Brazilian Society of Surgical Oncology recommendations on Merkel cell carcinoma surgical treatment.

BACKGROUND: Merkel cell carcinoma (MCC) is a rare neuroendocrine skin cancer with poor 5-year survival rates. Surgery and radiation are the current first-line treatments for local and nodal disease. OBJECTIVES: The Brazilian Society of Surgical Oncology developed this document aiming to guide the surgical oncology role in multimodal MCC management. METHODS: The consensus was established in three rounds of online discussion, achieving consensus on specific topics including diagnosis, staging, treatment, and follow-up. RESULTS: Patients suspected of having MCC should undergo immunohistochemical examination and preferably undergo pathology review by a dermatopathologist. Initial staging should be performed with dermatologic and nodal physical examination, combined with complementary imaging. Whole-body imaging, preferably with positron emission tomography (PET) or computed tomography (CT) scans, are recommended. Due to the need for multidisciplinary approaches, we recommend that all cases should be discussed in tumor boards and referred to other specialties as soon as possible, reducing potential treatment delays. We recommend that all patients with clinical stage I or II may undergo local excision associated with sentinel lymph node biopsy. The decision on margin size should consider time to recovery, patient's comorbidities, and risk factors. Patients with positive sentinel lymph nodes or the presence of risk factors should undergo postoperative radiation therapy at the primary site. Exclusive radiation is a viable option for patients with low performance. Patients with positive sentinel lymph node biopsy should undergo nodal radiation therapy or lymphadenectomy. In patients with nodal clinical disease, in addition to primary tumor treatment, nodal radiation therapy and/or lymphadenectomy are recommended. Patients with advanced disease should preferably be enrolled in clinical trials and discussed in multidisciplinary meetings. The role of surgery and radiation therapy in the metastatic/advanced setting should be discussed individually and always in tumor boards. CONCLUSION: This document aims to standardize a protocol for initial assessment and treatment for Merkel cell carcinoma, optimizing oncologic outcomes in middle-income countries such as Brazil.

Leiomyosarcoma of the inferior vena cava: Survival rate following radical resection.

Leiomyosarcoma (LMS) of inferior vena cava (IVC) is a rare neoplasm affecting approximately 1/100,000 people. The prognosis is poor and potential curative intent occurs through challenging operations, such as vena cava resection, occasionally multivisceral when required, and vascular reconstruction. There are few retrospective series regarding this retroperitoneal neoplasm, and the aim of the present study was to discuss the experience at the São Paulo Cancer Institute and Clinics Hospital of University of São Paulo Medical School, São Paulo, Brazil. The current study is a retrospective review of 7 patients treated in the two tertiary hospitals between 2005 and 2013. Oncological and operative aspects were discussed, primarily regarding surgical aspects highlighting en bloc resection, vascular reconstruction, and the overall survival and recurrence rates. All the patients were treated with radical intent, 4 of whom underwent multivisceral resection, with the kidney being the most resected organ. The location of the IVC tumor was described using Kulaylat's description and the median tumor size was 10 cm. Vascular reconstruction was necessary in 4 patients. The overall survival rate at 3 and 5 years was 100, and 25%, respectively. The disease-free survival rate at 3 and 5 years was 57 and 20%, respectively. In conclusion, IVC LMS is a rare and severe retroperitoneal neoplasm, with multivisceral resections remaining a surgical challenge. The treatment requires numerous experienced surgeons and the impact of microscopic free margins remains unclear. Vascular reconstruction depends on several aspects regarding primarily the topography of the tumor.

Acute appendicitis, inflammatory appendiceal mass and the risk of a hidden malignant tumor: a systematic review of the literature.

INTRODUCTION: Acute appendicitis is significantly common. Despite the increased use of computed tomography, the number of perforated cases has been stable in the past three decades. Between 2% and 6% of patients with acute appendicitis present appendiceal mass, often described as inflammatory phlegmon or abscess. Malignant tumors are confirmed by pathological analysis in 0.9-1.4% of all appendectomies performed to treat acute appendicitis. However, recent series demonstrate an elevated incidence of malignancies, ranging from 5.9 to 12%, in patients with inflammatory appendiceal mass. METHODS: The analysis was based on a systematic review of the literature. The articles were searched in PubMed for the period from 1987 to 2016. Articles presenting the incidence of the hidden malignancy among patients with appendiceal inflammatory mass were selected. Variables as age, interval appendectomy rate, the incidence of neoplasm, time to surgery, minimally invasive assessment, histology, right colectomy rate and morbidity were analyzed. RESULTS: A total of 13.244 patients were described as presenting acute appendicitis. Appendiceal tumor is present in approximately 1% of the appendectomies, while the rate of neoplasm varies from 10 to 29% in patients presenting appendiceal inflammatory mass. Interval appendectomies, despite been the minority of the procedures, disregard the higher morbidity associated with right sided colectomies. The review of literature also describes oncologic, histologic and clinical aspects of patients presenting appendiceal neoplasm, describing the most frequent histologic subtypes of this illness. CONCLUSION: Hidden appendiceal neoplasm in acute appendicitis are rare, fortunately. However, its incidence is much higher in patients presenting appendiceal inflammatory mass. Hence, interval appendectomy should be considered in this subgroup of patients.