Paraganglioma of urinary bladder.

Paraganglioma of the urinary bladder is extremely rare. In this report of a young man, hypertensive crisis and ventricular arrhythmia was provoked during cystoscopic evaluation of a bladder mass. A diagnosis of pheochromocytoma was considered following detection of high serum and urinary catecholamine levels. A preoperative meta-iodobenzylguanidine scan was, however, negative. The bladder mass was surgically removed following initiation of antihypertensive therapy. Pathological confirmation of extraadrenal pheochromocytoma was established. During a serial follow-up, serum and urine catecholamine levels were persistently elevated. This was explained by abnormalities on fluorodeoxyglucose positron emission tomography scan, which were considered to represent a metastatic malignant neuroendocrine tumour. The patient is on palliative chemotherapy for malignant paraganglioma. This case highlights variable presentation of pheochromocytoma, importance of having a high index of clinical suspicion for early recognition and prompt management and serious adverse consequence of a delayed diagnosis.

Rare manifestation of Behcet's syndrome: insight from multimodality cardiovascular imaging.

A young Jordanian man was hospitalised with fever of 2 months duration. Preadmission diagnostic assessment including CT thorax and abdomen were inconclusive. Right-sided pleuritic chest pain was present on admission. Pulmonary embolism was suspected on a ventilation/perfusion lung scan. Lower-extremity deep venous thrombosis was, however, absent. Echocardiogram to exclude a cardiac source of pulmonary embolism showed a right ventricle mass which was also present on retrospective review of prehospitalisation CT thorax. Tissue characterisation of this mass on cardiac MRI was not helpful. Empiric anticoagulation was started without reduction in size at 3 weeks. Due to ongoing diagnostic uncertainty, surgical resection was performed. Histopathology confirmed this mass to be a thrombus. With unabated fever, right ventricular thrombosis with pulmonary embolism and cutaneous and scrotal ulceration which evolved later in the hospital course, a diagnosis of Bechet's syndrome was considered and subsequently confirmed. Response to immunosuppressive therapy was prompt.

Immunoglobulin and T-cell receptor gene-gene rearrangement in pleural cavity-based T-cell rich B-cell lymphoma in an immunocompetent patient.

Body cavity-based lymphomas are fluid-based lymphomas that are not associated with a tumor mass or adenopathy which could explain the origin of the lymphomatous effusion. A distinct lymphoma that grows in the body cavity as a lymphomatous effusion in the absence of a tumor mass has been identified as a primary effusion lymphoma. This almost exclusively occurs in patients with acquired immunodeficiency syndrome (AIDS), who invariably have a history of Kaposi sarcoma. We report a rare case of a recurrent pleural effusion in an immunocompetent patient. There was no evidence of lymphadenopathy or an associated mass on computerized tomography of the chest, abdomen and pelvis. Serology for HIV, HHS-8, EBV and HTLV-1 were negative. Cytologic examination of the pleural fluid showed an elevated white cell count with 97% lymphocytes, mostly with T-cell markers. Bone marrow aspirate and biopsy were negative and bronchoscopy was unrevealing. Pleural biopsy was significant for >70% T-lymphocytes and some large atypical cells. Which had CD19, CD20 and weak bcl-2 positivity. Kappa and lambda light chains did not show distinct clonality. A preliminary diagnosis of T-cell rich B-cell lymphoma (TCRBCL) of the pleural cavity was made. The diagnosis was confirmed with DNA studies done on the pleural biopsy specimen using PCR and southern blot. Dual rearrangement of Ig heavy chain region and TCR-beta genes were identified. The patient responded to combination chemotherapy with cyclophosphamide, adriamycin, vincristine and prednisone. Our case is the first known case of pleural cavity-based TCRBCL and illustrates the role of gene rearrangement studies in such patients.