Endosonographic diagnosis of recurrent gastrointestinal stromal tumors associated with Carney's syndrome.
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.
Lipcan
Michael T
MT
Division of Gastroenterology and Hepatology, Thomas Jefferson University, 132 South 10th Street, Philadelphia, PA 19107-5244, USA.
Loren
David E
DE
Palazzo
Juan P
JP
Bibbo
Marluce
M
Dimarino
Anthony J
AJ
Cohen
Sidney
S
D002812:Chondroma / Q000175:diagnosis; D019160:Endosonography; D005260:Female; D046152:Gastrointestinal Stromal Tumors / Q000175:diagnosis*; D006801:Humans; D008175:Lung Neoplasms / Q000175:diagnosis; D008479:Mediastinal Neoplasms / Q000175:diagnosis; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local / Q000175:diagnosis; D009378:Neoplasms, Multiple Primary / Q000175:diagnosis*; D010236:Paraganglioma, Extra-Adrenal / Q000175:diagnosis; D012186:Retroperitoneal Neoplasms / Q000175:diagnosis; D013274:Stomach Neoplasms / Q000175:diagnosis*