MES2008 Poster Presentations (1) (41 abstracts)
Lymphocytic hypophysitis secondary to a ruptured Rathke's cleft cyst (RCC): a diagnostic and management challenge
Puja Mehta 1 , Frederico Roncaroli 2 , Amrish Mehta 2 , Maneesh Bhojak 2 , James Lawrence 3 , Emma Hatfield 2 , Karim Meeran 1 & Waljit Dhillo 2
1Hammersmith Hospital, London, UK; 2Charing Cross Hospital, London, UK; 3Salisbury District Hospital, Salisbury, UK.
Hypophysitis describes inflammatory pituitary lesions which can be classified into lymphocytic (autoimmune), granulomatous or xanthomatous. Rathke’s cleft cysts (RCC) are usually assymptomatic, benign tumours derived from remnants of Rathke’s pouch. We present a case of lymphocytic hypophysitis secondary to a ruptured RCC and the first reported case of post-surgical recurrence.
A 34-year-old female presented with secondary amenorrhoea and fatigue. There were no features of infection, autoimmunity or granulomatous disease. Investigations revealed panhypopituitarism and she was prescribed replacement corticosteroids, thyroxine and HRT. She subsequently developed diabetes insipidus and was commenced on desmopressin. MRI scans showed expansion of a peripherally enhancing pituitary mass abutting the optic chiasm and transsphenoidal hypophysectomy was performed. Post-operative imaging demonstrated initial tumour debulking and then re-growth at 14 months with chiasmal compression, requiring a second hypophysectomy. Review of the histology confirmed a lymphocytic hypophysitis with underlying ruptured RCC.
Her most recent MRI scan (7 months after the second hypophysectomy) demonstrated further expansion of the pituitary mass, and a trial of high-dose prednisolone has been commenced, with repeat MRI planned to assess response. If there is a sub-optimal response, we will consider further surgery and/or radiotherapy.
The contents of a ruptured RCC is thought to drive a sterile inflammatory or autoimmune process. There have been nine cases in the literature of hypophysitis associated with a ruptured RCC, only three of these cases involved lymphocytic infiltrates. We describe the fourth reported case, who required multidisciplinary team management. Our patient is the first case to exhibit recurrence after surgical debulking. Treatment options include high dose steroids, surgery and radiotherapy, although there is no consensus in the literature regarding management.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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