Gastrointestinal stromal tumor metastases to the pituitary: a rare entity.

Gastrointestinal stromal tumors (GISTs) are rare, but represent the most common mesenchymal tumor of the gastrointestinal tract. The paucity of intracranial metastasis makes treatment strategies difficult. This manuscript presents the first documented case report of a GIST that presented clinically with pituitary symptoms due to a pituitary metastasis.

Prospective trial comparing contrast swallow, computed tomography and endoscopy to identify anastomotic leak following oesophagogastric surgery.

BACKGROUND: Anastomotic leaks are a major complication of oesophagogastric surgery. We compare contrast swallow fluoroscopy, computed tomography (CT) with oral contrast and endoscopy in identifying anastomotic leaks following oesophagogastric surgery. METHODS: A prospective trial of 38 patients undergoing oesophagogastric resection was undertaken with informed consent and institutional review board (ethics committee) approval. Patients underwent all three investigations (over 24 hours) 1 week postoperatively. RESULTS: Eight (21%) had clinically apparent leaks. Three pseudo-leaks were suggested on contrast swallow but were confirmed normal on CT and endoscopy. Contrast swallow and CT missed one anastomotic leak each. Endoscopy identified anastomotic defects in three patients, in whom CT and contrast swallow were either normal or conflicting. CONCLUSIONS: Routine tests of anastomotic integrity are unnecessary. When clinically suspected, contrast swallow or CT with oral contrast will identify most leaks. Endoscopy is useful in cases where there are incongruous results.

Laparoscopic procedures in adults with ventriculoperitoneal shunts.

Until recently, the presence of a ventriculoperitoneal shunt (VPS) was considered an absolute contraindication to laparoscopy. In some cases, intraabdominal insufflation causes a rapid, sustained increase in intracranial pressure (ICP). Such intracranial hypertension may result in hindbrain herniation. To prevent this, the use of lower abdominal pressures, intraoperative ICP monitoring, intraoperative ventricular drainage, and distal shunt catheter clamping/externalization has been reported in some studies. However, other studies show that laparoscopy is safe even without VPS catheter clamping and with only routine anesthetic monitoring. Moreover, the risk of retrograde failure of the valve system has been shown to be minimal even with intraabdominal pressures as high as 80 mm Hg. We report how we managed a hydrocephalic adult with a VPS shunt undergoing laparoscopic cholecystectomy in the hope that our experience contributes to the successful management of such patients in the future.

Idiopathic cholangiopathy in a biliary cast syndrome necessitating liver transplantation following head trauma.

The development of total biliary casts is very unusual, especially in patients who have not undergone liver transplantation. The aetiology of these casts is uncertain but several factors are believed to play a role, including periods of fasting, haemolysis, cholangitis and recent surgery. Resultant bile stasis and/or gallbladder hypocontractility promote sludge and subsequent stone formation. Here we present the case of a previously well 66-year-old woman who developed a total biliary cast several weeks after being involved in a road traffic accident during which she sustained head injuries but no obvious liver insult. This cast was removed at laparotomy but the patient had resultant diffuse biliary tree abnormalities and persistent cholestasis and subsequently required a liver transplant. The possible aetiologies of biliary cast formation and subsequently cholangiopathy necessitating transplantation in this patient are described.

A case of spontaneous hypoglycaemia and impaired glucose tolerance in the same patient.

We present a rare case of an insulin-like growth factor-2 (IGF-2)-secreting tumour of the thorax. This patient demonstrated the combination of fasting hypoglycaemia and impaired glucose tolerance on oral glucose tolerance testing, which has not been previously described in this condition. A review of the literature of IGF-2-secreting intrathoracic tumours is presented here.

GIST with a twist--upregulation of PDGF-B resulting in metachronous gastrointestinal stromal tumor and dermatofibrosarcoma protuberans.

CASE REPORT: A 61-year-old male was referred following an incidental radiological discovery of an intra-abdominal mass. His medical history included excision of a lumbar dermatofibrosarcoma protuberans (DFSP) 5 years previously. A CT scan of the abdomen revealed a mass arising from the greater curvature of the stomach. Upper GI endoscopy was normal. He underwent successful laparoscopic resection of this mass. MATERIALS AND METHODS: The histology of the abdominal mass revealed a gastrointestinal stromal tumor (GIST) with poor prognostic indicators. Immunohistochemical analysis of the GIST and his previous DFSP was performed. RESULTS: Immunohistochemistry suggested a link between the GIST and his previous DFSP involving the PDGF signalling system. DISCUSSION: Both GIST and DFSP are extremely rare tumors. A mutation in the platelet-derived growth factor receptor alpha (PDGFR-alpha) has been described in 5-15% of GISTs. It has been shown that DFSP is frequently associated with a translocation between PDGF-B (Chr 22) and COL1A1 (Chr 17), causing continuous activation of PDGFR-beta. Literature review confirms that there are no previously reported cases of both of these tumors occurring in the same patient. CONCLUSION: We hypothesize that this patient may have a previously undescribed genetic mutation involving the PDGF signalling system, resulting in these two very rare malignancies. Immunohistochemistry studies confirmed the link on this occasion. Improvements in our understanding of the molecular biology of the PDGF system may novel therapeutic targets in the future.

Primary malignant melanoma of the oesophagus: a case report.

Primary malignant melanoma of the oesophagus is a rare neoplasm comprising less than 0.2% of all primary oesophageal neoplasms. There are fewer than 250 reported cases in worldwide literature. Several reports suggest that it has a mean survival rate of 2.2% at 5 years and a median survival rate of 10 months. A 48 year old male presented to our surgical service complaining of a three month history of progressively worsening dysphagia with associated regurgitation and unintentional weight loss of 14 kg. There was no prior history of cutaneous or ocular melanoma. He was treated with a combination of subtotal oesophageal resection and immunomodulatory therapy. We present herein a case of primary malignant melanoma of the oesophagus including the associated clinical, pathological and radiological findings.

Infiltrating immune cells, but not tumour cells, express FasL in non-small cell lung cancer: No association with prognosis identified in 3-year follow-up.

Non-small cell lung cancer (NSCLC) remains a difficult disease to treat and independent prognostic markers other than tumour stage and histology have not emerged. The immune cell content of solid tumours has been associated with tumour regression and at times, tumour progression. The involvement of immune cells in prognosis of NSCLC is poorly described. Poor immune responses within solid tumours have been linked with tumour production of immunosuppressive cytokines. Tumour expression of FasL is thought to disarm responses through the transduction of a death signal in Fas-expressing T cells. The existence of the 'tumour counterattack' in vivo has been questioned. We undertook to measure T cell and macrophage infiltration of the tumour bed in NSCLC and report the association between immune cell content and prognosis in a limited, 3-year analysis of survival (n = 113). In addition we investigated FasL expression (n = 45). T cells and macrophages were found to frequently infiltrate lung tumours, albeit in small numbers. Generally there were more T cells infiltrating than macrophages. T cell and macrophage numbers were not associated with prognosis. Lung tumours were found not to express FasL, although occasional immune cells surrounding tumour cells were strongly positive. FasL expression was not associated with prognosis in this series. Thus, immune cells infiltrating NSCLC are not capable of suppressing tumour growth, nor are they associated with tumour progression. We report that lung tumours do not express the FasL, and that although some immune cells are FasL positive, this is not a reflection of general immune cell activation.