Sacrococcigeal and vertebral chordomas. Report of three cases and review of the literature.

Three cases of sacrococcygeal and vertebral chordoma are described. Histogenetic and anatomopathological aspects with particular reference to differential diagnosis from similarly distributed neoplasias are discussed. Anti-cytoskeleton monoclonal antibodies were used to this purpose. The clinical profile of sacrococcygeal and vertebral chordomas is characterized by a pronounced metastatic potential; radiation treatment can only partially counter their biological behaviour, while chemotherapy has proved little or no effectiveness. Where possible, radical surgery is currently the only treatment to guarantee long-term survival or complete cure. Palliative surgery, associate with efficient painkilling, offers a better quality of life and slows down the progress of the disease.

[Palliative endoscopic treatment of esophageal neoplastic stenosis]. / Considerazioni sul trattamento palliativo endoscopico delle stenosi tumorali dell'esofago.

The authors examined a series of 231 patients suffering from unoperable neoplastic dysphagia of the esophagus and treated using prevalently palliative endoscopic methods (photocoagulation and/or intubation) during the period 1980-1991. They draw the following conclusions: a) endoscopic methods are better than surgical techniques; b) there are a greater number of indications for endoscopic intubation than for photocoagulation (approximately 2 to 1); c) some situation which are indicated for photocoagulation are not contraindicated for intubation; d) the sole contraindication for intubation is stenosis in which the proximal limit is less than 2 cm from the upper esophageal sphincter; e) contraindications for photocoagulation are long stenoses and/or those of the infiltrating type, and/or involving the upper third of the esophagus; f) sometimes the two methods may be complementary in the sense that intubation may be preceded by a few photocoagulation sessions in order to necrotize the vegetating portion of an infiltrating tumour; g) it is best to choose intubation wherever possible since this technique is less expensive and the quality of remaining life better, even if the percentage of severe and generally fatal complications (perforation) is still too high; h) the possible introduction of expandable metal prostheses might increase indications for intubation and reduce the number of severe complication.

[Perforated diverticulum of the cecum: conservative therapy or resection?]. / Il diverticolo perforato del cieco: terapia conservativa o resezione?

The diverticulum of the right colon is a rare disease in Western Countries but is very common in Eastern races. The etiology is usually congenital in the lesions limited to the cecum (true diverticulum). The diagnosis is rarely made preoperatively. The most useful help is from CT and US. Because the clinical features suggest an acute appendicitis, in most cases of appendicectomized patients the intervention is made without any other investigations. At laparotomy, when the diverticulum is surely identified, a conservative treatment is recommended (diverticulectomy or invagination). If there are some doubts an ileo-colic resection is more opportune. After recovery it is useful to study the colon with an Rx clisma.

[Intestinal malrotations in the adult]. / I difetti di rotazione intestinale nell'adulto.

The paper reports a case of intestinal malrotation in an adult patient with an anomalous location in the left hypochondrium of the cecum and vermiform appendix. The patient underwent explorative laparotomy for peritonitis due to acute appendicitis. Following a review of the literature, the anomaly was classified in embryological terms as a union defect during the third developmental stage of the umbilical loop. Attention is focused on the importance of an exact knowledge of the embryology of intestinal rotation in order to avoid technical surgical errors. In conclusion, the value of giving the patient precise iconographic documents is stressed in order to inform the surgeon who may have to operate on the patient's abdomen in the future.

[An unusual association: Jaffe-Linchtenstein syndrome, arcuate ligament syndrome and hepatic adenoma]. / Una insolita associazione: sindrome di Jaffè-Linchtenstein, sindrome del legamento arcuato e adenoma epatico.

A case of a woman affected by an unusual association of rare diseases, is presented. The patient was referred to our department for acute anaemia. Preoperative investigations revealed that the patient was affected by fibrous polyostotic dysplasia, so called Jaffè-Lichtenstein syndrome. The presence of skin brown spot and endocrine disorders requiring pill administration, allows to classify the patient as carrier of Albright syndrome. Moreover, the angiography pointed out a celiac trunc stenosis (Dunbar syndrome). The long-standing administration of the pill could be the cause of bleeding adenoma. The patient underwent hepatic resection. We did not treat the Dunbar syndrome because of poor symptoms. From literature, we review some opinions on the fibrous dysplasia of the bone.

[Lipoma of the large intestine]. / Il lipoma del grosso intestino.

A case of lipoma of the colon with a clinical picture of intestinal occlusion due to colo-colic invagination is reported. The invagination diagnosis was arrived at preoperatively by opaque clysis and echography. Surgical treatment consisted of segmentary resection owing to trouble in the affected ansa.

[Current surgical approach in the treatment of pancreatic pseudocysts]. / Attuale orientamento chirurgico nel trattamento delle pseudocisti pancreatiche.

INTRODUCTION: The paper evaluates the role of surgery in the treatment of pancreatic pseudocysts in the light of current knowledge. The authors underline the important part played by ultrasonography and CT in monitoring the evolution of pseudocysts: ETG on account of its high levels of sensitivity and specificity and CT due to its diagnostic accuracy which is equivalent to almost 100%; these methods allow the surgeon to decide if and when to operate. MATERIALS AND METHODS: A series of operations were carried out by the authors' division in 15 patients suffering from pancreatic pseudocysts. Emphasis is placed on the high percentage of emergency operations, 40%, due to complicated pseudocysts. External drainage was always used in emergency surgery, whereas in elective surgery it was preferred to use cystojejunostomy with a Roux-en-Y loop, followed by cystogastrostomy; cystoduodenostomy was only chosen in one case. The operative mortality rate was 20%. DISCUSSION: In elective surgery the operation which currently gives the best results is cystojejunostomy with a Roux-en-Y loop, but cystogastrostomy should not be overlooked due to the ease with which it is performed. A recent acquisition is the use of needle aspiration and CT- or ETG-guided percutaneous drainage in the treatment of post-necrotic pseudocysts, therefore after 4-8 weeks' maturity, both in a rapid-growth from and in the event of suppuration. Pancreatic resection is indicated for multiple atypical pseudocysts caused by retention, and this also serves to treat the underlying pancreopathy. Duodenocephalopancreatectomy is now considered to be excessive by the majority of authors. In emergency surgery, drainage of the pseudocysts is the method of choice using either a surgical or percutaneous method.

[Peptic ulcer: changing indications for gastric resection during the last 15 years]. / Ulcera peptica: cambiamento di indicazione alla resezione gastrica negli ultimi 15 anni.

In this study the authors want to underline the changing strategies for peptic ulcer disease. Considering the experience of two Surgery Departments in Piedmont, the G. Bosco Hospital of Turin and the S. Croce Hospital of Moncalieri, in the last 15 years, they observed a notable reduction of gastric resections owing to new drugs such as H2-antagonist and omeprazole in peptic ulcer therapy. The authors report, therefore, recent suggestions to gastric resection in the cases of complicated ulcer or not. Today pyloric stenosis due to chronic ulcer no longer operated and perforated ulcer is resected only when there is a great gastric hole, or a relapse perforation or a bleeding together to perforation. On the contrary hemorrhagic ulcer is operated when the bleeding is either notable, or relapse, or continuous, or untransfusible.

[Carcinoma of the gastric stump. Our experience]. / Il carcinoma del moncone gastrico. Nostra esperienza.

The authors have examined eight patients operated at the Department of Surgery "B" of the G. Bosco Hospital (Torino) from 1980 to 1992 for gastric stump cancer. All patients were male of about 72 years who were subjected to resection: 5 for gastric ulcer and 3 for duodenal ulcer. During the first operation they used the same recanalization technique after gastric resection: the semi-oral distal gastric jejunal anastomosis created by Hofmeister-Finsterer. The second operation was the subtotal gastric resection made on 6 patients, total gastrectomy in 1 case and explorative laparotomy in an inoperable case. The average of free time from disease was 32 years while it is 24 years in literature. The mean survival was 40 months. The 5-year survival was about 33%. The authors conclude that carcinoma of the gastric stump has a worst prognosis then carcinoma in normal stomach. The subtotal resection has the same results as in total gastrectomy.

[Angiolipoma of the cecum]. / L'angiolipoma del cieco.

The paper reports a case of angiolipoma localised on the ileocecal valve. No cases of intestinal angiolipoma have been reported in the literature. The authors analyse the anatomopathological diagnostic criteria used, together with the clinical characteristics of a pathology which, even when examined macroscopically, resembles a malignant neoplasia of the colon. The authors conclude by underlining the importance of histological diagnosis for a correct surgical choice.

The role of surgery in a case of diffuse mucormycosis with haematemesis and gastric necrosis.

Mucormycosis is a life threatening condition caused by invasion of fungi of the order Mucorales. Gastrointestinal invasion is very rare and often lethal, particularly in disseminated mucormycosis. We present the case of a 26-year-old woman from North Africa with type 2 diabetes who, after a cholecystectomy, developed unexplained septic shock and haematemesis due to gastric necrosis. Computed tomography (CT) revealed a disseminated fungal invasion of the lungs, kidney and paranasal sinuses. A gastrectomy and subsequent amphotericin B treatment resolved her condition. The number of patients with mucormycosis is increasing. Early diagnosis of high risk patients with CT and biopsies from which fungi are directly isolated must be followed by surgery and systemic amphotericin B infusion.

Von Hippel-Lindau and myotonic dystrophy of Steinert along with pancreatic neuroendocrine tumor and renal clear cell carcinomal neoplasm: Case report and review of the literature.

INTRODUCTION: Myotonic dystrophy of Steinert, DM1, is the most common adult muscular dystrophy and generally is not associated to development on multiple site neoplasm. Von Hippel-Lindau (VHL) disease is a dominantly inherited familial cancer syndrome that is associated to tumors such as hemangioblastoma of the retina or central nervous system, clear-cell renal carcinoma (RCC) and endocrine tumors, most commonly pheochromocytoma and non-secretory pancreatic islet cell cancers. No data exist in literature describing the coexistence of both DM1 and VHL. PRESENTATION OF CASE: Herein we report a case of renal and pancreatic neoplasm in a young adult female affected by DM1 and VHL simultaneously. DISCUSSION: DM1 is due to an unstable trinucleotide (CTG) expansion in the 30 antranslated region of the dystrophia myotonica-protein kinase (DMPK) gene, located on chromosome 19q13.3. Several molecular mechanisms thought to be determining the classical DM phenotype have been shown. VHL disease is characterized by marked phenotypic variability and the most common tumors are hemangioblastomas of the retina or central nervous system, clear-cell renal carcinoma (RCC) and endocrine tumors, most commonly pheochromocytoma and non-secretory pancreatic islet cell cancers. The pancreatic manifestations seen in patients with VHL disease are divided into 2 categories: pancreatic neuroendocrine tumor (PNET) as solid tumors, and cystic lesions, including a simple cyst and serous cystadenoma. The surgical approach for these cistic lesions is to consider as golden standard. Blansfield has proposed 3 criteria to predict metastatic disease of PNET in patients with VHL disease: (1) tumor size greater than or equal to 3cm; (2) presence of a mutation in exon 3; and (3) tumor doubling time less than 500d. If the patient has none of these criteria the patient could be followed with physical examination and radiological surveillance on a 2/3 years base.(4) If the patient has 1 criterion, the patient should be followed more closely every 6 months to 1 year. If the patient has 2 or 3 criteria, the patient should be considered for surgery given the high risk of future malignancy. Our patient owned only one criterion but in presence of a second malignant tumor. Our hypothesis for this rare findings is that both DM and VHL might be derived from genetic aberration and these might be linked to a major cancer susceptibility. As far as we know this is the first confirmed case of RCC and neuroendocrine pancreatic cancer occurring concurrently with VHL and, at the same time, DM1. According to this case report and the literature data a VHL should be ruled out in the presence of RCC presenting along with pancreatic cysts/tumor. CONCLUSION: As far as we know this is the first confirmed case of RCC and neuroendocrine pancreatic cancer occurring concurrently with VHL and, at the same time, DM1. Our hypothesis for the unusual findings is that both DM and VHL derived from genetic aberration and these are linked to a major cancer susceptibility.

[Biliary ileus. Presentation of 4 cases and review of the literature]. / L'ileo biliare. Presentazione di 4 casi e revisione della letteratura.

Gallstone ileus is a rare condition whose diagnosis may be puzzling. A series of 4 cases of gallstone ileus is presented. The last twenty years' literature was reviewed and compared with our results. Enterolithotomy was performed in all patients and no surgery was performed on the gallbladder and/or the fistula. The outcome was favourable in all cases, thus supporting our procedure.