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| Year : 2014 | Volume
: 57
| Issue : 4 | Page : 650-652 |
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| Primary granulomatous hypophysitis: An interesting entity |
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Subhashis Mitra1, Barnik Choudhury2, Hema Chakraborty1
1 Department of Pathology, Advanced Medicare and Research Institute (Vision Care Hospital), Kolkata, West Bengal, India
2 Department of Pathology, College of Medicine and JNM Hospital, West Bengal University of Health Sciences, Nadia, West Bengal, India
Click here for correspondence address and email
| Date of Web Publication | 11-Oct-2014 |
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How to cite this article:
Mitra S, Choudhury B, Chakraborty H. Primary granulomatous hypophysitis: An interesting entity. Indian J Pathol Microbiol 2014;57:650-2 |
Editor,
We would like to present a case of primary granulomatous hypophysitis in a 65-year-old woman. The patient presented with complaint of severe intermittent headache of 4 months duration, along with visual disturbances. She had no other symptoms, and a generalized physical examination was unremarkable.
MRI (Magnetic resonance imaging) scanning was recommended to identify any organic cause. MR imaging revealed a sellar mass isointense with grey matter, with bright contrast enhancement [Figure 1]. Further serological, hematological, and comprehensive endocrinological investigations were done; with normal results excluding elevated ESR (44 mm/1 st hour) and slight decrease in cortisol levels to 3 μg/dL (Normal = 5-25 μg/dL). The mass was diagnosed as non-functioning pituitary adenoma. | Figure 1: (a) T1-weighted axial MR scan showing sellar mass abutting optic chiasma, (b) T2-weighted coronal MR scan showing sellar mass, isointense with grey matter, (c) T1-weighted sagittal MR scan howing sellar mass (pituitary region), (d) T1-weighted sagittal MR scan showing intense contrast (gadolinium) enhancement of sellar mass
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Trans - sphenoidal microsurgery was done to debulk the pituitary mass, and excised tissue sent for histopathological examination. Microscopic examination revealed chronic inflammation, with several large, discrete epithelioid granulomas and few foci of pituitary parenchyma. Numerous Langhans and foreign body type giant cells, epithelioid cells, lymphocytes and variable degree of fibrosis were noted. Ziehl-Nielson(ZN/AFB) and periodic acid Schiff's(PAS) stains were done, but showed no evidence of acid fast bacilli or fungal elements [Figure 2]. A histopathological diagnosis of Idiopathic/Primary Granulomatous Hypophysitis (PGH) was given. | Figure 2: Section showing granulomas, lymphocytic infiltration and fibrosis (100×, H and E). Inset shows giant cells and lymphocytes (400×; (a,b) H and E, (c) Z-N stain)
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The patient was kept under observation, and further investigations done to rule out any systemic granulomatous diseases or hormonal dysfunction. No such evidence was found, and the patient's headaches and visual disturbances resolved within a week. Repeat endocrinological and neuroradiological examinations are normal, and the patient remains symptom-free 2 years post operatively.
Granulomatous hypophysitis was first reported in an antemortem case in 1980. PGH is a rare entity, accounting for less than 1% of all pituitary tumours. The male:female ratio is nearly 1:2, and incidence peaks at around 46 years of age. 39 confirmed cases of PGH have been reported in the English literature till date. [1],[2]
Symptoms arise due to sellar compression or pituitary parenchymal destruction. Headache, endocrine dysfunction, and visual disturbances are the major symptoms.
MRI shows a pyramidal sellar mass isointense with grey matter on T1/T2-weighted imaging, brightly enhancing with contrast. Preoperative diagnosis is difficult, and it is usually confused with pituitary adenoma. [3],[4] Hypophysitis maybe suspected on basis of stalk thickening, suprasellar extension, and loss of posterior pituitary hyperintensity. [5]
Primary hypophysitis exists as a spectrum ranging from lymphocytic hypophysitis to granulomatous hypophysitis, as well as xanthomatous and necrotizing forms. Lymphocytic hypophysitis is usually seen in peri-partum females, can remit spontaneously, and is associated with autoimmune diseases. Pathogenesis of PGH is unclear, though secondary causes include tuberculosis, sarcoidosis, syphilis, foreign body reactions, mycotic granulomas, and systemic immunologic diseases. Histopathology remains the gold standard of diagnosis in PGH. [4] Trans-sphenoidal surgery is recommended for all cases to aid unequivocal diagnosis, disease specific therapy, rapid decompression and resolution of symptoms, and to obviate unnecessary steroid therapy. [1],[2] Frozen section examination can be done intra-operatively to prevent excess removal of tissue. PGH, though rare, has been reported in India, and may be considered in differential diagnosis of a pituitary mass presenting with headache and vision defects, with or without any hormonal dysfunction. [2],[5]
 References | |  |
| 1. | Su SB, Zhang DJ, Yue SY, Zhang JN. Primary granulomatous hypophysitis: A case report and literature review. Endocr J 2011;58:467-73.  |
| 2. | Gundgurthi A, Kharb S, Garg MK, Brar KS, Bharwaj R, Gupta S, et al. Combined granulomatous and lymphocytic hypophysitis presenting as pituitary incidentaloma in a middle-aged woman. Indian J Endocrinol Metab 2012;16:846-9. |
| 3. | Kristof RA, Van Roost D, Schramm J, Wichers M. Primary granulomatous hypophysitis not responsive to pulsed high dose prednisolone therapy: Case report. J Neurol Neurosurg Psychiatry 1998;64:693-4. [ PUBMED] |
| 4. | Shi J, Zhang JM, Wu Q, Chen G, Zhang H, Bo WL. Granulomatous hypophysitis: Two case reports and literature review. J Zhejiang Univ Sci B 2009;10:552-8. |
| 5. | Bhansali A, Velayutham P, Radotra BD, Pathak A. Idiopathic granulomatous hypophysitis presenting as non-functioning pituitary adenoma: Description of six cases and review of literature. Br J Neurosurg 2004;18:489-94. |
Correspondence Address:
Dr. Subhashis Mitra
Consultant Pathologist, Department of Pathology, Advanced Medicare and Research Institute (Vision Care Hospital, 223, 230 Barkohala Lane, Mukundapur, Kalikapur, Kolkata - 700 099, West Bengal
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0377-4929.142713
[Figure 1], [Figure 2] |
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