| Article Access Statistics|
| Viewed||1480 |
| Printed||23 |
| Emailed||0 |
| PDF Downloaded||62 |
| Comments ||[Add] |
| Cited by others ||1 |
Click on image for details.
|LETTER TO EDITOR
|Year : 2016 | Volume
| Issue : 2 | Page : 343-345
Granulomatous hypophysitis – A rare pathology: Review of three cases
Biswaranjan Nayak, Ram Kumar Goyal, Rajiv Maharshi, Debabrata Biswal, Prafulla Kumar Sahoo
Department of Neurosurgery, Apollo Hospital, Bhubaneswar, Odisha, India
|Date of Web Publication||3-Mar-2016|
Department of Neurosurgery, Apollo Hospital, Bhubaneswar, Odisha
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Nayak B, Goyal RK, Maharshi R, Biswal D, Sahoo PK. Granulomatous hypophysitis – A rare pathology: Review of three cases. Neurol India 2016;64:343-5
Inflammatory pituitary lesions are extremely rare, with an estimated annual incidence of one case in 10 million individuals. Histologically, these can be granulomatous, lymphocytic, xanthomatous, and necrotizing hypophysitis. Granulomatous hypophysitis is a chronic inflammatory condition of the pituitary, first described by Simmonds in 1917. Majority of pituitary granulomas present with a specific lesion such as tuberculosis, sarcoidosis, syphilis, or histiocytosis-X. In the absence of any demonstrable causative agent, the process is termed as idiopathic granulomatous hypophysitis. Isolated involvement of the pituitary gland by tuberculosis (pituitary tuberculoma) is very rare. It was first reported as a case of sellar tuberculoma from Britain in 1924. Since then, only few case reports of this entity have been recorded, mostly from India and other developing countries. ,,,
Case 1: A 48-year old woman presented with frontal headache for 5 months along with gradual diminution of vision in both the eyes for 3 months. On examination, the patient was conscious, oriented, and afebrile, and her vitals were normal. She had bitemporal hemianopia. Other than the optic nerve, all other cranial nerves were normal. There were no motor or sensory deficits. Routine biochemical and hematological profiles were normal. Chest radiography did not reveal any pathology. Endocrinal study revealed a prolactin level of 48.37 (normal range, 1–25) ng/mL; GH, 2.4 (normal range, 0–3) ng/mL; T4, 6.6 (range, 5–15) μg/dL; T3, 84.71 (80–200) ng/dL; TSH, 2.19 (0.27–4.20) mIU/mL; and cortisol, 0.832 (5–25) mg/dL. Contrast T1-weighted (W) MRI study revealed an enhancing mass in the sella, with hypointensity at the center and with suprasellar extension; T2W MRI showed a homogenously hyperintense lesion. The tumor was accessed through a trans-sphenoidal approach, tumor debulking was done, and the sample sent for histopathological examination. The postoperative period was uneventful. The histopathological examination revealed chronic inflammatory cells, epithelioid cells, and multinucleated giant cells of Langhans type, with areas of necrosis suggestive of tubercular hypophysitis. Ziehl–Neelsen staining and culture of the caseous material were negative for acid fast bacilli (AFB). No fungus was isolated. The patient was advised standard antituberculous treatment for 18 months along with steroid replacement therapy. At follow-up, there was no headache and visual symptoms had also improved.
Case 2: Our second patient was a 41-year old man who presented with headache, gradual diminution of vision in both the eyes, and generalized weakness for 1 month. Upon examination, the patient was conscious, oriented, and afebrile, and his vitals were normal, with decreased vision; other cranial nerves were normal; there were no motor or sensory deficits. Routine biochemical and hematological profiles were normal. Chest radiography did not reveal any pathology. Endocrinal study revealed a prolactin level of 63.2 ng/mL; GH, 2.1 ng/mL; T4, 7.4 μg/mL; T3, 84.3 ng/dL; TSH, 3.79 mIU/mL; and cortisol, 6.3 mg/dL. Contrast T1W MRI study revealed an enhancing mass in the sella, with hypointensity at the center and with suprasellar extension; T2W MRI showed a homogenously hyperintense lesion [Figure 1]. The tumor was accessed through the trans-sphenoidal approach, tumor debulking was done, and the sample was sent for histopathological examination. The postoperative period was uneventful. Histopathological examination revealed focal replacement of the pituitary architecture by a lymphocytic infiltrate, noncaseating epithelioid cell granulomas with giant cells, and areas of fibrosis. Staining for acid-fast bacilli, spirochetes, and fungi was negative. On follow-up, there was no headache and visual symptoms had also improved.
|Figure 1: Contrast MRI showing a homogenously hyperintense enhancing lesion|
Click here to view
Case 3: Our third patient was a 40-year-old woman who presented with gradual progressive diminution of vision in both the eyes, frontal headache with intermittent vomiting for 2 years, and oligomenorrhea for 10 months. On examination, the patient was conscious, oriented, and afebrile, and her vitals were normal; vision in the left eye was 1/60; right eye, 5/60; with loss of temporal field of vision. Other than the optic nerve, all other cranial nerves were normal; there were no motor or sensory deficits. Routine biochemical and hematological profiles were normal. Chest radiography did not reveal any pathology. Her endocrinal study revealed a prolactin level of 6.30 ng/mL; GH, 2.4 ng/mL; T4, 0.92 μg/dL; T3, 3.2 ng/dL; TSH, 4.5 mIU/Ml; and cortisol, 47 mg/dL. Both T1W and T2W MRI study revealed a hyperintense mass in the sellar region with suprasellar extension [Figure 2]. The tumor was accessed through a left pterional approach, almost total tumor debulking was done, optic nerve and chiasmal decompression was carried out, and the tumor was sent for histopathological examination. The postoperative period was uneventful. Histopathological examination revealed several foci of granulomas comprising groups of epithelioid cells, mononuclear histocytes, and scattered lymphocytes along with Langhans-type giant cells. No fungus was isolated. Hence, keeping tubercular hypophysitis in mind, we started antituberculous therapy. The patient was advised standard antituberculous treatment. In the repeat MRI scan after a follow-up of 6 months, we again found a lesion of the same size in the sellar area. We then got the histopathology reviewed at a higher center and a diagnosis of sarcoidosis was made. On this basis, we started steroid therapy. The patient was followed up for 2 more years, unfortunately, after which she died owing to a cardiac event.
|Figure 2: (a) Preoperative MRI study revealed a hyperintense mass in the sellar region with suprasellar extension; (b) 6-month postoperative recurrence|
Click here to view
Intracranial tuberculomas account for 0.15%–4% of all intracranial tumors., Tuberculosis affecting the sellar region, however, is extremely rare, with no reported incidence or prevalence. Although the first reported case of a sellar tuberculoma was from Britain in 1924, the literature on sellar tuberculomas, unsurprisingly, largely arises from the Indian subcontinent and other developing countries. All our patients presented with a history of headaches and diminution of vision in both the eyes and some kind of pituitary dysfunction. One of the largest series reporting on intrasellar tuberculosis (18 cases) has described a 100% incidence of headache. Owing to their compressive effects, varying degrees of anterior pituitary dysfunction can develop later with or without central diabetes insipidus. Hypopituitarism with hyperprolactinemia occurs, causing galactorrhea and amenorrhea in female patients and decreased libido in their male counterparts. A concomitant history of systemic tuberculosis is not always present. Radiologically, the tumor is usually sellar or suprasellar, being isointense on T1W images and hyperintense on T2W sequences. Pituitary stalk thickening is often noted on MRI scans. The clinical and radiological features of these lesions mimic a typical pituitary adenoma. However, following surgery, histological appearances characteristically reveal necrotizing granulomatous inflammation with areas of caseation necrosis. AFBs are rarely demonstrated within the lesions, which in turn serves to widen the differential diagnosis to include other granulomatous conditions such as sarcoidosis and fungal infection. Some patients are even wrongly treated for those conditions. This tuberculoma can undergo caseation resulting in a pituitary abscess, or hemorrhagic infarction leading to pituitary apoplexy.
Surgery is usually indicated for obtaining biopsy samples and to confirm the diagnosis. Trans-sphenoidal route is the safest approach to avoid cerebrospinal fluid contamination; however, for very large masses, craniotomy can be performed. Intraoperative findings of a thickened hypophyseal stalk, a grayish-firm, nonsuckable mass with caseation, and a thickened dura serve as telltale signs of a tuberculoma. Anti tuberculous therapy is mandatory, and hormone replacement therapy may need to be instituted in the cases that present with signs and symptoms of hypopituitarism.
Granulomatous hypophysitis is a rare condition but must be considered in the differential diagnosis of pituitary masses in developing countries as the likelihood of occurrence of these lesions is higher. Surgical decompression and biopsy are mandatory for the diagnostic purpose. After confirming the diagnosis, definitive therapy should be instituted.
Financial support and sponsorship
No financial support of any kind for this study was received.
Conflicts of interest
There was no conflict of interest.
| » References|| |
Sautner D, Saeger W, Lüdecke DK, Jansen V, Puchner MJ. Hypophysitis in surgical and autoptical specimens. Acta Neuropathol 1995;90:637-44.
Simmonds M. Uber das vorkommen reisenzellen in der hypophyse. Virchows Arch Pathol Anat 1917;223:281-90.
Letchworth TW. Tuberculoma of the pituitary body. Br Med J 1924;1:1127.
Coleman CC, Meredith JM. Diffuse tuberculoma of the pituitary gland simulating tumour with postoperative recovery. Arch Neurol Psychiatry 1940;44:1076-85.
Manghani DK, Gaitonde PS, Dastur DK. Pituitary tuberculoma–A case report. Neurol India 2001;49:299-301.
Desai KI, Nadkarni TD, Goel A. Tuberculomas of hypophysitis cerebri: Report of five cases. J Clin Neurosci 2003;10:562-6.
DeAngelis LM. Intracranial tuberculoma: Case report and review of the literature. Neurology 1981;31:1133-6.
Maurice-Williams RS. Tuberculomas of the brain in Britain. Postgrad Med J 1972;48:679-81.
Sharma MC, Arora R, Mahapatra AK, Sarat-Chandra P, Gaikwad SB, Sarkar C. Intrasellar tuberculomas–An enigmatic pituitary infection: A series of 18 cases. Clin Neurol Neurosurg 2000;102:72-7.
Sinha S, Singh AK, Tatke M, Singh D. Hypophyseal tuberculoma: Direct radiosurgery is contraindicated for a lesion with a thickened pituitary stalk: Case report. Neurosurgery 2000;46:735-9.
Majumdar K, Barnard M, Ramachandra S, Berovic M, Powell M. Tuberculosis in the pituitary fossa: A common pathology in an uncommon site. Endocrinol Diabetes Metab Case Rep 2014;2014:140091.
Arunkumar MJ, Rajshekhar V. Intrasellar tuberculoma presenting as pituitary apoplexy. Neurol India 2001;49:407-10.
Ghosh S, Chandy MJ. Intrasellar tuberculoma. Clin Neurol Neurosurg 1992;94:251-2.
Husain N, Husain M, Rao P. Pituitary tuberculosis mimicking idiopathic granulomatous hypophysitis. Pituitary 2008;11:313-5.
[Figure 1], [Figure 2]
|This article has been cited by|
||Granulomatous and lymphocytic hypophysitis - are they immunologically distinct?
| ||Shilpa Rao,Anita Mahadevan,Tanmoy Maiti,Manish Ranjan,Shivayogi Durgad Shwetha,Arimappamagan Arivazhagan,Jitender Saini |
| ||APMIS. 2016; 124(12): 1072 |
|[Pubmed] | [DOI]|