巨大腹膜后肿瘤的术前评估和手术策略

腹膜后肿瘤（retroperitoneal tumor,RPT）发病较为隐匿,出现症状时肿瘤往往体积较大,多侵犯周围组织与脏器,尤其是腹腔重要血管,是RPT手术主要难点。累及大血管RPT的处理常被很多外科医生视为禁区,但手术仍是目前治疗该病唯一有效的方法,累及大血管RPT的处理决策是提高肿瘤手术切除率的关键。因此,术前对肿瘤累及血管程度的评估,术中对血管重建技术的应用对于肿瘤完整切除至关重要。术前应用影像学检查了解RPT与腹腔大血管之间的关系、浸润情况,手术中良好的术野为处理和控制血管出血创造了有利条件;累及大血管RPT在完整切除时,需要对一些重要的血管进行修复与重建,自体血管的使用技术已日益成熟,人工血管和补片的应用也为手术创造了条件。术前评估、手术策略、术后管理对肿瘤外科医生在累及大血管RPT的患者治疗过程中起着至关重要的作用。     

Radiation therapy for soft tissue sarcoma

Sarcomas are relatively uncommon tumors of mesenchymal origin. They may arise anywhere within the musculoskeletal system and are typically classified by clinical behavior into benign, borderline, or malignant. This article focuses primarily on the role of radiation for malignant soft tissue sarcoma and selected borderline tumors.     

Palliative surgery of soft tissue sarcoma

Despite an increasing trend towards early diagnosis of breast cancer,patients still present with locally advanced disease. Also, in some patients chemotherapy will fail, and local and regional recurrence will occur. This article outlines options for palliative care for such patients.     

Fast neutron therapy for soft tissue sarcoma

Seventy-nine patients with soft tissue sarcoma were treated with fast neutron therapy at the Hammersmith Hospital, MRC Cyclotron Unit. Sixty-six of these, treated between 1971 and 1983 were assessable. The histology was reviewed and graded in 82% of cases and tumors divided into groups according to maximum diameter. In sixteen patients, who were irradiated following complete macroscopic removal of tumor, there was 94% local control and 86% survived 5 years. Of the 50 patients who had gross tumors present 62% were greater than 10 cm in diameter, and 20 were recurrent after previous radiotherapy or surgery or both. Sixty-eight per cent of gross tumors completely regressed and local control was 52%. The main cause of death was metastatic spread, and median survival was 63 months for Grade 1 patients, 9 months for Grade 2, and 7 months for Grade 3. Thus, there was a significant advantage to patients with Grade 1 tumor but little difference between Grades 2 and 3. Twenty-seven patients experienced late complications of treatment, 67% of which involved the skin predominantly and were related to the low energy of neutrons used. Seventeen of the 27 had received previous radiotherapy. Neutron therapy given in this dose and fractionation produced a higher local control rate than photon therapy, but complications were more frequent. Since these mainly involved the skin a lower level of complications may be anticipated using higher energy neutrons which will have a more even distribution of dose and lower skin dosage. Forty-eight per cent of patients developed metastatic disease, indicating the need for effective systemic therapy, especially in Grades 2 and 3 tumors.     

Results of limb-salvage surgery with vascular reconstruction for soft tissue sarcoma in the lower extremity: comparison between only arterial and arterovenous reconstruction

BACKGROUND: Reports on vascular reconstruction in conjunction with limb salvage techniques for soft tissue sarcomas in the lower extremity have been published. The aim of this study was to investigate the necessity for venous reconstruction by comparing the results of arterial reconstruction alone and those of arteriovenous reconstruction. METHODS: Twenty-five (6.7%) of 373 patients underwent vascular resections for lower limb soft tissue sarcomas. Only arterial reconstruction was performed for 12 patients. Arteriovenous reconstruction was performed for 13 patients. The clinical results, complications, and postoperative function were compared between only arterial reconstruction group and arteriovenous reconstruction group. RESULTS: Limb salvage was achieved in 92% of all cases. No local recurrence has been observed. Nineteen patients (76%) are continuously free of disease. Histopathologic examination of the specimens showed that blood vessels had been infiltrated in 24%. The mean Musculoskeletal Tumor Society score was 70%. There were no significant differences in complication rate and postoperative function between two groups. CONCLUSIONS: Wide resection with vascular reconstruction for patients of soft tissue sarcomas involving major vessels in the lower limb provide long term local control and limb salvage. This study could not indicate the usefulness of additional venous reconstruction after vascular resection in the lower extremity.     

软组织肉瘤靶向治疗进展

化学治疗晚期软组织肉瘤疗效似已达到平台,靶向药物是进一步提高疗效的关键.尽管第1代靶向治疗药物开发的种类繁多,但仅有少数药物在前瞻性、随机的Ⅲ期临床试验中显示出生存优势.第2代靶向治疗药物多靶点抑制剂可同时阻断肿瘤多个信号传导,临床试验的初步结果表明,不良反应可以耐受,效果较好,治疗软组织肉瘤已显示出较好的应用前景.     

Radiation therapy alone for sarcoma of soft tissue

Fifty-one patients have been treated with radiation therapy alone for soft tissue sarcomas, with 72.5% eligible for more than 5-year follow-up. Thirty-six of 51 patients received a radiation dose of 6400 cGy (or its equivalent) or greater. Careful attention was paid to the radiation therapy technique, using multiple angled fields, tissue-compensating wedges, immobilization devices, etc., in order to preserve a good functional result. Eleven of 51 patients are alive with no evidence of disease. The overall 5-year survival and local control rates were 25.1% and 33% respectively. For patients treated to a dose of 6400 cGy or greater, the 5-year survival and local control were 28.4% and 43.5%, respectively. Local control was better for tumors less than 5 cm diameter (87.5%) than in tumors 5 to 10 cm diameter (53%) or greater than 10 cm (30%). Only four patients in this series had a grade I tumor, and no survival difference could be detected between Grade 2 and Grade 3 tumors. This series indicates that radiation therapy alone can control tumor in a portion of patients with soft tissue sarcomas.     

Surgical margins and reresection in the management of patients with soft tissue sarcoma using conservative surgery and radiation therapy

BACKGROUND: Patients with localized soft tissue sarcoma (STS) who present to specialist centers after undergoing apparent macroscopic total resection often have a significant incidence of residual tumor and may benefit from reresection of the tumor bed. The potential benefits of such reresection have not been documented adequately. METHODS: The clinicopathologic features and disease outcome for 666 consecutive patients with localized STS who presented after undergoing apparent macroscopic total tumor resection were analyzed to elucidate the relative merits of reresection. Actuarial univariate and multivariate methods were used to compare disease outcome of patients who presented with positive or uncertain microscopic resection margins according to whether they underwent reresection. All patients received adjuvant radiation therapy. RESULTS: Two hundred and ninety-five patients underwent reresection of their tumor bed, and residual tumor was found in 136 patients (46%), including macroscopic tumor in 73 patients (28%). Final resection margins among patients who underwent reresection were negative in 257 patients (87%), positive in 35 patients (12%), and uncertain in 3 patients (1%). Patients who did not undergo reresection had final margins that were negative in 117 patients (32%), positive in 47 patients (13%), and uncertain in 207 patients (56%). Local control rates at 5 years, 10 years, and 15 years for patients who underwent reresection were 85%, 85%, and 82%, respectively; for patients who did not undergo reresection, the respective local control rates were 78%, 73%, and 73% (P = 0.03). Reresection remained a significant determinant of local control when other prognostic factors were incorporated into a multivariate proportional hazards regression analysis. A similar beneficial effect of reresection was found for metastasis free survival and disease specific survival. CONCLUSIONS: Patients with localized STS who were referred to a specialist center after undergoing apparent macroscopic total resection of their tumor had a high incidence of residual tumor in their tumor bed and benefited from undergoing reresection, even if radiation was administered routinely.     

Results of surgery and radio-hyperthermo-chemotherapy for patients with soft-tissue sarcoma

Background. Between 1990 and 1999, we performed radio-hyperthermo-chemotherapy (RHC) in 44 patients with high-grade soft-tissue sarcomas of the limbs. Methods. Radiotherapy involved the delivery of radiation at a dose of 2 Gy once daily on 16 days, to give a total dose of 32 Gy. Hyperthermia was conducted once a week, with a total of five sessions. Chemotherapy was performed by implanting a reservoir and administering cisplatin (3 mg/kg) three times, and pinorubin (an adriamycin derivative; 1 mg/kg) twice by intra-arterial infusion, at weekly intervals. These drugs were administered alternately during hyperthermia sessions. Results. Tumor shrinkage was observed in 98% (43/44) of the patients. Of the 36 patients with M0 tumors, 30 were disease-free at final follow-up, 2 had no evidence of disease, 1 was alive with disease, and 3 had died of the disease. Amputation was required only in the first patient, and the affected limb was preserved in the other 43 patients. The surgical margin was wide in 9 patients and marginal in 29 patients, and intralesional excision was performed in 5 patients. There was recurrence in only 1 of the 44 patients. Conclusion. RHC is currently the most potent and relatively safe treatment method for high-grade soft-tissue sarcomas that is available clinically. Received: April 12, 2001 / Accepted: August 17, 2001     

Imaging evaluation of patients with soft tissue sarcoma

Imaging provides the clinician with crucial information in the diagnosis, staging, treatment planning, treatment evaluation, and post-treatment assessment of patients with soft tissue sarcoma. MRI, including contrast-enhanced sequences, usually is preferred for evaluating the primary site in extremity sarcomas and lesions of the head and neck. CT generally is preferred for imaging of the chest, abdomen, and pelvis, either in the evaluation of the primary site in those regions or for identifying metastatic disease. The experienced radiologist often can suggest a specific diagnosis or narrow differential diagnosis from the imaging characteristics, particularly with MRI. It is imperative that imaging be performed in a manner specific for the evaluation of soft tissue masses, and before biopsy or surgery, to provide the most accurate preoperative assessment and treatment planning [56, 57].     

Pathological femur fracture secondary to radiation therapy for soft tissue sarcoma

Bone fracture is a well known possible late complication of radiation treatment. Little has been written about fractures of long bones after irradiation. We present a case of femur bone necrosis secondary to postoperative radiation for a soft tissue sarcoma of the thigh 20 years earlier. Fixation of the diaphyseal fracture and radiological evolution are described.     

Results of multimodal therapy in Ewing's sarcoma: a retrospective analysis of 20 patients

A retrospective analysis was performed of 20 patients with Ewing's sarcoma treated by combined modality therapy, consisting of surgery, radiotherapy, and chemotherapy. Fourteen patients (70%) achieved complete remission and 5 patients (25%) were in partial remission at the end of treatment. One patient (5%) failed to respond to combined modality therapy. The overall 5 year actuarial survival was 64% and the disease free survival, 55%. Persistent or recurrent disease occurred in 8 patients (40%); one of them was salvaged by surgery and chemotherapy. Site and extent of primary lesion were prognosticators of patients' outcome. One patient developed radiation-related-sequelae. The efficacy of aggressive management consisting of wide surgery, radiotherapy and intermittent high dose chemotherapy is discussed.     

The histologic response of soft tissue sarcoma to radiation therapy

Twenty-seven patients with soft tissue sarcoma had preoperative radiotherapy, limb-sparing marginal surgical resection and whole-mount tumor histologic analysis. Incisional biopsy specimens before radiotherapy were reviewed for tumor type, grade, and extent of necrosis. Preoperative radiotherapy was given in either of two regimens: 13 patients received a mean total dose of 5250 cGy in one daily 180 to 200 cGy fractions and 14 patients a mean total dose of 4770 cGy in two daily fractions of 180 to 200 cGy separated by 4 hours. Twenty-one specimens had at least 80% necrosis or severely altered cells, a 3+ to 4+ response. Grade and size of the tumor appeared to be indicators of response to treatment rather than histologic type. Three of five patients (60%) with Grade 1, eight of 11 patients (73%) with Grade 2 lesions, and ten of 11 patients (91%) with Grade 3 tumors had 80% or greater necrosis or severely altered cells. For tumors 10 cm or less in greatest diameter, the 3+ to 4+ histologic response was seen in 12 of 14 patients (86%) whereas for lesions greater than 10 cm, this response was observed in nine of 13 patients (69%). For patients with Grade 2 or 3 soft tissue sarcoma, 13 of 14 patients (93%) treated with two fractions per day and two of four patients (50%) receiving one fraction per day exhibited significant response. All six patients treated twice daily for lesions greater than 10 cm had 3+ to 4+ histologic response compared to three of seven (43%) patients treated once per day. Therefore, grade and size of soft tissue sarcoma are important predictors of response to radiotherapy and preoperative twice daily radiotherapy may more likely permit the conservative surgical excision of sarcomas of borderline resectability.     

Clinical outcomes for patients with soft tissue sarcoma of the hand

BACKGROUND:

In an earlier report from the current study center regarding surgical treatment for patients with soft tissue sarcoma (STS) of the hand, it was concluded that repeat resection or amputation improves outcomes. Since then, the authors have aggressively sought to achieve negative resection margins, using standard or modified amputations when needed, and performing repeat resections to negative surgical margins when they were not achieved at the time of initial surgery. The current review was conducted to determine whether this approach resulted in better outcomes.

METHODS:

A retrospective review of 53 patients with STS of the hand who were treated between 1996 and 2005 was performed. Recurrence‐free survival (RFS) and functional outcome of hand‐preserving procedures were assessed according to the Musculoskeletal Tumor Society (MSTS) system.

RESULTS:

The median RFS was not reached at the time of last follow‐up. Of 53 patients, 6 (11%) had positive microscopic resection margins. Three patients underwent repeat resection to negative surgical margins, whereas another 3 patients did not. All 3 patients with positive microscopic surgical margins that were not re‐excised developed local disease recurrence; 2 patients also developed distant metastases. Two of the 50 patients with negative resection margins developed distant metastases. All 5 patients who developed local and/or distant disease recurrence had deep tumors. The median MSTS score was 29 (interquartile range, 27‐30). Patients who underwent more extensive resections, such as double ray amputations, had lower MSTS scores.

CONCLUSIONS:

Suboptimal biopsies and positive resection margins are associated with local and distant disease recurrence in patients with STS. The results of the current study suggest that aggressive surgical treatment can result in better clinical outcomes, and underscore that care must be taken when planning biopsies of hand tumors. Cancer 2011. © 2010 American Cancer Society.     

Contemporary role of amputation for patients with extremity soft tissue sarcoma

Introductionlimb-sparing surgery is the mainstream treatment for primary extremity soft tissue sarcoma (ESTS) at referral centers, following advances in surgical reconstructions and multimodal management. However, amputation is still needed in selected patients and has not yet been described for a ESTS cohort in a contemporary scenario.Material and methodsconsecutive patients who underwent surgery for primary ESTS from 2006 to 2018 were extracted from a prospectively collected database at our reference center. Patients receiving amputation for either primary tumor or local recurrence (LR) after limb-sparing surgery were selected for analysis.ResultsAmong 1628 primary ESTS, 29 patients underwent primary amputation (1.8%), 22/1159 (1.9%) for upper limb and 7/469 (1.5%) for lower limb ESTS. Patients were mainly affected by grade III FNCLCC (89.6%) of notable dimension (median size 16 cm, IQR 10–24). 65.5% of patients received preoperative treatments (systemic or regional chemotherapy, radiotherapy or chemo-radiation). Secondary amputation for LR was performed after a median of 23 months in 16/1599 patients (1%). Median survival time was 16.2 and 29.6 months after primary or secondary amputation respectively. Factors prompting the need for a primary amputation were most often a combination of multifocal disease, bone invasion and pain or neurovascular bundle involvement and relevant comorbidities, mainly for grade III tumors in elderly patients.ConclusionContemporary rate of amputation for ESTS at a reference center is extremely low. Still, amputation is required in selected cases with advanced presentations, especially in elderly, frail patients.     

软组织肉瘤治疗进展

肉瘤是一组少见的具有不同临床和病理特征的间叶来源的肿瘤。整体上肉瘤可分为两大类,软组织来源的肉瘤和骨肉瘤。肉瘤大约占成人恶性肿瘤的1%,占儿童恶性肿瘤的15%。软组织肉瘤包含50多种不同的组织学亚型,最常见的亚型包括未分化多形性肉瘤、胃肠间质瘤、脂肪肉瘤、平滑肌肉瘤、滑膜肉瘤和恶性外周神经鞘瘤等。手术是软组织肉瘤最主要的治疗方法,但术后复发率高,预后差,化疗疗效仍不理想。随着人们对其生物学行为认识的加深,近些年涌现的一系列新型靶向药物取得了不错的疗效。总之,软组织肉瘤的治疗需根据疾病的组织学亚型、分子遗传学特点、分期及预后因素采取包括手术、放疗、化疗以及分子靶向治疗在内的个体化治疗模式。      

Tumor-induced hypercalcemia in a patient with extensive soft tissue sarcoma: Effects of bisphosphonate therapy and surgery

Tumor-induced hypercalcemia (TIH) is a frequent complication of advanced cancer, but it has been rarely reported in patients with sarcoma. We describe the case of a young female patient with TIH and with an extensive synoviosarcoma of the left lower limb destroying the bony structures. Hypercalcemia was severe (18.3 mg/dl) and accompanied by low serum P_i and suppressed parathyroid hormone (PTH) and 1,25(OH)₂ vit D₃ serum concentrations. Hypercalcemia was successfully treated with ibandronate, a new third-generation bisphosphonate, and radical surgery was performed when the patient was normocalcemic. Circulating levels of PTH-related protein (PTHrP) were elevated at 22.5 pmol/L (NI <9) PTHrP levels did not change after successful therapy of TIH, in contrast with PTH, which increased sharply. PTHrP levels were normalized after radical surgery. Moreover, low serum Pi with reduced threshold for phosphate excretion and increased tubular calcium reabsorption supported the notion that PTHrP was indeed the essential mediator of paraneoplastic hypercalcemia in this case despite the extensive bone destruction. © 1996 Wiley-Liss, Inc.     

Impact of surgery in patients with metastatic soft tissue sarcoma: A monocentric retrospective analysis

Background and Objectives

The role of local surgical procedures in patients with metastatic soft tissue sarcoma is still undefined. Few retrospective studies have reported survival benefits for patients with pulmonary metastases after complete surgical resection. Treatment decisions are therefore mainly based on personal experiences rather than on reproducible knowledge.

Method

A total of 237 patients with metastatic sarcoma, treated between 1982 and 2015 at the University Hospital Tuebingen, Germany, were eligible for inclusion. Out of the 237 screened patients, 102 patients underwent at least one metastasectomy. Overall survival was defined as the primary endpoint in this study. For association of non‐linear relationship to the endpoint, significant prognostic factors were included into a recursive partitioning model. A subgroup analysis for long‐term survivors was also performed.

Results

The median overall survival was 64 months. The 3‐, 5‐, 10‐, and 20‐years overall survival rates were 70.7%, 50.3%, 24.7%, and 14.8%, respectively. The number of resections and the progression‐free intervals were independent prognostic factors in three statistical models.

Conclusion

Repeated resections of metastases from different localizations are a strong predictor for prolonged survival. We suggest that the progression‐free interval after metastasectomy should be considered as a predictive factor for benefit from further surgery.     

The clinical and functional outcome for patients with radiation-induced soft tissue sarcoma

BACKGROUND:

Radiation‐induced soft tissue sarcomas (RI‐STS) are rare, and it is believed that they are associated with a poor prognosis.The authors of this report compared the clinical and functional outcomes of adults who had extremity RI‐STS with the outcomes of adults with sporadic STS.

METHODS:

Forty‐four patients who were diagnosed with RI‐STS from 1989 to 2009 were identified from 4 prospectively collected databases. Patient demographics, surgical and adjuvant treatment parameters, and oncologic and functional outcomes were evaluated.

RESULTS:

The median latent period from irradiation of the primary condition to RI‐STS diagnosis was 16 years. The median radiotherapy dose used for the index condition was 45 gray. The median age at RI‐STS diagnosis was 56 years. The most common primary diagnoses were breast cancer (36.4%) and lymphoma (34.1%). The most common RI‐STS histologies were malignant fibrous histiocytoma (36.4%) and angiosarcoma (18.2%). Forty‐two patients underwent surgery, 13 patients received adjuvant radiotherapy, and 8 patients received adjuvant chemotherapy. Systemic metastases occurred in 50% of treated patients (n = 21), and 26% (n = 11) developed local recurrence, the risk of which was lower among patients who received reirradiation (P = .043). The 5‐year disease‐free interval (DFI) and overall survival (OS) rates for patients with RI‐STS who presented without metastasis were 36% and 44%, respectively. Patients who had International Union Against Cancer TNM stage III RI‐STS had a significantly worse DFI compared with patients who had stage III sporadic STS (multivariate analysis, P = .051). Eighteen patients with RI‐STS underwent functional assessment after surgery, and their results were comparable to those of patients with sporadic STS.

CONCLUSIONS:

Despite aggressive surgical treatment, patients who have RI‐STS remain at greater risk of both local and systemic recurrence compared with patients who have sporadic STS, but they can anticipate similar functional outcomes. Reirradiation can be relatively safe and effective if used properly. Cancer 2011. © 2011 American Cancer Society.     

介入化疗与保肢手术治疗软组织肉瘤

目的研究术前动脉介入化疗与手术治疗肢体软组织肉瘤。方法21例肢体软组织肉瘤，其中13例(62％)术前影像学显示肿瘤邻近大血管或骨骼侵犯，采用股动脉及肱动脉插管，化疗2—3周后，施行肿瘤切除及保留肢体功能的手术。结果介入化疗后病理检查，肿瘤有不同程度的坏死，体积缩小，周围水肿浸润带减少，肿瘤与邻近组织术中易分离，术后随访6—62个月，患肢功能基本正常。结论对四肢软组织肉瘤侵犯大血管、骨骼者，术前动脉介入化疗可提高切除率，降低局部复发及保存患肢。