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.

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.

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.

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.

Unusual Presentation of a Large Multi-Septated Cystic Hygroma: A Case Report and Comprehensive Literature Review

Background: Cystic hygromas are primarily found in the cervicofacial, thoracic, and abdominal regions, with limited occurrences in other areas. Despite existing literature on this condition, comprehensive descriptions and MRI findings of cystic hygromas in the extremities are rare. Aim: This case report aims to present a unique instance of a cystic hygroma in the left thigh. The objective is to provide detailed insights into the characteristics of this atypical presentation. Case Presentation: The case involves a 2-year-10 month-old girl with a cystic hygroma in her left thigh. The report includes a comprehensive description of the lesion’s clinical features and diagnostic evaluation, emphasizing the MRI findings to enhance understanding of this rare occurrence. Conclusion: This case report highlights the rarity of cystic hygromas outside the cervicofacial, thoracic, and abdominal regions, explicitly focusing on the occurrence in the left thigh. By presenting detailed insights into the clinical features, MRI findings, histopathological findings, and the surgical approach employed, this report contributes to the existing knowledge on this condition in atypical locations and informs future treatment strategies.

Multiple well-differentiated retroperitoneal liposarcomas with different patterns of appearance on computed tomography:A case report

BACKGROUND Primary retroperitoneal liposarcoma(PRPLS)is a rare soft tissue tumor with nonspecific clinical symptoms;it has different computed tomography(CT)image features according to pathological types.Some patients with a single tumor have been previously reported in the literature.We present an exceptional case of a PRPLS patient with multiple large tumors exhibiting different patterns of appearance on CT and confirmed as atypical lipomatous tumor/welldifferentiated liposarcoma by postoperative pathology.CASE SUMMARY A 64-year-old man presented with abdominal distension for 1 year.The patient was diagnosed with PRPLS based on physical examination,laparotomy,ultrasonography,CT scan,and surgery.Both of the tumors were completely resected through surgery and confirmed as atypical lipomatous tumor/welldifferentiated liposarcoma by postoperative pathology.The postoperative course was uneventful without recurrence or metastasis,as demonstrated by abdominalpelvic CT during an 18 mo follow-up.CONCLUSION Multiple large Well-differentiated liposarcomas with different patterns of appearance on CT image can occur simultaneously in the same patient,to which more attention should be paid to make an effective differential diagnosis.

Clinicopathologic analysis of angiomyofibroblastoma of the female genital tract

To study the clinicopathologic features and immunoprofile of angiomyofibroblastoma (AMF) with emphasis on differential diagnosis Methods Seven vulvar, two vaginal and one perineal angiomyofibroblastomas were evaluated by light microscopy and immunohistochemistry The immunohistochemical studies were performed on paraffin embedded sections using the standard ABC method with a panel of antibodies Results Angiomyofibroblastoma predominantly occurred in middle aged women in the genital region, especially in the superficial area of the vulva Clinically, most of the tumors presented as slowly growing painless masses and were often diagnosed as Bartholin’s gland cysts Histologically, the tumors were all well circumscribed and characterized by alternating hypocellular and hypercellular areas with abundant thin walled blood vessels The tumor cells were bland and spindle shaped or epithelioid and tended to concentrate around the vessels or cluster in small nests Immunohistochemically, the tumor cells expressed vimentin and estrogen receptor protein in all 10 cases and desmin in 9 cases Three cases showed weak or focal immunoreactivity to alpha smooth muscle actin and muscle specific actin All ten patients were treated by local excision Follow up showed a benign course with no signs of recurrence Conclusions Angiomyofibroblastoma is a distinctive neoplasm that has a propensity to occur in the female genital tract Recognition of this entity is important to avoid misdiagnosis with other vulvar angiomyxoid