Hepatic adenomatosis in glycogen storage disease type Ia: report of a case with unusual histology

Hepatic adenomatosis is a well-known complication of glycogen storage disease type Ia (von Gierke disease). Although most of these tumors have an appearance similar to sporadic hepatocellular adenomas, unusual histologic features have been reported, including Mallory hyaline, varying degrees of fibrosis, and aggregates of neutrophils. We report the fourth case of Mallory hyaline in the adenomas of glycogen storage disease type Ia in a 28-year-old woman undergoing segmental hepatectomy for enlarging liver nodules. Other prominent findings included steatohepatitis and nonspecific granulomatous inflammation--2 findings that are commonly seen in sporadic adenomas but not, to our knowledge, previously reported in glycogen storage disease type Ia.     

A Report Card on Provider Report Cards: Current Status of the Health Care Transparency Movement

BACKGROUND  

Public reporting of provider performance can assist consumers in their choice of providers and stimulate providers to improve quality. Reporting of quality measures is supported by advocates of health care reform across the political spectrum.     

Fatal disseminated adenoviral infection in a renal transplant patient

Immunosuppressed patients are more susceptible to adenoviral infection and carry a significantly higher mortality than immunocompetent patients. Renal transplant patients with adenoviral infection most often present with infection of the kidney and urinary tract within weeks to months of transplant surgery, suggesting reactivation of the latent adenovirus in the immunosuppressed host as the source of infection. We describe the first case of a fatal adenovirus infection after several years of immunosuppression in a kidney transplant patient. Postmortem examination of several tissues, using standard viral culture and polymerase chain reaction, was positive for adenovirus serotype 21. This case is unusual in that the fatal disseminated viral infection occurred after 6 years of immunosuppression, suggesting that the source of adenovirus was a novel infection rather than reactivation of latent infection, or infection from the transplanted tissue. Furthermore, this is the first report of adenovirus type 21 in an immunosuppressed patient.     

Aortic and mitral fenfluramine-phentermine valvulopathy in 64 patients treated with anorectic agents.

CONTEXT: Few published studies of the pathology of fenfluramine-phentermine (fen-phen) valvulopathy, described by Connolly and colleagues in 1997, have appeared. OBJECTIVES: To define temporal changes in the morphology of the stuck-on plaques and to determine whether the plaques progress or regress after cessation of fen-phen. METHODS: The available clinical information and pathology material from 35 aortic valves (AVs) and 43 mitral valves (MVs) from 64 patients were reviewed. RESULTS: The valves fell into 3 groups: 17 AVs and 28 MVs had fen-phen lesions only, 2 AVs and 7 MVs had fen-phen changes associated with other valve diseases, and 16 AVs and 8 MVs had no fen-phen changes. Fenfluramine-phentermine-attributable dysfunction was regurgitation in all instances. Typical plaques showed proliferation of myofibroblastic cells with myxoid stroma. Small vascular channels and slight lymphocytic accumulations were often present. Deeper parts of some plaques had dense fibroelastic tissue underlying typical plaque. CONCLUSIONS: Considerable individual variation in the time course of anorectic agent use and the severity of fen-phen valvulopathy was observed. Possible plaque regression could not be assessed from this study. The observations suggest that in some patients fen-phen-induced plaques may continue to have surface proliferation despite drug withdrawal.     

Urothelial carcinoma with rhabdoid features: report of 6 cases

Extrarenal rhabdoid tumors have been described in a variety of primary sites with only rare case reports of urothelial carcinomas with rhabdoid features in the literature. In this report, we describe the clinicopathologic characteristics, including clinical follow-up on 6 cases of urothelial carcinoma with prominent rhabdoid features. Four cases were retrieved from the consultation files of one of the authors and 2 were retrieved from the surgical pathology files at our institution. The patients were all men, with ages ranging from 53 to 86 years (mean, 66.5 years). Patients initially presented with hematuria or obstructive symptoms. The sites included bladder (n = 4) and renal pelvis (n = 2). All cases had a prominent rhabdoid component (mean, 60%), ranging from 40% to 80%. In addition to the rhabdoid component, multiple coexistent histological components were seen, including in situ urothelial carcinoma (carcinoma in situ) and high-grade papillary urothelial carcinoma (n = 2), poorly differentiated carcinoma with small-cell features (n = 1), sarcomatoid (n = 2), and a myxoid component (n = 2). All cases in this series had focal or diffuse positive staining with one or more cytokeratin markers (epithelial membrane antigen, CAM 5.2, AE1/AE3). Of the 6 patients, 4 were treated initially with surgery (radical cystoprostatectomy, n = 2; radical nephrectomy, n = 2). Of 6 patients, 2 died within 1 month, whereas a third patient died within 4 months. The remaining 3 patients were alive at 3, 3, and 9 months after diagnosis. The histological and immunohistochemical findings in this study serve to broaden the morphological spectrum of urothelial carcinomas with prominent rhabdoid features and add further evidence as to their poor prognosis.     

Clear cell hidradenoma: a mimic of metastatic clear cell tumors

Clear cell hidradenoma is a benign skin appendage tumor that may mimic conventional-type renal cell carcinoma. Histologically, clear cell hidradenoma contains small ductular lumens, focal apocrine and squamoid change, and a less prominent vascular pattern than renal cell carcinoma. Furthermore, immunohistochemical studies can aid in distinguishing the 2 tumors. Knowing the cytologic features of primary skin adnexal neoplasms helps distinguish them from cutaneous metastases, which are more commonly referred for fine-needle aspiration biopsy evaluation. Detailed clinical history, physical findings, and ancillary studies are essential for correct diagnosis and categorization of these tumors. We report the rare case of a patient with renal cell carcinoma who underwent excision of an axillary clear cell hidradenoma, which was clinically suggestive of cutaneous metastatic disease.     

Deformation eines koronaren Stents als Reanimationsfolge

Zusammenfassung Wir berichten über die Deformation eines koronaren Stents als Folge einer extrakorporalen Herzdruckmassage. Es handelt sich um den ersten publizierten Fall. Im Rahmen einer Akut-PTCA war je ein RCA- und ein LAD-Stent implantiert und deren Durchg?ngigkeit vor Verlassen des Katheterlabors angiographisch überprüft worden. Nach einer 30 Minuten sp?ter einsetzenden, erfolglosen Reanimation verstarb die Patientin im kardiogenen Schock. Die Obduktion zeigte einen stark deformierten LAD-Stent. Diese Beobachtung beweist die M?glichkeit der Deformation eines koronaren Stents durch Reanimationsma?nahmen. Eingegangen: 17. August 1998, Akzeptiert: 10. Dezember 1998     

Peripheral T-cell lymphomas, unspecified (or not otherwise specified): a review

Peripheral T-cell lymphomas (PTCL) comprises a heterogeneous group of haematological tumours, which originate from mature T-cells, and constitute less than 15% of all non-Hodgkin's lymphomas (NHLs) in adults. The current WHO classification recognizes nine distinct clinicopathologic peripheral T-cell NHLs, being the 'unspecified variant' (PTCL-U) the most common subtype. These neoplasms often present in advanced stage at diagnosis, and most commonly have an aggressive clinical course requiring prompt treatment. The rarity of these tumours requires additional studies to better understand their biology and search for new therapies which may hopefully improve the dismal outcome of most patients. This review aims to describe the pathobiological aspects as well the clinical characteristics and current therapeutic strategies of the PTCLs, with special attention to the group of PTCL-U.     

Deformation of a coronary stent as a sequela of resuscitation]

We report a case of LAD-stent deformation by extracorporeal cardiopulmonary resuscitation (CPR) shortly after stent implantation. To our knowledge, this is the first reported case. During an emergency PTCA, stents were implanted into the RCA and LAD. Patency was proven by angiography before the patient left the cardiac laboratory. Thirty minutes later the patient underwent CPR because of ventricular fibrillation. The patient died in cardiogenic shock. Postmortem examination showed a distinctly deformed LAD-stent. The case proves the possibility of a deformation of a coronary stent by resuscitation.