Mid-transverse colon cancer and extended versus transverse colectomy: Results of the Italian society of surgical oncology colorectal cancer network (SICO CCN) multicenter collaborative study.

INTRODUCTION: Transverse colon cancer (TCC) is poorly studied, and TCC cases are often excluded from large prospective randomized trials because of their complexity and their potentially high complication rate. The best surgical approach for TCC has yet to be established. The aim of this large retrospective multicenter Italian series is to investigate the advantages and disadvantages of both hemicolectomy and transverse colectomy in order to identify the best surgical approach. MATERIALS AND METHODS: This was a retrospective cohort study of patients with mid-transverse colon cancer treated with a segmental colon resection or an extended hemicolectomy (right or left) between 2006 and 2016 in 28 high-volume (more than 70 procedures/year) Italian referral centers for colorectal surgery. RESULTS: The study included 1529 patients, 388 of whom underwent a segmental resection while 1141 underwent an extended resection. A higher number of complications has been reported in the segmental group than in the extended group (30.1% versus 23.6%; p 0.010). In 42 cases the main complication was the anastomotic leak (4.4% versus 2.2%; p 0.020). Recovery outcomes also showed statistical differences: time to first flatus (p 0.014), time to first mobilization (p 0.040), and overall hospital stay (p < 0.001) were significantly shorter in the extended group. Even if overall survival were similar between the groups (95.1% versus 97%; p 0.384), 3-year disease-free survival worsened after segmental resection (78.1% versus 86.2%; p 0.001). CONCLUSIONS: According to our results, an extended right colon resection for TCC seems to be surgically safer and more oncologically valid.

Laparoscopic nephrectomy in children with Wilms tumor. Considerations after 10 years of experience.

Despite laparoscopy in children is considered safe and is routinely used for several procedures, even in neonates and in pediatric oncology, its role in the treatment of pediatric renal tumors is still controversial. This study analyzes the results of laparoscopic nephrectomy for Wilms Tumor (WT) in pediatric age compared with open nephrectomy after 10 years of experience in a single centre. From 1993 in our center of reference for pediatric oncology, 30 patients with WT have been treated. We performed 21 open nephrectomy and in the last 10 years 9 laparoscopic nephrectomy. In all patients treated laparoscopically, the same technique made by the same equip was used. Compared with patients treated by open surgery, we did not find a significant difference in terms of outcome and survival. In the open surgery group, two patients had lung relapse while in the other group there was one local relapse. These three children obtained and maintained a second complete remission with chemotherapy. Open surgery complications were a tumor rupture in two cases, and an episode of pancreatitis 10 days after surgery. In the laparoscopic group, there were two conversions to open surgery not considered as complications but a surgical choice for cystic areas present in the tumor. As far as complications and oncologic outcomes are concerned, both techniques showed similar results. In experienced hands, laparoscopy proves to be an attractive alternative to open surgery for pediatric renal tumors.

Importance of a careful clinical evaluation in the diagnosis of cystic pancreatic tumors.

INTRODUCTION: Intraductal papillary mucinous neoplasms (IPMN) are a rare group of pancreatic neoplasms. Often are asymptomatic and, when are symptomatic, patients complain sensation of weight in the abdomen or compression at the neighboring structures. In many cases the diagnosis is incidental, during a CT or MR performed for other raisons. CASE REPORT: We report a case of a 59-year-old woman with diagnosis of post-pancreatitis pseudocyst who, instead, was affected by an intraductal papillary mucinous neoplasm (IPMN), treated by us with pancreatoduodenectomy. DISCUSSION: The diagnosis of IPMN has increased in recent years thanks to an improvement in radiological investigation. The study of pancreatic lesions must be very careful and it is absolutely necessary that diagnostic imaging be accompanied by a correct clinical evaluation of the patient. CONCLUSION: A thorough anamnesis is required in patient with history of acute pancreatitis to avoid the mistake of exchanging an IPMN for a pseudocyst.

Metastatic neuroblastoma in infants: are survival rates excellent only within the stringent framework of clinical trials?

Introduction. SIOPEN INES protocol yielded excellent 5-year survival rates for MYCN-non-amplified metastatic neuroblastoma. Patients deemed ineligible due to lack or delay of MYCN status or late registration were treated, but not included in the study. Our goal was to analyse survival at 10 years among the whole population. Materials and methods. Italian and Spanish metastatic INES patients’ data are reported. SPSS 20.0 was used for statistical analysis. Results. Among 98 infants, 27 had events and 19 died, while 79 were disease free. Five- and 10-year event-free survival (EFS) were 73 and 70 %, and overall survival (OS) was 81 and 74 %, respectively. MYCN status was significant for EFS, but not for OS in multivariate analysis. Conclusions. The survival rates of patients who complied with all the inclusion criteria for INES trials are higher compared to those that included also not registered patients. Five-year EFS and OS for INES 99.2 were 87.8 and 95.7 %, while our stage 4s population obtained 78 and 87 %. Concerning 99.3, 5-year EFS and OS were 86.7 and 95.6 %, while for stage 4 we registered 61 and 68 %. MYCN amplification had a strong impact on prognosis and therefore we consider it unacceptable that many patients were not studied for MYCN and probably inadequately treated. Ten-year survival rates were shown to decrease: EFS from 73 to 70 % and OS from 81 to 74 %, indicating a risk of late events, particularly in stage 4s. Population-based registries like European ENCCA WP 11-task 11 will possibly clarify these data (AU)

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Metastatic neuroblastoma in infants: are survival rates excellent only within the stringent framework of clinical trials?

INTRODUCTION: SIOPEN INES protocol yielded excellent 5-year survival rates for MYCN-non-amplified metastatic neuroblastoma. Patients deemed ineligible due to lack or delay of MYCN status or late registration were treated, but not included in the study. Our goal was to analyse survival at 10 years among the whole population. MATERIALS AND METHODS: Italian and Spanish metastatic INES patients' data are reported. SPSS 20.0 was used for statistical analysis. RESULTS: Among 98 infants, 27 had events and 19 died, while 79 were disease free. Five- and 10-year event-free survival (EFS) were 73 and 70 %, and overall survival (OS) was 81 and 74 %, respectively. MYCN status was significant for EFS, but not for OS in multivariate analysis. CONCLUSIONS: The survival rates of patients who complied with all the inclusion criteria for INES trials are higher compared to those that included also not registered patients. Five-year EFS and OS for INES 99.2 were 87.8 and 95.7 %, while our stage 4s population obtained 78 and 87 %. Concerning 99.3, 5-year EFS and OS were 86.7 and 95.6 %, while for stage 4 we registered 61 and 68 %. MYCN amplification had a strong impact on prognosis and therefore we consider it unacceptable that many patients were not studied for MYCN and probably inadequately treated. Ten-year survival rates were shown to decrease: EFS from 73 to 70 % and OS from 81 to 74 %, indicating a risk of late events, particularly in stage 4s. Population-based registries like European ENCCA WP 11-task 11 will possibly clarify these data.

Thyroid hemiagenesis associated with multinodular goiter and Hashimoto's thyroiditis.

Thyroid hemiagenesis is a rare congenital abnormality in which one of the thyroid lobes is not developed. It can be associated with various thyroid diseases, such as Grave's disease, nodular goiter and thyroid neoplasm, rarely with hyperparathyroidism. We report a case of a 50-year old woman with left thyroid lobe agenesis diagnosed by ultrasonography and scintigraphy. Right thyroidectomy was performed and the histopathological examination showed diffuse hyperplasia, multinodular goiter and Hashimoto's thyroiditis. To our knowledge, this is the first description of multinodular goiter and Hashimoto's thyroiditis in a patient with thyroid hemiagenesis.

Unusual presentation of retroperitoneal Schwannoma: case report.

Schwannoma is a rare tumor that develops from the Schwann cells in the nerve sheath. A 42 years old woman was found incidentally to have a bulky mass in epigastric region. Abdominal ultrasonography CT and MRI have been of aid to know the position and size of the tumor. A massive capsulated retroperitoneal lesion was identified. It moved forward the hepatoduodenal ligament, inferior vena cava laterally and aorta medially. The mass is exte-riorized and detached from adhesions. There were no complications after the operation and the patient was discharged on the fourth post-operative day. The microscopically examina-tion showed features suggestive of Cellular Schwannoma. After 8 months during follow-up, the patient did not report any neurological deficit and control CT did not suggest the presence of recurrent disease.

Cystic pancreatic tumors: should we resect all of them?

OBJECTIVE: Pancreatic cystic tumors are relatively rare tumors and only 1% of them are malignant. They are often asymptomatic and detected as incidental findings through diagnostic imaging. Currently there are no universal guide lines for the correct clinical approach to pancreatic cystic lesions. Cross-sectional imaging demonstrates some typical morphological features that determine the pre-operative diagnosis of the pancreatic cystic lesions (serous or mucinous cystadenoma, intraductal papillary mucinous neoplasms). In addition, endoscopic ultrasonography permits the collection and analysis of the fluid content. The aim of this paper is to describe our case load in the management of pancreatic cystic neoplasms and propose some criteria for choosing between surgical or conservative approaches. PATIENTS AND METHODS: 12 patients with pancreatic cystic neoplasms were retrospectively evaluated. They were studied using cross-sectional imaging modalities (computed tomography and magnetic resonance); endoscopic ultrasonography was performed in 7 patients. RESULTS: In each patient a careful evaluation of several factors (age, comorbidity, imaging features, symptoms, life expectancy) conditioned our clinical decision. Among our 12 patients, surgical resection was performed in 7 cases. DISCUSSION: The treatment of pancreatic cystic lesions is still a dilemma because even in the presence of malignant potential, pancreatic surgery remains very complicated and demolitive. Many factors need to be considered in the management of cystic pancreatic tumors. The most important include histological type, location, size, age and clinical condition of the patient. CONCLUSIONS: A correct multidisciplinary pre-operative diagnosis is mandatory. Surgery should only be performed in selected cases.

Synchronous double cancers of the colon and the pancreas: a case report.

The authors report a clinical case of a synchronous double cancer of the colon and pancreas. Having carefully examined the scarce literature, they dwell on the incidence of this disease referring to frequencies that in the literature range from 0.06% and 0.55% while considering that some series do not show any association between the two neoplasms. The authors analyze the role of the colorectal follow-up. In fact, such a follow-up, even if only providing a relative improvement in disease-free survival, has allowed for a treatment of the two certainly not-advanced tumors ensuring the patient a good prognosis. Finally, the authors analyze how endoscopic ultrasound-guided fine-needle aspiration made possible a preoperative diagnosis of small pancreatic cancer, and show that, the positive predictive value (PPV) of this method was 100%.

Cystic pancreatic neoplasms: diagnosis and management emphasizing their imaging features.

The incidence of cystic pancreatic neoplasms increased in the past decade, due to the recent advances in multidetector computed tomography and magnetic resonance imaging; several pancreatic cysts are incidentally encountered during diagnostic exams performed for non-pancreatic diseases. Indeed, cystic pancreatic tumors are currently considered relatively rare, accounting for approximately 10% of all pancreatic neoplasms. Serous cystadenoma, mucinous cystadenoma, intraductal papillary mucinous neoplasms and solid-pseudopapillary tumor represent about 90% of all pancreatic primary cystic tumours. The non-optimal diagnostic preoperative accuracy in distinguishing benign from malignant cystic lesions ensures that up till now there are no well-defined guidelines regarding the management of cystic pancreatic neoplasms. Imaging findings often do not allow the diagnosis, because there is a considerable overlap among the cystic lesions; the best pre-operative characterization is obtained by the association of all diagnostic procedures available. For their different histology and behavior, cystic pancreatic neoplasms need to be managed according to various factors. In this review, the main elements necessary for their management are assessed--radiological features, tumour dimensions, patients' characteristics, the mode of clinical presentation and the associated oncologic markers. A multidisciplinary approach--including gastroenterologists, radiologists and surgeons--should be adopted in order to perform a differential diagnosis and a correct management.

Segmental chromosomal alterations lead to a higher risk of relapse in infants with MYCN-non-amplified localised unresectable/disseminated neuroblastoma (a SIOPEN collaborative study).

BACKGROUND: In neuroblastoma (NB), the presence of segmental chromosome alterations (SCAs) is associated with a higher risk of relapse. METHODS: In order to analyse the role of SCAs in infants with localised unresectable/disseminated NB without MYCN amplification, we have performed an array CGH analysis of tumours from infants enrolled in the prospective European INES trials. RESULTS: Tumour samples from 218 out of 300 enroled patients could be analysed. Segmental chromosome alterations were observed in 11%, 20% and 59% of infants enroled in trials INES99.1 (localised unresectable NB), INES99.2 (stage 4s) and INES99.3 (stage 4) (P<0.0001). Progression-free survival was poorer in patients whose tumours harboured SCA, in the whole population and in trials INES99.1 and INES99.2, in the absence of clinical symptoms (log-rank test, P=0.0001, P=0.04 and P=0.0003, respectively). In multivariate analysis, a SCA genomic profile was the strongest predictor of poorer progression-free survival. CONCLUSION: In infants with stage 4s MYCN-non-amplified NB, a SCA genomic profile identifies patients who will require upfront treatment even in the absence of other clinical indication for therapy, whereas in infants with localised unresectable NB, a genomic profile characterised by the absence of SCA identifies patients in whom treatment reduction might be possible. These findings will be implemented in a future international trial.

Metachronous liver metastases and resectability: Fong's score and laparoscopic evaluation.

BACKGROUND AND AIMS: The aim of this retrospective study was to establish whether Fong's risk score can predict rate of resectability and whether laparoscopic exploration with ultrasonography can reduce the number of useless laparotomies to any extent. MATERIAL AND METHODS: Fong's score was calculated for each of the 43 potential resectable patients. We analysed: the relation between score and resectability; the probability of unnecessary laparotomy with respect to each level of score; and which of the five Fong parameters was the most indicative of non-resectability. None of our patients was submitted to preoperative laparoscopic staging. RESULTS: All patients with Fong's score 0 were submitted to liver resection, whereas only 76.9% with score 1, 58.3% with score 2, and 66.6% with score 3. No patients had score 4 and 5. "CEA level" is the parameter that best predicts the "non-resectability" of metastases. In the subgroup with score 0-1, laparoscopy would have spared 12% of unnecessary laparotomies, whereas in subgroup 2-3 this percentage would have risen to 38.9. CONCLUSIONS: The above data allowed us to quantify statistically the risk associated with non-resectability of liver metastases in a directly proportional manner as the score progresses.

Idiopathic myenteric ganglionitis underlying acute 'dramatic' intestinal pseudoobstruction: report of an exceptional case.

Inflammation of the myenteric plexus of the gastrointestinal tract is a very rare pathological condition, with few reports in the medical literature. This pathological condition causes atonic gut motor dysfunction and is principally secondary to other diseases, being reported nearly solely as a paraneoplastic phenomenon in neuroendocrine lung tumors, including small cell carcinomas or neuroblastomas. In addition it can also be associated with disorders of the central nervous system, although it has rarely been described in Chagas disease. It has been named 'idiopathic myenteric ganglionitis' because no apparent causes can be demonstrated. We report the clinicopathologic findings of an exceptional case of a young woman affected by severe chronic constipation suddenly changing into acute intestinal pseudoobstruction with dramatic evolution. Relationships between ganglionitis, idiopathic constipation and acute intestinal pseudoobstruction as well as therapeutic implications are discussed.

Methodologies for the treatment of acute and chronic nononcologic pain in children.

Pain represents the most feared symptom of illness but for many years it has been underestimated in children. Only recently has analgesic treatment become an integral part in the assistance of ill children with acute pain, such as postoperative pain, and with chronic pain. Postoperative pain is a complication of surgery: postoperative pain prevention should replace the current theory and practice of postoperative treatment through the creation of ''pain services'' and adequate pain prevention planning. Pain prevention begins in the preoperative period, continues in the operating theaters and in the postoperative phase. A multimodal approach should employ techniques of loco-regional anesthesia using a variety of agents from opioids to nonsteroidal anti-inflammatory drugs or paracetamol. The techniques we currently use to control procedural pain are both medical and nonmedical. Nonmedical interventions such as distraction, muscular relaxation, and guided imagination for pain control are psychological techniques showing very good results in those children who must undergo various procedures. The medical treatment must guarantee both sedation and effective analgesia. Our medical approach includes local anaesthesia, conscious sedation, deep sedation, and general anaesthesia.

[Carcinoma of the Bellini collecting duct in paediatric patients: a case report and review of the literature]. / Carcinoma dei dotti collettori di Bellini in età pediatrica: descrizione di un caso e revisione della letteratura.

Collecting duct carcinoma is an uncommon variant of renal cell carcinoma that usually occurs at an earlier age compared to conventional renal cell carcinoma. It is characterised by an aggressive, often fatal, course. Renal cell carcinoma rarely occurs in paediatric patients, and is almost always in association with specific genetic alterations; the most common histotypes are the clear cell and chromophobe cell variants. Collecting duct carcinoma is rare, and only 8 cases have been reported in the literature. The authors describe the clinico-pathological features of a fatal collecting duct carcinoma in an 11-year-old boy.

Mortality from second tumour among long-term survivors of retinoblastoma: a retrospective analysis of the Italian retinoblastoma registry.

Survivors of retinoblastoma (Rb) are at high risk of dying from second malignant tumour. The occurrence of second malignant neoplasm (SMN) and related mortality in a cohort of 1111 cases from the Italian Retinoblastoma Registry was analysed, considering the possible role of both genetic and iatrogenic causes. Rb patients had a greater than 10-fold excess in overall mortality compared with the general population (standardized mortality ratio (SMR) 10.73, 95% CI 9.00-12.80). Their excess risk attributable to cancers other than Rb was 14.93 95% CI 10.38-21.49). Survivors of hereditary Rb had an SMR for all causes of 16.25 (95% CI 13.20-20.00), whereas their SMR for all cancers was 25.72 (95% CI 17.38-38.07). Survivors of unilateral sporadic Rb had an SMR of 4.12 from all cancers (95% CI 1.55-10.98) and a much higher excess for overall mortality (SMR 13.34, 95% CI 10.74-16.56). As expected, survivors of hereditary Rb had higher mortality from cancers of the bone (SMR 391.90, 95% CI 203.90-753.20) and soft tissue (SMR 453.00, 95% CI 203.50-1008.40), small intestine (SMR 1375.50, 95% CI 344.00-5499.70), nasal cavity (SMR 13.71, 95% CI 1.93-97.35) and cancers of the brain and central nervous system (SMR 41.14, 95% CI 13.2-127.55).

Embryonal tumor with abundant neuropil and true rosettes. A new entity or only variations of a parent neoplasms (PNETs)? This is the dilemma.

A rare embryonal brain tumor has been diagnosed in a 4-year-old boy. The mass, located at the pons and mesencephalon, has been histologically classified as an embryonal tumor containing abundant neuropil and true rosettes. After surgical complete removal of the neoplasia, the child received intensive combined chemotherapy and radiotherapy. He is alive and free of disease at 34 months from surgery. Difficulties in histological definition, possible suggestions for treatment proposals are discussed.

[Myasthenia gravis and intestinal resection: is dehiscence likely to occur?]. / Myasthenia gravis e resezione intestinale: deiscenza in agguato?

Experimental and clinical data have proved that anticholinesterase drugs are responsible for vigorous peristaltic contractions and for an increase of the intraluminal pressure, because they determine muscarinic effects on the smooth muscle of the intestine both in small and large intestine. Therefore, a greater incidence of intestinal anastomotic disruption has been supposed when anticholinesterases are used both in the early postoperative period, to reverse curarization, and in myasthenic patients. The authors report a case of a patient with myasthenia gravis who received maximal doses of pyridostigmine and underwent left hemicolectomy and small intestine resection and afterwards total colectomy in order to treat a sigmoid perforated diverticulitis. In the postoperative course an anastomotic leak developed after both surgical operations. The authors believe that pyridostigmine could have had an important role in the pathogenesis of the leak and assert that, when an intestinal resection has to be performed in a myasthenic patient, it could be useful to reduce in the preoperative period the administration of anticholinesterase drugs and always perform a protective ileostomy.