Peripancreatic paraganglioma: Lesson from a round table.

We described the case of a peripancreatic paraganglioma (PGL) misdiagnosed as pancreatic lesion. Surgical exploration revealed an unremarkable pancreas and a large well-defined cystic mass originating at the mesocolon root. Radical enucleation of the mass was performed, preserving the pancreatic tail. Histologically, a diagnosis of PGL was rendered. Interestingly, two previously unreported mutations, one affecting the KDR gene in exon 7 and another on the JAK3 gene in exon 4 were detected. Both mutations are known to be pathogenetic. Imaging and cytologic findings were blindly reviewed by an expert panel of clinicians, radiologists, and pathologists to identify possible causes of the misdiagnosis. The major issue was lack of evidence of a cleavage plane from the pancreas at imaging, which prompted radiologists to establish an intra-parenchymal origin. The blinded revision shifted the diagnosis towards an extra-pancreatic lesion, as the pancreatic parenchyma showed no structural alterations and no dislocation of the Wirsung duct. Ex post, the identified biases were the emergency setting of the radiologic examination and the very thin mesocolon sheet, which hindered clear definition of the lesion borders. Original endoscopic ultrasonography diagnosis was confirmed, emphasizing the intrinsic limit of this technique in detecting large masses. Finally, pathologic review favored a diagnosis of PGL due to the morphological features and immonohistochemical profile. Eighteen months after tumor excision, the patient is asymptomatic with no disease relapse evident by either radiology or laboratory tests. Our report strongly highlights the difficulties in rendering an accurate pre-operative diagnosis of PGL.

A look towards the clonal origin of metastatic pulmonary carcinosarcoma: Report of a patient with an unexpected long-term survival.

INTRODUCTION: Pulmonary carcinosarcoma is a rare histological subtype of non-small cell lung cancer, defined by the combination of epithelial and mesenchimal elements. Prognosis is usually dismal, with a median survival of about 6 months. The use of immunotherapy by blockade of PD1/PD-L1 immune checkpoint signaling has been shown to improve patients' survival. However, local aggressiveness and distant metastases are frequent. Spread to the gastrointestinal tract is seldom reported. The genetic landscape of the disease has only recently begun to emerge, pointing at TP53, KRAS, EGFR and MET as the most common mutated genes. CASE DESCRIPTION: We describe the case of a metastatic patient with 37 months overall survival, treated by an aggressive multimodal approach combining surgery, chemotherapy, radiotherapy and immunotherapy. To shed new light on the molecular basis for sarcomatoid component in lung carcinoma, we performed next generation sequencing analysis of the squamous and sarcomatoid component by the two sites. We demonstrated a clonal origin and hypermutability of the sarcomatous elements that may account for the good response to immunotherapy. Moreover, we identified some mutations involving TP53 and EGFR genes, targetable by already available drugs. CONCLUSIONS: We depicted a model of how a squamous cell carcinoma can differentiate during its natural history into sub-clonal populations with different features and may ultimately result in a neoplasm (i.e. pulmonary carcinosarcoma) showing clonal heterogeneity. Our data might contribute to a better understanding of the pathogenesis and molecular mechanisms of this rare tumor and open new ways for a more tailored approach.

A case of cervical fasciitis complicated by mediastinitis and pre-peritoneal abscess: the role of video-assisted thoracoscopic surgery.

Cervical necrotizing fasciitis (CNF) is the name for a group of bacterial infections characterized by necrosis of the fascia and subcutaneous cellular tissue of the neck. Descending necrotizing mediastinitis (DNM) is a well-known and feared complication of CNF that severely affects the prognosis. Abdominal involvement is rare. The mainstay of treatment is prompt and aggressive medical therapy and surgical debridement of all involved sites. The role of video-assisted thoracoscopic surgery (VATS) in this setting, even if already reported in the literature, is not universally adopted. We present a case of CNF complicated by DNM and pre-peritoneal involvement, treated with open surgical drainage of the neck and video-assisted thoracoscopic debridement of the mediastinal space.

Pancreatic endocrine tumors in multiple endocrine neoplasia type 1 syndrome: review of literature.

OBJECTIVE: To analyze the surgical approach to multiple endocrine neoplasia type 1 (MEN 1)-related pancreatic endocrine tumors (PETs). METHODS: We reviewed selected publications and our personal experience with MEN 1-associated PETs to delineate their general characteristics, current practice and controversies, preoperative imaging and intraoperative assessment, and appropriate therapeutic strategies including radical surgical procedures. RESULTS: The penetrance of PETs in the setting of MEN 1 is similar to that of parathyroid tumors, even though hyperparathyroidism is usually the first manifestation of MEN 1 syndrome. In contrast with the sporadic counterparts, MEN 1-related PETs are characterized by an early onset, multiplicity of lesions, variable expression of the tumors, and propensity for malignant degeneration. Both the histologic type and the size of these tumors correlate with malignant potential. CONCLUSION: The rationale for surgical considerations for these tumors is to curtail the malignant progression of the disease and to cure or aid in management of the associated biochemical syndromes. A surgical procedure is often the treatment of choice for PETs in patients with MEN 1. Monitoring of pancreatic peptides and use of diagnostic imaging allow an early pancreatic resection, in conjunction with prevention of metastatic PETs and improvement of long-term survival. Hepatic metastatic lesions can be successfully treated by surgical resection.

Is total parathyroidectomy the treatment of choice for hyperparathyroidism in multiple endocrine neoplasia type 1?

OBJECTIVE: The aim of the present report is to describe the results obtained with total parathyroidectomy (TPTX) guided by rapid intraoperative parathyroid hormone (PTH) evaluation, followed by immediate parathyroid autograft with fresh tissue. SUMMARY BACKGROUND DATA: Surgery for hyperparathyroidism (HPT) in multiple endocrine neoplasia type 1 (MEN1) is performed with various surgical approaches. METHODS: We report our 16-year experience of surgical treatment of 51 MEN1-HPT patients using TPTX and thymectomy. Forty-five patients underwent TPTX as the first surgical procedure, whereas for 6 patients, a parathyroid operation was the second surgical procedure. PTH intraoperative values less than 10 pg/mL, at the end of the surgery, were indicative for reimplantation of a few fragments ( approximately 7) of fresh parathyroid tissue in the brachioradial muscle of the forearm. Parathyroid autograft was performed in all patients, except 3 in whom the fourth parathyroid gland was not found. RESULTS: Persistent hypoparathyroidism occurred in 13 patients (25%), with higher incidence in patients undergoing a second surgical revision for cervical recurrence than in patients submitted to the first surgery. At follow-up, 5 recurrences ( approximately 10%) in the forearm were observed after a mean time of 7 +/- 5 (M +/- SD) years. No cervical recurrence was documented. The forearm recurrence was treated with removal of 1 or 2 enlarged fragments obtaining the resolution of HPT in all but 1 case. CONCLUSIONS: Based on the occurrence of complications in our experience, TPTX followed by autograft and guided by intraoperative PTH monitoring represents a better surgical option in MEN1-HPT compared with other surgical approaches.

Pancreatectomy in multiple endocrine neoplasia type 1-related gastrinomas and pancreatic endocrine neoplasias.

OBJECTIVE: The aim of this study was to evaluate the results of pancreatic resection in pancreatic endocrine neoplasias (PENs) in patients affected by multiple endocrine neoplasia type 1 (MEN1) syndrome. BACKGROUND: Since these tumors often show an indolent course, the role of diagnostic procedures and type of surgical approach are controversial. Experience with new diagnostic approaches and more aggressive surgery is still limited. METHODS: Sixteen MEN1 patients were referred to our Surgical Unit (1992-2003) and were operated on for the indications of hypergastrinism, hypoglycemia, and/or pancreatic endocrine neoplasias larger than 1 cm. Zollinger-Ellison syndrome (ZES) was present in 13 patients, 2 of whom experienced a recurrence after previous surgery. Preoperative tumor localization was carried out using ultrasonography (US), computed tomography (CT), endoscopic ultrasonography (EUS), somatostatin receptor scintigraphy (SSRS), or selective arterial secretin injection (SASI). Rapid intraoperative gastrin measurement (IGM) was carried out in 8 patients, and 1 patient also underwent an intraoperative secretin provocative test. RESULTS: Either pancreatoduodenectomy (PD) or total pancreatectomy (TP) or distal pancreatectomy was performed. There was no postoperative mortality; 37% complications included pancreatic (27%) and biliary (6%) fistulas, abdominal collection (6%), and acute pancreatitis (6%). EUS and SSRS were the most sensitive preoperative imaging techniques. At follow-up, 10 of 13 hypergastrinemic patients (77%) are currently eugastrinemic with negative secretin provocative test, while 3 are showing a recurrence of the disease. All patients affected by insulinoma were cured. CONCLUSIONS: MEN1 tumors should be considered surgically curable diseases. IGM may be of value in the assessment of surgical cure. Our experience suggests that PD is superior to less radical surgical approaches in providing cure with limited morbidity in MEN1 gastrinomas and pancreatic neoplasias.

Comparison of Wirsung-jejunal duct-to-mucosa and dunking technique for pancreatojejunostomy after pancreatoduodenectomy.

BACKGROUND: Pancreato-enteric reconstruction after pancreatoduodenectomy (PD) is still a source of debate because of the high incidence of complications. Among the various types of pancreato-jejunostomies we don't know yet which is the best in terms of anastomotic failure and related complications rates. Wirsung-jejunal duct-to-mucosa anastomosis (WJ) and "dunking" pancreato-jejunal anastomosis (DPJ) are the two most used ones worldwide but conflicting results are reported. To determine which is the safer anastomosis and to define when an anastomosis should be preferred, we retrospectively reviewed two groups of patients who underwent WJ or DPJ. METHODS: Twenty-three patients underwent PD with WJ (n = 17) with dilated (WJD) (n = 9) or not-dilated Wirsung's duct (WJND) (n = 8) or with a DPJ (n = 6) over a 3-year period at a single institution. RESULTS: The complications rate was high in all groups of patients (33.3% in WJD, 37.5% in WJND and 66.7% in DPJ). A pancreatic fistula developed in one patient in each group (11.1% in WJD, 12.5% in WJND and 16.7% in DPJ). All these patients were managed conservatively. Anastomotic disruption took place in the WJ patients especially in the WJND group (n = 2) compared to the WJD (n = 1) (25% vs. 11.1%) or DPJ groups (0%): these three patients required a re-operation. Overall, the anastomotic defects were higher in patients who underwent WJND (37.5%), compared to WJD (22.2%) and to DPJ (16.7%). However, no statistical differences were found among the groups. Delayed gastric emptying (DGE) and total parenteral nutrition (TPN) along with anastomotic defects were responsible for a prolonged hospital stay. CONCLUSIONS: Our results were not able to demonstrate any statistical difference between the two different techniques in preventing anastomotic failure. WJ can represent a valid choice in case of a dilated duct and a firm, fibrotic enlarged gland that could not be properly invaginated in a small jejunal loop. DGE may occur in those patients who experienced an anastomotic failure and required a TPN regimen with a prolonged hospital stay.

Small diameter H-graft porta-caval shunt performed at different stages of liver disease.

BACKGROUND: Partial porto-systemic shunts have been popularized because of reported low rate of mortality and morbidity (especially encephalopathy, liver failure and occlusion). To further investigate these assumptions, we retrospectively reviewed the results of partial porta-caval shunts performed at different stages of liver disease. METHODS: Twenty-nine cirrhotic patients underwent a partial porta-caval shunt with a ringed polytetrafluoroethylene interposition prosthesis of 8-mm (20 patients) or 10-mm (9 patients) in diameter. Pre and post-shunt porta-caval pressure was measured in all patients. Twelve patients (41.4%) belonged to Child A, 11 Child B (37.9%), and 6 Child C (20.7%). Eleven patients (37.9%) suffered from hepatic encephalopathy preoperatively. Twelve patients (41%) were operated on in emergency/urgency. RESULTS: Porta-caval pressure gradient, reduced significantly using either 8- or 10-mm prosthesis. The overall early mortality and morbidity were 13.8% and 48% respectively. The early mortality and morbidity were different between patients of Child A and B when compared to those of Child C (0 vs 66.6% and 34.8% vs 66.6% respectively). No patient re-bled early from varices. The overall late mortality and morbidity were 40% and 64% respectively. Shunt thrombosis and stenosis took place in 16% and 8% of the two groups of patients respectively; variceal re-bleeding occurred in 4 patients (16%). Encephalopathy occurred postoperatively in 5 patients (20%), acute in 3 patients (12%), and chronic in 2 (8%). The actuarial survival rate at 3 and 5 years was 92% and 75% for patients of Child A, 70% and 60% for patients of Child B, and 0% for patients of Child C. CONCLUSIONS: Our results indicate that partial porta-caval shunt with a small diameter interposition H-graft is an effective procedure for the treatment of variceal bleeding, as well as for the prevention of re-bleeding in patients of Child A and those of Child B, as an elective or emergency/urgency procedure, with a low rate of complications and encephalopathy. This technique could be used safely in patients with good liver function but they should be monitored closely because of the risk of shunt occlusion.