Natural history and experience with diagnosis and treatment of the Zollinger-Ellison syndrome.

With better methods of diagnosis, patients will be identified earlier in the course of their disease and will often have atypical and borderline manifestations of the syndrome. Serum gastrin measurements with calcium and especially with secretin challenge will be the most important method of diagnosis. Any patient with acid hypersecretion who has a high serum gastrin level that does higher on secretin infusion should be considered to have the Zollinger-Ellison syndrome. A firm diagnosis of the Zollinger-Ellison syndrome should be made, if at all possible, prior to operation. At operation, a thorough search of the pancreas, duodenum, stomach, greater and lesser omentum and liver should be made for primary and secondary gastrinomas. If the preoperative data firmly establish the diagnosis of the Zollinger-Ellison syndrome, a total gastrectomy should be carried out even if no primary tumor is found. Similarly, a total gastrectomy should be done even if there are massive hepatic metastases. If total gastrectomy is not performed, the patient is apt to die of complications of acid hypersecretion. The only possible exceptions to the rule of always performing a total gastrectomy are in asymptomatic patients with easily excisable tumors or patients with tumors of the duodenum that are easily excisable, providing that in both instances after the excision of the tumor the output of gastric acid as measured at operation is immediately halted. All possible metastatic tumor tissue should be removed. The more tumor tissue removed, the longer the patient will survive. Metastases should be treated aggressively. They do not disappear after total gastrectomy in our experience, and they may kill patients. Patients should be followed after operation with serial measurements of serum gastrin concentrations and by hepatic scintillation scans and hepatic angiography. If hepatic metastases develop, intrahepatic artery infusions of 5-fluorouracil may slow tumor growth.     

Myocardial infarction in the 34th week of gestation: case report

Acute myocardial infarction during pregnancy is a rare event that is often associated with a very high maternal mortality, estimated to be from 19 to 37%. During the last decades the incidence of myocardial infarction during pregnancy has increased . The main contributing factor could be a higher prevalence of the metabolic syndrome. The strongest predictors correlated with a myocardial infarction are hypertension, diabetes mellitus and advanced maternal age. In addition, improved diagnostic tools could explain the elevated incidence of myocardial infarction during pregnancy. In general gestation is not considered a risk factor for myocardial infarction but gravidity is accompanied by an increase in oestrogen and progesterone levels. It is generally accepted that oral contraceptives increase the risk of coronary heart disease. We present a case where a 37-year-old gravida was admitted to hospital with diffuse thoracic pain. In the patient's history, we found several putative reasons for the thoracic pain that pointed to a musculoskeletal cause. Based on an elevation of ischaemic heart markers and continuous non-specific thoracic pain we performed a primary Cesarean section. In the coronary angiography procedure that followed, a thrombotic occlusion of the ramus diagonalis was diagnosed. We here describe the differential diagnosis as well as the problems associated with diagnosing myocardial infarction in the third trimester of pregnancy.     

Small cell carcinoma of the cervix: a clinical study of 15 patients and review of the literature

Objective: Small cell carcinoma of the cervix is a very rare and aggressive tumor. Most gynecologic oncology centers have little experience with this tumor. The purpose of the study was to evaluate the University of Kansas’ experience with this tumor and compare findings with those found in current literature.Methods: Fifteen patients with small cell carcinoma of the cervix were treated at or in association with Kansas University Medical Center between 1977 and 1997. Clinical data including age of patient, pregnancy history, stage of tumor, recurrence, type of therapy, presenting symptoms, location of metastases, and overall survival were studied.Results: Age at diagnosis ranged from 20 to 83 years with a mean of 47. Two patients were nulliparous, 2 were primiparous, and 11 were multiparous. Five patients (33%) were stage I, 3 (20%) were stage II, 1 (7%) was stage III, and 6 (40%) were stage IV at diagnosis. Four patients (27%) had progressive courses without good response to treatment, 7 (47%) recurred at an average of 15 months. The patients were treated with surgery, radiation, chemotherapy, or a combination thereof. Extrapelvic metastases developed in 5 of 8 patients with stage I or stage II disease with distant nodes, liver, lung, and brain being common sites. Three patients (20%) developed brain metastases. Tumor lysis syndrome was encountered in one patient. One patient was alive and well 80 months after diagnosis and one patient was lost to follow-up. The remaining 13 died of their disease. Mean survival was 22.3 months for stage Ib, 40 months for stage II, 27 months for stage IIb, 8 months for stage III, and 19.2 months for stage IV.Conclusions: Small cell carcinoma of the cervix is a rare and aggressive variant of cervical cancer. Our experience with this tumor raises the question of increased incidence of central nervous system metastases with small cell carcinoma. Present therapy has not significantly improved outcome with this tumor. There is conflicting evidence regarding the etiologic role of an infectious agent. Tumor lysis syndrome is a possible risk when treating these patients.     

Choriocarcinoma with diffuse intraabdominal abscess and disseminated intravascular coagulation. A case report

BACKGROUND: Management of choriocarcinoma complicated by diffused intraabdominal abscess is difficult, especially when disseminated intravascular coagulation (DIC) ensues. CASE: A 29-year-old woman presented with massive vaginal bleeding, fever and severe abdominal pain. Choriocarcinoma with pulmonary and vaginal metastases was diagnosed along with diffused intraabdominal abscess. Hysterectomy and hypogastric artery ligation were performed after the fever and abdominal symptoms failed to respond to intravenous antibiotics. Although the patient developed DIC after surgery, transfusion, antibiotics and immediate combination chemotherapy improved her condition and controlled the malignancy. She was free of disease for > 20 months after treatment. CONCLUSION: Timely surgery, aggressive antibiotics and immediate postoperative chemotherapy are recommended for patients with choriocarcinoma complicated by intraabdominal abscess and DIC.     

Neonatal myocardial infarction due to thrombotic occlusion

Neonatal myocardial infarction is rare and its prognosis is poor. We describe the clinical course and autopsy findings of a newborn female with myocardial infarction. Her clinical course was rapidly progressive, becoming fatal before we could detect the cause. Autopsy demonstrated significant occlusion of the left coronary artery as well as evidence of new infarction, suggesting that the event occurred at birth. This case illustrates myocardial infarction as a possible cause of early neonatal death.     

Neonatal myocardial infarction due to thrombotic occlusion.

Neonatal myocardial infarction is rare and its prognosis is poor. We describe the clinical course and autopsy findings of a newborn female with myocardial infarction. Her clinical course was rapidly progressive, becoming fatal before we could detect the cause. Autopsy demonstrated significant occlusion of the left coronary artery as well as evidence of new infarction, suggesting that the event occurred at birth. This case illustrates myocardial infarction as a possible cause of early neonatal death.     

Takotsubo cardiomyopathy--transient left ventricular apical ballooning mimicking acute myocardial infarction.

Takotsubo cardiomyopathy is characterized by transient left ventricular dysfunction with clinical symptoms of chest pain, electrocardiographic changes of ST-segment elevation or T wave inversion, which mimics acute myocardial infarction in patients without angiographically significant coronary artery stenosis. We report a 75-year-old woman with a history of chest tightness who presented with typical pictures of takotsubo cardiomyopathy. Acute myocardial infarction was initially diagnosed based on the electrocardiographic changes and elevated troponin. Apical akinesis and ballooning with basal hyperkinesis were noted during left ventriculography. Coronary angiography, however, did not show significant coronary artery stenosis. Electrocardiography was normal 3 months later. Follow-up echocardiography did not show any wall motion abnormality. This patient remained well without chest pain or dyspnea over 24 months of follow-up. Optimal medical management of takotsubo cardiomyopathy remains unclear. This patient received diltiazem to prevent possible coronary artery spasm. The prognosis of this syndrome seems to be favorable except for occasional mortality due to left ventricular rupture or ventricular arrhythmia. Recurrence of this syndrome is rare.     

The significance of elevated peritoneal fluid phosphate level in intestinal infarction

Early diagnosis seems mandatory if the mortality of mesenteric vascular occlusion is to be altered. A model has been developed in which intestinal infarction has been produced by ligation of either the superior mesenteric vein or the superior mesenteric artery. Results of earlier work, using this model, have shown a significant rise in the serum inorganic phosphate level and an associated severe metabolic acidosis. This article has confirmed these results as being statistically significant. In this experiment, a significant rise in the inorganic phosphate level of the peritoneal fluid has been shown in the same model. We suggest that, in the patient with possible intestinal infarction, an elevated serum phosphate level, elevated peritoneal fluid phosphate level, base deficit and leukocytosis may be useful in making an earlier diagnosis of this disease. If an earlier diagnosis is accomplished, the morbidity and mortality of this lethal disease hopefully will be reduced.     

Limb sparing operations for sarcomas of the extremities involving critical arterial circulation

Seven patients underwent resection of a sarcoma of the extremity requiring excision of a vital artery and revascularization. These included osteosarcoma of the pubis, osteosarcoma of the distal femur, undifferentiated sarcoma of the thigh, liposarcoma of the thigh, liposarcoma of the popliteal space, chondrosarcoma of the proximal tibia and rhabdomyosarcoma of the thumb. Preoperative evaluation included computed tomographic scan, magnetic resonance imaging and angiography. Operation involved excision of the iliac artery in one instance, femoral artery in three, popliteal artery in two instances and radial artery in one. Wide local resection including revascularization was undertaken when the tumor could be resected with the artery but separated from the nerve and it was a low grade malignant disease or the patient refused amputation for a high grade malignant disease. In five patients, the tumor margins were adequate. One patient with an osteosarcoma of the pubis with distant disease had a palliative resection and one patient had positive margins but refused amputation. Coverage of the soft tissue and vascular grafts was achieved using a distant pedicle flap in two patients. Amputation was avoided and each patient remained ambulatory. Five patients remained free of disease with patent grafts at six months to six years of follow-up study. One patient died of late myocardial infarction and one who underwent palliative resection died eight months later of metastatic disease. Involvement of the major arterial circulation does not preclude adequate resection of sarcomas of the extremity with limb salvage.     

HELLP syndrome, multifactorial thrombophilia and postpartum myocardial infarction

A case of postpartum acute myocardial infarction with intraventricular thrombus occurred in a woman with HELLP syndrome. Since coronary artery disease was ruled out angiographically, the assumed pathophysiological mechanism for myocardial malperfusion was intermittend coronary vasospasm and thrombosis. There were several thrombophilic risk factors detectable (heterozygous factor V Leiden, low levels of antithrombin III, protein S deficiency), whose possible impact in this rare but severe clinical condition is discussed.     

Operative risk of general surgical procedures in patients with previous myocardial revascularization

Eighty general surgical procedures with general anesthesia were performed upon 73 patients who had undergone previous myocardial revascularization for significant coronary artery disease. No deaths occurred in this group of patients. Cardiac complications occurred in two patients. A silent myocardial infarction occurred in one patient while another required a permanent cardiac pacemaker for complete heart block. Six noncardiac complications developed in four patients. We concluded that patients with significant coronary artery disease who have undergone previous myocardial revascularization can tolerate subsequent general surgical procedures with limited risk.     

Fatal Myocardial Infarction Associated with Bromocriptine for Postpartum Lactation Suppression

Summary: Bromocriptine (Parlodel®) has attracted widespread controversy for its use for postpartum lactation suppression because of recent reports of cerebral and cardiovascular complications. This case describes a maternal death in which bromocriptine therapy may have triggered myocardial infarction in a patient with asymptomatic coronary artery disease. We suggest its use with caution, especially in patients with identifiable risk factors of coronary artery disease or arteriovascular disease.     

Fatal Myocardial Infarction Associated with Bromocriptine for Postpartum Lactation Suppression

Summary: Bromocriptine (Parlodel®) has attracted widespread controversy for its use for postpartum lactation suppression because of recent reports of cerebral and cardiovascular complications. This case describes a maternal death in which bromocriptine therapy may have triggered myocardial infarction in a patient with asymptomatic coronary artery disease. We suggest its use with caution, especially in patients with identifiable risk factors of coronary artery disease or arteriovascular disease.     

Complete myocardial revascularization using only pedicled arterial conduits in Kawasaki disease.

A 16-year-old boy with Kawasaki disease suffered from progressive angina and exercise intolerance for 1 year. Coronary angiography showed 60% stenosis of the left main coronary artery, a calcified aneurysm with total occlusion at the proximal left anterior descending artery (LAD), and another aneurysm with total occlusion at the middle portion of the right coronary artery. Aortocoronary bypass was done with the left internal mammary artery (IMA) anastomosed to the first obtuse marginal branch, the right IMA to the distal LAD, and the right gastroepiploic artery to the posterior descending artery. Graft patency was documented by follow-up coronary angiography 1 month after surgery. During follow-up, his rapid improvement led to an upgrade from New York Heart Association functional class III to class I. The results of the present case suggest that complete myocardial revascularization using arterial conduits in patients with Kawasaki disease with coronary artery occlusion is safe and effective.     

Arteriosclerosis in popliteal artery trifurcation as a predictor for myocardial infarction after arterial reconstructive operation

The frequency of myocardial infarction and mortality within 30 days after lower limb vascular reconstruction, in relation to the extent of atherosclerotic lesions in the trifurcation of the popliteal artery, have been retrospectively analyzed in 158 consecutive patients reconstructed in the aortoiliac region and 239 consecutive patients undergoing femorodistal bypass. Among the patients without trifurcational disease (TFD) none had myocardial infarction develop postoperatively, as compared with four of the 50 patients with TFD in the aortoiliac series (p less than 0.05) and 18 of the 174 patients with TFD in the femorodistal series (p less than 0.05). In nine instances, the cause of death was myocardial infarction. The strong correlation between postoperative myocardial infarction and the presence of TFD, may be due to a direct correlation between coronary artery disease and TFD. The finding is of practical importance in the selection of treatment for patients with circulatory disorders of the lower limbs. The finding facilitates the preoperative identification of patients liable to have myocardial infarction develop. Indications for operation can be made more stringent and optimal intraoperative and postoperative monitoring can be instituted.     

Brain metastases from endometrial carcinoma: a retrospective study

OBJECTIVE: The objective of this study was to examine the characteristics and treatment results of patients at our center with brain metastases from endometrial carcinoma. METHODS: Between January 1991 and March 2003, there were 1295 women referred to the London Regional Cancer Centre with the diagnosis of endometrial cancer, and eight of these women (0.6%) developed brain metastases. The medical records of these eight women were reviewed to assess patient and tumor characteristics at primary diagnosis. Treatment and clinical outcomes were analyzed. RESULTS: The majority of patients had poor prognostic factors associated with the primary tumor, including high grade and advanced stage. The median age at diagnosis of brain metastases was 66 years. The median interval between completion of primary tumor treatment and diagnosis of brain metastases was 2 months. Three patients had no other evidence of systemic disease, while five had disseminated disease. Four patients had a single brain metastasis, while four had multiple lesions. Seven patients received whole brain radiation therapy in addition to systemic steroids, of which six had temporary improvement or resolution in symptoms. Median survival following diagnosis of brain metastases was 3.5 months. CONCLUSIONS: Brain metastases from endometrial cancer are uncommon. Associated factors include high grade and advanced stage. The majority of women in this series presented with brain metastases shortly after completion of primary treatment for their endometrial cancer. The prognosis following brain metastases is generally very poor. Innovative treatment strategies are needed to improve the outcome of these patients.     

Postpartum Budd-Chiari syndrome with prolonged hypercoagulability state

Budd-Chiari syndrome after pregnancy is an extremely rare disease. Reported here is a case of postpartum Budd-Chiari syndrome with unusual features of prolonged hypercoagulability state. The disease occurred 2 weeks after delivery and despite massive anticoagulation treatment the patient developed severe hepatic vein occlusion, renal vein thrombosis, inferior vena cava thrombosis, and femoral artery thrombosis.     

Sertoli cell tumor in androgen insensitivity syndrome--a case report

A 26-year-old individual with androgen insensitivity syndrome was operated on for a 3200-g Sertoli cell tumor of the left gonad with retroperitoneal metastases. Six courses of bleomycin, etoposide, and cisplatin chemotherapy followed surgical treatment. Eighteen months after the initial surgery the patient is free of disease and in good health. The association of Sertoli cell tumor with androgen insensitivity syndrome is discussed and the relevant literature is briefly reviewed.     

Ovarian metastasis of a primary renal cell carcinoma: case report and review of literature

Ovarian metastases from renal cell carcinoma (RCC) are very rare, with only 23 cases reported in the literature. We report a case of 54-year-old women who developed bilateral ovarian metastasis 39 months after diagnosis of clear cell carcinoma. Total abdominal hysterectomy with bilateral salpingo-oophorectomy was carried out. Subsequently she was treated with sunitinib and her disease stabilized. She is still alive four years after diagnosis of the renal primary, and disease has stabilized on sunitinib. We conclude that, although rare, the possibility of metastatic RCC should be considered in the differential diagnosis of clear cell tumors of the ovary. Due to therapeutic and prognostic implications, it is very important to differentiate if the tumor is a primary ovarian tumor or a metastasis from a renal cell carcinoma. Early diagnosis of this rare metastatic tumor results in prompt treatment and prolonged patient survival.     

Recurrence and rapid metastasis formation of a granular cell tumor of the vulva

Histologically confirmed local recurrence of the rare entity of a granular cell tumor of the vulva was diagnosed in a 55-year-old patient with no signs of distant metastasis. Intraoperatively (local excision of mons pubis and inguinal lymphnodes), widespread regional metastasis with retroperitoneal lymphnode metastases were found. Postoperative restaging detected pulmonary, hepatic and skeletal metastases and the patient died of her disseminated disease within 4 months. Early diagnosis of the malignant phenotype of granular cell tumor using expression of S-100-protein, MIB-1, vimentin and p53 is suggested. Once a malignant granular cell tumor is diagnosed histologically, thorough staging procedure should be performed to exclude disseminated disease. If this is the case, radical surgery should be tried due to the lack of efficiency of radiotherapy and of systemic treatments.