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.

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.

Multiple clear-cell sarcomas of small intestine with parotid gland metastasis: A case report

Clear-cell sarcoma is a rare, malignant soft tissue tumor that displays melanocytic differentiation with a distinct molecular profile. It is rarely localized in the gastrointestinal tract. Herein we reported a case of multiple synchronous clear-cell sarcomas of the gastrointestinal tract with parotid gland metastasis. A 51-year-old male patient presented with a growing painless mass under the right ear. A preoperative positron emission tomography/computed tomography showed multiple intestinal masses and a mass in the right parotid with increased glucose uptake, and he underwent operative treatment with resection of three tumors in the jejunum and ileum and then received a right parotidectomy. Postoperative pathological examination showed that cells in the intestinal tumor were consistent with clear-cell sarcoma of the gastrointestinal tract, and the malignant cells in the parotid gland were similar to the intestinal tumor. Immunohistochemical studies revealed positive expression of HMB-45, Melan-A, and S-100. EWSR1 gene fusion transcripts were undetectable by fluorescence in situ hybridization.

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.

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.

Maxillofacial sarcomas: a ugandan epidemiological survey

We reviewed the case notes of 203 patients who were treated for sarcomas of the oral and maxillofacial region over a period of 5 years. There were 98 male cases (mean age 31.4 ± 12.8 years) and 105 female cases (mean age 29.3 ± 10.4). Kaposi’s sarcoma accounted for 82.8% cases, and rhabdomyosarcoma 6.9% followed by osteosarcoma and chondrosarcoma at 2.5% each. Except for Kaposi’s sarcoma, surgery in combination with radiotherapy and/or chemothe- rapy was the main stay of treatment. Survival data was not available for most of our patients.

Clinical analysis of 251 cases of soft tissue sarcomas

Objective To explore the therapeutic principles and prognostic factors of soft tissue sarcoma. Methods Two hundred and fifty-one patients with soft tissue sarcoma (STS) treated at Shanghai Cancer Hospital during 1986 -1990 were reviewed retrospectively. Results The 1-, 3 - , 5 - , 10 - year tumor-free survival rates were 67. 74% , 57. 16% , 52, 41% , 38. 60% , respectively.The overall survival rates for 1,3,5 and 10 years were 81. 01 % ,67.75% ,60.79% ,and 49.23% respectively. Log-rank test showed that the patients with different pathological findings, histological grades,mass location and size, anatomical depth, and surgical margin showed different outcomes. Whether the sarcomas invaded the vessels or metastasized would influence the survival rates. The patients who underwent different interventions or operations also had different outcomes. The prognosis of STS was associated with age,histological type,histological grade, tumor size, surgical margin and metastasis according to the Cox regression

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.

Surgical treatment outcomes of primary hepatic sarcomas: A singlecenter experience

BACKGROUND Primary hepatic sarcoma is a rare tumor originated from mesenchymal tissue.There are various pathologic types of primary hepatic sarcoma and the treatment outcome of this tumor was usually disappointing.Unlike hepatocellular carcinoma,outcome of primary hepatic sarcoma is not well-known due to it’s rarity.However,with development of medical technology,surgical treatment may lead to better survival.AIM To investigate the surgical outcomes of primary hepatic sarcoma,we gathered and analyzed the cases of a single institute.METHODS From August 2001 to September 2016,a total of nine patients were surgically treated for primary hepatic sarcoma after exclusion of cases with open and closure,early loss to follow-up and sarcomatoid hepatocellular carcinoma and sarcomatoid cholangiocellular carcinoma.Baseline characteristics,tumor characteristics such as tumor pathology,size and number,surgical and adjuvant treatments were reviewed.Tumor recurrence,and patient survival were analyzed with retrospective approach.RESULTS The enrolled participants included five patients with angiosarcoma and four patients with undifferentiated sarcoma.All patients experienced tumor recurrence at a median of 52 post-operative days.Only two patients survived and the 5-year survival rate was 29.6%.One patient with angiosarcoma who received central hepatectomy for primary tumor and received radiofrequency ablation for recurrent tumor still lives for 11 years.One patient with undifferentiated sarcoma received Rt.lobectomy for primary tumor followed by chemotherapy and radiation therapy still lives around 30 mo even though she got additional operation for recurrent tumor.Two patients who received living donor liver transplantation due to angiosarcoma died.Only adjuvant therapy was associated with survival gain(P=0.002).CONCLUSION Patients with primary hepatic sarcoma may gain survival benefit with surgical resection followed by adjuvant therapy,even though the outcome remains relatively poor.

ALDH Activity Correlates with Metastatic Potential in Primary Sarcomas of Bone

Osteosarcoma (OS), chondrosarcoma (CSA), and Ewings sarcoma (ES) are the most common primary malignancies of bone, and are rare diseases. As with all sarcomas, the prognosis of these diseases ultimately depends on the presence of metastatic disease. Survival is therefore closely linked with the biology and metastatic potential of a particular bone tumor’s cells. Here we describe a significant correlation of aldehyde dehydrogenase (ALDH) activity and the presence/absence of distant metastases in ten consecutive cases of human bone sarcomas. Additionally, cultured human CSA cells, which are historically chemo- and radio-resistant, may be sensitive to the ALDH inhibitor, disulfiram. While it is premature to draw broad conclusions from such a small series, the importance of ALDH activity and inhibition in the metastatic potential of primary bone sarcomas should be investigated further.

The role of radiotherapy in the management of localized soft tissue sarcomas

The combination of radiotherapy(RT) and function-preserving surgery is the most usual contemporary approach in the management of soft tissue sarcomas(STS). Pre- and postoperative RT result in similar local control rates, as shown by a landmark trial in extremity STS. In this review, the role of RT in the management of extremity STS will be discussed, but STS in other sites,including retroperitoneal STS, will also be addressed. The focus will consider various aspects of RT including strategies to reduce the volume of tissue being irradiated, dose, scheduling, and the possible of omission of RT in selected cases. Finally, technology advances through the use of intensity-modulated radiotherapy(IMRT), image-guided IMRT, intraoperative radiotherapy(IORT)and particle therapy will also be discussed.

Resection and reconstruction of pelvic and extremity soft tissue sarcomas with major vascular involvement: Current concepts

Soft tissue sarcoma accounts for approximately 1% of all cancers diagnosed annually in the United States. When these rare malignant mesodermal tumours arise in the pelvis and extremities, they may potentially encase or invade large calibre vascular structures. This presents a major challenge in terms of safe excision while also leaving acceptable surgical margins. In recent times, the trend has been towards limb salvage with vascular reconstruction in preference to amputation. Newer orthopaedic and vascular reconstructive techniques including both synthetic and autogenous graft reconstruction have made complex limb-salvage surgery feasible. Despite this, limb-salvage surgery with concomitant vascular reconstruction remains associated with higher rates of post-operative complications including infection and amputation. In this review we describe the initial presentation and investigation of patients presenting with soft tissue sarcomas in the pelvis and extremities, which involve vascular structures. We further discuss the key surgical reconstructive principles and techniques available for the management of these complex tumours, drawn from our institution's experience as a national tertiary referral sarcoma service.

Is there consensus regarding surgical treatment of bone sarcomas?

AIM To perform an Internet based survey on the surgical management of bone sarcomas in the lower extremity amongst sarcoma surgeons. METHODS All orthopedic surgical members of the Scandinavian Sarcoma Group were invited to participate in an online questionnaire. The questionnaire consisted of a clinical case involving resection of a malignant bone tumor. Several questions were asked, subdivided into categories. Among these, surgical/technical considerations, e.g., choice of implant; choice of antibiotics, dosage, and duration of treatment, choice of antithrombotic drug, initial start-up, dosage, and duration were included.RESULTS In terms of choice of implant fixation, the majority of surgeons preferred an uncemented prosthesis in younger patients until the age of 50. All participants administer intravenous prophylactic antibiotics for endoprosthetic reconstructive surgery. First choice of antibiotics was cephalosporin. Less common used was glycopeptide, penicillin, or a combination. Duration of prophylactic antibiotics ranged from less than one day to more than four days. All participants used low molecular weight heparins as antithrombotic prophylaxis and 55% of the participants answered that initial treatment was started preoperatively, 3% perioperatively and 42% postoperatively. Duration of the antithrombotic treatment ranged from five days to more than twenty-eight days.CONCLUSION The use of resection prosthesis in the treatment of bone sarcomas is a well-established procedure. However, therse is a significant discrepancy in the surgical treatment algorithm between the sarcoma centers. Still the treatment is mainly based on best clinical practice,due to the lack of evidence-based medicine in the surgical management of bone sarcomas.

Vulvar sarcomas: A 25 plus-year experience from Queensland

Objective: To review the characteristics of cases of vulvar sarcomas (VS) referred to the Queensland Centre for Gynaecological Cancer (QCGC) between mid 1984 and mid 2012. Methods: VS case data from QCGC were reviewed and analysed using the computer software Statistical Package for the Social Sciences (SPSS) 11.0. Results: Of the nine cases three died of their disease, one died of ovarian cancer and five are still alive and disease free. The mean age at diagnosis was 54.8 years. Time from onset of symptoms to diagnosis averaged one month. All diagnoses were confirmed histologically. The most common presenting complaint was a lump. Treatment in all but one case involved surgical excision, one case had chemotherapy alone and two cases had surgery followed by radiotherapy. Conclusions: To talk of five year survival for patients with such a rare cancer, so many histological types with variable cell differentiation plus the unknown of when sarcomatous cells metastasize from even early FIGO staged lesions is in our opinion taking generalisation to far. A multidisciplinary approach to management achieves the best results for each individual and pooling multicentre data will improve our understanding of this rare disease and benefit future patients.

High-Grade Extremity Myxofibrosarcoma with Synchronous Mesenteric Leiomyosarcoma: Double Primary Sarcomas

Here, we report the case of a 47-year-old male who presented with a painless palpable mass in the right shoulder. This extremity tumor was diagnosed as a high-grade myxofibrosarcoma after a wide excision. Simultaneously, a synchronous mesenteric mass was discovered, which proved to be a leiomyosarcoma.

The Importance of the Multidisciplinary Approach to Surgical Treatment of Extremity Soft-Tissue Sarcomas

Soft tissue sarcomas (STS) are rare neoplasm with frequencies around 1% of all neoplasms. Although it consists of a high heterogeneous group of tumors, surgery is the mean treatment. The STS surgery is still challenging and complex procedure is usually required: this is because STS requires different types of resection and reconstruction due to various tissue-commitments (nerve, arteries, skin and muscle). So, a multidisciplinary team must be prepared for STS approach to obtain the maximum local control and a limited extremity functional impairment. We, here, showing our experience, wish to introduce some technical contrivances in STS surgery, with special reference to tissue reconstruction. This may illustrate the necessity of a multidisciplinary team approach in this surgery.

Management of Phylloid Sarcomas: A Retrospective Study of 12 Cases

Background: Phylloid sarcomas are rare. There is not enough data to codify the management. Objectives: The objective was to study the clinical and therapeutic aspects and the fate of patients after a follow-up of at least 4 years. Thus contributing to the limited body of knowledge on these tumors. Methods: a retrospective analysis of the files from 2013 to 2017 was carried out and patients were followed up until 2021at Hassan II Hospital. Epidemiological, clinical and therapeutic aspects were studied. Survival was calculated using the Kaplan-Meier method. Results: We collected 12 charts of patients treated for phyllodeal sarcoma from 2013 to 2017. The median age was 43 years. The circumstance of discovery was marked by the presence of nodule in all patients. The coupled echo-mammography examination classified the nodules, ACR 4 in 7 patients and ACR 3 in 3 and ACR 5 in 2 patients. Histological examination revealed a phylloid sarcoma in 11 patients and a borderline phylloid tumor in 1 patient. All patients had radical surgery with positive margins in 2 patients, 16.66%. One patient had revision surgery. Histological examination of the surgical specimens showed phylloid sarcoma on all specimens. All patients had adjuvant radiotherapy with doses of 50 Gy in 25 fractions of 2 Gy and a boost of 10 Gy was done in one patient. The median spread of radiotherapy was 37 days. Grade 1 and 2 skin toxicities were noted in 5 and 3 patients respectively. The median time from surgery to radiotherapy was 2.95 months. 3 patients relapsed after 13.6 months of follow-up. The recurrence-free survival at 1 and 3 years was 83% and 75% respectively. Overall survival at 3 and 5 years was 83% and 75% respectively. Conclusion: This is a rare entity which requires randomized trials to codify its management. It would seem that the multidisciplinary approach, associating surgery ± radiotherapy, is a good option.

Management of Uterine Sarcomas

Uterine sarcomas are rare malignant tumors characterized by a great clinical and histopathological diversity. The aim of this work is to analyze the difficulties of diagnosis, therapeutic and prognosis posed by these tumors. Thirty-seven patients with uterine sarcoma, collected in the service Radiotherapy and Oncology, University Ibn Rochd of Casablanca between January 2000 and December 2007 were included in this study retrospective. The average patient age was 50 years (17-76). The bleeding was present in all patients, isolated in 54% of cases associated with pelvic pain in 24.6% and a mass abdomino-pelvic in seven patients. The average time of evolution was 10 months. The main histological type was found leiomyosarcoma. Twenty four patients in our series underwent total hysterectomy without annexial conservation. The surgery R0 was obtained in 43% of cases. The sarcomas were classified as stage IV in 51.4%. Adjuvant radiotherapy was indicated in 13 patients. After a mean of 20 months, half of patients evaluable presented a local relapse and/or metastatic, the third of cases were tumor progression while complete remission was maintained in 18.5% of cases. Uterine sarcomas are rare malignant mesenchymal tumor, which often occur in women after menopause. The main prognostic factors are hormonal status of the patient, stage clinical, histological type, histological grade and quality surgical excision. The management of uterine sarcomas is multidisciplinary, based mainly on surgery remains the only means of cure. Adjuvant radiotherapy allows decreased risk of local recurrence, with no impact on survival achieved at best 30% at 5 years. The role of chemotherapy remains confirm.

Clinical utility of circulating tumor DNA sequencing with a large panel in patients with advanced soft-tissue sarcomas

To the editor Soft-tissue sarcomas(STS)represent a very heterogeneous group of rare tumors including more than 100 different subtypes[1].Surgery and neo/adjuvant radiation therapy represent the cornerstone of treatment for STS.However,despite an optimal resection of the tumor,up to 40%of patients will develop metastatic relapse and will die from the disease[1].Doxorubicin represents the first-line standard of care for patients with advanced disease since the 1970s,despite several attempts to identify better regimens.The median overall survival(OS)of patients with metastatic disease is<18 months and has only modestly improved over the past 20 years[2].

Survival differences of low-grade versus high-grade head and neck pleomorphic dermal sarcomas and a review of a scalp case

Objective:To examine survival by tumor grade of pleomorphic dermal sarcomas(PDS)of the head and neck(H&N)and review a scalp PDS case.Methods:Patients in the Surveillance,Epidemiology,and End Results(SEER)database were included from 1980 to 2016 based on a diagnosis of H&N PDS.Survival estimates were performed using Kaplan–Meier analysis.Additionally,a case of a grade III H&N PDS is presented.Results:Two hundred-seventy cases of PDS were identified.The mean age at diagnosis was 75.1 years(SD:13.5).Two hundred-thirty-four(86.7%)patients were male.Eighty-seven percent of patients received surgery as a part of their care.The 5-year overall survival rates for grades I,II,III,and IV PDSs were 69%,60%,50%,and 42%,respectively(P=0.03).Conclusions:H&N PDS occurs most commonly in older-age males.Surgical management is frequently a part of H&N PDS care.Survival rates significantly decline based on tumor grade.