Document Detail

Infrasellar pituitary gangliocytoma causing Cushing's syndrome.
MedLine Citation:
PMID:  25183169     Owner:  NLM     Status:  Publisher    
INTRODUCTION: Pituitary gangliocytomas are uncommon neuronal tumours that may present with endocrine disorders, the most frequent being acromegaly caused by growth hormone hypersecretion. Cushing's syndrome is very rarely seen with gangliocytomas.
MATERIAL AND METHODS: We report the unique case of a 62 year-old woman whose clinical picture and endocrine testing clearly demonstrated adrenocorticotropin (ACTH)-dependent Cushing's syndrome. Pituitary magnetic resonance imaging showed a 12-mm homogeneous, infra- and retrosellar mass first diagnosed as pituitary macroadenoma. Transsphenoidal surgery was performed and allowed complete resection of the tumour with sparing of normal anterior pituitary. Very low postoperative serum cortisol and ACTH levels were observed in the early postoperative period and the patient is still in remission 18 months after surgery, thus demonstrating that the resected lesion was entirely responsible for the clinical picture.
RESULTS: Histological and immunocytochemical analyses demonstrated a benign tumour composed of mature neuronal cells suggestive of a gangliocytoma, expressing both ACTH and corticotropin-releasing hormone (CRH). The tumour was surrounded by a rim of pituitary tissue containing ACTH-producing endocrine cells. Careful analysis of the resected lesion did not reveal any pituitary microadenoma. We search literature for similar cases and retraced only nine cases of gangliocytomas associated with Cushing's syndrome. In most of them, the tumour was combined with either pituitary corticotroph adenoma or hyperplasia.
CONCLUSIONS: Our case represents a unique case of an infrasellar pituitary gangliocytoma which was able to cause Cushing's syndrome by both direct ACTH production and CRH-induced stimulation of neighbour normal corticotroph cells.
Marie-Eve Domingue; Etienne Marbaix; Jean-Luc Do Rego; Vincent Col; Christian Raftopoulos; Thierry Duprez; Hubert Vaudry; Dominique Maiter
Related Documents :
21373909 - The scaphocapitate fracture syndrome: report of a case and a review of the literature.
21820839 - Contiguous bilateral head and neck paragangliomas in a carrier of the sdhb germline mut...
21180489 - Partial kluver-bucy syndrome as a delayed manifestation of head injury.
2253249 - Acute poststreptococcal glomerulonephritis and acute rheumatic fever: occurrence in the...
14570349 - Primary human immunodeficiency virus type 1 infection in a patient with acute rhabdomyo...
24881749 - Neuroleptic malignant syndrome induced by combination therapy with tetrabenazine and ti...
18370699 - Relationship between antihypertensive drugs and metabolic syndrome.
24962639 - Phace syndrome, a series of six patients: clinical and morphological manifestations, pr...
24343879 - Prenatal ultrasound findings observed in the wolf-hirschhorn syndrome: data from the re...
Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2014-9-3
Journal Detail:
Title:  Pituitary     Volume:  -     ISSN:  1573-7403     ISO Abbreviation:  Pituitary     Publication Date:  2014 Sep 
Date Detail:
Created Date:  2014-9-3     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9814578     Medline TA:  Pituitary     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

Previous Document:  Do you feel alright? Attenuated neural processing of aversive interoceptive stimuli in current stimu...
Next Document:  Activation of CD1d-restricted natural killer T cells can inhibit cancer cell proliferation during ch...