Gastric stromal tumors in carney triad are different clinically, pathologically, and behaviorally from sporadic gastric gastrointestinal stromal tumors: Findings in 104 cases

Lizhi Zhang, Thomas Christopher Smyrk, William Francis Young, Constantine A. Stratakis, J. Aidan Carney

Research output: Contribution to journalArticle

136 Citations (Scopus)

Abstract

Carney triad, as originally described in 1977, was the association of 3 tumors: gastric epithelioid leiomyosarcoma [later renamed gastrointestinal stromal tumor (GIST)], extra-adrenal paraganglioma, and pulmonary chondroma. The disorder affected mostly young women and was not familial. We studied the clinical and pathologic features of the gastric neoplasm in 104 patients with the syndrome. Most (88%) were young women (mean age, 22 y), and the usual presentation was gastric bleeding. The tumors, commonly antral-based (61%), were multifocal, and ranged from 0.2 to 18.0 cm in dimension. Most (86%) featured round and polygonal (epithelioid) cells. Metastasis occurred in 49 patients (47%): to gastric lymph nodes (29%), liver (25%), and peritoneum (13%). Immunopositivity was detected in the tumors tested as follows: KIT, 100%; CD34, 75%; PKCθ, 21%; PDGFRA, 90%; and smooth muscle actin, 6%. Fourteen patients (13%) died of metastatic GIST at a mean age of 45 years (range, 30 to 69 y). Estimated 10 and 40-year survivals were 100% and 73%, respectively. Median survival time was 26.5 years (range, 16 to 60 y). There was no correlation between the National Institutes of Health tumor risk classification and the tumor behavior. Compared with sporadic gastric GISTs, the gastric stromal tumor in Carney triad showed distinctive features: female predilection, young patient age, epithelioid cell predominance, multifocality, frequent lymph node metastasis, serial tumor occurrence, and unpredictable behavior. Thus, the Carney triad gastric stromal tumor is different clinically, pathologically, and behaviorally from sporadic gastric GIST.

Original languageEnglish (US)
Pages (from-to)53-64
Number of pages12
JournalAmerican Journal of Surgical Pathology
Volume34
Issue number1
DOIs
StatePublished - Jan 2010

Fingerprint

Gastrointestinal Stromal Tumors
Stomach
Neoplasms
Epithelioid Cells
Lymph Nodes
Extra-Adrenal Paraganglioma
Neoplasm Metastasis
Chondroma
Survival
Leiomyosarcoma
Carney Triad
Peritoneum
National Institutes of Health (U.S.)
Stomach Neoplasms
Smooth Muscle
Actins
Hemorrhage
Lung
Liver

Keywords

  • Carney triad
  • Gastric gastrointestinal stromal tumor
  • Nonfamilial syndrome
  • Paraganglioma
  • Pulmonary chondroma

ASJC Scopus subject areas

  • Anatomy
  • Pathology and Forensic Medicine
  • Surgery
  • Medicine(all)

Cite this

Gastric stromal tumors in carney triad are different clinically, pathologically, and behaviorally from sporadic gastric gastrointestinal stromal tumors : Findings in 104 cases. / Zhang, Lizhi; Smyrk, Thomas Christopher; Young, William Francis; Stratakis, Constantine A.; Carney, J. Aidan.

In: American Journal of Surgical Pathology, Vol. 34, No. 1, 01.2010, p. 53-64.

Research output: Contribution to journalArticle

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abstract = "Carney triad, as originally described in 1977, was the association of 3 tumors: gastric epithelioid leiomyosarcoma [later renamed gastrointestinal stromal tumor (GIST)], extra-adrenal paraganglioma, and pulmonary chondroma. The disorder affected mostly young women and was not familial. We studied the clinical and pathologic features of the gastric neoplasm in 104 patients with the syndrome. Most (88{\%}) were young women (mean age, 22 y), and the usual presentation was gastric bleeding. The tumors, commonly antral-based (61{\%}), were multifocal, and ranged from 0.2 to 18.0 cm in dimension. Most (86{\%}) featured round and polygonal (epithelioid) cells. Metastasis occurred in 49 patients (47{\%}): to gastric lymph nodes (29{\%}), liver (25{\%}), and peritoneum (13{\%}). Immunopositivity was detected in the tumors tested as follows: KIT, 100{\%}; CD34, 75{\%}; PKCθ, 21{\%}; PDGFRA, 90{\%}; and smooth muscle actin, 6{\%}. Fourteen patients (13{\%}) died of metastatic GIST at a mean age of 45 years (range, 30 to 69 y). Estimated 10 and 40-year survivals were 100{\%} and 73{\%}, respectively. Median survival time was 26.5 years (range, 16 to 60 y). There was no correlation between the National Institutes of Health tumor risk classification and the tumor behavior. Compared with sporadic gastric GISTs, the gastric stromal tumor in Carney triad showed distinctive features: female predilection, young patient age, epithelioid cell predominance, multifocality, frequent lymph node metastasis, serial tumor occurrence, and unpredictable behavior. Thus, the Carney triad gastric stromal tumor is different clinically, pathologically, and behaviorally from sporadic gastric GIST.",
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