Langerhans' cell histiocytosis in an adult patient manifested as recurrent skull lesions and Diabetes Insipidus.

We describe a 57-year old female with the diagnosis of skull and meningeal Langerhans' cell histiocytosis who was treated with the combination of azathioprine and methotrexate. Although the skull lesions improved considerably on this regimen, the patient developed diabetes insipidus while the anterior pituitary function remained intact.

Growth without growth hormone (GH): a case report.

Growth without growth hormone (GH) has occasionally been described in patients with organic pituitary pathology, and even more rarely in patients with idiopathic pituitary hormone deficiency. The mechanism of growth without GH remains a mystery. We describe a 17-year old male who grew 38.5 cm in height over a 7-year period, despite the fact that he had established panhypopituitarism. The hypopituitarism was initially attributed to a presumptive hypothalamic hamartoma which was not, however, confirmed on subsequent and prolonged follow-up. Regular endocrine evaluation confirmed persistent anterior and posterior pituitary hormonal deficiencies with severe concomitant hyperinsulinemia as shown by an exaggerated insulin response to a standard oral glucose tolerance test. In our patient, a postulated mechanism could be the severe hyperinsulinemia, acting either through the insulin and/or IGF receptors and thus potentiating the mitogenic effect. This case illustrates that final height attainment within or above target height may occur in patients with idiopathic pituitary hormonal deficiency despite persistent, severe GH insufficiency.

Treatment of advanced neuroendocrine tumours with the radiolabelled somatostatin analogue octreotide.

Neuroendocrine tumours (NET) have a particular tendency to express functional receptors and/or uptake mechanisms. Radionuclides, such as (111)In-pentetreotide, a somatostatin analogue, which bind to somatostatin receptors, present an imaging modality that has been used for both the diagnosis and staging of NET. Scintigraphy with (111)In-pentetreotide can identify lesions beyond the diagnostic sensitivity of conventional imaging modalities. In addition, NET that demonstrate positive uptake to a diagnostic (111)In-pentetreotide can, in theory, be treated with these radionuclides, thus presenting a novel and evolving therapeutic modality in addition to other traditional therapeutic approaches. Although experience with (90)Y-DOTA-D-Phe(1)-Tyr(3)-octreotide therapy is still limited, preliminary studies have demonstrated useful activity in a variety of NET with limited side-effects. Large phase II clinical trials using (90)Y-DOTA-D-Phe(1)-Tyr(3)-octreotide therapy are still on-going and the results are expected to be presented soon. In order to improve the response rates obtained, newer somatostatin analogues are being developed and the combination with other beta-emitting particles in addition to (90)Y is being considered.