Nikica Grubor

Malignant stromal tumor of the stomach with giant cystic liver metastases prior to treatment with imatinib mesylate.

INTRODUCTIONnGastrointestinal stromal tumors (GISTs) are rare and account for 0.1%-3% of all gastrointestinal neoplasms. GISTs are most commonly located in the stomach (60%) and 20%-25% are malignant, with metastases involving the peritoneum or the liver. Cystic liver metastases are extremely rare. Only two previous cases of patients with cystic liver metastases, prior to treatment with imatinib mesylate, have been described so far.nnnCASE REPORTnWe reported a 52-year-old woman presented with a history of abdominal fullness and discomfort. Clinical examination revealed two palpable masses, first in the right upper abdomen and second left to the umbilicus. Examinations revealed 4 cystic metastases in the liver, 3 in the right lobe (including a huge one measuring 20.5 x 16 cm), and 1 in the left lobe, together with a primary tumor on the greater curvature of the stomach. Gastric tumor was removed with a Billroth II gastrectomy. Partial excision of the largest liver metastasis was performed for histopathology. Immunohistochemistry confirmed the diagnosis of a GIST in both tissue samples. After an uneventful recovery the patient was commenced on imatinib mesylate therapy. The patient remainsed symptom-free at 24 months follow-up.nnnCONCLUSIONnThis was the third reported case of gastric GIST with giant cystic liver metastases present prior to treatment with imatinib mesylate. Although extremely rare, GISTs may present with cystic liver metastases prior to treatment with imatinib mesylate, and should be considered in the differential diagnoses of patients with liver cysts of uncertain aetiology.

Portal cavernoma caused by a calcified hydatid cyst of the liver (case report).

A portal cavernoma is a network of porto-porto collateral dilated tortuous veins lying within the hepatoduodenal ligament, which develops as a sequel to portal vein obstruction. This can be the result of extra-hepatic portal vein obstruction from local extrinsic occlusion, or by a prothrombotic disorder, or both. A 56-year-old woman presented with right upper quadrant abdominal pain. Examination and investigations revealed the presence of gallstones, a cavernous portal vein, several calcified hydatid cysts within the liver, grade III haemorrhoids, but no oesophageal varicosities. She had no previous abdominal surgery, and had normal full laboratory workup, including inflammatory markers, clotting analyses, and thrombophilia screen. At open surgery it became apparent that the portal cavernoma had been caused by local pressure from a calcified hydatid cyst of the caudate lobe of the liver. To the best of our knowledge, this is the first report of portal cavernoma caused by a hydatid cyst of the liver.

Glucagonoma without glucagonoma syndrome

INTRODUCTIONnGlucagonomas are rare, frequently malignant tumours, arising from the Langerhans islets of the pancreas. They usually secrete large amounts of glucagon that can cause a characteristic glucagonoma syndrome, which includes necrolytic migratory erythema, glucose intolerance or diabetes, weight loss and sometimes, normochromic normocytic anaemia, stomatitis or cheilitis, diarrhoea or other digestive symptoms, thoromboembolism, hepatosplenomegaly, depression or other psychiatric and paraneoplastic symptoms. In certain cases, some or all glucagonoma symptoms may appear late, or even may be completely absent.nnnCASE OUTLINEnThe authors present a 43-year-old woman in whom an investigation for abdominal pain revealed a tumour of the body of the pancreas. During operation, the tumour of the body of the pancreas extending to the mesentery measuring 85 x 55 x 55 mm was excised. Histology and immunohistochemistry showed malignant glucagonoma, with co-expression of somatostatin in about 5% and pancreatic polypeptide in a few tumour cells. The recovery was uneventful. The patient stayed symptom-free with no signs of local recurrence or distant diseases 15 years after surgery.nnnCONCLUSIONnGlucagonoma syndrome may be absent in glucagonoma tumour patients so that in unclear pancreatic tumours the clinician should frequently request the serum hormone level (including glucagon) measurement by radioimmunoassay and the pathologist should perform immunohistochemistry investigation. Those two would probably result in discovery of more glucagonomas and other neuroendocrine tumours without characteristic clinical syndromes.

Serous microcystic adenoma of the head of the pancreas causing an obstructive jaundice

BACKGROUNDnSerous microcystic adenoma is a rare benign tumor of the exocrine pancreas originating from the ductal system and composed of a large number of small cysts covered by cuboid cells, filled with clear serous fluid and separated with fibrocolagenous stroma. Most frequently it appears in women in 7th and 8th decades, in the distal pancreas. It shows a very low malignant potential. In 2/3 of patients symptoms are uncharacteristic and in 1/3 they are absent When localised within the head of the pancreas it rarely causes an obstructive jaundice.nnnCASE REPORTnWe presented a 61-year-old female patient who for months had had mild and nonspecific abdominal symptoms developing to progressive obstructive jaundice. At surgery we revealed a rather large policystic mass of the head of the pancreas causing not only obstructive jaundice but also a venous stasis by compression and dislocation of the portomesenteric vein. The tumor was removed with pylorus preserving cephalic duodenopancreatectomy (Whipples procedure modified by Longmire-Traverso). Histology confirmed serous microcystic adenoma of the pancreas. The postoperative recovery was uneventful and preoperative symptoms disappeared.nnnCONCLUSIONnAlthough very rare, serous microcystic adenoma might appear within the head of the pancreas and has to be taken into consideration in differential diagnosis of cystic lesions of the head of the pancreas. Very rarely the tumour might cause obstructive jaundice. Surgical resection, which might be demanding, leads to complete recovery.

Cystic dystrophy of the duodenal wall in ectopic pancreas

INTRODUCTIONnCystic dystrophy of the duodenal wall is a rare complication of the ectopic pancreas that is characterized by cyst/s formation within thickened duodenal wall.nnnCASE OUTLINEnA 61-year-old male with recurrent abdominal pain, weight loss (about 25 kg) who had been moderate alcohol abuser and heavy smoker was presented. On ultrasonography, very thickened duodenal wall (2.5 cm), an enlarged head of the pancreas with cyst of 3 cm in diameter as well as dilated pancreatic duct (< 6 mm) were seen. Barium meal showed stenosis of the first and second part of the duodenum. CT and endoscopic ultrasound confirmed the ultrasonographic finding.The patient underwent surgery. The pathologic finding was established only on first two portions of the duodenum and limited part of the head of the pancreas along duodenum while the rest of the pancreas was normal. Due to poor general condition, gastrojejunostomy was performed. Although some improvement was evident, the patient did not become asymptomatic, and, therefore, four months later a cephalic duodenopancreatectomy was carried out which made him fully asymptomatic. A year later, the patient was symptom-free and in good health. Histologic examination showed a cystic dystrophy of the duodenal wall in the ectopic pancreas.nnnCONCLUSIONnUnless there are strong contraindications, cephalic duodenopancreatectomy is best treatment of the disease.

Inflammatory pseudotumours of spleen

INTRODUCTIONnInflammatory pseudotumours are benign lesions of unknown aetiology that can appear almost in any organ, sometimes along with infectious agents like Epstein-Barr virus. They are very rare in the spleen, with about 80 cases described in the world literature. The tumour is a single, very rarely multinodal, lesion. Middle-aged patients are mostly affected. Symptoms are either absent or uncharacteristic so that the tumours are discovered by chance or during the investigations for other diseases. Although the tumour may be suspected before surgery, the exact diagnosis is established by the histological and immunohistochemical examination of the spleen removed as lymphoma or some other lymphoproliferative disorder because they cannot be ruled out.nnnOUTLINE OF CASESnThe authors present 3 female patients 36, 48 and 56 years of age (average age 46.6 years) with the inflammatory pseudotumour of the spleen. Two patients complained of a mild pain in the epigastrium and below the left costal margin, while in the third patient the tumour of the spleen was discovered by chance during the examination after the traffic accident trauma. On examination, only slight epigastric tenderness was found in two patients. Except for the elevated white blood cells in one patient, laboratory data were within normal limits. All 3 patients were submitted to splenectomy as lymphoma of the spleen could not have been ruled out. Two patients developed transient postsplenectomy thrombocytosis which was treated with aspirin. The exact diagnosis of the tumours was established by the histological and immunohistochemical investigation.nnnCONCLUSIONnInflammatory pseudotumours of the spleen may not be so rare as it is believed. Thanks to the new diagnostic modalities (US, CT, MRI), an increasing number of reports of these tumours may be expected. They have to be taken into consideration in the differential diagnosis of solitary or multimodal lesions of the spleen. Splenectomy is a treatment of choice.

Tc-99m labeled RBC scintigraphy of an inflammatory pseudotumor of the spleen.

Inflammatory pseudotumors are very rare benign lesions of an unknown etiology usually discovered accidentally. We present a 26-year-old woman with a nodal lesion discovered in the spleen during a routine check-up by abdominal ultrasound. Radiocolloid and delayed Tc-99m RBC scintigraphy proved the existence of a delineated hypervascular lesion. Histology and immunohistochemistry of the lesion proved an inflammatory pseudotumor, with very dilated sinusoids with blood congestion. The exact diagnosis of inflammatory pseudotumor can be established only by histology and/or immunohistochemistry of the spleen.

Giant Cavernous Hemangioma of the Adrenal Gland: Case Report and Review of the Literature

Adrenal tumors are nowadays being detected with increasing frequency due to the widespread use of various radiological imaging techniques (CT, MRI, US). Incidentally discovered adrenal masses (incidentalomas) are shown in 1% to 5% of all abdominal CT scans performed. Cavernous hemangiomas of the adrenal gland are extremely rare, benign in nature and most usually non-functioning lesions. We report a case of a 50-year-old female who presented with flank pain and abdominal discomfort. MRI of the abdomen revealed a large, oval, adrenal tumor mass, embedded between the upper pole of the right kidney and inferior surface of the liver. Surgery was performed, and tumor was excised completely. Histopathological examination revealed a cavernous hemangioma of the adrenal gland.

Comparison of standard fibrinogen measurement methods with fibrin clot firmness assessed by thromboelastometry in patients with cirrhosis

BACKGROUNDnThe Clauss fibrinogen method and thrombin clotting time (TCT) are still routinely used in patients with cirrhosis to define fibrinogen concentration and clotting potential. The thromboelastometric functional fibrinogen FIBTEM assay evaluates the strength of fibrin-based clots in whole blood, providing information on both quantitative deficit and fibrin polymerization disorders.nnnOBJECTIVEnTo compare these three methods of assessing fibrinogen in patients with cirrhosis of different aetiologies, characterized by impairment in fibrinogen concentration as well as functional aberrance.nnnMETHODSnSixty patients with alcoholic and 24 patients with cholestatic cirrhosis were included (Child-Pugh score (CPs)A, n=24; B, n=32; C, n=28). All parameters were compared with those from a control group. Maximum clot firmness (MCF) in the FIBTEM test was assessed in regard to its relevance in detection of qualitative fibrinogen disorders in comparison with results obtained by standard measurement methods, i.e. the Clauss fibrinogen method and TCT.nnnRESULTSnWith increased cirrhosis severity, fibrinogen and FIBTEM-MCF levels significantly declined (p=0.002), while TCT was significantly prolonged (p=0.002). In all CPs groups, fibrinogen strongly correlated with FIBTEM-MCF (r=0.77, r=0.72, r=0.74; p<0.001), while cross-correlations of other assays were highly variable. The prevalence of decreased FIBTEM-MCF values (<9 mm) was significantly higher in advanced CPs categories (p=0.027), whereby the highest prevalence was detected in patients with CPsC (10/16; 62.5%). Nine of the 16 patients with decreased FIBTEM-MCF values had also decreased fibrinogen levels, while in the remaining 7 patients fibrinogen levels were within the reference range, indicating the possible presence of qualitatively altered fibrinogen that could be detected by FIBTEM-MCF.nnnCONCLUSIONSnFIBTEM-MCF may be considered as a reliable alternative to standard plasma fibrinogen measurement in cirrhotic patients, especially in evaluating fibrin polymerization disorders in these patients. Further studies are needed to evaluate the usefulness of this assay in predicting bleeding complications in cirrhotic patients as well as monitoring replacement treatment.

Mucinous cystadenomas of the liver

INTRODUCTIONnBiliary cystadenomas of the liver are rare benign tumors prone to malignant alteration so that a total excision is recommended.nnnOBJECTIVEnThe aim of the paper is to present our experience in treatment and to evaluate whether a simple ablation represents the appropriate treatment.nnnMETHODSnOver a 10-year period 25 patients (24 women) of the average age of 58 years suffering from cystadenomas of the liver, 18 in the right, 4 in the left and 3 in both lobes of the liver were operated. Twenty-three patients had a single lesion, while two patients had 3 and 6 lesions, respectively. Pain was present in 20, occasional vomiting in 4, discomfort in 2 and a sense of fullness in 2 patients.Three patients were jaundiced, 1 due to cystadenoma of the liver, 2 due to concomitant tumors of the head of the pancreas, while 5 patients had concomitant diseases.nnnRESULTSnA total ablation was performed in 22 patients, left lateral bisegmentectomy in 1 and partial excision in 2 patients. Six additional procedures were performed. Five cystadenomas of the liver had,,ovarian like stroma, all in women. A focal malignant alteration was found in 2 patients aged 66 and 79 years, respectively. Recurrence was registered in 1 female patient in whom a partial excision had been done. Two patients with concomitant malignancy and 1 patient who developed malignant histiocytosis six months after surgery, died after 1, 2 and 3 years, respectively.nnnCONCLUSIONnBiliary cystadenomas of the liver may be misdiagnosed as simple liver cysts, so that ,,frozen section histology is highly recommended. In most cases the tumor may be successfully treated by ablation up to the healthy liver tissue. Major liver resections are rarely necessary.