MARLENY NOVAES FIGUEIREDO DE ARAUJO
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- Desmoid tumors: clinical features and outcome of an unpredictable and challenging manifestation of familial adenomatous polyposis(2015) CAMPOS, Fabio Guilherme; MARTINEZ, Carlos Augusto Real; NOVAES, Marleny; NAHAS, Sergio Carlos; CECCONELLO, IvanBackground/Aims Desmoid tumors (DTs) are rare, locally invasive neoplasms that may affect 10-25 % of familial adenomatous polyposis (FAP) patients. Our aim was to evaluate incidence and clinical presentation among our patients, the potential impact on FAP outcome and to discuss treatment. Materials and methods Charts from 133 FAP (1977-2013) were reviewed. Patients with DTs were separated to retrieve demographic, clinical and management data. Follow-up was focused on disease evolution causing complications or death. Results 19 (14.3 %) DTs were diagnosed, either after previous trauma (16) or during FAP surgery (3). This group comprised 8 men (42.1 %) and 11 women (57.9 %) with an average age of 32.9 years. Intervals from surgical trauma to DTs ranged from 7 to 60 months. ECMs were detected in 12 (63.1 %) patients. DTs were located in the abdominal wall (8), abdominal cavity (8), abdominal wall and cavity (2) and left arm (1). Five patients (26.3 %) referred family history of DTs. Patients presented severe complications such as small bowel obstruction (4) and hydronephrosis (2), being directly responsible for death in three patients. Conclusions (1) DTs developed in 14.3 % of FAP, mostly after surgical trauma; (2) 30 % caused severe morbidity; (3) identification of clinical risk factors may help surgeons to develop screening and therapeutic decisions.
- Local excision of rectal cancer(2015) HABR-GAMA, A.; FIGUEIREDO, M. N.; JULIãO, G. P. São; PEREZ, R. O.Transanal Local Excision has become a very useful surgical tool for the management of selected cases of rectal cancer due to its low postoperative morbidity and minimal functional consequences. However, the considerably high local recurrence rates led to the introduction of preoperative therapies. Neoadjuvant chemoradiation therapy has been considered the preferred alternative in this setting and may result in significant rates of tumor regression allowing the procedure to be offered to a significant proportion of cases. On the other hand, this multimodality approach may also determine increased postoperative morbidity. In addition, completion or salvage total mesorectal excision in the case of local recurrence or the presence of unfavorable pathological features may also be a challenging task. Finally, accurate selection criteria for this minimally invasive approach are still lacking and may be influenced by baseline staging, post-treatment staging and final pathology information. Ultimately, selection of patients for this treatment modality remains a significant challenge for colorectal surgeons. In the present chapter, the rationale, surgical technique and outcomes of transanal local excision are detailed both after surgery alone or in the setting of multimodality therapy. © Springer-Verlag London 2015.
- Colorectal cancer risk in hamartomatous polyposis syndromes(2015) CAMPOS, Fabio Guilherme; FIGUEIREDO, Marleny Novaes; MARTINEZ, Carlos Augusto RealColorectal cancer (CRC) is a major cause of morbidity and mortality around the world, and approximately 5% of them develop in a context of inherited mutations leading to some form of familial colon cancer syndromes. Recognition and characterization of these patients have contributed to elucidate the genetic basis of CRC. Polyposis Syndromes may be categorized by the predominant histological structure found within the polyps. The aim of the present paper is to review the most important clinical features of the Hamartomatous Polyposis Syndromes, a rare group of genetic disorders formed by the peutz-Jeghers syndrome, juvenil polyposis syndrome and PTEN Hamartoma Tumor Syndrome (Bannayan-Riley-Ruvalacaba and Cowden Syndromes). A literature search was performed in order to retrieve the most recent and important papers (articles, reviews, clinical cases and clinical guidelines) regarding the studied subject. We searched for terms such as ""hamartomatous polyposis syndromes"", ""Peutz-Jeghers syndrome"", ""juvenile polyposis syndrome"", ""juvenile polyp"", and ""PTEN hamartoma tumour syndrome"" (Cowden syndrome, Bananyan-Riley-Ruvalcaba). The present article reports the wide spectrum of disease severity and extraintestinal manifestations, with a special focus on their potential to develop colorectal and other neoplasia. In the literature, the reported colorectal cancer risk for Juvenile Polyposis, Peutz-Jeghers and PTEN Hamartoma Tumor Syndromes are 39%-68%, 39%-57% and 18%, respectively. A review regarding cancer surveillance recommendations is also presented.