Intra-abdominal Tumor Research Articles

Desmoplastic small round cell tumor a case report of a rare intra-abdominal tumor of the young adult

Desmoplastic small round cell tumor is a rare and highly aggressive mesenchymal tumor described as a distinct clinico-pathological entity in 1989 by Gerald . These tumors occur mainly in the peritoneal cavity,although other primary sites, such as testicular, ovarian, thoracic, pulmonary, intracranial and head and neck regions have been reported. Clinical symptoms are non-specific. Diagnosis is based on histological analysis combined with immunohistochemical study. Histological study typically shows small round blue cells in nests separated by abundant desmoplastic stroma associated with a single chromosomal translocation t(11:22) (p 13; q 12) involving the EWSR1 and WT1 genes. The prognosis is particularly poor, with median survival ranging from 17 to 25 months. Management of this tumor remains difficult, and current regimens do not achieve a significant cure rate despite the use of aggressive treatments such as polychemotherapy.

Anaesthesia for Laparoscopic Abdominal Surgery in a Morbidly Obese Female with Hypothyroidism and Hypertension: A Case Report

Obesity is characterised by a body weight that surpasses the expected or ideal weight by over 10%, considering factors such as height, age, body build, and sex. Another way to define obesity is based on body fat content: a male is considered obese if body fat exceeds 25% of his total weight, while a female is considered obese if body fat exceeds 30%. Abdominal obesity, prevalent in 40% of women in India, poses significant challenges in the realm of anaesthesia, particularly during laparoscopic abdominal surgeries. The co-existence of morbid obesity, hypothyroidism, and hypertension present intricate medical conditions that demand precise management. During the surgical procedure, issues related to large intraabdominal tumours and airway maintenance impact respiratory and circulatory dynamics. Notably, intraoperative challenges, including bronchospasm, hypotension, and respiratory acidosis, were addressed to ensure the patient's well-being and a successful surgical outcome. The present case report provides insights into the comprehensive management of high-risk patients undergoing laparoscopic abdominal surgery. It underscores the significance of tailored anaesthesia strategies to mitigate complications and ensure a favourable postoperative recovery. This report presents the case of a 59-year-old female with obesity (BMI: 39.6 kg/m2 ), hypothyroidism, and hypertension who underwent laparoscopic abdominal surgery and discusses managing intraoperative challenges, such as bronchospasm, hypotension, and respiratory acidosis, and the postoperative care for successful patient recovery

Long-Term Outcomes After Abdominal Radiation for Wilms Tumor: A 20-Year Experience.

As radiation therapy (RT) for Wilms tumor (WT) evolves with more conformal techniques, it is necessary to evaluate patterns of failure and toxicity. We sought to determine the rate of local failure (LF) after abdominal RT in WT, specifically focusing on those with contained rupture treated with whole abdominal and pelvic RT (WAPRT) vs flank RT. Secondary objectives were to determine overall survival (OS), distant failure (DF), and late toxicities. A single institution retrospective study of 54 pediatric patients with WT treated with abdominal RT between May 2000 and October 2022. LF and DF were calculated through cumulative incidence function and OS by Kaplan-Meier method. The median age was 4.5 years and the median follow-up was 6 years. Most patients (91%) had favorable histology. Only 1 patient experienced LF, 15 months from completion of RT (cumulative incidence 2% at 5y). All patients who received unilateral flank radiation for contained rupture/spillage (n=13) experienced long-lasting intra-abdominal tumor control. A total of 5 patients experienced a DF at a median of 7 months, all in the lung. No patient relapsed in the lungs after upfront whole lung irradiation (n=16). OS was 96% at 5 years. Among 28 patients who followed through puberty, 4 female patients with prior WAPRT experienced hormonal irregularities/infertility. Unilateral flank radiation may be a viable alternative to WAPRT for contained rupture/spillage and should be further explored prospectively. Our results may also be utilized in the future for outcome and toxicity comparison as conformal radiation techniques evolve.

Abdominal wall mass resections: single-center closure practices and outcomes following oncologic resections of abdominal wall fascia.

Radical resections for abdominal wall tumors are rare, thus yielding limited data on reconstruction of defects. We describe surgical management and long-term outcomes following radical tumor resection. This was a single-center retrospective review of patients between January 2010 and December 2022. Variables included operative characteristics, wound complications, hernia development, tumor recurrence, and reoperation. A multivariable analysis compared wound morbidity for suture and mesh repairs while adjusting for defect width, fascial closure, and CDC wound class. 120 patients were identified. Mean follow-up was 3.9 ± 3.4years. Seventy-five (62.5%) of the masses were primary; most commonly desmoid (n = 25) and endometrioma (n = 27). Forty-five masses were metastases. Mean tumor width was 6.2 ± 3.4cm; mean defect width was 8.1 ± 4.1cm. Sixty-one patients (50.8%) had mesh placed, with variation in technique. Postoperative CT scans were available for 88 (73.3%) patients. Forty SSOs (33.3%), 11 SSIs (9.2%), and 18 (15%) SSOPIs occurred within 30 days. On multivariable analysis, increased defect width was associated with SSOPI (OR 1.17, p = 0.041) and CDC wound class II-III was associated with SSI (OR 8.38 and 49.1, p < 0.05) and SSOPI (OR 5.77 and 17.4, p < 0.05); mesh was not associated with these outcomes. Seven patients (5.8%) underwent 30-day reoperations and 35 (20.8%) required additional operations after 30 days. Thirteen percent developed abdominal wall (n = 8) or intra-abdominal tumor recurrence (n = 8) requiring reoperation. Twenty-seven (22.5%) patients developed hernias with a mean fascial defect width of 9.8 ± 7.2cm. Abdominal wall mass resections are morbid, often contaminated cases with high postoperative complication rates. Risks and benefits of mesh implantation should be tailored on an individual basis.

A Case of Intraabdominal Desmoid Tumor Requiring Differentiation from Regional Lymph Node Recurrence after Gastrectomy for Gastric Cancer

A Case of Intraabdominal Desmoid Tumor Requiring Differentiation from Regional Lymph Node Recurrence after Gastrectomy for Gastric Cancer

Chasing the uncommon: Intra-Abdominal Testicular Tumors case report

Undescended testicles, a common congenital anomaly, can increase the risk of developing testicular tumors. This case report presents the unique occurrence of an intra-abdominal testicular tumor in a 30-year-old male with a history of cryptorchidism. The patient experienced persistent hypogastric pain, and diagnostic evaluation revealed elevated tumor markers, further indicating the presence of a testicular tumor. Surgical exploration confirmed a 256g differentiated seminoma within the peritoneal cavity. The discussion highlights the increased risk of malignancy associated with cryptorchidism, the limited preventive impact of orchidopexy, and the challenges in early diagnosis due to the non-palpable nature of these tumors. It emphasizes the need for awareness, early diagnosis, and ongoing research to improve the management of intra-abdominal testicular tumors in individuals with cryptorchidism and enhance patient outcomes.

Pediatric Patient with Omental Cyst: A Rare Case (CT scan is not Enough to Differentiate from Other Abdominal Cysts)

Background. Omental cysts are rare intra-abdominal tumors in children. It is challenging to diagnose since other big abdominal cysts are difficult to distinguish from in clinical and CT imaging. Aim. This case report aims to accurately and thoroughly determine the diagnosis based on the anamnesis, clinical and imaging examinations to choose the best treatment for the patient. Case Report. A 4-year-old girl reported having an enlarged belly in the past two months; the anamnesis provided the diagnosis. A mobile, 15 cm by 15 cm mass with dull percussion was discovered during clinical investigations in the top border. It was determined by anamnesis, physical examination, and imaging that the diagnosis was an ovarian cyst. Instead of an ovarian cyst, we found an omental cyst during the operation, thus we had to conduct an excision and omentectomy and postoperatively achieved favorable results. She was discharged three day after. Discussion. An abdominal CT scan showed an ovarian cyst. In laparotomy, we found a giant omental cyst, not originating from the ovaries, and did an omentectomy to excision the cyst. CT scan shows that the giant abdominal cyst is difficult to distinguish between omental, mesenteric, and ovarian cysts. Precise identification of stomach cysts is necessary. Also, doctors need to read the CT scan image more carefully. Conclusion. Giant omentum cysts are difficult to distinguish clinically and imaging from ovarian and mesenteric cysts. There is a need for a more detailed history, physical examination, and support, as well as a more thorough reading of the CT scan.

Online Adaptive MRI-Guided Stereotactic Body Radiotherapy for Pancreatic and Other Intra-Abdominal Cancers.

A 1.5T MRI combined with a linear accelerator (Unity®, Elekta; Stockholm, Sweden) is a device that shows promise in MRI-guided stereotactic body radiation treatment (SBRT). Previous studies utilized the manufacturer's pre-set MRI sequences (i.e., T2 Weighted (T2W)), which limited the visualization of pancreatic and intra-abdominal tumors and organs at risk (OAR). Here, a T1 Weighted (T1W) sequence was utilized to improve the visualization of tumors and OAR for online adapted-to-position (ATP) and adapted-to-shape (ATS) during MRI-guided SBRT. Twenty-six patients, 19 with pancreatic and 7 with intra-abdominal cancers, underwent CT and MRI simulations for SBRT planning before being treated with multi-fractionated MRI-guided SBRT. The boundary of tumors and OAR was more clearly seen on T1W image sets, resulting in fast and accurate contouring during online ATP/ATS planning. Plan quality in 26 patients was dependent on OAR proximity to the target tumor and achieved 96 ± 5% and 92 ± 9% in gross tumor volume D90% and planning target volume D90%. We utilized T1W imaging (about 120 s) to shorten imaging time by 67% compared to T2W imaging (about 360 s) and improve tumor visualization, minimizing target/OAR delineation uncertainty and the treatment margin for sparing OAR. The average time-consumption of MRI-guided SBRT for the first 21 patients was 55 ± 15 min for ATP and 79 ± 20 min for ATS.

Cancer-associated splanchnic vein thrombosis: Clinical features upon diagnosis and short-term outcomes

IntroductionThe incidence of splanchnic vein thrombosis (SVT) in cancer patients has increased in recent years and its real clinical significance and management can be challenging. This study aimed to describe the clinical presentation and short-term outcomes of patients with cancer-associated SVT. Material and methodsThis was a retrospective observational study of consecutive patients with cancer-associated SVT diagnosed during the period 2015–2020. The primary objective was to describe the clinical presentation of SVT. Patients were clinically classified into two groups based on the presence of symptoms on SVT diagnosis. The main outcomes were overall and SVT-related mortality, major and non-major bleeding rates, and the thrombosis recurrence rate in the first 30 days of follow-up. ResultsThis study enrolled 203 patients. Intra-abdominal tumors (76 %) and metastatic disease (68 %) predominated. A total of 79 (39 %) patients without symptoms were diagnosed with SVT during a scheduled radiological test and were classified as “asymptomatic”, while 124 (61 %) patients presented some potential SVT symptoms and were considered as “symptomatic”. Although the 30-day outcomes showed no significant differences between the two groups, mortality in the asymptomatic group was slightly lower compared to the symptomatic group (3 % vs. 10 %, p = 0.085). ConclusionsAlmost 40 % of cases of cancer-associated SVT are asymptomatic. There were no significant differences in short-term outcomes between the symptomatic and asymptomatic patients. More studies are required to better define long-term management and outcomes in these patients.

836 Aetiology and Management Outcomes of Adult Mechanical Bowel Obstruction in Nigeria: A Systematic Review and Meta-analysis

Abstract Aim Adult mechanical bowel obstruction (AMBO) has been previously reported to be majorly caused by hernias in developing countries. In Nigeria, however, there has been a recent change in pattern with adhesions now being the leading cause. The changing pattern of the causes, clinical features, mode of management and factors influencing the outcome of adult patients managed for bowel obstruction in Nigeria necessitated this review. Method Relevant keywords relating to AMBO were used to conduct a search on PubMed, Google Scholar and AJOL. The search returned 452 articles which were subjected to title, abstract and full text screening, according to the inclusion and exclusion criteria. 10 articles met the inclusion criteria and were included in the final qualitative synthesis. Results The total sample size across the 10 studies was 1033. Adhesions, hernias and intra-abdominal tumours, which are responsible for 46.25%, 26.31% and 12.23% of cases respectively, were the major causes of AMBO. 65.6% of cases were managed operatively and 34.4% were managed conservatively. The meta-analysis revealed high morbidity and mortality rates of 31% (95% CI:17, 44) and 11% (95% CI: 6, 15), respectively, among adult patients managed for mechanical bowel obstruction in Nigeria. Conclusions Adhesion, which results majorly from appendicectomy is the most common cause of AMBO in Nigeria. This is unlike former reports where hernia was the most common cause. Morbidity results majorly from wound infection, recurrent adhesions and post-operative enterocutaneous fistula. The mortality rate is similar to reports from various West African studies, and it is significantly influenced by surgical intervention time.

870 Benign Cystic Mesothelioma- a Rare Intra-Abdominal Tumour

Abstract Introduction Benign cystic mesothelioma is a rare intra-abdominal tumour affects primarily women who are in reproductive. Since this pathology has a rare occurrence, a similar presentation, and similar imaging features, the diagnosis is based on histologic findings and there is no current standard treatment for this condition. Presentation of case In this case report, a 40-year-old female patient presented in the Emergency Department with a five-day history of right-sided, migratory, abdominal pain. She was systemically well, and her inflammatory markers were unremarkable. The patient was investigated for appendicitis, with a CT scan showing a multi-loculated cystic structure below the liver. Her case was discussed with the Basingstoke Peritoneal Malignancy Institute who felt this may demonstrate benign cystic peritoneal mesothelioma. As per their advice, and the thoughts of the local MDT team, the patient underwent a diagnostic laparoscopy, and the cystic mass was removed. The diagnosis of benign cystic peritoneal mesothelioma was then confirmed by histology. Discussion Based on literature, this is a rare and asymptomatic form of mesothelioma.Imaging is able to provide suspicion of this type of benign mesothelium, but only histological examination can provide a definitive diagnosis. Additionally, on review there are no guidelines regarding management and no standard treatment for this condition. The only effective treatment for a benign cystic mesothelioma is surgery, but no evidence-based strategies exist. Conclusions It is critical to publish more case reports on this condition once it is diagnosed in order to formulate guidelines and to advance towards a possible effective treatment solution.

Physiologic tension of the abdominal wall.

Tension-free abdominal closure is a primary tenet of laparotomy. But this concept neglects the baseline tension of the abdominal wall. Ideally, abdominal closure should be tailored to restore native physiologic tension. We sought to quantify the tension needed to re-establish the linea alba in patients undergoing exploratory laparotomy. Patients without ventral hernias undergoing laparotomy at a single institution were enrolled from December 2021 to September 2022. Patients who had undergone prior laparotomy were included. Exclusion criteria included prior incisional hernia repair, presence of an ostomy, large-volume ascites, and large intra-abdominal tumors. After laparotomy, a sterilizable tensiometer measured the quantitative tension needed to bring the fascial edge to the midline. Outcomes included the force needed to bring the fascial edge to the midline and the association of BMI, incision length, and prior lateral incisions on abdominal wall tension. This study included 86 patients, for a total of 172 measurements (right and left for each patient). Median patient BMI was 26.4kg/m2 (IQR 22.9;31.5), and median incision length was 17.0cm (IQR 14;20). Mean tension needed to bring the myofascial edge to the midline was 0.97lbs. (SD 1.03). Mixed-effect multivariable regression modeling found that increasing BMI and greater incision length were associated with higher abdominal wall tension (coefficient 0.04, 95% CI [0.01,0.07]; p = 0.004, coefficient 0.04, 95% CI [0.01,0.07]; p = 0.006, respectively). In patients undergoing laparotomy, the tension needed to re-establish the linea alba is approximately 1.94lbs. A quantitative understanding of baseline abdominal wall tension may help surgeons tailor abdominal closure in complex scenarios, including ventral hernia repairs and open or burst abdomens.

SP8.3 Aetiology and Management Outcomes of Adult Mechanical Bowel Obstruction in Nigeria: A Systematic Review and Meta-analysis

Abstract Aim Adult mechanical bowel obstruction (AMBO) has been previously reported to be majorly caused by hernias in developing countries. In Nigeria, however, there has been a recent change in pattern with adhesions now being the leading cause. The changing pattern of the causes, clinical features, mode of management and factors influencing the outcome of adult patients managed for bowel obstruction in Nigeria necessitated this review. Method Relevant keywords relating to AMBO were used to conduct a search on PubMed, Google Scholar and AJOL. 452 articles were subjected to title, abstract and full text screening, according to the inclusion and exclusion criteria. 10 articles met the inclusion criteria and were included in the final qualitative synthesis. Results The total sample size across the 10 studies was 1033. Adhesions, hernias and intra-abdominal tumours, responsible for 46.25%, 26.31% and 12.23% of cases, respectively, were the major causes of AMBO. 65.6% of cases were managed operatively and 34.4% were managed conservatively. The meta-analysis revealed high morbidity and mortality rates of 31% (95% CI: 17, 44) and 11% (95% CI: 6, 15), respectively, among adult patients managed for mechanical bowel obstruction in Nigeria. Conclusions Adhesion, which results majorly from appendicectomy is the most common cause of AMBO in Nigeria. This is unlike former reports where hernia was the most common cause. Morbidity results majorly from wound infection, recurrent adhesions and post-operative enterocutaneous fistula. The mortality rate is similar to reports from various West African studies and it is significantly influenced by surgical intervention time.

Solitary Fibrous Tumor in the Preperitoneal Space Mimicking an Intra-Abdominal Tumor

Solitary fibrous tumors (SFTs) are uncommon fibroblastic mesenchymal neoplasms that rarely metastasize. They were primarily considered intrathoracic tumors; however, recent studies have reported SFTs in extrathoracic locations. This report describes a rare case of an SFT in the preperitoneal space that mimicked an intra-abdominal tumor radiographically. A 67-year-old woman was diagnosed with an extrahepatic tumor through ultrasonography. Computed tomography revealed a nodule near the liver's left lobe at the upper abdominal midline. Laparoscopic tumor resection was performed to minimize undersampling and tumor seeding. Laparoscopy revealed a well-circumscribed tumor located in the preperitoneal space. The tumor was resected en bloc with a macroscopically negative margin. Histopathological examinations confirmed an SFT using immunohistochemistry. Adjuvant treatment was not administered. No residual lesions were reported at the 6-month and 1-year follow-ups. Although SFT rarely metastasizes, early diagnosis and treatment of SFTs should be emphasized to ensure optimal patient outcomes.

Mesenteric fibromatosis involving intestine mimicking a gastrointestinal stromal tumor

Mesenteric fibromatosis (MF) or intra-abdominal desmoid tumour is a rare proliferative disease affecting the mesentery. MF is a locally aggressive tumour that lacks metastatic potential, but the local recurrence is common. We report a case of 33-year-old female with chief complaints of mass in abdomen which was hard on palpation arising from the pelvis and extending up to the umbilicus. Magnetic resonance imaging (MRI) suggestive of soft tissue mass and exploration was planned. Intra-operatively a mass of 16×16×8 cm with attached ileum and caecum which was excised completely and sent for histopathological examination. Histopathology was suggestive of Mesenteric fibromatosis spindle cell type. Considering the rarity of this tumour and difficulties in diagnostic and therapeutic ambit, we believe it is justified to publish this case which came to our observation.

136: Intestinal Transplant in patients with multiple FAP complicated by intraabdominal desmoid tumor: A Case Series

136: Intestinal Transplant in patients with multiple FAP complicated by intraabdominal desmoid tumor: A Case Series

GOunder/Desmoid Tumor Research Foundation DEsmoid Symptom/Impact Scale (GODDESS©): psychometric properties and clinically meaningful thresholds as assessed in the Phase 3 DeFi randomized controlled clinical trial

PurposeThe GODDESS© tool was developed to assess Desmoid Tumor/Aggressive Fibromatosis (DT/AF) symptom severity and impact on patients’ lives. This study evaluated GODDESS©’s cross-sectional and longitudinal measurement properties.MethodsThe Phase 3, randomized placebo-controlled, DeFi study (NCT03785964) of nirogacestat in DT/AF was used to assess GODDESS©’s reliability, construct validity, responsiveness, and estimate of meaningful change thresholds (MCTs). Other patient-reported outcome (PRO) measures included Patient Global Impression of Severity (PGIS) in DT/AF symptoms, EORTC QLQ-C30, Brief Pain Inventory Short Form, and PROMIS Physical Function short-form 10a v2.0 plus 3 items.ResultsDeFi participants (N = 142) had a median age of 34 years (range: 18–76) and were mostly female (64.8%), with extra-abdominal (76.8%) or intra-abdominal tumors (23.2%). The GODDESS© symptom/impact scales showed internal consistency at baseline, cycles 4 and 7 (Cronbach’s α > 0.70) and test–retest reliability (intra-class correlation coefficient > 0.85). GODDESS© scales correlated moderately to highly with PRO measures capturing similar content and differentiated among PGIS and Eastern Cooperative Oncology Group groups. GODDESS© scales detected improvement over time. For the total symptom score, a 1.30-point decrease was estimated as the within-person MCT and a 1.00-point decrease as the between-group MCT. For the physical functioning impact score, estimated within- and between-group MCTs were 0.60-point and 0.50-point decreases, respectively. Few participants exhibited symptom worsening.ConclusionGODDESS© was found to be reliable, valid, responsive, and interpretable as a clinical trial endpoint in the pooled sample of DT/AF patients. Estimated MCTs can be used to define responders and assess group-level differences in future, unblinded, efficacy analyses.Trial registration number and registration dateNCT03785964; December 24, 2018.

Pathophysiologic Contributions of Visceral Adiposity to Left Ventricular Diastolic Dysfunction.

Visceral fat produces inflammatory cytokines and may play a major role in heart failure with preserved ejection fraction (HFpEF). However, little data exist regarding how qualitative and quantitative abnormalities of visceral fat would contribute to left ventricular diastolic dysfunction (LVDD). We studied 77 participants who underwent open abdominal surgery for intra-abdominal tumors (LVDD, n = 44; controls without LVDD, n = 33). Visceral fat samples were obtained during the surgery, and mRNA levels of inflammatory cytokines were measured. Visceral and subcutaneous fat areas were measured using abdominal computed tomography. Patients with significant LVDD had greater LV remodeling and worse LVDD than controls. While body weight, body mass index, and subcutaneous fat area were similar in patients with LVDD and controls, the visceral fat area was larger in patients with LVDD than in controls. The visceral fat area was correlated with BNP levels, LV mass index, mitral e' velocity, and E/e' ratio. There were no significant differences in the mRNA expressions of visceral adipose tissue cytokines (IL-2, -6, -8, and -1β, TNFα, CRP, TGFβ, IFNγ, leptin, and adiponectin) between the groups. Our data may suggest the pathophysiological contribution of visceral adiposity to LVDD.

Famitinib for familial adenomatous polyposis-associated aggressive desmoid tumors: A single-center exploratory study.

10513 Background: Desmoid tumour (DT) is a common extra-intestinal manifestation of familial adenomatosis polyposis (FAP), with high risk of recurrence and became the second leading cause of FAP patients’ death. The treatment of FAP-associated DTs are limited. Famitinib is a orally bioavailable, tyrosine kinase inhibitor against VEGFR-2, PDGFR, c-kit, and FGFR, and has shown antitumor activity in these patients (pts). Methods: This is a prospective, single arm study of FAP pts with progressing DT per RECIST v1.1, treated with famitinib 20 mg QD since 11/2021. The primary endpoint was objective response rate (ORR), with secondary endpoints of safety, progression-free survival (PFS), and quality of life (EORTC QLQ C30). We also collected the FFPE slices of DTs to test both genetic and proteomic backgrounds of pts with different therapeutic results. Results: Between Nov 24, 2021 and Aug 8, 2022, 11 pts were enrolled and evaluated every 9 weeks. The median follow-up time was 9.4 m (6.6 to 14.9 m). The median age was 36 (28-53) and 45% were male. Nine pts were diagnosed with intra-abdominal (IA) tumors while two had extra-abdominal (EA) DTs. Eight had stage III-IV DTs with rapid growth and severe symptoms. 10 of them had history of intestinal resection. Two pts were treated with dacarbazine and epirubicin before enrollment, while one patient received radiotherapy before famitinib. Five pts achieved partial response (PR) and six achieved stable disease (SD), with a median time to response of 7.0 months. The ORR was 45.5% and the disease control rate (DCR) was 100%. The 6-month PFS rate was 91%. The ORR of pts with IA DTs was 55.6%. Of adverse events (AEs) with famitinib, 87% were grade 1/2, the most frequently reported being hypertension (73%), neutropenia (64%), bilirubinemia (55%) and proteinuria (36%). Mean global health status/QoL before and after 6 months’ treatment was 58.3 vs 75.8 ( P= 0.09). Of all 11 pts, germline APC mutations scattered from codon 457 to 1578, and five of them detected somatic APC mutations from codon 547 to 1896 in DTs. Correlative study analysis will be presented. Conclusions: Famitinib demonstrated promising efficacy and a favorable safety profile in FAP-associated aggressive DTs.

Intra-abdominal desmoplastic small round cell tumor: The European pediatric Soft tissue sarcoma Study Group (EpSSG) experience.

This study describes the clinical findings of a consecutive series of pediatric and adolescent patients with a diagnosis of intra-abdominal desmoplastic small round cell tumor (DSRCT) prospectively enrolled in European pediatric Soft tissue sarcoma Study Group (EpSSG) protocols: the BERNIE study, the EpSSG MTS 2008 study, and the EpSSG NRSTS 2005 study. Patients aged less than 21years with a diagnosis of DSRCT arising in the abdomen were included. All trials recommended a multimodal approach including intensive multidrug chemotherapy and loco-regional treatment with surgery and/or radiotherapy whenever possible. The analysis included 32 cases (median age 13.7years, male:female ratio 1.5:1). Three patients had localized tumors, seven had regionally disseminated disease, and 22 extraperitoneal metastases. All but one patient received multidrug chemotherapy and 11 had maintenance chemotherapy. Loco-regional treatment consisted of surgery only in seven cases, surgery plus adjuvant radiotherapy in 10, and radiotherapy only in six. Among the 17 cases who had radiotherapy, six had irradiation of the primary site, 10 had whole abdominopelvic radiotherapy plus boost to macroscopic residual disease, and one had irradiation to lung metastases only. With a median follow-up of 76months (range: 18-124months), 5-year event-free and overall survivals were 19.7% and 21.0%, respectively. Event-free survival was significantly worse for patients who did not receive loco-regional treatment (p-value .007). The study confirmed that the outcome of patients with DSRCT remains dismal and did not improve over recent years despite an intensive multimodal treatment approach.