Multivisceral resection for retroperitoneal liposarcoma-is it worth it? A 20-year single-center experience.

PURPOSE: Soft tissue sarcomas are rare malignant tumors. Liposarcoma constitutes the most frequent histological subtype of retroperitoneal sarcoma. The prognosis of soft tissue sarcomas depends on clinical and histologic characteristics. OBJECTIVE: Evaluate variables that may be related to the overall and local recurrence-free survival in patients with retroperitoneal liposarcoma and discuss the need for visceral resection en-bloc for tumors. METHODS: A retrospective analysis was conducted of the medical records of 60 patients seen between 1997 and 2017 who underwent surgical resection of retroperitoneal liposarcoma. RESULTS: The overall survival rate at 5 years of follow-up was 75.22% (95% confidence interval [CI] 0.58-0.86). The probability of a local recurrence-free survival at 5 years of follow-up was 26.04% (95% CI 0.11-0.44). The multivariate analysis showed that dedifferentiated or pleomorphic tumors and R2/fragmented resection were associated with a shorter time to recurrence. No other characteristics markedly influenced the overall survival (P > 0.05). CONCLUSION: Patients with dedifferentiated or pleomorphic tumors and incomplete resection were associated with higher local recurrence rates than others. This study reinforces the need for complete and en-bloc resection with organs when there is clear involvement or technical surgical difficulty to maintain the tumor integrity.

Popliteal lymph node dissection for metastases of cutaneous malignant melanoma.

Popliteal lymph node dissection is performed when grossly metastatic nodal disease is encountered in the popliteal fossa or after microscopic metastasis is found in interval sentinel nodes during clinical staging of cutaneous malignant melanoma. Initially, an S-shaped incision is made to gain access to the popliteal fossa. A careful en bloc removal of fat tissue and lymph nodes is made to preserve and avoid the injury of peroneal and tibial nerves as well as popliteal vessels, following the previous recommendations. This rare surgical procedure was successfully employed in a patient with cutaneous malignant melanoma and nodal metastases at the popliteal fossa. The technique described by Karakousis was reproduced in a step-by-step fashion to allow anatomical identification of the neurovascular structures and radical resection with no post-operative morbidity and prompt recovery. Popliteal lymph node dissection is a rarely performed operative procedure. Following a lymphoscintigraphic examination of the popliteal nodal station, surgeons can be asked to explore the popliteal fossa. Detailed familiarity of the operative procedure is necessary, however, to avoid complications.

Discordance Between the Initial Diagnosis of Sarcomas and Subsequent Histopathological Revision and Molecular Analyses in a Sarcoma Reference Center in Brazil.

PURPOSE: To investigate the discordance in sarcoma diagnoses between nonspecialized institutions following revision by dedicated sarcoma pathologists at a reference center in Brazil and the relevance of molecular pathology in this context. METHODS: We conducted a retrospective analysis of sarcoma samples initially analyzed at outside laboratories and subsequently reviewed by two specialized pathologists between January 2014 and December 2020. After obtaining demographic and tumor characteristics, pathology results were matched and classified as complete discordance (CD; benign v malignant, sarcoma v other malignancies), partial concordance (similar diagnosis of connective tumor, but different grade/histological subtype/differentiation), and complete concordance (CC). The concordance for histology or grade, and the role of molecular assessments supporting the diagnosis were also independently determined. Statistical analyses were conducted through the kappa coefficient of agreement and adherence by χ2 test, χ2 test by Person, and Fisher exact test. RESULTS: In total, 197 cases were included, with samples obtained predominately from male patients (57.9%) and localized/primary tumors (86.8%). Following revision, the most frequent final diagnoses were undifferentiated pleomorphic sarcoma (17.8%), well-differentiated/dedifferentiated liposarcoma (8.6%), and leiomyosarcoma (7.6%). CD was found in 13.2%, partial discordance in 45.2%, and CC in 41.6% of reviews (P < .001). We found a concordance for histology or grade of 53.5% (P < .001) and 51.8% (P < .001), respectively. Molecular assessments, comprising next-generation sequencing panels (79.5%) and fluorescent in situ hybridization (20.5%), were performed in 44 (22.3%) cases, with findings classified as of diagnostic relevance in 31.8%. CONCLUSION: In nearly 60% of the cases, the initial sarcoma diagnosis was modified when revised by a reference center and dedicated pathologists, assisted by molecular pathology techniques. These results justify the assembly of referral networks in countries with limited health care resources.

Pressurized Intraperitoneal Aerosol Chemotherapy (PIPAC): The First Reported Case in Brazil Using Standardized Technique with the Capnopen® Nebulizer Device.

BACKGROUND Peritoneal metastasis is a common progression of abdominal-pelvic cancers, and it is associated with poorer oncological prognosis when compared to other metastasis sites. Its treatment has limited results, mainly because of poor bioavailability of chemotherapy within the abdominal cavity after systemic administration. Pressurized intraperitoneal aerosol chemotherapy (PIPAC) has been proposed as a novel method to deliver chemotherapy directly into the peritoneal surface; it combines the effectiveness and response of an intraperitoneal therapy with benefits of a minimally invasive approach. The laparoscopic capnoperitoneum is used to instill chemotherapy particles in a more efficient way for distribution and penetration when compared to peritoneal lavage. In the present study, we describe the first PIPAC performed in Brazil, according to the standard technique previously described with the Capnopen® nebulizer device, as well as technique details based on our literature review. CASE REPORT A 67-year-old man with pancreatic adenocarcinoma metastatic to the liver at first diagnosis underwent systemic treatment with the FOLFIRINOX protocol. After a major clinical response due to systemic treatment, pancreaticoduodenectomy was performed with resection and radiofrequency ablation of hepatic nodules. After 7 months of follow-up, the patient's condition evolved with symptomatic relapse in the peritoneum. Aiming at better control of this site, multiple PIPAC procedures were performed, showing excellent control of the peritoneal cavity disease. The patient had a sustained response in the peritoneal cavity and showed systemic disease progression 6 months after the first PIPAC procedure, which deceased at 20 months after the first PIPAC procedure and 42 months after the primary diagnosis. CONCLUSIONS This report shows that the PIPAC procedure is reproducible elsewhere, with safety and good functional results.

Brazilian Group of Gastrointestinal Tumours' consensus guidelines for the management of oesophageal cancer.

Oesophageal cancer is among the ten most common types of cancer worldwide. More than 80% of the cases and deaths related to the disease occur in developing countries. Local socio-economic, epidemiologic and healthcare particularities led us to create a Brazilian guideline for the management of oesophageal and oesophagogastric junction (OGJ) carcinomas. The Brazilian Group of Gastrointestinal Tumours invited 50 physicians with different backgrounds, including radiology, pathology, endoscopy, nuclear medicine, genetics, oncological surgery, radiotherapy and clinical oncology, to collaborate. This document was prepared based on an extensive review of topics related to heredity, diagnosis, staging, pathology, endoscopy, surgery, radiation, systemic therapy (including checkpoint inhibitors) and follow-up, which was followed by presentation, discussion and voting by the panel members. It provides updated evidence-based recommendations to guide clinical management of oesophageal and OGJ carcinomas in several scenarios and clinical settings.

Brazilian Group of Gastrointestinal Tumours' consensus guidelines for the management of gastric cancer.

Gastric cancer is among the ten most common types of cancer worldwide. Most cases and deaths related to the disease occur in developing countries. Local socio-economic, epidemiologic and healthcare particularities led us to create a Brazilian guideline for the management of gastric carcinomas. The Brazilian Group of Gastrointestinal Tumors (GTG) invited 50 physicians with different backgrounds, including radiology, pathology, endoscopy, nuclear medicine, genetics, oncological surgery, radiotherapy and clinical oncology, to collaborate. This document was prepared based on an extensive review of topics related to heredity, diagnosis, staging, pathology, endoscopy, surgery, radiation, systemic therapy and follow-up, which was followed by presentation, discussion, and voting by the panel members. It provides updated evidence-based recommendations to guide clinical management of gastric carcinomas in several scenarios and clinical settings.

High numbers of human skin cancers express MMP2 and several integrin genes.

BACKGROUND: Basal cell carcinomas (BCC), squamous cell carcinomas (SCC) and cutaneous malignant melanoma (MM) are solid skin cancers derived from different cell types, with different ability to metastasize. Several subtypes of integrins and matrix metalloproteinases (MMP) have been related to malignization and metastasis processes. This work aimed at a quantitative evaluation of skin cancers expressing eight integrins and MMP2 genes. METHODS: Expression of integrins and MMP2 genes was evaluated on fresh tumor biopsies from BCC, SCC and MM, and respective controls, by the reverse transcriptase polychain reaction (RT-PCR) technique. RESULTS: More than 90% tumors expressed alpha6a, beta1, beta3 and beta6 (non-melanoma), and alpha5a, alpha6a and MMP2 (MM). Up to 100% controls also expressed beta1 and beta3. The results were significant for alpha6a in BCC (p = 0.026), alpha6b in SCC (p = 0.035), alpha2a in BCC (p = 0.003), beta5 and beta6 in BCC (p = 0.005). MMP2 was expressed in 100% MM (p = 0.0004). CONCLUSION: Integrin subunits alpha2a and alpha6a would be interesting targets for BCC anti-tumor therapy, as well as alpha6b in case of SCC. The elevated number of BCC expressing alpha2 and alpha6, and of MM expressing alphav and MMP2, corroborate literature data.

Is there an oncological interest in the combination of CRS/HIPEC for peritoneal carcinomatosis of HCC? Results of a multicenter international study.

INTRODUCTION: Peritoneal metastasis (PM) of hepatocellular carcinoma (HCC) without distant spread are rare. The related prognosis is poor without standard treatment available. The role of cytoreduction surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) is poorly documented. METHODS: An international multicentric cohort was constituted by retrospective analysis of 21 patients undergoing CRS/HIPEC for PM of HCC between 1992 and 2016 from 10 reference centers of PSOGI. Data on clinical features, treatment strategies, and survival outcomes were analyzed. RESULTS: The median time interval from the diagnosis of PM to the procedure was 4.5 months. The median peritoneal cancer index was 14. Sixteen patients had complete cytoreduction (CCR0-1). Ten patients had grades 3 to 4 complications. The median duration of follow-up was 52.2 months. The median OS was 46.7 months. The projected 3y-OS and 5y-OS were 88.9 and 49.4% respectively. The median OS for patients with CCR0-1 resection was not reached whereas it was 5.9 months for those with CCR2-3 resection after CRS (p = 0.0005). The median RFS was 26.3 months and projected RFS at 3 years of 36.5 months Three prognostic factors were associated with improved RFS in the univariate analysis: preoperative chemotherapy (p = 0.0156), PCI >15 (p = 0.009), Number of chemotherapy agents used for HIPEC (p = 0.005). CONCLUSION: CRS/HIPEC is a safe and effective approach in selected patients with PM of HCC. CRS/HIPEC gives the patient a chance for a good relapse free and overall survival and should be considered as an option.

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.

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.

A proposal of Brazilian Society of Surgical Oncology (BSSO/SBCO) for standardizing cytoreductive surgery (CRS) plus hyperthermic intraperitoneal chemotherapy (HIPEC) procedures in Brazil: pseudomixoma peritonei, appendiceal tumors and malignant peritoneal mesothelioma

Cytoreductive surgery plus hypertermic intraperitoneal chemotherapy has emerged as a major comprehensive treatment of peritoneal malignancies and is currently the standard of care for appendiceal epithelial neoplasms and pseudomyxoma peritonei syndrome as well as malignant peritoneal mesothelioma. Unfortunately, there are some worldwide variations of the cytoreductive surgery and hypertermic intraperitoneal chemotherapy techniques since no single technique has so far demonstrated its superiority over the others. Therefore, standardization of practices might enhance better comparisons between outcomes. In these settings, the Brazilian Society of Surgical Oncology considered it important to present a proposal for standardizing cytoreductive surgery plus hypertermic intraperitoneal chemotherapy procedures in Brazil, with a special focus on producing homogeneous data for the developing Brazilian register for peritoneal surface malignancies.

Pancreaticoduodenectomy in patients with type 1 Neurofibromatosis: Report of two cases and literature review.

INTRODUCTION: Type 1 Neurofibromatosis (NF1) is one of the most common autosomal dominantly inherited multisystem disorders. It is associated with an increased risk of developing neurologic and gastrointestinal (GI) malignant neoplasms. The incidence of GI involvement is reported in 10-25% of patients. Less than 5% of NF1 patients with GI neoplasms manifest symptoms. The presence of synchronic gastrointestinal stromal and neuroendocrine tumors is rare in these patients. PRESENTATION OF CASES: The first case is a 37 year-old male patient with a history of abdominal pain for a few months. Imaging study showed a periampullary mass and a solid lesion at the third duodenal portion. He was submitted to a pancreatoduodenectomy and histological anaylisis showed two low-grade neuroendocrine tumors and a gastrointestinal stromal tumor. The second case is a 47 year-old female patient with a routine computed tomography scan showing a duodenal and a jejunal lesion. Duodenopancreatectomy was performed and histological analysis showed a neuroendocrine adenocarcinoma of the duodenum and two jejunal lesions compatible with GI tumors. DISCUSSION: GI symptoms such as jaundice, pain and bleeding in NF1 patients should prompt urgent admission Occasionally, associated gastrointestinal tumors may be incidentally found in asymptomatic NF1 patients. The presence of a periampullary or duodenal neoplasia such as neuroendocrine tumors should be evaluated. CONCLUSION: Although rare, the synchronic presentation of gastrointestinal tumors in patients with NF1 should be ruled out since it can lead to higher morbidity and mortality rates. Single-stage surgical management is feasable and yields satisfactory results.

Can we respect the principles of oncologic resection in an emergency surgery to treat colon cancer?

Patients with colorectal cancer admitted to the emergency room are generally at more advanced stage of the disease and are usually submitted to a resection with curative intent in a smaller scale. In such scenario, one of the aspects to be considered is whether the principles of oncologic resection are observed when those patients diagnosed with colon cancer are treated with surgery. We selected 87 patients with adenocarcinoma of colon and/or upper rectum submitted to an emergency surgical resection. The major variables reviewed retrospectively were: the extent of resection performed, the number of dissected regional lymph nodes and the overall survival rate. Intestinal obstruction was observed in 67 patients (77%) while perforation was found in 20 patients (23%). Seven (8%) specimens had circumferential compromised margins, all found in patients with T4 tumors combine with poor clinical status. The number of dissected regional lymph nodes was greater than, or equal to, 12 in 71% of patients. While the average days of stay in the ICU was 5.7 days, the median was 3 days. The morbidity and peri-operative mortality stood at 33.6% and 20%, respectively. The outcome of an emergency surgery of colorectal cancer observed in this study was similar to those found in the literature. The principles of oncologic resection were respected when considering and analyzing the extent of the resection, the surgical margins and the number of dissected lymph nodes.

A proposal of Brazilian Society of Surgical Oncology (BSSO/SBCO) for standardizing cytoreductive surgery (CRS) plus hyperthermic intraperitoneal chemotherapy (HIPEC) procedures in Brazil: pseudomixoma peritonei, appendiceal tumors and malignant peritoneal mesothelioma / Proposta de padronização da Sociedade Brasileira de Cirurgia Oncológica (BSSO/SBCO) para procedimentos de citorredução cirúrgica (CRS) e quimioterapia intraperitoneal hipertérmica (HIPEC) no Brasil: pseudomixoma peritoneal, tumores do apêndice cecal e mesotelioma peritoneal maligno

ABSTRACT Cytoreductive surgery plus hypertermic intraperitoneal chemotherapy has emerged as a major comprehensive treatment of peritoneal malignancies and is currently the standard of care for appendiceal epithelial neoplasms and pseudomyxoma peritonei syndrome as well as malignant peritoneal mesothelioma. Unfortunately, there are some worldwide variations of the cytoreductive surgery and hypertermic intraperitoneal chemotherapy techniques since no single technique has so far demonstrated its superiority over the others. Therefore, standardization of practices might enhance better comparisons between outcomes. In these settings, the Brazilian Society of Surgical Oncology considered it important to present a proposal for standardizing cytoreductive surgery plus hypertermic intraperitoneal chemotherapy procedures in Brazil, with a special focus on producing homogeneous data for the developing Brazilian register for peritoneal surface malignancies.

RESUMO A cirurgia citorredutora com quimioterapia intraperitoneal hipertérmica emergiu como um importante tratamento das neoplasias peritoneais e é, atualmente, o padrão de atendimento para neoplasias epiteliais do apêndice associadas à síndrome de pseudomixoma peritoneal, bem como para o mesotelioma peritoneal maligno difuso. No mundo, existem algumas variações reconhecidas das técnicas de cirurgia citorredutora e quimioterapia intraperitoneal hipertérmica, entretanto nenhuma técnica até agora demonstrou sua superioridade sobre o outra. Portanto, a padronização destes procedimentos poderia melhorar a prática clínica e permitir a comparação adequada entre os resultados. Neste cenário, a Sociedade Brasileira de Cirurgia Oncológica considera importante a apresentação de uma proposta de padronização de procedimentos de cirurgia citorredutora com quimioterapia intraperitoneal hipertérmica no Brasil, com um foco especial na produção de dados homogêneos para o desenvolvimento do registro brasileiro das neoplasias peritoneais.

Mid-humeral sentinel lymph node dissection in patients with primary cutaneous malignant melanoma.

In rare cases, lymphatic drainage from the malignant melanomas in the upper extremity may follow an unpredictable pattern (outside the axillary nodes), and these aberrant sentinel nodes may represent the only site of regional lymph node metastases. The precise anatomical landmarks and technical aspects of surgical exploration of these aberrant sentinel lymph nodes are rarely described in the literature, including aberrant sentinel mid-humeral lymph nodes. This report describes a step-by-step dissection of the mid-humeral sentinel lymph nodes in two patients with a primary malignant melanoma in the upper extremity, identified by lymphatic mapping. Recognition of precise regional anatomy and following a special surgical technique makes the procedure safe and successful, thus avoiding local complications and allowing a prompt recovery.

Peritonectomy for peritoneal carcinomatosis: long-term outcomes from a single Brazilian institution.

BACKGROUND: The objective of this study was to evaluate the long-term outcomes of a single institution, Hospital Sírio-Libanes in São Paulo, Brazil, regarding the treatment of peritoneal carcinomatosis. METHODS: Between October 2002 and October 2006, 46 consecutive patients were treated with radical cytoreduction and hyperthermic peritoneal chemotherapy. There were 21 patients with peritoneal surface malignancy (PSM) from colorectal origin (among whom 8 had an appendiceal primary), 15 with ovarian carcinomas, 2 with primary peritoneal mesotheliomas, and 8 with other cancers. The median age was 49 years (range 18-77 years). All patients were followed for a median of 20 months. Demographic data, tumor histology, the peritoneal carcinomatosis index (PCI), operative procedures (extension of resection, lymphadenectomy), and hyperthermic intraperitoneal chemotherapy (HIPEC) characteristics (drugs, temperature, duration) were prospectively recorded. Perioperative mortality and morbidity and the long-term outcome were assessed. RESULTS: Complete cytoreduction was achieved in 45 patients. The median PCI was 11, and the mean operating time was 17 h. There were no procedure-related deaths, but major morbidity was observed in 52% and included fistulas, abscesses, and hematologic complications. The overall Kaplan-Meier 4-year estimated survival was 56%. Among patients with PSM from colorectal carcinoma, the estimated 3-year survival was 70%. Nine (42%) patients had a recurrence, three with peritoneal disease. The median disease-free-interval was 16 months. The ovarian cancer patients had an estimated 4-year survival rate of 75% and median disease-free survival duration of 21 months. CONCLUSIONS: Cytoreductive surgery with HIPEC may improve survival of selected patients with peritoneal carcinomatosis, with acceptable morbidity.

Fístula esofagobrônquica tardia por corpo estranho devida a trauma fechado / Late esophageo-bronchial fistula after blunt chest trauma

Os autores discutem o diagnóstico e tratamento realizados em um paciente portador de fístula esofagobrônquica adquirida após trauma fechado, sem cura nove anos após esofagoplastia, que evoluiu com lesöes pulmonares supurativas do tipo broncoaspirativas. O paciente foi submetido, há três meses, a esofagectomia intratorácica com sutura e conseqüente fechamento da fístula do brônquio principal esquerdo.