Angela Maria Rosaria Ferrante

The Italian registry for adrenal cortical carcinoma: Analysis of a multiinstitutional series of 129 patients

BACKGROUND Adrenal cortical carcinoma is an uncommon tumor with a poor prognosis. The low incidence of this tumor makes it difficult to achieve reliable data on clinical manifestations, natural history, and the impact of therapies. The purpose of this study was to evaluate such aspects in a large series. METHODS A retrospective series of 129 cases (55 men and 74 women, mean age of 49 years) was collected from 18 surgical institutions. At the time of diagnosis 45.7% of patients had endocrine symptoms. One hundred twenty-four patients underwent surgery, which was considered curative in 91 cases and palliative in 33. Sixty-three patients had local disease, 48 had regional disease, and 43 had distant metastases. RESULTS This study confirmed a higher incidence in the 40- to 50-year-old population with a female prevalence; hormonal hyperincretion was more common in women, but it was not caused by advanced disease. The overall 5-year survival rate was 35%. Tumor stage and curative resection affected prognosis significantly. The influence of gender, side, age, and hormonal function has not been confirmed. Adjuvant therapies were ineffective in prolonging survival. Reoperated patients experienced better survival (mean, 41.5 months) than nonreoperated cases (mean, 15.6 months). CONCLUSIONS The poor prognosis of adrenal cortical carcinoma may be improved by early diagnosis and complete resection. Radical surgery is the sole effective therapy, particularly in early stages. Surgical treatment of recurrence seems to improve survival and should be attempted systematically. Adjuvant therapies obtained contrasting results, and their role should be evaluated in prospective multicentric trials.

Role of reoperation in recurrence of adrenal cortical carcinoma: results from 188 cases collected in the Italian National Registry for Adrenal Cortical Carcinoma.

BACKGROUND Recurrence of adrenal cortical carcinoma (ACC) after radical surgery is a common finding. Although successful reoperations have been reported with encouraging results, most published experiences are anecdotal and based on few cases. We report the results of surgical treatment for recurrent ACC in a multiinstitutional series. METHODS One hundred eighty-eight cases of ACC were collected in a national registry. A complete follow-up was obtained in 179 cases. At initial diagnosis 92 patients had local disease (stage I or II). One hundred seventy patients underwent surgical treatment, considered radical in 140; in this group, recurrent disease was observed in 52 cases (37%) after a mean disease-free interval of 21.7 months. RESULTS Adjuvant chemotherapy was ineffective in ameliorating the prognosis. The mean survival in 20 patients who underwent reoperation was significantly higher (15.85 +/- 14.9 months) than in nonreoperated cases (3.2 +/- 2.9 months). Five-year actuarial survival in reoperated patients is significantly better than in nonreoperated patients (49.7% versus 8.3%, respectively). CONCLUSIONS Although the prognosis of this tumor is still poor, surgery is the only effective therapy; reoperation allows survival comparable to that observed in patients without recurrent disease. An aggressive strategy for recurrent ACC is advisable until prospective studies demonstrate a real effectiveness for chemotherapy.

Adrenal cystic lesions: Report of 12 surgically treated cases and review of the literature

Adrenal cysts are rare (0, 064%–0, 18% in autopsy series) and less than 500 cases have been reported in the western literature. Incidental diagnosis of adrenal cysts, however, is reported with increasing rates. We observed 12 patients with adrenal cyst. Each of them had a careful laboratory and instrumental evaluation; all the patients were operated. In our series about 67% of the patients were symptomatic (6 patients with abdominal pain, 1 with palpable mass, 1 with hemorrhagic shock). No biochemical alteration was observed. Conversely we observed an unusual subclinically hyperfunctioning cystic adenoma, potentially progressive to a clinically recognizable endocrine syndrome. US, CT and MRI had a sensitivity of 66, 7%, 80% and 100% respectively. Adrenalectomy was performed in all patients. The pathological findings were: 1 epithelial cyst (cystic adenoma), 2 endothelial cysts (vascular cystic ectasia with adenomatous adrenocortical hyperplasia and 1 vascular cyst) and 9 pseudocysts. On the basis of these results, we conclude that a careful hormonal, morpho-functional and instrumental evaluation is indicated in all adrenal cysts, even if the available diagnostic procedures, even when combined, cannot always define their nature. Surgical excision, when possible by laparoscopic approach, is indicated in presence of symptoms, endocrine abnormalities (even when subclinic), complications, suspicion of malignancy and/or large size (>5 cm). Adrenal gland must be excised en bloc, also because of the possible presence of other adrenal lesions.

Prognostic factors in differentiated thyroid carcinoma: a multivariate analysis of 234 consecutive patients

Background and Objectives: The clinical characteristics and patient outcome of a group of patients treated for differentiated thyroid carcinoma (DTC) were analyzed in order to assess the relative influence of different prognostic factors.

Case series of resection of pelvic leiomyoma extending into the right heart: surgical safeguards and clinical follow-up

Objective To analyze the clinical features, surgical management and oncologic results of a series of six patients undergoing seven operations for resection of uterine leiomyoma extending into the right cardiac chambers. Methods A retrospective review of patients operated on for surgical resection of a pelvic leiomyomatous mass originating from the uterus and extending into the right cardiac chambers was performed. The most common symptoms at presentation were syncope and dyspnea; two patients were asymptomatic. Four patients had been misdiagnosed as having intracardiac thrombus or primary cardiac tumor. The intracardiac and upper intracaval portion was removed under circulatory arrest in moderate hypothermia; the remaining portion was removed by caval incision. In one patient with cardiogenic shock, the sole intracardiac portion of the mass was removed at primary surgery. A mean of 2.8 ± 1.5 years of follow-up was available, consisting of clinical and radiological tests (computed tomography scan, echocardiography). Results There were no cases of operative mortality in the present series. No recurrence was observed at the end of the follow-up in all cases of complete resection of the mass from its intracardiac to its pelvic end. Conversely, in the only case in which partial resection was performed due to the patients clinical condition, recurrence of the intracardiac involvement was observed 6 months after primary surgery. Conclusion Radical resection is curative for uterine leiomyomatosis extending into the right cardiac chambers. Surgery can be afforded with acceptable risks. A high level of suspicion for intracardiac extension of pelvic leiomyomatosis should be retained in the presence of a floating mass within the right cardiac chambers. Such a finding should prompt radiographic evaluation of the abdomen and the pelvis.

Digestive and nutritional consequences of pancreatic resections. The classical vs the pylorus-sparing procedure.

SummaryDigestive and nutritional alterations are a common occurrence after pancreatic resections. The authors report the results of a multiparametric evaluation performed in a group of 26 patients submitted to total or cephalic pancreatectomy. Patients were divided into two groups according to the surgical procedure; group A(n=13) included gastroresected patients and group B (n=13) included those submitted to pylorus-sparing pancreatic resection. Subclinical digestive and absorptive impairment has been found in 61.5% of group A patients; the nutritional status was clinically poor in four cases from the same group. Digestive alterations have also been found in 69.2% of group B cases, but nutritional status was always satisfactory in the whole group. The more positive results obtained with the pylorus-sparing technique encourage wider adoption of this procedure.

Paroxystic hypertension in a long-term hemodialyzed patient. Successful adrenalectomy for a dopamine-producing pheochromocytoma

Pheochromocytoma (Pheo) is an uncommon neoplasm producing blood pressure troubles and it may be undiagnosed in chronic dialyzed patients in whom hypertension is a common finding. The symptoms in Pheo syndrome depends on the prevalent catecholamine released, the most common being epinephrine (E) and norepinephrine (NE). Recently, a particular clinical picture has been described for dopamine (DA)-producing Pheos, in whom a normo-hypotensive status is more often observed. The authors report a case of mainly dopamine-producing Pheo in a long-term dialyzed patient, successfully treated with adrenalectomy. The main steps in diagnosis and preoperative management are described and debated also in view of the particular background produced by the end-stage renal failure. The common imaging techniques adopted for adrenal medullary neoplasms (US, CT, MIBG scintiscan) confirmed to be decisive for diagnosis; HPLC assay of plasma catecholamines is the only biochemical test available in these patients although its significance is questionable due to the poor knowledge of catecholamine metabolism in chronic renal failure. The clinical findings observed in this case seem in disagreement with those already reported in DA producing Pheos. Pheo in hemodialyzed patients is a rare event and it may be hidden by other more common causes of hypertension. However, more awareness from the medical staff allows to diagnose the neoplasm correctly by the currently available methods and to plan a safe surgical therapy also in high-risk patients.

Lectin Pathway of Complement Activation Is Associated with Vulnerability of Atherosclerotic Plaques

Inflammatory mechanisms may be involved in atherosclerotic plaque rupture. By using a novel histology-based method to quantify plaque instability here, we assess whether lectin pathway (LP) of complement activation, a major inflammation arm, could represent an index of plaque instability. Plaques from 42 consecutive patients undergoing carotid endarterectomy were stained with hematoxylin-eosin and the lipid core, cholesterol clefts, hemorrhagic content, thickness of tunica media, and intima, including or not infiltration of cellular debris and cholesterol, were determined. The presence of ficolin-1, -2, and -3 and mannose-binding lectin (MBL), LP initiators, was assessed in the plaques by immunofluorescence and in plasma by ELISA. LP activation was assessed in plasma by functional in vitro assays. Patients presenting low stenosis (≤75%) had higher hemorrhagic content than those with high stenosis (>75%), indicating increased erosion. Increased hemorrhagic content and tunica media thickness, as well as decreased lipid core and infiltrated content were associated with vulnerable plaques and therefore used to establish a plaque vulnerability score that allowed to classify patients according to plaque vulnerability. Ficolins and MBL were found both in plaques’ necrotic core and tunica media. Patients with vulnerable plaques showed decreased plasma levels and intraplaque deposition of ficolin-2. Symptomatic patients experiencing a transient ischemic attack had lower plasma levels of ficolin-1. We show that the LP initiators are present within the plaques and their circulating levels change in atherosclerotic patients. In particular, we show that decreased ficolin-2 levels are associated with rupture-prone vulnerable plaques, indicating its potential use as marker for cardiovascular risk assessment in atherosclerotic patients.

C-reactive protein binds to cholesterol crystals and co-localizes with the terminal complement complex in human atherosclerotic plaques

Inflammation is a part of the initial process leading to atherosclerosis and cholesterol crystals (CC), found in atherosclerotic plaques, which are known to induce complement activation. The pentraxins C-reactive protein (CRP), long pentraxin 3 (PTX3), and serum amyloid P component (SAP) are serum proteins associated with increased risk of cardiovascular events and these proteins have been shown to interact with the complement system. Whether the pentraxins binds to CC and mediate downstream complement-dependent inflammatory processes remains unknown. Binding of CRP, PTX3, and SAP to CC was investigated in vitro by flow cytometry and fluorescence microscopy. CRP, PTX3, and SAP bound to CC in a concentration-dependent manner. CRP and PTX3 interacted with the complement pattern recognition molecule C1q on CC by increasing the binding of both purified C1q and C1q in plasma. However, CRP was the strongest mediator of C1q binding and also the pentraxin that most potently elevated C1q-mediated complement activation. In a phagocytic assay using whole blood, we confirmed that phagocytosis of CC is complement dependent and initiated by C1q-mediated activation. The pathophysiological relevance of the in vitro observations was examined in vivo in human atherosclerotic plaques. CRP, PTX3, and SAP were all found in atherosclerotic plaques and were located mainly in the cholesterol-rich necrotic core, but co-localization with the terminal C5b-9 complement complex was only found for CRP. In conclusion, this study identifies CRP as a strong C1q recruiter and complement facilitator on CC, which may be highly relevant for the development of atherosclerosis.

Controversial results of the Revised Cardiac Risk Index in elective open repair of abdominal aortic aneurysms: Retrospective analysis on a continuous series of 899 cases

BACKGROUND Low reliability of Revised Cardiac Risk Index (RCRI) in predicting major cardiac events (MACE) among Vascular Surgery patients emerged in recent literature, suggesting procedure-specific risk evaluation - particularly in major surgery. METHODS-RESULTS Comorbidities, perioperative variables, RCRI and MACE were retrospectively analyzed in a consecutive series of 899 elective open abdominal aortic aneurysm (AAA) repairs performed at our Institution. Possible MACE predictors were studied through univariate/multivariable analysis (logistic regression, MVRE) and stepwise-backward elimination/odds ratio (MVR-SBE/OR). Patients were divided by clampsite in 2 subgroups: 1. infrarenal (690 cases); 2. pararenal (209 cases). RCRI resulted predictive for MACE in the whole dataset but its performance resulted lower for pararenal aneurysms (p = 0.11) than for infrarenal ones (p ≤ 0.00). Among RCRI covariates of the whole cohort, dilated cardiomyopathy (p ≤ 0.001), ischemic cardiopathy (p ≤ 0.01) and cerebrovascular disease (p ≤ 0.02) resulted predictive. Peripheral arteriopathy also related to MACE (p ≤ 0.03). At MVR-SBE/OR analysis, the following resulted to be MACE predictors: dilated cardiomyopathy (p ≤ 0.001), cerebrovascular disease (p ≤ 0.02), and surgical access (p = 0.04) in subgroup 1; previous myocardial infarction (p ≤ 0.01), congestive failure (p ≤ 0.03) and chronic pneumopathy (p = 0.04) in subgroup 2. CONCLUSIONS Predictability of RCRI in elective AAA surgery is influenced by clampsite and resulted to be lower in aneurysms requiring suprarenal clamping. Variables included in the RCRI show to have different weights when patients are stratified by clampsite. Some variables not included in the RCRI model significantly affect the onset of MACE. RCRI should be revised to elaborate a specific score for AAAs including further MACE predictors, to improve risk assessment and to support proper surgical strategy.