<b>Medical Journal Article Annotated Citations</b>

<b>Definition of Retroperitoneal: 
The omentum, or peritoneum, is a thin layer of tissue that covers all of the intestines, and carries the blood supply to them.  The area of the abdomen that is behind the peritoneum, outside of it, is called the retroperitoneum.  This would include the abdominal wall, and a leiomyosarcoma tumor on the abdominal wall [a retroperitoneal leiomyosarcoma] could also come into contact with liver, spleen, pancreas, major arteries and veins, and the ureters and kidneys. 

Latest Pubmed/Medline Search for Leiomyosarcoma with &&url  </b>


Am Surg 2000 Sep;66(9):832-6 
<b>Retroperitoneal soft-tissue sarcomas: prognosis and treatment of primary and recurrent disease. </b>
Bautista N, Su W, O'Connell TX. Department of Surgery, Kaiser Permanente Medical Center, Los Angeles, California 90027, USA. 

<b> Soft-tissue sarcomas of the retroperitoneum constitute a heterogeneous group of tumors with varying histology, potential for complete resection, and propensity for recurrent disease-making the development of effective treatment difficult and challenging. A retrospective review of 23 patients with retroperitoneal sarcomas from 1985 through 1998 was performed to assess the biological behavior and clinical outcomes and to identify factors that may influence prognosis and optimize treatment strategy.  </b>Liposarcomas were the most common pathology ,,,.  Leiomyosarcomas were the next most common pathology (30%); 43 per cent of these were of low grade. Low-grade sarcomas overall accounted for 62 per cent of the total group. Low-grade tumors independent of histologic type exhibited good prognosis for long-term survival with a median survival of 44 months. In contrast, intermediate- or high-grade tumors were associated with a median survival of only 9 months (P < 0.02). On the other hand, tumor histologic type independent of grade did not have a significant survival difference. Complete tumor resection was possible in 21 of 23 patients, which gives an overall resectability rate of 91 per cent. Eight patients (36%) remain disease-free after initial surgical treatment. However, <b>local recurrence was common; this occurred in 11 of 22 patients (50%). Local recurrence, however, did not preclude long-term survival.</b> Surgical resection of recurrent disease was done in nine patients with a median survival of 91 months (range 24-150 months). <b>Three patients had as many as three operations for recurrent disease. With subsequent recurrences there was a decrease in interval from approximately 4 years to 2 years, and 33 per cent of these patients developed tumor dedifferentiation to high grade. An aggressive surgical approach with reoperation can produce prolonged survival in patients with low-grade retroperitoneal sarcoma.</b>  <b>[Ed. This is combined sarcomas, not LMS alone.]</b>

&&url PMID: 10993610

World J Surg 1999 Jul;23(7):670-5 
<b>Primary retroperitoneal soft tissue sarcomas: results of aggressive surgical treatment. </b>
Malerba M, Doglietto GB, Pacelli F, Carriero C, Caprino P, Piccioni E, Crucitti P, Crucitti F. Istituto di Clinica Chirurgica, Universita Cattolica del Sacro Cuore, Largo Agostino Gemelli 8, 00168 Rome, Italy. 

,,, <b>The hospital records of 42 patients with primary retroperitoneal sarcomas who underwent surgical exploration at our unit from 1984 to 1995 were reviewed.</b>   A univariate analysis was used to identify the main clinical, pathologic, and treatment-related factors affecting long-term survival. Twenty-five patients (59.6%) underwent radical surgery. The 5-year survival and 5-year disease-free survival after radical resection were 48.1% and 38.8%, respectively. According to the univariate analysis of survival tumor classification (T), stage and gross surgical margins significantly affected prognosis. The study indicates that even though there are predetermined and unmodifiable tumor-related factors, such as tumor classification (T) and stage, that influence survival in patients with retroperitoneal sarcomas,<b> wide surgical excision offers a concrete chance for long-term survival.</b> <b>[Ed. This is combined sarcomas, not LMS alone.]</b>

&&url PMID: 10390584

Mod Pathol 1999 Jan;12(1):21-8 
<b>Retroperitoneal leiomyosarcomas unassociated with the gastrointestinal tract: a clinicopathologic analysis of 17 cases. </b>
Rajani B, Smith TA, Reith JD, Goldblum JR. Department of Anatomic Pathology, The Cleveland Clinic Foundation, Ohio 44195, USA. 

Data are limited on leiomyosarcomas within the abdomen and retroperitoneum, particularly if one discounts those associated with the gastrointestinal (GI) tract. Recently, some authors proposed that certain tumors in this location are more appropriately termed extra-GI stromal tumors, given their histologic resemblance to GI stromal tumors as opposed to conventional soft tissue leiomyosarcomas. We evaluated the clinical and pathologic features of 17 cases of leiomyosarcoma ,,,  Follow-up intervals ranged from 4 to 169 months (median, 47 mo). Fifteen (88%) of seventeen patients developed an adverse outcome, defined as the development of metastatic disease or death due to tumor. <b>Patients whose tumors had greater than 10 MFs/10HPFs had significantly shorter intervals to either metastasis or death than did those whose tumors had 10 or fewer MFs/10HPFs</b> (8.4 mo vs. 42 mo; P = .003). No other features correlated with time to adverse outcome. In conclusion, the majority of patients with leiomyosarcomas located within the abdomen or retroperitoneum progress to metastatic disease or die from their tumor. The only feature that is significantly associated with a shorter interval to either metastasis or death is more than 10 MFs/10HPFs. <b>[Metastatic disease does not preclude longterm survival. Ed.] </b>  

&&url PMID: 9950158


J Gastrointest Surg 1998 Mar-Apr;2(2):151-5 
<b>Complete hepatic resection of metastases from leiomyosarcoma prolongs survival. </b>
Chen H, Pruitt A, Nicol TL, Gorgulu S, Choti MA. Division of Surgical Oncology and Endocrine Surgery, Department of Surgery, The Johns Hopkins Medical Institutions, Baltimore, Md, USA. 

,,, To determine whether hepatic resection has a role in the management of metastatic leiomyosarcoma, medical records from 11 consecutive patients who underwent resection of isolated metastases from leiomyosarcoma between 1984 and 1995 were reviewed. All liver resections were for leiomyosarcomas originating in the viscera (n = 6) or retroperitoneum (n = 5). The average disease-free interval was 16 months. Five of 11 primary tumors were classified as low grade, whereas six were high grade. Hepatic resections included lobectomy or extended lobectomy (n = 4), segmentectomy and/or wedge resection (n = 5), and complex resection (n = 2). There were no operative deaths. Median survival of all patients after liver resection was 39 months. <b>Patients who underwent complete resection of hepatic metastases (n = 6) had a significantly longer survival than those who had incomplete resections </b>(n = 5) (P = 0.03, log-rank test). Furthermore, five of six patients who underwent complete resection are alive after hepatectomy with a median follow-up of 53 months. <b>Therefore, in selected patients with isolated liver metastases from visceral and retroperitoneal leiomyosarcomas, complete resection of hepatic metastases results in prolonged survival.</b>  

&&url PMID: 9834411 


Am Surg 1998 Jan;64(1):53-60; discussion 60-1 
<b>Leiomyosarcoma: a 45-year review at Charity Hospital, New Orleans. </b>
Hill MA, Mera R, Levine EA. Section of Surgical Oncology, Louisiana State Univ. Medical Center, New Orleans 70112,USA. 

Approximately 2 to 9% of all soft tissue sarcomas are leiomyosarcomas (LMS). LMS arises nearly exclusively as tumors in adults, with peak incidence occurring in the fifth and sixth decades. The purpose of this study was to analyze disease-specific survival and define prognostic factors in patients with this disease who were treated and followed at a single institution. Fifty-eight cases of LMS were identified in the Tumor Registry of the Medical Center of Louisiana at New Orleans (charity Hospital) from 1950 to 1995. Charts were reviewed and tissue blocks reexamined to confirm the diagnosis. Follow-up information was available for 56 of 58 (96%) patients. Univariate and multivariate analyses were performed to analyze which factors predict outcome. The median survival time was 138 months. Univariate analysis identified age (> 48 years), location (retroperitoneal vs other sites), and extent of disease as prognostic factors. Multivariate analysis revealed that only age and the extent of disease at presentation are independent prognostic indicators. Race, sex, and adjuvant therapy were not significant prognostic factors. Surgical resection remains the therapeutic mainstay for patients with LMS. The value of other treatment modalities is largely limited to surgical failures. The data show that the age of the patient and the extent of disease at presentation are the best predictors of long-term survival. LMS has a good prognosis when complete resection of localized lesions can be achieved. 

&&url PMID: 9457038 


Am Surg 1996 Jul;62(7):525-8; discussion 528-9 
<b>Hepatic and vena cava resection using cardiopulmonary bypass with hypothermic circulatory arrest. </b>
Sener SF, Arentzen CE, O'Connor B, Drueck C, Keeler T. Department of Surgery, Evanston Hospital, Illinois 60201, USA. 

When large hepatic or retroperitoneal tumors encroach upon hepatic veins or vena cava and make conventional resection hazardous, the most commonly used method of hepatic resection or vena cava reconstruction includes hepatic vascular exclusion, at times with venovenous bypass or aortic occlusion. These techniques result in warm liver ischemia, and may be accompanied by significant systemic hypotension, despite aggressive central venous preloading. <b>Hepatic lobe (two patients) and retroperitoneal sarcoma (one patient) resections were done in a cold, bloodless field without significant complications</b>. Standard cardiopulmonary bypass techniques with heparin and cardioplegia were used. Systemic circulatory arrest was done at 15 degrees C with isolated retrograde perfusion of the brain through the jugular veins. Hepatic vein and vena cava reconstructions were performed with arrest times of between 30 and 78 minutes. Blood loss was gradual and easily controlled, occurring during the rewarming phase when clot formation was inhibited by cold and heparin. 
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Gynecol Oncol 1995 Dec;59(3):333-7 
<b>Retroperitoneal leiomyosarcoma: eight cases and a literature review. </b>
Todd CS, Michael H, Sutton G. Department of Obstetrics/Gynecology, Indiana Univ. Medical Center,Indianapolis 46202, USA. 

Leiomyosarcoma, a rare malignancy of smooth muscle, may arise from the retroperitoneum and present with the same vague symptoms as a malignancy of the pelvic organs. The purpose of this paper is to review eight cases of retroperitoneal leiomyosarcoma and to illustrate the presenting symptoms, prognostic indicators, and outcomes of patients seen at Indiana University Medical Center between 1989 and 1994. Patients in this study were white females who presented between the ages of 46 and 73 (mean age of 61.4) with nonspecific presenting symptoms of weight loss and back pain (37.5% for each).Tumor size (mean diameter of 14.6 cm in the range 4 to 35 cm), tumor extension, and the presence of distant metastases were of greater prognostic significance than tumor grade (7/8 were grade II, 1/8 was grade I).<b> Surgical resection improved long-term prognosis; three of five patients undergoing resection are alive with no evidence of disease compared to zero of three treated nonsurgically.</b>  Due to location and vague presenting symptoms, this tumor continues to have a bleak prognosis and further evaluation and innovative treatment are required before a reasonable cure rate may be expected. 

&&url PMID: 8522250 


Chirurg 1995 Sep;66(9):899-904 
<b>[Intraoperative radiotherapy within the treatment concept of retroperitoneal soft tissue sarcomas].</b> [in German] 
Willeke F, Eble MJ, Lehnert T, Schwarzbach M, Hinz U, Wannenmacher M, Herfarth C. Chirurgische Universitatsklinik Heidelberg. 

<b>Sarcomas of the retroperitoneum are characterized by a high rate of local recurrence. External beam radiotherapy is known to improve local control after surgical therapy. To increase local dosage of radiotherapy without affection of sensitive structures we applied intraoperative radiotherapy (IORT) since 1991 in a dedicated operative unit. </b>To compare morbidity and tumor control we used a partly historic control group of patients treated since 1988 for retroperitoneal soft tissue sarcoma. 25 patients with a mean age of 53 years were operated. Tumor histology was dominated by liposarcoma and leiomyosarcoma, UICC stage IIB (T2 G2 N0 M0) was present in 45% of cases. Distant metastasis were diagnosed in 19% at therapeutic intervention. Tumor free margins were achieved in 55% while 29% showed microscopic and 16% macroscopic tumor residues after surgical intervention. 11 patients received IORT with a mean of 18 Gy, eight of those patients were treated additionally with a mean of 40,4 Gy externally. There were no differences in distribution of known risk factors for recurrence in the group of patients treated with or without IORT. The analysis showed no difference for perioperative morbidity. Tolerance for IORT and additional external beam radiotherapy was good. <b>Local tumor control tended to be improved by IORT (p = 0.082) while overall survival was not affected at a mean follow-up of 24 months.</b> 

&&url PMID: 7587564 


Int J Radiat Oncol Biol Phys 1994 Jul 30;29(5):1005-10 
<b>Outcome and prognosis in retroperitoneal soft tissue sarcoma. </b>
Catton CN, O'Sullivan B, Kotwall C, Cummings B, Hao Y, Fornasier V. Department of Radiation Oncology, Princess Margaret Hospital, Toronto, Canada. 

,,, The records of 104 patients with retroperitoneal soft tissue sarcoma (RSTS) managed with surgery and irradiation at Princess Margaret Hospital between 1975 and 1988 were retrospectively reviewed. ,,, Presentation was new primary disease, 74; primary recurrence, 20; metastases, 10. Pathology was liposarcoma for 42, leiomyosarcoma for 22, ,,, Grade was low for 36, high for 35, and 33 were not graded. Median tumor size was 17 cm. Grossly complete surgical excision was achieved for 45 (43%), of whom 6 (6%) also had clear surgical margins. Adjuvant postoperative irradiation was administered to 36 patients to a median dose of 40 Gy/20 fractions/4 weeks and 16 received adjuvant chemotherapy. Nine patients received no adjuvant postoperative radiotherapy. Gross residual tumor was present postoperatively in 57 patients. The overall 5- and 10-year survival rates were 36% and 14%, respectively. The locoregional relapse free rate (RFR) was 28% at 5 years and 9% at 10 years, and the distant RFR was 76% at 5 years and 60% at 10 years. For the 45 patients treated with complete excision, survival was 55% and 22% at 5 and 10 years, and locoregional RFR was 50% and 18% at 5 and 10 years. <b>Univariate analysis demonstrated that complete surgical removal was the only factor significant for improved survival, locoregional RFR, and distant RFR.</b> ,,, leiomyosarcoma histology [predicted] for a lower distant RFR, compared to other histologies (p = 0.003). Patients under 62 years had an improved survival (p = 0.002) and local RFR (p = 0.02), and patients presenting with recurrent disease had improved survival (p = 0.03). Sex, tumor size, or grade, or the use of adjuvant chemotherapy were not predictive for any of the endpoints tested.<b> Those who received adjuvant irradiation following gross surgical clearance experienced a prolonged median locoregional RFR over those who did not, and this approached statistical significance for those receiving radiation doses > 35 Gy.</b> (103 months vs. 30 months, p = 0.06). Statistical significance was reached (p = 0.02) if only the infield RFR was considered. ,,, <b>This study demonstrates that failure to achieve local control is the primary cause of treatment failure for patients with RSTS, and that postoperative irradiation in doses > 35 Gy after complete surgery delayed, but did not prevent local recurrence.</b> Improvements in outcome for patients with RSTS will require alternate treatment strategies, and preoperative irradiation with an aggressive surgical attempt at complete excision is currently under investigation. Publication Types:  Clinical trial 

&&url PMID: 8083069 


Cancer 1994 Feb 1;73(3):637-42 
<b>Resectable retroperitoneal soft tissue sarcomas. The effect of extent of resection and postoperative radiation therapy on local tumor control. </b>
van Doorn RC, Gallee MP, Hart AA, Gortzak E, Rutgers EJ, van Coevorden F, Keus RB, Zoetmulder FA. Department of Surgical Oncology, The Netherlands Cancer Institute, Amsterdam. 

,,, Soft tissue malignancies of the retroperitoneum are rare. Surgery is commonly considered the first choice of treatment. There is still no consensus concerning the precise value of the extent of surgery and the value of additional treatment modalities. ,,, From 1973 to 1990, 34 patients were initially treated by surgery for retroperitoneal soft tissue sarcomas at the Netherlands Cancer Institute. In a retrospective study, the prognostic effect of extent of primary surgery and postoperative radiation therapy was examined. ,,, Liposarcomas (47%) and leiomyosarcomas (29%) were the histologic types most commonly found. Grade of malignancy was defined as medium to high (II/III) in 44% and as low (I) in 38%. Complete (extended or marginal) resection of the tumor was achieved in 29 patients. After complete resection, the 5-year survival rate and the local recurrence rate were 35% and 63%, respectively. Extended surgery (performed in eight patients) showed a local recurrence rate of 50%. <b>Thirteen patients received high-dose postoperative radiation therapy, which was found to have a significant favorable effect on the recurrence-free interval (P < 0.01).</b> CONCLUSIONS. We conclude that complete (extended) resection followed by high-dose postoperative radiation therapy improves the prognostic outcome of patients with retroperitoneal soft tissue sarcomas. [NOTE: we don't have the figures broken down for lms vs. other sarcomas in terms of response to radiation. It might be worthwhile to try to get the complete article. Ed.] Publication Types: Clinical trial 

&&url PMID: 8299085 


Zentralbl Chir 1994;119(7):488-94 
[Results of surgical therapy of soft tissue sarcoma of the retroperitoneum]. [Article in German] 
Nagel M, Ockert D, Stoelben E, Saeger HD. Chirurgische Universitatsklinik Mannheim. 

Between 1.1.1973 and 30.6. 1993 57 patients underwent operation for soft-tissue sarcoma of the retroperitoneum.  The histological classification showed in 21 cases a liposarcoma and in 14 cases a leiomyosarcoma. Other histological types like fibrosarcoma, malignant fibrous histiocytoma or malignant schwannoma were only rarely seen. Complete resection (R-0-resection) was possible in 39 cases (73.5%). 6 patients underwent partial resection (R-2-resection) and 8 patients had only an explorative laparotomy because of an unresectable tumor. To realize a complete resection in 23 cases a multivisceral resection was necessary. The postoperative staging according to AJCC showed for 47.7% of the patients a stage III- or stage IV- disease. The clinical follow-up was observed between 3 months and 14.3 years. The cumulative 5-year-survival-rate for all patients was 38.2%.<b> The most important factor for prognosis was the complete resection, while other factors like sex and age of patients, size of the tumor and histological type did not predict outcome. After complete resection the cumulative 5-year-survival-rate was 46.7%, while after partial resection or explorative laparotomy no patient survived 5 years.</b> 

&&url PMID: 7524249
