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LETTER TO EDITOR
Year : 2016  |  Volume : 64  |  Issue : 5  |  Page : 1075-1079

Xanthogranuloma of the sellar region


1 Department of Neurosurgery, Ataturk Research and Training Hospital, Bilkent, Ankara, Turkey
2 Department of Neurosurgery, Ataturk Research and Training Hospital, Bilkent; Universiteler Avenue, Bilkent Street, Ankara, Turkey
3 Department of Neurosurgery, Koc University School of Medicine, Istanbul, Turkey
4 Department of Neurosurgery, Sanliurfa Balikligol State Hospital, Sanliurfa, Turkey
5 Private Practicing Neurosurgeon, Ankara, Turkey
6 Department of Pathology, Ataturk Research and Training Hospital, Bilkent, Ankara, Turkey

Date of Web Publication12-Sep-2016

Correspondence Address:
Ismail Bozkurt
Department of Neurosurgery, Ataturk Research and Training Hospital, Bilkent; Universiteler Avenue, Bilkent Street, Ankara
Turkey
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.190238

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How to cite this article:
Gurcay AG, Gurcan O, Kazanci A, Bozkurt I, Senturk S, Ferat M, Turkoglu OF, Beskonakli E, Orhun Yavuz H S. Xanthogranuloma of the sellar region. Neurol India 2016;64:1075-9

How to cite this URL:
Gurcay AG, Gurcan O, Kazanci A, Bozkurt I, Senturk S, Ferat M, Turkoglu OF, Beskonakli E, Orhun Yavuz H S. Xanthogranuloma of the sellar region. Neurol India [serial online] 2016 [cited 2019 Aug 20];64:1075-9. Available from: http://www.neurologyindia.com/text.asp?2016/64/5/1075/190238




Sir,

Xanthogranulomas (XGs), also known as cholesterol granulomas or xanthogranulomatous reactions are rare, benign lesions. These are forms of granulomatous lesions that are characterized by cholesterol clefts, foreign body multinucleated giant cells, marked hemosiderin deposits, foamy macrophage (xanthoma cells) accumulation, and fibrous tissue proliferation.[1],[2] XGs are considered to be a secondary tissue reaction to degeneration, hemorrhage, or inflammation. XGs may be observed in a variety of sites, including the middle ear, mastoid bone, paranasal sinuses, and intracranial region; however, they are usually found in the choroid plexus located in the trigone of the lateral ventricle.[3],[4]

Intracranial XGs are usually asymptomatic lesions and are found in 1.6–7% of all the central nervous system autopsies.[5] A lesion which has cholesterol tissue properties in the sellar region could be highly indicative of a craniopharyngioma. Moreover, the adamantinomatous variant of a craniopharyngioma has cholesterol deposits. However, XGs have distinct properties from this variant.[2],[6] They are rarely located in the sellar region.[1] Herein, we report a case of XG found in the sellar region along with a review of the literature.

A 45-year-old woman was admitted to our clinic with complaint of severe headache that was localized to the left retro-orbital region. Her neurological examination revealed left oculomotor palsy with an inability to rotate the eye inwards, accompanied by diplopia. Her visual acuity was measured as being 6/6 bilaterally. There was no hormonal pathology. Cranial computed tomography (CT) revealed an isodense sellar and suprasellar mass lesion without any sign of calcification. Cranial magnetic resonance imaging (MRI) demonstrated an intra– as well as suprasellar mass lesion, extending into the cavernous sinus, which had compressed the optic chiasma. The lesion appeared as a homogenously isointense mass on both T1 and T2 weighted scans. The mass lesion was also homogeneously enhancing with gadolinium [Figure 1] and [Figure 2]. Considering the diagnosis of a nonfunctional pituitary adenoma, the endonasal transsphenoidal approach was adopted to excise the lesion. During surgery, the mass was yellowish-brown in color and was soft and fragile. The mass was almost completely excised.
Figure 1: Preoperative coronal view of MRI showing the sellar-supra sellar lesion with parasellar extension

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Figure 2: Preoperative sagittal view of MRI showing the sellar-suprasellar lesion

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At the time of discharge, the patient was relieved of her headache and visual disturbance. Her postoperative MRI at 15-months revealed no lesion [Figure 3] and {Figure 4].
Figure 3: Postoperative coronal view of MRI showing excision of the lesion

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Figure 4: Postoperative sagittal view of MRI showing the excised lesion

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The histological examination revealed a granulomatous tissue with cholesterol clefts and hemosiderin deposits. The pituitary tissue with histiocyte infiltration and granuloma formation could be seen [Figure 5],[Figure 6],[Figure 7]. The chromogranin stain was used to confirm the neuroendocrine cells of the pituitary gland [Figure 8]. The tissue had a fibrous component, xanthoma cells (foamy macrophages), and multinucleated giant cells without any sign of squamous epithelium, as seen in a craniopharyngioma; and, ciliated cuboidal epithelium, as seen in a Rathke's cleft cyst. Neither mitosis nor necrosis were observed. The immunohistochemical examination did not show any signs of a pituitary adenoma.
Figure 5: Hematoxylin eosin, ×40 magnification, pituitary tissue with histiocyte infiltration

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Figure 6: CD 68 stained, ×200 magnification, histiocytes with granuloma formation

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Figure 7: Periodic acid–Schiff (PAS) stained, ×100 magnification, showing granuloma like structures

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Figure 8: Chromogranin stained, ×40 magnification, showing neuroendocrine cells in the pituitary tissue

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The spectrum of tumors in the sella include pituitary adenomas, craniopharyngiomas, Rathke's cleft cysts, metastatic neoplasms, autoimmune diseases and granulomatous inflammation.

Paulus et al., reported a series of 110 patients with a craniopharyngioma with different histological findings, including 37 cases with xanthogranulomatous properties. They, therefore, classified XGs of the sellar region as a distinct entity. In 2000, the World Health Organization accepted that XG of the sellar region differs from the adamantinomatous variant of craniopharyngioma in its histological features, age of onset, symptoms, and prognosis.[1],[7]

The histogenesis of XG in the sellar region is not known. The numerous etiologies include hemorrhage, inflammation, nonspecific tissue reaction, and degenerative changes.[3],[8]

XGs often present with hormonal deficits (hypopituitarism), headache, visual field deficits (bitemporal hemianopsia), hydrocephalus (by displacing the third ventricle posteriorly and resulting in foramen of Monro obstruction), generalized fatigue, weight loss, polyuria, polydipsia, and changes in consciousness.[1],[3],[4],[6],[9],[10] This case presented with severe headache and left oculomotor palsy, with an inability to look inward, and double vision without any hormonal deficits. XGs of the sellar region are predominantly observed in adolescents and adults.[9] Our patient was an adult aged 45-years.

Preoperatively, it is difficult to differentiate XGs from other sellar mass lesions. On a CT scan, a craniopharyngioma typically appears as a moderate to large, partially calcified mass, whereas no calcification is observed in a XG. Rathke's cleft cysts also present as intrasellar masses. It has been stated that cholesterol clefts appear as high signal intensity lesions in T1 scans and as low signal intensity lesions in T2 scans; hemosiderin deposits show T1 isointense and T2-low-signal intensity; xanthochromic cyst fluid shows T1 high-iso and T2 high intensities; and, areas of fibrosis show both T1 and T2 low-signal intensities.[3] In this case, the radiological examination and cranial CT scan revealed a sellar and suprasellar mass lesion without any sign of calcification, and MRI showed the mass to be a homogeneous isointense lesion on T1- and T2-weighted scans. These findings were similar to that seen with the radiological appearance of a chronic hematoma, an adamantinomatous craniopharyngioma, and a Rathke's cleft cyst because these pathologies may also have cholesterol and hemosiderin deposits and fibrosis.[9],[11]

[Table 1] shows an overview of the present literature regarding XGs of the sellar region. Most of the histopathological analysis revealed similar results and neuroendocrinological findings.
Table 1: Literature review of xanthogranulomas of the sellar region

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However, XGs of the sellar region have some distinct characteristics such as cholesterol clefts, foreign-body multinucleated giant cells, marked hemosiderin deposits, foamy macrophage (xanthoma cells) accumulation, and fibrous tissue proliferation. These features also overlap with those in lesions that could be seen in the sellar region such as an adamantinomatous craniopharyngioma and a Rathke's cleft cyst. On the other hand, no signs of squamous epithelium and ciliated cuboidal epithelium were observed, thus, allowing a more precise differential diagnosis. The immunohistochemical examination may be used to conclusively differentiate the lesion from a pituitary adenoma.

In conclusion, the properties of XGs are not well defined and diagnosis mostly depends on the histopathological examination after surgical intervention. XGs should be kept in mind when considering the differential diagnosis of sellar mass lesions.[29]

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest

 
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2.
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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8]
 
 
    Tables

  [Table 1]

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