MPNST of the abdominal wall in a patient with lynch syndrome: A case report of a rare presentation and unique association.

INTRODUCTION: The abdominal wall is an extremely rare location for malignant peripheral nerve sheath tumors (MPNSTs). Besides presenting a rare location of MPNST, the peculiarity of our case lies in its association with Lynch syndrome, which is to our knowledge the first reported case of its kind. PRESENTATION OF CASE: We present a case report of a 39-year-old male with a personal history of colonic cancer. Genetic counseling revealed Lynch syndrome with a heterozygous germline mutation in MLH1. Nine years after the right hemicolectomy, the patient presented with an asymptomatic lump in the abdominal wall. CT imaging showed a 3 cm mass in the aponeurosis of the right external oblique muscle. The patient underwent successful resection of the parietal tumor. Pathological examination revealed an MPNST. No additional treatment was warranted, and the patient exhibited no signs of relapse during the six months following the surgery. DISCUSSION: MPNSTs of the anterior abdominal wall are extremely rare and challenging. Some studies have investigated the presence of mismatch repair (MMR) deficiency in patients with sarcomas. Our case consolidates the hypothesis of an association between sarcomas and Lynch syndrome, which raises the question of the efficacy of immune checkpoint inhibitor therapy in these cases where treatment options remain limited. CONCLUSION: It is essential to have a deep understanding of the growth patterns of MPNSTs in the context of syndromes that predispose individuals to tumors, like Lynch syndrome. This knowledge is crucial for accurately predicting patient outcomes and developing appropriate plans for monitoring and treatment.

Exocrine Pancreatic carcinoma in Tunisia: A retrospective study about 158 cases.

BACKGROUND: Exocrine pancreatic carcinoma (EPC) occurs in the majority of cases with early locoregional spread and distant metastasis at diagnosis, leading to dismal prognosis and limited treatment options. Traditional cytotoxic chemotherapy provide only modest benefit to patients with EPC. Identification of different molecular pathways, overexpressed in pancreatic cancer cells, has provided the opportunity to develop targeted therapies with a crucial therapeutic role in this cancer setting. OBJECTIVE: Our aim is to study the epidemiological, clinicopathological characteristics, treatment modality and clinical outcome of pancreatic adenocarcinoma in Tunisian patients treated in the department of medical oncology Abderrahmane Mami Ariana. METHODS: This retrospective study concerned patients with exocrine pancreatic carcinoma treated between 2009 and 2012. We analysed the following data: Anamnesis, age, sex, delay to diagnosis(DD), symptoms, clinical exam, performance status, stage, therapeutic protocol and results. RESULTS: We collected 158 patients (113 males/45 females, SR 2.5) with a median age of 64 years (20-93). The median DD was 2 months (1-12). Abdominal pain, jaundice and weight loss were the most frequent symptoms, 88.6%, 43% and 55.1% of cases respectively. Performance status was < 2 in 56.9% (90 pts). Seric CA19-9 was increased in 86.6% of cases. Tumor was at stage III in 24.7% and stage IV in 58.2%. Surgery was done in 24.7% of cases (39pts), curative in 21 patients. Neoadjuvant chemotherapy(NACT) was administrated to 10.8% of patients, adjuvant to 13.9% (22 pts) and palliative chemotherapy(PCT) concerned 58.8% of patients. We used weekly Gemcitabine, Gemcitabine-CDDP, Gemcitabine-Oxaliplatine and LV5-FU2-CDDP in palliative setting respectively in 20%, 31.1%, 2.2 and 36.7% of cases. Median survival was 6 months (2-60) and the 1year overall survival at 38.8%. CONCLUSION: EPC remains a rare cancer in Tunisia. The prognosis is still grim worldwide and so does in our country. In this retrospective serie, we noted the predominance of locally advanced and metastatic cases with a long delay to diagnosis. Awareness campaigns have to be programmed to improve early diagnosis in EPC and improve outcomes.

Perioperative chemotherapy in locally advanced gastric cancer. A retrospective study about 25 cases.

BACKGROUND: Perioperative chemotherapy containing 5FU, Cisplatin ± Docetaxel is one of the most active regimens in advanced gastric cancer. OBJECTIVE: To report epidemiological and clinical profile and therapeutic results of perioperative chemotherapy in locally advanced gastric cancer in Tunisia. METHODS: Our retrospective study concerned patients with histologically confirmed advanced gastric cancer treated at the institute Salah Azaiez of Tunis. They received 2-3 cycles / 3 weeks of neoadjuvant chemotherapy based on FP (5FU and Cisplatin) or TPF (Docetaxel-Cisplatin-5FU). RESULTS: From 2010 to 2012, 25 patients with a median age of 60 years and 7.3 sex-ratio received neoadjuvant chemotherapy. Protocols used were TFP in 20 and FP in 5 patients, 17 patients receiving more than 2 cycles. Side effects were represented by mucositis grade 3 in 2 patients, neutropenia grade ¾ in 3 patients and renal failure in 5 patients. After neoadjuvant chemotherapy, we observed 40% of partial response and 20% of stable disease. Six patients (24%) underwent surgery, curative in 4 (R0 in 3 cases) by total gastrectomy and D2 lymphadenectomy and palliative in 2 cases due to peritoneal carcinomatosis. With a median follow up of 16 months, median overall survival was 16 months (2-21 months). CONCLUSION: Probably due to the very bulky and advanced stages of our gastric cancer cases, neoadjuvant chemotherapy using FP±T showed no benefit in the treatment of locally advanced gastric cancer in Tunisian patients.