Surgical treatment of a giant mesenteric fibromatosis mimicking a gastrointestinal stromal tumor: a case report and current treatment aspects.

Intra-abdominal fibromatosis (IAF), usually located at the mesenteric level, is a locally invasive tumor of fibrous origin. Although lacking the ability to metastasize, it has the tendency to recur. The case described here is a case of mesenteric fibromatosis with involvement of the bowel wall, which had the appearance of a gastrointestinal stromal tumor (GIST), a tumor with malignant behavior. This report outlines the fact that certain non-typical cases of IAF with involvement of the bowel wall can be misdiagnosed as GIST. It is of outmost importance to make an early and correct diagnosis in such equivocal cases, so that the appropriate treatment can be closed.

The prophylactic effect of L-arginine in acute ischaemic colitis in a rat model of ischaemia/reperfusion injury.

BACKGROUND/AIMS: The decreased synthesis of nitric oxide (NO) during ischaemia/reperfusion (I/R) has been implicated as the major underlying mechanism for the pathogenesis of acute ischaemic colitis (A.I.C.). The aim of this study was to investigate the prophylactic effect of L-arginine, a NO donor, on tissue injury during intestinal I/R, and compare its efficacy with that of exogenous vasodilators (molsidomine) and inert nitrogen-containing molecules (casein). MATERIAL AND METHODS: One hundred forty four Wistar rats underwent occlusion of the superior mesentery artery for 30, 60 and 90 min for induction of intestinal ischaemia, followed by 90 min of reperfusion. The rats were randomly assigned to receive L-arginine, molsidomine, or casein hydrolysate. In all groups, apart of the histological study, we determined the levels of serum malondialdehyde (MDA), a reliable marker indicating the degree of the tissue damage after intestinal I/R. RESULTS: Serum MDA levels were significantly lower in the L-arginine group compared to the untreated animals or those that had received molsidomine or casein, after a period of ischaemia of 90 minutes (p < 0.0005), as well as after a period of ischaemia of 60 or 90 minutes followed by a 90 minutes reperfusion (p = 0.011, and p < 0.0005, respectively). In addition, lesser histopathological damage was noted after the use of L-arginine compared to that caused by the administration of molsidomine and casein. CONCLUSION: These findings support a prophylactic effect of L-arginine in experimentally induced intestinal ischaemia. In short, L-arginine attenuates the degree of tissue damage in intestinal ischaemia and promotes healing of intestinal mucosa.

Non-parasitic splenic cysts: a report of three cases.

Primary splenic cyst is a relatively rare disease, and the majority of cases are classified as epithelial cysts. Three cases with nonparasitic splenic cysts are presented: two epithelial and one pseudocyst. All cases had an atypical symptomatology, consisted mainly of fullness in the left upper abdomen and a palpable mass. Preoperative diagnosis was established with ultrasonography and computerized tomography. Two cases with large cysts located in the splenic hilum were treated with open complete splenectomy. The most recent case, a pseudocyst, was managed laparoscopically with partial cystectomy. All cases did not have any problems or recurrence during follow-up. Laparoscopic partial cystectomy is an acceptable procedure for the treatment of splenic cysts, because it cures the disease preserving the splenic tissue. Complete splenectomy is reserved for cases in which cyst excision cannot be done otherwise.

Extraskeletal myxoid chondrosarcoma metastatic to the pancreas: a case report.

Extraskeletal myxoid chondrosarcoma (EMC) is a low-grade sarcoma characterized by developing metastases and local recurrence in high rate. It is mainly deep seated in the proximal extremities. The most common metastatic sites are the lungs, soft tissues, lymph nodes, bones and the brain. To our knowledge, no case of clearly defined EMC has been reported to date developing a metastasis in the pancreas. We describe a case of a man suffering from EMC who developed a single pancreatic metastasis 20 years after the initial diagnosis. A 49-year-old man was submitted to surgical excision of an EMC, in left thigh, 20 years ago. Fourteen years after the initial diagnosis a local recurrence in left thigh occurred. Multiple lesions of metastatic origin, in both lungs, were excised via thoracotomies until the time being. In 2003, as a part of a periodically performed imaging control, an abdominal CT scan was performed revealing a solid lesion in the pancreas. Distal pancreatectomy was performed. The histopathology of the excised specimen proved to be the one of metastatic lesion of EMC. The above-mentioned case of EMC is, as far as we know, the first one described developing a certain pancreatic metastasis.

Gardner's syndrome: a case report and review of the literature.

Gardner's syndrome is an autosomal dominant disease characterized by the presence of colonic polyposis, osteomas and a multitude of soft tissue tumors. The syndrome may present at any age from 2 mo to 70 years with a variety of symptoms, either colonic or extracolonic. We present a case of a 11-year-old female patient with Gardner's syndrome who presented with a lumbar area desmoid tumor and treated with resection of the desmoid, restorative proctocolectomy and ileal pouch anal anastomosis, A review of the current literature has been performed.

Colonic duplication in adults: report of two cases presenting with rectal bleeding.

Gastrointestinal duplication is an uncommon congenital abnormality in two-thirds of cases manifesting before the age of 2 years. Ileal duplication is common while colonic duplication, either cystic or tubular, is a rather unusual clinical entity that remains asymptomatic and undiagnosed in most cases. Mostly occurring in pediatric patients, colonic duplication is encountered in adults only in a few cases. This study reports two cases of colonic duplication in adults. Both cases presented with rectal bleeding on admission. The study was focused on clinical, imaging, histological, and therapeutical aspects of the presenting cases. Gastrografin enema established the diagnosis in both cases. The cystic structure and the adjacent part of the colon were excised en-block. The study implies that colonic duplication, though uncommon, should be included in the differential diagnosis of rectal bleeding.

The use of isobutylcyanoacrylate as a tissue adhesive in abdominal surgery.

BACKGROUND: The use of cyanoacrylate substances as tissue adhesives is of valuable aid in surgery, especially in cases of injuries of the intraabdominal organs, where the haemorrhage is very difficult to control. MATERIALS AND METHODS: We investigated the efficiency of isobutyl-2-cyanoacrylate as a tissue adhesive in the haemostasis and adhesion of different types of wounds in solid and hollow organs. Forty-six dogs underwent single-organ (26 dogs) and combined-organ (20 dogs) procedures; cuneiform excisions of the liver and the spleen, as well as incisions of the small intestine were carried out. The wound surfaces were coated with isobutyl-2-cyanoacrylate and approximated. RESULTS: The majority (91.3%) of the surgical operations were uncomplicated, in which a very good macroscopical and histological result was achieved. Histological examination of the surgical injuries, performed 4 months later, confirmed complete wound healing. CONCLUSION: Isobutyl-2-cyanoacrylate proved to be a very effective tissue adhesive for both solid and hollow organs, even for high risk surgical operations.

Recurrent liposarcomas of the abdomen and retroperitoneum: three case reports.

We report three cases of patients with liposarcomas of the abdomen who had been treated during the last 13 years (1980-1993). Two patients were men, aged 29 and 51 years, with tumors of the retroperitoneal space and the third patient was a woman aged 64 years with a tumor in the peritoneal cavity. Therapeutic treatment was as aggressive as possible excision of the tumor. In the case of the first male patient, the histological examination revealed a retroperitoneal myxoid liposarcoma which recurred 5 times within 13 years. In the second male patient, it revealed a well differentiated retroperitoneal liposarcoma of the sclerosing type which recurred 5 times within 5 years since the first treatment. Finally, the one female patient had 2 recurrences of myxoid liposarcoma of the abdomen 9 years after the first operation, presented with an infected mass and has been well since then.

The effect of various types of splenectomy on the development of B-16 melanoma in mice.

260 CB57BL/J6 mice were used in an experimental protocol designed to investigate the effects of 4 different varieties of splenectomy on the growth rate of subcutaneously implanted GB-16 melanoma. In addition, the mean and absolute survival of the mice, the histopathology of the tumour and the effects of the same procedures on the immunological status of the tumour-bearing animals as assessed by serum IgG levels and immunoelectrophoresis were determined. The effects of the timing of the splenectomy and the removal of the primary tumour after splenectomy on the above parameters were also annotated. The following were found: First, splenectomy performed 1 week after B-16 melanoma tumour implantation in mice i.e. in the early period of oncogenesis, lengthened the survival of the grafted experiments, delayed tumour growth, reduced the "activity" of the tumour and caused pseudoencapsulation of the tumour by fibrous tissue. It increased, but not by a statistically significant degree (p > 0.05), the circulating levels of the IgG immunoglobulin. Second, splenectomy performed 4 weeks prior to grafting of the same tumour did not affect the circulating IgG levels, nor did it prolong survival; however, it reduced the rate of tumour growth and pseudoencapsulation of the tumour was observed. Third, splenectomy at the early stages of oncogenesis in combination with surgical removal of the primary tumour increased absolute and mean survival, delayed the tumour growth rate, increased the time to relapse and reduced the "activity" of the pseudocapsulated tumour.

Expression of HLA-DR is reduced in tumor infiltrating immune cells (TIICs) and regional lymph nodes of non-small-cell lung carcinomas. A putative mechanism of tumor-induced immunosuppression?

BACKGROUND: Deregulation of MHC class II molecules consists of a favorable mechanism of tumor evasion from immune surveillance. Among these molecules, HLA-DR antigens are the predominant ones in cancer. In the present study we sought to investigate the ability of tumor infiltrating immune cells (TIICs) to express HLA-DR antigen in the primary tumor site and reactive regional lymph nodes (LNs) in non small cell lung cancer (NSCLC). MATERIALS AND METHODS: Material consisting of 60 NSCLCs with corresponding regional LNs was studied by immunohistochemistry for human leukocyte antigen D-region related (HLA-DR) expression. Control reactive LNs, regional to several different malignant and non-malignant disorders, were also included in the study. RESULTS: Primary tumor site investigation revealed positive HLA-DR cancer cells in 22% of cases, whereas TIICs rarely expressed HLA-DR antigens. The lack of HLA-DR expression in TIICs was gradually attenuated as the distance from the primary tumor site decreased. Regional LN investigation showed that all follicles (paracapsular and deep cortical ones) were HLA-DR-negative in 60% of the LNs; in the remaining 40%, the paracapsular follicles remained negative, while all deep cortical ones were positive. Interestingly, LNs possessing only HLA-DR-negative follicles were more proximal to the primary tumor site compared to those that had only the paracapsular follicles negative. All control reactive LNs, regional to several distinct malignant and non-malignant disorders, were found to be HLA-DR-positive. CONCLUSION: The impairment of HLA-DR expression, detected both in neoplastic and by-stander immune cells, may justify the immunosuppression observed in NSCLC. This phenomenon may be due to a putative soluble factor in the tumor environment secreted by cancer cells.

Experimental echinococcus infection in the mouse model: pericystic cellular immunity reaction and effects on the lymphoid organs of immunocompetent and thymectomized mice.

Echinococcus can infect man as an accidental intermediate host causing hydatid disease. The infection persists and the growth of the cysts advances, while the patient usually remains asymptomatic for years. Experimental Echinococcus infection in mice provides a well described model for the study of the parasite-host relationship that permits the evolution of the disease despite the activation of the host's immune system. The aim of the present study was to assess the immune response to Echinococcus infection in normal and thymectomized mice. For this purpose, a total of 150 mice, divided into three equal groups (A, B and C), were infected by intraperitoneal inoculation of live protoscoleses. The mice of groups B and C underwent thymectomy, two weeks prior and after the infection, respectively. The mice of each group were further divided into three subgroups and were sacrificed at three consecutive time points: 45 days, 3 and 6 months post the infection. The hydatid cysts that subsequently developed by the metacestode-lavral stage, along with the spleen and lymph nodes were excised from each mouse and histologically studied. The results revealed a marked activation of the cell-mediated immunity against the parasite at the early stages of the disease. The initial response of the host abated with time and was minimal six months after the infection suggesting a local immunosuppression state that could account for the advancement of the disease. In addition, the thymectomized mice exhibited a higher susceptibility to the infection, which corresponded to the weak and delayed cellular immunity response observed in these groups. These results suggest that the cell-mediated immunity is crucial for the defense against Echinococcus, especially early in the course of the disease where suppression of larval growth is critical for the final outcome of the infection.

A new rare allele at the CGG repeat polymorphism in the first intron of human c-H-ras gene.

We have examined a region in the first intron of the human c-H-ras gene containing a CGG repeat. This region was previously shown to be variable in length. The length variation was attributed to the presence of the CGG repeat after estimation of its electrophoretic mobility. In the present report we have characterized in detail this region by PCR-RFLP and automated sequencing, in a total of 102 histologically normal tissues from unrelated individuals affected by lung and breast cancer. Four alleles were detected and analysis of their internal sequence showed that the length alterations of this region were due to the presence of 5, 6, 8 and 9 CGG triplets respectively. The last three occur most often (44.1%, 34.8%, 20.6% respectively) and coincide with three previously reported alleles (Riggins et al, Hum Mol Genet 9: 775, 1992). The fourth allele consisting of 5 repeats is a rare one (0.5%), whilst alleles with 7, and a previously reported one suggested to comprise 11 repeats (1%) were not present in our cohort. This polymorphism coincides in position with an element that was previously shown to possess regulatory activity.

Vascular manifestations of Behçet's disease.

The purpose of this article is to present the spectrum of vascular disease accompanying Behçet's disease. Four cases of the disease with various vascular manifestations are presented. In the first case superior vena cava syndrome due to thrombosis was the presenting symptom. In the second case foot ischemia with toe necrosis was prevalent. In the third case the patient developed recurrent aneurysmal disease in large and medium size arteries requiring successive vascular surgery, and in the fourth case the patient had SVC thrombosis and ruptured femoral aneurysm. All four patients were young males. Although vascular complications of Behçet disease are uncommon they may pose serious therapeutic problems.

[Surgery of the adrenal glands in Cushing's syndrome]. / La chirurgie des surrénales pour syndrome de Cushing.

Our surgical experience for the Cushing syndrome, reviewed in 23 patients. The accuracy of localizing adrenal lesions increased recently. The present study shows that, adrenal surgery, after accurate diagnosis and localisation, can be performed with low morbidity and mortality.

[Complicated solitary biliary cysts]. / Les kystes biliaires solitaires du foie compliqués.

With development of ultra sound, solitary hepatic cyst (SHC) appears as a common and benign affection. Complications can occur in 10 per cent of cases. We report here four cases of complicated SHC: vena caval obstruction, intracystic bleeding, rupture, intracystic infection. Those four patients were successfully treated by partial excision of the cyst in the first 3 cases, and by percutaneous drainage with CT scan guidance in the latter. Complications of SHC occur only in large cysts, with a diameter up to 8 cm. So, small SHC do not require any treatment, while large SHC must be treated to avoid complications. Percutaneous aspiration and direct injection of alcohol can lead to recurrence. Surgical therapy by partial excision is successful, with low rates of mortality and morbidity.