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OBJECTIVE: To assess the prevalence and clinical significance of small-vessel vasculitis (SVV) surrounding an uninflamed temporal artery (TA) in patients diagnosed as having giant cell (temporal) arteritis (GCA) and/or polymyalgia rheumatica (PMR). METHODS: Patients with GCA and/or PMR (n = 490) were included in this multicenter prospective study. Slides of TA biopsy specimens were reviewed by 2 pathologists who were blinded with regard to clinical information. SVV was defined as aggregates of mononuclear inflammatory cells surrounding a capillary, distant from an uninflamed temporal artery. Clinical and biologic data of patients in the SVV group (n = 35) were compared with data of patients with biopsy-proven GCA (n = 280) and with negative TA biopsy findings (n = 175). RESULTS: SVV was diagnosed in 18 women and 17 men (mean +/- SD age 74.5 +/- 9.4 years). The group of patients with SVV had a higher proportion of men than in the entire GCA series, had systemic symptoms, headache, jaw claudication, and an abnormal temporal artery less frequently at clinical examination, but had symptoms of PMR more often than patients in the biopsy-proven GCA group (P = 2.6 x 10(-7), odds ratio 9.17 [95% confidence interval 3.44-24.4]). Levels of inflammation markers were significantly lower in the SVV group. Patients in the SVV group had fever less frequently than patients in the group with negative TA biopsy findings, but otherwise shared the same clinical (including PMR symptoms) and biologic features. Eighteen of the 94 patients with pure PMR (19%) had SVV. CONCLUSION: SVV is often neglected by pathologists, and appears to be strongly associated with PMR symptoms in patients with a clinical diagnosis of GCA and/or PMR. However, SVV as a new diagnostic criterion for PMR must be assessed in prospective studies.

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OBJECTIVES: To quantify and study the distribution of innervation of the left atrium and the pulmonary veins in humans. BACKGROUND: Damage to cardiac nerves has been hypothesized as the explanation for successful radiofrequency ablation of atrial fibrillation. METHODS: From January 2003 to September 2003, histologic and quantitative studies of innervation of the left atrium and the pulmonary veins was performed in 43 consecutive necropsied adult hearts (30 men and 3 women; mean age 45.5 +/- 12.4 years). The left atrium was sectioned in 1-cm slices from left to right, with the plane of section perpendicular to the long axis of the heart. Sections of the pulmonary veins at their ostia and sections 1 cm away of this structure also were obtained. Nerve fiber density was counted manually for each case and expressed as the mean number per slice. RESULTS: Numerous epicardial nerve fibers and ganglia having distinct patterns of distribution in the left atrium were found. Nerve density was significantly higher at the ostia of the four pulmonary veins than in their distal part (7.1 +/- 2.1 vs 5.2 +/- 1.3 for left upper pulmonary vein; 6.3 +/- 1.5 vs 5.2 +/- 1.7 for right upper pulmonary vein; 7.4 +/- 2 vs 5.9 +/- 2 for left lower pulmonary vein; 6.7 +/- 1.8 vs 3.9 +/- 1.3 for right lower pulmonary vein). The left superior vein was significantly more innervated than the right inferior vein (12.3 +/- 3 vs 10.6 +/- 1.4). Gradients of innervation were found from right to left (9.8 +/- 4.6 vs 18.5 +/- 6.6, P < .05) and from the front to the rear of the atrium (17.2 +/- 6.4 vs 20.7 +/- 6.5, P < .05). The same heterogeneous distribution was observed at the myocardial level but with thinner nerve fibers, making quantification difficult. Only very thin nerve fibers were present in the endocardium. CONCLUSIONS: The human left atrium exhibits several gradients of innervation at discrete sites. These findings may have clinical implications for radiofrequency ablation of atrial fibrillation.

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A series of 90 thymic epithelial tumors were reviewed and classified by histopathologic characteristics into the three major categories (A, B, and C) recognized by the WHO schema. Each tumor type was correlated with patient characteristics and clinical data (age, sex, presence of myasthenia gravis, tumor size and invasiveness, and completeness of resection), and with outcome (survival, recurrence, and metastasis). All tumors were categorized by the WHO schema. Myasthenia gravis was present in 32 patients, mostly young and with type B thymic epithelial tumors. Tumors were invasive in 56% of cases, but resection was total in 67% of patients and only partial in the rest. Five factors were shown by univariate analysis to be associated with a favorable prognosis: presence of myasthenia gravis (p = 0.0389), younger age (p = 0.0022), completeness of resection (p = 0.0001), noninvasiveness (p = 0.0138), and tumor type A or B, as opposed to type C (p = 0.0001). Prognosis for types A and B was not significantly different, suggesting that the subtypes of types A and B thymic epithelial tumors should be regarded as a morphologic continuum rather than as distinct histologic variants.

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Primary cardiac lymphoma is an extranodal non-Hodgkin's lymphoma exclusively located in the heart and/or pericardium, extremely rare in immunocompetent patients, and more frequent in immunodepressed patients. We present 3 retrospectives cases of primary cardiac lymphoma in immunocompetent patients and review 35 cases reported in the literature. Two patients were adults and one was a child. Primary cardiac lymphoma presented with constitutional symptoms in two cases and superior vena caval syndrome in one case. Diagnosis of a tumor mass was made in all cases by transthoracic echocardiography. Primary cardiac lymphoma arose in the heart right chambers in two cases. Histological diagnoses, obtained after thoracotomy, were diffuse large B-cell lymphoma in two cases, and Burkitt's lymphoma in one case. All three cases received chemotherapy, combined with radiotherapy in one patient. Of our patients, 2 are alive and asymptomatic 12 months and 33 months after diagnosis. In conclusion, diagnosis of primary cardiac lymphoma is difficult due to non-specific clinical manifestations and should be considered in patients with a cardiac mass sometimes with pericardial effusion. It is confirmed using transthoracic echocardiography and magnetic resonance imaging and certified using cytology or open biopsy. The only effective treatment is chemotherapy, but prognosis remains poor.

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BACKGROUND: Chest physicians have a limited experience of primary pulmonary sarcomas, which represent a particular entity among rare intrathoracic neoplasms. DESIGN: Retrospective review of medical records. PURPOSE: To study patients with primary sarcomas of the lung diagnosed in our pathology department in order to define their clinical characteristics, treatment, and prognosis. PATIENTS: The study group consisted of 12 patients, with a mean age of 53 years. RESULTS: The main symptoms were chest pain, and cough. Imaging findings consisted of: eight single peripheral opacities, three single parahilar opacities, and one lobar actelectasis. The histologic diagnoses confirmed in all cases by detailed immunohistochemical study were leiomyosarcoma (7), monophasic synovial sarcoma (2), one case each of malignant peripheral nerve sheath tumor (MPNST), epithelioid sarcoma, and malignant fibrous histiocytoma. Thoracic surgery done in nine cases consisted of six lobectomies with further parietal resection in two cases, and three pneumonectomies. Four patients received chemotherapy and two patients had radiation therapy postoperatively. Follow up available on 12 patients ranged from 3 to 144 (mean 42) months. Long term survival up to 3 years was observed in five patients. Median overall survival was 48 months. Overall 5-year survival rate was 38%. CONCLUSIONS: Primary sarcomas of the lung are a rare and aggressive malignancy. Treatment and prognosis do not differ from other soft tissue sarcomas.

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