| Popis: |
A 34-year-old woman presented eighteen months post-partum with blurred vision, polyuria, amenorrhoea, headache and general malaise. Comprehensive clinical examination revealed left superior temporal visual loss only. Investigations revealed pan-hypopituitarism; and MRI demonstrated a sellar mass involving the infundibulum and hypothalamus. Lymphocytic hypophysitis was initially suspected, and high dose glucocorticoids were commenced along with desmopressin and thyroxine. However, her vision rapidly deteriorated. At surgical biopsy, an irresectable grey amorphous mass involving the optic chiasm was identified. Histopathology was initially reported as granulomatous hypophysitis. However, despite ongoing glucocorticoids, her vision worsened to light detection only. Histopathological review revised the diagnosis to partially treated lymphoma. A PET scan demonstrated avid uptake in the pituitary gland in addition to splenic involvement, lymphadenopathy above and below the diaphragm, and a bone lesion. Excisional biopsy of an impalpable infraclavicular lymph node confirmed nodular lymphocyte-predominant Hodgkin’s lymphoma [HL]. Hyper-CVAD chemotherapy was commenced, along with rituximab; fluid balance management during chemotherapy (with its requisite large fluid volumes) was extremely complex given her diabetes insipidus. The patient is now in clinical remission. Panhypopituitarism persists; however, her vision has recovered sufficiently for reading large print and driving. Lymphoma involving the pituitary gland – whether primary or secondary - is exceedingly rare; and almost all reported cases are non-Hodgkin’s lymphoma. We have been able to identify only three cases of HL with pituitary involvement presenting pre-mortem, and two cases of HL and pituitary involvement identified post-mortem. However, in none of these cases were pituitary symptoms or signs the initial presentation; and to the best of our knowledge this is the first reported case of Hodgkin’s lymphoma presenting initially as hypopituitarism. Unless otherwise noted, all abstracts presented at ENDO are embargoed until the date and time of presentation. For oral presentations, the abstracts are embargoed until the session begins. Abstracts presented at a news conference are embargoed until the date and time of the news conference. The Endocrine Society reserves the right to lift the embargo on specific abstracts that are selected for promotion prior to or during ENDO. |
