Indication and surgical approach for reconstruction with endoprosthesis in bone-associated soft tissue sarcomas:Appropriate case management is vital

It is important for surgeons performing sarcoma surgery to know that bone resection and tumor prosthesis applications in soft tissue sarcomas(STS)have unique features in terms of indication,surgical approach and follow-up,in terms of the management of these cases.Some STS are associated with bone and major neurovascular structures.Bone-associated STS are generally relatively large and relatively deep-seated.Additionally,the tendency for metastasis is high.In some cases,the decision about which structures to resect is difficult.These cases are often accompanied by poor oncological and surgical outcomes.Management of cases should be done by a multidisciplinary team in advanced centers specialized in this field.The surgical team must have sufficient knowledge and experience in the field of limb-sparing surgery.Preoperative evaluation and especially good planning of bone and soft tissue reconstruction are vital.

Undifferentiated high-grade pleomorphic sarcoma of the common bile duct:A case report and review of literature

BACKGROUND Undifferentiated pleomorphic sarcoma(UPS)is a rare malignant mesenchymal tumor with a poor prognosis.It mainly occurs in the extremities,trunk,head and neck,and retroperitoneum regions.Owing to the lack of specific clinical manifestations and imaging features,UPS diagnosis mainly depends on pathological and immunohistochemical examinations for exclusive diagnosis.Here we report an extremely rare case of high-grade UPS in the common bile duct(CBD).There are limited available data on such cases.CASE SUMMARY A 70-year-old woman was admitted to our department with yellow eyes and urine accompanied by upper abdominal distending pain for 2 wk.Her laboratory data suggested significantly elevated hepatorenal function levels.The imaging data revealed calculous cholecystitis,intrahepatic and extrahepatic bile duct dilation with extrahepatic bile duct calculi,and a space-occupying lesion at the distal CBD.After endoscopic biliary stenting and symptomatic support therapy,CBD exploration and biopsy were performed.The frozen section indicated malignant spindle cell tumor of the CBD mass,and further radical pancreaticoduodenectomy was performed.Finally,the neoplasm was diagnosed as a high-grade UPS combined with the light-microscopic morphology and immunohistochemical results.CONCLUSION This extremely rare case highlighted the need for increasing physicians'vigilance,reducing the odds of misdiagnosis,and providing appropriate treatment strategies.

Unusual Anterolateral Fascio Cutaneous Tight Flap for Extremity Soft Tissue Reconstruction

Soft tissue sarcoma is rare neoplasms that affect mainly the extremities. Surgery is the mean treatment and the resection results in extensive muscle and skin loss. The anterolateral tight flap is a good option for sarcomas arising in knees, superior third of legs and the medial face of thigh. The anatomy knowledge and the multidisciplinary approach is very important for a successful reconstruction. The authors report a successful case treated with the anterolateral tight flap and a literature review.

New trends in the surgical management of soft tissue sarcoma: The role of preoperative biopsy

Soft tissue sarcoma(STS)accounts for 1%of all malignant neoplasms in adults.Their diagnosis and management constitute a challenging target.They originate from the mesenchyme,and 50 subtypes with various cytogenetic profiles concerning soft tissue and bones have been recognized.These tumors mainly affect middle-aged adults but may be present at any age.Half of the patients have metastatic disease at the time of diagnosis and require systemic therapy.Tumors above 3-5 cm in size must be suspected of potential malignancy.A thorough history,clinical examination and imaging that must precede biopsy are necessary.Modern imaging techniques include ultrasound,computed tomography(CT),new magnetic resonance imaging(MRI),and positron emission tomography/CT.MRI findings may distinguish low-grade from high-grade STS based on a diagnostic score(tumor heterogeneity,intratumoral and peritumoral enhancement).A score≥2 indicates a high-grade lesion,and a score≤1 indicates a lowgrade lesion.For disease staging,abdominal imaging is recommended to detect early abdominal or retroperitoneal metastases.Liquid biopsy by detecting genomic material in serum is a novel diagnostic tool.A preoperative biopsy is necessary for diagnosis,prognosis and optimal planning of surgical intervention.Core needle biopsy is the most indicative and effective.Its correct performance influences surgical management.An unsuccessful biopsy means the dissemination of cancer cells into healthy anatomical structures that ultimately affect resectability and survival.Complete therapeutic excision(R0)with an acceptable resection margin of 1 cm is the method of choice.However,near significant structures,i.e.,vessels,nerves,an R2 resection(macroscopic margin involvement)preserving functionality but having a risk of local recurrence can be an acceptable choice,after informing the patient,to prevent an unavoidable amputation.For borderline resectability of the tumor,neoadjuvant chemo/radiotherapy has a place.Likewise,after surgical excision,adjuvant therapy is indicated,but chemotherapy in nonmetastatic disease is still debatable.The five-year survival rate reaches up to 55%.Reresection is considered after positive or uncertain resection margins.Current strategies are based on novel chemotherapeutic agents,improved radiotherapy applications to limit local side effects and targeted biological therapy or immunotherapy,including vaccines.Young age is a risk factor for distant metastasis within 6 mo following primary tumor resection.Neoadjuvant radiotherapy lasting 5-6 wk and surgical resection are indicated for highgrade STS(grade 2 or 3).Wide surgical excision alone may be acceptable for patients older than 70 years.However,locally advanced disease requires a multidisciplinary task of decision-making for amputation or limb salvage.

Adjuvant therapy for orbital non-rhabdomyosarcoma soft tissue sarcoma:comparison of long-term outcome between radiotherapy and chemotherapy

AIM:To illustrate clinicopathological features of orbital non-rhabdomyosarcoma soft tissue sarcoma(NRSTS),and to compare the treatment outcome between postoperative radiotherapy(RT) and chemotherapy in a retrospective analysis nearly 20y.METHODS:A retrospective cohort study of 56 patients with orbital NRSTS were reviewed,34 of whom received postoperative RT,and 22 received postoperative chemotherapy.The clinicopathological features,local recurrence,metastases,and survival data were recorded.Survival analysis was performed using the Kaplan-Meier method.RESULTS:During follow-up(111.8mo,ranged 8-233mo) for 56 patients,19 patients of them developed local recurrence,and 7 patients developed distant metastases.Fifteen patients died during follow-up period.Overall survival rates considering the whole study group was 78.57% at 5y,and 72.16% at 10y after the initial diagnosis.Compared with chemotherapy,RT was associated with lower risk of local recurrence [hazard ratio for RT vs chemotherapy,0.263,95% confidence interval(CI),0.095-0.728,P=0.0015];with lower risk of distant metastasis(hazard ratio for RT vs chemotherapy,0.073,95%CI,0.015-0.364,P=0.0014);and with lower risk of death from disease(hazard ratio for RT vs chemotherapy,0.066,95%CI,0.022-0.200,P<0.0001).The 5-year survival rate in RT group was 97.06% compared to 50% in chemotherapy group.CONCLUSION:In patients with orbital NRSTS,postoperative RT provides better control of local recurrence,distant metastasis,and death from disease than chemotherapy.RT is the more preferrable adjuvant therapy compared to chemotherapy possibly.

Soft Tissue Sarcomas: Descriptive Study of 232 Cases Collected, over a Period of 10 Years, at the Hospital of Oncology, Department of the Hassan II Hospital FEZ

Soft-tissue sarcomas are uncommon tumors that have traditionally been managed by wide excisional surgery and radiotherapy;the use of chemotherapy has been reserved for advanced disease. Advances in multidisciplinary care have improved the evaluation and care of patients with this disease. Limb-conserving surgical paradigms, superior radiotherapy delivery, and novel adjuvant agents for specific tumors are now available. The objective of this study is to report the epidemiological, clinical, histological, therapeutic and evolutionary characteristics of soft tissue sarcomas at the oncology hospital: Chu Hassan II in FEZ, and to define the factors influencing patient survival. This is a retrospective study of 232 cases of soft tissue sarcoma, collected between January 2010 and June 2020. The eligibility criteria were an age greater than 16 years, and histological evidence of a soft tissue sarcoma excluding gastrointestinal stromal tumors (GIST). Items collected were: epidemiological, clinical, histological, radiological, and therapeutic. These are 232 cases, 120 Men and 112 Women, the mean age was 48.89 years (Extreme = 18 - 76 years). The tumor was localized to the extremities at (58.72%). The predominant histological type was Leiomyosarcoma in 61 cases (26.29%). The tumor stage was localized in (17.67%) of cases, locally advanced in 34.05% and metastatic in 44.08% of patients, all localized cases were treated surgically including (84%) conservative surgery and (16%) radical surgery. Radiation therapy was performed in 32.75% of patients. Chemotherapy was performed in 74.14% of patients. Age and tumor stage are prognostic factors influencing the survival of soft tissue sarcomas.

System describing surgical field extension associated with flap reconstruction after resection of a superficial malignant soft tissue tumor

BACKGROUND Flap reconstruction after resection of a superficial malignant soft tissue tumor extends the surgical field and is an indicator for potential recurrence sites.AIM To describe a grading system for surgical field extension of soft tissue sarcomas.METHODS Grading system:CD-grading is a description system consisting of C and D values in the surgical field extension,which are related to the compartmental position of the flap beyond the nearby large joint and deeper extension for the pedicle,respectively.C1/D1 are positive values and C0/D0 are negative.With a known location,1/0 values can be"p"(proximal),"d"(distal),and"b"(in the tumor bed),and the description method is as follows:flap type,CxDx[x=0,1,p,d or b].RESULTS Four representative patients with subcutaneous sarcomas who underwent reconstruction using fasciocutaneous flaps are presented.The cases involved a distal upper arm(elbow)synovial sarcoma reconstructed using a pedicled latissimus dorsi(pedicled flap:CpDp);a distal upper arm(elbow)pleomorphic rhabdomyosarcoma reconstructed using a transpositional flap from the forearm(transpositional flap:CdD0);an undifferentiated pleomorphic sarcoma in the buttocks reconstructed using a transpositional flap(transpositional flap:C0D0);and a myxofibrosarcoma in the buttocks reconstructed using a propeller flap from the thigh(pedicled flap:CdDd).CONCLUSION The reconstruction method is chosen by the surgeon based on size,location,and other tumor characteristics;however,the final surgical field cannot be determined based on preoperative images alone.CD-grading is a description system consisting of C and D values in the surgical field extension that are related to the compartmental position of the flap beyond the nearby large joint and deeper extension for the pedicle,respectively.The CD-grading system gives a new perspective to the flap reconstruction classification.The CD-grading system also provides important information for follow-up imaging of a possible recurrence.

Incidence, distribution of histological subtypes and primary sites of soft tissue sarcoma in China

Objective: Soft tissue sarcomas(STSs) are rare malignancies deriving from mesenchyme.In this study, we reported the epidemiology of STS in China using population-based cancer registry data.Methods: In 2017, qualified data from 339 cancer registries were included in the national database.All STS cases were retrieved based on the morphological and topographical codes of International Classification of Diseases for Oncology, and were categorized into different histological subtypes and primary sites accordingly.Nationwide new STS cases were estimated using incidence rate of STS and the national population, and were reported for gastrointestinal stromal tumor(GIST) and STSs other than GIST separately by sex and region.Distribution of histological subtypes and primary sites of STS were calculated, as well as primary sites of GIST.Results: Approximately 39,900 new STS cases occurred nationwide in China in 2014, accounting for 1.05% of overall cancer incidence.The crude incidence rate was 2.91/100,000 and generally increased with age.An overall female predilection was found.GIST was the most common histological subtype, followed by nerve sheath tumor and malignant peripheral nerve sheath tumor,leiomyosarcoma, liposarcoma, and fibrosarcoma.About 67.5% of GIST occurred in stomach while 1.4% were recorded outside the gastrointestinal tract.Connective, subcutaneous and other soft tissues were the most common primary site, of which extremities were the major subsite.Conclusions: The burden of STS is not serious in China relatively.However, due to their histological and topographical complexity, STSs should not be unnoticed, and more basic and clinical studies should focus on STSs.

LAG-3 expression on tumor-infiltrating T cells in soft tissue sarcoma correlates with poor survival

Objective: To elucidate the role and prognostic significance of lymphocyte activation-gene-3(LAG-3) in soft tissue sarcoma(STS).Methods: The expression of LAG-3 in patient and matched normal blood samples was analyzed by flow cytometry. The localization and prognostic values of LAG-3^+ cells in 163 STS patients were analyzed by immunohistochemistry. In addition, the expression of tumor-infiltrating CD3^+ T, CD4^+ T, and CD8^+ T cells and their role in the prognosis of STS were evaluated by immunohistochemistry. The effect of LAG-3 blockade was evaluated in an immunocompetent MCA205 fibrosarcoma mouse model.Results: Peripheral CD8^+ and CD4^+ T cells from STS patients expressed higher levels of LAG-3 than those from healthy donors.LAG-3 expression in STS was significantly associated with a poor clinical outcome(P = 0.038) and was correlated with high pathological grade(P < 0.001), advanced tumor stage(P = 0.016). Additionally, LAG-3 expression was highly correlated with CD8^+ T-cell infiltration(r = 0.7034, P < 0.001). LAG-3 was expressed in murine tumor-infiltrating lymphocytes, and its blockade decreased tumor growth and enhanced secretion of interferon-gamma by CD8^+ and CD4^+ T cells.Conclusions: LAG-3 blockade may be a promising strategy to improve the effects of targeted therapy in STS.

Current management of pediatric soft tissue sarcomas

Pediatric soft tissue sarcomas are a group of malignant neoplasms arising within embryonic mesenchymal tissues during the process of differentiation into muscle, fascia and fat. The tumors have a biphasic peak for age of incidence. Rhabdomyosarcoma(RMS) is diagnosed more frequently in younger children, whereas adult-type non-RMS soft tissue sarcoma is predominately observed in adolescents. The latter group comprises a variety of rare tumors for which diagnosis can be difficult and typically requires special studies, including immunohistochemistry and molecular genetic analysis. Current management for the majority of pediatric sarcomas is based on the data from large multi-institutional trials, which has led to great improvements in outcomes over recent decades. Although surgery remains the mainstay of treatment, the curative aim cannot be achieved without adjuvant treatment. Pre-treatment staging and risk classification are of prime importance in selecting an effective treatment protocol. Tumor resectability, the response to induction chemotherapy, and radiation generally determine the risk-group, and these factors are functions of tumor site, size and biology. Surgery provides the best choice of local control of small resectable tumors in a favorable site. Radiation therapy is added when surgery leaves residual disease or there is evidence of regional spread. Chemotherapy aims to reduce the risk of relapse and improve overall survival. In addition, upfront chemotherapy reduces the aggressiveness of the required surgery and helps preserve organ function in a number of cases. Long-term survival in low-risk sarcomas is feasible, and the intensity of treatment can be reduced. In high-risk sarcoma, current research is allowing more effective disease control.

HIGH DOSE IFOSFAMIDE, DOXORUBICIN, DACARBAZINE AND G-CSF FOR PATIENTS WITH METASTATIC OR LOCALLYADVANCED SOFT TISSUE SARCOMA

Objective: A pilot study to test the feasibility and efficacy of high dose IFO and standard dose ADR and DTIC with G-CSF support in treatment of advanced soft tissue sarcoma (STS). Methods: 35 patients of no prior chemotherapy with metastatic or locally advanced unresectable STS were treated by this regimen, including 18 rhabdomyosarcomas, 7 malignant fibrous histiocytomas, 2 neurofibrosarcomas, 2 fibrosarcomas, 2 leiomyosarcomas, 2 synoviosarcomas, and 2 malignant hemangiopericytomas. IFO dose was 2 g/m2 on day 1–5 (with mesna uroprotection), ADR 50mg/m2 on day 1 and DTIC 250 mg/m2 on day 1–5. G-CSF (2 μg/kg/d) was administered on day 6 to 15 or until recovery of leukocytes account. The cycles were repeated every 3 weeks. Result: There were five complete responses (CR including pathologic CR) and eleven partial responses for overall 46% objective response rate. Most responses were observed within two cycles. The median survival was 15 months. Following CR, two patients remain disease free at 45 and 28 months, respectively. 6/120 (5%) cycles were complicated by grade IV neutropenia, 46/120 (38%) cycles had grade III neutropenia. No patients had treatment-related deaths. Nonhematologic toxicity consisted predominantly of anorexia and vomiting. No other severe toxicities were seen, especially no severe cardiotoxicity. Conclusion: This regimen is well tolerated and has substantial benefits for patients with advanced soft tissue sarcomas.

Multidisciplinary treatment of soft tissue sarcomas:An update

Standard treatment for soft tissue sarcoma,based on complete surgical resection with or without adjuvant radiotherapy and chemotherapy,has not substantially changed during the last several decades.Nevertheless,recent advances have contributed to considerable improvement in the management of these patients;for example,new magnetic resonance imaging sequences such as diffusionweighted imaging and magnetic resonance imaging radiomics can better assess tumor extension and even estimate its grade.Detection of circulating genetic material(liquid biopsy)and next-generation sequencing are powerful techniques for genetic analysis,which will increase our understanding of the underlying molecular mechanisms and may reveal potential therapeutic targets.The role of chemotherapy in non-metastatic disease is still controversial,and there is a need to identify patients who really benefit from this treatment.Novel chemotherapeutic regimens have entered clinical praxis and can change the outcome of patients with metastatic disease.Advances in radiotherapy have helped decrease local adverse effects and sustain good local control of the disease.The following report provides an updated view of the diagnosis,treatment,and future perspectives on the management of patients with soft tissue sarcomas.

Primary clear cell sarcoma of soft tissue in the posterior cervical spine invading the medulla oblongata:A case report

BACKGROUND Clear cell sarcoma(CCS)is a rare and highly malignant soft tissue tumor,usually occurring in the deep soft tissues of the distal tendons and aponeurosis of the extremities,especially the feet and knees.CCS originating in the head and neck is extremely rare.The clinical manifestations of CCS in the head and neck are not typical,and the imaging manifestations have certain characteristics,but the diagnosis still depends on pathological examination and genetic testing.CASE SUMMARY A 33-year-old male patient had paroxysmal headache for more than 4 years,accompanied by nausea and vomiting,which could be relieved after rest.Computed tomography angiography showed a left paraspinal soft tissue mass.Contrast-enhanced imaging showed obvious uneven enhancement with adjacent bone lytic destruction.Magnetic resonance imaging examination showed isosignal on T_(1)-weighted images,slightly high signal on T_(2)-weighted images(T_(2)WI),high signal on Tirm fat suppression sequence,significantly high signal on diffusion weighted imaging,and obvious and uneven enhancement.The lesion invaded the anterior medulla oblongata through the left atlantoaxial foramen and compressed the cervical spinal cord on T_(2)WI.Primary CCS of soft tissue was diagnosed by pathology and genetic examination.CONCLUSION CCS should be considered in the differential diagnosis of soft tissue tumors of the head and neck,and their diagnosis depends on pathological examination and genetic testing.

Abdominal metastases of primary extremity soft tissue sarcoma: A systematic review

BACKGROUND Despite the fact that about one third of patients with primary localized extremity soft tissue sarcoma(e STS)will develop metastatic disease,abdominal metastases(AM)and retroperitoneal metastases(RM)constitute rare events.There is no clear consensus on how to achieve follow-up on patients with primary localized e STS following curative resection,especially regarding the surveillance of potential AM/RM.AIM To systematically analyse incidence,diagnosis,treatment and outcome of AM/RM in e STS patients.METHODS In this systematic review,899 studies available in Pub Med and published between 2000 and 2018 were screened,identifying 17 original articles focused on AM or RM in e STS.Article selection was based on the PRISMA guidelines,using the search terms(abdominal metastasis AND soft tissue sarcoma)and(soft tissue sarcoma metastasis abdomen).All studies published between January 1,2000 and December 31,2018 were screened.Further articles were identified by crosssearching article references,with the final search date being February 18,2019.Due to limited data and the different reporting techniques used,the present review focused on descriptive analysis of the included studies.RESULTS Of the 17 studies included,six original articles reported on incidence±diagnosis,therapy and outcome in AM and RM,whilst three original and eight case reports focused on diagnostic pathway,therapeutic procedures or outcomes without allowing conclusions regarding incidence of AM and RM.According to the former six studies,incidence of AM ranged from 0.9%-5.6%in patients with miscellaneous histological subtypes,and up to 12.1%in patients with myxoid liposarcoma.The most common histological subtypes that developed AM or RM were(myxoid)liposarcoma and leiomyosarcoma,but also rare subtypes such asepithelioid sarcoma,myxofibrosarcoma,synovial sarcoma,and malignant peripheral nerve sheath tumour had been reported to develop AM/RM.Surgery for AM/RM was performed in five of eight case-reports(62.5%)and in 20.8%-100.0%of original articles.In particular,patients with hepatic metastases undergoing metastasectomy had a survival benefit compared to patients treated with chemotherapy or best supportive care(>3 years vs<6 mo).CONCLUSION Patients with e STS should undergo surveillance with abdominal ultrasonography/computed tomography,or even whole-body-magnetic resonance imaging to detect AM/RM at an early stage.

Clear cell sarcoma of soft tissue in pleural cavity: A case report

BACKGROUND Clear cell sarcoma(CCS)of soft tissue is a rare malignant soft tissue sarcoma usually reported to locate at distal end of extremities and rarely at trunk.Herein,we report a case of CCS in pleural cavity.CASE SUMMARY A 31-year-old male was admitted for an uncertain mass in left pleural cavity in routine physical examination without any symptoms.A VATS surgery was performed to remove the tumor.The pathological finding displayed a cystic mass with 6.5 cm at the longest diameter,dark red in section and cysts could be found locally.A strong expression of S-100,HMB45 and Vimentin was detected in immunohistochemical staining,which was inclined to the diagnosis of the CCS of soft tissue.The patient refused chemotherapy,radiotherapy and targeted therapy because of the personal financial situation.Follow-up computed tomography scans were done at the 90th postoperative day and the 180th postoperative day,and no obvious sign of recurrence was found till now.CONCLUSION CCS of soft tissue also can be found in pleural cavity although in an extremely rare incidence.Radical resection is useful to improve the prognosis.

INVESTIGATION OF RELATIONSHIPS BETWEEN KI－67SCORE，DNA INDEX，AND HISTOLOGIC GRADEIN SOFT TISSUE SARCOMAS

Proliferative activity of soft tissue sarcomas(STS)in 31 cases was estimated by histologic grading, mitotic count, DNA analysis by flow cytometry, and immunohistochemical procedures with monoclonal antibody Ki-67.Aneuploid was found in 12 of 16 cases(75.0%) with Grade 3, and in 4 of 15 cases(26.7%) with Grade 1, 2(P=0.0121).Tumors with more than 100 Ki-67 positive tumor cells per 10 high power fields(HPF) had a higher rate of aneuploid(81.3%) than those with less than 100 Ki-67 per 10 HPF (26.7%)(P=0.0038). There were significant correlations between Grade and DI (r=0.4901, P=0.0051), Grade and Ki-67(r=0.4636, P=0.0086), Ki-67 and DI(r=0-6368, P=0.0001). The results indicate that DI and reactivity of tumor cells to Ki-67 may reflect Proliferative activity and be helpful for clinicians to judge the biological behaviour of tumors more accurately and objectively. Supplementary to the grading of STS, DI and Ki-67 score could be useful as Prognostic parameters for clinical investigation of multimodality therapy for individual Patients.

THE CLINIC－PATHOLOGIC ANALYSIS OF 66 CASES WITH SOFT TISSUE SARCOMA OF ABDOMEN

We accepted 66 cases with soft tissue sarcoma of abdomen testified by the Pathologists. The ratio of male to female was 1.5 to 1, but the aged-groups in female were more. The male incidences increased as the ages did. The mean age was 46.79 ±17. 04. The neoplasms arises more often in the abdominal wall below 30 years old, and it does mainly in the abdominal viscera and in the retroperitoneum over 30 years old. Fibrosarcoma Protuberance was the main type in the abdominal wall,and leiomyosarcoma was the main type in the abdominal viscera, and in the retroperitoneum, the most was liposarcoma, the following was leiomyosarcoma. Among the soft tissue sarcoma of the abdomen. the most was in retroperitoneum, and the following was in abdominal viscera. Therefore, it was difficult to diagnose early. The keys to the question were B-mode ultrasonic examining,computed tomography (CT) scanning and magnetic resonance imaging (MRI). Surgery was the primary therapeutic modality. The resectability was the major prognostic factor Radiotherapy and chemotherapy indicated a clear beneficial effect on soft tissue sarcoma of the abdomen and they can slightly decrease the recurrences and the metastases, but they had no obvious influence on the survival. In 5-year survival, the lesion of abdomen was the best, and those both of abdominal viscera and of retroperitoneum were the bad. To increase the 5-year survival, what counts are early diagnosis and the resectability.

Alveolar Soft Tissue Sarcoma: A Case Report and Literature Review

Soft alveolar sarcoma is a rare tumor (0.5% to 1% of soft-tissue sarcomas) of poor prognosis that often affects the young subject, with a moderate male predominance. The authors present the diagnostic and therapeutic course of a 60-year-old patient from Niger with no particular previous history, consulted for a painless swelling of the right arm evolving for 6 months, in which the diagnosis of alveolar sarcoma of the soft parts of the arm was selected. A monobloc surgical excision decision was made, with the anatomy and function of the limb retained, with the filling of the area resected with cement and fixed by a locked nail. A conventional three-course chemotherapy regimen was instituted. Alveolar sarcoma of the soft tissues is a rare tumor whose imagery remains unclear, but the diagnosis is based on surgical excision with histopathological examination and immunohistochemical analysis.

Gemcitabine and Docetaxel as a Second-Line Treatment in Advanced Soft Tissue Sarcomas

OBJECTIVE Promising anti-tumor activity in patients with metastatic or unresectable soft tissue sarcomas has been reported with gemcitabine and/or docetaxel. METHODS Forty patients with advanced soft tissue sarcomas refractory to first-line chemotherapy treatment were enrolled. They received combination of gemcitabine at dose of 900 mg/m2 on days 1 and 8 and docetaxel at dose of 100 mg/m2 on day 8, and had this regimen repeated every 3 weeks. If the patients had received the pelvic irradiation in advance, gemcitabine dose was reduced to 675 mg/m2 on days 1 and 8 and docetaxel to 75 mg/m2 on day 8, and had it repeated every 3 weeks. RESULTS Gemcitabine/docetaxel combination was well tolerated by the patients with an overall response of 20%. After median follow-up of 15 months, a median overall survival time was 12 months （95% CI 7.042-16.958） and a median progression free survival time was 6 months （95% CI 5.445-6.545）. The most common hematologic toxicity was neutropenia （47.5%） while mucositis was the most common nonhematologic toxicity （45%）. The 1- and 2- year survival rates were 50% and 15%, respectively. CONCLUSION This regimen of gemcitabine/docetaxel combination as second-line treatment for the patients with advanced soft tissue sarcomas is effective with acceptable toxicities. These results should be evaluated in a large phase III trial.

Mutational analysis of the PTEN gene in soft tissue sarcomas

Objective： This study was to investigate whether PTEN mutations play a role in the carcinogenesis of soft tissue sarcomas （STS）. Methods： Polymerase chain reaction-single strand conformation polymorphism （PCR-SSCP） was used to amplify 4 exons of PTEN and to analyze the conformation polymorphism, then DNA sequencing methods was used to detect point mutation of PTEN gene four exons of abnormal single strand conformation in soft tissue sarcomas. Results： Two of 86 cases showed 130th condon G→A missense mutation in the exon 8 of PTEN gene, and this mutation made Arg to change to Gin in PTEN protein structure 334th condon A→T missense mutation in the exon 8 of PTEN gene, and this mutation made Asn to change to Lys in PI-EN protein structure. Conclusion： These data indicated the existence of PTEN mutation in soft tissue sarcomas, but PTEN gene mutation rate is very low. PTEN mutation may prays an less important role in the development and malignant transformation of soft tissue sarcomas.