Extra Renal Rhabdoid Tumor: A Rare Cause of Congenital Soft Tissue Tumor

Rhabdoid tumors (RTs) are a well-defined entity in the kidney or central nervous system of infants or children. However, soft-tissue involvement is uncommon. It’s an exceptional neonatal tumor of soft tissue. The imaging characteristics of this tumor are not specific. Biopsy allows diagnosis;the histomorphological characteristics of rhabdoid tumors, their immunoreactivity to epithelial markers and vimentin, and the INI-1 loss are important tools for diagnosis. RT tumors are aggressive and have a rapidly fatal clinical course in most cases. Despite multidisciplinary therapy, the survival rate is very low. We report a rare case occurring in a male neonate who presents at birth with a voluminous right axillary mass. A CT scan showed a well-limited tumor mass with lobulated contours. An ultrasound-guided biopsy was performed on day 8, showing the morphology and immunoprofile of RT. The mass showed rapid growth. The child was admitted for respiratory distress at 3 weeks. A thoraco-abdominal CT showed an increase in the size of the mass with the appearance of multiple lymph nodes and pleural, hepatic, and renal metastases. The child died two days later.

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.

Bone and soft tissue tumors presenting as sciatic notch dumbbell masses: A critical differential diagnosis of sciatica

AIM To study the clinical findings and characteristic features in sciatic notch dumbbell tumors(SNDTs).METHODS We retrospectively reviewed the clinical outcomes and characteristic features of consecutive cases of SNDTs(n = 8). RESULTS Buttock masses occurred in three patients with SNDT(37.5%). Severe buttock tenderness and pain at rest were observed in seven patients with SNDTs(87.5%). Remarkably, none of the patients with SNDTs experienced back pain. Mean tumor size was 8.4 ± 2.0 cm(range, 3.9 to 10.6 cm) and part of the tumor mass was detected in 2 patients in the sagittal view of lumbar magnetic resonance imaging(MRI).CONCLUSION The clinical information regarding to SNDTs is scarce. The authors consider that above mentioned characteristic findings may facilitate the suspicion of pelvic pathology and a search for SNDT by MRI or computed tomography should be considered in patients presenting with sciatica without evidence of spinal diseases.

Magnetic resonance imaging of soft-tissue tumors of the extremities: A practical approach

Diagnosis of extremity soft-tissue tumors can be challenging.Characteristics of tumor margins can help precisely identify locally aggressive or non-aggressive behavior for surgical planning,but cannot differentiate benign from malignant lesions.Most malignant tumors can have inhomogeneous signals on T2-weighted images.Although a uniform signal on T2-weighted images can be a reliable indication of a benign lesion,a well-defined mass with homogeneous internal signal intensity does not definitively identify a benign lesion.Some common and distinctive soft-tissue lesions can have specific clinical and imaging features allowing a diagnosis without biopsy.These are known as determinate lesions.This illustrative report presents a diagnostic guide for extremity soft-tissue tumors based on tissue signal and morphological characteristics on magnetic resonance images.It is important for clinicians to be familiar with the imaging characteristics of common determinate lesions.

Metastatic Soft Tissue Tumors

Between 1998 and 2007, we treated 157 patients with malignant soft tissue tumors. Patients with obvious lymph node metastasis, metastasis from bone or soft tissue sarcoma, and with obvious advanced stage disease which had multiple metastases were excluded from the present analysis. Finally, sixteen of the 157 patients were diagnosed to have soft tissue metastasis from carcinomas or hematopoietic malignancies. There were 10 males and 6 females with an average age of 65 (range, 50 - 83) years. Four lesions were located in the abdomen and back, 3 in the thigh, 2 in the chest, and 1 lesion each in the upper arm, lower leg and buttock. There were 9 intramuscular and 7 subcutaneous metastases. The average tumor size was 6 cm (range 2 - 17 cm). The initial diagnoses were a primary soft tissue tumor in 8 cases, primary soft tissue tumor or metastatic soft tissue tumor in 6, and inflammatory lesions in 2. Four of the 6 cases were suspected to be a metastatic soft tissue tumor in the initial diagnosis based on a past history of malignancy. There were no characteristic findings of metastatic soft tissue tumors in the imaging studies. The primary origins were lung cancer in 6 cases, malignant lymphoma in 5, gastric cancer in 2, and esophageal cancer, pancreatic cancer and uterine cancer in 1 patient each. Treatments were administered to all cases, including radiotherapy and chemotherapy in 11, chemotherapy in 2, wide resection and chemotherapy in 1, radiotherapy in 1 and palliative therapy in 1. The final oncological status was continuous disease free survival in 1 patient, no evidence of disease in 1, alive with disease in 7, and dead of disease in 7 patients. The estimated one-year survival was 47% and the two-year survival was 0%.

HISTOLOGIC GRADE, AND CONTENT AND IMAGE ANALYSIS OF SOFT TISSUE TUMORS

Flow cytometry and image analysis technique were used to quantltate the nuclei of various soft tissue tumors. A single representing section from soft tissue sarcoma was used for histologic grading. Histologlc and cytometric comparative analyses showed that all 21 benign tumors were diploid. Among 62 cases of soft tissue sarcoma, 45(73%) were aneuploid. There was a significant difference in the nuclear area between benign and malignant tumors (P<0. 01), dlploid and aneuploid tumors (P<0. 05). The two new techniques are valuable In cellular quantitative measurement for soft tissue tumors.

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.

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.

Experimental Study of Multi-slice Spiral CT Perfusion Imaging in VX_ 2 Soft-tissue Tumor of Rabbits

An experimental animal model of malignant soft-tissue tumor was established to investigate the applied value of multi-slice spiral CT perfusion imaging preliminarily. Ten New Zealand white rabbits which were implanted with VX2 tumor in either proximal thigh were subjected to CT plain scan and perfusion scan two weeks later respectively, then the original perfusion images were transmitted to AW4.0 Workstation. The functional maps and perfusion parameters including blood flow （BF）, blood volume （BV）, mean transit time （MTT） and permeability surface （PS） were computed and analyzed. All the values of BF, BV and PS in VX2 soft-tissue tumors were obviously higher while the MTT-values were lower than those in the normal muscular tissues significantly. It was suggested that multi-slice spiral CT perfusion imaging is an accurate, convenient and relatively safe functional imaging technique, and can give a quantitative assessment to angiogenesis and blood perfusion of soft-tissue tumors.

Malignant giant cell tumor in the left upper arm soft tissue of an adolescent:A case report

BACKGROUND Giant cell tumor of soft tissue(GCT-ST)is an extremely rare low-grade soft tissue tumor that is originates in superficial tissue and rarely spreads deeper.GCT-ST has unpredictable behavior.It is mainly benign,but may sometimes become aggressive and potentially increase in size within a short period of time.CASE SUMMARY A 17-year-old man was suspected of having a fracture,based on radiography following left shoulder trauma.One month later,the swelling of the left shoulder continued to increase and the pain was obvious.Computed tomography(CT)revealed a soft tissue mass with strip-like calcifications in the left shoulder.The mass invaded the adjacent humerus and showed an insect-like area of destruction at the edge of the cortical bone of the upper humerus.The marrow cavity of the upper humerus was enlarged,and a soft tissue density was seen in the medullary cavity.Thoracic CT revealed multiple small nodules beneath the pleura of both lungs.A bone scan demonstrated increased activity in the left shoulder joint and proximal humerus.The mass showed mixed moderate hypointensity and hyperintensity on T1-weighted images,and mixed hyperintensity on T2-weighted fat-saturated images.The final diagnosis of GCT-ST was confirmed by pathology.CONCLUSION GCT-STs should be considered in the differential diagnosis of soft tissue tumors and monitored for large increases in size.

Characteristics of primary giant cell tumor in soft tissue on magnetic resonance imaging:A case report

BACKGROUND Primary soft tissue giant cell tumor(GCT-ST)is rare and has relatively low malignant potential.Most reports are pathological and clinical studies,while imaging studies have only been reported in cases of adjacent bone or with atypical cystic degeneration.With regard to the findings on magnetic resonance imaging(MRI)or ultrasonography,superficial masses can be further identified based on facial edema,skin thickening,skin contact,internal hemorrhage or necrosis and lobulation of the mass.Unlike deep-seated masses,MRI features do not always provide an accurate diagnosis for benign and malignant patients with superficial soft-tissue lesions.Thus,the application of diffusion-weighted imaging(DWI)to evaluate superficial soft tissue tumors is necessary.CASE SUMMARY A 36-year-old woman who had a suspected malignant tumor in the upper limb on ultrasound and computed tomography is reported.The signal intensity of the suspected tumor was heterogeneous on plain MRI;nodular and heterogeneous enhancement was observed in the tumor with irregular shapes and blurred margins on dynamic contrast-enhanced MRI.The lesion on DWI was hyperintense with a higher mean apparent diffusion coefficient(ADC)value.Finally,a GCT-ST was confirmed by pathology.This case suggests that GCT-ST should be distinguished as a benign soft tissue mass from giant cell-rich soft tissue neoplasms or malignant tumors.CONCLUSION The MRI features of the superficial GCT-ST in the upper limb included heterogeneous signal intensity within the lesion on T2-weighted image(T2WI)and T1-weighted fat-saturation spoiled gradient recalled echo(T1 FSPGR),nodular enhancement with blurred margins,irregular shapes,and a slow-increased enhancement.DWI could be used to differentiate a benign soft tissue mass from a malignant mass by the mean ADC value and provide more radiologic-pathologic information for the diagnosis of GCT-ST.Comprehensive imaging of primary GCT-ST could help complete tumor resection,and in turn likely prolong survival after surgery.

Contrast investigation of multi-slice spiral CT perfusion imaging and pathological findings in VX2 soft-tissue tumor of rabbits

Objective: To perform a contrast investigation of multi-slice spiral CT (MSCT) perfusion imaging and pathological findings in VX2 soft-tissue tumor of rabbits, and discuss the applicative value of multi-slice spiral CT perfusion imaging in soft-tissue tumors. Methods: 8 Newzealand white rabbits were implanted with 0.1 ml VX2 tumor tissue suspension in bilateral proximal thighs. 14 days and 21 days later, CT plain scan and perfusion scan were performed on these rabbits respectively, then the images were transmitted to AW4.0 workstation, the functional maps and perfusion parameters including blood flow (BF), blood volume (BV), mean transit time (MTT) and permeability surface (PS) were computed and analyzed. Subsequently, the rabbits were sacrificed, the tumors of which were taken out for pathological examination. The correlation between MSCT functional parametric images and pathological findings was analyzed. Results: All the values of BF, BV and PS of VX2 soft-tissue tumors were obviously higher while the MTT-values were lower than those of the normal muscular tissues significantly (P<0.001). Conclusion: Multi-slice spiral CT perfusion imaging is an accurate, convenient and relatively safe functional imaging technique, which can give a quantitative assessment to blood perfusion and angiogenesis of soft-tissue tumors.

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.

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.

Nd:YAG water mist laser treatment for giant gestational gingival tumor: A case report

BACKGROUND This case of gestational gingival tumor is huge and extremely rare in clinical practice.As the growth location of this gingival tumor is in the upper anterior tooth area,it seriously affects the pregnant woman's speech and food,causing great pain to the patient.The use of Nd:YGA water mist laser to remove the gingival tumor resulted in minimal intraoperative bleeding,minimal adverse reactions,and good postoperative healing,which is worthy of clinical promotion and application.CASE SUMMARY The patient,a pregnant woman,reported a large lump in her mouth on the first day of postpartum treatment.Based on medical history and clinical examination,the diagnosis was diagnosed as gestational gingival tumor.Postoperative pathological biopsy also confirmed this diagnosis.The use of Nd:YAG water mist laser to remove the tumor resulted in minimal intraoperative bleeding,clear surgical field of view,short surgical time,and good postoperative healing.CONCLUSION In comparison to traditional surgery,Nd:YAG water mist laser surgery is minimally invasive,minimizes cell damage,reduces bleeding,ensures a clear field of vision,and virtually eliminates postoperative edema,carbonization,and the risk of cross infection.It has unique advantages in oral soft tissue surgery for pregnant patients.Therefore,the clinical application of Nd:YAG water mist laser for the treatment of gestational gingival tumors is an ideal choice.

Sonographic Diagnosis of Bone Tumors

This paper presents the results of sonographic and radiographic exami nations in 48 patients clinically diagnosed as having bone tumors. Sonography revealed bone destruction in all 48 cases, elevated periosteum in 26 cases and soft tissue mass in 34 cases. The results obtained in this series demonstrate that mostbone tumors have their characteristic sonographic features such as giant cell tu-mors, malignant bone tumors, bone cysts, as well as metastatic lesions. Studyshowed that sonography has equally high accuracy in the diagnosis of these tumorscompared with radiography.

Clinical and imaging features of desmoid tumors of the extremities

BACKGROUND Desmoid fibroma is a rare soft tissue tumor originating from the aponeurosis,fascia,and muscle,and it is also known as aponeurotic fibroma,invasive fibroma,or ligamentous fibroma.AIM To investigate the clinical and imaging features of desmoid tumors of the extremities.METHODS Thirteen patients with desmoid fibroma of the extremities admitted to our hospital from October 2016 to March 2021 were included.All patients underwent computed tomography(CT),magnetic resonance imaging(MRI),and pathological examination of the lesion.Data on the diameter and distribution of the lesion,the relationship between the lesion morphology and surrounding structures,MRI and CT findings,and pathological features were statistically analyzed.RESULTS The lesion diameter ranged from 1.7 to 8.9 cm,with an average of 5.35±2.39 cm.All lesions were located in the deep muscular space,with the left and right forearm each accounting for 23.08%of cases.Among the 13 patients with desmoid fibroma of the extremities,the lesions were"patchy"in 1 case,irregular in 10,and quasi-round in 2.The boundary between the lesion and surrounding soft tissue was blurred in 10 cases,and the focus infiltrated along the tissue space and invaded the adjacent structures.Furthermore,the edge of the lesion showed"beard-like"infiltration in 2 cases;bone resorption and damage were found in 8,and bending of the bone was present in 2;the boundary of the focus was clear in 1.According to the MRI examination,the lesions were larger than 5 cm(61.54%),round or fusiform in shape(84.62%),had an unclear boundary(76.92%),showed uniform signal(69.23%),inhomogeneous enhancement(84.62%),and"root"or"claw"infiltration(69.23%).Neurovascular tract invasion was present in 30.77%of cases.CT examination showed that the desmoid tumors had slightly a lower density(69.23%),higher enhancement(61.54%),and unclear boundary(84.62%);a CT value<50 Hu was present in 53.85%of lesions,and the enhancement was uneven in 53.85%of cases.Microscopically,fibroblasts and myofibroblasts were arranged in strands and bundles,without obvious atypia but with occasional karyotyping;cells were surrounded by collagen tissue.There were disparities in the proportion of collagen tissue in different regions,with abundant collagen tissue and few tumor cells in some areas,similar to the structure of aponeuroses or ligaments,and tumor cells invading the surrounding tissues.CONCLUSION Desmoid tumors of the extremities have certain imaging features on CT and MRI.The two imaging techniques can be combined to improve the diagnostic accuracy,achieve a comprehensive diagnosis of the disease in the clinical practice,and reduce the risk of missed diagnosis or misdiagnosis.In addition,their use can ensure timely diagnosis and treatment.

The Application and Indications for Sentinel Lymph Node Biopsy in Pediatric Solid Tumors: Current Status and Future Directions

Oncologic staging of many solid organ malignancies involves an understanding of how cancers spread via the lymphatic system, and ultimately may involve evaluation of the primary nodal drainage basin by performing a sentinel lymph node biopsy. In the adult population, there are well established indications for sentinel lymph node biopsy in diseases like melanoma and breast cancer. However, its use and relevance in the pediatric population is less defined. This review details the history and development of sentinel lymph node biopsy technique, advanced lymph node mapping techniques currently under investigation, and the applications of sentinel node biopsy towards childhood cancers.

Primary Malignant Musculosqueletal Tumors of Members in Adult in Togo

Purpose: Primary malignant musculosqueletal tumors in adult are rare affections group and its treatment is still a real challenge today. The aim of this work was to describe the epidemiologic and treatment aspects of these tumors in the national reference center of our country. Materiel and Methods: It was a retrospective review of primary malignant musculosqueletal tumors treated in orthopedics and trauma unit of Sylvanus Olympio teaching hospital of Lomé, Togo from January 2000 to December 2014. Results: During the study period, 28 cases were selected and reviewed. There were 17 men (60.71%) and 11 women (39.29%). The average age was 32.7 years. There were 20 cases (71%) of primary malignant bone tumors (PMBT) and 8 cases (29%) of primary malignant soft tissues tumors (PMSTT). There were 6 (30%) of osteosarcoma. The tumor was located in the bones of the forearm and wrist/hand in 2 (10%) patients for each anatomical site;for the lower limb, around knee in 7 (35%) patients. Eight patients had PMSTT (28, 57%). There were three cases of rhabdomyosarcoma, 2 cases of fibrosarcoma. Tumors were located around knee in 4 cases and around ankle/foot in 3 cases. In the two groups of tumor, tumor resection was performed in 5 patients (17.86%) and limb amputation indicated in 23 patients (82.14%), was performed in 15 (53.57%) and 8 patients (37.78%), rejected it and left hospital against medical advice. Conclusion: Malignant musculoskeletal tumors are relatively rare in Togo. Their treatment is based on radical surgery which is often not supervised by adjuvant therapies. Ignorance, poverty of the population and embryonic state of diagnostic and treatment infrastructures are the obstacles to their care. Education and awareness must be integrated to care and fight against this group of diseases.

儿童非横纹肌肉瘤类软组织肉瘤的临床特点及预后

目的 分析儿童非横纹肌肉瘤类软组织肉瘤(NRSTS)的临床特点、治疗及预后影响因素。方法 回顾性分析2012年8月—2022年8月确诊并在我院接受规范治疗的NRSTS患儿的临床资料,通过Kaplan-Meier法分析患儿的预后及影响因素。结果 共纳入49例患儿,男27例,女22例,中位诊断年龄52.7 (0.8~149.6)个月。病理亚型有14种,包括婴儿型纤维肉瘤9例(18.4%)、肾外横纹肌样瘤8例(16.3%)、炎性肌纤维母细胞瘤7例(14.3%)、肝未分化胚胎性肉瘤7例(14.3%)、其他病理亚型18例(36.7%)。在多学科综合治疗下,初治完全缓解43例,部分缓解1例,有效率89.8%。复发15例(30.6%),中位复发时间14 (3~40)个月,复发后中位生存时间16个月。中位随访时间37(4~125个月),3年OS为(74.9±6.6)%,3年EFS为(59.4±7.3)%。Ⅰ+Ⅱ期、Ⅲ+Ⅳ期的3年OS分别为(94.4±5.4)%vs(56.9±10.4)%(P=0.003),3年EFS分别为(72.9±9.6)%vs(46.2±10.3)%(P=0.042)。COG危险度分组低危组和中高危组3年OS分别为100%vs(62.0±9.2)%(P=0.009)。结论 儿童NRSTS治疗以多学科综合治疗为主,完全手术切除是预后良好的关键,COG中高危组预后不佳,复发患儿预后较差。