A very advanced case of a T cell peritoneal lymphomatosis.

Small-bowel lymphoma is not a common disease, accounting for 15-20% of primary extranodal gastrointestinal lymphomas. Peritoneal lymphomatosis is considered a rare and aggressive presentation. We describe the case of a 55 years-old man affected by T-cell intestinal lymphoma, presenting with diffuse abdominal involvement, bowel dysfunction, severe ascites and pleural effusion, who underwent surgery. Clinical course led dramatically to death. Preoperative cytology and radiologic investigations did not yield diagnosis and were unable to differentiate between peritoneal carcinosis and lymphomatosis. It is suggested that, in such advanced cases, with rapidly deteriorating clinical conditions and huge systemic involvement, surgery is not indicated. On the contrary, maximum effort has to be spent to obtain a preoperative diagnosis.

Neoadjuvant accelerated concomitant boost radiotherapy and multidrug chemotherapy in locally advanced rectal cancer: a dose-escalation study.

OBJECTIVES: To determine the maximal and safely dose of preoperative radiotherapy and concurrently intensified chemotherapy regimen (raltitrexed plus oxaliplatin) in locally advanced rectal cancer patients. METHODS: Patients with cT3-T4 and/or cN≥1 or locally recurrent rectal cancer were sequentially assigned to 4 treatment schedules of chemoradiation: standard radiotherapy (50.4 Gy/5.5 wk) plus raltitrexed (cohort A), accelerated radiotherapy (55 Gy/5 wk) plus raltitrexed (cohort B), standard radiotherapy plus raltitrexed and oxaliplatin (cohort C), accelerated radiotherapy plus raltitrexed and oxaliplatin (cohort D). Patients were treated in cohorts of 6 to 12 per group. The maximal tolerated dose was exceeded if more than one-third of patients in a given cohort experienced dose-limiting toxicity (DLT). DLT was defined as any grade ≥3 toxicity according to the Radiation Therapy Oncology Group criteria. RESULTS: Forty-six consecutive patients were enrolled. In cohort A, 6 patients received the planned treatment with no DLT. In cohort B, 1 of 8 patients experienced a DLT. In cohort C, a DLT occurred in 2 of 6 patients and therefore, a cohort expansion was required. Three of 16 patients treated at this dose level experienced a DLT. In addition, cohort D was expanded and DLT was found in 4 of 16 patients. Therefore, the maximal tolerated dose was not exceeded at any treatment level. CONCLUSIONS: An intensified regimen of chemoradiotherapy delivering raltitrexed and oxaliplatin concurrently with concomitant boost radiotherapy (55 Gy/5 wk) can be safely administered in patients with locally advanced rectal cancer. On the basis of these results, this intensified regimen could be tested in a phase II study.

[Gastrointestinal stromal tumors]. / Tumori stromali gastrointestinali.

Gastrointestinal stromal tumor (GIST) account for 1% of all gastrointestinal neoplasms and are the most common mesenchymal tumor of gastrointestinal tract. There are considered to originate fom the intestinal cell of Cajal, an intestinal pacemaker cell, characterized usually express the KIT protein on immunohistochemistry. The stomach (40-60%) and small intestine (30-40%) are the most common locations. Diagnosis of these tumors is difficult to establish, because symptoms are vague and traditional diagnostic tests are not specific. GISTs shows a wide variety of clinical behaviours ranging fom benign to frankly malignant, making the outcome totally unpredictable. Surgery is the standard treatment of local GIST while Imatinib (tyrosine kinasi inhibitor) is considered as the standard treatment of metastatic disease. Resistence to Imatinib is also becoming a major clinical problem but new tirosyne kinase inibitor are being studied to improve the treatment and survival. The present paper is a review of the salient features of epidemiology, pathophysiology, diagnosis, therapy and prognostic factors of GIST

[Presentation, treatment and prognosis of intraductal papillary mucinous neoplasm]. / Aspetti clinici, trattamento e prognosi della neoplasia papillare intraduttale mucosecernente.

Intraductal papillary mucinous neoplasms (IPMNs) are rare tumours rising from the pancreatic duct epithelium. They are characterized by intraductal papillary growth and thick mucin secretion; mucin fills the Wirsung and/or branch pancreatic ducts and may cause ductal dilatation. IPMNs are classified into three types, according to the site of involvement: main duct type, branch duct type, and combined type. Most branch type IPMNs are benign, while the other two types are frequently malignant. Recent advances in diagnostic imaging have led to an increased frequency of diagnosis of IPMNs, but the clinical features of them can range broadly from benign, borderline, and malignant non-invasive to invasive lesions, and their management has not yet been clearly defined. The most of patients are asymptomatic. The possibility of malignancy is increased in cases which large mural nodules are presented. Presence of a large branch type IPMN and marked dilatation of the main duct indicate the existence of adenoma. Not infrequently, synchronous or metachronous malignancies may be developed in various organs. Endoscopic retrograde cholangiopancreatography (ERCP), endoscopic ultrasonography (EUS), intraductal ultrasonography, and magnetic resonance cholangiopancreatography (MRCP) are the most valuable imaging techniques for diagnosis of these lesions. Prognosis is excellent after complete resection of benign and non-invasive malignant IPMNs. Total pancreatectomy should be reserved for patients with resectable but extensive IPMN involving the whole pancreas; its benefits must be balanced against perioperative risks.

Risk factors associated with pancreatic fistula after distal pancreatectomy, which technique of pancreatic stump closure is more beneficial?

AIM: To identify risk factors related to pancreatic fistula in patients undergoing distal pancreatectomy (DP) and to determine the effectiveness of using a stapled and a sutured closed of pancreatic stump. METHODS: Sixty-four patients underwent DP during a 10-year period. Information regarding diagnosis, operative details, and perioperative morbidity or mortality was collected. Eight risk factors were examined. RESULTS: Indications for DP included primary pancreatic disease (n=38, 59%) and non-pancreatic malignancy (n=26, 41%). Postoperative mortality and morbidity rates were 1.5% and 37% respectively; one patient died due to sepsis and two patients required a reoperation due to postoperative bleeding. Pancreatic fistula was developed in 14 patients (22%); 4 of fistulas were classified as Grade A, 9 as Grade B and only 1 as Grade C. Incidence of pancreatic fistula rate was significantly associated with four risk factors: pathology, use of prophylactic octreotide therapy, concomitant splenectomy, and texture of pancreatic parenchyma. The role that technique (either stapler or suture) of pancreatic stump closure plays in the development of pancreatic leak remains unclear. CONCLUSION: The pancreatic fistula rate after DP is 22%. This is reduced for patients with non-pancreatic malignancy, fibrotic pancreatic tissue, postoperative prophylactic octreotide therapy and concomitant splenectomy.

Presentation and management of pancreatic cystic neoplasms.

Pancreatic cystic neoplasms are less frequent than other pancreatic tumors, but because of the wide availability and improvement of modern imaging methods, these neoplasms are being recognized with increasing frequency and it is often possible to be differentiated preoperatively not only from other cystic pancreatic disorders but also from one another. Most patients have no symptoms while clinical signs are not really useful in the clinical work up, and when they are present, they never help us to identify the type of pathology. Treatment differs with the diagnosis. Serous cystic neoplasms are uniformly benign and usually do not mandate resection unless this lesion is symptomatic. In contrast, mucinous cystic neoplasms and intraductal papillary mucinous neoplasms have a premalignant or malignant tendency, and therefore should be managed aggressively by pancreatic resection; in the absence of invasive disease, prognosis is excellent after appropriate surgery, but the presence of invasive malignancy signifies a poor prognosis. Solid pseudopapillary neoplasms have nonaggressive behavior and their management is related to the extension of the disease. The purpose of this article is to review the types of pancreatic cystic neoplasms, their diagnosis, indications for surgical treatment, and outcome.

Acute limb compartment syndrome: a review.

Acute limb compartment syndrome (LCS) is a limb-threatening and occasionally life-threatening condition caused by bleeding or edema in a closed muscle compartment surrounded by fascia and bone, which leads to muscle and nerve ischemia. Well-known causative factors are acute trauma and reperfusion after treatment for acute arterial obstruction. Untreated compartment syndrome usually leads to muscle necrosis, limb amputation, and, if severe, in large compartments, renal failure and death. Alertness, clinical suspicion of the possibility of LCS, and occasionally intracompartmental pressure (ICP) measurement are required to avoid a delay in diagnosis or missed diagnosis. Open fasciotomy, by incising both skin and fascia, is the most reliable method for adequate compartment decompression. The techniques of measuring ICP have advantages and disadvantages, whereas the pressure level that mandates fasciotomy is controversial. Increased awareness of the syndrome and the advent of measurements of ICP pressure have raised the possibility of early diagnosis and treatment. This review reports LCS, including etiology, pathophysiology, diagnosis, ICP measurement, management, and outcome.

[Retroperitoneal Castleman's disease. A report of two cases and analysis of the literature]. / Malattia di Castleman retroperitoneale. Due casi clinici e analisi della letteratura.

Castleman's disease is a rare disorder of the lymphoid tissue with three possible histological variants--the hyalin-vascular type, with a good prognosis, the plasma-cellular type and the mixed type; the latter two are both more aggressive than the hyalin-vascular type. Two clinical types of this disease have already been described: the localized or unifocal type and the multicentric or multifocal type. The aetiology of Castleman's disease remains unclear due to polymorphic clinical features that give rise to many diagnostic and treatment problems. Its diagnosis, therefore, can only be confirmed by histological examination. Surgical treatment is the treatment of choice in patients with Castleman's disease, but radical removal of the tumour mass, especially in the multicentric type, is not always possible. We are still in no position to draw definitive conclusions as to treatment, because there are only a few reports with different regimens regarding patients with multicentric Castleman's disease. A better understanding of the pathogenesis of this rare disorder may help in deciding the best treatment approach. In this study, we report two cases of Castleman's disease, one hyalin-vascular and the other plasma-cellular, both of which were unifocal and located in a retroperitoneal-pararenal site. We also analyse the main clinical, diagnostic and treatment problems associated with this rare condition, with an overview of the literature.

Surgical management of chronic pancreatitis.

BACKGROUND: Treatment of chronic pancreatitis (CP) is a challenging condition for surgeons. During the last decades, increasing knowledge about pathophysiology of CP, improved results of major pancreatic resections, and integration of sophisticated diagnostic methods in clinical practice have resulted in significant changes in surgery for CP. DATA SOURCES: To detail the indications for CP surgery, the surgical procedures, and outcome, a Pubmed database search was performed. The abstracts of searched articles about surgical management of CP were reviewed. The articles could be identified and further scrutinized. Further references were extracted by cross-referencing. RESULTS: Main indications of CP for surgery are intractable pain, suspicion of malignancy, and involvement of adjacent organs. The goal of surgical treatment is to improve the quality of life of patients. The surgical approach to CP should be individualized according to pancreatic anatomy, pain characteristics, baseline exocrine and endocrine function, and medical co-morbidity. The approach usually involves pancreatic duct drainage and resection including longitudinal pancreatojejunostomy, pancreatoduodenectomy (Whipple's procedure), pylorus-preserving pancreatoduodenectomy, distal pancreatectomy, total pancreatectomy, duodenum-preserving pancreatic head resection (Beger's procedure), and local resection of the pancreatic head with longitudinal pancreatojejunostomy (Frey's procedure). Non-pancreatic and endoscopic management of pain has also been advocated. CONCLUSIONS: Surgical procedures provide long-term pain relief, a good postoperative quality of life with preservation of endocrine and exocrine pancreatic function, and are associated with low early and late mortality and morbidity. In addition to available results from randomized controlled trials, new studies are needed to determine which procedure is the most effective for the management of patients with CP.

[Chronic pain after inguinal hernia mesh repair: possible role of surgical manipulation of the inguinal nerves. A prospective multicentre study of 973 cases]. / Il dolore cronico dopo ernioplas ica inguinale con protesi: il possibile ruolo della manipolazione chirurgica dei nervi del canale inguinale.

The aim of the study was to evaluate whether the surgical treatment reserved for the ilioinguinal, iliohypogastric and genital branches of the genitofemoral nerves, during open hernia mesh repair, is effective in reducing chronic post-operative pain. A multicentre prospective study involving 11 Italian Institutions led to the recruitment of 973 cases of hernioplasty. All surgeons were asked to report whether or not each nerve had been identified and preserved or divided. The main endpoint of the study was the evaluation of moderate-severe chronic pain at 6 months and 1 year. Overall, presence of groin pain at 6 months and 1 year follow-up was 9.7% and 4.1%, respectively. Pain was mild in 7.9% and moderate-to-severe in 2.1% at 6 months, and mild in 3.6% and moderate-to-severe in 0.5% at 1 year. Univariate and multivariate analysis showed that lack of identification of nerves is significantly correlated with presence of chronic pain, the risk of developing inguinal pain increasing with the number of nerves not detected. Likewise, division of nerves was clearly correlated with presence of chronic pain. The present findings indicate that identification and preservation of nerves during open inguinal hernia repair reduce chronic incapacitating groin pain.

Influence of preservation versus division of ilioinguinal, iliohypogastric, and genital nerves during open mesh herniorrhaphy: prospective multicentric study of chronic pain.

OBJECTIVE: To evaluate whether the various surgical treatment reserved for ilioinguinal, iliohypogastric, and genital branch of the genitofemoral nerves, during open hernia mesh repair, is effective in reducing chronic postoperative pain. BACKGROUND: Interest in chronic groin pain following herniorrhaphy has escalated, in recent years, due both to treatment and legal implications. However, much debate still exists concerning which treatment to reserve for the 3 inguinal sensory nerves. METHODS: A multicentric prospective study involving 11 Italian institutions led to the recruitment of 973 cases of hernioplasty. All surgeons were asked to report whether or not each nerve had been identified and preserved or divided. The main endpoint of the study was the evaluation of moderate to severe chronic pain at 6 months and 1 year. RESULTS: Overall, the presence of groin pain at the 6-month and 1-year follow-up was 9.7% and 4.1%, respectively. Pain was mild in 7.9% and moderate to severe in 2.1%, at 6 months, and mild in 3.6% and moderate to severe in 0.5%, at 1 year. Univariate and multivariate analysis showed that lack of identification of nerves is significantly correlated with presence of chronic pain, the risk of developing inguinal pain increasing with the number of nerves not detected. Likewise, division of nerves was clearly correlated with presence of chronic pain. CONCLUSIONS: The present findings indicate that identification and preservation of nerves during open inguinal hernia repair reduce chronic incapacitating groin pain and that, in the majority of patients with chronic pain at 6 months, the pain at 1 year is resolved only with conservative or medical treatment.

Pancreatoduodenectomy for tumors of Vater's ampulla: report on 94 consecutive patients.

Evaluation of prognostic factors of adenocarcinoma of Vater's ampulla is still a matter of debate. The aim of this study was to evaluate retrospectively factors that influence early and long-term outcomes in a 20-year single-institution experience on ampullary carcinoma. A total of 94 consecutive patients with ampullary carcinoma or adenoma with severe dysplasia were managed from 1981 to 2002. Among them, 64 underwent pancreatoduodenectomy, and the remaining 30 submitted to surgical (n = 5) or endoscopic (n = 25) palliative treatment. Demographic, clinical, and pathologic data were collected, and a comparison was made between patients who did or did not undergo resection. Standard statistical analyses were carried out in an attempt to establish a correlation between clinical variables, intraoperative and pathologic factors, and survival in patients with resection. A total of 85 (90.4%) patients had potentially resectable lesions due to the extent of the tumor, but only 64 (68%) underwent curative resection. The surgical morbidity rate was 34.3%. Postoperative mortality was 9.3%, with no deaths among the 38 more recently treated patients. Median survivals were 9 and 54 months for nonresected and resected patients, respectively. The overall 5-year survival was 64.4% for patients undergoing pancreatoduodenectomy. Survival was found to be significantly affected by resection, tumor size, tumor grade, and tumor infiltration. Patients with negative lymph nodes show a trend toward longer survival. In a multivariate analysis, only the depth of tumor infiltration influenced patient survival. Pancreatoduodenectomy is the treatment of choice for ampullary carcinoma and adenomas with high-grade dysplasia, with a good chance of long-term survival. Surgical resection remains the most important factor influencing outcome.