Aggressive fibromatosis of the right colon mimicking a gastrointestinal stromal tumour: a case report.

Aggressive fibromatosis is a rare type of intra-abdominal desmoid tumour that usually involves the small bowel mesentery. It is a locally-invasive lesion, with a high rate of recurrence, but without metastatic potential. Aggressive fibromatosis is seen more often in young female patients. This case report presents the radiological, intraoperative and histopathological findings from a 37-year-old female patient that presented with epigastric pain and a palpable mass in the right hemiabdomen. Histological and immunohistochemical examinations of the resected tumour, including positive staining for beta-catenin, confirmed a postoperative diagnosis of desmoid type fibromatosis. This specific case showed that desmoid type fibromatosis of the colon can mimic gastrointestinal stromal tumours (GIST) based on its clinical presentation, computed tomography and magnetic resonance imaging findings. Differential diagnosis between desmoid type fibromatosis and GIST is clinically very important due to the different treatments and follow-up protocols that are implemented for these lesions.

[Solid solitary hamartoma of the spleen].

INTRODUCTION: Hamartoma of the spleen is a rare, sometimes asymptomatic similar to hemangioma benign tumor of the spleen, which, owing to the new diagnostic imaging methods, is discovered with increasing frequency. It appears as solitary or multiple tumorous lesions. CASE OUTLINE: We present a 48-year-old woman in whom, during the investigation for Helicobacter pylori gastric infection and rectal bleeding, with ultrasonography, a mass 6.5x6.5 cm in diameter was discovered by chance within the spleen. Splenectomy was performed due to suspected lymphoma of the spleen. On histology, tumor showed to be of mixed cellular structure, with areas without white pulp, at places with marked dilatation of sinusoids and capillaries to the formation of "blood lakes" between which broad hypercellular Billroth's zones were present. Extramedullary hematopoiesis was found focally. The cells that covered vascular spaces were CD34+ and CD31+ and CD8- and CD21-. CONCLUSION: Hamartoma has to be taken into consideration always when well circumscribed hypervascular tumor within the spleen is found, particularly in children. Although the diagnosis of hamartoma may be suspected preoperatively, the exact diagnosis is established based on histological and immunohystochemistry examinations. Treatment is most often splenectomy and rarely a partial splenectomy is possible, which is recommended particularly in children.

Radiofrequency ablation for Barrett's esophagus high-volume center initial results.

INTRODUCTION: Barrett's esophagus (BE) is a well established precursor of esophageal adenocarcinoma. Both, surveillance and therapeutic strategies have been proposed over the years. Recent deve-lopment of endoscopic radiofrequency ablation (RFA) brought new perspectives in the treatment of BE, with excellent initial results. METHODS: The study of 40 pts with macroscopically visible BE on endoscopy and biopsy proven goblet cells presence, was conducted from January 2010 until March 2012. In all pts a complete symptomatic, endoscopic and manometric evaluation was performed. Initially RFA HALO 90 and 360 were performed in 28 and 12 pts respectively. Repeated treatments were conducted in 7 pts. The overall number was 50, while the mean number of RFA procedures per patient was 1.25. RESULTS: The mean circumferential length and maximal extent of BE were 1.61 and 3.29 cm respectively. We did not encounter esophageal perforation or hemorrhage during the procedure. Complications were transient short-term retrosternal pain (23 pts) and dysphagia (11 pts). Three months after the RFA mean values of cumulative symptom and heartburn score dropped significantly (p < 0.05). Functional diagnostics did not disclose any statistically significant decrease of lower esophageal sphincter pressure or esophageal body contraction amplitudes. One year foIlow-up was obtained in 26 pts and revealed a complete macroscopically visible BE eradication. So far, in 19 pts a laparoscopic Nissen fundoplication was performed up to 3 months after complete RFA BE eradication. CONCLUSION: HALO RFA procedure is safe and very effective in the treatment of pts with BE, does not lead to esophageal function impairment, and produces no long term and serious side effects.

[Mucinous cystadenoma of pancreatic head with focal malignant alteration in a 52-year-old man].

Mucinous cystadenomas of the pancreas are rare tumors appearing usually within the body and the tail of the pancreas in a young and middle-aged women. They rarely communicate with the pancreatic duct and occasionally may become malignant. The authors present a patient with a number of rare features. In a 52-year-old male, we did a radical pylorus-preserving cephalic duodenopancreatectomy for a mucinous cystadenoma within the head of the pancreas, which perforated into the main pancreatic duct causing chronic obstructive pancreatitis having few foci of malignant alteration. The postoperative recovery was uneventful, but three months later the patient died due to exacerbation of the underlying serious heart disease.

[Stromal tumour of duodenal autonomous nerves (plexosarcoma)].

Gastrointestinal tumours arising from autonomous nerves of Meisner's or Auerbach's plexus (plexomas and plexosarcomas) are rare tumours in only 87 cases described in the literature up to 2001. We present a very rare case of gastrointestinal stromal tumour (plexosarcoma) of the third and fourth portion of the duodenum, 130 x 98 x 87 mm in diameter, arising from its back wall, with central necrosis of the well circumscribed tumour, which communicated with the duodenum through an ulceration of 15 x 7mm in diameter, spreading towards the great vessels of the retroperitoneum. It was gradually and carefully removed, together with 17 cm of the duodenum and few centimetres of the jejunum with end-to-end duodenojejunostomy below the Vater's papilla. During the removal of the tumour, the superior mesenteric artery, being within the tumour's capsule, was accidentally ligated but not transsected. In spite of the removal of the ligature, the artery became thrombosed due to damage of the intima by ligature so that it had to be resected and reanastomosed. After otherwise uneventful recovery, except for a mild pus discharge through the drain, not far from the arterial anastomosis, the patient suddenly started bleeding on the 13th day after surgery. At emergency reoperation, a rupture of the mesenteric artery above the thrombosed anastomosis was found. In spite of absence of the arterial pulsation within the mesentery, the bowel looked vital and the back flow from the artery was satisfactory. The arterial rereconstruction was not possible, so the artery was ligated. The postoperative recovery was surprisingly uneventful. The patient was discharged ten days after surgery and has stayed symptom-free so far.

[Angiectatic pseudocyst of the pancreatic head].

BACKGROUND: Arteriovenous malformations of the pancreas are very rare, most frequently congenital ones, but tending to increase frequency, predominantly due to the introduction and use of new diagnostic modalities. They usually present with gastrointestinal bleeding and abdominal pain. CASE REPORT: The autors presented a 52-year-old woman in whom, during the investigation for the abdominal pain and gastrointestinal bleeding, an ultrasonography showed a hypoechogenic lesion within the head of the pancreas 3 cm in diameter. Endoscopic ultrasound showed an irregular hypoechogenic lesion. Computed tomography showed a low density area within the lesion (< 10HU). At endoscopic retrograde cholangiopancreatography, a cystic lesion within the head of the pancreas was filled with contrast from the normal pancreatic duct. Angiography showed an arteriovenous malformation within the head of the pancreas. The patient underwent pylorus preserving cephalic duodenopancreatectomy. The diagnosis of arteriovenous malformation, probably of congenital origin, was confirmed on histology. CONCLUSION: Arteriovenous malformations should be considered in patients with gastrointestinal bleeding of unknown etiology. In this case, the lesion was taken into account preoperatively, based on color Doppler ultrasonography and angiography. The best results of treatment can be achieved with resection, but it has to be performed before an eventual portal hypertension takes place.

[Hemangioma of the spleen].

Although the most frequent benign tumors of the spleen, hemangiomas are very rare, much rarer than hemangiomas of the liver. They manifest as localized (either single or multiple) or diffuse lesions, vary from solid to cystic, histologically from capillary to cavernous. Usually, they are small in size (1-3 cm), rarely larger and very rarely of large size. A 67-year old woman admitted to Institute of Hematology, CCS, for investigation of the left upper abdominal pain, loss of appetite, loss in weight and malaise. As the investigation showed a number of hypoechogenic lesions within the enlarged, diffusely non-homogenic spleen, splenectomy was indicated. The spleen weighing 2600 grams was removed, in which the number of lesions histologically corresponded to hemangioma of the spleen. Postoperative recovery was uneventful. The patient has remained symptom free more than two years after surgery.

[Infected giant liver cyst].

Congenital liver cysts are not as rare as it was thought in the past. Thanks to new imaging diagnostic techniques, these cysts have been discovered with increasing frequency. Uncomplicated congenital liver cysts are usually asymptomatic or followed by mild symptoms. Various complications of these cysts were reported. Infection of the congenital liver cyst is, however, very rare complication. A 65-year old man with huge infected congenital central liver cyst containing 3400 ml of pus from which Escherichia coli was cultivated was presented. The patient had a number of other histologically confirmed congenital cysts of the liver and the cyst of the left kidney. The patient was cured by pus evacuation, partial excision of the cyst wall and drainage of the cavity and subphrenic space. The uninfected cysts were operated using deroofing technique. A year after surgery the patient remained symptom free.

[Aggressive intraabdominal fibromatosis].

BACKGROUND: Intraabdominal or mesenteric fibromatosis is a rare benign nonmetastatic neoplasm that appears as a sporadic lesion or in patients with familiar polyposis. Its evolution is unpredictable. About 10% of the cases shaw a very aggressive growth. CASE REPORT: We presented a 22-year-old woman in whom an aggressive intraabdominal fibromatosis had appeared during the first pregnancy as a well circumscribed ovoid tumor, involving the terminal ileum, the caecum, the ascending colon, the right kidney, the ureter, and the right common iliac artery. The tumor was excised with right colectomy, nephroureterectomy and resection of the involved artery using arterial reconstruction with graft interposition. Two years after the surgery the patient developed an inoperable tumor recurrency with a fatal outcome. CONCLUSION: In spite of a successful surgical excision during the original surgery intraabdominal or mesenteric fibromatosis might have an aggressive evolution leading to an inoperable tumor recurrency and a fatal outcome.

[Inflammatory pseudotumor of the spleen].

BACKGROUND: Inflammatory pseudotumors are benign tumor lesions of an unknown etiology that can appear almost at any organ, sometimes along with infectious agens, like Epstein-Barr virus. They are extremely rare in the spleen, with less than 80 cases described in the world literature. It is a single, very rarely multinodal lesion. Middle aged patients are mostly affected. Symptoms are either absent or uncharacteristic, so that the tumors are discovered by chance or during investigations of other diseases. CASE REPORT: We presented a 56-year-old woman in whom a nodal lesion of 2 cm in diameter had been discovered in the spleen during a routine check-up after a mild traffic accident. The lesion had grown up to 6 cm in diameter during a 5-year follow-up causing a mild pain over the upper left abdomen and left shoulder. Fine needle biopsy failed to show the exact nature of the lesion. Splenectomy was performed due to a suspected lymphoproliferative disorder. Histology and immunohistochemistry of the lesion of the upper pole of the spleen showed inflammatory pseudotumor. Epstein-Barr virus was not present. The patient developed postsplenectomy thrombocytosis (up to 850 x 10(9)/1) which settled down within 10 weeks. CONCLUSION: . Inflammatory pseudotumors of the spleen are extremely rare. The exact diagnosis can be established only by histology and/or immunohistochemistry of the spleen removed for a suspected lymphoproliferative disorder, most frequently lymphoma. The patient was cured with splenectomy.

[Lymphoepithelial cyst of the pancreas].

Lymphoepithelial cysts of the pancreas are very rare with less than 35 cases described in the literature. A 49-year old male with a cystic tumor in the tail of the pancreas of 8 cm in diameter discovered during the investigation for mild pain in the upper abdomen was presented. The tumor was easily removed during the open surgery. It contained yellowish dense pus-like fluid whose culture remained sterile. Histology showed the lymphoepithelial cyst of the pancreas. Postoperative recovery was uneventful and preoperative pain disappeared. Eight and a half years later, he died due to complications after orthopedic surgery of the spine.

[Villous duodenal adenoma with II and III grade dysplasia and carcinoma in situ treated with cephalic duodenopancreatectomy].

BACKGROUND: Villous duodenal adenomas are rare, tend to malignant alteration and recurrency if surgical procedures are not sufficiently radical. They are mostly localized in the second portion of the duodenum and close to a papilla and are prone to malignant alteration. CASE REPORT: In this paper we presented a 79-year-old female patient already operated on twice elsewhere due to obstructive jaundice caused by villous duodenal adenoma, using, firstly, cholecystectomy and T-tube drainage of the choledoclus, and, then, an unsuccessful attempt of radical surgery--duodenopancreatectomy. The patient was some time wearing a T-tube drainage, and, then, submitted to endoscopic papillotomy, endoscopic stenting, and, finally to the insertion of a self-expandable metallic stent which got clogged three months later causing chalangitis. Three years following the first surgery, the patient presented to our institution and submitted to cephalic duodenopancreatectomy. Histology confirmed villous duodenal adenomas with II and III grade dysplasia and carcinoma in situ. The limph nodes were tumour-free. The postoperative recovery was somewhat prolonged due to cardiologic difficulties and a mild infection of the wound. CONCLUSION: Villous duodenal adenoma is curable if treated correctly and in a proper time. Radical treatment delay increases the risk for malignant alteration which was the case with our patient who was submitted to it three years after the histologic confirmation of the diagnosis.

[Solitary fibrous tumor of the visceral pleura of the right lung base].

Solitary fibrous tumors are relatively frequent. They may occur throughout the body. About 20% of tumors develop in the thorax, out of which vast majority is localized on its wall. Within thorax, they usually originate from pleura, most frequently the visceral one. Authors present a 67- year old male in whom the tumor was diagnosed during the investigation for dyspnea. During operation, solitary, well circumscribed, firm, ruber-like tumour, 11 x 10 x 9 cm in diameter, covered with serosa, arising from the visceral pleura of the base of the right lung was easily excised. The classical histological and immunohistochemical examinations confirmed the diagnosis of benign fibrous tumor. Postoperative recovery was uneventful. Preoperative symptoms disappeared.

[Desmoid tumor within lesser sac].

Desmoid tumors or fibromatoses comprise a number of benign fibrous proliferative lesions that have local infiltrative growth and tendency to recur after incomplete excision. They never metastasize. The authors present a 31-year-old woman who, due to epigastric pain and palpable mass detected on presentation, underwent the excision of firm tumorous mass, 210 x 140 x 115 mm in diameter, from the lesser sac. Compressing the splenic vein, the tumor caused left-sided portal hypertension which subsided after the mass was removed. The recovery was uneventful. The histological examination verified typical desmoid tumor. Twelve years after surgery, the patient remained symptom-free with no signs of recurrence.

[Bilateral angiomyolipoma of the kidney in patient with tuberous sclerosis].

Angiomyolipomas are relatively frequent tumours of the kidney. It is believed that about 10 million people worldwide have such a tumour. About 1/10 of these 10 million are patients who suffer from tuberous sclerosis. The tumours are frequently bilateral, slow growing, and usually asymptomatic, as well as being rare in children. Due to the benign nature of angiomyolipomas, surgical treatment and embolisation of the tumours are generally not recommended, unless renal function is endangered, the symptoms are severe, or the kidney in question becomes completely dysfunctional. This is particularly the case in patients with tuberous sclerosis in whom these tumours are either already bilateral or may become so. We present a 24-year-old woman with tuberous sclerosis in whom bilateral kidney tumours were diagnosed 7 years earlier and in whom we carried out a left nephrectomy of a 5300 gram angiomyolipoma, which caused pain and complete loss of function. Although tumourous, the right kidney was functional, so it was left untouched. After an uneventful recovery, a close follow-up was recommended, as well as HLA typing, as it is highly probable that the right kidney will gradually become inadequate or completely dysfunctional, so that haemodialysis and/or kidney transplantation along with nephrectomy will become necessary.

[Solid cystic pseudopapillary tumor of the pancreas]. / Solidni cisticni pseudopapilarni tumor pancreasa.

Solid-cystic-pseudopapillary tumor (SCPT) of the pancreas, described by Frantz (1959), is a very rare clinical pathologic entity with relatively low grade malignant potential. The tumor is more frequent in the body and tail of the pancreas. The majority of patients are young females. About 60% of patients are asymptomatic. Complications such as rupture, bleeding or secondary infections are rare. Metastases of the tumor and local recurrence after surgical treatment are also rare. Prognosis is excellent after complete surgical removal. It is difficult to make a preoperative diagnosis of pancreatic SCPT. The exact diagnosis is based on histological findings. Differential diagnosis should consider pancreatoblastoma, non neoplastic cysts, pancreatic pseudo-cysts and hydatid cyst. This is a case report of 39-year old woman who was admitted to our institution with abdominal discomfort and palpable abdominal mass in the upper abdomen. US and CT scan revealed round neoformation of 60 mm in diameter located in the body of the pancreas. Imaging features were not specific enough to allow for precise diagnosis. Curative R0 left spleno-hemipancreatectomy was performed. Histology of the resected specimen revealed solid and cystic-pseudopapillary tumor of the pancreas. The patient was discharged on postoperative day 7 in a good condition. The patient is well 48 months after the operation, with no impairment of pancreatic endocrine or exocrine function.