Obstruction of the right coronary artery ostium by an aortic valve papillary fibroelastoma

Aortic valve papillary fibroelastomas are rare benign tumours which may present with the sequele of embolisation, coronary occlusion or sudden death. We report a case of a 68 year old lady presenting with unstable angina who appeared at coronary angiography to have thrombus in the right coronary ostium. Increasing right territory ischaemia prompted echocardiography, which diagnosed a mass in the right coronary ostium. Urgent surgery was undertaken, demonstrating chronic impaction of an aortic valve fibroelastoma in the right coronary ostium. Aortic valve replacement and coronary bypass grafting to the distal right coronary artery (RCA) was performed. This case illustrates a common cardiology presentation caused by the complication of a rare tumour.     

Primary cardiac angiosarcoma with right coronary-to-right atrium fistula

Primary tumours of the heart are rare. About 25% of all cardiac tumours are malignant and the most common of these is the angiosarcoma. We present a 61-year-old male with a right atrial angiosarcoma that was detected on coronary angiography. The tumour showed marked vascularity and a right coronary-to-right atrium fistula, and the patient underwent surgical resection. Pathological examination of the tumour was consistent with a cardiac angiosacoma and the diagnosis was also confirmed by immuno-histochemistry. He consequently underwent chemotherapy, however the patient died 60 days after the surgery.     

A critical reappraisal of MIB-1 labelling index significance in a large series of pituitary tumours: secreting versus non-secreting adenomas

Pituitary tumours are usually benign neoplasia, but may have a locally aggressive or malignant evolution. This study aimed to identify factors which mostly influence their proliferative activity, in order to clarify its value for clinical and research purposes. The proliferative index was determined in a prospective series of 132 pituitary tumours as the percentage of monoclonal antibody MIB-1-immunopositive cells and referred to as the MIB-1 labelling index (LI). Its distribution was analysed according to both univariate and multivariate models. A life-threatening pituitary tumour is presented separately. The mean LI was 1.24+/-1.59%, with significant differences between clinically secreting (CS) and clinically non-secreting (CNS) adenomas. In CS adenomas (n=65), LI was highly variable and markedly influenced by pre-operative pharmacological treatment (0.80+/-1.03 vs 2.06+/-2.39% in treated vs untreated cases, P=0.009); it decreased with patient's age (P=0.025, r=0.28) and increased with tumour volume and invasiveness. The influence of pre-operative treatment and macroscopic features on LI in this group was confirmed by multivariate analysis. In CNS adenomas (n=67), LI distribution was less variable than in CS adenomas (P<0.0001), it was age-independent and correlations with tumour volume, invasiveness or recurrence did not reach significance. In a rapidly growing parasellar tumour, the mean LI was 24% at first surgery and exceeded 50% at second surgery performed 4 months later. LI should be interpreted according to hormone secretion and pre-operative treatment. Unusually high LI values deserve particular attention.     

Hepatic angiomyolipoma pre-operatively diagnosed by imaging

An asymptomatic patient with pre-operatively diagnosed solitary angiomyolipoma is reported. The tumour was a well defined, fat density mass on computerized tomography (CT) and magnetic resonance imaging (MRI), and a hyperechoic nodule with a sharp margin on ultrasonography (US). The lesion was hypervascular on arteriography and CT during hepatic angiography. Chemical shift MRI confirmed the fat component within the lesion. Although a review of the literature shows this tumour to be rare and difficult to differentiate from a malignant lesion pre-operatively, the pre-operative diagnosis of angiomyolipoma is considered feasible when the characteristic findings as described here are present.     

Percutaneous portal vein embolisation in preparation for extended hepatic resection of primary nonresectable liver tumours

BACKGROUND: In patients with malignant primary and secondary liver tumours or proximal bile duct carcinoma radical surgery is superior to all other therapeutic modalities in terms of survival and quality of life. Radical resection, however, often requires the removal of a large amount of liver parenchyma, resulting in a marked reduction of functional liver tissue with the risk of liver failure. AIM: Preoperative partial portal vein embolisation induces hypertrophy of the controlateral liver and thereby increases the safety of extended liver resections. PATIENTS AND METHODS: Between January 1997 and February 2001 we applied this strategy in 19 patients with primary and secondary nonresectable hepatobiliary malignancies, in whom the estimated amount of the remnant liver was < or =25% of the liver volume. RESULTS: The increase in volume ranged between 7 and 245%. Radical extended liver resection was performed in 13 patients (68%) without mortality. After a mean observation time of 22 months patient survival was 19 months with six tumour-related deaths during the second year after surgery. The remaining seven patients are alive and well with tumour recurrence in one. CONCLUSION: Preoperative partial portal vein embolisation allows more patients with previously unresectable liver tumours to benefit from a potentially curative resection.     

Localization of gastroenteropancreatic tumours by angiography

Neuroendocrine tumours are seen on arteriography as diffusely enhancing masses without tumour vessels and without arteriovenous shunting. In 70 patients with surgically proven tumours, the sensitivity of angiography was 68% for extrapancreatic and 86% for hepatic lesions. Hepatic metastases have always been easier to demonstrate arteriographically than the primary tumour because of the absence of overlying bowel. Portal venous sampling is a sensitive technique for detecting functioning gastroenteropancreatic tumours. Sampling the small veins about the pancreatic head yielded a sensitivity of 62% but this is an invasive procedure in which considerable experience is required. Intra-arterial secretagogue, secretin for gastrinomas and calcium for insulinomas, selectively injected into the pancreatic and the hepatic arteries produce a diagnostic gastrin or insulin gradient respectively. The localization sensitivity of arterial stimulation with venous sampling is 77-89% for gastrinoma and 92% for pancreatic insulinoma. Recently, spiral CT in conjunction with selective intra-arterial rather than intravenous injection of contrast may increase the detection sensitivity of duodenal and pancreatic gastrinomas.     

Hepatic artery embolisation--new approach for treatment of malignant carcinoid syndrome

A transcatheter embolisation was carried out for treatment of a patient suffering from hepatic metastases of a malignant carcinoid tumour. Recurrent and very severe carcinoid symptoms could be observed; a bronchial carcinoid supposingly the primary tumour without characteristic symptoms was removed six years before. The carcinoid symptoms became resistant to pharmacological agents and finally ended in life-threatening clinical complications. The transcatheter hepatic artery embolisation was successfully performed and repeated three months later. After embolisation relief of carcinoid symptom and a significant decrease in 5-hydroxyindole acetic acid (5-HIAA) urinary excretion lasting for eight months could be observed. There were no serious complications with adequate pharmacological cover, however, a transient fever, leucocytosis, abdominal pain and an increase in serum transaminase activities occurred after the procedure. The transcatheter hepatic artery embolisation should be a method of choice for treatment of patients with carcinoid metastases producing severe carcinoid symptoms resistant to pharmacological agents.     

Echocardiography of an operated case of a myxoma of the tricuspid valve

The case of a 6 year old child with a tricuspid valve myxoma is presented. Early diagnosis by echocardiography, confirmed by angiography, resulted in surgical cure before any symptoms appeared. This is the only reported echocardiographic study of a myxoma inserted on the tricuspid valve found in a review of the literature of 6 cases of right ventricular myxoma. The tricuspid valves were found to be thickened and prolapsed into the right atrium. The amplitude of excursion of the anterior leaflet was greatly increased, its mass projecting into the right ventricular outflow tract in diastole and remaining there until the end of right ventricular ejection. The echocardiographic signs localising the precise origin of the tumor in the tricuspid valve are developed. The differential diagnosis with right atrial myxoma prolapsing into the ventricular cavity, benign and malignant right ventricular tumours and tricuspid valve vegetations is discussed. The precision of the diagnostic information obtained by echocardiography suggests that angiography may not be essential any longer, especially when the risk of embolisation and acute obstruction are taken into consideration.     

Non-myxomatous cardiac tumours: twenty-year experience

Eighty-eight patients underwent surgery for various cardiac tumours from January 1978 to June 1998 at our Institute. Seventy-seven tumours were myxomas, 10 were non-myxomatous and one was secondary cardiac tumour. Case records of the patients with non-myxomatous primary cardiac tumours and one secondary tumour were reviewed. Six of these primary tumours were benign and four, malignant. Age of the patients ranged from 26 days to 47 years. Among patients (3 children, 8 adults) with non-myxomatous primary cardiac tumours, dyspnoea on exertion was the commonest symptom and was the cause of presentation in seven out of 11 patients. Of the eight adults, six were in New York Heart Association functional class II/III and two in class IV. Echocardiographic diagnosis was possible in all the patients. Complete excision of the tumour was possible in all benign and two of the four malignant tumours. Incomplete resection was done in the secondary tumour. Of the six benign tumours, three were rhabdomyomas and one each of fibroma, haemangioma and lipoma. The malignant tumours were one each of fibrosarcoma, angiosarcoma, unclassified sarcoma and malignant mesothelioma. The secondary tumour was a malignant thymoma. Follow-up ranged from 1 to 10 years (mean 7.2 years). Of the patients with benign tumours, four out of six are alive; one patient died on the first post-operative day and one lost to follow-up. Two of the four patients with malignant cardiac tumours died, one was lost to follow-up and one is alive two years after surgery. The patient with secondary malignant thymoma to the superior vena cava was lost to follow-up three months after an uneventful recovery from surgery.     

Pancreatic endocrine tumors with digestive effects. Physiopathological and clinical aspects

Endocrine tumours of the gastro-intestinal tract, especially pancreatic, play an important role in the regulation of digestive function and in the embryogenesis of the endocrine cells of the body. Histologically, about 50 p. 100 of these tumours are composed of several types of endocrine cell. However, in the majority of cases, the clinical and biological syndrome that they produce is related to the hypersecretion of only one of the peptide hormones secreted. One point of interest is the lack of correlation between tumour size, plasma concentration of the secreted peptide hormones, the effects of these endocrine oversecretions on the target organ(s) and the severity of the clinical presentation. This suggests phenomena of adaptation at each stage (tumour, peritumoral tissues and target organ). The diagnosis of gastro-intestinal endocrine tumors has been significantly improved by the development of reliable radioimmunological assays of the digestive hormones and modern techniques of medical imagery (ultrasonography, CAT scanning, selective portal angiography, aspiration biopsy under ultrasonic control). The prognosis of these tumours which are often malignant is better than that of gastro-intestinal adenocarcinoma. This justifies therapeutic intervention with the twin aim of controlling the effects of endocrine hypersecretion and tumour reduction. Surgical excision of the tumour should always be considered; if the tumour process is malignant chemotherapy will be required currently based on the association of 5-fluoro-uracil and streptozotocin.     

Medullary Thyroid Carcinoma

The clinical features of a 45 year old woman with proven medullary carcinoma of the thyroid are described. The disease ran a typical prolonged course over 12 years with local recurrence of the tumour. Generalised spread of the carcinoma was associated with persistent and intractable diarrhoea for which no local bowel pathology could be ascertained. She also had hilar lymphadenopathy and a recurrent bloody malignant pericardial effusion with cardiac tamponade. Biochemical investigations revealed normal thyroid function with no extrathyroidal uptake of a thyroid marker, and normal calcium and phosphorous metabolism. Plasma calcitonin assays showed a grossly elevated level of 8 MRC milli units/ml, which rose after an intravenous infusion of calcium chloride. Biopsy of tumour metastases demonstrated small spindle shaped neoplastic cells arranged in large alveolar groups and amyloid stroma. This case demonstrates many of the typical clinical, biochemical and histological features of the disease and adds further evidence that medullary carcinoma of the thyroid is a tumour not of thyroid tissue but of the parafollicular or C cells of the thyroid which produce calcitonin. Furthermore, pre-operative assay of plasma calcitonin may be of diagnostic value in cases of thyroid tumours.     

Neoplastic heart disease -- the Muenster experience with 108 patients

BACKGROUND: Tumours of the heart are rare. Different histological subtypes are known. The most common tumour entity is benign cardiac myxoma. Malignant heart tumours are less common. Tumours originating in other organs such as the kidney may also affect the heart by tumour progression via the inferior caval vein. A large experience with surgical treatment of different types of heart tumours is presented. METHODS: Between January 1989 and April 2004, 108 patients with a heart tumour were included in a database. All patients underwent radical surgical resection, except for 2 patients who had malignant lymphoma of the heart. RESULTS: Histological findings included 78 myxomas (72.2 %), and 6 other benign cardiac tumours in 5.6 % of the patients. Primary malignant heart tumours were seen in 10 (9.2 %) and renal cell carcinoma with cardiac involvement in 6 (5.6 %) patients. Eight patients presented with tumour metastases inside the heart (7.4 %). Mean overall survival was 12.7 years for myxoma patients and 5.6 years for patients with other benign heart tumours. Patients with primary malignant heart tumours survived 5.5 years on average. CONCLUSIONS: Heart tumours are rare, but usually life-threatening. Radical surgical resection is the therapy of choice and may offer excellent long-term survival, even in cases with malignant heart tumours.     

Cardiac tumours simulating collagen vascular disease.

Cardiac tumours can mimic collagen vascular disease and they are often accompanied by profound systemic upset. Both benign and malignant tumours may present in this way. Three cases of cardiac tumour, two malignant and one benign, are reported with just such a presentation. A review of fifteen similar case reports showed that a spectrum of different collagen vascular diseases was diagnosed and treated before the true diagnosis emerged. In half of these cases the cardiac tumour was only diagnosed at necropsy. The diagnosis of collagen vascular disease should not be made in the absence of corroborative laboratory data. In cases of malignant cardiac tumour, and less commonly with atrial myxoma, M mode and cross sectional echocardiography may not exclude the diagnosis. There may be a good response to steroid treatment in cases of suspected but not confirmed collagen vascular disease in which the true diagnosis is cardiac tumour.     

Cardiac tumours simulating collagen vascular disease

Cardiac tumours can mimic collagen vascular disease and they are often accompanied by profound systemic upset. Both benign and malignant tumours may present in this way. Three cases of cardiac tumour, two malignant and one benign, are reported with just such a presentation. A review of fifteen similar case reports showed that a spectrum of different collagen vascular diseases was diagnosed and treated before the true diagnosis emerged. In half of these cases the cardiac tumour was only diagnosed at necropsy. The diagnosis of collagen vascular disease should not be made in the absence of corroborative laboratory data. In cases of malignant cardiac tumour, and less commonly with atrial myxoma, M mode and cross sectional echocardiography may not exclude the diagnosis. There may be a good response to steroid treatment in cases of suspected but not confirmed collagen vascular disease in which the true diagnosis is cardiac tumour.     

Parathyroid hormone-related peptide in pancreatic neuroendocrine tumours associated with hypercalcaemia

BackgroundHypercalcaemia is a common paraneoplastic syndrome. In the context of pancreatic neuroendocrine tumours, it is occasionally caused by secretion of parathyroid hormone-related peptide (PTH-rP).Case outlinesTwo patients are reported in whom persistent hypercalcaemia was traced to a large neuroendocrine pancreatic tumour hypersecreting PTH-rP. Resection of the tumour reduced serum levels of calcium and PTH-rP transiently in each case until the patient developed bulky metastatic disease. A 33-year-old woman remained hypercalcaemic after the removal of all four hyperplastic parathyroid glands had rendered circulating parathormone levels undetectable. Radical distal pancreatectomy was followed over the next 4 years by operative debulking of liver metastases, multiple hepatic artery embolisations.octreotide injections and repeated admissions for intravenous fluid and biphosphonate therapy. A 41-year-old man presented with hypercalcaemia as well as features of somatostatinoma syndrome. Symptomatic improvement after radical distal pancreatectomy was short-lived, and hepatic artery embolisation failed to control his rapidly progressive disease.DiscussionMalignant hypercalcaemia associated with a neuroendocrine pancreatic tumour hypersecreting PTH-rP is difficult to treat and can be life-threatening. Aggressive surgical treatment is recommended initially, while somatostatin analogues and hepatic artery embolisation are alternative therapeutic options for metastatic disease.     

Multiple malignant tumours in the elderly

Aging of the population and technical improvements may contribute to increase the possibility of multiple malignant tumours to be diagnosed. Over a period of 18 years, 2,749 consecutive autopsies of elderly patients were performed. In addition to macroscopic examination of every single organ, bone marrow and prostate were routinely stained and microscopically examined. One hundred and sixty-seven patients with multiple malignant tumours were found. Mean age was 81.1 years for females and 80.1 years for males. Mean interval between tumours was 2.4 years for females and 1.0 year for males. First tumours were most of the time diagnosed clinically (n = 121 vs. 46) whereas second tumours were diagnosed at autopsy (n = 121 vs. 46). Origin and metastastic sites of tumours were not different between the first and second tumour. Prostate and breast were the most frequent organs involved in males and females. Multiple malignant tumours in the elderly are not rare and the second tumour is frequently diagnosed after death despite occurring relatively soon after the diagnosis of the first tumour. Multiple malignant tumours may represent a significant clinical challenge leading to further diagnostic procedures and differentiated therapeutic approaches.     

Right-left shunt caused by sarcoma of the right atrium

A 70 year old woman was admitted for right ventricular failure and cyanosis of recent onset. Echocardiography showed a very large, homogenous, immobile, smooth-contoured mass filling the right atrium. Right atrial pressures were raised but the other intracardiac pressures were normal at catheterisation. Right heart angiography confirmed the voluminous right atrial mass and dilatation of the hepatic veins and showed early opacification of the left heart chambers. A right-to-left shunt was confirmed by oximetry which showed significant desaturation of the blood in the left atrium and ventricle (saturation 78% in the left ventricle). The tumour was also documented by a thoracic CT scan. At surgery, a very large, malignant right atrial tumour was resected which histological examination showed to be an angiosarcoma. The interatrial septum seemed to be intact: there was no true atrial septal defect but a persistent foramen ovale was found. After resection of the tumour the right atrium was reconstructed. The initial postoperative period was uncomplicated but the patient died nine months later of metastatic disease particularly affecting the liver and brain. The clinical presentation of malignant cardiac tumour is very variable but a right-to-left interatrial shunt through a patent foramen ovale has only been reported previously in 2 cases of primary malignant tumours (a rhabdomyosarcoma and an angiosarcoma) and in 1 case of a secondary cardiac metastasis.     

Primary tumors of the heart. Diagnostic, anatomic and therapeutic aspects

Primary cardiac tumours are rare. They are usually benign, the most common ones being left atrial myxomas. The authors report their experience of 16 cardiac tumours operated between 1978 and 1985. The patients were 12 adults and 4 children. The tumours were benign in 14 cases and malignant in 2 cases. Complete (14 cases) or incomplete ablation of the tumour was carried out under cardiopulmonary bypass with an early mortality of 6.25 per cent (1 case) and late mortality of 12.5 per cent (the two malignant tumours). The tumours were identified as myxomas in 11 cases, rhabdomyomas in 2 cases, fibroma in 1 case and malignant sarcomas in 2 cases. The average follow-up period of the 13 survivors is 30 months (range 4 months to 5 years) with excellent clinical and anatomical results. Clinical and paraclinical diagnosis of these tumours does not pose any major problems nowadays because of the reliability of the methods of investigation. However, the nature of the tumour is not always predictable and the operative findings are fundamental, not so much for the treatment which is relatively stereotyped but for the prognosis. Age is not a significant prognostic factor as excellent results may be obtained in the very young and the very old despite a sometimes precarious preoperative clinical condition. The benign or malignant nature of the tumour is the only real factor which affects the prognosis.     

Unilateral testicular tumour associated to congenital adrenal hyperplasia: Failure of specific tumoral molecular markers to discriminate between adrenal rest and leydigioma

Testicular adrenal rest tumours are frequently associated with congenital adrenal hyperplasia (CAH). These ACTH-dependent tumours cannot be easily distinguished histologically from Leydig-cell tumours. We report the case of a 30-year-old man who was explored for infertility, azoospermia and unilateral testicular tumour. High levels of 17-OH progesterone and ACTH, low cortisol and undetectable gonadotropins levels, associated to bilateral adrenal hyperplasia, led to the diagnosis of CAH by 21-OH deficiency with a composite heterozygoty. The testicular tumour was first considered as adrenal rest. However, histological analysis of this unilateral painful tumour showed a steroid-hormone-secreting cell proliferation with atypical and frequent mitosis. To discriminate between a benign adrenal rest tumour and a possible malignant leydigioma, tumoral expression of specific gene products was analyzed by RT-PCR. No 11-beta-hydroxylase nor ACTH receptor mRNAs could be found in the tumour, which did not behave like usual adrenal rest cells. For this unilateral testicular tumour, the lack of adrenal-specific markers associated with a high rate of mitosis and pleiomorphism supported a leydigian origin with malignant potential. However, lack of tumoral LH-R mRNA expression and a tumour-free 3-year follow-up led us to retain the diagnosis of adrenal rest tumour with loss of adrenal gene expression and progressive autonomous behaviour.