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PubMed2006

Perioperative outcome in sarcoma surgery.

INTRODUCTION: Soft tissue sarcomas (STS) are rare tumors accounting for less than 1% of all malignancies. Although disease-specific surgical management is increasingly important, only few data are available for STS. Here, we analyze a single institution setting focusing on perioperative surgical and clinical parameters. METHODS: Prospectively gathered data of all adult patients undergoing surgery for STS including gastrointestinal stroma tumors (GIST) between October 2001 and October 2004. Patients undergoing only biopsy or ambulatory surgery were excluded. Statistical analysis was performed using SAS(R) software and patient's data from a computerized sarcoma registry. RESULTS: 159 patients with a median age of 60.2 years underwent a total of 179 operations. Three major sites of occurrence were notified: the visceral cavity (VIS) (36.3%), the retroperitoneum (RET) (31.3%), and the extremities (EXT) (27.4%). GIST (53.9%) were the most common type in the VIS, liposarcoma (62.5%) in the RET, and either liposarcoma (30.6%) or malignant fibrous histiocytoma (28.6%) in the EXT. Recurrence was treated in more than half of the patients with RET STS, and in almost one third of the EXT lesions, while primary occurrence dominated in the VIS. Median operation times in the VIS, RET, and EXT were 210, 240, and 120 min, respectively. Blood loss was 300, 500, and 50 ml for VIS, RET, and EXT operations. Morbidity was 26.2, 30.4, and 34.7% in VIS, RET, and EXT operations, respectively (reoperation rates were 9.4, 5.4, and 14.3%). Mortality was 1.5, 8.9, and 2.0% for VIS, RET, and EXT. Length of hospital stay in the groups was comparable. CONCLUSION: STS surgery of a single surgical unit contains predominantly VIS, RET, and EXT tumors. The STS subtype varies with location, as does length of operation, blood loss, morbidity, mortality, and reoperation rate. These data are helpful for planning the perioperative management of adult patients with STS and can be used for prognostic analyses.

Aged↗
PubMed1994

Primary intrathoracic malignant mesenchymal tumors: pictorial essay.

Primary mesenchymal intrathoracic tumors are unusual. They can originate from the lung, the pleura, or the mediastinal structures. These sarcomas have protean, nonspecific imaging features. This pictorial essay illustrates a large number of these tumors, describes the plain film findings, and emphasizes the contribution of the new imaging modalities. The cross-sectional display and the high contrast resolution computed tomography (CT) and the ability to image vascular structures and chest wall with magnetic resonance (MR) allow excellent delineation of tumor extent and assessment of chest wall or vascular invasion. Early recognition of recurrence or metastases can be facilitated. In rare instances, the intrinsic characteristics of the tumor allow a specific diagnosis.

Adult↗
PubMed2002

Soft-tissue tumours of the perineum.

AIM: Primary perineal tumours in adults are rare entities that have been infrequently reported (one clinical series of nine patients with sarcoma and isolated case reports). We present our experience of perineal tumours better to characterize and define the natural history of this condition. METHODS: Perineal tumours occurring in adults (>18 years) from January 1990 were identified from the Royal Marsden Hospital's Sarcoma Unit prospective database. RESULTS: Nineteen perineal soft-tissue tumours (12 malignant and seven benign) were evaluated and treated at the Royal Marsden Hospital during this period. Liposarcoma (n=3) and aggressive angiomyxoma (n=4) were the most frequent histological subtypes. Three malignant and four benign tumours were larger than 10 cm. Most tumours were located deep to the deep fascia. Local recurrence in those with sarcoma occurred in one of five patients with negative microscopic margins and in one of two patients who had positive microscopic margins. One patient each with aggressive angiomyxoma and fibromatosis recurred locally. CONCLUSION: Aggressive treatment in the form of wide local excision is associated with fewer local recurrences in adults with primary perineal soft-tissue tumours. The aim of surgical treatment should be to obtain negative resection margins without causing disturbance to urinary or anorectal function.

Adolescent↗
PubMed2006

[Retroperitoneal sarcomas imaging].

Retroperitoneal sarcomas are rare neoplasms. The aim of this article is to expose imaging protocols and useful radiological signs, to show the core biopsy technique for diagnosis, to precise imaging key points before treatment and for survey.

Biopsy, Needle↗
PubMed1984

Subcutaneous pseudosarcomatous polyvinylpyrrolidone granuloma.

A 77-year-old man developed an induration of the buttock following repeated injections of an antihypertensive remedy containing polyvinylpyrrolidone (PVP). The excised tumor had a cellular, pseudosarcomatous appearance with abundant extracellular material, and resembled a myxoid liposarcoma. All of the staining reactions and infra-red spectrophotometry of the deposition indicated the presence of PVP. The awareness of such a cellular PVP granuloma is important for surgical pathologists so as to avoid the diagnosis of sarcoma.

Aged↗
PubMed2000

Intra-abdominal metastases in musculoskeletal sarcomas.

This study examined the incidence, histological type, clinical symptoms, and prognosis in patients with intra-abdominal metastases of musculoskeletal sarcomas. The medical records of 505 patients with musculoskeletal sarcomas were reviewed for examples of intra-abdominal metastases. The incidence of intra-abdominal metastases (excluding lung) was: 4% in the liver (20 patients), 1.2% in gastrointestine (6 patients), 0.8% in pancreas (4 patients), and 0.8% on the peritoneal surface (4 patients). Patients with a previous hisory of lung metastases and those with high-grade liposarcoma tended to show metastasis in the intra-abdominal organs. Most patients with liver metastasis had no symptoms. Patients with gastrointestinal metastasis had abdominal pain, anemia, and melena. Patients with pancreatic metastasis had diabetes and jaundice. Six patients underwent surgical treatment, and two of them survived for more than 2 years. Metastases within the abdomen must be considered as a possible site for dissemination of musculoskeletal sarcomas, especially in patients with advanced disease and those with liposarcoma.

Adolescent↗
PubMed1982

Computed tomography in the management of soft tissue tumors.

Forty-six patients with primary soft tissue sarcomas underwent computed tomographic evaluation. Among 21 patients with retroperitoneal sarcomas, computed tomography was misleading in three patients and accurate and helpful in 18 patients. It was also useful in detecting recurrence by follow-up scanning and determining response to chemotherapy. Among 16 patients with tumors of the extremity, computed tomography demonstrated clinically helpful findings in only two patients and was misleading in two others. Computed tomographic scans were helpful and accurate in evaluating six sarcomas involving the trunk and abdominal wall. Computed tomographic scans were more accurate than ultrasonography or xeroradiography when patients had both imaging modalities. Computed tomographic scans should not supplant clinical examination for tumors of the extremity but are valuable in the preoperative evaluation of sarcomas arising at other sites. They are also valuable in the postoperative follow-up study of patients and may detect a recurrence prior to clinical examination.

Adult↗
PubMed2004

Management of soft tissue sarcomas (STS) in first isolated local recurrence: a retrospective study of 83 cases.

PURPOSE: To analyze the management and clinical outcome of patients treated for a first isolated local recurrence of soft tissue sarcomas (trunk or extremities) and to identify prognosis factors. METHODS AND MATERIAL: Between 1980 and 1999, 83 adult patients were included in the study. Mean age was 61 years. Mean tumor size was 6 cm. Most sarcomas were located in extremities (n=74), were deep (n=60), and proximal (n=53); 30 involved nerves or vessels. Histologic subtypes were mainly grade 2 (42%) or 3 (36%) histiocytofibrosarcomas (49%) and liposarcomas (20%). Surgical treatment of recurrences consisted in wide excision (29 cases), marginal resection (43 cases), 5 patients requiring amputation. Final results were R0 (n=33), R1 (n=47) or R2 (n=3) resection. Besides surgery, 6 patients received neo-adjuvant and 7 others adjuvant chemotherapy. Twenty three patients received post-operative external beam radiotherapy (EBRT) (mean dose 55 Gy) and 26 interstitial 192Ir low dose rate brachytherapy (BCT) (mean dose 45 Gy for BCT alone, 22 Gy when associated with EBRT), 19 patients being re-irradiated. RESULTS: Mean follow up was 13 years. Thirty-seven (45%) patients relapsed, 62% of whom presenting an isolated local recurrence. Nineteen patients developed distant metastases. Multivariate analysis showed only tumor depth (P=0.05) and re-resection for primary R1 resection (P=0.018) being independent prognosis factors for tumor control, radiotherapy (EBRT and/or BCT) being significant in univariate analysis (P=0.05). Overall survival rate was 73%, 54%, and 47% at, respectively, 3.5 and 10 years, and was 65%, 35% and 32% after a further local recurrence. Multivariate analysis showed trunk (P=0.0001) or inferior extremity locations (P=0.023), symptomatic (P=0.001), high grade (P=0.01), deep (P=0.01) tumors, and the occurrence of a further local failure (P=0.004) as unfavorable characteristics for overall survival. CONCLUSIONS: A first isolated local recurrence of STS increases mainly the risk of a subsequent local relapse. Quality of local treatment is decisive. When a conservative treatment is feasible, it should combine surgical resection and radiotherapy, BCT being the best suited in previously irradiated patients. Efforts have to be pursued to increase quality of the treatment of primary tumors, at best performed in centers that have expertise in this field.

Adult↗
PubMed2005

Expression of epidermal growth factor receptor, ERBB2 and KIT in adult soft tissue sarcomas: a clinicopathologic study of 281 cases.

BACKGROUND: Little is known about the expression of receptor tyrosine kinases in adult soft tissue sarcomas (STS). In the current study, the authors analyzed the expression of epidermal growth factor receptor (EGFR), ERBB2, and KIT in 281 patients with STS who were treated in a single institution. Verification of the presence of an association with prognosis was performed. METHODS: The current study included 281 adult patients with STS of the extremity and trunk who were diagnosed and treated in the National Cancer Center, Tokyo. Expression was assessed using immunohistochemical stains for EGFR, ERBB2, and KIT on formalin-fixed, paraffin-embedded tissue sections by standard avidin-biotin peroxidase complex technique and EGFR detection system. RESULTS: Positive staining of EGFR was observed in 168 of 281 (60%) patients. Positive staining was common in pleomorphic malignant fibrous histiocytomas (89%), myxofibrosarcomas (89%), synovial sarcomas (76%), malignant peripheral nerve sheath tumors (89%), and leiomyosarcomas (73%). It was less common in well differentiated liposarcomas (38%), fibrosarcomas (36%), and myxoid liposarcomas (6%). In contrast, positive staining of ERBB2 and KIT was very limited. Increased levels of EGFR were significantly associated with a decreased probability of overall survival (P = 0.01), although by univariate analysis; probability of overall survival at 5 years was 64% in patients with increased levels of EGFR and 79% in patients without such overexpression. The overexpression of EGFR was significantly associated with histologic grade (P < 0.001). Moreover, stratified log-rank test revealed that there is an interrelation between EGFR overexpression and histologic grade. CONCLUSIONS: EGFR overexpression was found to be a negative prognostic factor of adult STS, which is strongly associated with histologic grade. STS patients with EGFR overexpression may benefit from treatment with currently available biospecific inhibitors for EGFR.

Adult↗
PubMed2002

"In situ preparation": new surgical procedure indicated for soft-tissue sarcoma of a lower limb in close proximity to major neurovascular structures.

BACKGROUND: When soft-tissue sarcomas occur near neurovascular structures, preoperative images cannot always reveal the accurate relationship between the tumor and these structures. Therefore, in some patients, neurovascular structures are sacrificed unnecessarily. In other patients, neurovascular structures are preserved with an inappropriate margin, followed by local recurrence. The objective of this study was to evaluate a new surgical method, "in situ preparation" (ISP), which enables the preparation of neurovascular bundles and the intraoperative evaluation of the surgical margin without contamination by tumor cells. With this method, additional procedures, including pasteurization, alcohol soaking, and distilled water soaking of the preserved neurovascular bundle can also be performed to preserve the continuity of vessels. METHODS: Between April 1992 and December 1998, 18 patients with soft-tissue sarcoma were operated on using ISP. The tumor and neurovascular structure were lifted en bloc from the surgical bed and separated from the field by the use of a vinyl sheet. The consistency of the neurovascular structures was preserved. The tissue block could be freely turned around and the neurovascular structure was separated from the block through the nearest approach. The margin between the tumor and neurovascular structure was evaluated, and an additional procedure, such as pasteurization, alcohol soaking or distilled water soaking, was performed, according to the safety of the surgical margin. RESULTS: Only one patient showed recurrence after ISP. Complications after ISP were arterial occlusion in two patients and nerve palsy in three patients. The main cause of these complications was the long period of pasteurization; modified additional procedures could prevent such complications. CONCLUSIONS: ISP is a useful method with which to ensure a safe surgical margin and good functional results.

Adult↗
PubMed1995

Deletion in human chromosome region 12q13-15 by integration of human papillomavirus DNA in a cervical carcinoma cell line.

In human cervical carcinomas papillomavirus DNA is frequently integrated in the cell genome. We have cloned the integration site of human papillomavirus-18 DNA in human chromosome region 12q13-15 present in the SW756 cervical carcinoma cell line. Viral DNA is broken from nucleotides 2643 to 3418 in the E1 and E2 open reading frames, resulting in a deletion of 775 bases of viral DNA. Cloning and sequence analysis of the rearranged and germline alleles shows that there is no homology between the target cellular and viral DNA, suggesting it is a nonhomologous recombination. The target cellular region is called papillomavirus associated locus 2 (PAL2). The 5'- and 3'-flanking probes derived from the hybrid viral-cellular clone detect completely different germline restriction fragments in DNA from cells with normal chromosome 12. There is no overlap between the restriction maps of the target germline clones obtained with 5'- and 3'-flanking probes. Probes from these germline clones beyond the breakpoint position do not detect any DNA rearrangement in SW756 cells DNA. These data prove that there is a deletion of cellular DNA as consequence of the integration, with an estimated minimum size of 14 kilobases. Both cellular flanking probes are outside the amplicon of this chromosome region identified in the OSA and RMS13 sarcoma cell lines, comprising SAS-CHOP-CDK4-MDM2 genes and where translocation breakpoints are located in liposarcomas. The integration at 12q13-15 might have been selected by its contribution to the tumor phenotype.

Alleles↗
PubMed1998

[Low grade malignant fibromyxoid sarcoma].

Two cases of low-grade fibromyxoid sarcoma are presented. In a 31-year-old male the tumour arouse in the scapular soft tissues and local recurrence occurred. In a female of 45, the neoplasm was located in the thigh. Histologically, the neoplasms with low cellularity of deceptively benign-appearing small fibroblastic spindle cells demonstrated alternating dense fibrous and loose myxoid areas, showing a mainly whorled and swirling growth pattern. In myxoid areas a prominent vascular component was present. Mitoses and cellular atypia were absent. Tumor cells showed staining with anti-vimentin and occasionally anti-actin antibodies. As a distinctive soft-tissue sarcoma, low-grade fibromyxoid sarcoma had to be distinguished from variety of benign and malignant soft tissue tumours such as neurofibroma, liposarcoma, myxoid MFH and others.

Adult↗
PubMed1989

[Combined treatment of soft tissue sarcoma].

The five-year results of surgical and combined (irradiation + operation) treatment of 451 patients with sarcoma of the soft tissues are analysed with due account for the principal prognostic factors. It is shown that the survival of patients may be prolonged if the operation is supplemented by irradiation. Comparison of three variants of combined treatment showed the advantages of combined treatment with subsequent postoperative irradiation. Radiotherapy does not increase the number of postoperative complications.

Adolescent↗
PubMed2007

Analysis of germline and tumor mutations of p53 gene in familial occurrence of soft tissue sarcomas.

BACKGROUND: Familial soft tissue sarcomas are extremely rare. There is little information available on the clinical features and molecular findings of the hereditary occurrence of mesenchymal tumor. PATIENTS AND METHODS: A woman and her younger brother had malignant fibrous histiocytoma (pleomorphic type) and liposarcoma (pleomorphic type) in the lower limbs, respectively. Analysis of p53 mutations in exons 5-9 of the tumor and in germ-line was done. RESULTS: A guanine to adenine substitution occurred in CGC, codon 175 of exon 5 in p53 gene, to CAC in the tumor sample of Case 1. Likewise, a thymine to cytosine substitution occurred in TTT, codon 270 of exon 8 in p53 gene, to TCT in tumor sample of Case 2. Germline mutations were not seen in the either patients. CONCLUSIONS: Different missense mutations of p53 were detected in each tumor, however no germline mutations of p53 were found. The alteration of codon 175 in Case 1 is relatively common mutation. On the contrary, the mutation in codon 270 in Case 2 was extremely rare in cancers. Further molecular investigation is needed to understand the mechanism in familial occurrence of sarcomas.

Female↗
PubMed1992

SAS amplification in soft tissue sarcomas.

Gene amplification is an important mechanism of increased gene expression in a number of human solid tumors. We have recently identified and cloned sequences from a novel DNA amplification unit in malignant fibrous histiocytoma. The amplified sequences are derived from chromosome 12q13-14 and encode a gene designated SAS (sarcoma amplified sequence). In the present study, a series of soft tissue sarcomas was studied to characterize further the phenomenon of SAS amplification. Seven of 22 (32%) malignant fibrous histiocytomas and three liposarcomas contained SAS amplification. Strikingly, all of the tumors with SAS amplification occurred in central sites (i.e., in the abdominal or inguinal regions) rather than in the extremities (i.e., in the arms of legs). These observations demonstrate that SAS amplification occurs with a significant frequency in mesenchymal tumors and is particularly associated with abdominal disease.

Adult↗
PubMed1975

Growth rate of pulmonary metastases in human sarcomas.

The growth rate of spherical pulmonary metastases was studied in 15 patients with osseous and soft tissue sarcoma. The median volume doubling time (Dt) was 25 days. The time from diagnosis of the primary tumor to occurrence of pulmonary metastases correlated directly with the length of Dt. No correlation between Dt and tumor histology was observed. The effect of therapy on the tumor growth curve was studied in 1 patient. The therapeutic implications derived from the quantitative evaluation of tumor growth rate are discussed.

Adolescent↗
PubMed2005

Nerve tolerance to high-dose-rate brachytherapy in patients with soft tissue sarcoma: a retrospective study.

BACKGROUND: Brachytherapy, interstitial tumor bed irradiation, following conservative surgery has been shown to provide excellent local control and limb preservation in patients with soft tissue sarcomas (STS), whereas little is known about the tolerance of peripheral nerves to brachytherapy. In particular, nerve tolerance to high-dose-rate (HDR) brachytherapy has never been properly evaluated. In this study, we examined the efficacy and radiation neurotoxicity of HDR brachytherapy in patients with STS in contact with neurovascular structures. METHODS: Between 1995 and 2000, seven patients with STS involving the neurovascular bundle were treated in our institute with limb-preserving surgery, followed by fractionated HDR brachytherapy. Pathological examination demonstrated that 6 patients had high-grade lesions with five cases of negative margins and one case with positive margins, and one patient had a low-grade lesion with a negative margin. Afterloading catheters placed within the tumor bed directly upon the preserved neurovascular structures were postoperatively loaded with Iridium-192 with a total dose of 50 Gy in 6 patients. One patient received 30 Gy of HDR brachytherapy combined with 20 Gy of adjuvant external beam radiation. RESULTS: With a median follow-up of 4 years, the 5-year actuarial overall survival, disease-free survival, and local control rates were 83.3, 68.6, and 83.3%, respectively. None of the 7 patients developed HDR brachytherapy-induced peripheral neuropathy. Of 5 survivors, 3 evaluable patients had values of motor nerve conduction velocity of the preserved peripheral nerve in the normal range. CONCLUSION: In this study, there were no practical and electrophysiological findings of neurotoxicity of HDR brachytherapy. Despite the small number of patients, our encouraging results are valuable for limb-preserving surgery of unmanageable STS involving critical neurovascular structures.

Adolescent↗