[Neuroendocrine carcinoma of the breast: a rare entity]. / Carcinoma neuroendocrino della mammella: una rara evenienza.

Nowadays neuroendocrine breast cancer is a rare entity, though the presence of neuroendocrine cells is often detected within breast cancers. Most of these tumours are associated with conventional ductal or globular breast cancers. We describe the case of neuroendocrine cancer of the breast and discuss its clinical, radiological and cytological aspects.

[Gastrointestinal stromal tumour in emergency surgery]. / GIST intestinali nell'emergenza chirurgica.

Although rare, gastrointestinal stromal tumours (GIST) are the most common mesenchymal neoplasms of the gastrointestinal tract. We present our experience with the treatment of 8 patients affected by gastrointestinal stromal tumours of the ileum and colon. These cases were characterised by acute bleeding, intestinal obstruction or diffuse abdominal pain, and all of them underwent an emergency surgical treatment. We performed six partial resections of the ileum, one right hemicolectomy and one left hemicolectomy. Three patients died after surgical procedures for disease relapse, one died 10 years later due to other complications, and the others are still alive.

[Gastric mesenchymal tumours (GIST)]. / Tumori mesenchimali gastrici (GIST): aspetti clinici e revisione critica delle metodologie diagnostiche.

The incidence of gastrointestinal stromal tumours (GIST) has increased in recent years. A number of authors have attempted to define the actual nature of these tumours. Immunohistochemistry highlighting the positivity of tyrosine-kinase (CD117/c-Kit) has revealed the difference between gastrointestinal stromal tumours and other mesenchymal tumours and, therefore, the possibility of medical rather than surgical therapy. We retrospectively reviewed 19 patients affected by primary gastric GIST, who underwent surgery in recent years with subsequent follow-up. Gastroscopy and gastrointestinal tract radiography were used not only to obtain the diagnosis but also to establish the size, density, contours, ulceration, regional lymphadenopathy, mesenteric infiltration and the presence of metastases. The aim of this study was to evaluate the roles of endoscopy and radiology in this pathology and the advantages and limitations of each individual technique.

[Retroperitoneal and mesenteric fibrosis. An uncommon "carcinoid syndrome"]. / Fibrosi retroperitoneale e mesenterica. Una "sindrome da carcinoide" di non frequente riscontro.

Retroperitoneal fibrosis is an uncommon disease in which dense fibrous tissue proliferates in the retroperitoneum. It frequently consists in an abdominal mass involving alimentary structures, ureters with obstruction, and vascular elements with stenosis. This pathological event may be associated with a history of ergotamine usage or a wide range of conditions including malignancy, injuries and infections. In the case described here, the cause was a midgut carcinoid tumour, releasing high concentrations of serotonin and other metabolites directly into the peritoneal fluid. Because carcinoid tumours of the bowel can remain silent for many years it is possible that similar cases of retroperitoneal fibrosis may be identified only many years after onset. For that reason, a long history of bowel obstruction must be considered a kind of unusual but no less important carcinoid syndrome.

Parachordoma of the gastric serosa: report of a myxoid mimicry in an unusual location.

The authors report a case of an incidentally discovered myxoid stromal tumor occurring in the outer wall of the stomach of a 65-year-old woman. The tumor was phenotypically consistent with parachordoma (or myoepithelioma), showing a chordoid microscopic appearance of tumor cells loosely arranged within a myxoid or hyaline background. Immunoreactivity for high-molecular-weight keratins, S100 protein, and vimentin was detected. In this report, the authors address the differential diagnosis of parachordoma and the unusual location of this neoplasm.

[Inflammatory pseudotumour of the spleen: a case report and review of the literature]. / Pseudotumore infiammatorio della milza: presentazione di un caso e revisione della letteratura.

The case of a 64-year-old man presenting dysuria and haematuria is described. The cause of these symptoms was related to a splenomegaly compressing the left kidney and renal pelvis. A splenectomy was performed. Macro- and microscopic examination of the mass revealed an inflammatory pseudotumour of the spleen, composed of a variable mixture of polyclonal lymphocytes, eosinophils, neutrophils, plasmacells, foamy hystiocytes, giant cells and fibrous tissue. The aetiology is unknown. An inflammatory pseudotumour of the spleen is clearly a reactive lesion resulting from a variety of causes, particularly vascular thrombosis, infections or autoimmune mechanisms. Splenectomy is both diagnostic and curative.

[Inflammatory pseudotumour of the liver: case report and review of the literature]. / Pseudotumore infiammatorio del fegato: presentazione di un caso e revisione della letteratura.

We report a case of inflammatory pseudotumour of the liver in a 53-year-old woman who over the previous month had presented malaise, fever and right-sided hypochondralgia. On physical examination the liver was tender at palpation 2 cm below the right costal margin. Laboratory data were normal. Abdominal ultrasonography revealed a focal lesion, measuring 5 cm in diameter, located between the sixth and seventh segments of the liver, just beneath the liver capsule, characterised by a uniformly low level of echogenicity, a round shape with ill-defined margins and very good sound transmission. CT scan disclosed an unexpected and somewhat ambiguous pattern, with coexistence of inflammatory and neoplastic patterns. The patient was therefore submitted to a diagnostic US-guided percutaneous liver biopsy. The microbiological examination proved sterile, while the histological features revealed chronic inflammatory tissue, with fibroblasts and a number of necrotic components. For this reason, despite a strong orientation towards an inflammatory process, we could not rule out the possibility of a necrotic tumour. After two weeks of antibiotic therapy without results, the patient underwent a hepatic bisegmentectomy and a cholecystectomy. Pathological examination of the surgical specimen confirmed the biopsy findings and was suggestive of an inflammatory pseudorumour of actinomycotic origin. The patient was discharged on postoperative day 14 in good general condition, and today, after a follow-up of 5 years, she is still well without any recurrence of disease. Inflammatory pseudotumour of the liver is an unusual lesion that can mimic hepatic malignancy in its presentation and imaging. Despite the clear inflammatory nature of the mass it is almost impossible to detect any aetiological agent. Histologically, the lesion consists in interlacing bands of fibrous connective tissue, containing microscopic suppurative foci, granulocytes, neutrophils, lymphocytes, plasma cells, foamy histiocytes and a few sulphur granules suggestive of actinomycosis. The diagnosis can be made only histologically by US/CT guided-biopsy or, in a limited number of cases, directly by intraoperative frozen sections. Treatment for hepatic inflammatory pseudotumours is controversial: some Authors report cases with spontaneous regression of the disease with or without antibiotic/steroid therapy, while others favour early resection in patients unresponsive to medical therapy. This latter surgical approach is justified not only by the difficult diagnosis but also by the need to prevent complications related to the clinical course of the disease.

Gastric wall necrosis from organo-axial volvulus as a late complication of laparoscopic gastric banding.

We present a case of massive gastric wall necrosis associated with volvulus of the stomach occurring 17 months after laparoscopic gastric banding. The 19- year-old female was admitted to our hospital with acute abdominal pain with rapid deterioration from massive necrosis of a distended stomach which perforated distal to the gastric band, accompanied by splenic infarction. Total gastrectomy and spenectomy were required. We present this very rare complication which should be recognized and treated early, so that a less drastic operation is possible.

[Non-functioning neuroendocrine tumor of the pancreas: report of a clinical case and review of the literature]. / Tumore neuroendocrino non funzionante (BINT) del pancreas: descrizione di un caso clinico e revisione della letteratura.

The authors report the case of a 26-year-old woman, with a palpable abdominal mass, dyspepsia, pain and weight loss. These symptoms were caused by a non-functioning or biologically inactive neuroendocrine tumour (BINT), weighing 510 g and located in the tail of the pancreas. The treatment opted for was a surgical resection consisting in a distal pancreatectomy. The results of the operation were satisfactory and the woman is now alive and in good health, without recurrence, after 7 years. The authors then go on to analyse the concept of non-functioning neuroendocrine tumours and the problems relating to their symptoms, location and nature. These tumours, which arise, from the pancreatic islet cells, fail to produce a clinical syndrome owing to insufficient peptide production or insufficient release, or concurrent secretion, of inhibitory peptides by the tumour or production of biologically inactive molecular forms of the peptides (without clinical effects). The value of tumour markers and the indications and type of surgery are also discussed according to the different patterns of tumour spread (local, locoregional and metastatic disease). It is stressed that a complete surgical resection is the only curative treatment for these tumours. Encouraging results have been recently obtained by adjuvant treatment with somatostatin analogues, chemotherapeutic agents and/or inteferon.