Retroperitoneal soft tissue sarcoma

The objective of the current study was to define the optimal treatment for patients with retroperitoneal soft tissue sarcomas (RPS). The authors conducted a review of the pertinent literature and found that the majority of patients had locally advanced RPS at the time of diagnosis. Surgery was the mainstay of treatment, and macroscopic total excision was feasible in approximately 50-67% of patients. The 5-year probability of local control was approximately 50%, and the likelihood of developing distant metastasis was approximately 20-30%. The 5-year survival rate was approximately 50%, and deaths due to recurrent RPS continued to occur after 5 years. Postoperative radiotherapy (RT) decreased the likelihood of local recurrence, although it demonstrated no impact on survival. Adjuvant chemotherapy had no proven efficacy. The factor that was found to have the greatest impact on the prognosis for patients with RPS was the ability to achieve a macroscopic total resection. The authors concluded that RPS usually is advanced at the time of diagnosis. The optimal treatment was complete resection. Most patients experienced recurrent disease after surgery, and the most common site of failure was local. Adjuvant RT reduced the risk of local recurrence, but its impact on survival was questionable.     

Retroperitoneal sarcoma treated by surgery

A retrospective analysis of extensive surgery with resection of retroperitoneal sarcoma and total or partial resection of adjacent organs in 15 patients is presented. Dominating symptoms were a palpable mass (80%). The tumor was felt in all patients. In 9 of 10 patients, ultrasonography showed a solid tumor with retroperitoneal location. In 14 patients (93%) the tumor was removed, in 11 of them adjacent organs also. Five of the patients were subjected to more than one operation because of tumor recurrence. Two patients died postoperatively. Six patients are alive without tumor recurrence; and three, with recurrence 18 to 62 months after the first operation. Four patients died 5–24 months after primary operation. In seven patients (50%), the tumor recurred locally. Surgery for recurrent tumor was beneficial. Complete surgical removal provides the most effective therapeutic approach. Routine second-look operation within a year after primary surgery may be a way to improve the results. Adjuvant cytostatic therapy could be considered.     

Soft tissue sarcoma in adults.

Soft tissue sarcomas are a heterogeneous group of tumors, consisting of numerous histiotypes that all share a putative common mesenchymal origin. Although prognosis of these tumors is determined by clinical parameters (size, location, and resection margin status) and pathologic features (mitotic activity and necrosis), the histologic subtype has never been shown to be a consistent independent prognostic factor. Some relevant differences among these histiotypes are emerging, in specific biological parameters such as proliferation indices, in integrin expression profiles, and with regard to drug sensitivity. Several biological factors are considered to be prognostically important. Most attention is directed to regulators of cell-cycle progression. The significance of p53 dysregulation is confirmed by the inhibition of cellular proliferation, both in in-vitro and in in-vivo sarcoma models, after reintroduction of wild type p53. A multidisciplinary approach is essential for the optimal treatment of soft tissue sarcomas. Multimodality treatment has led to a patient-tailored approach with limb-sparing resections integrated with external and/or interstitial irradiation. The value of chemotherapy both in the neoadjuvant and the adjuvant setting, although of critical value in other sarcomas such as Ewing's sarcoma and osteosarcoma, remains to be established for soft tissue sarcomas.     

Retroperitoneal mass presentations of B-immunoblastic sarcoma

A clinicopathologic analysis of nine patients with B-immunoblastic sarcoma (B-IBS) presenting as a bulky lymph node-based retroperitoneal mass is reported. The histologic and immunologic findings, similar to those reported in B-IBS presenting in various other nodal and extranodal sites, support the recognition of this aggressive large cell non-Hodgkin's lymphoma (NHL) as a distinct pathologic entity. The patients, with a mean age of 60.5 years, presented for evaluation of abdominal pain and a palpable abdominal mass. Four patients were Stage II or IIE, one Stage III, and four Stage IV; eight of nine had B symptoms. Chemotherapy (cyclophosphamide, doxorubicin, vincristine, prednisone, bleomycin [CHOP-B] or cyclophosphamide, doxorubicin, vincristine, methotrexate with leucovorin rescue, cytarabine [ACOMLA]) yielded significant palliation in five patients (mean survival, 12.3 months); three untreated patients and one receiving radiation treatment (XRT) died within 1 month from diagnosis. There was a striking predilection for pleuropulmonary involvement in disseminating disease. The initial blood lymphocyte count correlated significantly with survival (correlation coefficient, 0.84). The one durable complete remission (CR) was obtained in a patient who received substantial surgical debulking before chemotherapy.     

Retroperitoneal sarcoma: a population-based analysis of epidemiology, surgery, and radiotherapy

BACKGROUND: No population-based studies of retroperitoneal sarcoma (RPS) have been conducted, and the use and timing of adjuvant radiotherapy for RPS is controversial. The objective of this study was to examine the incidence and treatment of RPS, specifically regarding the use of adjuvant radiotherapy. METHODS: The Surveillance, Epidemiology, and End Results (SEER) database was used to evaluate the incidence of RPS over a 29-year period (1973-2001). The rate of surgery, the rate and timing of adjuvant radiotherapy, and the influence of demographic factors on treatment were evaluated. RESULTS: A total of 2348 cases of RPS were identified. The mean annual incidence of RPS was 2.7 cases per 10(6) persons and did not change significantly over time (2.6 in 1973 vs. 2.8 in 2001; P = .92). Most patients (1654; 70.4%) underwent surgical resection. Radiotherapy was used in 428 patients (25.9%) who underwent surgery; radiation was given postoperatively in 366 (85.5%), preoperatively in 20 (4.7%), and intraoperatively or unknown in 42 (9.8%). Patients who received any adjuvant radiotherapy were on average 5 years younger than those who underwent surgery alone (P < .0001). Radiotherapy was more commonly used among whites than African Americans (25.8% vs. 16.7%; P = .02) and there was significant variation in the use of adjuvant radiotherapy by geographic location (P = .003). On multivariate analysis, race (P = 0.004), age (P < .0001), and geographic location (P = .006) were independently associated with the use of adjuvant radiotherapy. CONCLUSION: The incidence of RPS, a rare disease, appears stable. Most patients who undergo surgery do not receive any adjuvant radiotherapy, and very few receive preoperative radiotherapy. Differences in adjuvant radiotherapy use related to demographic and geographic factors suggest that at least some treatment variations reflect differences in individual and institutional practice patterns.     

Retroperitoneal follicular dendritic cell sarcoma in a young woman: Diagnosis and treatment challenges

IntroductionFollicular dendritic cell sarcoma (FDCS) is an uncommon tumor that usually arises in lymph nodes, especially in the cervical, mediastinal, or axillary areas, but rarely in extranodal sites. Few cases have been reported in English literature so far. The scarcity may be partially due to under-recognition of this entity. Through this case report we analyzed the difficulties of clinical and pathological diagnosis of this rare tumor with its unusual location mistaken it with gynecological cancer’s iliac lymph nodes metastases. We also discussed its systemic treatment options.Case reportA 48-year-old woman presented with a loss of weight and epigastralgia. Computed tomography (CT) showed a mass of 5 cm of diameter, located close to iliac vessels. Investigation for gynecologic cancers was negative and a partial tumor resection was performed. Pathological examination readdressed the diagnosis of FDCS. Microscopically, the tumor was composed of a proliferation of spindle to ovoid cells arranged in fascicles, whorls and storiform pattern, accompanied by sprinkling of small lymphocytes. The nuclei of the tumor cells were elongated spindled or ovoid shape with vesicular chromatin and distinct small nuclei. Immunohistochemically, the tumor cells were positive for CD21, CD23 but negative for any type of cytokeratin. Even pathological diagnosis was misleading, therapeutic management was more challenging with this unusual location particularly associated with an aggressive clinical course. Two lines of chemotherapy gave different responses.ConclusionClinical and pathological diagnosis of retroperitoneal FDCS needs vigilance. Both lymphoma and sarcoma chemotherapy regimens are effective. Due to this pathology’s rareness we highlighted a lack of treatment consensus and proposed options.     

Adjuvant role of radiation in soft tissue sarcoma in adults.

Forty-seven patients with soft tissue sarcomas were analyzed retrospectively. Treatment was non-randomized between surgery alone, preoperative radiation of 5,000 rads/5 weeks, postoperative radiation of 6,000 rads/6 weeks following total gross tumor removal, and postoperative radiation (variable dosees) for residual gross or unresectable tumor. Preoperative radiation was not statistically better for local control or survival compared to postoperative radiation after local excision of gross tumor. Wide excision, higher postoperative radiation dosage, and/or enlarged radiation portals perhaps would improve results. Additional adjuvant therapies, such as chemotherapy or immunotherapy, needs to be investigated.     

Retroperitoneal soft tissue sarcoma: prognostic factors and therapeutic approaches

AIMS AND BACKGROUND: Retroperitoneal sarcomas are a rare group of malignant soft tissue tumors with a generally poor prognosis. The aim of the study was to assess clinical, pathological and treatment-related factors affecting prognosis in patients with retroperitoneal sarcomas. METHODS AND STUDY DESIGN: The hospital records of 73 patients who underwent surgical exploration at our unit for primary retroperitoneal sarcomas between 1984 and 2003 were reviewed. Factors influencing overall and disease-free survival were analyzed for all patients and for those who underwent complete surgical resection. RESULTS: The complete resectability rate was 69.8% (51/73). Operative mortality and morbidity rates were 2.7% and 21.9%, respectively. For patients who underwent complete resection, the 5-year survival rate was 58.3%, whereas it was 0% in cases of incomplete or no resection (P < 0.001). Local recurrence rate was 37.2%. Incomplete gross surgical resection and microscopic infiltration of margins were the most important independent predictors of a poor prognosis. CONCLUSIONS: The present study confirmed the importance of an aggressive surgical management for retroperitoneal sarcomas to offer these patients the best chance for long-term survival.     

Retroperitoneal sarcoma with infected necrosis: an unfavourable prognostic factor

Purpose. To report the phenomenon of infected retroperitoneal sarcoma (RPS).Method. Two case reports.Results. Both patients died soon after laparotomy.Discussion. Infected RPS is identified as an entity not clearly documented in the literature. It should probably be added to the list of poor prognostic factors when planning the management of patients with RPS.     

Retroperitoneal soft tissue sarcoma: effect of hyperthermic total abdominal perfusion

AIMS AND BACKGROUND: Soft tissue sarcomas (STS) of the retroperitoneum are rare tumors. Surgery remains the principal modality of therapy in the management of primary and recurrent retroperitoneal STS. However, little is known about the effect of regional chemotherapy using hyperthermic total abdominal perfusion (HTAP). We analyzed independent prognostic variables in 33 patients with STS in the retroperitoneum admitted from November 1990 through December 1996. METHODS AND STUDY DESIGN: Data regarding patients' age, gender, tumor size, histological tumor type, tumor location, type of operation (primary or secondary surgery), extent of surgical management (marginal or extended), use of HTAP, tumor grade, and tumor stage according to the TNM classification were examined by univariate and multivariate analyses. RESULTS: All 33 patients underwent complete resections (marginal or extended). Eleven of them received locoregional chemotherapy by HTAP. The overall cumulative 5-year survival rates in patients with stage IIA and advanced disease were 82% and 22%, respectively (log-rank test, P<0.01). Using Cox's proportional hazard model, tumor stage, use of HTAP and type of operation were found to have significant influence on overall survival (P<0.05). CONCLUSIONS: Our results showed that complete resection along with HTAP chemotherapy may improve survival in patients with retroperitoneal STS. These phase II data could be used to support the initiation of a phase III trial to test HTAP in patients submitted to complete resection of retroperitoneal STS.     

Soft-tissue sarcoma in adolescents and young adults

International Journal of Clinical Oncology - Soft-tissue sarcoma is a rare cancer that accounts for approximately 1% of all malignant tumors. Although they occur in various age groups, soft-tissue...     

Undifferentiated embryonal sarcoma of the liver in adults

BACKGROUND: Undifferentiated embryonal sarcoma of the liver (UESL), a rare tumor that predominantly affects children, generally has been considered an aggressive neoplasm with an unfavorable prognosis. More recent reports have indicated that modern multimodal treatment and supportive care improve the survival of children with UESL. Data regarding the treatment and survival of adults have not been reviewed comprehensively, and only a few adult patients with UESL have been reported in the literature. METHODS: The authors analyzed demographics, treatment, and actuarial survival of all reported cases of UESL in patients aged >or=15 years (n = 67 patients). In addition, 1 case is presented of a patient with UESL who was treated successfully at the authors' institution. RESULTS: The median survival of all patients with UESL who were analyzed was 29 months. Patients who underwent complete tumor resection followed by adjuvant chemotherapy survived over a median follow-up of 28.5 months and had significantly better survival compared with patients who underwent surgical treatment alone. Patients who underwent an incomplete tumor resection had a tendency toward poorer outcomes. CONCLUSIONS: To the authors' knowledge, this is the first report to demonstrate a significant effect on survival for adjuvant chemotherapy after complete surgical resection of UESL in adults. The role of neoadjuvant chemotherapy was not evaluated in this study. In the case study presented herein, combined therapy with surgery and chemotherapy led to a complete, sustained remission that has lasted for >6 years to date.     

Retroperitoneal soft tissue sarcoma: an analysis of radiation and surgical treatment

PURPOSE: To evaluate the clinical outcomes of patients with localized retroperitoneal soft tissue sarcoma (STS) treated with complete surgical resection and radiation. METHODS AND MATERIALS: The medical records of 83 patients were reviewed retrospectively. Sixty patients presented with primary disease and the remaining 23 had recurrence after previous surgical resection. RESULTS: With a median follow-up of 47 months, the actuarial overall disease-specific survival (DSS), distant metastasis-free survival, and local control (LC) rates were 44%, 67%, and 40%, respectively. Of the 38 patients dying of disease, local disease progression was the sole site of recurrence for 16 patients and was a component of progression for another 11 patients. Multivariate analysis indicated that histologic grade was associated with the 5-year rates of DSS (low-grade, 92%; intermediate-grade, 51%; and high-grade, 41%, p = 0.006). Multivariate analysis also indicated an inferior 5-year LC rate for patients presenting with recurrent disease, positive or uncertain resection margins, and age greater than 65 years. The data did not suggest an improved local control with higher doses of external-beam radiation (EBRT) or with the specific use of intraoperative radiotherapy (IORT). Radiation-related complications (10% at 5 years) developed in 5 patients; all had received their EBRT postoperatively. CONCLUSIONS: Although preoperative radiation therapy and aggressive surgical resection is well tolerated in patients, local disease progression continues to be a significant component of disease death. In this small cohort of patients, the use of higher doses of EBRT or IORT did not result in clinically apparent improvements in outcomes.     

Retroperitoneal sarcomas.

BACKGROUND: The evaluation and treatment of retroperitoneal sarcomas are challenging because the tumors are relatively rare and frequently present with advanced disease in an anatomically complex location. METHODS: We reviewed the literature on experience in the management of retroperitoneal sarcomas, and we present our own experience in the treatment of these tumors. RESULTS: The identification of prognostic factors other than the adequacy of resection has been inconsistent. Due to a lack of associated symptoms, retroperitoneal sarcomas smaller than 5 cm are rare. Computed tomography is the most useful tool in the evaluation of retroperitoneal tumors. Surgery, radiation therapy, and chemotherapy are treatment options, but the most important factor in the treatment of primary tumors is complete surgical resection. The role of neoadjuvant and adjuvant therapies is not defined and should be considered within the context of clinical trials. CONCLUSIONS: Early referral of patients with retroperitoneal soft tissue tumors will help to ensure that they will receive the benefits of multidisciplinary evaluation and treatment of their disease and ready access to clinical trials.     

Cancer chemotherapy in the elderly: a series of 51 patients aged>70 years

Summary A total of 2,238 new cancer patients were treated in our institution in 1988; among the 423 (18.9%) who were>70 years old, 51 underwent chemotherapy. The median age was 75.8 years, and the Karnofsky performance status (KPS) was 70% for 40 patients. Malignancies were hematopoietic in 24 cases (47%) and digestive in 15 patients (29%), and 12 subjects (24%) had other types of cancers. The first chemotherapy course was given at the full dose to 23/51 (45.1%) patients. The drug dose was reduced for 28/51 (54.9%) patients, due in 25 cases to the subjects being>70 years old. Neither age, KPS, pretreatment assessment, nor cancer extent was correlated with the modifications made to the first cycle. An overall toxicity of grade 3+4 (WHO grading scale) was noted in 10 subjects (19.6%). Although these elderly patients were probably selected, analysis of their charts did not evidence an increase in chemotherapy toxicity, regardless of the dose they received.Presented at the EORTC Pharmacokinetics and Metabolism Group Meeting, Bordeaux, November 1990     

Clinical outcomes of surgical resections for primary liver sarcoma in adults: results from a single centre.

BACKGROUND: Primary hepatic sarcoma is a rare tumour with a poor prognosis. METHODS: From 1997 to 2002 eight patients had liver resection for primary sarcoma of the liver at our institution. The clinical characteristics, imaging findings, surgical procedures, adjuvant therapy and outcome were retrospectively reviewed. There were two patients each with angiosarcoma (AS), leiomyosarcoma (LMS), and undifferentiated embryonal sarcoma (UES), one patient with epithelioid hemangioendothelioma (EHE) and one patient with malignant peripheral nerve sheath sarcoma (PNSS). RESULTS: The most common presenting symptoms were right upper quadrant pain and fever. Typical imaging findings were a heterogenous mass with poorly defined margins, pseudocapsule and aberrant vasculature. Preoperative diagnosis of a primary liver sarcoma was made in 7/8 cases, either by fine needle aspiration (n = 5) or angiography (n = 2). Five right hepatectomies and three trisegmentectomies were performed. An R (0) resection was possible in three cases. Two patients developed complications and there was one death. Adjuvant chemoradiotherapy was administered to 5/7 patients. Systemic chemotherapy led to tumour regression in both patients with UES which enabled a second hepatic resection. CONCLUSIONS: The majority of patients with primary liver sarcoma present with right upper quadrant pain, fever and a liver mass. Differentiating the rare primary liver sarcoma from the much more common hepatocellular carcinoma (HCC) may aid in planning therapy. Patients with resectable tumours should be referred for surgery. Liver resection combined with adjuvant chemotherapy are the mainstays of treatment for UES in the adult.     

Prognostic factors of extremity soft tissue sarcoma in adults. A single institutional analysis

PurposeTo analyse the prognostic factors for patients treated with limb sparing surgery and radiation for extremity soft tissue sarcoma (E-STS).Patients and methodsMedical records of 87 patients with limb sparing surgery and radiation for E-STS were reviewed retrospectively. Disease-free survival (DFS) and disease-specific survival (DSS) were estimated and factors potentially influencing these outcomes were analysed.ResultsWith a mean follow-up of 69 months, most recurrences occurred within the first 2 years. Extent of resection margin was found to improve DFS (P = 0.002) and DSS (P = 0.002). Brachytherapy combined with external beam radiotherapy (EBRT) improved DFS (P = 0.034) and DSS (P = 0.019). Tumor size (< 10 cm) was related to DSS (P = 0.043) and its relation to DFS was almost significant (P = 0.057). Short time interval between surgery and radiotherapy (≤ 50 days) had an impact only on DSS (P = 0.030).ConclusionExtent of resection margin and use of brachytherapy combined with EBRT seem to improve the prognosis of E-STS. Small tumor size and short time interval between radiotherapy and surgery seem also to improve the outcome of E-STS. This study was limited by inadequate power and low number of recurrences. Larger randomised studies are needed to confirm these results.     

Ewing sarcoma in adults treated with modern radiotherapy techniques

Background and purposeTo evaluate local control and survival outcomes in adults with Ewing sarcoma (ES) treated with radiotherapy (RT).Material and methodsRetrospective review of all 109 patients age ⩾18 treated for ES with RT to the primary site at Memorial Sloan Kettering Cancer Center between 1990 and 2011. RT was used as the definitive local control modality in 44% of patients, preoperatively for 6%, and postoperatively for 50%.ResultsMedian age at diagnosis was 27 years (range, 18–67). The 5-year local failure (LF) was 18%. Differences in LF were not identified when evaluated by modality of local control (RT versus combined surgery and RT), RT dose, fractionation, and RT technique. However, margin status at time of resection significantly predicted LF. The 5-year event-free survival and overall survival rates were 44% and 66% for patients with localized disease, compared with 16% and 26% for metastatic disease (p = 0.0005 and 0.0002). Tumor size, histopathologic response to chemotherapy, and treatment on or according to a protocol were also significantly associated with survival.ConclusionsThis series of adults treated with modern chemotherapy and RT had prognostic factors and outcomes similar to adolescents with ES. All adults with ES should be treated with an aggressive, multidisciplinary approach.