Long-term quiescence of ectopic Cushing's syndrome caused by pulmonary neuroendocrine tumor (typical carcinoid) and tumorlets: spontaneous remission or therapeutic effect of bromocriptine?

In 1990, a 55-yr-old woman was admitted to the Medical Department of our hospital for severe hypercortisolism complicated by secondary diabetes mellitus and serious hypokalemia. Although inferior petrosal sinus sampling did not show any significant difference between central and peripheral ACTH concentration, suggesting an ectopic source of ACTH secretion, diagnostic imaging was negative and Cushing's disease due to hyperplasia of the pituitary intermediate lobe was suspected. Medical treatment with bromocriptine and cyproheptadine led to a rapid and stabile normalization of adrenal function, so that after two months cyproheptadine was stopped and bromocriptine was tapered to a smaller dose. An attempt to discontinue medical treatment, carried out 3 yr later, was followed by a quick increase of ACTH and cortisol levels, which were normalized by the resumption of the bromocriptine. Adrenal function remained normal until 1994 when hypercortisolism relapsed despite the treatment. Chest radiography and computed tomography (CT) scan detected a 6 mm nodule in the middle lobe of the lung which proved to be a neuroendocrine tumor, with immunohistochemical positivity for ACTH. Nests of neuroendocrine cells (tumorlets) were also demonstrated in the surrounding lung tissue. After the lobectomy, the patient recovered completely from Cushing's syndrome and no symptoms and/or signs of recurrence have been observed over the subsequent follow-up period. Although cyclical spontaneous Cushing's syndrome could not be excluded, there was strong evidence that medical treatment with bromocriptine might have played a key role in long-lasting remission. To our knowledge, this is the second case described in literature of Cushing's syndrome caused by neuroendocrine lung tumor responsive to bromocriptine.

Long-term results of aggressive surgical treatment of primary and recurrent retroperitoneal liposarcomas.

The aim of this study was to establish the role of surgery in the treatment of retroperitoneal liposarcomas. Data concerning 28 patients submitted to surgery for retroperitoneal liposarcoma in our department over the period from 1972 to 1999 were reviewed retrospectively and analysed. Seventy-four operations were performed; in 54% of the operations it was necessary to resect contiguous organs (kidney 60%, colon 50%, adrenal gland 35%). In 89%, grossly curative resection was achieved at the first operation; 20 patients had at least one local recurrence after first operation (median time interval: 22 months). The mean follow-up was 80 months; median survival time was 51 months and 5-year actuarial survival time 51%. Patients with low-grade liposarcoma showed a statistically significant improvement (P < 0.001) in median survival (153 months) versus those with medium- (37 months) and high-grade sarcomas (8 months). At present surgery is still the treatment of choice in the treatment of primary and recurrent liposarcoma; in the case of low-grade liposarcomas especially, an aggressive surgical approach can result in long-term survival.

[Perforation of the esophagus during pneumatic dilatation in achalasia]. / La perforazione dell'esofago nella dilatazione pneumatica per acalasia.

Esophageal perforation is a serious complication of pneumatic dilatation. We studied the cases of 4 patients (2 men and 2 women, mean age 58 years, range 56-62) who had surgical treatment for achalasia, two of which had had previous dilatation. The main symptoms were pain and dyspnea. Pneumomediastinum was present in all patients, pleural effusion in 2 and cervical emphysema in 1. Esophagographic results showed evidence of perforation in all four cases and gastric patches were surgically placed on the esophageal tear within 12 hours. Three patients received enteral nutrition for an average of 13 days. Mean hospital stay was 14 days. No post-operative complications were exhibited although one patient did develop gastroesophageal reflux 3 months later and underwent surgery to repair a hernia in the thorax 5 years later. Early and aggressive treatment is considered the best therapy and the gastric patch, in our opinion, is an effective and reliable technique for esophageal perforation repair in achalasia patients.

Strangulated traumatic hernia of the diaphragm. A report of two cases.

The herniation of abdominal viscera in the thorax can immediately follow diaphragmatic rupture or be delayed even years after the injury. The herniated viscera can strangulate; this consequence may lead to a dangerous misdiagnosis which could be lethal for the patient. Radiological procedures, serial chest X-ray studies, CT and MRI scans are mandatory to confirm diagnosis. The insertion of a naso-gastric tube is a very helpful method in ruling out hypertensive pneumothorax in the presence of an air-fluid level in the thorax. We report 2 cases of strangulated traumatic hernia of the diaphragm occurring just a few hours (case 1) and 18 months (case 2) after the trauma. During thoracotomy, a rupture of the left diaphragmatic cupola was demonstrated with herniation of the stomach in case 1, the stomach, spleen and transverse colon in case 2. No postoperative mortality or morbidity were detected.

Non ductal-adenocarcinoma neoplasms of the pancreas.

Pancreatic Non Ductal-Adenocarcinoma Neoplasms (PNDAN) represent about 20% of pancreatic and periampullary tumors and should be considered in differential diagnosis with ductal adenocarcinoma in the presence of isolated pancreatic mass. From January 1992 to December 1998, 238 patients were operated on for pancreatic and periampullary masses. Fifty-five patients had PNDAN: 24 endocrine tumors, 7 serous cystadenomas, 6 intraductal papillary-mucinous tumors, 5 acinar carcinomas, 4 mucinous cystadenomas, 3 metastatic tumors, 2 cystic papillary tumors, 2 solid cystadenocarcinomas, 1 neurilemmoma, and 1 pancreatoblastoma; 19 were benign and 36 were malignant or borderline tumors. A correct preoperative diagnosis was obtained in 58% of the cases. In all other cases, diagnosis was achieved intraoperatively. Major (18 pancreaticoduodenectomies, 17 left splenopancreatectomies, 1 total pancreatectomy) and minor resections (5 central pancreatectomy, 10 enucleations) were performed; curative surgical operations were carried out on 39/55 patients (curative resectability: 71%). Operative mortality and morbidity were 1.8% and 21.8%, respectively. Three and 5-year actuarial survival for malignant or borderline PNDANs are 65% and 40% versus 31% (3-year) for ductal adenocarcinoma of pancreatic head treated by pancreaticoduodenectomy (p-value = 0.03). We believe that pancreatic masses that are not ductal adenocarcinomas, can be aggressively resected even if large in size, resulting in a better outcome than ductal adenocarcinoma itself.

[Thymoma, aplastic anemia, hypogammaglobulinemia and malignant pulmonary neoplasm: a case report]. / Timoma, anemia aplastica, ipogammaglobulinemia e neoplasia polmonare maligna: presentazione di un caso.

A 67 year old man presented with non-invasive thymoma, associated aplastic anemia and important hypogammaglobulinemia; the postoperative course has been characterized, three months later, by thrombocytopenia (kept under control with steroid therapy) and, two years later, by squamous lung cancer, not susceptible of surgical treatment. The patient died five years after operation because of progression of the lung cancer. Anemia improved only partially after operation; there where no effects on hypogammaglobulinemia. Thymoma has been reported in literature in 50% of patients with aplastic anemia, 7-13% of adult patients with hypogammaglobulinemia is affected by thymoma, in 21% of patients that presented with thymoma other tumors have been discovered through clinical history.

[Stromal tumors of the stomach. Our experience with 25 patients]. / I tumori dello stroma gastrointestinale a localizzazione gastrica. Nostra esperienza in 25 casi.

Stromal tumors (GIST) represent 5% of gastric neoplasms. Twenty-five patients with GIST underwent surgical operation: the tumor was benign, malignant, and borderline in 11, 12, and 2 cases, respectively. Main symptoms were abdominal pain (36%), and digestive haemorrhage (32%); 4 patients (16%) complained of abdominal mass. In 5 patients the diagnosis was incidental. Surgical operations (12 local resections, 9 partial gastric resections, and 4 total gastrectomies) were macroscopically curative in all the patients. In 3 patients the resection was extended to liver (1 case), spleen, pancreatic body-tail, and left kidney (1 case), and diaphragm (1 case) because of contiguous involvement of these organs. Postoperative mortality and morbidity were 4% and 20%, respectively. A patient with benign GIST passed away 36 months after operation because of breast cancer disease; other 9 patients are alive from 3 months to 25 years after operation. Three patients with low grade malignant GIST are well at mean follow up of 53 months. The 9 patients with high grade neoplasms are all dead (median survival time: 18 months). The 2 patients with borderline tumors are alive without evidence of disease at 3 and 8 years.

Inflammatory pseudotumor of the liver: case report and review of the literature.

Inflammatory pseudotumors of the liver (IPL) are extremely rare focal lesions of the parenchyma. Up to now, the ethology of IPL has not been completely understood. Usually the clinical presentation is with fever, chills, hepatic mass. The fine needle biopsy shows a large amount of inflammatory cells, while the most common imaging techniques are not specific and do not reach a definitive preoperative diagnosis between a benign and a malignant tumor. From the examination of the Literature, the Authors found a mortality rate of 40% among patients treated by antibiotic therapy, while surgical procedures were successful in all but one case. Moreover, in Authors' case, successfully treated by hepatic resection, the preoperative diagnostic procedures were not helpful in differential diagnosis with a malignant lesion. For these reasons, the Authors believe surgery is the best therapeutic choice in case of a suspected IPL without an early clinical resolution after antibiotic therapy.

[Diagnosis and treatment of traumatic diaphragmatic hernia with delayed presentation]. / Diagnosi e trattamento dell'ernia traumatica del diaframma a tardiva presentazione.

AIM: To evaluate the clinical picture, diagnostic techniques and most appropriate treatment in traumatic diaphragmatic hernia with delayed presentation on the basis of personal experience and in the light of other published studies. EXPERIMENTAL DESIGN: Review of cases treated. SETTING: Patients treated in University General Surgery wards. PATIENTS: Those patients in whom diagnosis was made some time after trauma and after the acute event were selected from a group of patients with traumatic diaphragmatic hernia. SURGERY: All patients underwent surgery to reduce hernia and repair the diaphragmatic lesion. MEASUREMENTS: All clinical findings were examined together with the tests performed and the type of treatment carried out. RESULTS: The diagnosis was made between 3 months and 3 years after the injury. Three patients presented manifest symptoms of high intestinal occlusion on entry. Radiological alterations were present in standard chest X-rays in all patients and digestive tract contrast radiography was positive for the diagnosis of hernia in 3 out of 4 cases in which it was performed; a preoperative diagnosis of hernia was obtained in 4 cases. Patients were operated using a thoracotomy (3 cases) or combined laparothoracotomy access (2 cases); the diaphragmatic lesion, localised in all cases in the cupula of the left hemidiaphragm, was repaired using separate sutures in non-reabsorbable material without the use of grafts. One patient died postoperatively owing to septic complications. CONCLUSIONS: traumatic diaphragmatic hernia with delayed presentation involves severe complications that increase morbidity and operating mortality.

Delayed traumatic hernia of the diaphragm presenting with hypertensive pneumothorax. Case report and review of the literature.

The diagnosis of traumatic hernia of the diaphragm can be obtained at the time of injury or months-years after the trauma. The Authors report a case of traumatic hernia of the diaphragm, diagnosed 3 years after a blunt thoracic trauma in a 47-year-old man. The patient was admitted to the hospital for a pneumothorax caused by perforation of the herniated colon. He underwent colonic resection and reduction of the herniated viscera but unfortunately he died of septic shock on the 40th postoperative day. Pneumothorax is a very rare complication of traumatic diaphragmatic hernia and few cases are reported in literature. The diagnosis in the delayed phase is not easy since the correlation with the trauma is not always clear.

[Rupture of the diaphragm caused by closed trauma. Case contributions and review of the literature]. / La rottura del diaframma da trauma chiuso. Contributo casistico e revisione della letteratura.

AIM: Retrospective evaluation of 19 diaphragmatic ruptures due to blunt trauma. MATERIALS AND METHODS: We collected all patients with thoracic and/or abdominal blunt trauma who were admitted to the department of surgery (Clinica Chirurgica and Chirurgia generale C) from 1970 to 1995. We selected patients with ascertained diaphragmatic rupture. RESULTS: We considered 17 cases of TDR (15 males and 4 females). Mean age was 38 years (range 16-67). Radiologic findings were consistent with TDR in 10 cases out of 17 (58.8%). Right hemidiaphragm was injured in 6 cases (31.6%). 10 patients (52.6%) presented at operation with intrathoracic visceral herniation. 8 patients underwent laparotomy, 7 both laparotomy and thoracotomy, 4 thoracotomy alone. Perioperative mortality was 15.7% (3 patients). DISCUSSION AND CONCLUSIONS: The clinical features were complicated by a large number of associated lesions; radiologic diagnosis is comparatively easy if visceral herniation into the thorax is present, repeated radiologic examinations facilitate diagnosis. The surgical access is determined by concomitant associated injuries which may require urgent operation.

Lymphoepithelial cyst of the pancreas. Report of two cases and review of the literature.

CONCLUSION: Lymphoepithelial cyst of the pancreas (LC) is a very rare benign lesion and preoperative diagnosis is difficult. Conservative surgery seems to be the appropriate therapy in symptomatic patients or when a precise preoperative diagnosis is not achieved. The benign behavior of all reported cases suggests that the asymptomatic patients with a certain morphological preoperative diagnosis might be clinically followed up. The histogenesis of LC remains to be elucidated. BACKGROUND: LC of the pancreas is a cyst that is histologically characterized by a fibrous tissue, a lymphoid component and a lining squamous epithelium. METHODS: Clinical and pathological findings of two personal cases are reported with review of the literature. RESULTS: A 56-yr-old man, complaining of epigastric pain, and a 47-yr-old man, with a history of alcohol abuse, were admitted to hospital. In both cases the lesion was detected with abdominal ultrasound but a certain diagnosis was obtained only after histological examination of the resected cysts.

Mastectomy and immediate breast reconstruction: oncological considerations and evaluation of two different methods relating to 88 cases.

The authors consider 88 cases of mastectomy and immediate breast reconstruction mainly performed using the skin expander plus prosthesis method or latissimus dorsi myocutaneous flaps. At the same time, 53 patients underwent contralateral mastopexy for symmetry. The mean follow-up was 21 months (range 2-102). Progressive disease was observed in nine cases: one patient presented scar relapse, one axillary relapse, two contralateral tumor, two contralateral tumor and distant metastasis, three distant metastases and one death from distant metastasis. Reconstruction complications were capsular contracture in 12 cases, infection in nine, skin necrosis in two, skin expander breakage in three and implant dislocation in one. The final result was judged good in 54 cases, fair in nine, poor in 11 and unevaluable in 14. In conclusion immediate breast reconstruction does not seem to interfere with the disease or oncological therapy. After analysing separately, and comparing the results and complications of the two main techniques used, latissimus dorsi seems to be the most reliable method in the majority of cases but skin expanders can be a good technique in patients with small and firm breasts and without complicating risk factors.

[Hepatobiliopancreatic surgery in patients over 70 years old: which anesthesia?]. / Chirurgia epatobiliopancreatica nei pazienti oltre i 70 anni: quale anestesia?

In the last years the criteria of operability have been extended to elderly patients with hepato-pancreatic-biliary diseases. We selected 46 patients (in the seventies or older, class 3 or 4 of ASA score, affected by hepato-pancreatic-biliary neoplasms) in order to evaluate the behavior of these patients undergoing to different anaesthesiological techniques. Randomly, we treated 24 patients (group A) in general anaesthesia, and 22 patients (group B) in peridural anaesthesia. We considered mortality rate, morbidity rate, as sepsis, wound infection, pleuritis, and pneumonias. The data were analyzed by chi2-test and Fisher's exact test (p < 0.05). Mortality rate was similar in the two groups (A = 4.1, B = 4.5) (p = ns), and no complications were determined by the different anesthesiologic procedures. Pleuritis was present in 44% of group A vs 45% of group B (p = ns). Atelectasis areas were present in 58% of group A vs 27% of group B (p = ns), pneumonia was present in 33% of group A vs 9% of group B: this value was significant (p = 0.049). There were no differences between the two groups regarding wound infection rate (only one case in group B). We think that pulmonary diseases can be determined by intubation and mechanical ventilation. We show a significant reduction of pneumonia in the patients that underwent peridural anaesthesia. For this reason, peridural technique can be safely extended to elderly patients with hepato-pancreatic-biliary diseases.

[Role of surgical splanchnicectomy in the treatment of pancreatic carcinoma]. / Il ruolo della splancnicectomia chirurgica nel trattamento del carcinoma pancreatico.

Palliative surgical procedures offer considerable benefit for the patients with unresectable pancreatic cancer: surgical splanchnicectomy performed in conjunction with biliary-enteric by-pass offers good results as regard pain relief without increased morbidity and mortality. We treated 25 patients with unresectable pancreatic cancer by mean of biliary-enteric by-pass plus bilateral splanchnicectomy performed through different surgical approaches. In this series of patients postoperative mortality was nil, mean survival time was 7.2 months (range 3-14 months). Preoperatively, we assessed all patients as affected by visceral pain: Scott-Huskisson 10 mark-scale value in quantitative assessment of pain was equal or above the 7th mark in 87.5% of patients. One month later in the postoperative follow-up, 96% of the patients had a significant reduction in pain intensity from a preoperative median of 7 mark to a postoperative median of 1.5 mark (p = 0.0001). The mean period free of pain recurrence was 4.8 months. However, after 6 months only 46% of survivors were pain-free with such rate decreasing further to a 10% of survivors after 8 months. Nevertheless, the patients had around 70% of their survival span free of pain. We strongly believe that failure in relief of pain is due to a mistake in preoperative evaluation of the type of pain (somatic and not visceral, or both) and to the onset of somatic pain in the course of the disease rather than to surgical technical errors. Recurrence of pain has been considered inevitable in the biological progression of unresected cancer, and would be treated by combination of therapies, such as non steroidal anti-inflammatory drugs, transaortic coeliac plexus block, narcotics and cervical cordotomy.

Retroperitoneal paragangliomas: role of immunohistochemistry in the diagnosis of malignancy and in assessment of prognosis.

OBJECTIVE: To present our experience of 10 patients with extra-adrenal retroperitoneal paragangliomas, and assess prognostic tests. DESIGN: Retrospective study of casenotes. SETTING: University hospital, Italy. SUBJECTS: 10 Patients who presented with paragangliomas between 1970 and 1991. MAIN OUTCOME MEASURES: Histological and immunohistochemical results, and outcome. RESULTS: All tumours were completely resected and there was no operative mortality. Of the 8 patients who had no metastases at presentation 3 died of recurrence 3, 5, and 10 years later, respectively; 4 were alive and free of disease 2-7 years after diagnosis. The 2 patients with synchronous bone metastases at presentation died 1 and 4 years later. Immunohistochemical analysis of type I cells (chromogranin A and neurone-specific enolase) showed little correlation with progression of disease, but there was a correlation between the presence of type II cells (S100 protein) and good prognosis. CONCLUSIONS: Excision is the treatment of choice for paraganglioma. Immunohistochemical techniques may provide useful information about prognosis, in particular about those patients who are at increased risk of recurrence. Long term follow up is essential, because successful management of recurrence is dependent on early recognition.

[Pulmonary carcinoid tumors]. / I tumori carcinoidi polmonari.

27 patients suffering from carcinoid of the lung (18 females and 9 males, middle age 52 years, range 26-68) underwent surgery in our department. The neoplasms were located at the pulmonary hilum in 21 cases. The diagnosis was occasional in 6 cases, cough (51.8%) and recurrent bronchitis (37%) were the most frequent symptoms. No instances of carcinoid syndrome were detected. Preoperative staging ruled out pathologic mediastinal lymph nodes or hematogenous metastases. 26 patients underwent complete excision of the neoplasm (11 lobectomies, 9 pneumonectomies, 4 bilobectomies, 1 segmental resection, 1 bronchial wedge resection). Histologically, 4 cases were categorized as atypical carcinoids. Two patients died within 1 year, one suffering from atypical carcinoid because of disease progression, and an other one (suffering from atypical carcinoid) who underwent only at exploratory thoracotomy followed by chemotherapy. A patient suffering from typical carcinoid died within 1.5 years because of gallbladder carcinoma. From our experience and from the literature review it appears that carcinoids has to be considered as malignant neoplasms and treated according to.

[Iatrogenic rupture of the esophagus: presentation of several cases]. / Le rotture iatrogeniche dell'esofago: presentazione di alcuni casi.

Four cases of iatrogenic rupture of the oesophagus are presented. The site of the lesion was in the cervical tract in 1 case and in the thoracic tract in the other 3 cases. Their etiology was pneumatic endoscopic dilatation for achalasia in 2 cases, endoscopic insertion of a Celestin tube for carcinoma of the thoracic tract of the oesophagus in 1 case, and diagnostic endoscopy in the last one. Instrumental findings were relevant in all cases. All patients underwent surgery. In the patients suffering from achalasia, the rupture was repaired by a patch of the gastric fundus. The patient suffering from carcinoma underwent an oesophageal resection, the one with cervical perforation underwent a mediastinal drainage. There were no deaths or considerable post operating complications. In a patient suffering from achalasia gastro-oesophageal reflux was demonstrated after some months following the operation.

[Primary germinal tumors of the mediastinum: our experience in 9 cases]. / I tumori germinali primitivi del mediastino: nostra esperienza in nove casi.

9 patients (8 males 1 female, middle age 24.3 years, range 20-41) affected by primary mediastinal germ cell tumor were surgically treated in our department. They were 4 seminomas, 2 embryonal carcinomas, 1 malignant teratoma and 2 benign teratomas. Three patients were asymptomatic; cough, dyspnea and chest pain were the most frequently observed symptoms. The staging work-up did not show signs of metastatic disease in the malignant types. Benign teratomas underwent complete excision. In one patient suffering from seminoma radiotherapy and chemotherapy caused complete remission of the disease. In the others cases remission of the neoplasm was obtained by resection and adjuvant therapy in 3 cases, by neoadjuvant treatment and excision of the residual mass in 3 cases. Among the patients suffering from seminoma, 2 are dead at 60 months since initial treatment and 2 are alive at 132 and 120 months respectively. Among the patients with malignant nonseminomatous tumours, 2 are alive at 60 and 36 months and 1 patient is dead at 13 months. Two patients with benign teratoma are alive at 189 and 168 months respectively. At present a multimodality treatment including surgery, radiotherapy and cisplatin-based combination chemotherapy, give the most satisfactory results in the treatment of malignant mediastinal germ cell tumours.

[Thoracoplasty today. Review of a technique of the past]. / La toracoplastica oggi. Rivisitazione di una tecnica del passato.

Thoracoplastic operations had ruled the field in the past as treatment of choice in the surgical antituberculous collapse therapy; they have had a notable revival during the last years as an efficient therapeutic modality in the treatment of chronic empyemas of the most various etiologies. They are thoracoplasties done with particular modalities, imposing, setting out case by case to suppress the infected pleural space. It is usually joined with a thoracotomy and it permits also the execution of other surgical intrathoracic manoeuvres such as decortications, treatment of fistulas, myoplasties. Situations such as chronic empyemas, without any solution before, have found a brilliant solution by the use of this operation.