Tumour of the juxtaoral organ.

The juxtaoral organ is a normal and constant structure of the oral cavity. It consists of benign epithelial nests. We describe an intraoral tumour of the juxtaoral organ in a child. The tumour was not diagnosed after clinical and radiological examinations because it is extremely rare. A histological examination revealed a tumour of the juxtaoral organ, presumed to be neuroid hamartoma. This is only the second time that a tumour of the juxtaoral organ has been described in a child. We also describe the location, the embryology, the histology and the function of this organ. This is important because this structure can be confused with carcinomas of the oral cavity when examining frozen sections.

Elevated plasma level of lipotropin revealing an occult carcinoid tumor with normal plasma adrenocorticotropin.

The increases in the level of plasma lipotropin (LPH) and in the LPH/ACTH ratio are considered diagnostic tools in ectopic ACTH syndrome. However, plasma ACTH is also elevated in this syndrome. We report a case of a small carcinoid tumor with an increase in both ACTH and LPH in plasma before surgery. Eight months after the tumoral resection, plasma LPH alone was increased again, whereas plasma ACTH and plasma and urinary cortisol remained normal in this apparently cured patient. This repeated abnormality was the only available feature that allowed successful removal of the occult tumoral residue.

Lower limb giant cell arteritis and temporal arteritis: followup of 8 cases.

Among 8 patients with giant cell arteritis (GCA) (6 women, 2 men) whose clinical presentations were compatible with temporal arteritis (TA), 6 were followed for 37-105 (mean 74.9) months, one died shortly after treatment onset, and the last was asymptomatic (10 mg steroids/day) when lost to followup at 29 months. All 8 patients had bilateral leg claudication of recent onset; for 6 patients, this was the first symptom. All leg angiograms showed multiple, bilateral, long and smooth stenoses, thromboses, or both. Biopsies of diseased leg arteries from 4 patients provided histological proof of GCA; another case was histologically proven post mortem. Among the 5 patients who met at least 3 American College of Rheumatology criteria of GCA or TA, 3 without histologically documented leg GCA also had biopsy proven temporal GCA (n = 1), or headaches and claudication and angiographic inflammatory arteritis of the arms (n = 2). All patients received steroids; 3 had bypasses, one with endarterectomy. Five are asymptomatic after 24-100 months of steroids (mean 50.6). Revascularization was not successful; one amputation was necessary. Large artery involvement in GCA can affect the legs. Bilateral and rapidly progressive intermittent claudication of recent onset is the most common symptom, even in the absence of headaches or the presence of a silent inflammatory syndrome. Early diagnosis allows rapid initiation of steroid therapy, which is usually able to generate a sufficiently good response to avoid vascular surgery.

Expression of the chemokine RANTES in pulmonary Wegener's granulomatosis.

Wegener's granulomatosis (WG) is an inflammatory, destructive, angiotropic lesion. The inflammatory process involves accumulation of macrophages, lymphocytes, and polymorphonuclear neutrophils. We studied 6 lung biopsy specimens from patients with WG to characterize the cellular infiltrate and to analyze the mechanism of immune cell recruitment. We show that lymphocytes accumulating in WG lesions are mostly memory CD4(+)CD45RO(+) T lymphocytes and, although less numerous, CD8(+)CD45RO(+) T lymphocytes. Few if any B lymphocytes or natural killer cells are present within lesions. The chemokine RANTES (regulated upon activation in normal T cells, expressed and secreted) has been reported to recruit memory T lymphocytes and macrophages selectively. We used reverse-transcription polymerase chain reaction, in situ hybridization, and immunohistochemistry to study its production in WG. RANTES was expressed at a higher level in WG lungs than in normal controls, especially around microabscesses. As visualized immunohistochemically in serial sections with anti-RANTES monoclonal antibody, RANTES production was produced mainly by macrophages. Expression of the gene coding for interferon-gamma (IFN-gamma), a potent RANTES inducer, was also studied. Its expression was also much stronger in WG than in controls. Our observations are consistent with a cascade of events leading to the recruitment of immune cells in WG, sequentially involving production of IFN-gamma by T lymphocytes and RANTES production by macrophages, leading to the homing of memory T-helper lymphocytes and macrophages. HUM PATHOL 32:320-326.

[Costal chondroma and chondrosarcoma]. / Chondromes et chondrosarcomes costaux.

The aim of this study was to report two cases of chondrosarcoma located on the chest wall, in order to emphasize the difficulty encountered by the pathologist to differentiate a chondrosarcoma from a chondroma and the importance, in our opinion, of performing a large resection with wide margins in all cases.

Differential gene expression in mesothelioma.

To investigate the molecular events controlling malignant transformation of human pleural cells, we compared constitutive gene expression of mesothelioma cells to that of pleural cells. Using cDNA microarray and high-density filter array, we assessed expression levels of > 6500 genes. Most of the highly expressed transcripts were common to both cell lines and included genes associated with stress response and DNA repair, outcomes consistent with the radio- and chemo-resistance of mesothelioma. Interestingly, of the fewer than 300 genes that differed between cell lines, most functioned in (i) macromolecule stability, (ii) cell adhesion and recognition, (iii) cell migration (invasiveness), and (iv) extended cell division. Expression levels of several of these genes were confirmed by RT-PCR and could be useful as diagnostic markers of human mesothelioma.

Necrotizing granulomata of the aortic valve in Wegener's disease.

Wegener's disease is an inflammatory disease of unknown etiology, characterized by a granulomatous-necrotizing general vasculitis. Cardiac involvement in the form of aortic pathology is not frequent. We report a case of Wegener's granulomatosis which required prosthetic aortic valve replacement for aortic valve insufficiency. Microscopic examination of the valve demonstrated histopathology typical of Wegener's disease.

In vitro effects of desflurane, sevoflurane, isoflurane, and halothane in isolated human right atria.

BACKGROUND: Direct myocardial effects of volatile anesthetics have been studied in various animal species in vitro. This study evaluated the effects of equianesthetic concentrations of desflurane, sevoflurane, isoflurane, and halothane on contractile parameters of isolated human atria in vitro. METHODS: Human right atrial trabeculae, obtained from patients undergoing coronary bypass surgery, were studied in an oxygenated (95% O2-5% CO2) Tyrode's modified solution ([Ca2+]o = 2.0 mM, 30 degrees C, stimulation frequency 0.5 Hz). The effects of equianesthetic concentrations (0.5, 1, 1.5, 2, and 2.5 minimum alveolar concentration [MAC]) of desflurane, sevoflurane, isoflurane, and halothane on inotropic and lusitropic parameters of isometric twitches were measured. RESULTS: Isoflurane, sevoflurane, and desflurane induced a moderate concentration-dependent decrease in active isometric force, which was significantly lower than that induced by halothane. In the presence of adrenoceptor blockade, the desflurane-induced decrease in peak of the positive force derivative and time to peak force became comparable to those induced by isoflurane. Halothane induced a concentration-dependent decrease in time to half-relaxation and a contraction-relaxation coupling parameter significantly greater than those induced by isoflurane, sevoflurane and desflurane. CONCLUSIONS: In isolated human atrial myocardium, desflurane, sevoflurane, and isoflurane induced a moderate concentration-dependent negative inotropic effect. The effect of desflurane on time to peak force and peak of the positive force derivative could be related to intramyocardial catecholamine release. At clinically relevant concentrations, desflurane, sevoflurane, and isoflurane did not modify isometric relaxation.

Desmoid fibromatosis of the shoulder and of the upper chest wall following a clavicular fracture.

A desmoid tumor of the shoulder girdle infiltrating the upper chest wall and weighing 1500 g was almost completely removed in an 18-year-old man, 27 months after a bifocal fracture of the clavicule. Thirteen years later, the patient was free of recurrence. The interval time between trauma and diagnosis, as the particular characteristics of aggressive fibromatosis, strongly support a major causal role of the clavicular fracture in the occurrence of this tumor.

Fine deletion mapping of chromosome 8p in non-small-cell lung carcinoma.

Several somatic genetic alterations have been described in non-small-cell lung carcinomas (NSCLC). Recurrent chromosomal deletions have suggested the presence of tumor-suppressor genes specifically involved in lung carcinogenesis. For one of these, 2 non-overlapping regions have been proposed on the short arm of chromosome 8, encompassing the LPL and NEFL genes. The LPL region has been extensively studied in NSCLC and other cancer types. Two genes, N33 and PRLTS, have been identified, but the small number of mutations excludes their involvement in the vast majority of tumors. In order to delineate a reliable region of deletional overlap on chromosome 8p in NSCLC, a series of 77 NSCLC was studied for 34 microsatellite polymorphisms distributed on chromosome 8p, using multiplex-PCR amplification. After purification of tumor nuclei by flow cytometry based on either the abnormal DNA index or the presence of a high expression of cytokeratin, allelic losses on chromosome 8p were observed in 39% of cases. Measurement of DNA index showed that 62% of tumors were hyperploid; allelic losses were more frequent in hyperploid than in diploid tumors (54% vs. 14%; p < 10(-4)). Deletions of part of the short arm were observed in 7 instances. Our data allow definition of an interval of common deletion, flanked by the loci D8S511 and D8S1992, where the putative tumor-suppressor gene might be localized.

[Pleural fibromas: a review based on 12 cases]. / Les fibromes pleuraux: mise au point à partir de douze observations.

The authors report 12 cases of pleural fibroma, which is a rare benign tumor, usually discovered incidentally. However, these tumors are sometimes associated with a paraneoplastic syndrome, or may lead to compressive symptoms related to their large size. Radical resection is mandatory because recurrence may occur, sometimes several years later with a malignant behavior. Long-term follow-up is therefore necessary to detect any recurrence, particularly when certain prognostic factors are initially present.

Detection of cardiac myxoma by F-18 FDG PET.

Intracardiac tumors occur infrequently and are difficult to diagnose with CT and MRI. The authors describe the successful imaging of a right atrial myxoma with F-18 FDG PET.

Recurrent left-sided heart leiomyosarcoma: should heart transplantation be legitimate?

Leiomyosarcoma of the heart is an uncommon primary malignant tumor with poor postoperative survival that may be measured in months. A leiomyosarcoma of the left atrium was diagnosed in a 47-year-old man. Initial admission was for acute pulmonary edema requiring emergency surgery. The tumor involved the left atrial cavity, and a radical resection was performed. Six months later an isolated myxomatous recurrence was detected. Heart transplantation was then performed. The patient is in good health 20 months after operation with no evidence of residual disease or recurrence. The literature has been reviewed. Surgical resection is not an adequate treatment for leiomyosarcoma of the left atrium and early heart transplantation probably offers the only hope for these patients.

[Cystic lymphangioma of the mediastinum. Apropos of 3 cases, review of the literature]. / Les lymphangiomes kystiques du médiastin. A propos de trois cas, revue de la littérature.

Cystic hygromas are benign cysts frequently discovered incidentally on chest X ray films. Although CT scan provides helpful information about the size, density, and site of the cysts, it cannot establish a precise diagnosis concerning its nature. Complete removal of the cyst is the treatment of choice, and allows precise diagnosis on histological examination. However, surgical excision is sometimes difficult, due to the size and extension of the cysts, infiltrating mediastinal planes, enveloping great vessels, displacing mediastinal organs without invasion. The difficulty of completely eradicating certain cysts explains cases of insidious progression with compressive recurrence.

[Leiomyosarcoma of the heart and great vessels]. / Les léiomyosarcomes du coeur et des gros vaisseaux.

Leiomyosarcomas (LMS) of the heart are exceptional primary malignant tumours with a catastrophic prognosis and a mean survival measured in months. Extensive radical surgical resection clearly remains the most appropriate treatment. We report three cases observed over a 3-year period, consisting of an LMS of the inferior vena cava, an LMS of the pulmonary artery trunk and an LMS of the left atrium. The first case was treated by radical resection and reconstruction by autologous vein graft of the cavorenal junction, the second case was treated by extensive resection and prosthetic reconstruction of the pulmonary artery bifurcation and the third case was treated by a first radical resection of the left atrium, requiring total cardiectomy and orthotopic heart transplantation for local recurrence at the sixth month. The survical was significantly improved compared to other treatment options (chemotherapy, radiotherapy). The first patient is still alive without recurrence at two years; the second died 12.5 months after the surgical procedure and the medium-term follow-up of the transplanted patient revealed cerebral and hepatic metastases nine months after transplantation. The authors review the literature concerning these extremely rare malignant tumours. Recent progress of diagnostic investigations, such as spiral CT with reconstruction, MRI, positron emission tomography (PET), are now able to establish the diagnosis more rapidly and therefore allow more radical surgical resection. This resection, possibly combined with venous reconstruction, must be associated with adjuvant therapies. Heart transplantation should be considered among the treatment options for leiomyosarcomas of the heart, in order to improve the poor prognosis of these lesions affections a young population.

[Etiology and diagnostic of viral bronchopneumonias]. / Etiologie et diagnostic des bronchopneumopathies virales.

Community viral bronchopneumonias are frequent, mainly in children, and can be associated to all respiratory viruses: influenza- and parainfluenzavirus, respiratory syncytial virus, adenovirus, rhinovirus. The diagnostic method which proves viral infection of the respiratory tissues is selected as the direct detection by an immunofluorescence assay of viral infected cells in respiratory samples. In them, viral isolation or nucleic acid detection by PCR provide an amplification of the viruses. By using PCR-hybridation techniques viral detection is overall increased of 1.5 times for respiratory syncytial virus, 1.9 for parainfluenzavirus 3, 4 for rhinovirus and 10 times for adenovirus. This increased sensitivity raises questions about the meaning of the detection of viral sequences in nasal aspirates, with or without clinical signs. Cytomegalovirus (CMV) is a major agent of pneumonia in immunocompromised patients. All virological markers of CMV infection have to be sought (antigenemia, viremia...), but specific inclusions in pulmonary cells are the single diagnosis criteria. As pulmonary biopsies are rarely available and CMV inclusions rarely found in BAL, it has been reported useful to look for high viral loads or late m-RMA transcripts in these samples. Adenovirus pneumonia are unfrequent in these patients and mostly associated to rare or atypical strains. Such PCR-hybridization systems deserves also to be used in these cases.

Pulmonary artery trunk leiomyosarcoma.

Pulmonary artery leiomyosarcomas are rare tumors. They cause symptoms suggestive of recurrent pulmonary emboli. As in the case reported here, the diagnosis is virtually never considered initially, despite modern diagnostic imaging methods, but usually made at autopsy or at histologic examination of material removed from the pulmonary artery at surgery. The prognosis remains poor and prolongation of life up to 6 months has not been clearly demonstrated with radical excision in conjunction with radiotherapy. The difficulty is the accurate diagnosis required to consider these tumors for curative resection, which is the only hope for a longer disease-free course.