Pagetoid variant of actinic keratosis with or without squamous cell carcinoma of sun-exposed skin: a lesion simulating extramammary Paget's disease.

BACKGROUND: Extramammary Paget's disease usually occurs in anogenital skin. We present five cases of squamous cell carcinoma in situ of sun-exposed skin and non-squamous cell carcinoma in situ actinic keratosis that displayed atypical keratinocytes disposed in intraepithelial cell nests and immunohistochemical staining simulating extramammary Paget's disease. METHODS AND RESULTS: Two pilot cases--one squamous cell carcinoma in situ and one non-squamous cell carcinoma in situ actinic keratosis with formation of intra-epidermal nests of atypical keratinocytes with a pagetoid spread pattern--were encountered at our institution. Fifty-four consecutive cases of squamous cell carcinoma in situ including bowenoid actinic keratosis and 34 cases of non-squamous cell carcinoma in situ actinic keratosis were reviewed to identify pagetoid spread of atypical cells. Representative sections of all cases with pagetoid spread of atypical keratinocytes were submitted for special stains for mucin, and immunostaining for cytokeratin 7 (CK7), cytokeratin 20 (CK20), cytokeratin CAM 5.2 (CAM 5.2), carcinoembryonic antigen (CEA), vimentin and S100 protein. In the group of squamous cell carcinoma in situ, 10 cases displayed pagetoid spread of atypical keratinocytes with cytoplasm ranging from clear to pale and atypical hyperchromatic nuclei. One review squamous cell carcinoma in situ was multicentric with three separate lesions. The atypical keratinocytes tended to form well to poorly defined cell groups extending from the basal cell layer to the corneal layer. No similar cases were identified in the group of non-squamous cell carcinoma in situ actinic keratosis. Two pilot cases and three of 10 review cases with a total of seven separate lesions displayed a moderate to marked immunohistochemical reactivity for CK7 similar to extramammary Paget's disease. CEA immunoreactivity was also detected in two of these cases. In addition, two of 44 squamous cell carcinomas in situ without pagetoid spread of atypical keratinocytes showed a moderate reactivity for CK7 in very occasional atypical keratinocytes. The remaining seven squamous cell carcinomas in situ with pagetoid spread of atypical keratinocytes were not immunoreactive for CEA and CK7. Immunostaining for CK20, vimentin, S100 protein was negative in all atypical cells in all study cases. CONCLUSIONS: Actinic keratosis, particularly squamous cell carcinoma in situ of sun-exposed skin, may have histopathological and immunohistochemical features similar to extramammary Paget's disease and probably represents a variant of actinic keratosis. Awareness of the pagetoid variant of actinic keratosis arising in sun-exposed skin is helpful to avoid the over-diagnosis of extramammary Paget's disease.

Aortic medial changes associated with bicuspid aortic valve: myth or reality?

OBJECTIVE: To determine whether aortic medial changes are more severe in patients who require aortic valve replacement of congenitally bicuspid aortic valves (BAV) than in patients who require replacement of tricuspid aortic valves and acquired valvular disease (AVD). DESIGN: Aortas from autopsies of 14 patients with BAV and 25 with AVD were histologically assessed by two 'blinded' cardiovascular pathologists and analyzed independently with computer-aided morphometry. The aortic valves were examined for valvular fibrosis and calcification. SETTING: The patient population was from a tertiary-care facility. PATIENTS: Patients were selected by retrospective review of autopsy records for patient deaths after aortic valve replacement, over the period 1984 to 1995. RESULTS: There were no significant differences in age (P=0.89), sex (P=0.94), prevalence of systemic arterial hypertension (P=0.37), valvular degenerative changes (P=0.10 and P=1.0) or heart weights (P=0.60) between the two groups. Histological scores for aortic medial degenerative changes including elastic fragmentation, fibrosis and medionecrosis were not statistically different between the groups. However, morphometry demonstrated less elastic tissue in patients with BAV (P=0.003). CONCLUSION: Routine microscopy shows no significant difference in the degree of aortic medial degenerative changes between patients with BAV and AVD. However, morphometry shows less elastic tissue in the aortas of BAV patients. This may explain the anecdotal increase in aortic fragility and propensity for aortic dissection in these patients.

Reliability of transthoracic echocardiography in the assessment of aortic valve morphology: pathological correlation in 178 patients.

OBJECTIVE: To examine patient characteristics affecting the utility of transthoracic echocardiography in determining aortic valve morphology, particularly for the diagnosis of congenital bicuspid aortic valve (BAV). DESIGN: A retrospective comparison of preoperative echocardiographic determination of aortic valve morphology with pathological findings of the explanted valves. SETTING: A tertiary referral centre. PATIENTS: Consecutive patients who had aortic valve replacement between July 1994 and April 1996, and had preoperative echocardiograms. RESULTS: Of 313 patients, 181 (58%) had preoperative echocardiography. Three of the valves were excluded because they were too fragmented for pathological determination of valvular morphology. In the remaining 178 patients, aortic valvular morphology was determined by echocardiography in 104 (58%). Multivariate analysis showed that echocardiography was successful less often in women (odds ratio 0.44, P = 0.03) and in patients with densely calcified valves (odds ratio 0.69, P = 0.02), whereas age had no effect (odd ratio 0.99, P = 0.42). In those with adequate echocardiographic images, echocardiography had both a high sensitivity (0.92) and a high specificity (0.96) for the diagnosis of BAV. CONCLUSIONS: Echocardiography is a useful tool for the diagnosis of BAV, although suboptimal images may pose a problem in many patients, particularly women and patients with heavily calcified valves. When adequate images are obtained, transthoracic echocardiography can reliably identify aortic valvular morphology in most patients.

Implantable cardioverter-defibrillators and the pathologist: comment and cautionary notes.

This paper briefly reviews the components of, the clinical uses of, the techniques to place, and the complications related to implantable cardioverter-defibrillators (ICDs). Information useful in the specific identification of ICDs is presented. A series of recommendations for the autopsy examination or postmortem explantation of ICDs by the pathologist is given. Because of the serious risk of injury to the pathologist possible with postmortem discharges of ICDs which have not been deactivated, and because of the risk of device explosion if the ICD is incinerated, a number of cautionary notes are provided. A brief case with occurrence of accidental postmortem discharge of an active ICD is also presented.

Postinfarct cardiac free wall rupture: the relationship of rupture site to papillary muscle insertion.

The objective of this study was to examine for any relationship between the sites of papillary muscle insertion in left ventricular free wall (LVFW) and the site of LVFW rupture postinfarct. Twenty-five consecutive patients with LVFW rupture (12 men and 13 women, mean age 72.3 yr, range = 48 to 93) at the University of Ottawa Heart Institute during the period of July 1, 1988 to 1992 were studied. Clinical charts were reviewed, and the Formalin-fixed hearts were re-examined grossly and microscopically. Each patient died of tamponade or after surgery for tamponade. Eight (32%) of the 25 infarcts were anteroseptal or anterior, 11 of 25 (44%) were lateral, and 6 of 25 (24%) were inferior or inferoseptal. Four (16%) of the 25 infarcts were less than 2 days old, 9 of 25 (36%) were 2 to 5 days old, and 12 of 25 (48%) were 5 to 10 days old. A separate pre-existent and healed infarct was noted in 36% of patients; however, rupture adjacent to these areas of old infarct occurred in only 8% of cases. In 15 of 25 (60%) cases, free wall rupture occurred in the lateral wall between and at the level of the two papillary muscles. In a further 5 of 25 (20%) cases, the rupture was beside one of the papillary muscles but in anterior or posterior walls. In 20 of 25 (80%) cases, the endocardial tear associated with the LVFW rupture was within 1 cm of the base of one of the papillary muscles as they inserted in LVFW.(ABSTRACT TRUNCATED AT 250 WORDS)

Cicatricial changes in a Ionescu-Shiley bioprosthesis.

The mitral Ionescu-Shiley standard-profile prosthesis of a 53-year-old woman who had it placed 12 years previously is described. The observed cicatricial retraction of one of the bioprosthesis' cusps is unusual, and it contributed to the bioprosthetic dysfunction which necessitated this valve's replacement.

Tissue fragments recovered at cardiac surgery masquerading as tumoral proliferations. Evidence suggesting iatrogenic or artefactual origin and common occurrence.

The entity described in the literature as a "distinctive cardiovascular lesion" resembling histiocytoid hemangioma and more recently referred to as "mesothelial/monocytic incidental cardiac excrescence" may not be a true proliferative lesion. Rather, it may represent an artefact produced by suctioning of the pericardial cavity during cardiac surgery. This hypothesis was explored by comparing two index cases of cardiac histiocytoid hemangioma-like lesions (HLLL) to (a) the contents of extracorporeal bypass pump (ECBP) filters in 22 random cardiac surgical cases, and (b) material adherent to mediastinal and pericardial drains in 15 random post-cardiac surgery cases. In 18 of the 22 ECBP filter cases (82%) and two of the 15 postsurgery cases (13%), tissue fragments indistinguishable from the HHLL index cases were identified. These filter and drain fragments had light microscopic, immunohistochemical, and ultrastructural features identical to those of the index cases, as well as to the HHLLs described in the literature. In neither index case, nor in the study patients, were any proliferative lesions identified at surgery. Three study cases subsequently came to autopsy, and no proliferative lesions were found. This and other evidence strongly suggest that these lesions not only are artefactual, but also are a common occurrence. They are likely produced during cardiac surgery by the cardiotomy suction, with compaction of friable mesothelial strips, other tissue debris, and fibrin into tumor-like fragments that may be transported around the operative site on the suction tip.

Foreign materials found in the cardiovascular system after instrumentation or surgery (Including a guide to their light microscopic identification).

Foreign materials are often seen by light microscopy in surgical or autopsy specimens sent from the cardiovascular clinical services. This paper describes the range of materials that may be recovered in such specimens and provides a pictorial guide to aid in their identification. The spectrum of medical and nonmedical foreign materials that may be encountered in the cardiovascular system is discussed.

Regurgitation of fat and marrow emboli into coronary veins during resuscitation.

Three groups of patients were examined at autopsy for the presence of fat and marrow emboli in the lungs, heart, and other organs. Group 1 was composed of patients with massive pulmonary thromboembolism and attempted cardiopulmonary resuscitation; group 2, patients with pulmonary thromboembolism and without attempted cardiopulmonary resuscitation; and group 3, patients without pulmonary thromboembolism and with attempted cardiopulmonary resuscitation. The results confirm that pulmonary fat and marrow emboli are frequent in those patients who are resuscitated. A novel observation is the finding of multiple coexistent cardiac venous emboli in those resuscitated patients who have pulmonary artery obstruction with pulmonary thromboemboli. These findings suggest that these emboli regurgitate from the right side of the heart to the coronary sinus and cardiac veins in cases of pulmonary artery hypertension.