Low-grade Soft Tissue Sarcoma Research Articles

Papers Presented at the 2005 Meeting of the Musculoskeletal Tumor Society

Papers Presented at the 2005 Meeting of the Musculoskeletal Tumor Society

133 INVITED Modulating radiotherapy approaches for sarcoma heterogeneity

Needle core biopsy (NCB) of soft tissue sarcomas (STSs) presents problems for French Federation Nationale des Centers de Lutte Contre le Cancer (FNCLCC) histological grading because small sample size hinders determination of necrosis and mitotic activity. We graded 53 STSs on NCB using a modified FNCLCC grading system that substitutes Ki-67 immunoexpression for mitotic count and uses a radiological assessment of necrosis, and compared the results with those obtained by conventional FNCLCC grading of the corresponding untreated, surgically resected specimen. Forty-eight of the 53 tumors were classified as malignant on NCB (concordance = 91%). The modified FNCLCC grade correctly separated high-grade (grades II and III) from low-grade sarcomas in 70% of cases and predicted the traditional FNCLCC grade given to the resected specimen in 49% of cases. Ki-67 scores of 2 or 3 were observed in 5 tumors classified as low-grade neoplasms on NCB but upgraded to a high-grade dedifferentiated liposarcoma on resection. Underestimated NCB grades were commonly encountered with lipomatous tumors due to sampling error, whereas Ki-67 or radiologic necrosis scores higher than the corresponding histological scores were responsible for the vast majority of overestimated NCB grades. Our FNCLCC grading scheme replacing conventional mitosis counting and histologic assessment of necrosis with surrogate markers is useful in separating high- and low-grade STSs on NCB for STS treatment planning. High Ki-67 rate should raise suspicion of a higher-grade component, particularly with fatty tumors.

Positronenemissionstomographie (PET) beim Weichteilsarkom

Positron emission tomography (PET) uses the significantly increased glucose metabolism to differentiate malignancies from healthy tissue. This method is well established in the oncologic diagnostics of solid epithelial tumours and also in breast cancer. In low-grade soft tissue sarcomas specificity and sensitivity are limited, however. Not only conventional systems, but also dual headed coincidence cameras requiring considerably less technical and economical expenditure can be used to produce PET-scans. We have studied the outcome of this technology in 13 patients with different types of soft-tissue sarcoma. It is our impression that both conventional and gamma camera based PET-systems result in similar results in soft-tissue sarcoma. Furthermore, in one of the cases presented, PET was able to detect metastases considerably earlier than conventional radiography.

A Systematic Overview of Radiation Therapy Effects in Soft Tissue Sarcomas

A systematic review of radiation therapy trials in several tumour types was performed by The Swedish Council of Technology Assessment in Health Care (SBU). The procedures for evaluation of the scientific literature are described separately (Acta Oncol 2003; 42: 357-365). This synthesis of the literature on radiation therapy for soft tissue sarcomas (STS) is based on data from five randomized trials. Moreover, data from 6 prospective studies, 25 retrospective studies and 3 other articles were used. In total, 39 scientific articles are included, involving 4 579 patients. The results were compared with those of a similar overview from 1996 which included 3 344 patients. The conclusions reached can be summarized as follows: <list id="l1" type="5"> <item>The well-established prognostic factors for tumour-related death from STS--histological grade, tumour size and age--are well documented.</item><item>The importance of superficial versus deep site as well as the anatomic site is also reaffirmed to some extent.</item><item>There is strong evidence that adjuvant radiotherapy improves the local control rate in combination with conservative surgery in the treatment of STS of extremities and trunk in patients with negative, marginal or minimal microscopic positive surgical margins. A local control rate of 90% has been achieved.</item><item>Improvement is obtained with radiotherapy added in the case of intralesional surgery, but the local control rate is somewhat lower. More studies are needed on this issue.</item><item>For STS in other anatomic sites, retroperitoneum, head and neck, breast and uterus, there is only weak indication of a benefit for the local control rate, with the use of adjuvant radiotherapy.</item><item>There is still insufficient data to establish that preoperative radiotherapy is favourable compared to postoperative radiotherapy for local control in patients presenting primarily with large tumours. One small study has shown a possible survival benefit for preoperative radiotherapy.</item><item>There is fairly good evidence to suggest that the preoperative setting results in more wound complications.</item><item>There is no randomized study comparing external beam radiotherapy and brachytherapy. The data suggest that external beam radiotherapy and low dose rate brachytherapy result in comparable local control for high-grade tumours.</item><item>Some patients with low-grade soft tissue sarcomas benefit from external beam radiotherapy in terms of local control.</item><item>Brachytherapy with low dose rate for low-grade tumours seems to be of no benefit, but data are sparse.</item><item>The available data are inconclusive concerning the effect of intraoperative high dose rate radiotherapy for retroperitoneal STS. Further studies are needed.</item><item>Neutron radiotherapy might be beneficial for patients with low- and intermediate-grade tumours considered inoperable and for those operated with intralesional margins. More severe side effects for neutrons have been registered.</item><item>In two small studies investigating hyperfractionation schedules there was no indication of improvements compared to daily fractions of 2 Gy. Further studies should be encouraged.</item><item>One small study using preoperative limb perfusion with TNFα melphalan and ±interferon γ combined with postoperative radiotherapy in the case of marginal or positive surgical margin has shown excellent local control without enhanced morbidity.</item></list>

Allotype imbalance or microsatellite mutation in low-grade soft tissue sarcomas of the extremities in adults.

The ability to repair DNA double-strand breaks is essential to maintain chromosomal stability. Virtually all soft tissue sarcomas contain chromosomal instabilities, including clonal aberrations and cytogenetic aberrations. However, the relevance of DNA-dependent protein kinase (DNA-PK) in the pathogenesis of soft tissue sarcoma has not been clarified. The main aim of this work is to compare the prognostic impact of genotypic imbalance in low-grade soft tissue sarcomas of the extremities, and to correlate this with the translational level of DNA-PK. This study investigated 28 adult low-grade malignant spindle cell tumours of the extremities, predominantly fibrosarcomas, for loss of heterozygosity (LOH) and microsatellite mutation on flanking regions of each DNA-PK subunit, with identical immunophenotypes. Twelve different polymorphic markers flanking the specific loci of three subunits comprise the genetic map of DNA-PK, at 22q13, 2q35, and 8q11. Translational activity was also analysed by western blot and conventional immunohistochemistry. The overall sarcoma 5-year survival rate was 61.7%. LOH was identified in the specific coding region of DNA-PK in 39.29% for the DNA-PK catalytic subunit (cs), 17.86% for Ku70, and only 7.14% for Ku80. A positive LOH for DNA-PKcs was shown to be a significant factor for poor survival (log rank test p = 0.0160). Immunoreactivity and immunoblot results correlated with the loss of DNA-PKcs allotype in soft tissue sarcoma (Fisher's exact test p = 0.0037). Ku70 and DNA-PKcs were almost identical in terms of immunoreactivity. In conclusion, whereas microsatellite mutation seems an uncommon event during the evolution of low-grade fibrosarcoma of the extremities in adults, the loss of DNA-PKcs defines a biologically more aggressive subset.

Radiation therapy for low-grade soft tissue sarcoma.

One hundred eight patients with low-grade soft tissue sarcoma treated at the authors' institution from 1984 to 1999 were reviewed retrospectively. Low-grade sarcomas were defined as those designated as Grade I of III or Grade I or II of IV. Patients who presented with recurrent tumors or who had multiple procedures at outside institutions were excluded. The patient's gender, age at diagnosis, histologic diagnosis, site and depth of primary involvement, and specimen size at resection were noted. Involvement of periosteum, bone, and neurovascular structures also was recorded. The decision to use radiation therapy was made case-by-case. Indications for preoperative radiation therapy included tumor adjacent to the neurovascular bundle or bone where a marginal surgical margin was anticipated. Indications for postoperative radiation therapy included followup resection of an unsuspected malignancy. Patients were evaluated according to treatment rendered. Sixty-six patients were treated with surgery alone, whereas 42 patients had excision of the tumor in conjunction with radiation therapy. The overall local control rate was 97.2% (105 of 108). There were no statistically significant differences in local recurrence, wound complication, fibrosis, or survival between the two groups. Patients treated with radiation therapy had an increased incidence of postoperative lymphedema. Kaplan-Meier 10-year survival rates were approximately 90% for both groups.

Telomerase activity and hTERT mRNA expression can be heterogeneous and does not correlate with telomere length in soft tissue sarcomas.

In a previous study, we showed that telomerase activity (TA) and human telomerase reverse transcriptase (hTERT) mRNA expression were undetectable in benign mesenchymal lesions and low-grade soft tissue sarcomas (STSs), but detectable in about 50% of intermediate-/high-grade STSs. We wondered if this lack of TA or hTERT mRNA expression could be related to the tumor sample examined and if there was a relationship between the former 2 parameters and telomere length. Two separate tumor samples from 37 STSs were examined for telomerase activity, using the telomerase repeat amplification protocol (TRAP) assay and for hTERT mRNA expression, using RT-PCR. Telomere length was determined in each tumor sample, using the terminal restriction fragments (TRF) technique. Significant variations in telomere length, TA and hTERT mRNA expression between 2 samples of the same tumor were observed in 27%, 11% and 27% of STSs, respectively. Telomere length did not correlate with TA or hTERT mRNA expression. Despite great intratumoral heterogeneity in telomere length, short and long telomeres were more often seen in the low/intermediate-grade and high-grade STS categories, respectively. Few STSs that showed a TRF pattern suggestive of alternative lengthening of telomeres (ALT) may contain ALT subpopulations.

Testicular metastasis Of dermatofibrosarcoma protuberans: A case report - An Ultrasonography approach

Dermatofibrosarcoma protuberans (DFSP) is a highly recurrent low-grade soft tissue sarcoma, which is usually located on the trunk. In a review of the literature there were several reports of patients with dermatofibrosarcoma with histologically documented metastasis. In all of these, there was local recurrence and subsequent metastasis to local lymph nodes and heamatogenous spread to the lungs. We present a case of dermatofibrosarcoma protuberans first time reported in the literature with metastasis to the testis evaluated by Colour Doppler sonography.

Is radiotherapy important for low-grade soft tissue sarcoma of the extremity?

Radiotherapy and limb-preserving surgery has replaced amputation and compartmental resection for treatment of soft tissue sarcomas. However, the role of radiotherapy in low-grade tumors remains unclear. This study reviews the outcomes of 132 patients who received multimodality treatment for low-grade soft tissue sarcoma. Large primary tumors (> 5 cm) and the absence of radiotherapy correlated with local recurrence. Radiotherapy was most effective in patients operated on with marginal margins. Patients who were treated with wide surgical margins or had small tumors (< or =5 cm) showed no benefit with adjuvant radiotherapy. Size greater than 5 cm and local recurrence correlated with metastasis. Radiotherapy appears to be important in the management of low-grade soft tissue sarcoma. The principles of local treatment for low-grade soft tissue sarcoma should be the same as for high-grade tumors with a combination of surgery and adjuvant radiotherapy. In a subset of patients with small and widely excised tumors, consideration may be given to withholding radiotherapy. Local recurrence and metastasis from low-grade soft tissue sarcoma may occur as long as 1 decade after primary tumor resection. Long-term review of patients with low-grade tumors may be indicated.

Prospective evaluation of soft tissue masses and sarcomas using fluorodeoxyglucose positron emission tomography.

The differentiation of soft tissue sarcoma (STS) from benign masses is difficult owing to their clinical and radiological similarities. Accurate staging is hindered by the large number of sites at which metastases may be found. This study examined the value of whole-body [18F]2-fluoro-2-deoxy-D-glucose positron emission tomography (FDG PET) in patients presenting with soft tissue masses. Thirty patients with a soft tissue mass suspected to be malignant were evaluated with FDG PET. The images were evaluated qualitatively and quantitatively for uptake of FDG to determine whether benign lesions could be differentiated from malignant tumours, and for the presence of metastases. Thirty-one masses were removed from 30 patients; 12 were benign and 19 were malignant STSs. Using qualitative assessment of the FDG PET images, all the high-grade STSs (n = 12) were correctly identified, but low-grade STS (n = 7) could not be differentiated from a benign lesion. Using a quantification assessment, there was a 95 per cent sensitivity and a 75 per cent specificity in diagnosing STS. Three patients had metastases at presentation; two were correctly identified by FDG PET. FDG PET has a role in distinguishing high-grade STS from low-grade or benign STS and may have a role in staging malignant tumours.

Immunohistochemical detection of bcl-2 and p53 proteins and apoptosis in soft tissue sarcoma: their correlations with prognosis.

Information on prognostic factors is essential to establish appropriate therapeutic modalities for soft tissue sarcoma (STS). To evaluate the biological nature and prognostic factors of STS, p53 and bcl-2 expression was immunohistochemically studied on paraffin-embedded sections from 70 patients with STS in the extremities and trunk. In addition, the degree of apoptosis was examined by in situ end-labeling. Histologic diagnoses in these cases were malignant fibrous histiocytoma in 29 cases, liposarcoma in 11, synovial sarcoma in 11, leiomyosarcoma in 5, malignant neurogenic tumor in 5, and others in 9. Tumor cells in 31 of 70 cases (44%) showed positive nuclear staining for p53 protein. There was no correlation between p53 expression and tumor size, histologic grade, argyrophilic nucleolar organizer region (AgNOR) count, cellularity and extent of neerosis. Expression of p53 did not correlate with survival of patients. Tumor cells in 24 of 56 cases (43%) were positive for bcl-2 protein expression. The frequency of bcl-2 expression in the tumor cells showed a direct proportion to tumor size (> or = 10 vs. < 10 cm) but inverse proportion to AgNOR counts and cellularity. The 5-year survival rate in patients with bcl-2-positive tumors (87%) was more favorable than in those with bcl-2-negative tumors (53%; p < 0.05). The frequency of apoptosis in low-grade STS was significantly higher than that in the intermediate and high-grade STS (p < 0.001). Extent of necrosis, a well-known prognostic indicator in STS, was not correlated with the frequency of apoptosis. Multivariate analysis showed that cellularity, bcl-2 and AgNOR counts were independent prognostic factors in patients with STS. The current study revealed that STS with a higher expression of bcl-2 had lower proliferative activity and larger size than those without. Immunohistochemical detection of bcl-2 is useful for predicting prognosis in patients with STS.

Benign and low-grade tumors of the soft tissues: role for radiation therapy.

There is a misconception in some oncology circles that low-grade tumors are not likely to be controlled by clinically feasible radiation doses. This is so despite the abundant data that radiation alone achieves a high probability of permanent and complete responses of basal cell carcinomas of the skin, well-differentiated squamous cell carcinoma of the glottis, etc. Patients are not as concerned about the rate of response as about the probability that the response will ultimately be complete and permanent. The evidence available is that the low-grade soft tissue sarcomas respond as well as the high-grade ones. However, the difference between the low- and the high-grade sarcomas is that for low-grade sarcomas the time to achieve an observable response may be long. In this chapter we consider the response of fibromatosis and dermatofibrosarcoma protuberans (DFSP) to radiation alone or combined with surgery.KeywordsLocal ControlSoft Tissue SarcomaPositive MarginLocal FailureDesmoid TumorThese keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

Follicular Dendritic Cell Tumor

Follicular dendritic cell (FDC) tumor is an extremely rare malignant neoplasm with approximately 17 well-documented cases in the literature. We report 13 additional cases of this distinctive neoplasm. There were seven men and six women, with a mean age of 46.5 years (range, 27-62 years). There was involvement of cervical lymph nodes (six cases), mediastinum (three cases), axilla, tonsil, spleen, and peripancreatic soft tissues (one case each). The neoplasms were grey to tan, ranging in size from 1 to 13 cm. They were formed by oval to spindle cells with eosinophilic cytoplasm growing in sheets and fascicles, with a focal storiform pattern and whorls reminiscent of those seen in meningioma. The nuclei were oval or elongated with thin nuclear membranes, inconspicuous or small eosinophilic nucleoli, and clear or dispersed chromatin. Typically, the tumor cells were intimately admixed with small lymphocytes, with a prominent perivascular cuffing. Multinucleated tumor cells were present in seven cases. Necrosis, marked cellular atypia, high mitotic rate, and/or abnormal mitoses were present in seven cases. The tumor cells were positive for CD21 (10 of 11), CD35 (10 of 11), Ki-M4p (seven of eight) Ki-FDRC1p (six of seven), vimentin (five of nine), and S100 protein (five of nine). One case stained with actin. In situ hybridization, done in six cases, did not show Epstein-Barr virus RNA sequences. Ultrastructural examination of eight cases showed long, complex, occasionally interdigitating cytoplasmic processes joined by desmosomes. The behavior of these tumors is more akin to that of a low-grade soft tissue sarcoma than a malignant lymphoma and is characterized by local recurrences and occasional metastases. Two patients died of tumor, two were alive with recurrent or metastatic disease, eight were alive with no disease, and one was lost to follow-up.

Cellular schwannoma: A clinicopathologic, DNA flow cytometric, and proliferation marker study of 70 patients

CancerVolume 77, Issue 3 p. 591-593 CorrespondenceFree Access Cellular schwannoma: A clinicopathologic, DNA flow cytometric, and proliferation marker study of 70 patients Steven I. Hajdu M.D., Corresponding Author Steven I. Hajdu M.D. Department of Laboratories, North Shore University Hospital, Manhasset, New YorkDepartment of Laboratories, North Shore University Hospital, Manhasset, New York===Search for more papers by this author Steven I. Hajdu M.D., Corresponding Author Steven I. Hajdu M.D. Department of Laboratories, North Shore University Hospital, Manhasset, New YorkDepartment of Laboratories, North Shore University Hospital, Manhasset, New York===Search for more papers by this author First published: 1 February 1996 https://doi.org/10.1002/(SICI)1097-0142(19960201)77:3<591::AID-CNCR25>3.0.CO;2-ZCitations: 2AboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat No abstract is available for this article. References 1 Casadei GP, Scheithauer BW, Hirose T, Manfrini M, Van Houton C, Wood MR. Cellular schwannoma: a clinicopathologic, DNA flow cytometric, and proliferation marker study of 70 cases. Cancer 1995; 75: 1109– 19. 2 Hajdu SI. Schwannomas. Mod Pathol 1995; 8: 109– 15. 3 Hajdu SI. Pathology of soft tissue tumors. Philadelphia: Lea & Febiger, 1979. 4 Sordillo PP, Helson H, Hajdu SI, Magill GB, Kosloff C, Golby R, et al. Malignant schwannoma-clinical characteristics, survival and response to therapy. Cancer 1981; 47: 2503– 9. 5 Hajdu SI. Differential diagnosis of soft tissue and bone tumors. Basel: S. Karger AG, 1989. 6 Donohue JH, Collin C, Friedrich C, Godbold J, Hajdu SI, Brennan MF. Low-grade soft tissue sarcomas of the extremities. Analysis of risk factors for metastases. Cancer 1988; 62: 184– 93. 7 Hajdu SI. Peripheral nerve sheath tumors. Histogenesis, classification, and prognosis. Cancer 1993; 72: 2549– 52. 8 Harkin JC, Reed RJ. Tumors of the peripheral nervous system. Washington, D.C.: Armed Forces Institute of Pathology, 1969. 9 Reed RJ, Harkin JC. Tumors of the peripheral nervous system. Supplement. Washington, D.C.: Armed Forces Institute of Pathology, 1983. 10 Ducatman BS, Scheithauer BW, Piepgras DG, Reiman HM, Ilstrup DM. Malignant peripheral nerve sheath tumors: a clinicopathologic study of 120 cases. Cancer 1986; 57: 2006– 21. 11 Russell DS, Rubenstein LJ. Pathology of tumors of the nervous system. 5th edition. London: E. Arnold, 1989. 12 Russell WO, Cohen J, Enzinger F, Hajdu SI, Heise R, Martin RG, et al. A clinical and pathological staging system for soft tissue sarcomas. Cancer 1977; 40: 1562– 70. 13 Hajdu SI, Shiu MH, Brennan MF. The role of the pathologist in the management of soft tissue sarcomas. World J Surg 1988; 12: 326– 31. Citing Literature Volume77, Issue31 February 1996Pages 591-593 ReferencesRelatedInformation

Extraskeletal myxoid chondrosarcoma. Long-term experience with chemotherapy.

Extraskeletal myxoid chondrosarcoma (EMC) is a rare low-grade soft tissue sarcoma that has been reported to have an indolent nature history, and relatively good prognosis. The majority of primary tumors are located in the extremities and they tend to be bulky at presentation. Studies with long-term follow-up have revealed the development of distant metastases in virtually all patients, eventually resulting in death. We reviewed our experience with EMC over the last three decades. The patient population was identified through a search of the database maintained by the Departments of Patient Studies, Pathology, and Melanoma-Sarcoma Medical Oncology. Eleven patients with histologically confirmed diagnosis of EMC were identified. The median age was 59 (37-81 years), and there were nine males and two females. Nine patients had an extremity location and the remaining two had a chest wall and abdominal wall primary, respectively. The median size of the primary tumor was 10 cm (range: 4-17 cm) in maximum dimension. Ten of the eleven patients received chemotherapy, mainly with doxorubicin- and dacarbazine-based regimens. One patient is currently on beta-interferon. No objective responses were noted, to a median of 4 (2-6) cycles of chemotherapy. Three patients were treated with ifosfamide as a second-line chemotherapy without any benefit. Three patients have expired, two patients are alive with no evidence of disease, and six patients are alive with disease. The median follow-up is 5 years (range: 1.33-17 years) from diagnosis. Although small numbers preclude adequate assessment, there is no evidence of efficacy of standard soft-tissue sarcoma chemotherapy in patients with EMC.

A staging system for soft‐tissue sarcoma and its evaluation in relation to treatment

In order to define the significant factors for a staging system of soft-tissue sarcomas (STS), histologic and clinical findings in 190 adult patients with localized STS in the extremities and trunk were reviewed. The male-to-female ratio was 1.21. The histologic grading of tumors was defined according to the criteria recently proposed by us: tumors were low-grade in 65 cases, intermediate-grade in 57 cases and high-grade in 68 cases. The initial surgical procedure was as follows: intracapsular excision in 9 cases, marginal excision in 104 and wide local excision in 77, including 15 amputations. The mode of treatment was surgery alone (101 patients), surgery and chemotherapy (58), surgery and radiotherapy (22) and surgery and combined chemo- and radiotherapy (9). Univariate analysis revealed histologic grade, sex, tumor size and tumor depth to be significant prognostic factors. Multivariate analysis revealed histologic grade to be the only independent factor for prognosis. Significant clinical factors in each histologic grade were then evaluated. In the low-grade group, local recurrence significantly affected prognosis. Most of the patients with local recurrence had had marginal resection as the initial surgical procedure. No clinical factors affecting prognosis in the intermediate-grade group could be determined. In the high-grade group, patients with wide local excision and adjuvant chemotherapy had a better prognosis than those with marginal excision with or without adjuvant chemotherapy and wide local excision without chemotherapy (p = 0.09). In conclusion, histologic grade was the only significant factor for the staging of STS. On the basis of our staging system, different modalities of treatment for each grade of STS might be indicated; adequate surgery is essential for the prevention of local recurrence, which resulted in reduced mortality in patients with low-grade STS. For high-grade STS, the prevention of distant metastasis by combined extensive surgery and adjuvant chemotherapy may make long-term survival possible.

Long-term outcome in 87 patients with low-grade soft-tissue sarcoma.

To describe the long-term clinical outcome of patients with low-grade soft-tissue sarcoma and identify factors that may predict or determine their prognosis. Retrospective chart review with multivariate analysis. Large research hospital and referral center. All patients treated between 1975 and 1990 at the National Cancer Institute (Bethesda, Md) who had a confirmed diagnosis of low-grade soft-tissue sarcoma. Surgery and radiation therapy. Local recurrence and overall survival. For patients with nonretroperitoneal lesions, overall survival was excellent, with a history of recurrence, a positive surgical margin, and an absence of adjuvant radiation therapy significantly associated with increased risks of local recurrence. Patients with retroperitoneal lesions not only had an increased risk of local recurrence, but significantly poorer overall survival. Low-grade soft-tissue sarcomas are associated with excellent overall survival, especially those confined to nonretroperitoneal sites. The risk of local recurrence after resection with negative margins and/or adjuvant radiation therapy is very low and most recurrences can be controlled with further therapy.

Management of Extremity Soft-Tissue Sarcomas

Management of extremity soft-tissue sarcomas requires accurate clinical staging, pathologic diagnosis, surgical resection with a wide margin, and adjuvant radiation therapy for high-grade lesions. Brachytherapy offers a clean benefit in local control for large (> 5 cm) high-grade sarcomas. The role of radiation therapy for small high-grade and all low-grade soft-tissue sarcomas still is being evaluated. Survival is function of histologic grade, age of patient, size and location of the tumor, metastases, and adequacy of the surgical resection. Efficacy of chemotherapy has yet to be proven.

Dermatofibrosarcoma protuberans of the foot ?surgical treatment with defect covering by radial forearm flap Report of a case with review of the literature

Dermatofibrosarcoma protuberans (DFSP) is an unusual cutaneous neoplasm that originates in the dermis. This tumor belongs to the so-called low grade soft tissue sarcomas. The recurrence rate after simple surgical treatment is very high, and initial radical surgical treatment is indicated. In recurrences, the infiltrative growth often leads to invasion of vital structures and may ultimately lead to amputation of extremities. In the case reported, there was an initial misdiagnosis of dermatofibroma; the lesion recurred and was reported as DFSP. It was resected radically, and the defect was covered by a radial forearm flap. The radial forearm flap offers an effective one-stage method of soft tissue reconstruction which allows early mobilization and rehabilitation. After excision of a recurrence on the margin of the free flap 36 months after the first treatment, a deep infiltrating recurrence 12 months later made amputation necessary. The location of DFSP on the plantar aspect of the foot has not been reported previously. A brief review of the literature is presented.

Low-grade soft tissue sarcomas of the extremities. Analysis of risk factors for metastasis.

Of 130 patients with low-grade localized soft tissue sarcomas of the extremities treated at Memorial Hospital between 1968 and 1978, 18 (14%) developed metastases. The risk of metastasis could not be predicted by the clinical or pathologic size, tumor depth, proximal location, recurrence before presentation or subsequent to treatment at our institution, the type of surgical and adjuvant therapies, and the adequacy of surgical margins. The median age of patients with metastases was 10 years older than the other patients (57.5 years and 47.5 years, respectively). Of the more common sarcomas in this study, malignant peripheral nerve tumors and malignant fibrous histiocytomas had the highest prevalence of metastasis (27% (three of 11) and 17% (three of 18), respectively). Although patients with low-grade extremity sarcomas treated during the first 5 years of this review had an increased rate of metastasis, no other patient, tumor, or treatment variable examined significantly differed between the two time periods. Although grade alone is a good predictor of the risk of metastasis from soft tissue sarcomas of the extremity, 14% of patients in this series with low-grade histology developed metastases. Further review of larger numbers of these tumors should be undertaken in an effort to more definitively identify risk factors predictive of metastatic potential in low-grade sarcomas.