TY - JOUR
T1 - Endosonographic diagnosis of recurrent gastrointestinal stromal tumors associated with Carney's syndrome
AU - Lipcan, Michael T.
AU - Loren, David E.
AU - Palazzo, Juan P.
AU - Bibbo, Marluce
AU - Dimarino, Anthony J.
AU - Cohen, Sidney
PY - 2005/1
Y1 - 2005/1
N2 - This is a report of a patient who manifests all of the features of Carney's syndrome, including gastrointestinal stromal tumors (GISTs), extra-adrenal paragangliomas, and pulmonary chondromas. The patient underwent surgical resection of a gastric GIST; a retroperitoneal, nonfunctional paraganglioma; and a mediastinal, catecholamine-secreting paraganglioma (pheochromocytoma). Recently, new gastric GISTs were diagnosed by endoscopic, ultrasound-guided, fine-needle aspiration (EUS-FNA) biopsy and were resected. Recurrence of stromal tumors following complete resection is common in Carney's syndrome and presents considerable management challenges. This case illustrates several important points: (1) Carney's syndrome, although rare, should be considered in patients with foregut GISTs; (2) GISTs associated with Carney's syndrome, like sporadic gastric GISTs, may have a more indolent clinical course; and (3) EUS-FNA may be useful for the diagnosis and management of GISTs in this syndrome.
AB - This is a report of a patient who manifests all of the features of Carney's syndrome, including gastrointestinal stromal tumors (GISTs), extra-adrenal paragangliomas, and pulmonary chondromas. The patient underwent surgical resection of a gastric GIST; a retroperitoneal, nonfunctional paraganglioma; and a mediastinal, catecholamine-secreting paraganglioma (pheochromocytoma). Recently, new gastric GISTs were diagnosed by endoscopic, ultrasound-guided, fine-needle aspiration (EUS-FNA) biopsy and were resected. Recurrence of stromal tumors following complete resection is common in Carney's syndrome and presents considerable management challenges. This case illustrates several important points: (1) Carney's syndrome, although rare, should be considered in patients with foregut GISTs; (2) GISTs associated with Carney's syndrome, like sporadic gastric GISTs, may have a more indolent clinical course; and (3) EUS-FNA may be useful for the diagnosis and management of GISTs in this syndrome.
KW - Chondroma/diagnosis
KW - Endosonography
KW - Female
KW - Gastrointestinal Stromal Tumors/diagnosis
KW - Humans
KW - Lung Neoplasms/diagnosis
KW - Mediastinal Neoplasms/diagnosis
KW - Middle Aged
KW - Neoplasm Recurrence, Local/diagnosis
KW - Neoplasms, Multiple Primary/diagnosis
KW - Paraganglioma, Extra-Adrenal/diagnosis
KW - Retroperitoneal Neoplasms/diagnosis
KW - Stomach Neoplasms/diagnosis
UR - http://www.scopus.com/inward/record.url?scp=11144300198&partnerID=8YFLogxK
UR - https://www.webofscience.com/api/gateway?GWVersion=2&SrcApp=purepublist2023&SrcAuth=WosAPI&KeyUT=WOS:000226225400021&DestLinkType=FullRecord&DestApp=WOS
U2 - 10.1053/j.gastro.2004.10.042
DO - 10.1053/j.gastro.2004.10.042
M3 - Article
C2 - 15633134
SN - 0016-5085
VL - 128
SP - 169
EP - 174
JO - Gastroenterology
JF - Gastroenterology
IS - 1
ER -