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The presentation of syphilitic aortitis is often atypical and available serological tests are non-specific. The diagnostic gold standard remains direct identification of microorganisms in tissue. We present a case of syphilitic aortitis that presented as a mediastinal mass and report the use of polymerase chain reaction for Treponema pallidum to diagnose syphilitic aortic disease.

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BACKGROUND: Infliximab is a humanized antibody against tumor necrosis factor alpha (TNF-alpha) that is used in the treatment of Crohn's disease and rheumatoid arthritis. Approximately 147,000 patients throughout the world have received infliximab. Excess TNF-alpha in association with tuberculosis may cause weight loss and night sweats, yet in animal models it has a protective role in the host response to tuberculosis. There is no direct evidence of a protective role of TNF-alpha in patients with tuberculosis. METHODS: We analyzed all reports of tuberculosis after infliximab therapy that had been received as of May 29, 2001, through the MedWatch spontaneous reporting system of the Food and Drug Administration. RESULTS: There were 70 reported cases of tuberculosis after treatment with infliximab, for a median of 12 weeks. In 48 patients, tuberculosis developed after three or fewer infusions. Forty of the patients had extrapulmonary disease (17 had disseminated disease, 11 lymph node disease, 4 peritoneal disease, 2 pleural disease, and 1 each meningeal, enteric, paravertebral, bone, genital, and bladder disease). The diagnosis was confirmed by a biopsy in 33 patients. Of the 70 reports, 64 were from countries with a low incidence of tuberculosis. The reported frequency of tuberculosis in association with infliximab therapy was much higher than the reported frequency of other opportunistic infections associated with this drug. In addition, the rate of reported cases of tuberculosis among patients treated with infliximab was higher than the available background rates. CONCLUSIONS: Active tuberculosis may develop soon after the initiation of treatment with infliximab. Before prescribing the drug, physicians should screen patients for latent tuberculosis infection or disease.

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BACKGROUND: Arteriovenous malformations are composed of large, tortuous arteries and misshapen, veinlike structures. They are extremely uncommon in the genital tract. CASE: An arteriovenous malformation in a Bartholin gland presented as intermittent vaginal bleeding in a 43-year-old woman. CONCLUSION: Persistent unexplained bleeding from Bartholin's gland requires surgical excision.

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The differential diagnosis of melanocytic lesions is fraught with difficulty and a common source of litigation either if a lesion misreported as 'benign' recurs locally or re-presents with nodal metastases or if an atypical naevus is called 'malignant' leading to a cosmetically unsatisfactory wider resection, unwarranted anxiety about prognosis and adverse life insurance prospects. Several authors have claimed that there are valid morphological criteria which, alone or in combination, enable reliable distinction between benign and malignant melanocytic lesions. Others question these criteria and, doubting the extent to which unequivocal diagnoses can be rendered in all cases, believe that the diagnosis is purely subjective and that most diagnostic errors are non-negligent. To address these issues, expert opinions were commissioned from three sets of authors. Okun, Edelstein & Kasznica emphasize that a significant minority of melanocytic lesions are so borderline morphologically that diagnostic uncertainty is allowable and that such uncertainty can be handled responsibly. Kirkham, in favouring the methodical use of criteria, concedes that they are 'largely opinion-based rather than evidence-based, but do go beyond mere subjective pattern analysis'. In agreement with Okun and his colleagues. Slater emphasises that no single feature is reliable by itself and that all aspects, including clinical details, should be interpreted together; he has no hesitation in reporting the diagnosis as 'uncertain' in doubtful cases. In the absence of a specific marker pathognomonic of melanocytic malignancy, the diagnosis will continue to rely on the judicious application of morphological criteria with a small proportion of elusive cases in which diagnostic uncertainty should not be concealed.

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BACKGROUND/PURPOSE: Angiogenesis plays an integral role in wound healing and tissue remodeling. The authors hypothesized that inhibition of angiogenesis would reduce intraabdominal adhesion formation. METHODS: In 98 C57BL6/J mice, a 2-cm midline laparotomy was performed and a 5 mm2 SILASTIC (Dow Corning, Midland, MI) patch fixed to the right side of the peritoneum. Mice were injected with normal saline (n = 54) or TNP-470, an inhibitor of angiogenesis (n = 44; 30 mg/kg every other day over 6 days before surgery until 10 days after surgery). Animals were killed on postoperative days 10, 15, 35, and 55. Adhesions to the SILASTIC (Dow Corning) patch were scored based on their extent, type, and tenacity. Angiogenesis was quantified digitally as the area of vascularized peritoneum over the patch. RESULTS: At day 10, when TNP-470 was stopped, the percentage of vascularized peritoneum over the patch was less in treatment animals than in controls (P = .004). At day 35, the patch in treatment animals was completely covered by vascularized peritoneum, similar to controls. Adhesions in TNP-470 animals were reduced at day 10 compared with controls (P<.05) and remained reduced off treatment at day 55. CONCLUSIONS: Angiogenesis appears to play an important role in the development of intraabdominal adhesions, because the extent of early neovascularization correlates with adhesion formation. Perioperative treatment with TNP-470, a potent endothelial cell inhibitor, reduced vessel ingrowth over the patch and was associated with a sustained reduction in adhesion formation.

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Papillary fibroelastomas are uncommon benign tumors usually involving the heart valves, which historically have been diagnosed at autopsy. With the advent of echocardiography, however, the number of patients diagnosed in life has increased. Papillary fibroelastomas represent a surgically treatable cause of cerebrovascular and cardiovascular ischemia and infarction making their identification clinically important. We report three unusual cases of papillary fibroelastoma; two patients presenting with symptoms of cerebrovascular ischemia and one presenting with myocardial infarction. We also present a comprehensive review of the literature and provide a compilation of all case reports to date.

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Cathepsin B (CB) is involved in the hydrolysis of thyroglobulin (Tg) and thought to be regulated by thyroid stimulating hormone (TSH) in the normal thyroid. Our analyses of 91 thyroid tissues from 71 patients with Graves' disease (GD), multinodular goiter (MNG), papillary carcinoma (PC), or follicular carcinoma (FC), demonstrated a 2-fold increase in expression of CB in GD and an average increase of 1.5-fold in MNG (varying from 10-fold below normal to 6-fold above normal in MNG nodules), as might be predicted by the altered functional status of thyroid follicular cells in those diseases. However, CB activity was not downregulated in conjunction with the known "blocking effect" of malignancy on many thyroid functions, but rather increased on average 9-fold in papillary carcinomas (n = 33), and also showed a marked increase in 2 follicular carcinomas. Activity measurements were confirmed by CB protein detection on Western blot with moderately increased CB protein levels demonstrated in GD, variable expression in nodules of MNG, and markedly increased protein expression in carcinomas. In all diseased states, increased protein was detected primarily as overexpression of the 27 kd heavy chain of 2-chain mature CB and less frequently as overexpression of 31 kd single-chain mature CB. However, an additional 35 kd protein form was noted in 3 of 9 PCs, 1 of 2 FCs, and 1 of 4 GD cases but in none of 10 MNG cases. In conjunction with elevated CB activity plus additional protein bands on Western blots, altered patterns of CB immunohistochemical staining were observed, irrespective of the type of thyroid disease, suggesting certain common changes in CB expression, posttranslational processing, and vesicular trafficking. In summary, GD and MNG thyroid tissues demonstrated altered CB expression in keeping with predicted functional changes in thyroid follicular cells, while increased CB expression in carcinomas indicated a more pathological role for CB in thyroid cancers, possibly related to the processes of invasion or metastasis.

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We present a patient with a distinctive lesion of the skull base, which, at imaging, showed expansile destruction of the sphenoid bone, hemorrhage with fluid-fluid levels, and contrast enhancement of a solid portion. These features ordinarily suggest either giant cell tumor or aneurysmal bone cyst; however, pathologic examination confirmed instead a rare variant of osteosarcoma of the telangiectatic type. Although imaging findings can indicate any of these possibilities, biopsy is an essential step in arriving at the diagnosis.

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Varicella pneumonia usually resolves after treatment, and occasionally miliary calcification develops on the roentgenogram of the chest years afterward. A case of varicella pneumonia is presented that followed a previously unreported course. In this case, usual interstitial pneumonitis (UIP) developed. The pneumonitis responded well clinically and radiographically to corticosteroid treatment. The role of viral pneumonia in the cause of UIP is discussed.

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A case of a rare condition of congenital right anterior high origin of the diaphragm in a stillborn fetus is reported. Associated findings at autopsy were a hornlike subdiaphragmatic intrathoracic accessory lobe of the liver and a lobulated right atrial appendage of the heart. At the superiormost aspect of the malpositioned right anterior diaphragmatic leaf a small phrenic nerve hamartoma was found. The phrenic nerve itself appeared small and not well developed. The phrenic nerve lesion may have been a concomitant or secondary hamartomatous change. Careful clinical and pathological search for concomitant anomalies in diaphragmatic lesions is emphasized.

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The aim of this study was to assess the role of MIB-1 in the classification of cervical squamous lesions and to compare it with a novel proliferation marker, topoisomerase II alpha (TP II alpha). We classified 46 archival uterine cervical cases into 6 groups: normal (n = 3); human papillomavirus (n = 15); mild (n = 10), moderate (n = 7), and severe (n = 6) cervical intra-epithelial neoplasia; and invasive carcinoma (n = 5). The formalin-fixed, paraffin-embedded tissue sections were stained with monoclonal antibodies to Ki-67 (MIB-1) and TP II alpha with a standard streptavidin immunohistochemical technique, with antigen retrieval to assess enzyme presence. The results were based on nuclear staining and percentage of positivity. We found that the mean percentage of positive nuclei increased from normal through increasing grades of dysplasia to its maximal level in invasive carcinoma. The level of positive nuclei in the epithelium also generally increased from basal to full thickness with progression of the lesions. The correlation between the percentage of nuclei positive for both antibodies with the use of linear regression was close, with an r value of 0.85. Our conclusions were that MIB-1 is an adjunct in the classification of squamous lesions of the uterine cervix and that TP II alpha is a useful proliferation marker in this setting.

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Small bowel adenocarcinomas account for 3% of gastrointestinal malignancies, and 20 to 25% of these arise in the ileum. Clinical presentation is variable, and early diagnosis is difficult. A 56-year-old postmenopausal woman presented with crampy abdominal pain, anorexia, and weight loss. Pelvic examination and ultrasound revealed a 6 x 8-cm complex right adnexal mass. At laparotomy, en bloc resection of the right adnexa and the densely adherent ileal segment was performed along with a hysterectomy and a left salpingo-oophorectomy. The final pathology showed a moderately differentiated invasive adenocarcinoma of the ileum with a malignant enterotubal fistula. This is the first case reported in the literature of an ileal adenocarcinoma with a tubal fistula masquerading as an adnexal mass.

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BACKGROUND: Midtrimester genetic amniocentesis is a commonly performed procedure, with acknowledgment of some risk to mother and fetus. CASE: We present an unusual case of midtrimester genetic amniocentesis with bowel injury and resulting septic shock, adult respiratory distress syndrome, and disseminated intravascular coagulation. A total abdominal hysterectomy and bilateral salpingoophorectomy were required for resolution of sepsis. The patient also required prolonged ventilatory support postoperatively. CONCLUSION: Although relatively safe, genetic amniocentesis can result in serious morbidity, and attention to technique should be maintained.

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Systemic embolization is a common complication of left atrial myxoma; however, coronary embolism leading to acute myocardial infarction is rare. The use of echocardiography has increased the detection of intracardiac tumors when signs and symptoms are not evident. Echocardiography is the diagnostic procedure of choice in the initial evaluation of patients with suspected left atrial myxoma.

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Aseptic meningitis is a rare complication of relapsing polychondritis. We describe a 60-year-old man who developed a prolonged episode of aseptic meningitis for which no cause could be determined and, that resolved spontaneously. He then developed classic relapsing polychondritis 14 months later. He subsequently had another episode of prolonged meningitis complicated by hydrocephalus. No infectious cause for the meningitis could be determined after extensive investigation including meningeal biopsy. The patient responded to corticosteroids and antituberculous therapy.

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A case of fatal cardiac larva migrans in a 10-year-old boy is described. The autopsy findings were quite dramatic, with a bosselated, sessile polypoid mass involving the left ventricular myocardium and protruding into the ventricular lumen. The precise morphologic characterization of the zoonotic ascarid larva was impaired by advanced resorption of the larva by an inflammatory infiltrate. Nonetheless, morphometry of the larval remnants strongly suggested the raccoon ascarid, Baylisascaris procyonis, as the causative agent.

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