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BRIEF COMMUNICATION
Year : 2021  |  Volume : 9  |  Issue : 2  |  Page : 80-83

A rare case report of hypophysitis in pituitary macroadenoma mimicking pituitary apoplexy


1 Department of Neuro Ophthalmology, Aravind Eye Hospital, Madurai, Tamil Nadu, India
2 Department of General Ophthalmology, Aravind Eye Hospital, Puducherry, India

Date of Submission26-Jul-2020
Date of Decision26-Nov-2020
Date of Acceptance12-Jan-2021
Date of Web Publication31-Jul-2021

Correspondence Address:
Sivagami Nachiappan
C/o Aravind Eye Hospital, Cuddalore Main Road, Thavalakuppam, Puducherry - 605 007
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jcor.jcor_123_20

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  Abstract 


Hypophysitis, a rare inflammatory disorder of the pituitary gland is often misdiagnosed as mass lesion. Granulomatous hypophysitis presenting clinically as pituitary apoplexy is rare. A 23-year-old woman with pituitary macroadenoma presented with sudden diminution of vision in the right eye with features clinically mimicking pituitary apoplexy. Imaging revealed pituitary macroadenoma with hypophysitis. After a course of high-dose intravenous corticosteroids, her visual acuity improved to 6/6. Although apoplexy and hypophysitis have subtle clinical and radiological differences, it is essential to differentiate them to avoid unnecessary invasive procedures when conservative treatment can suffice.

Keywords: Hypophysitis, pituitary apoplexy, pituitary macroadenoma


How to cite this article:
Kowsalya A, Nachiappan S, Kumar M. A rare case report of hypophysitis in pituitary macroadenoma mimicking pituitary apoplexy. J Clin Ophthalmol Res 2021;9:80-3

How to cite this URL:
Kowsalya A, Nachiappan S, Kumar M. A rare case report of hypophysitis in pituitary macroadenoma mimicking pituitary apoplexy. J Clin Ophthalmol Res [serial online] 2021 [cited 2022 Jul 2];9:80-3. Available from: https://www.jcor.in/text.asp?2021/9/2/80/322786



Sudden vision loss in pituitary macroadenoma though rare, occurs mostly due to pituitary apoplexy. There are very few surgical emergencies in ophthalmology, and pituitary apoplexy is one such condition. It is defined as an acute clinical syndrome characterized by a triad of headache, vomiting, and sudden visual impairment, and can be associated with ophthalmoplegia, altered consciousness, and hemodynamic instability. Hypophysitis is a rare inflammatory disorder of the pituitary gland that can often be misdiagnosed as a mass lesion. It represents <1% of patients who present with symptoms of hypopituitarism and sellar compression.[1]

Although granulomatous hypophysitis has been previously described in literature, its clinical presentation as pituitary apoplexy is rare.[2] We report a patient with hypophysitis manifesting clinically as pituitary apoplexy who with timely intervention had complete visual recovery.


  Case Report Top


A 23-year-old woman with headache and seizures for 2 years was diagnosed to have pituitary macroadenoma elsewhere. She presented to our Neuro-Ophthalmology Department after discontinuing treatment for 2 days with complaints of the sudden decrease in vision in the right eye (RE) with headache. Her best-corrected visual acuity (BCVA) was 5/60 in RE and 6/6 in the left eye (LE). Anterior and posterior segment examination was normal in both eyes (BE) except for relative afferent pupillary defect in RE. Automated perimetry and color vision could not be performed in RE due to low vision. On Bjerrum tangent screen, tubular vision restricted to central 10° was noted. Color vision and visual fields were normal in LE.

Magnetic resonance imaging of the brain done 3 weeks back showed enlarged pituitary gland measuring 1.78 cm × 1.14 cm × 1.60 cm with convex upper surface giving the “Figure of 8/Snow man” appearance with mild indentation over the optic chiasma [Figure 1]a and [Figure 1]b.
Figure 1: (a) Contrast enhanced magnetic resonance imaging – T1 weighted coronal section showing enlargement of pituitary gland with mild indentation over optic chiasm. Convex upper surface gives “Figure of 8” appearance – (Yellow arrow) (b) Contrast enhanced magnetic resonance imaging – T1 Weighted Sagittal section showing enlargement of pituitary gland with significant involvement of anterior pituitary (red arrow)

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With prolactin levels of 88.2 ng/ml (Normal: 2.5–29.2 ng/ml in nonpregnant women) and thyroid profile indicating severe hypothyroidism (Free T3 <1.00 pg/ml [Normal: 2.1–3.8 pg/ml], Free T4 <0.40 ng/dl [Normal: 0.7–2.0 ng/dl], TSH >150 μIU/ml [Normal: 0.2–2.5 μIU/ml]), she was prescribed levothyroxine 100 μg and bromocriptine 1.25 mg daily. Follicular-stimulating hormone, luteinizing hormone, adrenocorticotropic hormone, and growth hormone levels were normal.

To rule out pituitary apoplexy as the cause of sudden vision loss, neuroimaging was advised. Computed tomography of the brain showed diffuse enlargement of the pituitary gland with ring-enhancing lesion suggestive of pituitary macroadenoma with hypophysitis [Figure 2]. There was no intralesional calcification or hemorrhage as anticipated in pituitary apoplexy. A reduction in the size of the mass to 1.0 cm × 1.0 cm × 0.9 cm could be attributed to the bromocriptine therapy.
Figure 2: Computed tomography of brain - Axial section showing diffuse enlargement of pituitary gland with ring enhancing lesion suggestive of pituitary macroadenoma with hypophysitis (blue arrow)

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In view of inflammatory pathology, intravenous methylprednisolone 1 g per day was given for 3 consecutive days. Her uncorrected visual acuity in the RE improved to 6/6 on the 4th day. Color vision and visual fields returned to normal. She was advised tapering the course of oral prednisolone starting with 40 mg/day followed by a 10 mg reduction every week.

Two weeks after complete recovery of vision in RE, her vision in RE remained 6/6 but the vision in LE dropped to 6/60 with constriction of visual fields to central 10°. She consulted a neurosurgeon and repeat neuroimaging revealed pituitary macroadenoma with no evidence of apoplexy. However, in view of the recurring visual symptoms, transnasal endoscopic excision of the pituitary mass was performed under general anesthesia. Histopathology reported numerous round to oval cells with vesicular nuclei. Stippled chromatin and moderate eosinophilic cytoplasm with uniform nuclear morphology were found suggestive of pituitary adenoma [Figure 3]. Adenomas tend to show uniform morphology and cell type with uncommon cytologic atypia, whereas normal pituitary on the other hand, has mixed cell types on histopathological examination. After surgical excision, BCVA was 6/6 in BE with normal color vision and visual fields.
Figure 3: Histopathology image of resected specimen suggestive of pituitary adenoma

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During the last visit, 2 years after the surgical excision, her vision, color vision, and visual fields remained the same. She was on thyroxine replacement therapy with no recurrence of visual symptoms.


  Discussion Top


Our patient, though presented with clinical features of apoplexy, was radiologically found to have hypophysitis and with high dose corticosteroids had complete recovery of vision.

Pituitary apoplexy is characterized by acute infarction or hemorrhage of the pituitary gland usually occurring in a pituitary adenoma. The various differential diagnoses include subarachnoid hemorrhage, bacterial meningitis, midbrain infarction, cavernous sinus thrombosis, migraine, hemorrhagic infarction in Rathke's cyst, and aneurysms.

Hypophysitis is an autoimmune disease portrayed by inflammation and cellular infiltration of the pituitary gland. Rarely, hypophysitis has been reported in association with pituitary adenoma.[3],[4],[5] Hypophysitis can be classified histologically as lymphocytic, granulomatous, and xanthomatous subtypes.[2] Most cases of granulomatous hypophysitis are secondary to pituitary adenoma or systemic granulomatous diseases, notably sarcoidosis, syphilis, and tuberculosis.

Granulomatous hypophysitis is a close differential for pituitary apoplexy and typically presents with features of hypopituitarism and compressive symptoms. Although it is a rare diagnosis, it is imperative to consider hypophysitis when faced with a clinical picture of severe headaches and visual disturbances, as medical treatment would suffice in treating the inflammation. However, an apoplexy would need surgical decompression. Reduction in size of the lesion can be achieved by steroids due to their anti-inflammatory effect and medical treatment may help recover pituitary function.

Psammoma bodies represent the process of dystrophic calcification and are commonly seen in papillary thyroid carcinoma, serous cystadenocarcinoma of the ovary, meningioma, and rarely in other neoplastic and nonneoplastic lesions.[6]

It is imperative to emphasize that hypophysitis is a radiological diagnosis. In neuroimaging, features such as hemorrhage or necrosis are indicative of pituitary apoplexy, whereas hypophysitis on the other hand is characterized by cystic areas and ring enhancement.

Surgical decompression, usually by transsphenoidal route, is indicated if consciousness or vision is impaired despite glucocorticoid replacement and electrolyte support.[7],[8] Our patient subsequently needed surgical decompression in view of the recurrent visual symptoms.


  Conclusion Top


It is crucial to differentiate apoplexy from hypophysitis so as to avoid unnecessary invasive procedure when conservative treatment would suffice. At the same time, as ophthalmologists, we must refer them to a neurosurgeon for intervention at an appropriate time as relapses may have more severe visual and systemic impact.

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.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Chung CH, Song MS, Cho HD, Jeong du S, Kim YJ, Bae HG, et al. A case of idiopathic granulomatous hypophysitis. Korean J Intern Med 2012;27:346-9.  Back to cited text no. 1
    
2.
Husain Q, Zouzias A, Kanumuri VV, Eloy JA, Liu JK. Idiopathic granulomatous hypophysitis presenting as pituitary apoplexy. J Clin Neurosci 2014;21:510-2.  Back to cited text no. 2
    
3.
McConnon JK, Smyth HS, Horvath E. A case of sparsely granulated growth hormone cell adenoma associated with lymphocytic hypophysitis. J Endocrinol Invest 1991;14:691-6.  Back to cited text no. 3
    
4.
Moskowitz SI, Hamrahian A, Prayson RA, Pineyro M, Lorenz RR, Weil RJ. Concurrent lymphocytic hypophysitis and pituitary adenoma. Case report and review of the literature. J Neurosurg 2006;105:309-14.  Back to cited text no. 4
    
5.
Holck S, Laursen H. Prolactinoma coexistent with granulomatous hypophysitis. Acta Neuropathol 1983;61:253-7.  Back to cited text no. 5
    
6.
Das DK. Psammoma body: A product of dystrophic calcification or of a biologically active process that aims at limiting the growth and spread of tumor? Diagn Cytopathol 2009;37:534-41.  Back to cited text no. 6
    
7.
Capatina C, Inder W, Karavitaki N, Wass JA. Management of endocrine disease: Pituitary tumour apoplexy. Eur J Endocrinol 2015;172:R179-90.  Back to cited text no. 7
    
8.
Glezer A, Bronstein MD. Pituitary apoplexy: Pathophysiology, diagnosis and management. Arch Endocrinol Metab 2015;59:259-64.  Back to cited text no. 8
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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