PMC9744597_01
Clinical image of the patient showing (a) gynaecomastia and absent body and facial hair (b) perianal ulcers.
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He was obese and had gynaecomastia (Figure 1(a)). After two and half years, patient developed painful perianal ulcers with serosanguineous discharge (Figure 1(b)).
MRI sella showing (a) enhancing lesion involving hypothalamus and infundibular stalk 14 x 8 mm, isointense on T1W and hypointense on T2W at 3 years after 1st presentation, and (b) increase in size of enhancing mass lesion in the upper part of stalk 20 x 17 x 15 mm, 2 years after biopsy.
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MRI sella was abnormal for loss of posterior pituitary bright spot but a normal stalk and anterior pituitary (Figure 2(a)). Repeated MRI showed an enhancing lesion involving the suprasellar area (hypothalamus) and thickened stalk (Figure 2(b)).
A panel of photomicrograph of perianal lesion showing sheets of epithelioid like cells with abundant amount of eosinophilic cytoplasm having mildly pleomorphic round to oval nuclei with nuclear grooving. Admixed with these cells are scattered eosinophils, lymphocytes, and plasma cells (a) (HE, x25). (b) The cell morphology is brought out better in high-power photomicrograph (HE, x400). (c) Immunohistochemical staining showing atypical histiocytic cells positive for CD1a, Langerin, and S-100 (not shown) confirming a diagnosis of Langerhans cell histiocytosis (by peroxidase anti-peroxidase, x500 on paraffin-embedded tissue sections).
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Immunohistochemical (IHC) studies showed atypical histiocytic cells which stained positively for CD1a, Langerin, and S-100, confirming the presence of Langerhans cells (Figure 3).