ABSTRACT
Sarcomas are a heterogenous group of malignant tumors with origin or mesenchymal differentiation, they comprise 1-2% of all solid tumors. Retroperitoneum is the second most frequent site affected. Prognosis is worse compared to the limbs, with a 5y OS of 36-58%, and 50-60% patients will relapse. Dedifferentiated liposarcomas (ddLPS) are more aggressive, it is known that presence of a de-differentiated component increases the probability of distant recurrence and lowers OS. There is little information about the specific impact of each type of de-differentiation. To determine if the presence of myogenic differentiation markers in DDLPS is an adverse prognostic factor. A retrospective, observational, analytic cohort study was performed. Cases identified from the electronic clinical files from the National Cancer Institute in Mexico City, we included cases from January 1st 2005 to December 31st 2016. We correlated the presence of expression of myogenic markers (Smooth muscle actin, Calponin, H-caldesmon, Desmin and Myogenin) in the dedifferentiated component of DDLPS with overall survival and surgical outcomes. One hundred and forty-three cases were analyzed. Eighty-two were liposarcomas, and 38 had a dedifferentiated component. Of these 38 cases, 21(55.3%) were males and, 17(44.7%) were females. Median age was 54.1(27-79) years, median tumor size was 28 cm (13-56). Most patients had locally advanced disease: 32(84.2%) were in stage IIIB. 2.6% had metastatic disease and 5(13.2%) had stage Ib at diagnosis. Myogenic marker expression was found in 18.4% of cases; these patients had a worse median survival than cases with no myogenic expression: 18 months (95% CI 15.4-20.5) vs 32 months (95% CI 21.8-42.1) p = 0.01, we also found a relation with higher postoperative morbidity in these cases (p = 0.045). The presence of myogenic differentiation markers might be associated with a worse prognosis, in our series it corelated with worse OS, however it is not a common event. Relation with surgical morbidity is to be analyzed in further studies.
ABSTRACT
Retroperitoneal sarcomas are rare entities. They have a tendency of growing slowly, rendering the patient apparently healthy for long periods of time, before diagnosis. Besides, they have a worse prognosis than sarcomas arising in extremities, with a higher local recurrence rate and lower 5-year survival rate. We describe a case of a 71-year-old male patient, who had a very well succeeded treatment of a large retroperitoneal sarcoma with the combination of chemoembolization, systemic chemotherapy, surgery, and radiation therapy. Initially, it was noticed in an incidental way he had a large retroperitoneal mass (15 cm × 10 cm × 9.2 cm) through magnetic resonance, when he was 63. The case was considered inoperable by the treating physicians. After neoadjuvant therapy, the residual tumor could be completely excised by the responsible surgeon. With a follow-up of >5 years, since the end of treatment, the patient remains in complete remission and, probably, cured from his illness. Large retroperitoneal sarcomas are still a great challenge for oncologists. According to the medical literature, chemoembolization can benefit some patients, but most of them in a palliative setting. In our report, we believe its contribution was critical for a great outcome. In selected cases, it is possible this procedure may be an additional therapeutic modality, as part of a multidisciplinary approach.
ABSTRACT
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.
ABSTRACT
INTRODUCTION: Retroperitoneal sarcomas are a rare group of malignant soft tissue tumors with a generally poor prognosis. The aim of the study was to define the demographic characteristics and prognostic factors for patients with retroperitoneal sarcomas (RPS) in a Tertiary Referral Center at Mexico. METHODS: A retrospective study of patients with RPS treated from January 2005 to December 2012 at the National Cancer Institute at Mexico. Patient, tumor and treatment variables were analyzed including use of adjuvant therapy and survival status. Survival and local recurrence curves were estimated using the Kaplan-Meier method. RESULTS: Ninety-five patients with a mean age of 47 years with retroperitoneal sarcoma were included. Median follow-up was 25 months (range 1-108 months). The average tumor size was 23.7 cm. Histology, 58 (61.1%) were liposarcoma, 14 (14.7%), leiomyosarcomas and 23 (24.2%) were from other histologies. In 64 (67.4%) patients were high-grade malignancies. The median survival was 51 months for patients with complete resection, 25.1 months for those with incomplete resection, and 4.4 months for those with unresectable tumors. Complete resection (p = 0.0001), and liposarcoma (p = 0.03) were prognostic factors for overall survival. CONCLUSION: In this study of patients with retroperitoneal, complete resection and liposarcoma histology are prognostic factors related to the disease-free and overall survival. Patients approached with curative intent should undergo aggressive attempts at complete surgical resection.