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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.
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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.