Juxtaglomerular cell tumor is a rare renal neoplasm arising from the juxtaglomerular apparatus. Approximately 70 cases have been reported in the English literature since it was first described by Robertson et al in 1967. This tumor has been considered benign and resection has so far been curative. In this paper, we report the first metastatic juxtaglomerular cell tumor. The 15-cm tumor occurred in the right kidney of a 46-year-old man. It invaded the renal vein, and was treated by radical nephrectomy in 1995. The diagnosis at that time was renal cell carcinoma. The patient was well for 6 years and then developed bilateral lung masses, which were resected. Microscopically, the tumors from the kidney and the lungs were similar, consisting of solid sheets of uniformly round-to-polygonal cells intermixed with abundant delicate vasculature. Both renal and pulmonary tumors were positive for vimentin, renin, and only focally to CD34. Electron microscopic studies performed on the paraffin-embedded renal tumor and formalin-fixed lung tumor revealed the typical rhomboid crystals of proto-renin. In consideration of the characteristic morphologic features, immunohistochemistry, and the presence of rhomboid crystals of proto-renin, the diagnosis was modified to malignant juxtaglomerular cell tumor.
Clear cell sarcoma (soft-part melanoma) is a very rare entity with a distinctive histopathologic and molecular profile. Herein, we present the sixth reported case of a primary gastrointestinal clear cell sarcoma discovered in a 21-year-old woman. The patient underwent numerous tests prior to the diagnosis of her small bowel pathology, including the use of capsule endoscopy, which allowed for visualization and final localization of the tumour. Additionally, we discuss this rare type of sarcoma that affects young adults and has a poor prognosis characterized by the balanced chromosomal translocation t(12;22)(q13;q12) with special emphasis on the necessity for pathologists to be able to distinguish it from melanoma -- potentially a major pitfall in diagnosis.
Concentrated ethanol has been used to induce controlled myocardial infarct in patients with hypertrophic obstructive cardiomyopathy. We report the acute and early follow-up results of an alternative agent, n-butyl cyanoacrylate (n-BCA) glue, in a dog model. In 11 mongrel dogs, we injected n-BCA into different branches of the left anterior descending artery. Biplane left ventriculogram and coronary angiogram were performed before and after injection. In the surviving animals, we performed programmed stimulation (PS) to test for inducible ventricular tachycardia (VT) 48 days later. Following euthanasia, the removed hearts were studied with computer tomography (CT) and gross and histologic examination. Three dogs were lost before injection. Four dogs died within 2 hr to 4 days, and four animals survived 48 days. Accidental embolization of n-BCA into nontarget vessels was documented in four subjects. In the n-BCA-injected animals, homogeneous circumscribed scar was demonstrated by CT and histology. The glue was confined strictly to the tributary of the injected vessel, infiltrating arterioles of 14 mum. There was intense granulomatous reaction (GR) in the vessel wall and in the surrounding myocardium. Remote areas were unaffected. Monomorphic VT was not inducible with PS. We report a feasibility study of n-BCA injection to selected coronary arteries of dogs to cause controlled myocardial infarction. We demonstrated that the glue does not escape from the target artery through capillaries or small collateral vessels and thus produces a sharply demarcated and homogeneous scar, which is confined strictly to the supply zone of the injected vessel. Improvement of the delivery system is necessary to eliminate inadvertent embolization. Long-term follow-up is needed to study the GR induced by n-BCA.
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