Primary Retroperitoneal Sarcoma Research Articles

Prognostic role of cyclin D1 in retroperitoneal sarcomas.

Cyclin D1 regulates the G1 checkpoint of the cell cycle and is overexpressed in a number of cancers. This study was designed to determine if cyclin D1 overexpression had prognostic value in patients undergoing surgery with curative intent for primary retroperitoneal soft-tissue sarcoma. Tissue was available for analysis on 79 patients who underwent resection between September 1983 and May 1997. Clinicopathologic data and follow-up was obtained from a prospective sarcoma database and a patient and family interview. Immunohistochemical analysis was used to determine overexpression (> or = 5% of nuclei labeled). Survival was analyzed using the Kaplan-Meier method, and statistical analysis was performed by using log rank testing and the Cox regression model. Median follow-up was 3.5 years. On univariate analysis of disease-specific mortality, significant prognostic factors were high grade (n = 42, 53%), positive microscopic margins (n = 36, 46%) or macroscopic margins (n = 15, 19%), and cyclin D1 overexpression (n = 37, 47%). On multivariate analysis, macroscopically positive margins (P = 0.02) and the combination of high grade and cyclin D1 overexpression (P = 0.04) both were associated independently with poor survival. High grade retroperitoneal sarcomas demonstrating cyclin D1 overexpression have had an extremely poor prognosis. Continued study of multiple biological markers, exemplified by cyclin D1, may aid characterization of tumor behavior and response to treatment in this diverse group of tumors.

Prognostic factors in retroperitoneal sarcoma: a multivariate analysis of a series of 165 patients of the French Cancer Center Federation Sarcoma Group.

Surgery is the main prognostic factor in retroperitoneal sarcoma. However, despite progress, surgery alone is rarely curative, and analysis of the causes of failures and of other prognostic factors are warranted to ascertain treatment orientations. Data of patients treated from 1.80 to 12.94 for primary retroperitoneal sarcoma were extracted from the French Federation of Cancer Centers Sarcoma Group registry. Univariate and multivariate analysis were performed for initial local control and for local and general outcome. One hundred sixty-five patients (median age, 54 years; range, 16--82 years) were identified. Median tumor size was 15 cm (range, 2--70 cm); 31% of tumors presented with neurovascular or bone involvement. Liposarcoma, leiomyosarcoma, and malignant fibrous histiocytoma represented 66% of the tumors. Eighty-four percent of the tumors were of high or intermediate grade. Twenty patients had initial metastases. Multimodality treatment included surgery (150 patients), radiotherapy (92 patients), and chemotherapy (77 patients). Complete excision was achieved in 94 of 145 nonmetastatic patients. Median follow-up was 47 months (range, 3--160 months). Actuarial overall 5-year survival rate (median) was 46% (51 months). The main prognostic factors for survival were initial metastases and surgery, which represented the major treatment-linked factor. High-grade of tumors affected local recurrence, metastatic recurrence, and survival. Adjuvant radiotherapy was significantly associated with reduced local recurrence. Various evolutive patterns were observed with histologic subtypes. Aggressive surgery remains mandatory in retroperitoneal sarcoma, but a randomized trial is needed to evaluate the place of radiotherapy for local control.

Retroperitoneal sarcomas.

The approach to the management of retroperitoneal tumors begins with a complete history and physical examination. Imaging of the abdomen and pelvis by computed tomography (CT) provides both an imaging modality and a method by which to obtain tissue for diagnosis. Because a histologic diagnosis is essential in treatment planning, adequate tissue can usually be obtained by a CT-guided core biopsy. If the diagnosis is sarcoma, additional tests necessary for staging include plain chest radiography and evaluation of the liver by either CT scan or magnetic resonance imaging (MRI). The treatment options for primary retroperitoneal sarcomas include chemotherapy, radiation therapy, surgery, or a combination of these modalities; therefore, a multidisciplinary group best manages treatment planning. Primary radiation therapy for cure is seldom effective for retroperitoneal sarcomas but can provide palliation in select cases. Systemic chemotherapy for chemosensitive lesions, such as poorly differentiated liposarcoma, malignant fibrous histiocytoma (MFH), synovial cell sarcoma, and primitive neuroectodermal tumors (PNET), can be useful when used in a neoadjuvant manner. Consequently, surgical resection continues to be the mainstay of treatment for retroperitoneal sarcomas and requires en bloc resection of the primary tumor. Frequently this includes adjacent organs such as colon, small bowel, kidney, adrenal, and pancreas. Postoperative adjuvant therapy with chemotherapy or radiation has not been proven to be of any additional benefit. Overall treatment results are predominantly influenced by tumor stage, grade, size, and margins of surgical resection. Follow-up CT scans at 6-month intervals and surgical resection of recurrences can be valuable.

Primary retroperitoneal soft tissue sarcomas: results of aggressive surgical treatment.

A retrospective study was undertaken to evaluate the results of surgical treatment in a series of patients with primary retroperitoneal sarcomas consecutively treated by the same surgical team. The hospital records of 42 patients with primary retroperitoneal sarcomas who underwent surgical exploration at our unit from 1984 to 1995 were reviewed. 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, wide surgical excision offers a concrete chance for long-term survival.

Long-term results of intraoperative electron beam radiotherapy for primary and recurrent retroperitoneal soft tissue sarcoma

Long-term results of intraoperative electron beam radiotherapy for primary and recurrent retroperitoneal soft tissue sarcoma

Primary Retroperitoneal Sarcomas

Primary retroperitoneal sarcomas may present with symptoms and signs that mimic common musculoskeletal disorders of the extremities that are quite remote from the source of the problem. This often misleads the clinician and results in delays in diagnosis. The authors present 6 patients with retroperitoneal sarcoma who had a common or nonspecific orthopaedic condition of the extremity. Delays in diagnosis ranged from 2 to 30 months. No patient survived his or her tumor. Clinicians should be alerted to the possibility of a retroperitoneal tumor that presents primarily or initially with extremity signs and symptoms but with few or no clues of the presence of a localized sarcoma in the retroperitoneal space.

Resectability of retroperitoneal sarcomas: a matter of surgical technique?

The management of retroperitoneal sarcomas has been hampered by the difficulty in complete resection, the resectability rate in the literature being about 53%. In a review of the last 88 consecutive patients with retroperitoneal sarcomas the resectability rate was 95%. At a mean follow-up of 48 months, the local recurrence rate was 17% following wide resection and 59% following local excision (P = 0.0002). For patients with minimum follow-up of 5 years, the local recurrence rate was 39% for those with primary tumours and 57% for those referred with local recurrence. Local recurrence diminished the rate of long-term survival. The 5- and 10-year survival rates for the primary retroperitoneal sarcomas (n = 55) were 66% and 57% and for those referred with locally recurrent sarcoma (n = 33) 57% and 26%, respectively. The 5-year survival rate varied significantly with the grade of the tumour, from 88% for Grade I to 44% for Grade III tumours (P = 0.006). In conclusion, with modern surgical techniques the resectability rate of retroperitoneal sarcomas is about 95%, and the survival rate of the primary tumours approximates that of the primary soft tissue sarcomas of the extremities.

Intraoperative electron beam radiation therapy for retroperitoneal soft tissue sarcoma

From December 1981 to December 1989, 20 patients with primary or recurrent retroperitoneal sarcoma received 4000 to 5000 cGy of external beam radiation therapy (EBRT) in conjunction with surgical resection and intraoperative radiation therapy (IORT). Seventeen of 20 patients underwent complete (14 patients) or partial (3 patients) resection. Three patients had shown evidence of metastases after EBRT by the time of surgery. The 4-year actuarial local control and disease-free survival rates of the 17 patients undergoing resection were 81% and 64%, respectively. Twelve patients received IORT at the time of resection for microscopic disease (10 patients) or gross residual sarcoma (2 patients). Of the ten patients receiving IORT for microscopic tumor, one patient has died of local failure and peritoneal sarcomatosis and two patients have died of distant metastases only. The remaining seven patients are disease-free. One patient treated for gross residual sarcoma has experienced a local failure 1 year after IORT and is without disease 7 years after salvage chemotherapy. The other patient treated for gross residual sarcoma has died of local failure. Five patients did not receive IORT at the time of resection because of the extensive size of the tumor bed. Three of these patients are disease-free with one patient alive with lung metastases and one patient dying of hepatic metastases. Aggressive radiation and surgical procedures appear to provide satisfactory resectability and local control with acceptable tolerance.

Prognostic Factors in Primary Retroperitoneal Soft-Tissue Sarcomas

We analyzed independent treatment variables (age, sex, signs and symptoms, site, size, histopathologic findings, grade, and clinical presentation) and treatment-dependent variables (resectability, type of operation, surgical margins, surgical boundaries, microscopic margins, adjuvant radiotherapy, and adjuvant chemotherapy) in 80 patients with primary retroperitoneal soft-tissue sarcomas admitted from 1982 through 1988. Both univariate and multivariate analysis of survival and disease-free survival were performed. The major factor in survival outcome was the ability to completely resect the lesion. When the 62 patients who underwent complete resection were examined, the only independent prognostic factor for both survival and disease-free survival was grade. We conclude that completeness of resection and grade of the lesion are primary determinants of survival. Once all tumor is macroscopically removed, no advantage could be demonstrated by more extensive surgical resection or current adjuvant therapy.

Management of Primary and Recurrent Soft-tissue Sarcoma of the Retroperitoneum

From 1982 to 1987, 114 patients underwent operation at Memorial Sloan-Kettering Cancer Center for soft-tissue sarcoma of the retroperitoneum. A retrospective analysis of these patients defines the biologic behavior, surgical management of primary and recurrent disease, predictive factors for outcome, and impact of multimodality therapy. Complete resection was possible in 65% of primary retroperitoneal sarcomas and strongly predicts outcome (p less than 0.001). The rate of complete resection was not altered by histologic type, size, or grade of tumor. These patients had a median survival of 60 months compared to 24 months for those undergoing partial resection and 12 months for those with unresectable tumors. Forty-nine per cent of completely resected patients have had local recurrence. This is the site of first recurrence in 75% of patients. These patients undergo reoperation when feasible. Complete resection of recurrent disease was performed in 39 of 88 (44%) operations, with a 41-month median survival time after reoperation. Tumor grade was a significant predictor of outcome (p less than 0.001). High-grade tumors (n = 65) were associated with a 20-month median survival time compared to 80 months for low-grade tumors (n = 49). Gender, histologic type, size, previous biopsy, and partial resection versus unresectable tumors did not predict outcome by univariate analysis. Adjuvant radiation therapy and chemotherapy could not be shown to have significant impact on survival. Concerted attempt at complete resection of both primary and recurrent retroperitoneal soft-tissue sarcoma is indicated.

Primary retroperitoneal sarcomas

A retrospective analysis of 20 adult patients with primary retroperitoneal sarcomas demonstrated that most patients presented with pain and a palpable abdominal mass. Liposarcomas and leiomyosarcomas were the most common tumors. Seven of the 20 patients (35 percent) had complete tumor excision. Operative morbidity was 18 percent with no mortality. After complete resection, the 5 year survival rate was 43 percent. Thirteen patients underwent partial excision of tumor, with an operative morbidity rate of 29 percent and a mortality rate of 15 percent. No patient in our series lived more than 2 years after only partial excision. Anatomic restrictions to wide resection and local recurrence were the most important factors determining survival. Aggressive, complete surgical resection in treating primary retroperitoneal sarcomas is strongly recommended.

Primary retroperitoneal sarcomas. A review of 36 cases.

A retrospective study of 36 patients with primary retroperitoneal sarcomas treated at The Norwegian Radium Hospital is presented. The median age at presentation was 57 years. The most common presenting symptom was abdominal pain. Leiomyosarcoma and liposarcoma were the most common histologic subtypes. The median survival in the whole series was 25 months. Patients with completely resected tumors had a longer median survival (59 months) than patients with incomplete resection (16 months) but the difference was not statistically significant. The malignancy grade seemed to be the most important prognostic factor and patients with low grade tumors had a significantly better outlook than those with high grade tumors.

Improved survival following complete excision of retroperitoneal sarcomas.

Charts and slides of 47 patients with primary retroperitoneal sarcomas (excluding pediatric rhabdomyosarcoma) were reviewed to determine clinical presentation, histologic features, extent of surgical resection, operative morbidity and mortality, use of radiation and/or chemotherapy, and survival data. Most patients presented with pain and a palpable mass. Leiomyosarcomas and liposarcomas were the most common tumors. Eighteen of the 47 patients (38%) had complete tumor excision; 68% required resection of adjacent organs. Operative morbidity was 33% with no mortality. After complete resection, the disease-free 5-year survival was 50% and the overall survival was 70% at 5 years; 10-year disease-free survival was 25% with an overall 58% survival at 10 years. Eleven patients (61%) developed recurrent disease with a median interval of 5 years following complete excision. Six patients received adjuvant radiation and/or chemotherapy with four remaining disease-free from 46 to 61 months. Eighteen patients underwent partial excision of tumor and 11 patients underwent biopsy only; these groups had similar survival curves with only 4% alive at 5 years. Their operative morbidity was 18% and mortality was 7%; median time to clinical evidence of tumor progression was 12 months. Sixty per cent of these patients received therapeutic radiation and/or chemotherapy, but their survival was the same as those undergoing surgery alone. These data emphasize the importance of an aggressive surgical approach in the treatment of retroperitoneal sarcomas. Complete tumor resection and total excision of recurrences will allow many patients long-term survival.

Primary Retroperitoneal Sarcomas: The Role of Surgical Treatment

Malignant tumors arising from the anatomic structures of the retroperitoneal space are rare [l--5]; therefore, it has been difficult to accurately assess their prognosis and clearly delineate the role that surgical excision plays in their overall therapy. Using their embryologic origin and histologic characteristics, Ackerman [6] categorized the malignant tumors of the retroperitoneal space as follows: tumors of mesodermal origin including liposarcoma, leiomyosarcoma, fibrosarcoma, rhabdomyosarcoma, lymphangiosarcoma, lymphoma, hemangiosarcoma and xanthogranuloma; tumors of nervous origin including schwannoma, neuroblastoma, malignant paraganglioma and malignant pheochromocytoma; and tumors arising from embryonic remnants, primarily malignant teratoma and chordoma. Most malignant retroperitoneal tumors are of mesodermal origin as documented in three important reports. In 1954, Pack and Tabah [5] studied 103 cases; 54 percent of them were sarcomas and 24 percent lymphomas. In 1967, Braasch and Mon [I] analyzed 88 cases; 43 percent of them were sarcomas and 32 percent lymphomas. In 1970, Gill et al [7] found that 70 percent of their 22 cases were sarcomas and 15 percent were lymphomas. In all three series, malignant retroperitoneal tumors originating from nervous tissue or embryonic remnants, or with undifferentiated histologic features, comprised less than 25 percent of the cases analyzed. Because lymphomas originating in the abdominal cavity frequently involve the retroperitoneal space and their course and management differ often from those of sarcomatous tumors, they were excluded. This study attempts to elicit factual information that would provide reliable guidelines for establishing the best plan for diagnosis and therapy, and an accurate

Primary retroperitoneal sarcomas: The role of surgical treatment

Attempting to understand the role surgical excision plays in the therapeutic efforts to provide cure or palliation in patients with retroperitoneal sarcomas, the literature on the subject was reviewed and 13 such cases were analyzed. A palpable abdominal mass was recorded in 10 patients. Intravenous pyelography suggested the diagnosis in nine patients. The diagnosis was difficult to establish before celiotomy, which was performed in 12 patients. In six the tumor was unresectable; it was partially resected in five and completely removed in one. This study suggests that the primary mode of spread of these tumors is direct invasion. The only objective benefit of operation was to provide a tissue-proven diagnosis. Radiation therapy, after the diagnosis was established, appeared to be the most effective therapeutic measure for retroperitoneal sarcomas. The value of additional chemotherapy could not be established.