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  Table of Contents    
CASE REPORT  
Year : 2019  |  Volume : 62  |  Issue : 3  |  Page : 448-450
Xanthomatous hypophysitis: A rare case report with review of literature


1 Department of Pathology, Kasturba Medical College, Mangalore, Manipal Academy of Higher Education, Karnataka, India
2 Department of Neurosurgery, Kasturba Medical College, Mangalore, Manipal Academy of Higher Education, Karnataka, India

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Date of Web Publication26-Jul-2019
 

   Abstract 


Hypophysitis is classified into primary and secondary. Xanthomatous hypophysitis is one of the rare types of primary hypophysitis. A 55-year-old female presented with headache, vomiting, and blurring of vision. She also had endocrine dysfunction in the form of low serum T3, T4, and low cortisol levels. MRI scan showed a sellar expansile lesion suggestive of pituitary macroadenoma. Microscopy showed pituitary tissue replaced by inflammatory infiltrate made up of foamy histiocytes arranged in sheets along with lymphoplasmacytic infiltrate. Interspersed areas of fibrosis, hyalinization, few congested and sclerosed blood vessels were seen. Compressed residual pituitary tissue was identified at the periphery. Xanthomatous hypophysitis is a rare entity which can mimic as pituitary adenoma both clinically and radiologically. Accurate diagnosis at an early stage with postsurgical steroid therapy may help to prevent permanent pituitary damage.

Keywords: Hypophysitis, pituitary adenoma, xanthogranulomatous

How to cite this article:
Kini H, Rao R, Pai M. Xanthomatous hypophysitis: A rare case report with review of literature. Indian J Pathol Microbiol 2019;62:448-50

How to cite this URL:
Kini H, Rao R, Pai M. Xanthomatous hypophysitis: A rare case report with review of literature. Indian J Pathol Microbiol [serial online] 2019 [cited 2019 Aug 21];62:448-50. Available from: http://www.ijpmonline.org/text.asp?2019/62/3/448/263477





   Introduction Top


To date more than 100 cases of hypophysitis have been documented.[1] While many cases have been described postmortem several are now being diagnosed antemortem.

Primary and secondary hypophysitis are recognized. Primary hypophysitis (PH) is characterized by chronic inflammation that primarily involves the pituitary gland. Based on the histological findings lymphocytic hypophysitis (LH), granulomatous hypophysitis (GH), xanthomatous hypophysitis (XH), xanthogranulomatous hypophysitis (XGH), and necrotizing (NH) variants occur. Of these LH is the commonest while XH is the least common.[1],[2]

On the contrary, secondary hypophysitis results from mass effect due to local lesions like germinoma, Rathke's cleft cyst, craniopharyngioma, pituitary adenomas, and systemic diseases like tuberculosis, syphilis, sarcoidosis, and Wegener's granulomatosis.[3]

These inflammatory lesions may mimic pituitary adenomas clinically, radiologically, and endocrinologically. Unlike pituitary adenomas, these inflammatory lesions generally have decreased hormonal function in those cases that present with endocrinal abnormalities. Sometimes diagnosing these lesions with appropriate tests may be difficult. While the current imaging modalities like magnetic resonance imaging (MRI) can be used to diagnose these lesions, experience, and a high index of suspicion is required for accuracy.[1]

Here, we report a 55-year-old woman who presented with hypopitutarism clinically, that was attributed to be due to a pituitary macroadenoma radiologically, but histological features showed that it was primary XH.


   Case Report Top


A 55-year-old female presented with the complaints of vomiting and headache of 4 days duration. She also had blurring of vision. On clinical examination she had bitemporal hemianopia. Higher mental functions and cranial nerve examination was normal. Laboratory tests revealed low serum T3 (0.263 ng/dL), T4 (4.38 μg/dL), TSH (1.79 mlU/L), and serum cortisol levels (0.794 μg/dL). Her ESR was high (90 mm/h). MRI scan showed a sellar expansile lesion suggestive of pituitary macroadenoma. Transsphenoidal endoscopic resection of the tumor was done and sent for histopathological examination. Grossly multiple irregular, grey white tissue bits amounting to 1 cm was received. Microscopy showed pituitary tissue replaced by inflammatory infiltrate made up of foamy histiocytes arranged in sheets along with lymphoplasmacytic infiltrate [Figure 1]a and [Figure 1]b. Interspersed areas of fibrosis, hyalinization, few congested and sclerosed blood vessels were seen [Figure 1]c and [Figure 1]d. There was absence of any cyst lining with collumnar ciliated epithelium to suggest ruptured Rathke's cleft cyst. Compressed residual pituitary tissue was identified at the periphery [Figure 2]a. The foamy macrophages showed positive staining with CD68 marker on immunohistochemistry [Figure 2]b.
Figure 1: (a) Showing sheets of foamy macrophages with adjacent fibrous areas (H and E, ×10). Inset shows high power view of the foamy macrophages (H and E, ×40). (b) Showing chronic inflammatory infiltrate consisting of lymphocytes and plasma cells. A sclerosed blood vessel also seen (H and E, ×10). (c) Areas showing fibrosis with inflammation (H and E, ×10). (d) Showing sclerosed blood vessels (H and E, ×10)

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Figure 2: (a) Showing fibrotic area with adjacent compressed pituitary tissue (H and E, ×10). Inset shows high power view of the pituitary cells. (b) Foamy macrophages showing positive staining with CD68 on immunohistochemistry (IHC, ×10). Inset shows high power view of the macrophage with cytoplasmic positivity for CD68

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   Discussion Top


Hypophysitis is an infrequent cause of pituitary mass lesions that is often mistaken to be a pituitary adenoma both clinically and radiologically. It can involve pituitary gland as well as pituitary stalk.[4] The clinical presentation of PH is variable and are mainly due to inflammatory irritation of sellar and parasellar structures.[5] The clinical features include four categories of symptoms including: sellar compression, hypopituitarism, diabetes insipidus, and hyperprolactinemia.

Sellar compression results in headache, nausea, vomiting, and blurred vision. Endocrine dysfunction is also common due to partial or complete deficit of the anterior pituitary hormones, mainly adrenocorticotropic hormone followed by TSH, gonadotropins, and prolactin. One-third of patients can have diabetes insipidus due to deficiency of the posterior pituitary hormones.[5]

Hypophysitis is subclassified into LH, GH, XH, XGH, and NH variants.[5] According to the site of inflammation, hypophysitis can be further subdivided into adenohypophysitis and infundibuloneurohypophysitis.

XH was first reported in 1998. In literature, a very few number of XH cases have been reported so far.[5] XH has been shown to have a predilection for young women.[6] Current knowledge of their etiopathogenesis, natural course, and prognosis are limited in the literature.

Xanthoma cells (lipid-containing cells) are uncommonly seen in hypophyseal lesions. It can be seen in both primary as well as secondary hypophysitis. In primary conditions XH, XGH, xanthogranuloma of the sellar region all represent continued spectrum of the same disease process.[7]

The histological features of XH consist of foamy histiocytic infiltration of the adenohypophysis, small lymphocytes, and cyst like areas of liquefaction but there should not be true cyst formation, granulomas, or adenoma. Fibrosis and hemosiderin pigments were later added as additional features of XH.[7] The foamy cells are histiocytes and are positive for CD68 and negative for CD1a and S100. In our case also histology revealed foamy histiocytes in sheets, fibrosis and lymphoplasmacytic infiltrate, and few congested sclerosed blood vessels.

Xanthogranulomatous inflammation (also known as cholesterol granuloma) is commonly known in extrapituitary sites like middle ear, mastoid, paranasal sinuses, and choroid plexus. They have also been reported in association with adamantinomatous craniopharyngioma and they represent tissue reaction to hemorrhage and degeneration.[7]

Clinical and laboratory findings suggest an autoimmune basis for PH[5] LH is also known as autoimmune hypophysitis because of its association with other autoimmune diseases, presence of lymphoplasmacytic infiltrate in the histopathology and response to steroid therapy. Rare entities like Castleman's disease accompanied by lymphocytic thyroiditis has been reported in the literature.[8] Lymphocytic adenohypophysitis is commonly seen in females in their third to fourth decade of life often in late pregnancy or in postpartum period.[9]

In contrast to this, XH in association with other autoimmune diseases is rarely reported in the literature. Only two cases are documented. One was associated with ulcerative colitis and the other with Hashimoto's thyroiditis.[6]

Duan et al.[10] in his study identified seven cases of XH out of 1221 pituitary specimens. Of these seven cases six cases had concurrent evidence of ruptured Rathke's cleft cyst. Thus XH could be the result of reactive response to the ruptured cyst contents.

XH has got overall good prognosis if treated promptly through transsphenoidal resection of the mass. The resumption of the normal endocrine function however may be delayed or permanently lost in some cases. Information on long-term follow up of these patients is limited. Use of steroid therapy in XH is not well documented unlike lymphocytic/autoimmune hypophysitis.

In conclusion XH may clinically and radiologically mimic a pituitary adenoma. Accurate diagnosis by being aware of this entity and postsurgical steroid therapy may assist in preserving pituitary function in some cases.

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.
Tashiro T, Sano T, Xu B, Wakatsuki S, Kagawa N, Nishioka H, et al. Spectrum of different types of hypophysitis: A clinicopathologic study of hypophysitis in 31 cases. Endocr Pathol 2002;13:183-95.  Back to cited text no. 1
    
2.
Gutenberg A, Caturegli P, Metz I, Martinez R, Mohr A, Brück W, et al. Necrotizing infundibulo-hypophysitis: An entity too rare to be true? Pituitary 2012;15:202-8.  Back to cited text no. 2
    
3.
Caturegli P, Newschaffer C, Olivi A, Pomper MG, Burger PC, Rose NR, et al. Autoimmune hypophysitis. Endocr Rev 2005;26:599-614.  Back to cited text no. 3
    
4.
Laws ER, Vance ML, Jane JA Jr. Hypophysitis. Pituitary 2006;9:331-3.  Back to cited text no. 4
    
5.
Aste L, Bellinzona M, Meleddu V, Farci G, Manieli C, Godano U, et al. Xanthomatous hypophysitis mimicking a pituitary adenoma: Case report and review of the literature. J Oncol 2010;2010:195323.  Back to cited text no. 5
    
6.
Joung JY, Jeong H, Cho YY, Huh K, Suh YL, Kim KW, et al. Steroid responsive xanthomatous hypophysitis associated with autoimmune thyroiditis: A case report. Endocrinol Metab (Seoul) 2013;28:65-9.  Back to cited text no. 6
    
7.
Burt MG, Morey AL, Turner JJ, Pell M, Sheehy JP, Ho KK, et al. Xanthomatous pituitary lesions: A report of two cases and review of the literature. Pituitary 2003;6:161-8.  Back to cited text no. 7
    
8.
Luo C, Lu Y, Jiang L, Wu X. Castleman's disease with autoimmune hypophysitis. Acta Neurochir (Wien) 2010;152:1811-2.  Back to cited text no. 8
    
9.
La Torre D, Falorni A, Bocci EB, Gerli R. Autoimmune hypophysitis. In: Shoenfeld Y, Cervera R, Gershwin ME, editors. Diagnostic Criteria in Autoimmune Diseases. 1st ed. New York: Humana Press; 2008. p. 255-8.  Back to cited text no. 9
    
10.
Duan K, Asa SL, Winer D, Gelareh Z, Gentili F, Mete O, et al. Xanthomatous hypophysitis is associated with ruptured rathke's cleft cyst. Endocr Pathol 2017;28:83-90.  Back to cited text no. 10
    

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Correspondence Address:
Ranjitha Rao
Department of Pathology, Kasturba Medical College, Light House Hill Road, Mangalore - 575 001, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/IJPM.IJPM_319_18

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