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Biomedical subjects

J N Eble

Publications and source records attributed to J N Eble.

At least 37 records · Page 2Linked to original sources
PubMed1999

Ureteral rhabdomyosarcoma.

Rhabdomyosarcoma is a malignant tumor well known to urologists. These tumors arise from the genitourinary system in 20% to 25% of cases, most commonly from the bladder, prostate, vagina, and paratesticular region. This is the first reported case of a rhabdomyosarcoma arising from the ureter. The radiographic findings and ureteroscopic appearance of this tumor suggested a benign fibroepithelial polyp; however, a ureteroscopic biopsy and subsequent nephroureterectomy revealed an embryonal rhabdomyosarcoma.

Adult↗
PubMed1999

Diagnosis and classification of renal cell carcinoma.

Although substantial agreement exists in most aspects of diagnosis and staging of renal cell carcinoma, further work is needed to resolve unsettled issues regarding precursor lesions, grading, and the prediction of outcome for individual patients.

Carcinoma, Renal Cell↗
PubMed1998

Cystic embryonal sarcoma of kidney: a case report.

BACKGROUND: Morphologic analysis of malignant renal tumors of childhood and adolescence has resulted in the identification of a variety of tumor types with characteristic histology and clinical behavior. The authors report a case of renal sarcoma in a 19-year-old male that differs in morphology from the various established categories of primitive renal tumors. METHODS: Sections taken from the nephrectomy specimen were stained by routine methods and by immunohistochemistry for stromal and epithelial markers, and for proliferation markers. In addition, ultrastructural studies were undertaken. RESULTS: The tumor, which the authors termed cystic embryonal sarcoma, was comprised of poorly differentiated malignant mesenchyme in a myxoid stroma. Numerous epithelial-lined cysts were present. The distribution of the cysts and proliferation kinetics of cyst-lining epithelial cells suggested that they were derived from entrapped renal tubules. The tumor showed early recurrence postoperatively and after aggressive chemotherapy. The pathologic features and clinical behavior of the tumor resemble those of 2 previously reported cases and an additional 25 cases from the files of the National Wilms' Tumor Study Pathology Center. CONCLUSIONS: The clinical and histologic features of cystic embryonal sarcoma differ from those of other renal tumors of childhood and adolescence, and the tumor appears to be a novel form of renal malignancy.

Adult↗
PubMed1998

Microsatellite instability is uncommon in young patients with renal cell carcinoma.

Hereditary non-polyposis colorectal cancer (HNPCC) is an autosomal, dominantly inherited syndrome which predisposes to the development of colorectal cancer and, to a lesser extent, other extracolonic neoplasms. Widespread microsatellite instability is a feature of nearly all tumors from patients with hereditary non-polyposis colorectal cancer. It was recently found that most patients under age 35 with colorectal cancer also have widespread microsatellite instability in their tumors and that many of these patients have the germline mutations of mismatch repair genes that characterize hereditary non-polyposis colorectal cancer. Renal cell carcinoma has occasionally been reported in patients with hereditary non-polyposis colorectal cancer, and some renal cell carcinomas have been shown to have microsatellite instability at multiple loci. For this reason, we sought to address the possibility that some young patients with renal cell carcinoma may have hereditary non-polyposis colorectal cancer by screening their tumors for microsatellite instability. Thirty-two patients under the age of 45 with renal cell carcinoma were examined for the presence of microsatellite instability in their tumor tissue. Microsatellite instability was not observed in any of the tumors. The absence of microsatellite instability in renal cell carcinomas from young patients suggests that these patients are unlikely to have hereditary non-polyposis colorectal cancer and would generally not benefit from genetic screening for this syndrome.

Adult↗
PubMed1998

Extensively cystic renal neoplasms: cystic nephroma, cystic partially differentiated nephroblastoma, multilocular cystic renal cell carcinoma, and cystic hamartoma of renal pelvis.

Predominantly cystic renal neoplasms have been the source of diagnostic confusion and controversy. In this review, the authors analyze the clinical and pathological features of four entities that consistently exhibit a diffusely cystic growth pattern, are strikingly similar in their gross appearances, and are not separable by preoperative imaging studies. Based on the literature, this review concludes that tumors in young children that have been classified as cystic nephroma and cystic partially differentiated nephroblastoma likely represent a single entity, and all should be considered highly cystic Wilms' tumors with little or no capacity for invasion or metastasis and diagnosed as cystic partially differentiated nephroblastoma. Conversely, cystic nephroma in adults has no discernible connection with Wilms' tumor or nephrogenic rests and should be considered a benign composite neoplasm of stroma and epithelium of unknown histogenesis, which may rarely become malignant with secondary development of a sarcoma. Multilocular cystic renal cell carcinoma appears to be unrelated to cystic nephroma and if the following criteria are met, it appears to be a neoplasm with an intrinsically cystic growth pattern, and no, or at most little, malignant potential: (1) an expansile mass is surrounded by a fibrous wall, (2) the interior of the tumor entirely is composed of cysts and septa with no expansile solid nodules, and (3) the septa contain aggregates of epithelial cells with clear cytoplasm. Cystic hamartoma of the renal pelvis is a rare, complex tumor composed of stroma with a prominent smooth muscle component and a variety of epithelial elements.

Adenocarcinoma, Clear Cell↗
PubMed1998

Angiomyolipoma of kidney.

Angiomyolipoma of the kidney is a clonal neoplasm, apparently part of a family of neoplasms derived from perivascular epithelioid cells. Early angiomyolipomas are small nodules composed of HMB-45-reactive spindle cells in the renal capsule, cortex, or medulla. Most angiomyolipomas are asymptomatic, and they are more common than previously appreciated, approaching 13 per 10,000 adults. They are much more prevalent in patients with tuberous sclerosis, where they often are accompanied by cysts and occasionally by renal cell carcinoma. That they are rarely diagnosed before puberty in patients without tuberous sclerosis, that large ones are more common in women than men, and that they occasionally grow rapidly during pregnancy suggest that hormones may play a role in stimulating the growth of angiomyolipoma. Pathologists should recognize that the presence of multiple angiomyolipomas is, at the least, presumptive evidence for the diagnosis of tuberous sclerosis and include this in their reports. Typical angiomyolipomas are benign but may have alarming properties: nuclear pleomorphism and mitotic activity, extension into the vena cava (21 cases), and spread to regional lymph nodes (39 cases), without malignant progression. The most common serious complication of renal angiomyolipoma is hemorrhage. Epithelioid angiomyolipoma is a recently recognized variant with malignant potential. Angiomyolipoma and lymphangiomyoma are closely related, and tumors with features of both have occurred. Angiomyolipomas have occurred in other organs in association with renal angiomyolipoma.

Adolescent↗
PubMed1998

Papillary and metanephric adenomas of the kidney.

Papillary renal adenomas are the commonest neoplasms of the renal tubular epithelium, occurring in up to 40% of adults. If these criteria are met, the diagnosis of adenoma can be made with confidence: (1) papillary, tubular, or tubulopapillary architecture; (2) diameter less than or equal to 5 mm; and (3) does not histologically resemble clear cell, chromophobe, or collecting duct renal cell carcinomas. They are composed of epithelial cells with variable amounts of cytoplasm with variable tinctorial qualities including clear. Metanephric adenoma and metanephric adenofibroma are closely related neoplasms that appear to be benign. Although they have a morphological resemblance to Wilms' tumor, there is some genetic evidence relating them to papillary adenoma and papillary renal cell carcinoma. Metanephric adenoma and metanephric adenofibroma occur at all ages, have a 2:1 predominance of female patients, and are associated with polycythemia. No instance of malignant behavior has been reported. The adenomatous component is composed of uniform small cells arranged in tubular or papillary architectural patterns. The fibromatous component is moderately cellular and composed of bland spindle cells.

Adenofibroma↗
PubMed1998

Collecting duct carcinoma of kidney.

Collecting duct carcinoma of the kidney is a rare neoplasm with only approximately 100 cases described in the literature to date. The tumor occurs in a wide age range and predominantly in males. Typical collecting duct carcinoma consists of a grossly infiltrative neoplasm centered on the renal medulla. The usual histological pattern is that of a tubular or tubulopapillary carcinoma with a desmoplastic stroma that often contains neutrophils. Less commonly, collecting duct carcinoma has a papillary architecture with desmoplasia and infiltrative growth. Collecting duct carcinoma spread aggressively and many patients have metastases at the time of presentation. Origin in the collecting duct is suggested by medullary location, dysplasia of the epithelium in collecting ducts outside the tumor, and reactivity with antibody to high molecular weight cytokeratin and Ulex europaeus agglutinin. No unifying cytogenetic or molecular genetic features have been discovered thus far. The concept of collecting duct carcinoma is expanding and should include the recently described renal medullary carcinoma that is associated with sickle cell trait.

Adolescent↗
PubMed1997

Efferent vagal innervation of the canine atria and sinus and atrioventricular nodes. The third fat pad.

BACKGROUND: The purpose of this study was to investigate the functional pathways of efferent vagal innervation to the atrial myocardium and sinus and atrioventricular (AV) nodes. METHODS AND RESULTS: Using vagally induced atrial effective refractory period shortening, slowing of spontaneous sinus rate, and prolongation of AV nodal conduction time as end points of vagal effects, we determined the actions of phenol and epicardial radiofrequency catheter ablation (RFCA) applied to different sites at or near the atrial myocardium to inhibit these responses. We found that efferent vagal fibers to the atria are located both subepicardially and intramurally or subendocardially. Most efferent vagal fibers to the atria appear to travel through a newly described fat pad located between the medial superior vena cava and aortic root (SVC-Ao fat pad), superior to the right pulmonary artery, and then project onto two previously noted fat pads at the inferior vena cava-left atrial junction (IVC-LA fat pad) and the right pulmonary vein-atrial junction (RPV fat pad) and to both atria. A few vagal fibers may bypass the SVC-Ao fat pad and go directly to the IVC-LA or RPV fat pad and then innervate the atrial myocardium. Vagal fibers to the sinus and AV nodes also converge at the SVC-Ao fat pad (a few fibers to the sinus node go directly to the RPV fat pad) before projecting to the RPV and IVC-LA fat pads. Long-term vagal denervation of the atria and sinus and AV nodes can be produced by RFCA of these fat pads and results in vagal denervation supersensitivity. Vagal denervation prevents induction of atrial fibrillation in this model. CONCLUSIONS: The newly described SVC-Ao fat pad receives most of the vagal fibers to the atria and sinus and AV nodes. Elimination of the fat pads with RFCA selectively vagally denervated the atria and sinus and AV nodes.

Adipose Tissue↗
PubMed1997

The Heidelberg classification of renal cell tumours.

This paper presents the conclusions of a workshop entitled 'Impact of Molecular Genetics on the Classification of Renal Cell Tumours', which was held in Heidelberg in October 1996. The focus on 'renal cell tumours' excludes any discussion of Wilms' tumour and its variants, or of tumours metastatic to the kidneys. The proposed classification subdivides renal cell tumours into benign and malignant parenchymal neoplasms and, where possible, limits each subcategory to the most commonly documented genetic abnormalities. Benign tumours are subclassified into metanephric adenoma and adenofibroma, papillary renal cell adenoma, and renal oncocytoma. Malignant tumours are subclassified into common or conventional renal cell carcinoma; papillary renal cell carcinoma; chromophobe renal cell carcinoma; collecting duct carcinoma, with medullary carcinoma of the kidney; and renal cell carcinoma, unclassified. This classification is based on current genetic knowledge, correlates with recognizable histological findings, and is applicable to routine diagnostic practice.

Humans↗
PubMed1997

Epithelioid angiomyolipoma of the kidney: a report of five cases with a prominent and diagnostically confusing epithelioid smooth muscle component.

We report five angiomyolipomas with a prominent component of epithelioid smooth muscle cells that occurred in patients from 20 to 48 (mean, 36) years of age. The tumors often posed problems in diagnosis, particularly with regard to distinction from renal cell carcinoma. Two patients had tuberous sclerosis. Two patients with more than 5 years' follow-up are alive and well. The epithelioid smooth muscle cells typically formed sheets that in two tumors were traversed by hyaline cords. The epithelioid cells ranged from medium sized and polygonal with slightly pleomorphic nuclei and eosinophilic cytoplasm to giant cells with prominent nucleoli. Hemorrhage, necrosis, and clusters of foamy macrophages were present in three tumors. Mitotic figures were easy to find in two of the tumors, but they were absent in the others. Obvious elements of typical angiomyolipoma were present in two tumors. The others contained only scattered, thick-walled blood vessels or a few fat cells suggestive of typical angiomyolipoma. None of the tumors was positive for low- or high-molecular-weight cytokeratins or epithelial membrane antigen. Actin was detected in the epithelioid areas in four tumors. Melanoma-associated antigens related to the gp100-cl gene product, HMB-45 and HMB-50, were present in all the tumors. Another melanoma-associated antigen, CD63 (NKI/C3), also was present in all the tumors, a finding suggesting that angiomyolipoma has features in common with melanoma beyond premelanosomal structures.

Adult↗
PubMed1997

The Muir-Torre syndrome in a black patient with AIDS: histopathology and molecular genetic studies.

In 1981, a black man had adenocarcinoma of the colon. In 1986, he had a sebaceous adenoma and the diagnosis of the Muir-Torre syndrome was established. The patient was found to be HIV sero-positive in 1986, and 8 years later fulfilled the CDC criteria for AIDS. During 1989 to 1993 the CD4 count was > 200 cells/ml and the patient had 2 sebaceous tumors, 1 basal cell carcinoma and 1 keratoacanthoma. In 1994 to 1996, the CD4 count was < 200 cells/ml and the patient developed 18 sebaceous tumors and a poorly differentiated adenocarcinoma of the finger which metastasized to axillary lymph nodes. Microsatellite analysis of tumor DNA from a sebaceous adenoma and adenocarcinoma of the finger revealed widespread microsatellite instability. The interaction of AIDS with the behavior of the tumors in the Muir-Torre syndrome has not previously been reported. Although our patient had an increase in the number of new sebaceous tumors at the same time he experienced deterioration of the immune system, he is doing well 15 years after resection of adenocarcinoma of the colon and 16 months after metastatic poorly differentiated adenocarcinoma of the skin. This follows the previously observed tendency for cancers of the Muir-Torre syndrome, especially those displaying widespread microsatellite instability, to be less lethal than their histologically similar counterparts in people without Muir-Torre syndrome.

Acquired Immunodeficiency Syndrome↗
PubMed1997

Carcinoma of the urinary bladder: a review of its diverse morphology.

The microscopic appearances of carcinomas of the urinary bladder are reviewed, with emphasis on patterns that have been highlighted only recently or account for major problems in differential diagnosis. Approximately 80% of bladder cancers are of urothelial (transitional cell) type. The major new information pertaining to that category is the appreciation of the extent to which they may have unusual patterns that, on occasion, lead to their misinterpretation as benign. The homology between some of these variants and benign epithelial abnormalities is emphasized and helps in understanding them: nested carcinomas and von Brunn's nests, small tubules and nephrogenic adenoma, carcinomatous glands and cystitis glandularis, cystic carcinomas and cystitis cystica. Other peculiar patterns seen in urothelial carcinoma include silt-like spaces, clefts, and angulated nests with spikes. Differences in opinion have been apparent in recent contributions concerning carcinomas of the bladder with a prominent component of spindle cells (sarcomatoid carcinomas) and whether they represent a category distinct from carcinosarcoma. Irrespective of this academic distinction, the potential for some epithelial-derived tumors of the bladder to be dominated by or even have an exclusive component of spindle cells is important. Three other major categories of primary bladder cancer are squamous cell carcinoma, adenocarcinoma, and undifferentiated carcinoma. The differences in the frequency of subtypes of adenocarcinoma originating in the bladder mucosa and urachus has recently been emphasized, as has the spectrum of undifferentiated carcinomas. In some bladder cancers, the stroma has unusual features. These are carcinomas with pseudosarcomatous stroma, osseous or cartilaginous metaplasia, or osteoclast-type giant cells. The diversity of appearances of bladder cancer has important implications in differential diagnosis.

Adenocarcinoma↗
PubMed1997

Papillary renal cell carcinoma: a clinicopathologic and immunohistochemical study of 105 tumors.

Papillary renal cell carcinoma is the second most common carcinoma of the renal tubules and has been characterized genetically. Its morphologic features are incompletely characterized. It has been suggested that the presence of cytokeratin 7 is specific for papillary renal cell carcinoma. Collecting duct carcinoma might have papillary architecture, and it has been suggested that reaction with the Ulex europaeus lectin is specific for it. Sections from 105 papillary renal cell carcinomas larger than 12 mm in diameter from 100 patients were studied, and immunohistochemical reactions were performed on 91. The tumors formed two morphologic groups. Type 1 (67 tumors) consisted of papillae and tubular structures covered by small cells with pale cytoplasm and characterized by small oval nuclei with inconspicuous nucleoli, frequent glomeruloid papillae, papillary edema, foamy macrophages in papillary cores, and psammoma bodies. Type 2 (38 tumors) consisted of papillae covered by large cells with abundant eosinophilic cytoplasm and characterized by pseudostratification and large spherical nuclei with prominent nucleoli, glomeruloid papillae, psammoma bodies, edematous papillae, and foamy macrophages in papillary cores are uncommon. Type 2 tumors were larger, more common in patients younger than age 40, and more frequently Stages 3 or 4 than were Type 1 tumors. Pseudocapsules were common in both and often were infiltrated by carcinoma. Sarcomatoid foci were found in five tumors. Eleven were stage T1, 54 T2, 23 T3, and 12 T4. Reaction for cytokeratin 7 was strong or moderate in 48 of 61 Type 1 tumors, and reaction was null in 24 of 30 Type 2 tumors. No tumor reacted with U. europaeus lectin.

Adolescent↗