Gastrointestinal stromal tumor: Our experience

S 1001 Background: The Carney complex, a rare dominantly inherited syndrome of multiple neoplasia associated with cardiocutaneous manifestations, was first described by Carney in 1985. In 2000, mutations were identified in the PRKAR1A gene on chromosome 17q22-24. PRKAR1A is a tumor suppressor gene that encodes the protein kinase A regulatory alpha subunit. Approximately 65% of patients affected by the Carney complex have mutations of PRKAR1A. About 80 types of mutations in the PRKAR1A gene have been identified in patients with the Carney complex with different penetrance and manifestations of disease. Case Report.We report the case of a 61-year-old man with atrial myxoma, patchy skin pigmentation and multiple tumors in which a new and unique mutation of the PRKAR1A gene (substitution of guanine with adenine at nucleotide position 973) was identified. This particular alteration has never been described before. The family history showed that his father had an atrial myxoma. The clinical history began in 1974, when an anaplastic seminoma of the right testis was diagnosed. The patient was submitted to orchiectomy. Over the following 10 years, he underwent inguinal lymphoadenectomy, resection of repeated cutaneous lesions, and resection of lung metastases. Histology demonstrated recurrence of leiomyosarcoma. He received repeated courses of chemotherapy and local radiotherapy. The follow-up was negative until September 2008, when a control computed tomography (CT) scan showed a huge right atrial mass and a retroperitoneal mass. The atrial lesion was surgically removed and identified as myxoma. In November 2008, he underwent distal pancreatectomy. Histology demonstrated acinar cell adenocarcinoma. In April 2010, because of evidence of a retroperitoneal mass, he underwent gastric and diaphragmatic resection for a recurrence of adenocarcinoma of pancreatic origin. In January 2011, he was referred to our institution with signs of high occlusion caused by a huge epigastric mass; surgical excision of the jejunum was performed. Histology confirmed the diagnosis of high-grade leiomyosarcoma. In February 2012, he underwent removal of a new retroperitoneal mass with consensual left nephroadrenalectomy and wedge resection of the greater gastric curve. Histological examination is in progress but the initial suspicion is a neuroendocrine carcinoma of adrenal origin, a typical lesion of the Carney complex. Gastrointestinal stromal tumor: Our experience C. Gennaro*, M. Garino, G.R. Fronda, S. Sandrucci 1 Oncological Surgery Unit, University of Turin, Italy 2 7Th Surgical Unit, S. Giovanni Battista Hospital Torino Italy * Corresponding author: Carlo Gennaro, Oncological Surgery Unit, University of Turin, Italy, Cso Dogliotti 14 10126 Torino, Italy. E-mail address: karlogen@gmail.com (C. Gennaro). Background: Over the past decade, gastrointestinal stromal tumors (GIST) have become the most common sarcoma of the intestinal tract, and this pathology is now recognized as a unique class of tumor. GIST could best be treated by integratiing surgery and target therapy. The aim of surgery must be a R0 resection, possibly avoiding neoplasm rupture to minimize the risk of recurrence. Methods: At the 7 General Surgery Department of S.G. Battista Hospital of Turin, we performed 58 operations for GIST in 54 patients from 1995 to 2009. The lesions were classified according to the criteria described by Miettinen and Fletcher: tumor size, mitotic rate, tumor site, and presence of metastases. None of the patients was lost to follow-up. Preoperative diagnostic work-up included endoscopy with and without ultrasound and computed tomography (CT), magnetic resonance imaging (MRI) and videocapsule in some cases. The lesions were classified as either gastric or non-gastric, and then according to risk: low-to-very low, intermediate, and high risk. Results: Gastric lesions made up 71% of cases; the remaining lesions involved the small intestine, rectum, peritoneum, or liver. Duodenum localization was considered separately. R0 resection was performed in 100% of cases of lesions with low-to-very low risk, in 94% of cases of GISTs with intermediate risk, and in 66% with high-risk tumors. Laparoscopic surgery was used in 12% of cases. The medium diameter of the gastric tumor was 4 cm (range, 3-7); a gastric wedge resection was performed in all cases. Pathological histology of the specimens was graded as low-to-very low and intermediate in 85% and 15% of cases, respectively. Adjuvant imatinib therapy was given only when there was evidence of progression of disease or of macroand microscopic residual disease in highand intermediate-risk GISTs in 77% and 28% of cases, respectively. The Kaplan-Meier survival rate was 84%, 62%, and 41% at 5, 8, and 10 years, respectively, for high-risk GIST and 92% and 78% at 5 and 10 years, respectively, for intermediate-risk tumors. There was a significant difference in recurrence of disease at 1, 3, and 5 years. Conclusions: Surgery alone, without lesion rupture, could be curative for low and very-low-risk GIST and R0 operations for intermediate-risk GIST. Aggressive GIST with a high risk of recurrence could be controlled with a combination of aggressive surgery and target therapy with imatinib. Robotic resection of paracardial and prepyloric gastrointestinal stromal tumors W. Petz*, L. Casali, M. Parodi, D. Belotti, F. Uccelli, P.P. Bianchi Unit of Minimally Invasive Surgery. European Institute of Oncology, Italy * Corresponding author: Wanda Petz. European Institute of Oncology, Via Ripamonti, 435; 20141 Milan. Italy. Tel.: +39.02.94372053; Fax +39.02.94379215. E-mail address: wanda.petz@ieo.it (W. Petz). Background: Surgical resection with negative margins is the treatment of choice for gastrointestinal stromal tumors (GIST). A robotic approach can allow a minimally invasive approach even to gastric GIST located near the esophagogastric junction or the pylorus. Methods: Robotic wedge resections were reviewed in 3 patients (age, 42, 44 and 78 years, respectively) with a gastric GIST located at the antrum, immediately proximal to the pylorus in 1 and on the posterior gastric wall near the esophagogastric junction in the remaining 2 cases. Diagnosis was based on imaging findings obtained with computed tomography (CT) and endoscopic ultrasound and confirmed by biopsy. With the patients positioned in dorsal decubitus and legs open, 1 optical supraumbilical trocar, 3 8-mm robotic trocars were inserted in the left and right hypocondrium and 2 accessory trocars in the left flank. Intraoperative ultrasonography confirmed the exact localization of the lesion. In the proximally located GIST, mobilization of the greater curvature was achieved by sectioning the short vessels; a gastrotomy was then performed and the tumor resected using a harmonic scalpel. The gastrotomy was closed with two-layer absorbable running sutures. Results: The operating time was 180 minutes; blood loss was negligible; no intraoperative or postoperative complications occurred. Oral feeding was started on the third postoperative day after a negative radiographic contrast study, and the patients were discharged home on the fourth postoperative day. Histopathological examination confirmed the diagnosis of GIST and the negative margins of the surgical specimens. Conclusions: Robotic resection of gastric GIST is a feasible and safe procedure. Robotic assistance allows accurate dissection of tumors in the paracardial and prepyloric region, and facilitates fine surgical gestures such as a gastric wall suture. Surgical treatment of atypical lipomatous tumors (well-differentiated liposarcoma) C. Mussi*, A. Ardito, A. Brocchi, L. Cozzaglio, P.F. Bagnoli, A. Kasangian, D. Strada, M. Cimino, P.G. Colombo, V. Quagliuolo 1 Department of Surgical Oncology, IRCCS Istituto Clinico Humanitas, Milan, Italy Department of Pathology, IRCCS Istituto Clinico Humanitas, Milan, Italy * Correspondence to: C Mussi, MD e Cancercenter e Department of Surgical Oncology e Istituto Clinico Humanitas e via Manzoni 56 e Rozzano (Mi), Italy. Phone: +39 02 82244597. E-mail address: chiara.mussi@cancercenter.humanitas.it (C. Mussi).