Radoje Colovic

Primary malignant fibrous histiocytoma of the spleen and liver

Malignant fibrous histiocytoma (MFH) is a distinct and pleomorphic form of sarcoma, which usually occurs in soft tissues but can be found in bones, kidney, larynx, lung, heart, and even aorta. Since the first description of MFH of the spleen by Govoni et al. in 1982, only 10 case have been reported in the literature worlwide. We report on a 45-yr-old female with MFH of the spleen and liver, with special emphasizes on immunohistochemical findings.

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

INTRODUCTION Gastrointestinal 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. CASE REPORT We 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. CONCLUSION This 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.

Fibromyxoid sarcoma of the pancreas

INTRODUCTION Fibromyxoid sarcoma is a rare mesenchymal neoplasm, usually appearing in the soft tissue of the extremities, less frequently in the groin, trunk, neck, and upper extremities. Within the abdomen, the tumour is usually localised within the retroperitoneum. CASE OUTLINE We present a 56-year-old woman in whom, during the routinely performed investigation for atacks of choking with lots of bronchial secretion, and arterial hypertension, an ultrasonographer found a tumour within the head of the pancreas 6 x 6 cm in diameter. At operation, a dark pink, lobulated soft tumour, surrounded by a tiny capsule, clearly different from the completely normal pancreatic tissue of the posterior side of the head of the pancreas, was easily and ideally excised. The postoperative recovery was stormy. She developed postoperative pancreatitis, temporary biliary and duodenal fistula, which all settled by conservative treatment. The histology of the 80 g weighing tumour showed a circumscribed fibromyxoid sarcoma of low malignancy. Immunohistochemistry showed diffuse vimentin and CD34 strong positivity, as well as focal anti-SMA and anti-EMA immunopositivity. Six months after surgery, she died with signs of cerebrovascular insult, asthmatic status, and recurrent suppurative abdominal fistula, probably related to the previous pancreatitis. Ultrasonography showed a possible liver secondary. The exact cause of death was not confirmed as the autopsy was refused by the family. CONCLUSION Primary sarcomas of the pancreas are very rare, but should be considered in differential diagnosis of pancreatic neoplasms. To the best of our knowledge, there has been no previously described fibromyxoid sarcoma of the pancreas.

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.

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.

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

BACKGROUND Serous 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. CASE REPORT We 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. CONCLUSION Although 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.

Coronary artery revascularization prior to abdominal nonvascular surgery

BACKGROUND Does preoperative revascularization of the myocardium reduce cardiac risk in noncardiac surgery? The aim of this study was to evaluate the clinical effectiveness of preoperative cardioprotection by coronary artery revascularization in abdominal nonvascular surgery under general anesthesia. MATERIALS AND METHODS The observational clinical study included 111 consecutive patients with angiographically verified coronary artery disease. Two stratification groups of patients were compared, those with coronary artery revascularization (34 patients, 30.6%) and those without coronary artery revascularization (77 patients, 64.9%), in relation to frequency of perioperative cardiac complications. The patients were followed up until the 30th postoperative day. During operation and in the following 72 postoperative hours, the patients were monitored by continuous ST-T segment recording. Twelve-lead electrocardiography was performed immediately after surgery and on postoperative days 1, 2, and 7 as well as 1 day before discharge. Serum troponin T levels were controlled at 6, 24, and 96 h postoperatively. RESULTS The number of patients with major cardiac complications was 0 (0.0%, n=34) in the revascularized myocardium group and 10 (12.9%, n=77) in the nonrevascularized myocardium group (P<.05). Three patients in the nonrevascularized myocardium group died of acute myocardial infarction, congestive heart failure, and malignant arrhythmias, respectively, with severe coronary artery stenosis verified angiographically. CONCLUSIONS Preoperative cardioprotection by coronary artery revascularization significantly reduces morbidity and mortality in patients who have undergone abdominal nonvascular surgery. Patients with severe coronary artery stenosis and indication for coronary artery revascularization independently of noncardiac surgery should first undergo cardiosurgical intervention prior to elective abdominal nonvascular surgery.

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.

Tubular adenoma of the gallbladder with squamous metaplasia

Adenomas of the gallbladder are rare tumors. They may be papillar or tubular. Tubular adenoma appears in middle aged and older patients, although it may develop even in children. This is a case report of forty-year old woman in whom a polypoid lesion was discovered in the gallbladder during regular ultrasonographic control after the liver surgery for hemangioma. The gallbladder was removed during an open surgery. A polypoid tumour of 20x7x5mm in size on wide pedicle was found in the gallbladder. Histology showed tubular adenoma with mild epithelial dysplasia in some places and with antral and squamous metaplasia. The recovery was uneventful.

Primary pancreatic non-Hodgkin's lymphoma

Diffuse large-cell B lymphoma of the pancreas is a rare disease, representing less than 1% of all non-Hodgkins lymphomas and less than 0.9% of all malignant tumours of the pancreas. About 150 cases of the disease have been observed so far. The tumours are more frequent in the head of the pancreas then in other parts of the organ. They are usually larger (average size of 8 cm) and are non-resectionable. As a rule, exact diagnosis is based on the histology and the immunohistology of the specimen taken during open surgery performed for general diagnosis of the pancreatic tumour. Very rarely can a very reliable and experienced cytopathologist establish a proper diagnosis based on material obtained from a fine needle biopsy. The disease usually responds positively to immunochemotherapy according to protocol R-CHOP. Occasionally, additional radiotherapy may be required. We present two women, 66 and 49 years old, in whom a diagnosis of large-cell B lymphoma of the pancreas was established, based on the histology and the immunohistochemistry of a specimen taken during open surgery performed in order to remove pancreatic tumours, which turned out to be non-resectionable. After immunochemotherapy, the symptoms disappeared and the tumours shrank, in one patient after additional radiotherapy. The authors would like to point out the importance of a proper histological diagnosis, which permitted the application of immunochemotherapy alone or together with additional radiotherapy with at least temporarily favourable results.