Endocrine Abstracts

We report a case of multisystem Langerhans cell histiocytosis (LCH) with pituitary involvement nearly 20 years after initial presentation. LCH is a rare disorder in which granulomatous deposits occur at multiple sites within the body often involving the hypothalamo-pituitary axis.

A 48-year-old gentleman had Histiocytosis X initially involving groin in1987, responding poorly to treatment with topical nitrogen mustard and radiotherapy. Involvement of external auditory canals in 1988 requiring several meatoplasties. Scalp involvement in 1990 requiring chemotherapy. He then developed gum and mandibular disease in 1992, which was resected. Perianal disease treated with pelvic radiotherapy in 1993. Relapse in axilla 1995 and pituitary involvement diagnosed November 2006. He also has hypothyroidism and Type 2 diabetes mellitus (diet controlled) both diagnosed in 2001.

In 2006 he presented with erectile dysfunction, initial investigations showed low LH, FSH and testosterone. TRH test showed delayed response suggestive of hypothalamic disease. Prolactin levels were normal. GnRH Test showed impaired testosterone and gonadotrophin response in keeping with pituitary disease. A glucagon stimulation test showed an impaired growth hormone response, but a normal cortisol increase. MRI pituitary showed an empty sella. There was no evidence of diabetes insipidus (DI). Bone mineral densitometry (BMD) was normal. He has good glycemic control.

Our patient has partial hypopituitarism. He was already on levothyroxine, testosterone injections were started. We plan to review his symptomatology regarding growth hormone deficiency once other hormone deficiencies are corrected.

Twenty percent of patients with multisystem involvement have a progressive disease course despite treatment. The cases reported in literature had DI and most had panhypopituitarism. Our patient currently doesn’t have DI. Ongoing monitoring needs to check for this developing and for the occurrence of pan- hypopituitarism