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CASE REPORT
Ahead of print publication  

Dual intracranial primaries: A rare occurrence


 Department of Radiation Oncology, GCRI, Ahmedabad, Gujarat, India

Date of Submission15-May-2021
Date of Acceptance23-May-2021
Date of Web Publication12-Jul-2021

Correspondence Address:
Aastha Shah,
Department of Radiation Oncology, GCRI, Ahmedabad, Gujarat
India
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jrcr.jrcr_11_21

  Abstract 


The incidence of two or more intracranial malignancies of different cell types is extremely rare with an incidence of <0.9% of all primary central nervous system tumors. Herein, we report the rare combination of pituitary macroadenoma, benign condition with glioblastoma multiforme, highly aggressive malignancy in a 33-year-old male with the initial complaints of intermittent headache and vomiting. The clinical presentation, radiological characteristics, and treatments received by the patient are briefly discussed along with review of literature. The patient was treated with surgery followed by chemoradiotherapy and medical management. Post radiation, contrast-enhanced computed tomography scan of the brain showed postoperative changes with the resolution of the pituitary lesion.

Keywords: Glioblastoma multiforme, chemoradiotherapy, pituitary macroadenoma, surgery, rare



How to cite this URL:
Shah A, Patel M, Suryanarayan U. Dual intracranial primaries: A rare occurrence. J Radiat Cancer Res [Epub ahead of print] [cited 2021 Sep 23]. Available from: https://www.journalrcr.org/preprintarticle.asp?id=321220




  Introduction Top


The detection rates of brain tumors are increasing in the present age due to the availability of computed tomography (CT) and magnetic resonance imaging (MRI). However, the simultaneous occurrence of dual intracranial malignancies with different cell types is extremely rare with the incidence of <0.9% of all primary brain tumors.[1],[2] The most common combination is the simultaneous occurrence of meningioma and glioma, followed by meningioma and neuroma, and meningioma and pituitary adenoma.[1],[3] Here, we report a rare combination of pituitary macroadenoma, benign condition with glioblastoma multiforme, highly aggressive malignancy. The pituitary adenoma in this report was of functioning variety; however, literature reports occurrence of functioning as well as nonfunctioning adenomas both. The nonsyndromal association of these synchronous co-occurrences is in itself rare.


  Case Report Top


A 33-year-old male presented with complaints of intermittent episodes of headache and vomiting for 3 months which were aggravated for the last 3 days. On eliciting a further history, we found out that the patient had no history of any generalized or focal convulsions, no difficulty in vision, and no history of any trauma or any previous surgery. The patient had no previous history of radiation exposure and familial-related diseases were absent.

On physical examination, the patient was well oriented to time, place, and person. There was no difficulty in vision, speech, hearing, and memory. Power in all the four limbs was 5/5, and bowel and bladder controls were unaltered. Routine blood investigations including complete hemogram, renal and liver function tests, coagulation profile, and viral markers were done which were unremarkable. Contrast-enhanced MRI of the brain was done which showed 3.2 cm × 2.9 cm × 2.8 cm lesion in the left frontal lobe involving the insular cortex. The lesion was heterogeneously hyperintense on T2-weighted image (T2w) and fluid-attenuated inversion recovery (FLAIR) and isointense on T2-weighted image (T1w) images with internal areas of restricted diffusion suggestive of peritumoral edema. On administration of gadolinium contrast, irregular capsular enhancement with nonenhancing central necrosis was seen. Midline shift of 4 mm toward the right side was seen. These findings suggest high-grade glioma. Another 1.4 cm × 1.3 cm × 1.5 cm well-defined homogeneous sellar mass was seen showing suprasellar extension which was isointense on T1w images and mild hyperintense on T2w and FLAIR images. This second lesion showed homogeneous postcontrast enhancement, and was seen abutting optic chiasma (left more than right), floor of third ventricle and bilateral cavernous internal carotid artery, highly suggestive of pituitary macroadenoma. Following this, the patient was advised for a hormonal profile which showed raised serum prolactin 21.77 ng/mL (normal level: 4.04–15.2 ng/mL), rest of the hormones including serum thyroid-stimulating hormone, T3 and T4, serum cortisol, serum follicle-stimulating hormone, luteinizing hormone, and growth hormone. Although there was no tissue diagnosis of sellar lesion, the clinical, laboratory, and radiographic features favored the diagnosis of pituitary adenoma.

The left frontal lesion was addressed by left frontotemporal craniotomy and gross total excision of left frontal space-occupying lesion was done. The histopathology showed features of necrosis, endothelial proliferation, atypia, and mitosis which confirmed the diagnosis of glioblastoma multiforme. Pituitary adenoma lesion was not addressed surgically but was addressed by medical management and radiation. The postoperative CT scan showed postoperative changes in the left frontal region with intact pituitary macroadenoma. The patient was started on tablet cabergoline 0.5 gram once a week, a dopamine agonist as prescribed by the endocrinologist taking into consideration the weight of the patient. The patient was planned for radiation using conformal technique (three-dimensional conformal radiation therapy), as shown in [Figure 1], to a dose of 60 Gray in 30 fractions at 2 Gray per fraction with concurrent capsule temozolomide to a dose of 75 mg/m2 body surface area. Following radiation, hormonal profile was repeated where the values of serum prolactin decreased to a value of 18.81 ng/mL and the patient was continued on the same dose of tablet cabergoline.
Figure 1: Radiation planning involving the pituitary lesion along with the left frontal region tumor bed

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One month post radiation, the patient came on follow-up with no any complaints and was started on capsule temozolomide to a dose of 150 m/m2 body surface area. Hormonal profile at the end of 4 months was repeated where the levels of serum prolactin reduced to 1.79 ng/mL. Contrast-enhanced CT scan of the brain showed postoperative changes with the resolution of the pituitary lesion.


  Discussion Top


A malignant glioma is believed to comprise a heterogeneous population of cells which is not the case seen with a benign lesion. Gliomas represent 50% of all the primary intracranial malignancies of which glioblastoma multiforme constitutes about 40%–50%.[4] Secondary glioblastoma has a better prognosis as compared to primary ones. Pituitary adenomas are benign and slow-growing tumors which arise from oral ectoderm, of them majority are prolactinomas. However, the simultaneous occurrence of two or more intracranial malignancies with different pathologies is a rare finding with only a few such cases having been reported in literature. Most of the cases have occurred after radiotherapy or have an association with familial tumor syndromes.[3] The pathogenesis of such co-occurrence of intracranial tumors remains unclear. But from a number of reported cases, the possible different hypotheses are explained. One tumor may act as an irritating agent for the local proliferation and growth of the other tumor localized in juxtaposition.[5] When the tumors were adjacent to each other, surgical trauma, ionizing radiation, and genetic factors may influence tumorigenesis.[6] However, collision tumors may be mediated via locally acting oncogenic factors, particularly platelet-derived growth factor subunit alpha-R (PDGF-alpha-R), which is the main receptor in astrocytoma.[7] The co-occurrence of endodermal and neuroectodermal tumors is extraordinarily rare, as seen in the present case.[8] To our knowledge, co-occurrence of glioblastoma with pituitary adenoma has been reported five times in the literature. Among these, three cases were reported as glioblastoma with prolactin-secreting pituitary adenoma (prolactinoma) and our case will be the fourth one to be reported. Miyagi et al. reported a case of simultaneous co-existing triple intracranial tumors of multicentric glioblastoma along with prolactinoma and transitional meningioma in a 77-year-old female.[9] Furtado et al. described a case of glioblastoma in a 36-year-old female co-occurring in a treated case of prolactinoma.[4] Al Shalawi et al. reported a case of simultaneous co-occurrence of glioblastoma and a prolactin-secreting pituitary adenoma.[1] Naydenov et al. reported a case of co-occurrence of 2 different brain tumors such as glioblastoma and pituitary adenoma in association with colorectal cancer as a nonfamilial Turcot syndrome.[3] Haciyakupoglu et al. reported a case of simultaneous co-existing glioblastoma and null cell pituitary adenoma in a 61-year-old patient.[2] All these five cases of glioblastoma with pituitary adenoma had no history of radiation exposure, and only one case has an association with tumor syndrome.[3] Moreover, our case had no association with any history of radiation exposure or presence of any familial and tumor syndromes. Although tissue biopsy was not performed for sellar lesion in our patient, the clinical, biochemical, and radiological profiles were suggesting prolactinoma.

Due to no similarities relating to gene mutation between glioblastoma multiforme and pituitary adenoma and no prior radiation history and no familial history suggests unknown mechanism for tumorogenesis of such a rare co-occurrence.


  Conclusion Top


The coexistence of dual intracranial neoplasms is a rare occurrence and further studies are required to find out the possible etiopathogenesis and a genetic linkage to such conditions.

Informed consent

The patient has duly consented to report his case.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Alshalawi A, Alkhaibary A, Basalamah A, Alassiri AH, Alarifi A. Glioblastoma and prolactinoma: A rare simultaneous occurrence. J Surg Case Rep 2019;2019:rjz030.  Back to cited text no. 1
    
2.
Haciyakupoglu E, Sav A, Haciyakupoglu S, Walter J. Multiple intracranial tumors: Coexistence of a glioblastoma and null cell pituitary adenoma within the same patient. Clin Neurol Neurosurg 2014;120:120-3.  Back to cited text no. 2
    
3.
Naydenov E, Marinov M, Nachev S. Two different primary brain tumors, glioblastoma multiforme and pituitary adenoma, in association with colorectal carcinoma: An unusual case of nonfamilial Turcot's syndrome? J Neurol Surg A Cent Eur Neurosurg 2012;73:410-2.  Back to cited text no. 3
    
4.
Furtado SV, Venkatesh PK, Ghosal N, Hegde AS. Coexisting intracranial tumors with pituitary adenomas: Genetic association or coincidence? J Cancer Res Ther 2010;6:221-3.  Back to cited text no. 4
    
5.
Tajika Y, Kubo O, Takeshita M, Tajika T, Shimizu T, Kitamura K. An intracranial collision tumor composed of intrasellar gangliocytoma and pituitary adenoma. No Shinkei Geka 1989;17:1181-6.  Back to cited text no. 5
    
6.
Hakan T, Armagan S, Aker FV, Celik L. Meningioma and glioblastoma adjacent in the brain. Turk Neurosurg 1998;8:57-60.  Back to cited text no. 6
    
7.
Tokunaga T, Shigemori M, Hirohata M, Sugita Y, Miyagi J, Kuramoto S. Multiple primary brain tumors of different histological types–Report of two cases. Neurol Med Chir (Tokyo) 1991;31:141-5.  Back to cited text no. 7
    
8.
Naik H, Vernon V, Gade P, Bhople L, Guha A. Anaplastic astrocytoma and pituitary macroadenoma within the same patient: A rare case of intracranial collision tumor. Neurol India 2018;66:857-60.  Back to cited text no. 8
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9.
Miyagi A, Maeda K, Sugawara T, Sawada T, Tsubokawa T. Triple primary intracranial tumors of different cell types: A case report. No Shinkei Geka 1995;23:531-6.  Back to cited text no. 9
    


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