Panhypopituitarism associated with severe retroperitoneal fibrosis.
A 43-year-old man, with a history of central diabetes insipidus diagnosed 3 years previously, complained about reduced libido. An MRI scan showed a suprasellar lesion just below the supraoptic recess of the third ventricle. A stereotactically guided biopsy revealed fibrous glia, but no other specific tissue and no inflammatory cells. Two months later the patient presented with fatigue and muscular weakness. Tertiary adrenal failure and hypothyroidism were diagnosed by endocrine function tests and therapy with levothyroxine and hydrocortisone was started. Another 2 months later the patient was admitted with giddiness, nausea, peripheral oedema and oliguria. Radiological imaging and an open transperitoneal kidney exploration showed severe fibrosis around both ureters. Histological examination confirmed the diagnosis of idiopathic retroperitoneal fibrosis. Presumably the suprasellar tumour was the first manifestation of retroperitoneal fibrosis. Once the diagnosis 'idiopathic retroperitoneal fibrosis' is confirmed, fibrotic manifestations and complications involving extra-retroperitoneal tissues including the endocrine system, should be sought.
Braun
J
J
Medizinische Klinik I, Schwerpunkt Endokrinologie, Klinikum der J.W. Goethe-Universität, Frankfurt am Main, Germany.
Schuldes
H
H
Berkefeld
J
J
Zanella
F
F
Jonas
D
D
Usadel
K H
KH
Badenhoop
K
K
D000328:Adult; D003346:Corticotropin-Releasing Hormone; D003520:Cyclophosphamide / Q000627:therapeutic use; D003919:Diabetes Insipidus / Q000097:blood / Q000150:complications; D004359:Drug Therapy, Combination; D005640:Follicle Stimulating Hormone / Q000097:blood; D006801:Humans; D006854:Hydrocortisone / Q000627:therapeutic use; D007018:Hypopituitarism / Q000097:blood / Q000175:diagnosis / Q000209:etiology*; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007986:Luteinizing Hormone / Q000097:blood; D008279:Magnetic Resonance Imaging; D008297:Male; D012185:Retroperitoneal Fibrosis / Q000097:blood / Q000150:complications* / Q000175:diagnosis; D013739:Testosterone / Q000097:blood; D013973:Thyrotropin-Releasing Hormone; D013974:Thyroxine / Q000627:therapeutic use
D007166:Immunosuppressive Agents; D013739:Testosterone; D013973:Thyrotropin-Releasing Hormone; D003520:Cyclophosphamide; D007986:Luteinizing Hormone; D005640:Follicle Stimulating Hormone; D003346:Corticotropin-Releasing Hormone; D013974:Thyroxine; D006854:Hydrocortisone