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Background Neuroblastoma (NB) is the most common extracranial solid tumor in children. It is known for high heterogeneity and concealed onset. In recent years, the mechanism of its occurrence and development has been gradually revealed. The purpose of this study is to summarize the clinical characteristics of children with NB and abnormal chromosome 10, and to investigate the relationship between the number and structure of chromosome 10 abnormalities and NB prognosis. Methods Chromosome G-banding was used at the time of diagnosis to evaluate the genetics of chromosomes in patients with NB and track their clinical characteristics and prognosis. All participants were diagnosed with NB in the Medical Oncology Department of the Beijing Children’s Hospital from May 2015 to December 2018 and were followed up with for at least 1 year. Results Of all 150 patients with bone marrow metastases, 42 were clearly diagnosed with chromosomal abnormalities. Thirteen patients showed abnormalities in chromosome 10, and chromosome 10 was the most commonly missing chromosome. These 13 patients had higher LDH and lower OS and EFS than children with chromosomal abnormalities who did not have an abnormality in chromosome 10. Eight patients had both MYCN amplification and 1p36 deletion. Two patients had optic nerve damage and no vision, and one patient had left supraorbital metastases 5 months after treatment. Conclusions The results indicated that chromosome 10 might be a new prognostic marker for NB. MYCN amplification and 1p36 deletion may be related to chromosome 10 abnormalities in NB. Additionally, NB patients with abnormal chromosome 10 were prone to orbital metastases.

Background and objectives Retroperitoneal liposarcoma (RPLS) are common soft tissue sarcomas of adulthood. The aim of this study is to show resectability of even giant liposarcomas and to identify factors associated with recurrence and survival in primary retroperitoneal liposarcomas. Methods We retrospectively reviewed the records of patients with retroperitoneal liposarcoma. Seventy-seven patients met inclusion criteria. Out of these 10 patients with primary giant, dedifferentiated retroperitoneal liposarcomas were operated with en bloc compartment resection with intention of radical resection. Treatment consisted of neoadjuvant radiochemotherapy and surgical resection or surgical resection. Results In 6 patients, neoadjuvant radiochemotherapy was performed; 3 patients were treated with surgical resection alone and 1 patient received adjuvant chemotherapy. The median diameter of tumor size was 360 mm (300 to 440 mm). Operative outcome showed complete resection in all 10 patients. Local tumor free survival was in median 19 month. Tumor recurrence was seen in 3 of 4 patients (75%) without neoadjuvant radiochemotherapy, and in 2 of 6 patients (33%) after neoadjuvant radiochemotherapy in 2 years follow-up. Conclusion Even in case of giant retroperitoneal liposarcoma, complete resection is possible and remains the principal treatment. The rate of recurrence was improved in patients with neoadjuvant radiochemotherapy.

BackgroundCavernous hemangioma, as a rare tumor, is difficult to differentiate from retroperitoneal lymphoma and paraganglioma. They are more difficult to excise completely through open surgery and traditional laparoscopic surgery. The study aimed to evaluate the role of DaVinci surgical system in laparoscopic resection of parapelvic cavernous hemangioma.Case presentationA 46-year-old female, who diagnosed as parapelvic cavernous hemangioma accompanying with thrombosis and calcification, was performed laparoscopic resection using DaVinci surgical system under general anesthesia. The patient well recovered without recurrence or spread of the lesion after operation for 3 months as well as hydronephrosis was significantly relieved.ConclusionLaparoscopic resection of parapelvic cavernous hemangioma under the help of DaVinci surgical system was feasible and safe.

Background Cystic lymphangioma is a rare, benign developmental disease, mostly affecting the cervical and axial regions. The clinical features of abdominal cystic lymphangioma (ACL) are reported among pediatric patients but are less well known in adults. The purpose of this study was to demonstrate the clinical characteristics of ACL in Chinese adults and describe our experience in treating this disease. Methods We conducted a single-center, non-interventional, retrospective study of 12 adult patients with ACL admitted to Peking Union Medical College Hospital in Beijing, China from November 1984 through August 2017. The demographic, clinical, laboratory, imaging, histopathologic, and therapeutic data were collected. Results Detailed information on seven males and five females was available. The mean age at diagnosis was 39.1 (SD 17.3) years. The mean duration of follow-up was 6.9 years. Six (50%) patients were asymptomatic, and abdominal pain was the leading symptom for three (25%) patients. The cysts were evaluated by ultrasound (n = 8, 67%), CT (n = 10, 83%), and MRI (n = 4, 33%). Therapeutic modalities include laparotomy (n = 6, 50%), laparoscopy (n = 4, 33%), and aspiration (n = 2, 17%), with variable outcomes. The mean postsurgical hospital stay was 6.8 days. Complete excision was accomplished in eight patients, and one recurrence was observed during follow-up. Of patients who underwent partial resection, one experienced disease relapse. Conclusions This is the first report on an Asian cohort of adult patients with ACL. Typical imaging features could lead to timely diagnosis and treatment of ACL. Radical resection is recommended with a longer period of follow-up. The analysis of this cohort deepens our understanding of adult ACL.

Background Pheochromocytoma (PCC) and Paraganglioma (PGL) are rare neuroendocrine neoplasms. These tumors harbour disastrous consequences during surgery due to catecholamine hypersecretion if they are undiagnosed or prepared inadequately preoperatively. Case presentation A 41- year- old lady presented with mild left flank discomfort. She had experienced recurrent anxiety attacks accompanied by palpitations and headache which were managed previously as panic attacks. Radiologic investigations showed a retroperitoneal mass that located anteromedial to the left kidney, separated from the left adrenal gland and adherent to the 4th duodenal segment. During admission, her vital signs showed slight elevation of blood pressure (140\90–160\110) mmHg, thus 24-h urine metanephrine and normetanephrine were requested and the results revealed normal values. Upper gastrointestinal endoscopy failed to pass beyond the 3th duodenal segment and showed no pathologic evidence. According to her findings, a diagnosis of duodenal gastrointestinal stromal tumor (GIST) was suspected. During laparotomy, crises of hypertension and tachycardia followed by severe hypotension made the resection of the misdiagnosed mass very tricky. Immunohistochemical staining studies confirmed the diagnosis of paraganglioma. Conclusion Paraganglioma is a life threatening disease and should always be considered as a differential diagnosis of asymptomatic retroperitoneal mass. The aim of our study is to present a challenging case of an undiagnosed retroperitoneal paraganglioma and to alarm our colleagues from such troubles.

Background Retroperitoneal sarcomas (RPS) include a heterogeneous group of rare malignant tumours, and various treatment algorithms are still controversially discussed until today. The present study aimed to examine postoperative and long-term outcomes after resection of primary RPS. Patients and methods Clinicopathological data of patients who underwent resection of primary RPS between 2005 and 2015 were assessed, and predictors for overall survival (OS) and disease-free survival (DFS) were identified. Results Sixty-one patients underwent resection for primary RPS. Postoperative morbidity and mortality rates were 31 and 3%, respectively. After a median follow-up time of 74 months, 5-year OS and DFS rates were 58 and 34%, respectively. Histologic high grade (5-year OS: G1: 92% vs. G2: 54% vs. G3: 43%, P = 0.030) was significantly associated with diminished OS in univariate and multivariate analyses. When assessing DFS, histologic high grade (5-year DFS: G1: 63% vs. G2: 24% vs. G3: 22%, P = 0.013), positive surgical resection margins (5-year DFS: R0: 53% vs. R1: 10% vs. R2: 0%, P = 0.014), and vascular involvement (5-year DFS: yes: 33% vs no: 39%, P = 0.001), were significantly associated with inferior DFS in univariate and multivariate analyses. Conclusions High-grade tumours indicated poor OS, while vascular involvement, positive surgical resection margins, and histologic grade are the most important predictors of DFS. Although multimodal treatment strategies are progressively established, surgical resection remains the mainstay in the majority of patients with RPS, even in cases with vascular involvement.

Background Gastric gastrointestinal stromal tumor (GIST), intro-abdominal and retroperitoneal neoplasms are distinct tumors arising from different cell layers; therefore, coexistence of such tumors is relatively rare. Case presentation A man complained of early satiety for 2 mouths, whose upper gastrointestinal (GI) endoscopy showed a tumor arising from the greater curvature of gastric body and extending into the lumen. Abdominal computed tomography (CT) revealed coexistence of gastric, intro-abdominal and retroperitoneal masses. Wedge resection for gastric tumor, resection for intro-abdominal and retroperitoneal tumors were done. The postoperative histological examination suggested simultaneous development of a gastric GIST, intro-abdominal and retroperitoneal myxoid liposarcomas. Conclusion Although both GISTs and liposarcomas originate from mesenchymal tissues, simultaneous development of a gastric GIST, intro-abdominal and retroperitoneal liposarcomas is the first such case to be reported in the literature.

Background Patients with a primary pure seminoma in the testis who have elevated serum alpha-fetoprotein are rare and should be treated as patients with nonseminomatous germ cell tumors. However, nonpalpable testicular tumors in this condition have never been reported. We describe a case of nonpalpable pure testicular seminoma with elevated serum alpha-fetoprotein presenting retroperitoneal metastasis. Case presentation A 29-year-old Asian man was referred to our hospital with right flank pain. Computed tomography showed a mass located between his aorta and inferior vena cava, but a testicular tumor was not detected. His serum levels of lactate dehydrogenase, alpha-fetoprotein, and DUPAN-2 were high. Although no tumor or nodule was palpable in his testis, ultrasonography revealed multiple low echoic lesions in his right testicular parenchyma. He was diagnosed with right testicular cancer with retroperitoneal lymph node metastasis and underwent right high orchiectomy. A pathological examination revealed pure seminoma and no nonseminomatous components were found in the specimen. Three courses of induction systemic chemotherapy (cisplatin, etoposide, and bleomycin) normalized his serum alpha-fetoprotein and DUPAN-2 levels. Three additional courses of chemotherapy (etoposide and bleomycin) were performed, and treatment was completed with laparoscopic retroperitoneal lymph node dissection. Pathology of the dissected specimen showed fibrous and necrotic tissue with no viable cells. He is alive without recurrence 54 months after orchiectomy. Conclusions We report a case of pure testicular seminoma with elevated serum alpha-fetoprotein and DUPAN-2 presenting retroperitoneal metastasis. We recommend an ultrasound examination of bilateral testes when large retroperitoneal tumors are detected in young men, even if a mass is not palpable in the scrotum.

Introduction Lymphangiomas are rare, benign tumors. An intra-abdominal location of these lesions is rarer still and there are only a few reports describing laparoscopic resection for retroperitoneal lymphangiomas, especially in tumors that mimic pancreatic tumors. Case presentation We present the case of an asymptomatic 30-year-old Japanese woman in whom a cystic tumor was found incidentally in close approximation to the pancreas. Because the tumor was located in the retroperitoneal space and the body of the pancreas was compressed, we were unable to distinguish a cystic lymphangioma from cystic pancreatic tumors. We started the procedure laparoscopically with five ports. The tumor was in fact separated from the pancreas and was dissected free from the body of the pancreas using scissors and laparoscopic coagulating shears. The left gastric vessels, which were compressed by the tumor, were preserved. As we realized that the tumor was connected to the retroperitoneal lymphatic tissue, we completed the procedure by performing a cystectomy without rupture. The specimen was extracted using a plastic bag. Our patient was discharged on postoperative day 7 without any complications. There is no evidence of recurrence during a >2-year observation period. Conclusions In addition to the therapeutic significance in differentiating between a cystic lymphangioma in close approximation to the body of the pancreas and a pancreatic cystic neoplasm, the laparoscopic approach is feasible and effective. Electronic supplementary material The online version of this article (doi:10.1186/s13256-015-0760-z) contains supplementary material, which is available to authorized users.

Background Retroperitoneal tumors (RTs) develop insidiously and are generally seen as large masses, and 50% of RTs are larger than 20 cm at the time of diagnosis. In this article, we share our experience of 5 years of surgical management of RTs. Methods We evaluated 28 RT cases operated on in three education hospitals in Turkey from January 2008 onwards, with regard to patients’ demographic characteristics, complaints, weight loss figures, the location and size of the tumor, blood transfusion, intra-operational time, metastases (in malignant cases), additional organ resection, histological grade, local recurrences, average life expectancy, and post-operative treatment methods. Results The mean age of the patients was 49 years (range, 18 to 78 years). Twenty (71.43%) were female, and 8 (28.57%) were male. The primary complaint was abdominal pain in 18 patients (64.28%). CT scans were performed in 17 (61%) patients, 10 (35.4%) underwent abdominal MR imaging, and 1 (3.6%) underwent both abdominal CT and abdominal MR imaging. A mass was palpated in the pelvis (suprapubic region) in seven (25%) of the patients during physical examination. The largest tumors were detected in the left lumbar area. The mean tumor size was 12.78 cm (range, 2 to 30 cm). The mean intra-operational time was 192 min (range, 70 to 380 min). The mean hospitalization period was 11 days (range, 8 to 23 days). Seven (25%) patients were reported to have benign tumors, while 21 (75%) were reported to have malignant tumors. The most frequently seen malignant pathology was liposarcoma (eight cases; 38.09%) followed by leiomyosarcoma (five cases; 23.8%) and malignant fibrous histiocytoma (four cases; 19.04%). The earliest local recurrence was detected in the 12th month and the latest in the 28th month. A total of 11 (52.3%) of the total of 21 malignant cases experienced local recurrence within 3 years. The 3-year average life expectancy was 85.7% in the 18 malignant cases. Conclusions Due to the low response rate of all but two types of RT to chemotherapy, the best remaining treatment option is surgery with wide resection margins, whereby all macroscopic traces of tumor are removed.

Background Retroperitoneal lipomas are an extremely rare condition with only 17 cases described in the literature since 1980. They can reach enormous size and cause significant abdominal symptoms. The most important differential diagnosis is the well-differentiated liposarcoma, which preoperatively often may not definitely be ruled out. Case presentation We present the case of a 73 year-old Caucasian patient with a giant retroperitoneal lipoma of 9 kg measuring 55 cm in diameter. The patient presented with abdominal pain and swelling that had been slowly progressive for the last 15 years. On computerized tomography an immense retroperitoneal tumor was revealed. Intraoperatively, the tumor did not show any signs of infiltrative growth, therefore sole tumor extirpation was performed. Conclusion Retroperitoneal lipomas are not clearly distinguishable from well-differentiated liposarcomas on imaging and even biopsies may be misleading. Moreover, abdominal symptoms, i.e. pain, obstipation and dysphagia may occur due to mechanical displacement. Therefore, surgical exploration with complete oncological resection is the therapy of choice if malignity cannot be ruled out. Electronic supplementary material The online version of this article (doi:10.1186/s13104-015-1038-7) contains supplementary material, which is available to authorized users.

Hemangiomas of the gastrointestinal tract and mesentery are uncommon benign vascular lesions. While spontaneous bleeding is the hallmark of the gastrointestinal tumor variant, clinical signs of mesenteric hemangiomas are mostly unspecific. Despite the increasing imaging quality of computerized tomography (CT), in most cases the final diagnosis is established through surgery and histopathologic analysis of a macrobiopsy. We present a case report of a 20-year-old female patient who was admitted with progressive abdominal distension and suffered from persistent abdominal pain for 3 months. A large retroperitoneal tumor mass was detected on the CT scan. Due to radiographic signs of an intraabdominal liposarcoma, an explorative laparotomy was performed revealing a large hemangioma originating from the mesosigmoid. Although rare, gastrointestinal hemangiomas should be kept in mind by oncological visceral surgeons as one differential diagnosis of large intraabdominal tumorous masses, especially in young adults.

Background Recent studies have demonstrated that pathological analysis of retroperitoneal residual masses of patients with testicular germ cell tumors revealed findings of necrotic debris or fibrosis in up to 50% of patients. We aimed at pursuing a clinical and pathological review of patients undergoing post chemotherapy retroperitoneal lymph node dissection (PC-RPLND) in order to identify variables that may help predict necrosis in the retroperitoneum. Methods We performed a retrospective analysis of all patients who underwent PC-RPLND at the University Hospital of the University of São Paulo and Cancer Institute of Sao Paulo between January 2005 and September 2011. Clinical and pathological data were obtained and consisted basically of: measures of retroperitoneal masses, histology of the orchiectomy specimen, serum tumor marker and retroperitoneal nodal size before and after chemotherapy. Results We gathered a total of 32 patients with a mean age of 29.7; pathological analysis in our series demonstrated that 15 (47%) had necrosis in residual retroperitoneal masses, 15 had teratoma (47%) and 2 (6.4%) had viable germ cell tumors (GCT). The mean size of the retroperitoneal mass was 4.94 cm in our sample, without a difference between the groups (P = 0.176). From all studied variables, relative changes in retroperitoneal lymph node size (P = 0.04), the absence of teratoma in the orchiectomy specimen (P = 0.03) and the presence of choriocarcinoma in the testicular analysis after orchiectomy (P = 0.03) were statistically significant predictors of the presence of necrosis. A reduction level of 35% was therefore suggested to be the best cutoff for predicting the absence of tumor in the retroperitoneum with a sensitivity of 73.3% and specificity of 82.4%. Conclusions Even though retroperitoneal lymph node dissection remains the gold standard for patients with residual masses, those without teratoma in the primary tumor and a shrinkage of 35% or more in retroperitoneal mass have a considerably smaller chance of having viable GCT or teratoma in the retroperitoneum and a surveillance program could be considered.

To report an unplanned interim analysis of a prospective, one-armed, single center phase I/II trial (NCT01566123). Between 2007 and 2013, 27 patients (pts) with primary/recurrent retroperitoneal sarcomas (size > 5 cm, M0, at least marginally resectable) were enrolled. The protocol attempted neoadjuvant IMRT using an integrated boost with doses of 45–50 Gy to PTV and 50–56 Gy to GTV in 25 fractions, followed by surgery and IOERT (10–12 Gy). Primary endpoint was 5-year-LC, secondary endpoints included PFS, OS, resectability, and acute/late toxicity. The majority of patients showed high grade lesions (FNCLCC G1:18%, G2:52%, G3:30%), predominantly liposarcomas (70%). Median tumor size was 15 cm (6–31). Median follow-up was 33 months (5–75). Neoadjuvant IMRT was performed as planned (median dose 50 Gy, 26–55) in all except 2 pts (93%). Gross total resection was feasible in all except one patient. Final margin status was R0 in 6 (22%) and R1 in 20 pts (74%). Contiguous-organ resection was needed in all grossly resected patients. IOERT was performed in 23 pts (85%) with a median dose of 12 Gy (10–20 Gy). We observed 7 local recurrences, transferring into estimated 3- and 5-year-LC rates of 72%. Two were located outside the EBRT area and two were observed after more than 5 years. Locally recurrent situation had a significantly negative impact on local control. Distant failure was found in 8 pts, resulting in 3- and 5-year-DC rates of 63%. Patients with leiomyosarcoma had a significantly increased risk of distant failure. Estimated 3- and 5-year-rates were 40% for PFS and 74% for OS. Severe acute toxicity (grade 3) was present in 4 pts (15%). Severe postoperative complications were found in 9 pts (33%), of whom 2 finally died after multiple re-interventions. Severe late toxicity (grade 3) was scored in 6% of surviving patients after 1 year and none after 2 years. Combination of neoadjuvant IMRT, surgery and IOERT is feasible with acceptable toxicity and yields good results in terms of LC and OS in patients with high-risk retroperitoneal sarcomas. Long term follow-up seems mandatory given the observation of late recurrences. Accrual of patients will be continued with extended follow-up. NCT01566123 .

Carcinosarcoma is a rare biphasic malignancy consisting of intermixed epithelial and mesenchymal elements. Carcinosarcoma is particularly rare among children. We accepted a 7 year old patient with retroperitoneal carcinosarcoma. The tumor was totally resected and no recurrence is found 11 months after operation. Literatures has been reviewed and there are few reports of primary retroperitoneal carcinosarcoma in children up to date. So we report the patient's clinical character, surgical resection, pathological and immunohistochemical analysis.

Background Extra-adrenal myelolipomas are rare benign tumours. Other soft tissue tumours such as well-differentiated liposarcomas appear morphological almost identical. Preoperative imaging and especially biopsy are important tools to diagnose these lesions. Case presentation We report a very seldom case of a simultaneous myelolipoma of the adrenal gland in association with an extra-adrenal myelolipoma in an 75-year-old man. With a review of the literature we describe and discuss the aetiology, differential diagnosis and treatment of patients with respect to adrenal and extra-adrenal lesions. Conclusion The appearance of a simultaneous adrenal and extra-adrenal myelolipoma is a rare incident. We conclude that such lesions should be considered in the differential diagnosis of a fat-containing tumour in the retroperitoneal tissue/compartment.