Sarcoma patients should be treated in high volume referral sarcoma centers. Compartmental resection is proposed as the best treatment option in retroperitoneal sarcoma patients.
Institute of Oncology ...Ljubljana is the only referral sarcoma center in Slovenia. Having a population of 2.1 million poses a unique situation. We manage all sarcoma patients in the country and operate on patients with soft tissue tumors of extremities, trunk, and abdomen. Data for all consecutive patients surgically treated from January 1999 to December 2018 for primary localized retroperitoneal sarcoma was extracted from a prospective surgical database. Data about the incidence of sarcoma patients in Slovenia was extracted from the Cancer Registry of Republic of Slovenia. Clinicopathologic variables and the outcome were analyzed.
In total, 89 patients were included in the study. Median age was 62 years. Dedifferentiated liposarcoma was the most common histology (38.2%). Median tumor size was 21 cm. Compartmental resection was performed in 47.2% (42/89). Postoperative complication grade 3a or higher according to Clavien-Dindo classification had 30.3% (27/89) of patients. The 30-day and 90-day mortality rate was 2.2% and 5.6%. Median follow-up was 62.1 months. Corresponding 5-year overall survival was 67.2%, 5-year disease-specific survival was 72.6%, and 5-year local recurrence-free survival was 81.5%, respectively.
Results from our institution show that referral sarcoma centers may achieve very good results in management of retroperitoneal sarcoma patients, despite not meeting the criteria for high volume hospitals, as long as they have multidisciplinary team, appropriate facilities, and expertise.
Full text
Available for:
IZUM, KILJ, NUK, PILJ, PNG, SAZU, UL, UM, UPUK
Izhodišča: Retroperitonealni sarkomi so izredno redki, zato naj zdravljenje bolnikov z retroperitonealnimi sarkomi poteka v referenčnem centru. Temeljno zdravljenje je kirurško. Priporočen tip ...operacije je kompartment resekcija.
Metode: Onkološki inštitut Ljubljana je edini referenčni center za sarkome v Sloveniji. V raziskavo so bili vključeni bolniki s primarnim lokaliziranim retroperitonealnim sarkomom, zdravljeni pri nas v obdobju od januarja 1999 do junija 2020. Opredelili smo preživetje, kakovost kirurškega zdravljenja in zaplete.
Rezultati: Vključenih je bilo 100 bolnikov. Srednja starost je bila 62 let. Srednja velikost tumorja je bila 21,5 cm. Najpogostejši histološki podtip je bil dediferenciran liposarkom (39 %). Kompartment resekcija je bila opravljena v 24 %, multivisceralna resekcija pa v 25 %. Zaplete po posegu je imelo po klasifikaciji Clavien-Dindo stopnje 3a ali višje 29 % bolnikov, pri 58,6 % (17/29) je bila potrebna ponovna operacija. Zgodnja in pozna smrtnost po operaciji sta bili 3 % in 5 %. Srednji čas sledenja je bil 55,1 mesecev. 5-letno celokupno preživetje je bilo 67,8 %. Kumulativna verjetnost za lokalno ponovitev bolezni po 5 letih je bila 16,9 %, za oddaljene zasevke pa 21,4 %. Ocena ASA in izguba krvi med operacijo sta bila neodvisna napovedna dejavnika celokupnega preživetja.
Zaključek: Retroperitonealni sarkomi sodijo med redke vrste raka. Naši rezultati zdravljenja bolnikov z retroperitonealnimi sarkomi so zelo dobri in primerljivi z rezultati drugih referenčnih centrov iz tujine. Potrjujejo tudi ključno vlogo referenčnega centra pri obravnavi in zdravljenju teh bolnikov.
Leiomyosarcoma is a rare malignant mesenchymal tumour. Some cases of leiomyosarcoma of the renal vein (LRV) have been reported in the literature, but no analysis of data and search for prognostic ...factors have been done so far. The aim of this review was to describe the LRV, to analyse overall survival (OS), local recurrence free survival (LRFS) and distant metastases free survival (DMFS) in LRV world case series and to identify significant predictors of OS, LRFS and DMFS.
Cases from the literature based on PubMed search and a case from our institution were included.
Sixty-seven patients with a mean age of 56.6 years were identified; 76.1% were women. Mean tumour size was 8.9 cm; in 68.7% located on the left side. Tumour thrombus extended into the inferior vena cava lumen in 13.4%. All patients but one underwent surgery (98.5%). After a median follow up of 24 months, the OS was 79.5%. LRFS was 83.5% after a median follow up of 21.5 months and DMFS was 76.1% after a median follow up of 22 months. Factors predictive of OS in univariate analysis were surgical margins, while factors predictive of LRFS were inferior vena cava luminal extension and grade. No factors predictive of DMFS were identified. In multivariate analysis none of the factors were predictive of OS, LRFS and DMFS.
Based on the literature review and presented case some conclusions can be made. LRV is usually located in the hilum of the kidney. It should be considered in differential diagnosis of renal and retroperitoneal masses, particularly in women over the age 40, on the left side and in the absence of haematuria. Core needle biopsy should be performed. Patients should be managed by sarcoma multidisciplinary team. LRV should be surgically removed, with negative margins.
Cancer presenting at the medial site of the breast may have a worse prognosis compared with tumors located in external quadrants. For medial tumors, axillary lymph node staging may not accurately ...reflect the metastatic potential of the disease.
Eight-thousand four-hundred twenty-two patients randomly assigned to International Breast Cancer Study Group clinical trials between 1978 and 1999 were classified as medial site (1,622; 19%) or lateral, central, and other sites (6,800; 81%). Median follow-up was 11 years.
A statistically significant difference was observed for patients with medial tumors versus those with nonmedial tumors in disease-free survival (DFS; 10-year DFS, 46% v 48%; HR, 1.10; 95% CI, 1.02 to 1.18; P = .01) and overall survival (10-year OS 59% v 61%; HR, 1.09; 1.01 to 1.19; P = .04). This difference increased after adjustment for other prognostic factors (HR, 1.22; 95% CI, 1.13 to 1.32 for DFS; and HR, 1.24; 95% CI, 1.14 to 1.35 for OS; both P = .0001). The risk of relapse for patients with medial presentation was largest for the node-negative cohort and for patients with tumors larger than 2 cm. In the subgroup of 2,931 patients with negative axillary lymph nodes, 10-year DFS was 61% v 67%, and OS was 73% v 80% for medial versus nonmedial sites, respectively (HR 1.33; 95% CI, 1.15 to 1.54; P = .0001 for DFS; and HR 1.40; 95% CI, 1.17 to 1.67; P = .0003 for OS).
Tumor site has a significant prognostic utility, especially for axillary lymph node-negative disease, that should be considered in therapeutic algorithms. New staging procedures such as biopsy of the sentinel internal mammary nodes or novel imaging methods should be further studied in patients with medial tumors.
Karcinom Merklovih celic (MCC) je redek, agresiven in pogosto smrten nevroendokrini kožni karcinom. Zanimanje zanj se v zadnjem času veča zaradi naraščanja incidence in možne virusne povezave. V ...prispevku so opisani klinična slika, zamejitveni sistem in zdravljenje ter izkušnje Onkološkega inštituta Ljubljana (OIL). Prikazan je pregled serije 40 bolnikov, zdravljenih na OIL od 1994 do 2011.
Klasifikacija, diagnostika in zdravljenje stromalnih ter mezenhimskih neoplazem prebavil je v zadnjih 30 letih doživela obsežen napredek. Gastrointestinalni stromalni tumorji (GIST) predstavljajo ...manj kot 1 % vseh malignih tumorjev prebavil.Klinična slika je odvisna od mesta, velikosti in malignega potenciala GIST. Razsejani GIST predstavlja primer uspešnega zdravljenja s tarčno terapijo, saj se je z odkritjem KIT in PDGFR signalne poti ter tirozin kinaznih inhibitorjev, kot staimatinib mesilat in sunitinib, prognoza te bolezni pomembno izboljšala. Izhod zdravljenja slovenskih bolnikov z razsejanim GIST je primerljiv z izhodom zdravljenja bolnikov, ki so bili zdravljeni v pomembnih večjih mednarodnih kliničnih raziskavah, srednji čas do progresa bolezni pri naših bolnikih je 52 mesecev in srednje preživetje 72 mesecev. Uporabljali smo protokol sledenja, ki je poleg kontrastne računalniške tomografije trebuha vključeval tudi preiskavo trebuha z ultrazvokom. S tem smo ob primerljivem preživetju izboljšali kakovost življenja naših bolnikov in zmanjšali stroške zdravljenja. ; slv - slovenski
Background. Retroperitoneal sarcomas are malignant tumors with an aggressive course of disease. Cause of death is local disease in 50% and disseminated disease in only 25%. We made a retrospective ...analysis of surgical treatment of retroperitoneal sarcomas in order assess the effect of this treatment modality on the course of the disease.In the years between 1975 and 2000, 155 patients were surgically treated for primary retroperotneal sarcoma at the Institute of Oncology in Ljubljana. Only 81 of 155 patients received the first treatment at our Institute, while other patients had been at least once operated on elsewhere before the admission to our Institute. Of these patients, 40 required a second surgery for the residual disease and 31 for recurrence. In 23 patients, metastatic spread was found at diagnosis. Our treatment approach was aggressive. We surgically removed the recurrent sarcomas and metastases wherever accessible. Operability at the first surgical treatment performed at the Institute of Oncology was 92%. Therefore, 44 patients underwent 3 or more surgeries for sarcoma. The highest number of operations performed in one patients was 9 (2 patients). In 127 patients, the tumor block resection involved at least one additional organ (up to 6).Results. Complications were not sparse; perioperative mortality was 8.4%. The survival depended upon the metastatic spread at diagnosis, tumor grade and oncologic surgery type.Conclusions. Despite complications, only complete resection without microscopic resuduum and contamination yields a long-term survival to the patients with retroperitoneal sarcoma.
PDF
