The management of locally recurrent soft-tissue sarcoma.

Thirty-eight patients with locally recurrent soft-tissue sarcomas of various histologic types and grades but with no evidence of distant metastases were studied. Twenty-five patients had more than one local recurrence. Most primary lesions had been initially treated by simple or "wide local" excision with removal of little or no surrounding normal tissue. The most common site for recurrence was the extremity. Sixteen patients received preoperative intra-arterial Adriamycin (30 mg/day X three days) and radiation therapy (3500 R over ten days) followed by wide resection of the recurrence and the previous operative field. Tumor-free margins were confirmed microscopically. There were no subsequent local recurrences in this group. In five patients, no preoperative therapy was used, and the resection was incomplete because of proximity to vital structures. In these patients, postoperative radiation therapy and chemotherapy could not prevent continued local tumor progression. Amputation for control of local recurrence was necessary in only three patients. the remaining patients underwent either resection alone or resection in combination with postoperative radiation and/or chemotherapy. Life-table analysis of these 38 patients shows an unexpectedly high predicted five-year survival of 76% (87% for patients whose local recurrence could be completely resected). This high salvage rate clearly justifies aggressive treatment of patients with local recurrences alone and warrants attempts to salvage functional extremities.     

Management of extremity soft-tissue sarcoma

Soft-tissue sarcomas remain relatively uncommon neoplasms, but they present major problems for both the patient and clinician. The identification of prognostic variables based on both clinical and histologic factors has allowed comparisons to be made between various therapeutic regimens. In extremity sarcoma, a more conservative operation utilizing adjuvant radiotherapy has decreased the need for radical surgery and maximized function without loss of survival benefit. The identification of high-risk groups, that is, patients with high-grade, large lesions, has highlighted the inadequacies of treatment in some areas. Similarly, this has led to more aggressive and innovative developmental programs. The progress, understanding, and therapeutic management of extremity lesions have not been matched in visceral and retroperitoneal tumors. Current efforts are directed at more appropriate use of radical surgery, maximizing conservative function-sparing operations with the use of adjuvant local therapy, and development of new regimens directed at prevention and treatment of metastatic disease.     

Management of small soft-tissue sarcoma of the extremity in adults.

To determine the significance of small (< or = 5 cm in diameter) soft-tissue sarcoma of the extremity, 174 adult patients were identified from information that had been entered prospectively into a database of 1742 patients between July 1982 and December 1990. Median follow-up was 48 months. The majority of tumors were high grade (n = 114; 66%). Local recurrence (n = 17) was seen in patients with both high-grade (11%) and low-grade tumors (7%). Distant metastases were seen in 7% of high-grade tumors and in no low-grade tumors. The overall 5-year survival rate was 94% for all patients. Grade, depth, location, type of operation, and sex did not affect 5-year survival or local recurrence-free survival. Neither postoperative adjuvant chemotherapy nor radiation therapy resulted in superior 5-year survival or local recurrence-free survival when compared with no postoperative treatment. The prognosis of these lesions is favorable, and no additional prognostic factors were identified. Inclusion of these patients into adjuvant therapy trials examining survival is inappropriate.     

复发性软组织肉瘤21例疗效分析

目的 总结复发性软组织肿瘤外科治疗疗效,探讨综合治疗策略.方法 回顾我院骨肿瘤科2005年1月至2011年3月收治的21例复发性软组织肉瘤患者的随访资料,对患者的疗效进行分析.结果 21例患者中,采用截肢术7例、扩大切除术12例、边缘切除2例.9例术后出现复发和转移,其中4例转移者死亡、l例术后转移并带瘤生存.结论 合适足够的外科手术切缘是防止局部复发的关键,彻底完整的切除肿瘤及综合放化疗是治疗复发性软组织肿瘤成功的关键.     

Ewing's sarcoma in the retroperitoneum

The patient was a female aged 18. Hematuria was discovered at an examination two years previously at which time the patient was complaining of right lumbar pain. The patient visited our hospital with a chief complaint of right abdominal tumor in June, 1988. A retroperitoneal tumor, 77 X 45 mm in size, was observed by CT and ultrasonic examination, and the right kidney was in hydronephrosis. An operation was performed in September, 1988, based on a diagnosis of retroperitoneal tumor and hydronephrosis. The tumor was seen to have involved the right ureter. Subsequently the tumor was surgically removed; right total nephroureterectomy and partial cystectomy were performed at that time. The removed tumor was 90 X 70 mm in size and 120 g in weight; it was histopathologically diagnosed as extraskeletal Ewing sarcoma. A systemic examination was carried out postoperatively, but no abnormalities were observed. As of September, 1990, the patient had had no recurrence and was being kept observation on an outpatient basis. There have been a total of 210 case reports regarding extraskeletal Ewing sarcoma published worldwide, including our case and 26 cases in Japan, so far as we know. However those of a lesion of retroperitoneal origin are extremely rare, numbering only 11 cases worldwide and 2 Japan. Herein we report our case of extraskeletal Ewing sarcoma with reference to the other 209 cases reported in the literature to date.     

Surgical resection of primary soft-tissue sarcoma of the extremities]

We evaluated 5 patients who had soft tissue sarcoma of the extremity treated by limb sparing surgery. All tumors were adjacent to bone, with no features of infiltration. According to the Surgical Staging System, all tumors were IIB. The mean follow-up was 13 months. To achieve wide resections of the tumors in such cases we recommend resections of the tumor with part of the bone, which is in direct contact with the tumor. Wide resection margins were achieved in all patients and none had local recurrence during the observation period.     

Alveolar soft-part sarcoma of the retroperitoneum

A 33-year-old woman was referred to Iida Municipal Hospital because of left back pain. Computed tomography showed a tumor (17 x 11 x 10 cm) in the left retroperitoneal space. T1- and T2-weighted magnetic resonance imaging showed an inhomogeneous mass with marginal blood vessels. The tumor was resected via lumbar oblique incision with the thoraco-abdominal approach. The tumor weighed 1800 g and consisted of nests of 5-100 large, loosely arranged, polygonal cells, surrounded by capillaries, resembling alveoli. The tumor cells were rich in cytoplasm, containing periodic acid-Schiff- and diastase-positive granules and typical crystals. The pathological diagnosis was alveolar soft-part sarcoma (ASPS). Alveolar soft-part sarcoma is a rare soft-tissue tumor that accounts for approximately 0.5-1% of soft-tissue sarcomas. Such tumors originating in the retroperitoneal space are extremely rare. Herein is reported a case of ASPS of the retroperitoneum with radiological and pathological findings.     

Hepatic metastases from soft-tissue sarcoma.

OBJECTIVE: Hepatic metastases from soft-tissue sarcoma are evaluated to define treatment and its limitations. METHODS: From 981 adult patients with diagnoses of soft-tissue sarcoma, 65 patients with hepatic metastases were studied. RESULTS: An intra-abdominal primary site was present in 61 of 65 patients, with 85% high-grade leiomyosarcoma. Hepatic resection was performed in 14 patients (22%). All patients have had recurrences after hepatic resection-11 of 14 in the liver--with a median survival of 30 months. Chemotherapy resulted in partial response in three patients and no complete responses. Survival is not influenced by grade, type, primary site, disease-free interval, chemotherapy, or hepatic resection. CONCLUSIONS: The uncommon response to conventional chemotherapy does not support its use in the treatment of hepatic metastases from soft-tissue sarcoma. Extent of disease limits the application and success of hepatic resection for soft-tissue sarcoma, and anything less than complete resection is not indicated.     

Diagnosis and management of retroperitoneal soft-tissue sarcoma.

Retroperitoneal soft-tissue sarcomas are locally invasive tumors that remain occult for long periods and grow quite large due to the abdominal cavity's remarkable ability to accommodate these slowly expanding masses with a paucity of attendant symptoms. An open biopsy is required to establish diagnosis definitively. Despite improved imaging techniques and preoperative and intraoperative patient management, resectability has not changed significantly in the past 20 years. Even with an aggressive operative approach, only one half the tumors can be resected completely, and of those, more than 90% recur locally and result in the death of the patient. The addition of adjuvant radiotherapy or chemotherapy has not altered this pattern of local failure, in contrast to promising results with extremity soft-tissue sarcoma. Because of the rarity of these tumors, there is an urgent need to establish a national retroperitoneal sarcoma registry and to form cooperative intergroup studies to evaluate, treat, and apply innovative multimodality combination therapies to these otherwise lethal tumors.     

Molecular alterations in bone and soft-tissue sarcoma.

Understanding the molecular alterations evident in human cancer offers the potential for more meaningful diagnosis and more accurate prediction of cancer risk as well as the hope of developing novel therapies. In this review, several of the molecular abnormalities identified in both bone and soft-tissue sarcoma are outlined. Some sarcomas exhibit consistent gene translocations, which produce a unique oncoprotein that can serve as a molecular fingerprint for the sarcoma subtype. Mutations in tumour suppressor genes result in cell proliferation that is not regulated by normal cell cycle control genes. Amplification (multiple copies) of genes that act as positive controls on cell division have a similar effect on increasing cell proliferation. Finally, the understanding of drug resistance and sarcoma response to signalling systems such as IGF ligand and receptors may help in designing new therapies based on molecular changes in the cancer.     

Retroperitoneal soft-tissue sarcomas: prognosis and treatment of primary and recurrent disease

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. Liposarcomas were the most common pathology (61%); 79 per cent of these were of low grade. 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, local recurrence was common; this occurred in 11 of 22 patients (50%). Local recurrence, however, did not preclude long-term survival. Surgical resection of recurrent disease was done in nine patients with a median survival of 91 months (range 24-150 months). 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.     

Selection bias in treatment of soft-tissue sarcoma.

We analysed 256 patients with primary soft-tissue sarcoma of the extremities diagnosed between 1970 and 1990 to see if tumour-related and host-related prognostic factors influenced both the selection of operation and the margin of clearance obtained at surgery. Amputation was more often performed in young patients, in those with distal tumours, and in those with deep-seated tumours. Inadequate surgical margins (those with a high risk of local recurrence) were more common in patients with deep-seated and large tumours than in patients with superficial and small tumours. These features of the tumour and the host, which have been shown to be prognostic for survival, also influenced the choice and performance of surgical procedures.     

Unexpected resection of soft-tissue sarcoma

Sixteen referred patients were reviewed after excision of an unexpected soft-tissue sarcoma of the extremities. Eight tumors were located in the muscle deep to the fascia, and 8 lesions exceeded the size of 5 cm. The lack of awareness by the primary physician towards the possibility of a malignant lesion was striking, although 11 of 16 tumors presented as a newly formed mass. No imaging studies were done in 11 patients. Fine needle biopsy was unsuccessfully performed in 3 patients. Eleven lesions (63%) were high-grade. Resection margins were intralesional in 12 (75%) and marginal in 4 (25%) patients. Surgical oncologic rules were disregarded in 7 cases, including opening of probably uninvolved joints. Postoperative magnetic resonance imaging (MRI) scans showed a poor negative predictive value for residual tumor. Repeated resection, including three amputations, revealed residual tumor in 10 patients (63%). Four patients received adjuvant local radiation, with additional chemotherapy in 2 of them. At an average follow-up of 4.5 years (range 15-149 months), 4 patients (25%) had developed distant metastases with a local recurrence in 3 (19%). There was one tumor-related death (6%). Physicians' alertness towards the possible malignancy of an enlarging mass cannot be overemphasized. Evaluation by adequate imaging techniques, biopsy, and definitive resection and reconstruction should be performed by an oncologically trained orthopaedic surgeon. Inadequate primary excision leads to a high local recurrence rate and more mutilating surgery and obscures the long-term prognosis.     

The role of multimodality therapy in soft-tissue sarcoma.

Soft-tissue sarcomas are uncommon malignancies. The development during a period of 8 years, in one institution, of a prospective data base incorporating more than 1600 patients with these tumors is described. The most common sites for occurrence are the extremities, but they can occur in any of the soft tissues of the body. Liposarcoma and leiomyosarcoma are the most common histopathologic conditions identified. Prognostic factors for both recurrence and survival include site, histopathology, size, grade, and adequacy of resection. A prospective randomized trial of the use of adjuvant radiation by the brachytherapy technique in extremity lesions has shown a decrease in local recurrence, but no impact on survival. Eligible patients not randomized to the trial show no difference in local recurrence or survival, regardless of whether they received adjuvant radiation.     

Extravisceral primary hydatid cyst of the retroperitoneum

BACKGROUND: Hydatid cyst (HC) continues to be endemic in the Mediterranean countries, such as Turkey. Living in a rural area is an important risk factor for the disease. HC is most commonly seen in the liver and lungs, but retroperitoneal hydatid cyst is very rare. The objective of this study was to evaluate the clinical and radiographic findings and surgical treatment of this unusual lesion. METHODS: Between 1979 and 2004, 14 cases with primary retroperitoneal hydatid cyst were treated surgically at our clinic. RESULTS: Symptoms included flank pain in eight (57.1%) and palpable mass in six patients (42.8%). The cyst was located in the right retroperitoneum in seven patients (50%), left retroperitoneum in five patients (35.7%), retrovesical region in one patient (7.1%) and paravesical region in one patient (7.1%). Surgical approaches were right paramedian extraperitoneal approach in four patients, left paramedian extraperitoneal approach in two patients and midline transperitoneal approach in eight patients. Total pericystectomy was chosen as the surgical procedure in all patients except in five (35.7%), who had partial cystectomy for cysts located near the vital structures. There were no complications and mortality postoperatively. CONCLUSION: A primary HC of the retroperitoneum is a distinct clinical entity that must be considered when caring for a patient with a retroperitoneal mass in endemic regions. It should be treated after the diagnosis is confirmed without any delay because of secondary spillages due to perforations and other possible complications.     

Incidence, investigations and staging of soft-tissue sarcoma

Soft-tissue sarcomas account for only 1% of all malignant lesions. The Canadian Sarcoma Group encourages the investigation and management of these tumours at tertiary institutions, where a multidisciplinary team can handle the complex problems. Staging of these tumours implies accurate anatomic determination of the extent of disease, the histogenesis and grade of the tumours and the presence of regional or distant metastases. Arteriography, computed tomography and magnetic resonance imaging can accurately define the tumour before biopsy. The biopsy should be muscle-splitting to minimize contamination of additional compartments and should allow inclusion of the biopsy site at definitive surgical resection. It should be done at the institution where definitive management will be performed. Regional lymph-node involvement can be detected using magnetic resonance imaging or gallium scanning, whereas for distant metastases, specifically of lung, computed tomography is the method of choice. To date no one staging system for soft-tissue sarcomas has been universally accepted. A hybrid, encompassing the advantages of each system, is being formulated.     

Prognostic factors predictive of survival for truncal and retroperitoneal soft-tissue sarcoma.

OBJECTIVE: The authors identified prognostic factors relevant to clinical outcomes (especially survival) in truncal and retroperitoneal soft-tissue sarcoma. SUMMARY BACKGROUND DATA: These results can be used to optimize surgical management and select patients most likely to benefit from novel therapeutic strategies in future trials. METHODS: A retrospective analysis was performed of a prospectively compiled database of 183 consecutive patients with truncal and retroperitoneal sarcomas seen at the Brigham and Women's Hospital and the Dana Farber Cancer Institute between 1970 to 1994. RESULTS: For truncal sarcoma, multivariate analysis showed that high-grade histology was associated with an eightfold increased risk of death compared with low-grade histology (p = 0.001). In addition to grade, gross positive margin of resection (p = 0.001), microscopic positive margin (p = 0.023), and tumors greater than 5 cm in size (p = 0.018) were important independent prognostic factors for survival. In this series, postoperative radiation therapy for truncal sarcoma was associated with a 2.4-fold decreased risk of death compared with truncal sarcoma patients receiving no adjuvant radiation therapy, having adjusted for the other prognostic factors (p = 0.030). In contrast, for retroperitoneal sarcoma, multivariate analysis showed that high-grade and intermediate-grade histology were associated with a five- to sixfold increased risk of death compared with low-grade histology (p = 0.009). In addition to grade, gross positive margin of resection (p = 0.001) and microscopic positive margin (p = 0.004) were important independent prognostic factors for survival in retroperitoneal sarcoma. Patients who received either preoperative or postoperative chemotherapy for retroperitoneal sarcoma had a 4.6-fold (p = 0.002) and 3-fold (p = 0.010) increased risk of death, respectively, compared with patients receiving no adjuvant chemotherapy, having adjusted for the other prognostic factors. CONCLUSIONS: The histologic grade and the margin of resection are prognostic for survival in both truncal and retroperitoneal soft-tissue sarcoma. Tumor size was an independent prognostic factor for truncal sarcoma, but not for retroperitoneal sarcoma. Postoperative adjuvant radiation was beneficial to overall survival for truncal sarcoma. In this series of patients receiving a heterogeneous mixture of chemotherapeutic regimens-either as preoperative "neoadjuvant" therapy or as postoperative "adjuvant" therapy, there were no beneficial effects on survival compared with nonrandomized patients not receiving chemotherapy.