Retroperitoneal liposarcoma : current insights in diagnosis and treatment

Retroperitoneal liposarcoma (RLS) are rare, biologically heterogeneous tumors that present considerable challenges due to their size and deep location. As a consequence, the majority of patients with high grade RLS will develop locally recurrent disease following surgery, and this constitutes the cause of death in most patients. Here, we review current insights and controversies regarding histology, molecular biology, extent of surgery, (neo)adjuvant treatment, and systemic treatment including novel targeted agents in RLS

Not all pediatric intestinal polyps are alike

Background/Aims : In childhood, clinical presentation of intestinal polyps is variable. Painless rectal red blood loss is the most common presenting sign. Most polyps are sporadic, isolated and benign. However, it is important to correctly identity exceptions. Hare inherited polyposis syndromes need to be recognized because of their increased risk of intestinal and extra-intestinal malignancies. Furthermore, a correct diagnosis and treatment of rare gastro-intestinal malignancies is crucial. Methods : Between 2016 and 2018 we encountered 4 different types of intestinal polyps. A database search was performed and patient tiles were checked for clinical manifestations and histopathology. Literature was searched to recapitulate red flags for these syndromes, probability of underlying genetic disorders and diagnostic criteria. Results : Between 2016 and 2018, 28 patients presented at the Ghent University Hospital with 30 juvenile polyps. Furthermore, we diagnosed juvenile polyposis syndrome, Li Fraumeni syndrome and familial adenomatous polyposis (FAP) in 1 patient each, whilst 2 FAP patients were in follow-up. Each of these diagnoses has a different lifetime risk of (extra)-intestinal malignancy and requires a different approach and follow-up. Histopathology and genetic testing play an important role in identifying these syndromes in pediatric patients. Conclusion : Although most intestinal polyps in childhood are benign juvenile polyps that require no follow-up, rare inherited syndromes should be considered and correctly diagnosed since adequate follow-up is necessary to reduce morbidity and mortality from both gastrointestinal and extraintestinal complications and malignancies

Routine surgical pathology in general surgery

Background: Although pathological analysis provides the definitive diagnosis for most resection specimens, recent evidence suggests that such analysis may be omitted for certain routine samples. This was a retrospective analysis of the value of routine histopathological examination performed in daily general surgical practice. Methods: All specimens from routine appendicectomies, cholecystectomies, haemorrhoidectomies and inguinal hernia repairs performed between 1993 and 2002 were included. The analysis included a comparison of histological and macroscopic diagnoses, review of preoperative and peroperative findings, and an evaluation of the consequences of routine histopathological assessment on patient management and costs. Results: With the exception of hernia specimens, the rate of submission for routine pathological evaluation was 100 percent. No hernia sac specimen from more than 2000 interventions revealed aberrant histological findings. Of 311 haemorrhoidectomy specimens three showed malignancy, all of which had a suspicious macroscopic appearance. Of 1465 appendices, only one (0(.)1 per cent) had a potentially relevant histological diagnosis that was not suspected macroscopically. Among 1523 cholecystectomy specimens, all adenomas (0(.)6 per cent) and carcinomas (0(.)4 per cent) were suspected macroscopically or developed in association with a known disease. Conclusion: The rarity of incidental histological findings relevant to patient management, especially in the absence of macroscopic abnormalities, suggests that routine histological examination of certain specimens may be omitted. A more elementary role for macroscopic examination of the specimen by the surgeon and the pathologist is proposed

A true laparoscopic herniotomy in children : evaluation of long-term outcome

Thirteen years of experience with a laparoscopic technique mimicking, as closely as possible, the classical open technique are presented in this report. Consecutive laparoscopic herniotomies in 385 children (178 girls and 207 boys; age 0.13 to 16.8 years) with a total of 525 hernia repairs were included into a retrospective review. Overall, 95% of children were treated on a day-care basis; all operations were completed laparoscopically. Mean operation time was 32.1 minutes (26.2 for unilateral hernias and 34.5 for bilateral). In total, 10.1% of children presented with clinically bilateral hernias, whereas 29.2% of clinical unilateral hernias had a patent contralateral processus vaginalis. We discovered femoral hernias in 2.6% and direct inguinal hernias in 0.5% of the cases. In 79 cases, a concomitant of umbilical herniorraphy was performed. There were no cases of testicular malposition or atrophy. We had a 1% hernia recurrence rate. Cosmetic outcome was excellent. Laparoscopy provides the surgeon with a superior diagnostic tool, facilitating the diagnosis of any kind of inguinal hernia or controlateral patent processus vaginalis and its repair in the same session; surgery- and anesthesia-related stress is minimized. This makes it an interesting alternative to the open repair of inguinal hernias in children, providing an excellent view on the cord structures and leaving them, as well as the testis, untouched

An atypical case of Burkitt lymphoma with diffuse peritoneal, omental and periportal invasion

Diffuse peritoneal and especially periportal invasion of Burkitt lymphoma is very rarely seen. We present a case of a 16-year-old boy presenting with abdominal pain and constipation, without improvement after enemas. Abdominal ultrasound revealed a diffuse infiltrating peritoneal mass, omental caking, ascites, and a periportal mass, confirmed by computed tomography (CT). No lymphadenopathies were seen. Biopsy was performed and the diagnosis of Burkitt lymphoma was made. Although very rare, it is important to include lymphoma in the differential diagnosis of diffuse peritoneal and periportal invasion in children, as it can mimic peritoneal carcinomatosis