Calciphylaxis After Aortic Valve Replacement in a Patient with End-Stage Renal Disease.

In this study, we report a case of a patient on dialysis who presented necrotic lesions on the legs and penile ulceration 7 years after a mechanical aortic valve replacement. The diagnosis of calciphylaxis was not confirmed even after skin biopsy, and multidisciplinary management was not initiated until the patient was admitted with septic shock. Cardiovascular surgeons should be aware of warfarin-induced calciphylaxis, whose pathophysiology differs from that of atherosclerosis. Considering poor long-term survival of dialysis patients, mechanical valves should be reserved only for those patients whose estimated survival is longer than the time taken for a biological valve to deteriorate.

Engrailed Homeobox 1 and Cytokeratin 19 Are Independent Diagnostic Markers of Eccrine Porocarcinoma and Distinguish It From Squamous Cell Carcinoma.

OBJECTIVES: The diagnostic utility of En1 in the histopathologic differentiation of eccrine porocarcinoma (EPC) from invasive squamous cell carcinoma (SCC) was investigated. METHODS: Expression of En1 and CK19 in 16 cases of EPC was immunohistochemically examined and compared with that in 32 cases of SCC. RESULTS: In all 16 EPCs, En1 was expressed in 3% to 100% of tumor cells. In 20 of the 32 SCCs, En1 was expressed in 3% to 90% of tumor cells. A total of 13 of the 16 EPCs and five of the 32 SCCs were judged as En1 positive, with a cutoff value of 25%. In addition, 11 of the 16 EPCs and four of the 32 SCCs were CK19 positive. The frequencies of En1- and CK19-positive cases were significantly higher in EPCs than in SCCs. In a logistic regression analysis for predicting EPC, En1 and CK19 were independent markers. When expression patterns of En1 and CK19 were combined, none of the 32 SCCs was both positive. In contrast, 15 of the 16 EPCs were positive for either En1 or CK19. CONCLUSIONS: A combination of En1 and CK19 expression can improve the accuracy of histologic diagnosis of EPC.

Radiologically indeterminate pulmonary cysts in Birt-Hogg-Dubé syndrome.

Birt-Hogg-Dubé (BHD) syndrome is an inherited disease characterized by recurrent pneumothorax. We report some unusual clinicopathologic features of the lung in a Japanese family with this syndrome presenting with recurrent pneumothorax. Radiologic imaging did not show detectable lesions; however, at video-assisted thoracic surgery (VATS), multiple diffusely distributed microcysts were visible on the pleura. This characteristic morphologic feature was common to all affected family members. The proband underwent genetic testing and BHD syndrome was diagnosed. Although many patients with BHD syndrome with pneumothorax show obvious pulmonary cysts, this case suggests that radiologically indeterminate cysts have the potential to cause pneumothorax.

An autopsy case of chronic active Epstein-Barr virus infection (CAEBV): distribution of central nervous system (CNS) lesions.

A 27-year-old Japanese man developed recurrent respiratory and central nervous system (CNS) symptoms, and hemophagocytic syndromes with a clinical course of 6 years. CT demonstrated multiple nodular lesions in the bilateral lungs, and MRI revealed multiple abnormal intensity areas in the brain and spinal cord. Cerebrospinal fluid (CSF) examination disclosed mild pleocytosis and the presence of Epstein-Barr virus (EBV)-DNA detected by polymerase chain reaction (PCR). The patient died of a hemorrhagic shock associated with a hemophagocytic syndrome. A postmortem study revealed massive hemorrhage in the abdominal cavity and iliopsoas muscles, as well as diffuse infiltration of lymphocytes and/or macrophages into the lungs, liver, kidneys, spleen, cardiac muscle, bone marrow, and CNS. The severe involvement was demonstrated in the CNS, especially in the spinal cord and brainstem. The CD3 positive cells of the brainstem were EBV-encoded RNA 1 positive. This is the first autopsy case of chronic active EBV infection (CAEBV) in which severe and extensive CNS involvement was demonstrated.

A case of malignant eccrine spiradenoma metastatic to intramammary lymph node.

Eccrine spiradenoma (ES) is a fairly common, benign, cutaneous tumor originating from the sweat glands. In contrast, the malignant counterpart of ES, malignant eccrine spiradenoma (MES), is extremely rare. A long-standing lesion rarely begins to enlarge rapidly. A growth that results in ulceration or discoloration may be associated with malignant transformation. We present the first reported case of this tumor metastasizing to an intramammary lymph node (IMLN). The uncommon metastasizing focus of the periumbilical MES and its histopathological similarity with a primary breast carcinoma made the diagnosis difficult.

Proposal for two strategies to prevent remnants of gastric cancers after endoscopic mucosal resections: fluorescein electronic endoscopy and rapid stump diagnosis based on pit patterns.

BACKGROUND: The aim of the present study was to establish strategies to prevent cancer remnants after gastric endoscopic mucosal resection (EMR). METHODS: Whether surgical stumps could be diagnosed by pit patterns was examined on 38 well-fixed EMR materials. Furthermore, a rapid stump diagnostic method, which enables pit patterns of EMR material to be observed within 10 min, was developed. This rapid stump diagnosis was tested in 6 EMR cases of early gastric cancers, one of which was absolutely obscure to routine endoscopy. Fluorescein electronic endoscopy had been performed to reveal the extent of this cancer before EMR. RESULTS: Adenocarcinomas showed irregular pit patterns that were significantly different from those of normal gastric mucosae. Among the 38 cases, all 20 EMR materials that were diagnosed as stump (-) and 5 that were diagnosed as stump (+), based on pit patterns, were confirmed by tissue sections to be stump (-) and stump (+), respectively. Six of the 13 EMR materials diagnosed as stump (+/-) by pit patterns were finally diagnosed as stump (+) by tissue sections. These data indicate that cancer remnants could be avoided if the stumps were judged positive or suspiciously positive just after EMR, and additional resections were continuously done until the stumps became negative. Rapid stump diagnosis based on pit patterns was successful in all 6 tested cases. The extent of the early gastric cancer that was absolutely obscure to routine endocscopy was clearly and exactly revealed by fluorescein electronic endoscopies done before EMR and subsequent rapid stump diagnosis. CONCLUSION: It is probable that fluorescein electronic endoscopy, as a precise preoperative examination, and rapid stump diagnosis, based on pit patterns, will become effective strategies to prevent cancer remnants after EMR.

Fluorescein electronic endoscopy: a novel method for detection of early stage gastric cancer not evident to routine endoscopy.

BACKGROUND: Fluorescence endoscopy with fluorescein sodium in the stomach was evaluated by using a newly developed fluorescence electronic endoscopic system. METHODS: Sixteen patients with early stage gastric cancer diagnosed by white light endoscopy and chromoendoscopy underwent fluorescein electronic endoscopy before surgery. The resection specimens underwent thorough histopathologic evaluation. RESULTS: About 10 seconds after intravenous injection of fluorescein, fluorescence appeared and immediately spread throughout the gastric surface. A few minutes later, differentiated early stage gastric cancers with more abundant stroma than surrounding normal mucosa exhibited significantly stronger fluorescence, and those with less stroma exhibited weaker fluorescence than the surrounding normal mucosa. Undifferentiated early stage gastric cancers, in which the stroma became wider because foveolae were collapsed from malignant invasion, expressed stronger fluorescence intensity. In all cases, the borders of early stage gastric cancers were clearly demonstrated. Among the 16 patients, 6 accompanying flat lesions and 1 tiny lesion not evident by routine endoscopy were detected. The extent of the cancers, as determined by fluorescence endoscopy, were similar to those determined histopathologically. CONCLUSIONS: Fluorescein electronic endoscopy is useful in determining the extent within the mucosa of gastric cancers when this is obscure by standard endoscopic observation, and for detecting extremely early stage cancer that is not evident by conventional endoscopic observation.