title: Hypothalamic germinoma masquerading as superior mesenteric artery (SMA) syndrome.
abstract: OBJECTIVE
  To report a case of superior mesenteric artery (SMA) syndrome secondary to hypothalamic germinoma.
  
  
  METHODS
  We describe the clinical presentation, diagnostic work-up, management, and clinical course of a patient admitted with SMA syndrome who was subsequently found to have a hypothalamic germinoma.
  
  
  RESULTS
  An adolescent boy was admitted to the surgical ward with progressive weight loss over a 2 year period and postprandial vomiting. He was diagnosed with SMA syndrome based on evidence of proximal duodenal dilatation, extrinsic compression of the distal duodenum, and a narrowed aortomesenteric angle (16°). Investigations performed to exclude thyrotoxicosis unexpectedly revealed secondary hypothyroidism and further evaluation demonstrated evidence of pan-hypopituitarism. Psychiatric evaluation excluded anorexia nervosa and bulimia. Magnetic resonance imaging (MRI) of the brain revealed a heterogeneously enhancing hypothalamic lesion, but a normal pituitary gland. Hormone replacement with hydrocortisone, desmopressin, testosterone, and thyroxine resulted in weight gain and resolution of gastrointestinal symptoms. A transventricular endoscopic biopsy subsequently confirmed a hypothalamic germinoma and he was referred to an oncologist.
  
  
  CONCLUSIONS
  SMA syndrome secondary to severe weight loss is an uncommon cause of upper gastrointestinal obstruction. While there have been reports of poorly controlled diabetes mellitus and thyrotoxicosis manifesting as SMA syndrome, there are no published reports to date of SMA syndrome secondary to hypothalamic/pituitary disease. Management of SMA syndrome is conservative, as symptoms of intestinal obstruction resolve with weight gain following treatment of the underlying cause. Awareness of this uncommon presentation of endocrine cachexia/hypothalamic disease will prevent unnecessary laparotomies and a misdiagnosis of an eating disorder.
authors.affiliation: Department of Medicine, University of Malaya, Kuala Lumpur, Malaysia. shireene.vethakkan@gmail.com
authors.forename: Shireene R
authors.initials: SR
authors.lastname: Vethakkan
authors.forename: Yogeswari
authors.initials: Y
authors.lastname: Venugopal
authors.forename: Alexander T B
authors.initials: AT
authors.lastname: Tan
authors.forename: Sharmila S
authors.initials: SS
authors.lastname: Paramasivam
authors.forename: Jeyakantha
authors.initials: J
authors.lastname: Ratnasingam
authors.forename: Rohaya A
authors.initials: RA
authors.lastname: Razak
authors.forename: Azmi
authors.initials: A
authors.lastname: Alias
authors.forename: Fauziah
authors.initials: F
authors.lastname: Kassim
authors.forename: Karen
authors.initials: K
authors.lastname: Choong
mesh_terms: D001932:Brain Neoplasms / Q000150:complications* / Q000000981:diagnostic imaging; D018237:Germinoma / Q000150:complications* / Q000000981:diagnostic imaging; D006801:Humans; D007031:Hypothalamus / Q000000981:diagnostic imaging*; D008297:Male; D011859:Radiography; D013478:Superior Mesenteric Artery Syndrome / Q000000981:diagnostic imaging / Q000209:etiology*; D055815:Young Adult