Symptoma

Carney Triad

Carney's Syndrome

A 26-year-old woman presented with dizziness and several episodes of melena over 3 days. Physical examination revealed pale conjunctiva but normal hemodynamic status. [ncbi.nlm.nih.gov]

[…] is the most frequently present. [scielo.br]

Clinical description Most patients initially present with two of the three tumors (incomplete Carney's triad). The main symptoms at presentation are gastrointestinal bleeding, epigastric pain, anemia and palpable abdominal mass. [orpha.net]

  • Fishing

    CGH, fluorescent in situ hybridization (FISH) and loss-of-heterozygosity (LOH) studies Tumours that showed losses of 1q region by CGH (samples CTRS9, CTRS5T, CTRS6, CTRS7, CTRS10, CTRS13, CTRS19, CTRS21 and CTRS36) were subjected to interphase FISH, using [ncbi.nlm.nih.gov]

  • Marfanoid Habitus

    MEN2B ( 4 ), one of the syndromes, also included nonendocrine tumors, mucosal neuromas, and congenital anomalies, including marfanoid habitus and other connective tissue abnormalities. [academic.oup.com]

  • Hyperthermia

    Adjuvant therapy, including radiation, chemotherapy, and hyperthermia, was ineffective for the primary tumor and its metastases. [academic.oup.com]

  • Recurrent Infection

    All except one of the patients studied were asymptomatic. (3) In our patient, the pulmonary component appeared as an endobronchial obstructive lesion, leading to signs and symptoms of pulmonary suppurative disease with recurrent infections. [scielo.br]

  • Papilledema

    Typical and atypical Carney’s triad presenting with malignant hypertension and papilledema. J Pediatr Hematol Oncol. 2001 Nov;23(8):519-24. PMID: 11878780 Vogl TJ, Lehnert T, Wetter A, Mack MG, Wurster MG. Interventional radiology in Carney triad. [gistsupport.org]

Metaiodobenzylguanidine scanning may be helpful in the diagnostic workup. [journals.aace.com]

  • Chest X-Ray Normal

    Table 1 Patients with the triad of gastric leiomyosarcoma, functioning extra-adrenal paraganglioma and pulmonary chondroma (Carney triad) Patient Id Sex Age Tumours Other Reference CTRS2 1 F 15 GIST, PGL Chest X-ray normal; metastatic GIST; multiple PGLs [ncbi.nlm.nih.gov]

  • Gastric Filling Defect

    Imaging studies showed one or more gastric filling defects, sometimes with evidence of ulceration. [academic.oup.com]

  • Atelectasis

    A computed tomography scan of the chest confirmed atelectasis and revealed a small calcified lesion in the right lower lobe bronchus ( Figure 2 ), in addition to bronchiectasis with probable impacted mucus in the collapsed pulmonary lobe. [scielo.br]

  • Gastric Lesion

    Whole body magnetic resonance scan performed to investigate the possibility of Carney triad demonstrated a gastric lesion. This was resected and found to be a gastrointestinal stromal tumor. No evidence of paragangliomata was found on imaging. [ncbi.nlm.nih.gov]

    Abdominal CT showed an exophytic gastric lesion ( Fig. 1 D). Laparoscopy with gastric biopsy demonstrated that this lesion was a gastrointestinal stromal tumor (GIST). Total gastrectomy was performed. The patient was discharged in good condition. [journalpulmonology.org]

    The gastric lesion was usually the presenting tumor (75%), followed by the lung lesion (15%) and the paraganglionic tumor (10%). Twenty percent of the patients had adrenocortical adenoma(s), and 10% had esophageal leiomyoma(s). [academic.oup.com]

Management and treatment The treatment of choice for GIST and its metastases (overall rate: 50%) is surgical resection. GIST associated with Carney's triad are mostly indolent. [orpha.net]

GISTs of the Carney triad involve predominantly the body and the antrum of the stomach, are generally multifocal, and have a better prognosis than sporadic GISTs. [ncbi.nlm.nih.gov]

The prognosis is better than in adult GIST, with a slow course of progression even after metastasis has occurred. However, many patients require repeated surgeries for recurring tumors. [gistsupport.org]

Prognosis CT is a chronic, persistent and indolent disease (Carney, 1999). The majority of patients survive for many decades either apparently disease-free or with hepatic or peritoneal metastasis. [atlasgeneticsoncology.org]

The prognosis of Carney triad is known to be chronic, persistent and indolent. [eymj.org]

Carney triad is a rare syndrome of unknown etiology characterized by having at least two out of three following neoplasms: gastrointestinal stromal tumor, pulmonary chondroma and extra-adrenal paraganglioma. [ncbi.nlm.nih.gov]

[…] doi: 10.1097/PCR.0000000000000175 Case Reviews Abstract Author Information Authors Article Metrics Metrics Carney triad is a rare, multitumoral, nonfamilial syndrome of unknown etiology. [journals.lww.com]

Summary Epidemiology Less than 100 cases have been reported worldwide. Carney's triad primarily affects young women (mean age of onset 20 years). [orpha.net]

Epidemiology Less than 100 cases have been reported worldwide. Carney's triad primarily affects young women (mean age of onset 20 years). Clinical description Most patients initially present with two of the three tumors (incomplete Carney's triad). [rarediseases.info.nih.gov]

(See Pathophysiology and Etiology.) [emedicine.medscape.com]

The pathophysiology and genetics of congenital lipoid adrenal hyperplasia. N Engl J Med 335: 1870, 2005. CrossRef Google Scholar 9. Andersson HC, Frentz J, Martinez JE, Tuck-Muller CM, Bellizaire J. [link.springer.com]

View Article PubMed Google Scholar Rozanski A, Blumenthal JA, Davidson KW, Saab PG, Kubzansky L: The epidemiology, pathophysiology, and management of psychosocial risk factors in cardiac practice: the emerging field of behavioral cardiology. [trialsjournal.biomedcentral.com]

If stenting prevents the progression of chronic kidney disease and lowers blood pressure, it has the potential to prevent serious health consequences, including adverse cardiovascular and renal events. [nejm.org]

Because of its association with poor mental and cardiovascular health, the prevention of PTSD development after MI is of high clinical relevance. [trialsjournal.biomedcentral.com]

Women with Carney complex may be at a high risk for recurrent atrial myxomas that lead to multiple strokes. [22] Early identification of a female patient with Carney complex may facilitate stroke prevention. [emedicine.medscape.com]