Rathke's cleft cyst associated with pituitary granulomatosis with polyangiitis: An unusual combination of hypothalamus-pituitary region pathologies

The authors present an unusual case of a patient suffering from visual deficit due to pituitary granulomatosis with polyangiitis (GPA) associated with Rathke's cleft cyst (RCC). The patient was referred to our Neurosurgery Department presenting right eye amaurosis, third cranial nerve palsy, and left temporal hemianopsia. Magnetic resonance imaging documented a sellar or suprasellar lesion with solid and cystic components. The dura mater of the skull base was also strongly enhanced. The patient underwent surgery. Histologic examination revealed RCC associated with pituitary GPA. To our knowledge, this is the first reported case of concomitant pituitary GPA and RCC. Pituitary involvement in GPA is rare, usually diagnosed in hormonal dysfunctions. The patient in case first presented optic chiasm compression, probably due to inflammation of both the pituitary gland and the previously asymptomatic RCC. We focus on the symptoms that led us to diagnose GPA pituitary involvement and on the peculiar and unusual Magnetic resonance imaging of the case presented.

• Publication year

  2017

• Venue

  Radiology Case Reports

• Publication date

  2017-10-31

• Fields of study

  Medicine

• Identifiers
  DOI 10.1016/j.radcr.2017.09.018PMID 29552263PMCID 5853151
• External record

  Open on Semantic Scholar

• Source metadata

  Semantic Scholar, PubMed

• No claims are published for this paper.

• No concepts are published for this paper.

• Pituitary Involvement in Granulomatosis With Polyangiitis

  2015influential reference
• Rathke’s cleft cysts

  2012cited by this paper
• Spontaneous involution of Rathke cleft cysts: is it rare or just underreported?

  2010cited by this paper
• Diabetes Insipidus as a Complication of Wegener's Granulomatosis and Its Treatment with Biologic Agents

  2009influential reference
• Treatment of antineutrophil cytoplasmic antibody associated vasculitis: a systematic review.

  2007cited by this paper
• Fatal inflammatory hypophysitis

  2007cited by this paper
• Off-label use of drug-eluting stents.

  2007cited by this paper
• Hypophysitis

  2006cited by this paper
• Central Nervous System Involvement in Wegener Granulomatosis

  2006cited by this paper
• Rathke's cleft cysts.

  2006cited by this paper
• The epidemiology of Wegener's granulomatosis. Estimates of the five-year period prevalence, annual mortality, and geographic disease distribution from population-based data sources.

  1996cited by this paper
• Neurological involvement in Wegener's granulomatosis: An analysis of 324 consecutive patients at the Mayo Clinic

  1993cited by this paper
• Experiences in transantrosphenoidal hypophysectomy.

  1961cited by this paper

• No citing papers are available for this paper.