [Figure 1]b. On MR spectroscopy, there was increase in choline/creatine ratio (4.9) and marked increase in N-acetyl aspartate and creatine peaks. The second mass lesion was noted in the sellar and suprasellar regions. It was hyperintense on T1WI, heterogeneously hyperintense on T2WI and FLAIR images, and showed peripheral enhancement on postcontrast study [Figure 2]a and [Figure 2]b. Superolaterally, it was stretching bilateral optic nerves and optic chiasma and laterally abutting the cavernous portion of bilateral internal carotid arteries with well-maintained flow voids. Preoperative laboratory analysis showed that no pituitary hormones were excessively secreted.
|Figure 1: (1a) Preoperative magnetic resonance imaging image showing an ill-defined mass lesion in the right frontal lobe which is hyperintense on T2-weighted images (1b) Gadolinium-enhanced magnetic resonance imaging showing mild patchy enhancement of the right frontal lesion. (2a and b) Saggital and axial contrast enhanced T1W images showing another mass lesion was in the sellar and suprasellar region which showed peripheral enhancement. Superolaterally it was stretching bilateral optic nerves and optic chiasma and laterally abutting the cavernous portion of bilateral internal carotid arteries with well maintained flow voids. (3a-c) Post operative MRI images showing no evidence of residual Lesion in either of the tumour locations|
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|Figure 2: (4a and b) Preoperative magnetic resonance axial and saggital images demonstrating a mass lesion appearing as iso-intense on T1 weighted sequence. (5a and b) Preoperative magnetic resonance axial and coronal images demonstrating the mass lesion as hyper-intense on T2 weighted sequences in the left cer-bellopontine angle, suggesting an epidermoid cyst. (6a) Intra-operative photograph showing pearly white flaky appearance typical of the epidermoid cyst. (6b) Intra-operative photograph showing the co-existing mass lesion(*) near the internal acoustic meatus suggestive of schwannoma. (7) Post operative CT image showing no evidence of residual lesion|
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Treatment was planned in a single transcranial approach, with a combined removal of the frontal lesion and subsequent removal of the sellar-suprasellar tumor through a right fronto-temporal craniotomy. The frontal lesion was grayish, hard nodular with cystic components, and gross total tumor removal was achieved. The sellar-suprasellar tumor was also subsequently removed through the same approach. It was soft, suckable, and grayish colored with some necrotic areas within, and gross total tumor removal was done. Postoperatively, the patient had no new neurological deficits.
Histological examination of the right frontal tumor revealed anaplastic astrocytoma WHO Grade III. On immunohistochemistry, tumor cells were positive for glial fibrillary acidic protein and focally for synaptophysin. MIB-1 labeling index was 6%–8% in the highest proliferation areas. The histolopathologic diagnosis of sellar lesion showed pituitary adenoma with apoplexy. Postoperative MRI showed no residual tumor in either of the locations [Figure 3]a, [Figure 3]b, [Figure 3]c. The patient was discharged on day 8 with recommendation of adjuvant radio-chemotherapy.
|Figure 3: (8a-c) MRI brain revealed a 3.0x2.2x1.9 cm sized ill defined mass in right frontal lobe. It was heterogeneously hypo intense on T1WI, hyper intense on T2WI and FLAIR images. No enhancement on post contrast study.The above findings were suggestive of a low grade glioma. (9) Another 2.4x1.8x1.6 cm sized well defined round extra axial broad based mass was noted in right parafalcine location in the right high frontal region.It showed intense homogenous post contrast enhancement suggestive of a meningioma. (10a and b) Post op CT images showing Near total excision of right frontal glioma and falcine meningioma|
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A 65-year-old man presented with complaints of mild intermittent headache for 6 months and imbalance while walking associated with tendency to fall toward the left side for 3 months. The patient also reported progressive decreased hearing in the left ear. On tuning fork testing, the Weber lateralized to the right and the Rinne test was positive in the left ear. Cerebellar signs were positive. Pure tone audiogram revealed severe sensorineural hearing loss “notch” at 2000 Hz on the left side and hearing sensitivity within normal limits in the right ear.
A contrast-enhanced MRI showed a mass lesion in the left cerebellopontine angle (CPA) which extended inferiorly to the left cerebellomedullary junction and superiorly till the tentorium cerebelli. This mass predominantly demonstrated isointense on T1 [Figure 4]a and [Figure 4]b and hyperintense on T2WIs [Figure 5]a and [Figure 5]b and showed areas of restricted diffusion-weighted images without contrast enhancement, compatible with an epidermoid cyst. It measured approximately 4.5 cm (anteroposterior) × 3.0 cm (transverse) × 5.5 cm (craniocaudal). No other lesion could be visualized in the preoperative imaging.
|Figure 4: (11a) MRI Brain showed diffuse fusiform enlargement of intra orbital segment of the right optic nerve measuring 2.5x1.2x1.3 cm. It was hypo-intense on T1W, hyper-intense on T2W/FLAIR images and showed heterogenous enhancement on post contrast study suggestive of optic nerve glioma. (11b) Another ill defined lobulated non enhancing extra axial heterogenous lesion of size 2.1x2.8x2.2 cm was seen in left Cerebellopontine angle cistern.The lesion was hypo intense on T1W, hyper intense on T2W and showed restricted diffusion with corresponding low ADC values suggestive of epidermoid cyst. (12) Follow up MRI at 3 months showed no residual lesion in the right orbit and no increase in the size of epidermoid cyst|
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A left retromastoid suboccipital craniotomy was performed in the lateral semi-sitting position. After opening the dura, cerebello medullary cistern was opened and cerebrospinal fluid was drained to make cerebellum lax. The mass in the left CPA was encapsulated, pearly white, and flaky extending into the prepontine cistern and the foramen of Luschka compressing the brainstem [Figure 6]a. Tumor was seen encasing cranial nerves 5, 6, 7, and 8. Tumor could be sucked and some solid parts were removed in piecemeal fashion.
Once the major part of the tumor was removed, we could see another encapsulated mass lesion adherent to the 7th and 8th nerve complex near the internal acoustic meatus [Figure 6]b. It was firm in consistency and moderately vascular. This mass lesion was excised completely keeping 7th and 8th cranial nerves intact. Endoscope assistance was taken to visualize and remove the hidden parts of tumor. The completeness of resection was eventually assessed by endoscope.
Postoperatively, the patient made an uneventful recovery and computed tomography brain showed no residual lesion [Figure 7]. Histopathological examination (HPE) showed vestibular schwannoma (WHO Grade I) associated with an epidermoid cyst on the same side of CPA. He was discharged after 10 days with no fresh neurological deficits and asked to follow-up after 1 month for MRI brain.
A 45-year-old female presented with a history of focal convulsions involving the left upper and lower limbs. Neurological examination was within normal limits. MRI brain revealed a 3.0 cm × 2.2 cm × 1.9 cm-sized ill-defined mass in the right frontal lobe involving the white matter. It was heterogeneously hypointense on T1WI and hyperintense on T2WI and FLAIR images [Figure 8]a and [Figure 8]b. There was no enhancement on postcontrast study [Figure 8]c. The above findings were suggestive of a low-grade glioma. Another 2.4 cm × 1.8 cm × 1.6 cm-sized well-defined round extra-axial broad-based mass was noted in the right parafalcine location in the right high frontal region. It was iso-intense on T1WI and heterogeneously hypointense on T2WI and FLAIR images. It showed intense homogeneous postcontrast enhancement suggestive of a meningioma [Figure 9]. A right fronto-temporo-parietal craniotomy with Simpsons Grade II excision of parafalcine meningioma along with gross total excision of right frontal glioma was done. Postoperative images showed complete excision of both lesions [Figure 10]a and [Figure 10]b. On HPE, it was suggestive of low-grade glioma and psammomatous meningioma Grade I. The postoperative course was uneventful and the patient was discharged on the 8th postoperative day.
A 14-year-old male presented with decreased vision and proptosis of the right eye. His visual acuity was finger counting at 2 ft in the right eye and 6/6 in the left eye. Rest of the neurological examination was normal. MRI brain showed diffuse fusiform enlargement of intra-orbital segment of the right optic nerve measuring 2.5 cm × 1.2 cm × 1.3 cm. It was hypointense on T1W, hyperintense on T2W/FLAIR images, and showed heterogeneous enhancement on postcontrast study [Figure 11]a. Imaging findings were suggestive of optic nerve glioma.
Another ill-defined lobulated nonenhancing extra-axial heterogeneous lesion of size 2.1 cm × 2.8 cm × 2.2 cm was seen in the left CPA cistern. The lesion was hypointense on T1WI, hyperintense on T2WI, and showed restricted diffusion with corresponding low apparent diffusion coefficient values suggestive of epidermoid cyst [Figure 11]b. He underwent right pterional craniotomy with orbitotomy and gross total excision of optic nerve lesion. No intervention was done for epidermoid cyst as it was asymptomatic. HPE of orbital tumor was suggestive of ganglioglioma (WHO Grade I). The postoperative course was uneventful. Follow-up MRI at 3 months showed no residual lesion in the right orbit and no increase in the size of epidermoid cyst [Figure 12].
| Discussion|| |
Multifocal primary lesions of a single histological type and multiple metastatic lesions of the brain have been published in the literature. However, the simultaneous occurrence of multiple primary brain tumors with different cell types is rare with the reported incidence of 0.3% and their occurrence is not well understood. The most common tumor associated with the simultaneous presence of other histologically different tumor is meningioma. The most frequently described combination is meningioma associated with glioma followed by schwannoma and pituitary adenoma.,
Giromini et al. reported the case of a primary central nervous system lymphoma in a child 6 months after extirpation of an astrocytoma, emphasizing the concept of collision tumors and their differential diagnosis. Coexistence of oligo-dendroglioma with anaplastic astrocytoma, juvenile pilocytic astrocytoma, pleomorphic xanthoastrocytoma, and metastatic breast carcinoma has been described in published literature. Poeata et al. reported three different cases describing simultaneous occurrence of meningioma with glioblastoma, pituitary adenoma, and acoustic schwannoma.
Phakomatoses, such as neurofibromatous-2, and a previous history of irradiation are well-known causes for the development of multiple primary brain tumors. Several hypotheses have been proposed to explain the coincidence of two completely separate primary brain tumors of different histogenesis in the same patient: (1) tumors can develop entirely coincidentally; (2) the initial tumor can act as a stimulus on the surrounding cerebral parenchyma or meningeal tissue to induce a new tumor in different tissue; (3) a carcinogenic stimulus may develop tumors in different tissues simultaneously; or (4) a residual embryonic structure becomes the basis for subsequent multiple brain tumor development. Furthermore, several studies have addressed the role of endogenous as well as exogenous hormonal therapy on the risk of meningioma formation.
Russel and Rubinstein made the hypothesis that the first tumor provides a stimulus for the surrounding brain parenchyma or meninges to induce a new tumor in a different tissue. Alexander proposed that residual embryonic structures form the basis for the development of subsequent multiple brain tumors. Some authors held carcinogenic stimuli responsible for the simultaneous development of different brain tumors and other attributed it as entirely coincidental.
Glioma may develop due to neoplastic transformation of the reactive glial cells surrounding a meningioma. This process may be mediated by locally acting oncogenic factors. The most suspected substance is platelet-derived growth factor (PDGF) subunit alpha-R, which is the main receptor in astrocytoma. Astrocytoma growth is probably stimulated by PDGF in an autocrine mechanism. It is possible to develop a meningioma as secondary malignant neoplasm due to transformation of the arachnoid cells in response to the growth of a subjacent glioma or after radiation therapy.
| Conclusions|| |
Co-existences of multiple intracranial tumors of different histological lineage that are seen should be reported more frequently, but are very rarely reported in the pertinent literature. Notably of the fact that none of our patients had any family of phakomatosis makes it a unique case series in the relevant literature. Syndromic association should be ruled out in patients of histologically different multiple brain tumors. Thorough clinical and imaging studies are mandatory in these cases to provide optimal treatment. However, based on our case and published literature, we assume that increased research work could provide significant understanding in the development of multiple primary brain tumors.
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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