Typical and atypical Carney's triad presenting with malignant hypertensionand papilledema

Citation
K. Valverde et al., Typical and atypical Carney's triad presenting with malignant hypertensionand papilledema, J PED H ONC, 23(8), 2001, pp. 519-524
Citations number
13
Categorie Soggetti
Pediatrics
Journal title
JOURNAL OF PEDIATRIC HEMATOLOGY ONCOLOGY
ISSN journal
10774114 → ACNP
Volume
23
Issue
8
Year of publication
2001
Pages
519 - 524
Database
ISI
SICI code
1077-4114(200111)23:8<519:TAACTP>2.0.ZU;2-N
Abstract
This report encourages clinicians to consider a diagnosis of Carney's triad in patients with multifocal gastric stromal sarcoma, extraadrenal paragang lioma (predominantly mediastinal), or pulmonary chondroma. The authors cond ucted a retrospective 20-year survey at the Hospital for Sick Children and identified two children with Carney's triad. One child, presenting atypical ly with papilledema and fundal hemorrhages from malignant hypertension and benign intracranial hypertension from chronic iron-deficiency anemia, is th e second patient ever to date be described with the complete Carney's triad of neoplasms at diagnosis. Another child presented more typically with gas tric stromal sarcoma and pulmonary chondroma without paraganglioma. Carney' s triad is a rare differential diagnosis for "idiopathic" hypertension or i ron-deficiency anemia from chronic gastrointestinal bleeding. If missed, pa tients with Carney's triad may have the debilitating physical and mental co nsequences of chronic iron deficiency and may die of untreated prolonged hy pertension and metastatic leiomyosarcoma.