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The surgical and functional outcome of limb-salvage surgery with vascular reconstruction for soft tissue sarcoma of the extremity.

BACKGROUND: This study compared the surgical, oncological, and functional outcomes of patients undergoing limb-salvage surgery for extremity soft tissue sarcoma with vascular resection and reconstruction with the outcomes of those undergoing limb-salvage without vascular reconstruction. METHODS: Nineteen patients were identified from a prospective soft-tissue sarcoma database who underwent vascular resection and reconstruction as part of their limb-salvage surgery and who were followed up for at least 1 year or until death. Each of these 19 patients was case-matched to 2 additional patients on the basis of tumor location, size, and depth; patient age; and timing of radiation. To compare functional outcome, a subset of patients was case-matched with additional criteria including wound-complication status, motor nerve sacrifice, similar preoperative function as determined by the Toronto Extremity Salvage Score, and no metastases at diagnosis or the 1-year follow-up. RESULTS: Patients in the vascular reconstruction group were more likely to require a muscle transfer (53% vs. 18%; P = .008), experience a wound complication (68% vs. 32%; P = .03), experience deep venous thrombosis (26% vs. 0; P = .003), experience significant limb edema (87% vs. 20%; P = .001), and ultimately require an amputation (16% vs. 3%; P = .07). Patients who underwent vascular reconstruction had only slightly lower Toronto Extremity Salvage Score scores 1 year after surgery (78.5 vs. 84.2; P = .35). There were no significant differences in local or systemic tumor relapse between the two groups. CONCLUSIONS: Vascular reconstruction is a feasible option in limb-salvage surgery for soft tissue sarcoma but is associated with an increased risk for postoperative complications, including amputation. Although function is not significantly worse after vascular reconstruction, the results are less predictable.

Adult↗

[The prognostic significance of inflammatory cells in malignant human soft tissue tumors. Malignancy grading].

These studies are based on an analysis of 160 spindle cell and/or pleomorphic malignant soft tissue tumors of adults and are aimed at providing an answer to the question of whether or not inflammatory cells in tumor tissue are of prognostic importance with regard to overall survival time. In a univariate analysis, granulocytes and lymphocytes did not show any statistical correlation with survival periods, whereas such correlation was revealed with significance to survival periods by plasma cells and mast cells. With multivariate analysis used and presence of necrotic areas included, only mast cell infiltration remained to be significantly correlated with survival time. Also presented in this paper is a proposed scheme of malignancy grading in which two properly established indicators are scored together with "mast cell infiltration", that is "mitosis count" and "necrosis amount". The three grades of malignancy resulting from this grading procedure are shown to be related with high significance to overall survival time.

Adult↗

The value of nuclear DNA and texture analysis by digital image processing in the diagnosis of lipomatous and leiomyomatous tumours.

The present study investigates whether the quantitative chromatin pattern description carried out by means of the digital cell image analysis of Feulgen-stained nuclei can contribute valuable diagnostic information on sarcomas. A series of 77 soft tissue tumours was consequently studied. This series included 9 benign lipomas versus 26 malignant liposarcomas and 26 benign leiomyomas versus 16 malignant leiomyosarcomas. Of the 26 liposarcomas, 14 were primary and 12 recurrent tumours. Of the 16 leiomyosarcomas, 13 were primary and three recurrent tumours. The results show that the combined use of principal-components analysis and the discriminant analyses of digital data obtained by means of the computer-assisted microscope analysis of Feulgen-stained nuclei made it possible to obtain a clear-cut distinction between the three histopathological groups relating to the lipoma/liposarcoma group of soft tumours. In contrast, while a clear-cut distinction could be made between the recurrent leiomyosarcomas and the primary leiomyosarcomas, such a distinction was not possible between the benign leiomyomas and the malignant primary leiomyosarcomas. This feature, along with previous ones obtained through DNA ploidy level determination, suggests to us that leiomyomas, or at least some of them, are in the process of malignant transformation. In other words, leiomyomas might be the pre-malignant counterpart of leiomyosarcomas, a feature that the present results do not suggest for lipomas versus liposarcomas.

Adult↗

Pleomorphic liposarcoma: a clinicopathologic analysis of 19 cases.

Pleomorphic liposarcoma is a variant of liposarcoma defined morphologically by the presence of pleomorphic lipoblasts. Because of its rarity, there are limited studies with long-term follow-up information. Nineteen pleomorphic liposarcomas were studied. Unequivocal pleomorphic lipoblasts were required for inclusion. In each case, the following features were noted: tumor site; tumor size; tumor depth; predominant histologic pattern (epithelioid or malignant fibrous histiocytoma [MFH]-like); extent of necrosis (absent, less than 15%, or at least 15%); mitotic counts; treatment and clinical follow-up. Patients were 11 females and 8 males, aged 33-87 years (mean, 64.5 y; median, 70 y). Tumors involved the extremities (13 patients: intramuscular in 10, subcutaneous in 2, depth unknown in 1), retroperitoneum (4 patients), mediastinum (1 patient), and paratesticular region (1 patient). Size ranged from 4.5--31 cm (mean, 11.9 cm; median, 12.0 cm). Predominant pattern was epithelioid in 7 and MFH-like in 12. Necrosis was present in 15 (79%) and was extensive (36 15%) in 14 patients. Mitotic counts ranged from 0.2--3.4/10 high-power fields (mean, 1.4; median, 1.4) by the average-count method and from 1--6/10 high power fields by the highest count method (mean, 2.9; median, 3.0). All patients were treated surgically; 10 patients received adjuvant chemotherapy and/or radiation therapy. On follow-up of 18 patients (range, 2--129 mo; mean, 35.4 mo; median, 23 mo) nine (50%) were dead of disease (range, 2--48 mo; mean, 20.1 mo; median, 12 mo), one died of other causes 2 months after diagnosis, two were alive with disease, five were disease free, and one was alive at 129 months (tumor status unknown). Five had recurrences (range, 3--28 mo; mean, 14.4 mo; median, 8 mo), and four of five (80%) with recurrences were dead of disease. Metastases developed in eight patients (range, 4--48 mo; mean, 19.5 mo; median, 11.5 mo), most commonly to the lungs. In conclusion, pleomorphic liposarcoma is a rare tumor of adulthood that occurs most commonly in the deep, soft tissues of the extremities. It behaves as a high-grade sarcoma that frequently metastasizes, most commonly to the lungs. Although this tumor has a wide range of histologic appearances, no clinical or pathologic feature is predictive of a more aggressive clinical course.

Adult↗

Gynaecological presentation of retroperitoneal tumours.

OBJECTIVE: To illustrate the problems associated with mistaken pre-operative diagnosis following gynaecological presentation of patients with retroperitoneal tumours. DESIGN: A case series of five referrals. RESULTS: Non-gynaecological tumours were not suspected in each case and hence there was a failure to undertake further pre-operative investigation and referral to a specialised soft tissue sarcoma service. This resulted in four of the patients having an unnecessary laparotomy with an inappropriate transperitoneal biopsy undertaken when the retroperitoneal tumour was discovered. The mistaken diagnosis of ovarian malignancy lead to increased morbidity, compromise of potential for a long disease free interval and/or possibly lessened the chance of cure in each case. CONCLUSIONS: Misinterpretation of clinical signs and an over-reliance on ultrasound diagnosis were the commonest causes of inappropriate management of these patients. Gynaecologists should consider more frequently the other, less common differential diagnoses of a pelvic mass. This is especially true in circumstances with a predominantly solid tumour, where there are clinical signs of vascular or rectal displacement, or where there is ultrasound evidence of ureteric obstruction. The more frequent utilisation of a computerised tomography scan with intravenous and oral contrast with referral before inappropriate transperitoneal biopsy are recommended as complete en bloc surgical excision at the first laparotomy is the treatment of choice in virtually all primary retroperitoneal tumours.

Adult↗

The value of MDM2 and CDK4 amplification levels using real-time polymerase chain reaction for the differential diagnosis of liposarcomas and their histologic mimickers.

Atypical lipomatous tumor/well-differentiated liposarcoma (ALT/WDL) and dedifferentiated liposarcoma (DDL) are reported to have murine double-minute type 2 (MDM2) and cyclin-dependent kinase 4 (CDK4) amplification as a characteristic genetic alteration. To evaluate the diagnostic utility of this gene abnormality, we analyzed 19 liposarcomas, 21 malignant fibrous histiocytomas, 3 leiomyosarcomas, 5 malignant peripheral nerve sheath tumors, 23 lipomas, and 28 nonneoplastic fat tissues using real-time polymerase chain reaction (PCR) and fluorescence in situ hybridization (FISH). In real-time PCR, all ALT/WDLs and DDLs had both MDM2 and CDK4 amplifications. The amplification levels in ALT/WDLs and DDLs were significantly higher than those in the other sarcomas, lipomas, and nonneoplastic fat tissues (P < .05); however, those in the other sarcomas and lipomas were not significantly higher than those in nonneoplastic tissues. In FISH, all ALT/WDLs and DDLs had both MDM2 and CDK4 amplifications, and all of the myxoid/round cell liposarcomas, leiomyosarcomas, malignant peripheral nerve sheath tumors, and all but one of the malignant fibrous histiocytomas did not have the amplifications. In this study, MDM2 and CDK4 amplifications were confirmed in ALT/WDLs and DDLs, and the amplification levels were significantly higher than those in the other tumors. An analysis of MDM2 and CDK4 amplification using real-time PCR, as well as FISH, is useful for the differential diagnosis of liposarcomas and their histologic mimickers.

Adipose Tissue↗

Treatment of metastatic sarcoma to the liver with bland embolization.

BACKGROUND: The authors evaluated the impact of bland particle embolization on survival in patients with metastatic sarcoma to the liver. METHODS: Twenty-four patients with liver-dominant metastases from sarcoma were treated with particle embolization from 1996 to 2002. Primary tumors included 16 gastrointestinal stromal tumors (GISTs), 7 intestinal leiomyosarcomas, and 1 liposarcoma. Thirteen patients had known extrahepatic disease. Embolization was performed by using polyvinyl alcohol or trisacryl microspheres to effect stasis in the target vessel(s). Follow-up images to assess response were obtained 4 weeks after the procedure. Decrease in the size of the target lesion by >25% or development of >50% necrosis on follow-up imaging was considered a treatment response. RESULTS: Nineteen patients had metachronous liver metastases, and the median disease-free interval was 22 months (range 10-156 months) from resection of the primary tumor. Ten patients underwent prior liver resection for metastatic disease. Of 15 evaluable patients, 9 patients (60%) had a radiographic response. The median follow-up for all patients was 21 months. The median follow-up for surviving patients was 59 months. Overall survival from the time of initial embolization was 62% at 1 year, 41% at 2 years, and 29% at 3 years. Patients who had radiographic evidence of response survived significantly longer than patients who did not respond (63 months vs. 19 months; P < .007). Patients with GIST survived significantly longer than patients with visceral leiomyosarcoma (median, 36 months vs. 18 months; P < .03). CONCLUSIONS: Bland embolization was efficacious in some patients with metastatic sarcoma to the liver. Radiographic evidence of response was correlated with improved survival. This regional therapy may enter the treatment algorithm for patients who have unresectable disease or disease that has failed conventional therapies.

Adult↗

Soft tissue sarcomas involving the pelvis.

BACKGROUND AND OBJECTIVES: Soft tissue sarcomas (STS) of the true pelvis are rare tumors and there is little information in the literature related to pelvic STS. The purposes of this review were to understand the anatomic extension of these tumors to better plan surgical treatment and to determine the outcome of these patients. METHODS: Eighteen consecutive patients presenting between 1987 and 1995 with soft tissue sarcomas involving the true pelvis were retrospectively reviewed at minimum follow-up of 18 months. Cross-sectional imaging was reviewed for each patient to determine the anatomical location of the lesions. RESULTS: The tumors were confined to the true pelvis in 4 patients, extended to the retroperitoneum in three cases, and extended to the thigh in 11 patients. Adjuvant radiation was administered to all but 2 patients who had received radiation to the region in the past and all patients underwent surgical resection (local resection in 13 patients and hindquarter amputation in 5 patients). Surgical resection had a high rate of morbidity and complications including positive resection margins in nine individuals. Of the 18 patients, 11 died at a mean time of 15.5 (2-58) months from surgery, 4 were alive with evidence of disease at a mean time of 44.3 (18-68) months, and 3 were alive with no evidence of disease at a mean time of 57 (43-71) months. CONCLUSIONS: Soft tissue sarcoma of the pelvis is fortunately a rare disease with a high risk of local and systemic disease progression despite treatment with irradiation and surgical resection.

Adult↗