Gastrointestinal stromal tumor in Carney's triad with laparoscopic total gastrectomy: a case report

Abstract

Introduction: Carney's triad is a rare syndrome characterized by the co-occurrence of gastric gastrointestinal stromal tumor (GIST), pulmonary chondroma, and extra-adrenal paraganglioma. We present a case of a young woman with GISTs associated with this triad.

Case presentation: A 28-year-old woman was identified with multiple gastric tumors and a right lung nodule during a routine health check-up. CT scans and upper gastrointestinal endoscopy revealed a 50 mm mass on the lesser curvature of the stomach, along with two additional gastric lesions and a 20 mm nodule in the right lung. The patient had a history of right middle lobectomy at the age of 19 for pulmonary chondroma. During surgery, enlarged lymph nodes were observed, indicating metastasis, which necessitated a total gastrectomy with radical (D2) lymph node dissection. Pathological examination confirmed seven GISTs, with immunohistochemical staining positive for KIT (+), DOG1 (+), and negative for SDHB (-). The postoperative course was uneventful, and the patient was discharged on the seventh postoperative day. Despite opting out of adjuvant imatinib therapy, she remains disease-free 2 years postoperatively.

Conclusions: This case underscores the necessity of total gastrectomy with lymph node dissection due to the high incidence of metastasis in GISTs associated with Carney's triad. Further research is required to determine the optimal extent of lymph node dissection in such cases.

Keywords: Carney’s triad; Gastrointestinal stromal tumors (GISTs); Laparoscopic total gastrectomy.

Fig. 1

Preoperative CT; there were four multiple gastric masses (white arrows)

Fig. 2

Chest plain CT; there was a nodule with coarse calcifications in the upper lobe of the right lung

Fig. 3

Endoscopic examination; there were multiple submucosal tumors in the stomach

Fig. 4

Intraoperative findings; a There was an enlarged lymph node on the lesser curvature, and rapid pathological examination revealed it to be metastasis of GIST. b We changed the surgery plan from a local resection to a total gastrectomy with radical lymph node dissection with Roux-en-Y reconstruction

Fig. 5

Pathological examination; there were three submucosal tumors that were not detected preoperatively, totaling seven lesions (black squares)

Fig. 6

Pathological stainings; a hematoxylin eosin staining of the #3 lymph node removed during surgery, b immunoreactivity with KIT antibodies. c Tumors were succinate dehydrogenase B deficient