Squamous cell carcinoma developing in necrobiosis lipoidica.

We describe a case of squamous cell carcinoma arising in long-standing necrobiosis lipoidica in a type 1 female diabetic patient. The tumour and the skin lesion were successfully excised and repaired with full thickness skin graft. The development of squamous cell carcinoma in association with this skin disorder is rare (only four cases reported in literature since 1966), but should be considered in chronic, non-healing and recalcitrant ulcers developing within areas of necrobiosis lipoidica.     

Cardiac tamponade because of left atrium direct invasion by a large cell neuroendocrine metastatic carcinoma of the lung

A 53-year-old man presented with cardiac tamponade due to directinvasion of left atrium by a large cell neuroendocrine metastaticlung adenocarcinoma. Direct invasion of left atrial cavity bythis type of lung tumour is rare. When cardiac involvement issuspected, two-dimensional echocardiography is the first-linediagnostic tool. Computerized tomography and magnetic resonanceimaging can provide additional anatomical information and, asin this case, further delineation of the relation between intracavitymasses and normal structures, including the mural site of attachmentand tumour extension.     

Complete response of a primary cutaneous T-cell-rich B cell lymphoma treated with interferon α2a

Primary B cell lymphomas of the skin are rare disorders and therapy is not yet standardized. A 45-year-old male patient presented 4 years after surgical removal of a subcutaneous tumour of the leg. On examination a reddish nodular tumour was found in the same region. The biopsy revealed a T-cell-rich primary B cell lymphoma of the skin. Interferon α2a was given perilesionally at a dosage of 9 MU three times a week. The treatment was well tolerated, had no severe side-effects and induced a complete remission, both clinically and histologically, during a 12-months course. Received: 2 December 1997 / Accepted: 9 December 1997     

Multicentric reticulohistiocytosis with arthritis and cardiac infiltration: regression following treatment for underlying malignancy.

The case is reported of a 63 year old man presenting with a rapidly destructive symmetrical polyarthritis and widespread papular nodular skin lesions, confirmed by a biopsy to be due to multicentric reticulohistiocytosis. Biventricular cardiac failure developed secondary to extensive myocardial infiltration with multicentric reticulohistiocytosis, a complication of this disease which has not previously been reported. The joint, skin, and cardiac manifestations of multicentric reticulohistiocytosis substantially regressed following resection of an associated squamous cell carcinoma. This report adds to the small amount of published work which suggests that multicentric reticulohistiocytosis can be a paraneoplastic disease that may respond to treatment directed at the underlying tumour.     

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.     

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.     

Cardiac presentation of ALK positive anaplastic large cell lymphoma

Cardiac involvement as an initial presentation of malignant lymphoma is a rare occurrence. We report the case of an immunocompetent 29-year-old male who presented with syncope and arrythmias secondary to a ventricular cardiac mass. Transcutaneous cardiac biopsy was non-diagnostic, therefore an open cardiac biopsy was performed from which a provisional diagnosis of a cardiac inflammatory pseudotumour was made. Six months after presentation, he developed several subcutaneous lesions with systemic symptoms. Histological and immunophenotypic review of the initial cardiac biopsy revealed features consistent with a diagnosis of CD30, ALK1 positive anaplastic large cell lymphoma (ALCL). Despite intensive treatment with combination chemotherapy, there was significant progression of disease, and he died 11 months after diagnosis. The overall prognosis of cardiac lymphoma remains poor, which may be due to the often late presentation of the tumour. To our knowledge, this is the first reported case of a cardiac ALK positive ALCL. Although rare, cardiac presentation of ALCL should be added to the list of differential diagnoses of cardiac lymphomas.     

Papillary fibroelastoma of aortic valve: diagnosis and surgical management

Papillary fibroelastoma (PFE) is a rare and benign cardiac tumour that mainly affects the valves. This tumour has the potential to cause serious life threatening thromboembolic complications. Herein, we describe successful excision of an aortic valve papillary fibroelastoma. The importance of valve sparing tumour resection is emphasised.     

Pericardial hemolymphangioma. Apropos of a case

A case of pericardial hemolymphangioma revealed by palpitations is reported. X-ray films of the chest provided a diagnosis of mediastinal tumour. Full evaluation was performed using echocardiography, computerized tomography of the mediastinum, coronary arteriography and cardiac ventriculography. The tumour could be removed in toto. Pericardial hemolymphangioma is a rare benign vascular tumour. It is often discovered accidentally during examinations for non-suggestive cardiac functional disorders. The threat of compression or invasion of adjacent structures and that of hemopericardium by bleeding make surgical excision mandatory.     

Left atrial leiomyosarcoma: A rare cause of mitral valve (pseudo)stenosis

A 54-year-old lady presented with a mass in the left atrium causing an acute mitral valve pseudo-stenosis. The tumour mass was assumed to be a myxoma. The patient was scheduled for tumour mass extirpation to clear the obstruction of the mitral orifice. Histological finding from the acquired specimen showed a leiomyosarcoma, as a rare primary malignant cardiac tumour.     

A rare case of aortic valve myxoma: an unusual cause of embolic stroke

Aortic valve myxoma is an extremely rare cardiac tumour. We describe a 60-year-old woman found to have an aortic valve myxoma and a patent foramen ovale (PFO) presenting as a stroke. In view of the recurrent cerebral ischaemic events, the aortic valve myxoma was surgically removed and the PFO was closed. To the best of our knowledge, this is the first reported case of aortic valve myxoma with a PFO. We also highlight the importance of identifying a possible cardiac source of embolism in a stroke patient and considering this rare tumour as a cause in patients with a cryptogenic stroke.     

Involvement of platelet‐derived growth factor ligands and receptors in tumorigenesis

Platelet‐derived growth factor (PDGF) isoforms and their receptors have important roles during embryogenesis, particularly in the development of various mesenchymal cell types in different organs. In the adult, PDGF stimulates wound healing and regulates tissue homeostasis. However, overactivity of PDGF signalling is associated with malignancies and other diseases characterized by excessive cell proliferation, such as fibrotic conditions and atherosclerosis. In certain tumours, genetic or epigenetic alterations of the genes for PDGF ligands and receptors drive tumour cell proliferation and survival. Examples include the rare skin tumour dermatofibrosarcoma protuberance, which is driven by autocrine PDGF stimulation due to translocation of a PDGF gene, and certain gastrointestinal stromal tumours and leukaemias, which are driven by constitute activation of PDGF receptors due to point mutations and formation of fusion proteins of the receptors, respectively. Moreover, PDGF stimulates cells in tumour stroma and promotes angiogenesis as well as the development of cancer‐associated fibroblasts, both of which promote tumour progression. Inhibitors of PDGF signalling may thus be of clinical usefulness in the treatment of certain tumours.     

Primary cardiac paraganglioma with dual coronary blood supply presenting with angina chest pain

Primary cardiac paraganglioma (pheochromocytoma) is very rare, constituting only 1% of cardiac tumours. A case of a 44-year-old woman presenting with angina chest pain and a tumour with dual blood supply from both the right and left coronary arteries is reported.     

Primary cardiac lymphoma causing right atrial occlusion

Primary cardiac lymphomas (PCL) are extremely rare, and diffuse large B-cell lymphoma is a highly aggressive subtype. We report a case that was initially diagnosed as chronic right heart dysfunction. Detailed investigations revealed a large lobulated tumour occluding the right atrium, infiltrating the inter-atrial septum, the roof of the left atrium, and the aortic root. Despite adequate surgical debulking and initial successful tricuspid annuloplasty, the patient succumbed to multi-organ failure. Pathological analysis of the resected tumour confirmed a diffuse large B-cell lymphoma with a proliferation rate of 100%. What is unique about this case is the size of this rare cardiac tumour, which we believe to be one of the largest described in the literature for a purely intra-cardiac PCL, its aggressive growth rate, and the relatively mild symptomatology until a late stage of the disease.     

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.     

Fieber,Vorhofflimmern und Angina pectoris bei einem 58-jährigen Patienten

Primary cardiac lymphoma (PCL) respresents a very rare type of cardiac tumour. This report illustrates a case of PCL in an immunocompetent 58-year-old man presenting with atrial fibrillation and febrile syndrome. Comprehensive imaging [computer tomography (CT), cardiac magnetic resonance imaging (cMRI), 3-dimensional transesophageal echocardiography (3D-TEE)] identified a large right atrial tumour, leading to pericardial effusion. Isolated cardiac involvement was confirmed by positron emission tomography (PET)-CT. A diffuse large B-cell lymphoma (DLBCL) was diagnosed based on the results of a TEE-guided biopsy. A normalized PET scan (PETAL study) indicated complete remission following R-CHOP 14 immunochemotherapy. Thus, an interdisciplinary and multimodal approach avoided unnecessary cardiac surgery.     

Is Dacron carcinogenic? Apropos of a case and review of the literature

Primary malignant cardiac tumours are extremely rare. The authors report a case of primary cardiac lymphoma nine years after implantation of a double leaflet mitral valve prosthesis. Malignant lymphoma is a haematological form of sarcoma. Exceptionally rare, it is a tumour of the immune system occurring principally in immuno-depressed patients. It typically presents as a nodular or diffuse myocardial infiltrate explaining its clinical expression as cardiac failure and atrioventricular block. In view of the usual degree of infiltration, surgery is rarely possible. Survival after "pure" medical therapy (chemotherapy alone or associated with radiotherapy) is 6 to 8 months after diagnosis. Dacron has been implicated in the pathogenesis of primary cardiac sarcoma. Oppenheimer demonstrated experimental induction of sarcoma in the rat by subcutaneous implantation of polymers. In conclusion, although primary cardiac lymphoma is a rare condition, it should be considered, as with thrombosis, a possible differential diagnosis of acute dysfunction of cardiac valvular prostheses.     

A left atrial paraganglioma patient presenting with compressive dysphagia

Paragangliomas are rare neoplasms of neural crest origin arising in the chromaffin (pheochromocytoma) and chemoreceptor (chemodectoma) tissues. Only a few cases of paragangliomas have been reported in the heart. Most of the cardiac paragangliomas are located in, or adjacent to, the left atrium. The biological activity indicative of catecholamine production has rarely been shown in cardiac paragangliomas. Patients with cardiac paragangliomas may present with hypertension, or with various obstructive or compressive symptoms, depending on the location of the tumour. A left atrial paraganglioma compressing the esophagus causing dysphagia is presented. A 37-year-old female patient underwent work-up for dysphagia to solids. Transesophageal echocardiography showed the presence of a large mass in the dilated left atrium. The tumour was removed completely intact and was a benign noncatecholamine-secreting paraganglioma. The patient's dysphagia was relieved. The clinical perspectives of cardiac paraganglioma and cardiac dysphagia are discussed.     

A rare cause of Sister Mary Joseph's nodule

Sister Mary Joseph's nodule is an important physical sign. It usually represents umbilical metastasis of intra abdominal malignancy. Its association with intra abdominal desmoplastic small round cell tumour is extremely rare. We describe a rare case of sister Mary Joseph's nodule associated with desmoplastic small round cell tumour. We conclude that differential diagnosis of Sister Mary Joseph's nodule should include desmoplastic small round cell tumour, especially in adolescents and young adults.