The first use of laparoscopic resection surgery for recurrent gastrointestinal stromal tumors (GISTs) and paragangliomas as a result of Carney-Stratakis syndrome (CSS) was highlighted in a case report published in the Asian Journal of Endoscopic Surgery.

The 34-year-old female patient received laparoscopic local gastrectomy for 4 gastric tumors and a liver subsegmentectomy of segment 7. A nodule anterior to the inferior vena cava, presumed to be peritoneal dissemination, was also removed laparoscopically.

The laparoscopic approach was chosen for 3 main reasons, the authors said. The first is that it would allow for observation of organs and can be helpful in detecting small extra-gastric nodules. Also, CSS has a higher chance of re-recurrence which would require a third surgery, and performing each laparoscopically could reduce adhesions, poly-surgery, and related complications. Thirdly, patient satisfaction with the cosmetology of the wounds will be a factor since CSS commonly occurs in young female patients.


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The tumors removed from the stomach were diagnosed as GISTs and the liver tumor was diagnosed as liver metastasis, all of which were positive for KIT and CD34 protein expression. Genetic testing of the GISTs found no mutations in the KIT or platelet-derived growth factor receptor alpha (PDGFRA) genes. All tumors also tested negative through immunohistochemistry for succinate dehydrogenase-B, consistent with CSS.

The nodule removed near the inferior vena cava was a paraganglioma positive for chromogranin A and synaptophysin expression. The patient previously underwent surgical resection for multiple gastric GISTs, liver metastasis, and a retroperitoneal tumor. Afterward, she was treated with imatinib but computed tomography (CT) scans revealed disease recurrence 5 years after surgery.

Adjuvant chemotherapy was not prescribed following the more recent resection surgery after a diagnosis of recurrent CSS since CSS lacks mutations in the KIT or PDGFRA genes for the agents to target. On follow-up, the patient was still alive with no recurrence after 3 years and CT monitoring for any recurrence every 6 months.

“We present a rare case of resection of recurrent GISTs and paraganglioma in a patient with CSS. As CSS is very rare, it is important that such cases are reported,” the authors said.

Reference

Fukada A, Takahashi T, Kurokawa Y, et al. Laparoscopic resection for recurrent gastrointestinal stromal tumors and paraganglioma in a patient with Carney-Stratakis syndrome: a case report. Asian J Endosc Surg. Published online July 6, 2022. doi:10.1111/ases.13104