Xanthogranulomatous Hypophysitis: A Rare And Often Mistaken ...

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Abstract

Xanthogranulomatous hypophysitis (XGH) is a very rare form of pituitary hypophysitis that may present both clinically and radiologically as a neoplastic lesion. It may either be primary with an autoimmune aetiology and can occur in isolation or as a part of autoimmune systemic disease or secondary as a reactive degenerative response to an epithelial lesion (e.g. craniopharyngioma (CP), Rathke's cleft cyst, germinoma and pituitary adenomas) or as a part of a multiorgan systemic involvement such as tuberculosis, sarcoidosis or granulomatosis. It may also present with a variation of symptoms in children and adults. Our case series compares the paediatric and adult presentations of XGH and the differential diagnoses considered in one child and two adult patients, highlighting the wide spectrum of this condition. Endocrine investigations suggested panhypopituitarism in all three patients and imaging revealed a suprasellar mass compressing the optic chiasm suggestive of CP or Rathke's cleft cyst in one patient and non-functioning pituitary macroadenoma in two patients. Magnetic resonance imaging (MRI) demonstrated mixed signal intensities on T1- and T2-weighted sequences. Following endoscopic transsphenoidal surgery, histological analysis revealed necrotic material with a xanthogranulomatous reaction confirming XGH in two patients and a necrobiotic granulomatous chronic inflammatory infiltrate with neutrophils in one patient, which is not typical of current descriptions of this disorder. This case series describes the wide spectrum of XGH disease that is yet to be defined. Mixed signal intensities on T1- and T2-weighted MRI sequences may indicate XGH and diagnosis is confirmed by histology. Histological variation may indicate an underlying systemic process.

Learning points: XGH is a rare form of pituitary hypophysitis with a wide clinical and histological spectrum and can mimic a neoplastic lesion.XGH primarily presents with growth arrest in children and pubertal arrest in adolescents. In adults, the presentation may vary.A combination of hypopituitarism and mixed signal intensity lesion on MRI is suggestive of XGH and should be considered in the differential diagnosis of sellar lesions.Radical surgery is the treatment of choice and carries an excellent prognosis with no recurrence.

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Figures

Figure 1

Figure 1

(A and B) MRI image…

Figure 1

(A and B) MRI image of patient 1 demonstrating the pituitary lesion pre-surgery.

Figure 1 (A and B) MRI image of patient 1 demonstrating the pituitary lesion pre-surgery.
Figure 4

Figure 4

(A) Patient 1. Haematoxylin and…

Figure 4

(A) Patient 1. Haematoxylin and eosin-stained section showing numerous cholesterol clefts (arrowheads). Brown,…

Figure 4 (A) Patient 1. Haematoxylin and eosin-stained section showing numerous cholesterol clefts (arrowheads). Brown, haemosiderin pigment is present (arrow) and there is an area of necrosis (star). Scale bar, 100 μm. (B and C) Patient 2. In addition to cholesterol clefts, there is an aggregate of multinucleate giant cells (arrow, B). In C, the cholesterol clefts are associated with new fibrosis. Scale bars, 100 μm. (D, E and F) Patient 3, first biopsy. An area of lymphoplasmacytic cells surrounded by paler macrophages, with a loose, vague granulomatous appearance (D, scale bar, 100 μm). In areas, foamy macrophages are admixed with neutrophils (E, scale bar, 50 μm), while F shows a somewhat foamy multinucleate macrophage (arrow) in a background of lymphocytes, histiocytes and plasma cells (scale bar, 50 μm). (G and H) Patient 3, second biopsy. Low-power view (G) showing dense fibrous tissues with a lymphoplasmacytic infiltrate. There is an area of necrosis towards the top of the field (scale bar, 100 μm). At high power (H), there is a prominent foamy macrophage infiltrate with admixed lymphocytes and histiocytes.
Figure 2

Figure 2

(A and B) MRI image…

Figure 2

(A and B) MRI image of patient 2 demonstrating the pituitary lesion pre-surgery.…

Figure 2 (A and B) MRI image of patient 2 demonstrating the pituitary lesion pre-surgery. (C) MRI image of patient 2 demonstrating the pituitary lesion post-surgery.
Figure 3

Figure 3

(A and B) MRI image…

Figure 3

(A and B) MRI image of patient 3 demonstrating the pituitary lesion post-surgery.

Figure 3 (A and B) MRI image of patient 3 demonstrating the pituitary lesion post-surgery.
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