Minimal-change lesion nephrotic syndrome with renal oncocytoma.

A renal mass was found in a 49-year-old man presenting with idiopathic nephrotic syndrome. The excised tumor was a well-encapsulated renal oncocytoma. Examination of the kidney revealed a minimal-change glomerular lesion and moderate arterionephrosclerosis. Nephrotic range proteinuria persisted through a postoperative course of prednisone therapy, but diminished and cleared within the subsequent two-year period. Renal function has remained stable and proteinuria has not recurred over a four-year follow-up. The clinical course suggests a previously unreported relationship between renal oncocytoma and minimal-change lesion nephrotic syndrome.

Renal lesions in acute rheumatic fever.

In the past 4 years, four patients with acute rheumatic fever and abnormalities of renal function, urinary sediment, or both have undergone percutaneous renal biopsy at this institution. Distinctly different renal lesions were found and included focal glomerulonephritis, classic acute exudative poststreptococcal glomerulonephritis, mesangioproliferative glomerulonephritis, and severe interstitial nephritis. In each case, the clinical abnormalities were transient. In this report we describe the clinical and pathologic findings in each of these patients to stress the variability of renal lesions that may accompany acute rheumatic fever.

Seminoma metastatic to ureter.

A case of metastatic seminoma to the ureter is presented. The metastasis formed a polypoid intraluminal mass that led to complete obstruction of the ureter, leading to advanced hydronephrosis of the kidney. This is the second case in the world literature of a primary testicular tumor metastasizing to the ureter.

Oncocytoma of kidney.

Two cases of oncocytoma of the kidney are presented. The clinical and pathologic characteristics of this rare tumor are described and compared with those of renal cell carcinoma.

Alport's syndrome associated with macrothrombopathic thrombocytopenia.

The combined occurrence of hereditary nephritis with nerve deafness (Alport's syndrome) and macrothrombocytopathic thrombocytopenia is very rare. The authors have had the opportunity to study such a case in a 20-year-old man who had been followed since birth. The clinical history, renal biopsy, platelet studies, and autopsy findings are presented. The renal pathologic findings are well defined; however, the hemostatic abnormalities and the hearing loss are not well characterized. In this paper, an attept is made to clarify the diverse platelet functional and morphologic abnormalties.

Treatment of Goodpasture's syndrome with plasmapheresis. A case report and review of the literature.

A case is reported of antiglomerular basement membrane antibody-induced Goodpasture's syndrome in which the patient required hemodialysis and was treated with immunosuppressive agents and plasmapheresis. A severe (80 per cent) cresentic lesion was reversed, and creatinine was stabilized at 2.5 mg/dl at one year follow-up. Earlier reports of therapy without plasmapheresis showed that 88 per cent of the patients would either die or require long-term hemodialysis. Fifteen other reported cases of Goodpasture's syndrome in which the patients were treated with plasmapheresis are reviewed. When reported, short-term follow-up showed that nine of these patients were alive without need of dialysis, five wee receiving dialysis, and only two had died. This suggests that plasmaheresis and immunosuppressive therapy may reverse the renal lesion in some patients with Goodpasture's syndrome.

Renal failure with posttransplant renin-angiotensin mediated hypertension.

Ten days after receiving a kidney transplant, severe hypertension and renal failure developed in a patient with a previously functioning graft. Although moderate blood pressure control was achieved with dialysis and antihypertensive medications, the graft did not function well. A trial of sar-ala-angiotensin II (an angiotensin II antagonist) was associated with a dramatic fall in blood pressure. Subsequently, the patient's own two kidneys were removed, the blood pressure fell to normal on the operating table, and within days graft function improved. The possible explanation for the changes in graft function, including angiotensin II-mediated changes of glomerular filtration rate, are discussed.

Peritoneal mesothelioma in a male pseudohermaphrodite with asymmetrical gonadal differentiation.

A diffuse peritoneal mesothelioma occurring in a patient with male pseudohermaphroditism and asymmetrical gonadal differentiation (mixed gonadal dysgenesis) is described. Malignancies of mesodermal origin, usually derivatives of the urogenital ridge, appear to occur with increased frequency in male pseudohermaphrodites. This appears to be the first reported instance of a mesothelioma in a male pseudohermaphrodite with mixed gonadal dysgenesis. The clinical and pathologic features of this tumor, including the electron microscopic findings, are presented.

Basosquamous (transitional cloacogenic) carcinoma of the sigmoid colon.

A case of basosquamous or so-called transitional cloacogenic carcinoma of the sigmoid colon, which arose above the pelvic brim at the peritoneal reflection, is reported. We were not able to find a report of this histologic type of tumor arising this far from the pectinate line of the anus, which is the most common primary site of this neoplasm. Possibilities as to the histogenesis of this tumor at this site are stated. The neoplasm also produced parathyroid hormone (PTH) and also possibly adrenal corticotrophic hormone (ACTH), which had not been previously reported for this specific neoplasm.

Type IV glycogen-storage disease. Light-microscopic, electron-microscopic, and enzymatic study.

The case of a 14-month-old Latin American girl with the diagnosis of Type IV glycogen-storage disease is reported. The diagnosis was reached on the basis of the typical clinical manifestations, the light- and electron-microscopic findings, and the demonstration of absence of the branching enzyme alpha-1,4-glucan:alpha-1,4-glucan 6-glucosyl transferase in the liver and in the cultured skin fibroblasts.

Histiocytic medullary reticulosis with parallel increases in serum acid phosphatase and disease activity.

Two months after presenting with autoimmune hemolytic anemia, a 57-year-old man developed increased serum acid phosphatase activity. Subsequently, abnormal histiocytes were found in the bone marrow and blood, and 8 months after the onset of his illness lymph node biopsy led to the diagnosis of histiocytic medullary reticulosis. At that time the level of serum acid phosphatase activity was more than 60 times the upper limit of normal and then paralleled the activity of his disease during temporary responses to chemotherapy, rising to over 90 times normal at the time of his death 1 year after presentation. No prostatic malignancy was demonstrated by biopsies or at autopsy, and electrophoretic studies of the serum enzyme suggested that it was of extraprostatic origin. It is postulated that the acid phosphate present in the serum at levels heretofore described only in metastatic carcinoma of the prostate may have arisen from the abnormal histiocytes.

Extraskeletal myxoid chondrosarcoma: a clinicopathological study.

Extraskeletal myxoid chondrosarcoma is a rare tumor of cartilagenous origin, with only 41 previously reported cases. It has a distinctive histologic pattern and should be distinguished from chondrosarcoma of bone because of its different behavioral pattern. The light microscopic, histo-chemical and electron microscopic features of such a tumor are reported.

Sweat gland carcinoma: a clinico-pathological reappraisal.

A case of sweat gland carcinoma arising from the right fifth finger which later metastasized to the regional lymph nodes and the lungs is the subject of this report which attempts to establish further the true characteristics of this definite pathological entity. The diagnosis of sweat gland carcinoma has been loosely applied to a variety of lesions, and it was not until recently that more exact and appropriate criteria were used to establish the diagnosis of sweat gland carcinoma. The case reported in this manuscript possesses most if not all the criteria necessary to make a diagnosis of sweat gland carcinoma. Further review of the literature shows that this is a rare and aggressive neoplasm as this case illustrates. Radical surgery of the primary lesions with radical regional lymph node dissection whenever possible is recommended for the initial treatment, and combination chemotherapy and radiotherapy may be employed in the palliative treatment of the more advanced forms of the disease.