Primary cardiac lymphoma. Report of a case

Malignant non-Hodgkins lymphomas have a secondary cardiac localisation in 20% of cases. However, a cardiac primary site is rare (44 cases described up to now). A positive diagnosis is rarely made before death. There is great interest in echocardiography, a non-invasive method, to identify these tumours early. The prognosis remains nevertheless gloomy. We report the case of a child aged 8 years, admitted with a scenario of low output right cardiac insufficiency. Chest radiography identified cardiomegaly with a prominent right border, and the electrocardiograph showed right auricular hypertrophy. A tumour mass infiltrating the right atrium, the right ventricle and the lateral face of the left ventricle was discovered on trans-thoracic echocardiography. Investigation for tumour spread was negative. The patient died before operation in a state of extreme low output. The histology favoured a highly malignant non-Hodgkins lymphoma type B.     

Left maxillary sinus tumour‐induced hypophosphataemic osteomalacia and combined with thyroid papillary carcinoma: a case report and literature review

Tumour‐induced osteomalacia (TIO) is a rare disease characterised by hypophosphataemia and clinical symptoms of osteomalacia. Herein we report the case of a 29‐year‐old man who was admitted to hospital with progressive bone pain and was diagnosed with TIO caused by maxillary sinus tumours. In the preoperative evaluation, it was found that the patient had thyroid malignant tumours at the same time. Two operations were performed separately on the left maxillary sinus tumour and thyroid tumour after complete examination. After tumour resections, the symptoms of bone pain were relieved and the level of blood phosphorus was restored, long‐term replacement therapy was needed for thyroid. When a patient is diagnosed with TIO, it is necessary to screen for the presence of other malignant tumours and explore the treatment options in order to benefit patients preferably.     

Primary myxoid mediastinal liposarcoma

Mediastinal liposarcomas (LPS) are rare tumours. We report a case of primary myxoid LPS in a 22-year-old woman suffering from cough, dyspnoea on exercise and asthenia for 3 weeks. Thoracic MRI showed a large tumour on the right side. After neoadjuvant chemotherapy, a complete resection was performed, followed by adjuvant thoracic irradiation. Eighteen months after the diagnosis, no sign of recurrence was detected. Mediastinal LPS include a heterogeneous group of bulky tumours, the progression of which depends on the histological type. The prognosis is dominated by the operability of the tumour. Adjuvant therapies are not established.     

Fibroelastoma of the tricuspid valve presenting with syncope

Cardiac fibroelastomas are rare benign tumours. Initially observed as a chance finding at autopsy or during cardiac surgery, the diagnosis has become more common since the introduction of echocardiography. These tumours are usually asymptomatic. When the left heart valves are involved, embolic complications may be threatening. When the right heart valves are affected they are usually asymptomatic. The authors report the case of a fibroelastoma of the tricuspid valve responsible for several syncopal attacks. The tumour was excised with preservation of the native valve. There has been no recurrence of syncope since surgery. The authors believe this to be the second reported case of fibroelastoma of the tricuspid valve presenting with syncope.     

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.     

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.     

Secondary tumors of the right heart. Echocardiographic aspects. Apropos of 6 cases in the adult

Secondary cardiac tumours are rare but but are now more frequently diagnosed by echocardiography. We report 6 cases of intracardiac metastases affecting the right heart which were diagnosed by 2D echocardiography. In 3 cases, a very mobile, oval-shaped tumour was visualised within the right atrium prolapsing into the tricuspid orifice in diastole like a myxoma but associated in 2 cases with signs of invasion of the inferior vena cava. Two other non-mobile tumours were observed causing massive invasion of the right atrium and the last case was of an infiltrating tumour of the right ventricle resulting in pulmonary infundibular obstruction. In the light of our experience and a review of the literature, it is difficult to distinguish secondary tumours of the right atrium from myxomas especially when the tumours are mobile and when it is impossible to visualise a pedicle inserted on the interatrial septum or tumoral invasion of the inferior vena cava. At the ventricular level, the diagnostic signs differ according to whether there is tumoral invasion of the cavity or infiltration of the muscular wall. These cases illustrate the value of 2D echocardiography in the diagnosis of intracardiac metastases, sometimes even in the absence of clinical signs.     

Abscess of the mitral annulus mimicking left atrial tumour - case report

A case of a 70-year-old female with hypertension, atrial fibrillation and angina pectoris, admitted to the hospital due to echocardiographically detected left atrial tumour, is presented. Differential diagnosis included thrombus, myxoma, infectious or neoplastic tumour. The patients underwent surgery. Histopathological examination revealed the presence of an abscess in the left atrium. This report underlines the difficulties in the diagnosis of cardiac tumours.     

Application of radionics on primary and metastatic tumours of the liver

The aim of our study is to present our initial experience on the use of radiofrequency ablation (RFA) on hepatic tumours. Four patients with tumours of the liver were submitted to RFA. The first patient underwent an emergency left lobectomy due to rupture of a tumour of the left hepatic lobe. A synchronous tumour was detected on the right lobe. He has been submitted to four sessions of RFA. The second patient was submitted to transverse colon resection (adenocarcinoma) and a year later three metastatic tumours were detected. Two sessions of RFA have been applied in this case. The third patient, with adenocarcinoma of the sigmoid and one metastatic lesion on the right hepatic lobe, and the fourth patient with breast cancer and metastatic lesion of the liver, underwent one session of RFA each. The use of RFA on metastatic or primary tumours of the liver enriches the possibilities of therapeutic treatment.     

Granular cell tumour in a patient with Crohn's disease treated with infliximab: coincidence or causal relationship?

Granular cell tumour (or Abrikossoff's tumour) was first described by Abrikossoff in 1926. This is a rare benign neoplasm of unclear histogenesis that is generally believed to be of nerve sheath origin. Usually, it presents as a solitary lesion, located mainly in the subcutaneous tissue of the head, or the neck, or in the oral cavity, such as a tongue lesion, although it may develop anywhere in the body. Approximately 1-2% of granular cell tumours are malignant. Granular cell tumours are extremely rare in patients with inflammatory bowel disease. To the best of our knowledge, granular cell tumours have never been reported in association either with Crohn's disease or scheduled infliximab treatment. Herein, we report a case of a granular cell tumour that presented as a subcutaneous skin nodule of the right lumbar area without any associated local or systemic symptoms in a 41-year-old woman with Crohn's disease who was receiving scheduled treatment with infliximab (5 mg/kg every 8 weeks) for 7 years.     

Huge right atrial myxoma revealed by heart failure

We report a case of 34 years old female with a diagnosis of a big right Atrial myxoma revealed by echocardiography. The treatment consisted on a surgical excision of the tumour under extra corporal circulation. The patient made a good post-operative recovery. Authors showed the clinical signs of these tumours to be various, and echocardiography to be the definitive diagnosis procedure.     

Appendiceal tumour--retrospective clinicopathological analysis.

Appendiceal tumours are rare and often discovered unexpectedly in an acute situation in which decision-making is difficult. We report the spectrum of appendiuar tumours seen in our institution over a period of more than 10 years, and discuss the clinicopathological behaviour, investigations, surgical procedures and outcomes in these patients. We have also reviewed the literature with regard to appendiceal tumours. Appendicular tumours were identified from the database of 1646 appendictomies (18% in children) performed in single centre and case notes were reviewed. Clinical presentation, investigations, histopathology, surgical procedures and outcome were analysed. Twelve patients with appendiceal tumours were identified (0.72%): 8 carcinoid, 2 mucinous (mucocele) and 2 adenocarcinoma. All the patients with a carcinoid tumour presented with features suggestive of acute appendicitis and were diagnosed postoperatively following appendicectomy and formal histology. No further surgical intervention was required as these lesions were less than 1cm away from the base of the appendix. One of the patient with mucinous cystadenoma presented acutely and underwent an appendicectomy; in the other patient with chronic pain, apreoperative MRI suggested the diagnosis leading to a planned hemicolectomy as the lesion was close to the base of the appendix. While one of the patient with an adenocarcinoma localized to the appendix did well following a right hemicolectomy, the other patient with disseminated disease succumbed within a year. Carcinoid tumours are the commonest appendiceal tumours, which present often as acute appendicitis. While appendicectomy would be adequate in most of these patients, in patients with a cystadenoma close to the base of the appendix or in case of a carcinoma, a right hemicolectomy is the appropriate option. While the prognosis is good in patients with carcinoid tumour and cystadenoma, it remains dismal in patients with disseminated malignant disease.     

Abscess of the mitral annulus mimicking left atrial tumour -- case report

A case of a 50-year-old female with hypertension and diabetes, admitted to the hospital due to echocardiographically detected left atrial tumour, is presented. Differential diagnosis included thrombus, myxoma and infectious tumour. The patient underwent surgery. Histopathological examination revealed the presence of an abscess in the left atrium. This report underlines the difficulties in the diagnosis of cardiac tumours.     

Massive Left Atrial Sarcoma Presenting With Severe Congestive Heart Failure

Primary intimal sarcomas of the left atrium are extremely rare and highly aggressive tumours. To our knowledge, only 4 cases have been reported to date. We report on a 42-year-old man who presented with signs of congestive heart failure and was found to have an enormous left atrial bilobed tumour with obstruction of the mitral valve. The patient underwent a surgical excision of the sarcoma. The tumour recurred 8.5 months later and the patient required right pneumonectomy.     

Cardiac tumours: non-invasive detection and assessment by gated cardiac blood pool radionuclide imaging.

Four patients with cardiac tumours were investigated by gated cardiac blood pool radionuclide imaging and echocardiography. Contrast angiocardiography was performed in three of the cases. Two left atrial tumours were detected by all three techniques. In one of these cases echocardiography alone showed additional mitral valve stenosis, but isotope imaging indicated tumour size more accurately. A large septal mass was detected by all three methods. In this patient echocardiography showed evidence of left ventricular outflow obstruction, confirmed at cardiac catheterisation, but gated isotope imaging provided a more detailed assessment of the abnormal cardiac anatomy. In the fourth case gated isotope imaging detected a large right ventricular tumour which had not been identified by echocardiography. Gated cardiac blood pool isotope imaging is a complementary technique to echocardiography for the non-invasive detection and assessment of cardiac tumours.     

Primary cardiac tumours--operative treatment of 20 patients

This report describes a series of 20 patients operated on for a primary cardiac tumour. The majority of the tumours (16) were benign myxomas; 12 of them were located in the left atrium, two in the right atrium and two were biatrial. Two lipomas were found; one was epicardial and the other was located in the left atrium. The only intraventricular tumour was a malignant left ventricular myosarcoma. The propensity of intracardiac tumours to embolize was distinctive. Nine of the 16 myxomas presented with peripheral embolization, and in two patients surgery was complicated by fatal perioperative cerebral embolization of myxomatous tissue. Furthermore, in one patient embolization of a left atrial lipoma necessitated amputation of her left arm before cardiac surgery. Late postoperative recurrences were found in two patients with atrial myxomas. In one of them, reoperation showed that the tumour had grown at that site in the interatrial septum where the original pedicle had been excised. One patient developed severe mitral valve regurgitation and underwent replacement with a prosthetic valve at reoperation. Otherwise our late follow-up study showed that the results of surgery were usually excellent even though mild echocardiographic abnormalities were not uncommon. Our experience emphasizes the embolic potential of intracardiac myxomas and suggests, furthermore, that to avoid recurrences excisions with wide margins should be preferred. Echocardiography is an optimal method for the follow-up of these patients.     

Sclerosing haemangioma with lymph node metastasis

Sclerosing haemangioma (SH) of the lung is an intrapulmonary tumour composed of polygonal and cuboidal cells arranged in a heterogeneous pattern. SH is generally regarded as benign or very low-grade; only rarely have cases of lymph node metastasis been reported. We report a case of SH with metastasis to the regional lymph nodes in an 18-year-old male, whose left lung tumour was identified incidentally on routine CXR. The left upper lobe was removed and the regional lymph nodes dissected. The tumour consisted of proliferative cuboidal and polygonal cells arranged in a papillary pattern within the sclerotic stroma. Subcapsular metastasis was observed in a lymph node. SH with lymph node metastasis was diagnosed. The patient was disease-free at follow up after 2 years. Lobectomy with nodal dissection is suggested for the treatment of pulmonary SH, especially in the case of large tumours with enlargement of regional lymph nodes. Investigation of more cases and long-term follow up are necessary to assess the clinical significance of lymph node metastasis.     

Myxoid leiomyosarcoma of the left atrium: a rare malignancy of the heart and its comparison with atrial myxoma

Primary tumours of the heart are rare. The majority of these tumours are benign, with myxomas located in the left atrium being the most common form. Almost all malignant tumours are sarcomas and occur preferentially in the right side of the heart. An exception to this rule is leiomyosarcoma, a rare form of primary cardiac sarcoma that occurs predominantly in the left atrium, as does cardiac myxoma. The case of a 53-year-old woman who presented with symptoms of mitral valve stenosis and pulmonary hypertension is reported. Cardiac catheterization, angiography and echocardiography revealed a left atrial mass that was interpreted as atrial myxoma. At the time of operation, the myxoid appearance of the tumour mass further supported this assumption. The tumour, including a wide rim of atrial septum, was removed with cautery. Histopathological examination unexpectedly showed that the tumour was not an atrial myxoma but rather a myxoid variant of a primary leiomyosarcoma. Immunohistochemistry and electron microscopy confirmed the diagnosis. Local radiotherapy was considered but deemed contraindicated in view of the longstanding pulmonary hypertension. Two months after excision, a repeat echocardiogram indicated recurrence of tumour in the left atrium, and the patient died a few days later. The preferential left atrial location and the frequently myxoid appearance of primary leiomyosarcomas of the heart make it particularly difficult to differentiate them preoperatively from atrial myxomas. The authors recommend resection of all atrial myxoid tumours with a wide (at least 1 cm) margin, combined with intraoperative frozen section diagnosis, because complete surgical resection appears to correlate with prolonged survival in the few reported cases of atrial leiomyosarcomas. In cases of incomplete initial resection or local recurrence in the absence of metastatic disease, heart transplantation may be a valid option in appropriately selected patients.     

Tumours or mediastinal pseudo-tumours of Castleman? One observation (author's transl)

Through a routine examination a Castleman tumour of left hilary topography was discovered in a 30 years old man. No recurrence followed exeresis. With regard to this case, the authors underlined the infrequency of these tumours with no preferential mediastinal topography and its occurrence at any age. Diagnosis is often reached after surgery by histological analysis of the tumour which enabled the distinction in two types according to Flendrig: type I plasmocytic with inflammatory signs; type II hyalino-vascular without any inflammatory signs. Surgery usually advised is not without any drawbacks because of the vascularization of the tumour and its adherence to neighbouring organs. But Castleman tumours still set nosological problems and the debate remains opened on the question of a nodal origin or a true neo-formation.     

Bronchogenic glomangiomyoma with local intravenous infiltration.

Most glomus tumours occur in the dermis and subcutaneous tissues. Lung glomus tumours are quite rare. The current authors present the first reported case of a lung-derived glomangiomyoma, the rarest variant of glomus tumour. A 56-yr-old female was admitted with haemoptysis. Chest computed tomography showed an approximately 5-cm-diameter mass in the right lower lobe with mucoid impaction. After a right lower lobectomy, a diagnosis of glomangiomyoma was made. The tumour had grown endobronchially and its maximal diameter was 5.5 cm. Although cytologically benign, glomus tumour cells had visibly infiltrated neighbouring vessels. These results suggest that a bronchogenic glomangiomyoma has a low-grade malignancy potential and warrants close follow-up.