Common Soft Tissue Tumors Research Articles

Guyon's canal resulting from lipoma: A case report and review of the literature.

IntroductionCompression of the ulnar nerve at the wrist is a rare condition. Clinical presentations of ulnar nerve compression at the wrist and hand can vary widely due to the complex anatomic course of ulnar nerve at the wrist. Lipoma is a common benign soft tissue tumor and rarely causes neuropathy. We present a rare case of Guyon's canal syndrome caused by lipoma.Case reportsWe describe a case with acute ulnar nerve deficit. Clinical examination, EMG and MRI suggested Guyon's canal syndrome. Surgical exploration revealed a well-encapsulated fatty tumor within Guyon's canal. Histopathology demonstrated mature adipose tissue consistent with lipoma. Post-operative course was uneventful and the sensory symptom had subsided at the three month follow up.Discussion and conclusionThere are many etiologies of Guyon's canal syndrome which can be classified into groups. Symptoms of ulnar nerve compression at the wrist can vary depend on the location of the lesion. Treatment options depend on severity and duration of symptoms, previous treatment attempts, and underlying etiology. Lipoma is not a common cause of Guyon's canal with few case reports in the literature. Surgery to open and release the roof of Guyon's canal and excision of the lipoma can yield good outcome in most reports.

HISTOPATHOLOGICAL STUDY OF SOFT TISSUE TUMOURS AND CORRELATION OF HISTOLOGIC GRADE OF SARCOMAS WITH PROLIFERATIVE MARKER KI-67

INTRODUCTION:Soft tissue tumours have diagnostic and therapeutic challenge due to a wide variety of histomorphological patterns. Soft tissue sarcomas are relatively infrequent and constitute less than 1 % of all malignant neoplasms. Immunohistochemical evaluation of Ki-67 is a rapid prognostic tool to assess the cellular proliferation better than mitotic index. METHODS: A prospective study was conducted with a total of 100 cases of soft tissue tumors to analyze the frequency and histomorphology. Soft tissue sarcomas were graded by FNCLCC grading system and were subjected to Ki-67 evaluation. Spearman's Rho and Pearman's correlation coefcients were calculated to determine the correlation between the histologic grade and Ki-67 index. RESULTS: Soft tissue tumours constituted only about 1.3% of all neoplasms. Benign tumours (50%) outnumbered intermediate (11%) and malignant soft tissue tumours (39%). Soft tissue tumours were more common in males (52%) than females (48%) and frequently observed in the sixth decade. The sites of predilection were upper extremities for benign tumours and lower extremities for malignant tumours. Most common benign soft tissue tumour was Neurobroma (19%) followed by Schwannoma (14%). Undifferentiated pleomorphic sarcomas were the most common malignant soft tissue tumour (28%). In FNCLCC grading, most of the sarcomas were grade III (42%). Ki-67 index was low in grade I sarcomas, variable in grade II sarcomas and high in grade III sarcomas. Spearman's rho and Pearman's correlation R values were 0.77 and 0.75 respectively and p value is less than 0.05. CONCLUSION: There is positive correlation between the histologic grade and Ki-67 proliferative index in soft tissue sarcomas. Ki-67 index can be used as an independent prognostic factor to predict the risk of distant metastasis. Prospective evaluation of Ki-67 should be carried out in patients with soft tissue sarcomas for planning adjuvant treatment modalities

A case of gastrointestinal stromal tumor metastasized to the left ventricular myocardium

BackgroundGastrointestinal stromal tumors (GISTs), which are the most common soft tissue tumors of the gastrointestinal tract, originate from Cajal interneurons. The main metastatic sites of GISTs are the liver and intra-abdominal cavity, and metastasis to the heart is rare.Case presentationThe patient was a 78-year-old man who was diagnosed with a rectal GIST 20 years previously. Since then, he had undergone repeated operations for metastasis. A follow-up thoracoabdominal computed tomography scan 4 months prior to the operation revealed GIST metastasis to the left ventricular myocardium. The patient wanted the tumor removed and consequently underwent an operation. The surgical findings showed a 3-cm × 3-cm mass in the lateral wall of the left ventricle. The mass was resected from the left ventricular wall in the shape of a tear drop. The left ventricular cavity was closed with a 4–0 polypropylene mattress suture and continuous suture. Postoperative histopathological findings showed nodular tumor growth consisting of bundles of spindle-shaped cells in the myocardium. The margins were negative. Immunostaining showed c-KIT (CD17) positivity and CD34 positivity, consistent with GIST metastasis.ConclusionsThis case involved GIST metastasis to the heart muscle, which has rarely been reported worldwide.

Deep Learning of Rhabdomyosarcoma Pathology Images for Classification and Survival Outcome Prediction

Rhabdomyosarcoma (RMS), the most common malignant soft tissue tumor in children, has several histologic subtypes that influence treatment and predict patient outcomes. Assistance with histologic classification for pathologists as well as discovery of optimized predictive biomarkers is needed. A convolutional neural network for RMS histology subtype classification was developed using digitized pathology images from 80 patients collected at time of diagnosis. A subsequent embryonal rhabdomyosarcoma (eRMS) prognostic model was also developed in a cohort of 60 eRMS patients. The RMS classification model reached a performance of an area under the receiver operating curve of 0.94 for alveolar rhabdomyosarcoma and an area under the receiver operating curve of 0.92 for eRMS at slide level in the test data set (n=192). The eRMS prognosis model separated the patients into predicted high- and low-risk groups with significantly different event-free survival outcome (likelihood ratio test; P =0.02) in the test data set (n=136). The predicted risk group is significantly associated with patient event-free survival outcome after adjusting for patient age and sex (predicted high- versus low-risk group hazard ratio,4.64; 95% CI,1.05-20.57; P =0.04). This is the first comprehensive study to develop computational algorithms for subtype classification and prognosis prediction for RMS histopathology images. Such models can aid pathology evaluation and provide additional parameters for risk stratification.

Pedunculated Vulvar Hemangioma: A Rare Case Report

Hemangiomas are one of the most common benign soft tissue tumors. Vascular tumors of the female genitalia are unusual and pedunculated vulvar hemangiomas are extremely rare .Usually they are asymptomatic and quite small in size. Here we report a rare case of pedunculated vulvar hemangioma in a 46 year old patient who presented in the gynecology department with a painless mass in vulva for duration of 2 years.

Sub-Clavicular Hibernoma: A Rare Diagnosis of Lipomatous Tumor

Lipomatous tumors are the most common soft tissue tumors, including a large variety of benign and malignant lesions. Hibernoma is a benign lipomatous tumor originating from the brown adipose tissue inherited from the fetus. The diagnosis is often incidental, since the large majority are asymptomatic or very slow growing. Differential diagnosis with other lipomatous tumors is often challenging. Hence the diagnostic work-up is large and must be multidisciplinary. Biopsy and large resection with sane margins are the standard of care. We describe a 48-year-old male patient with a history of a painless, mobile, slow growing right sub-clavicular mass apparently evolving for eleven years. This patient underwent ultrasound, mammography, MRI, CT Scan and ultrasound guided large core biopsy at different points in time. We describe this case of a well-documented hibernoma of the sub-clavicular region in line with the current literature.

ALTERNATE TECHNIQUE IN THE EXCISION OF AN ORAL MUCOCELE: A CASE SERIES

Mucocele is the most common benign soft tissue tumour occurring in the oral cavity. Even though, it is the most common minor salivary gland lesion with good prognosis following its removal, its recurrence rate is just not rare, but bothersome. Its recurrence rate was signicantly differed according to the location of the lesion and age of the individual. Various surgical techniques were proposed in literature and each of them found to have limitations. In this report, we presented a case series of three patients with oral mucocele on lower lip and we also reported a technique, that involved use of alginate impression material to clearly dene the margins of the lesion into adjacent submucosal tissues for better access of the lesion. This technique is found to be effective, safe and economical way of treating a mucocele, ensuring complete removal and minimal chance of a recurrence.

Giant lipoma over the back

Lipoma is one of the most common soft tissue tumor arising from the mesenchyme. It is slow growing, encapsulated, and usually benign in nature. Tumors over the back, shoulder, and neck region have a high propensity to assume large size thereby getting redefined as a giant lipoma when they exceed 10 cm in width or weigh more than 1000 grams. MRI is the investigation of choice for evaluating giant lipomas. Fine needle aspiration cytology (FNAC) or frozen section may be pertinent in suspected cases of liposarcoma. Complete surgical incision is the treatment of choice. A case of a giant lipoma on the back of a 64-year-old lady is presented with a view to revisit conceptual understanding of the clinical evaluation, investigation, and management of giant lipomas.

NanoString Digital Molecular Profiling of Protein and microRNA in Rhabdomyosarcoma.

Simple SummaryNanoString digital profiling methods are novel techniques to identify biologic markers from human formalin-fixed, paraffin-embedded cancer tissue. We have applied NanoString Digital spatial profiling and microRNA profiling methods in non-alveolar rhabdomyosarcoma, a common soft tissue tumor in young adults and children with variable prognosis. Our results have highlighted aberrant miRNA expression and over-expression of several members of PI3-AKT, MAPK and apoptosis signaling pathways in fusion-negative rhabdomyosarcoma, particularly in tumors with unfavorable prognosis. INPP4B, an entry molecule in the PI3/AKT pathway, was significantly over-expressed in tumors with poor prognosis, confirmed by traditional immunohistochemistry. Several microRNAs had increased expression in association with poor patient prognosis. These results highlight the utility of NanoString digital profiling as a screening method to identify prognostic biomarkers of interest in rhabdomyosarcoma from formalin-fixed paraffin-embedded tissue.Purpose: Rhabdomyosarcoma (RMS) exhibits a complex prognostic algorithm based on histologic, biologic and clinical parameters. The embryonal (ERMS) and spindle cell-sclerosing RMS (SRMS) histologic subtypes warrant further studies due to their heterogenous genetic background and variable clinical behavior. NanoString digital profiling methods have been previously highlighted as robust novel methods to detect protein and microRNA expression in several cancers but not in RMS. Methods/Patients: To identify prognostic biomarkers, we categorized 12 ERMS and SRMS tumor cases into adverse (n = 5) or favorable (n = 7) prognosis groups and analyzed their signaling pathways and microRNA profiles. The digital spatial profiling of protein and microRNA analysis was performed on formalin-fixed, paraffin-embedded (FFPE) tumor tissue using NanoString technology. Results: The detectable expression of several component members of the PI3K/AKT, MAPK and apoptosis signaling pathways was highlighted in RMS, including INPP4B, Pan-AKT, MET, Pan-RAS, EGFR, phospho-p90 RSK, p44/42 ERK1/2, BAD, BCL-XL, cleaved caspase-9, NF1, PARP and p53. Compared to cases with favorable prognosis, the adverse-prognosis tumor samples had significantly increased expression of INPP4B, which was confirmed with traditional immunohistochemistry. The analysis of microRNA profiles revealed that, out of 798 microRNAs assessed, 228 were overexpressed and 134 downregulated in the adverse prognosis group. Significant over-expression of oncogenic/tumor suppressor miR-3144-3p, miR-612, miR-302d-3p, miR-421, miR-548ar-5p and miR-548y (p < 0.05) was noted in the adverse prognosis group. Conclusion: This study highlights the utility of NanoString digital profiling methods in RMS, where it can detect distinct molecular signatures with the expression of signaling pathways and microRNAs from FFPE tumor tissue that may help identify prognostic biomarkers of interest. The overexpression of INPP4B and miR-3144-3p, miR-612, miR-302d-3p, miR-421, miR-548y and miR-548ar-5p may be associated with worse overall survival in ERMS and SRMS.

Differential diagnosis of lipoma and atypical lipomatous tumor/well-differentiated liposarcoma by cytological analysis.

BackgroundAdipocytic tumors are the most common soft tissue tumors, with lipomas and atypical lipomatous tumor/well‐differentiated liposarcomas (ALT/WDL), which comprise most cases. Preoperative differential diagnosis of lipoma or ALT/WDL can provide important information for decisions regarding treatment. We evaluated the cytological findings of 20 cases of lipoma and ALT/WDL.MethodsFluorescence in situ hybridization (FISH) was performed on formalin‐fixed paraffin‐embedded specimens (FFPE) to examine mouse double minute 2 homolog (MDM2) amplification in all cases. Tissue samples were collected from the center of the surgical materials, stained with Pap, and evaluated for 12 cytological parameters by six cytotechnologists.ResultsThe findings regarding large atypical cells, multinucleated cells, and nuclear pleomorphism were highly concordant among the cytotechnologists and were associated with MDM2 amplification. Large atypical cells, considered a highly specific feature of ALT/WDL, were not observed in lipoma cases. However, the sensitivity of the large atypical cell findings was not high (67%); therefore, comprehensive evaluation of multinucleated cells and pleomorphism is crucial for predicting ALT/WDL diagnosis. FISH of MDM2 on Pap‐stained specimens was performed in four cases. In two, the results were similar to those of MDM2 FISH performed on FFPE sections and were reproducible, whereas in the other two, the signal could not be evaluated because of the strong background coloration.ConclusionsCytology specimens may be useful for the preoperative diagnosis of adipocytic tumors, particularly if the FISH conditions for Pap‐stained specimens and the detection accuracy of MDM2 amplification can be improved.

Paratesticular Rhabdomyosarcoma in a 30 Months Old Child at the Lagoon Mother and Child Teaching Hospital (CHUMEL) in Cotonou

Introduction: Rhabdomyosarcoma is the most common soft tissue tumor in children. Modern multidisciplinary approaches make it possible to better characterize the different entities and to adapt the treatment accordingly. Paratesticular localization is rare and aggressive. We report here a case of paratesticular localization in a 30 months old boy. Observation: The parents would have noticed a small, painless and rapidly evolving testicular mass in the 6 months old child. Not having health insurance, they went to the hospital only 5 months later. This was followed by a total ablation of the mass (without orchidectomy) and a pleiomorphic rhabdomyosarcoma had been concluded. No chemotherapy was performed and the mass recurred 2 months later. This time, its ablation was followed by a series of non-adapted chemotherapy sessions with irregular follow-ups leading 8 months later to the child’s admission at our department. He presented with a degraded general condition, associating an infectious and anemic syndrome, an important increase of the scrotal mass and the occurrence of a tumoral mass in the left iliac fossa. The tumoral nature of these masses was confirmed on abdominal ultrasound and scan. The tumor was classified stage II (TNM: initial Tumors, Nodes, Metastasis). The multidisciplinary medical staff indicated a total ablation of the two masses, followed by a new session of adapted chemotherapy, in the absence of radiotherapy means. The child died in the immediate surgery follow-ups due to cardiac arrest. Conclusion: In our context of insufficient technical facilities, only early detection and adapted imperative chemotherapy, would have enabled a durable remission in front of the paratesticular rhabdomyosarcoma.

Giant Preperitoneal Lipoma: An Unusual Case Report with Literature Review

Lipomas are the most common and benign soft tissue neoplasms that can occur throughout the body. Intraabdominal lipomas, especially those located in the preperitoneal region, are very rarely detected. These lipomas can be manifested with discomfort or bloating, pain or palpable mass in the abdomen, and many of them can be detected incidentally during a radiological examination or surgery for a different reason. Surgical resection is necessary only in cases of pain, cosmetic reasons, rapid growth or uncertainty in the diagnosis. Lipomas such as mesentery, omentum and retroperitoneum are very rare in the abdominal cavity. The number of reported cases of parietal peritoneum is 7 so far. Lipomas can reach large volumes very rarely. In the literature, only one excessively large lipoma case was reported. In this case report, the diagnosis and treatment of a very rare giant preperitoneal lipoma detected in a female patient is presented with literature review.

Primary Dedifferentiated Liposarcoma of the Colon - Report of a Rare Case with Review of Literature

Liposarcoma is the most common malignant soft tissue tumor in adults. Extremities and retroperitoneum are the common sites of involvement of liposarcoma. Liposarcoma of the gastrointestinal tract is rare and the colon is an extremely uncommon site. Mesenchymal tumors of the bowel are mainly represented by gastrointestinal stromal tumors (GIST). A sixty-one-year-old male patient presented with abdominal pain and distension. Contrast-enhanced computerized tomography (CECT) showed a circumferential

Pedunculated Intraperitoneal Lipoma Presenting as Ovarian Torsion: A Case Report

A lipoma is a very common (prevalence 39%) benign soft tissue tumour that is composed of mature fat. Lipomas, though common on external surface of body, also have been reported in mediastinum, chest wall, thorax, retroperitoneal areas and pelvis. In Gastrointestinal Tract (GIT), lipomas are mainly found in the submucosa and subserosa of the small and large intestine. An eightyear-old female child presented with a feature suggesting torsion of the dermoid ovarian cyst. The clinical features included sudden abdominal pain, vomiting and other symptoms suggesting a differential diagnosis of appendicitis or an ovarian cause. Serum markers for carcinoma were normal. The Contrast Enhanced Computed Tomography (CECT) showed a right iliac fossa fat density with few internal septations and Magnetic Resonance Imaging (MRI) confirmed the torsion of the pedunculated mass and histopathological examination showed the mass as lipoma. The site of the lipoma, presented in this case, is rare. Though the presentation of intraperitoneal lipoma is rare, this should be considered as differentials in young patients presenting with features of ovarian torsion. The presentation of intraperitoneal lipoma especially in young patient is rare which is mostly an incidental finding in a laparotomy and mostly present as a case of obstruction or abdominal pain.

The Effect of Direct and Indirect EZH2 Inhibition in Rhabdomyosarcoma Cell Lines.

Simple SummaryRhabdomyosarcoma is the most common soft tissue tumor in children. Its two major subtypes show epigenetic alterations that are associated with poor prognosis. Therefore, targeting these epigenetic alterations by pharmacological intervention could be a therapeutic approach. We investigated two different types of substances that interfere with the epigenetic process of histone methylation. We performed studies in two cell lines that carry characteristics of the major rhabdomyosarcoma subtypes. The aim of this study was to find out if the substances differ in their effect on tumor-related cellular functions and to find out if the tumor subtypes differ in their response to the substances. These findings may contribute to a better assessment of the feasibility of pharmacological intervention directed against histone methylation in subtypes of rhabdomyosarcoma.Enhancer of Zeste homolog 2 (EZH2) is involved in epigenetic regulation of gene transcription by catalyzing trimethylation of histone 3 at lysine 27. In rhabdomyosarcoma (RMS), increased EZH2 protein levels are associated with poor prognosis and increased metastatic potential, suggesting EZH2 as a therapeutic target. The inhibition of EZH2 can be achieved by direct inhibition which targets only the enzyme activity or by indirect inhibition which also affects activities of other methyltransferases and reduces EZH2 protein abundance. We assessed the direct inhibition of EZH2 by EPZ005687 and the indirect inhibition by 3-deazaneplanocin (DZNep) and adenosine dialdehyde (AdOx) in the embryonal RD and the alveolar RH30 RMS cell line. EPZ005687 was more effective in reducing the cell viability and colony formation, in promoting apoptosis induction, and in arresting cells in the G1 phase of the cell cycle than the indirect inhibitors. DZNep was more effective in decreasing spheroid viability and size in both cell lines than EPZ005687 and AdOx. Both types of inhibitors reduced cell migration of RH30 cells but not of RD cells. The results show that direct and indirect inhibition of EZH2 affect cellular functions differently. The alveolar cell line RH30 is more sensitive to epigenetic intervention than the embryonal cell line RD.

Development and validation of a novel diagnostic model for initially clinical diagnosed gastrointestinal stromal tumors using an extreme gradient-boosting machine

IntroductionGastrointestinal stromal tumor (GIST) is the most common gastrointestinal soft tissue tumor. Clinical diagnosis mainly relies on enhanced CT, endoscopy and endoscopic ultrasound (EUS), but the misdiagnosis rate is still high without fine needle aspiration biopsy. We aim to develop a novel diagnostic model by analyzing the preoperative data of the patients.MethodsWe used the data of patients who were initially diagnosed as gastric GIST and underwent partial gastrectomy. The patients were randomly divided into training dataset and test dataset at a ratio of 3 to 1. After pre-experimental screening, max depth = 2, eta = 0.1, gamma = 0.5, and nrounds = 200 were defined as the best parameters, and in this way we developed the initial extreme gradient-boosting (XGBoost) model. Based on the importance of the features in the initial model, we improved the model by excluding the hematological features. In this way we obtained the final XGBoost model and underwent validation using the test dataset.ResultsIn the initial XGBoost model, we found that the hematological indicators (including inflammation and nutritional indicators) examined before the surgery had little effect on the outcome, so we subsequently excluded the hematological indicators. Similarly, we also screened the features from enhanced CT and ultrasound gastroscopy, and finally determined the 6 most important predictors for GIST diagnosis, including the ratio of long and short diameter under CT, the CT value of the tumor, the enhancement of the tumor in arterial period and venous period, existence of liquid area and calcific area inside the tumor under EUS. Round or round-like tumors with a CT value of around 30 (25–37) and delayed enhancement, as well as liquid but not calcific area inside the tumor best indicate the diagnosis of GIST.ConclusionsWe developed a model to further differential diagnose GIST from other tumors in initially clinical diagnosed gastric GIST patients by analyzing the results of clinical examinations that most patients should have completed before surgical resection.

GIANT CELL TUMOUR OF THE TENDON SHEATH OF THE THUMB – A CASE REPORT

Giant cell tumor (GCT) of the tendon sheath is the second most common benign soft tissue tumor of the hand. Magnetic resonance imaging (MRI) is the investigative tool of choice for both diagnosis and treatment planning. The current standard treatment of choice is excision or amputation of the involved part as the recurrence rates are very high. Hence, it is necessary that the surgeon ensures complete removal of the tumor and removal of residual satellite nodules if any. There are many hypotheses proposed regarding the etiology of these lesions, but still the opinions are varied.

Multiple symmetric and multiple familial lipomatosis

Lipomas are the most common soft tissue tumors and are malignant in only 1% of cases. Lipomatosis is defined as the presence of multiple benign lipomas on the body, without lipoatrophy. Their impact on quality of life is significant. Different entities have been described such as symmetrical multiple lipomatosis (MSL), also called Madelung's disease and familial multiple lipomatosis (FML). MSL occurs preferentially in men (but also women) who are alcohol abuser. There are different subtypes of the disease, the most classic of which affects the upper body and the nuchal region with a buffalo hump appearance. A metabolic component with obesity is frequent. In contrast to Dercum's disease, there is no pain. SAOS, complications of the metabolic syndrome and of alcohol abuse including cancers, may be associated and should be screened. FML has been little described in the literature since Brodie's first report in 1846. FML occurs preferentially in the third decade but equally in women and men. Its autosomal dominant component is classically accepted with variable penetrance within the same family. Association with naevi, angiomas, polyneuropathies and with gastrointestinal comorbidities has been reported. Interestingly, and in contrast with most lipodystrophy disorders, the patients show an insulin sensitivity profile. A better understanding of the underlying pathophysiological mechanisms would open up avenues on therapeutic research, since treatments are only symptomatic to date.

Total Laparoscopic Hysterectomy without Mini-Laparotomy for a 7060g Myomatous Uterus with Cystic Degeneration.

<h3>Study Objective</h3> Uterine leiomyomas are the most common benign gynecological soft tissue tumors in women. Although the laparoscopic approach is a standard treatment option, it is more difficult to achieve minimally invasive hysterectomy in cases with giant myomatous uteri. The laparoscopic removal of a myomatous uterus over 7000g without mini-laparotomy has not been reported in literature. Here we report the surgical challenges in the case of a patient with a 7060g myomatous uterus with cystic degeneration. <h3>Design</h3> Stepwise demonstration of the technique with narrated video footage. <h3>Setting</h3> An urban general hospital. <h3>Patients or Participants</h3> A 54-year-old woman was referred to our department for treatment for a huge fibromatous uterus. She suffered pelvic pressure and abdominal distension for several months. A magnetic resonance imaging scan showed a cystic-degenerated fibromatous uterus measuring 27.5cm × 21.3cm × 29.5cm with no suggestion of malignancy. <h3>Interventions</h3> We performed a laparoscopic hysterectomy and bilateral salpingo-oophorectomy. Intraoperatively, we aspirated the contents of the degenerative cyst of the uterus for mass reduction, and then closed the cyst hole by suturing to prevent leakage of the contents. This was followed by our usual surgical steps. We removed the specimen transvaginally. The total weight of the uterus removed was 7060g, including 5300g of cyst contents. The operation lasted for 79 minutes, and the intraoperative blood loss was 50g. No intraoperative complications occurred. <h3>Measurements and Main Results</h3> The histology confirmed the diagnosis of leiomyoma. The patient's postoperative course was unremarkable with no complications. <h3>Conclusion</h3> Uterus size has been considered a barrier to the use of laparoscopy for hysterectomy. We believe this is the first reported laparoscopic hysterectomy without mini-laparotomy in a patient with a giant uterus, over 7000g. Though appropriate case selection is necessary, this case demonstrates the efficiency and safety of laparoscopic hysterectomy for giant myomatous uterus cases.

Synovial Sarcoma in Children, Adolescents, and Young Adults: A Report From the Children's Oncology Group ARST0332 Study.

Synovial sarcoma (SS) is the second most common malignant soft tissue tumor in children. ARST0332 evaluated a risk-based treatment strategy for young patients with soft tissue sarcoma designed to limit therapy for low-risk (LR) disease and to test neoadjuvant chemoradiotherapy for unresected higher-risk disease. Newly diagnosed patients with SS age < 30 years were assigned to four treatment arms based on disease features: A (surgery only), B (55.8 Gy radiotherapy [RT]), C (ifosfamide and doxorubicin [ID] chemotherapy plus 55.8 Gy RT), and D (neoadjuvant ID and 45 Gy RT, then surgery and RT boost based on margins followed by adjuvant ID). Patients treated in Arms A and B were considered LR, arms C and D without metastases as intermediate-risk (IR), and those with metastases as high-risk (HR). Of the 146 patients with SS enrolled, 138 were eligible and evaluable: LR (46), IR (71), and HR (21). Tumors were 80% extremity, 70% > 5 cm, 70% high-grade, 62% invasive, 95% deep, and 15% metastatic. Treatment was on arm A (29.7%), B (3.6%), C (16.7%), and D (50%). There were no toxic deaths and four unexpected grade 4 adverse events. By risk group, at a median follow-up of 6.8 years, estimated 5-year event-free survival was LR 82%, IR 70%, and HR 8%, and overall survival was LR 98%, IR 89%, and HR 13%. After accounting for the features that defined risk category, none of the other patient or disease characteristics (age, sex, tumor site, tumor invasiveness, and depth) improved the risk stratification model. The risk-based treatment strategy used in ARST0332 produced favorable outcomes in patients with nonmetastatic SS relative to historical controls despite using RT less frequently and at lower doses. The outcome for metastatic SS remains unsatisfactory and new therapies are urgently needed.