[Intracerebral extension of cervical paraganglioma]. / Extension intracérébrale d'un paragangliome cervical.

A large paraganglioma invaded the neck and skull, with extension into the right supratentorial cerebral hemisphere through the calvarium. Immunohistological markers were found useful for the diagnosis. The patient died rapidly. Despite no evidence of metastasis, the usually benign paraganglioma can have a malignant course.

[Undifferentiated carcinomas of the thyroid corpus. Apropos of 10 cases]. / Carcinomes indifférenciés du corps thyroïde. A propos de dix cas.

Ten undifferentiated thyroid carcinomas detected between 1976 and 1991, were reviewed by means of immunohistochemical techniques. These tumors were highly aggressive with a mean survival rate of three months after the histological diagnosis. They were predominant in women and always occurred in old people (mean age 63.1 years). Four tumours were composed of differentiated trabecular areas. Another one was included in a microvesicular adenoma and a sixth one occurred as a recurrence of a papillary carcinoma surgically treated four months previously. Six tumours were cytokeratin-positive and two of them showed a cytokeratin-vimentin coexpression. These results, like those of an electron microscopic analysis of 1 case, confirm the epithelial origin of these tumours. Undifferentiated carcinomas must be distinguished from poorly differentiated carcinomas and from malignant non Hodgkin lymphomas which have a better prognosis and a different therapeutic approach. The immunohistochemistry and the electron microscopy are useful to identify undifferentiated thyroid carcinomas.

Centrofacial malignant granulomas. Clinicopathologic study of 40 cases and review of the literature.

An important problem in the treatment of centrofacial ulcerations is to establish a precise diagnosis, since similar clinical and microscopic findings can result from many different causes (as in the centrofacial malignant granuloma syndrome [CFMG]). A comprehensive surgical biopsy protocol (known as SNFMI/GMCF), involving microbiology, parasitology, immunology and pathology laboratories, allowed us to evaluate and to treat 40 cases of CFMG, who form the basis of this report. In 13 of them, specific diagnoses were found and curative treatments could be given. In the remaining 27, the optical microscopy pattern met the criteria for CFMG without identifiable origin or the presence of so-called lethal midline granulomas; however, a more precise evaluation with the help of immunofluorescence studies led to the recognition of malignant lymphoma (ulcerative lymphoma of the midface [ULM]). Most of these lymphomas belonged to the T cell lineage; the others were of B lymphoid origin, or, more rarely, of histiocytic origin. Patients with ULM received radiotherapy and chemotherapy with a response rate of 70.3%; however, the toxicity was significant, with frequent occurrence of chemotherapy-induced neutropenia followed by severe infectious facial cellulitis. Six patients were enrolled in a preliminary open trial of treatment with recombinant alpha-2b interferon with little success. Three patients were treated with radiation therapy only, and survived. Thus, CFMG is a syndrome with specific causes and treatments, requiring multiple extensive biopsies to make the correct diagnosis. The recognition of ULM as the cause of the previously called "lethal midline granulomas" leads logically to the use of chemotherapy with growth factors in order to ameliorate its bad prognosis.

[Heart conduction disorders in long-term treatment with chloroquine. Two new cases]. / Troubles de la conduction cardiaque au cours d'un traitement prolongé par chloroquine. Deux nouvelles observations.

Cardiac complications are exceptional in long-term chloroquine therapy; congestive heart failure and restrictive cardiomyopathy may develop, but disorders of conduction are more frequent. The characteristics of these disorders emerge from 12 cases in the literature and from 2 personal cases. The usual disorder is fascicular block which may become a complete, syncopal, atrioventricular block, as in one of our 2 patients. The time elapsed between the beginning of treatment and the occurrence of these disorders (2 to 30 years) and the total dose of chloroquine received (100 to 2,500 g) are extremely variable. Retinopathy or neuromyopathy is present in 64 and 35 percent of the cases respectively. The diagnosis is confirmed by endomyocardial biopsy with electron microscopic study which shows vaculoar myopathy with numerous large secondary lysosomes containing a dense material of lamellar structure (myelinic figures, curvilinear bodies). Regression of heart conduction disorders after withdrawal of chloroquine seems to be inconstant and incomplete. The rare occurrence of this complication raises the question of genetic predisposition. We believe that chloroquine therapy should be contra-indicated in patients with a history of conduction disorders and that a 6-monthly electrocardiographic control of these patients would be justified.

[Coronary pathology after heart transplantation]. / Pathologie coronaire après transplantation cardiaque.

Graft coronary disease is a frequent and devastating complication with rapid development after heart transplantation. Until now, non-invasive and invasive methods have proved to be insensitive in the prediction and detection of the early stages of this disease. Conventional arteriography is considered as the only reliable means of diagnosis, but it remains insensitive in the accurate evaluation of the severity of graft coronary lesions (obliterative, diffuse and distal lesions). Precise quantitation of coronary lumen changes may be a sensitive method for the accurate evaluation of graft coronary disease and help in the understanding of the natural course of development of this disease. The pathogenesis of the disease is still unclear. It is possible that graft coronary disease is a consequence of non-treated low-grade cellular rejections. In most cases, retransplantation must be considered as the unique solution.

[Multiple cholesterol embolism mimicking periarteritis nodosa]. / Embolies multiples de cholestérol simulant une périartérite noueuse.

Ten men aged 56 to 84 were hospitalized with a diagnosis of periarteritis nodosa, whereas they had multiple cholesterol embolism. The diagnosis was corrected post mortem in the first 3 patients and subsequently in live patients. The particularly misleading clinical manifestations were neurological (polyneuritis in 5 cases, mononeuritis in 1, central nervous system disorders in 3), pulmonary (alveolar haemorrhage in 2 cases, respiratory failure of unknown mechanism in 4) and pericardial (2 cases). Five patients had eosinophilia (more than 500 eosinophils/mm3). The elements that led to the correct diagnosis were the presence of vascular risk factors in all 10 patients (but hyperlipidaemia in only one), severe complications of the atheromatous disease in all cases, a precipitating or aggravating factor in 8 patients (anticoagulant therapy in 7, arteriography in 6) and the finding of purple or necrotic toes (6 cases). Histological (5 cases) and/or ophthalmological (2 cases) evidence was obtained in only 6 patients. Seven patients died 1 to 3 years after the onset of the disorders. Studies on low-density lipoprotein metabolism are in progress to determine the mechanism of clinical manifestations unexplainable by embolism.

[The value of biopsy of the accessory labial salivary glands for the diagnosis of amylosis]. / Intérêt de la biopsie des glandes salivaires accessoires labiales pour le diagnostic d'amylose.

Diagnosis of amyloidosis depends on the demonstration of amyloid deposits in biopsies using specific stains. Recently, in addition to classical biopsies (kidney, liver, gum, skin, rectal mucosa), labial salivary gland biopsy has been recommended as safe diagnostic method. In our recruitment, it allowed the fortuitous discovery of amyloidosis in three patients suffering from rheumatoid polyarthritis or spondylarthritis. In five other patients (2 cases of familial amyloidosis, 1 dysglobulinemia, 2 primary cardiac amyloidosis), biopsy was performed for systematic search of amyloidosis. In five of these eight cases, a sicca syndrome was associated with the salivary deposits. These deposits were stained with congo red viewed in polarized light and with T thioflavine. Besides, Wright's method allowed to know the AL or AA type of amyloidosis and thus to guide the treatment. On the whole, labial salivary gland biopsy is a highly sensitive method for diagnosis of primary and secondary amyloidosis.

[A malignant salivary lymphoepithelial lesion with a poorly differentiated epidermoid carcinoma. Apropos a case]. / Lésion lymphoépithéliale maligne salivaire avec carcinome épidermoïde peu différencié. A propos d'une observation.

A case of malignant lymphoepithelial lesion of the submandibular gland is reported in a 71-years-old woman. The carcinoma showed a poorly differentiated pattern and its epidermoid nature was obvious after immunohistochemistry and electron microscopy. The epithelial nests were lying in an abundant lymphoid stroma consisting of small lymphocytes. A recurrence occurred ten months after surgical treatment. This observation is compared with those already related in the literature.

[Coronary disease in patients after heart transplantation]. / Coronaropathie du transplanté.

The thinking about coronary artery disease in heart transplant patients has changed recently. This term includes not only the traditional accelerated delayed atherosclerosis of the transplant, but also proximal inflammatory vascularitis associated with severe acute rejection and distal vascularitis, which is independent of interstitial rejection. This term is also used to describe purely atheromatous lesions of the large coronary vessels which usually occur in high risk patients. The morphology, significance and prognosis of these various vascular lesions are discussed on the basis of material obtained from biopsies, transplantectomies and autopsies in the cardiovascular surgery service of the la Pitié Hospital.

An immunohistochemical study of the distribution of lysozyme, lactoferrin, alpha 1-antitrypsin and alpha 1-antichymotrypsin in salivary adenoid cystic carcinoma.

The immunohistochemical expression of lysozyme (Ly), lactoferrin (La), alpha 1-antitrypsin (alpha 1-AT), and alpha 1-antichymotrypsin (alpha 1-Ach) was described, and their distributions were compared to each other in 28 cases of adenoid cystic carcinoma (ACC) of the salivary glands. ACC materials were obtained from the parotid gland (7), the submandibular gland (4), the sublingual gland (8), and minor oral salivary glands (9). Histopathologically, ACC was classified into cribriform (14), tubular (3), and basaloid or solid patterns (11). Positive staining for Ly was found in 1 case of solid ACC in the sublingual gland; La was found in 4 cases (2 cribriform, 1 tubular, 1 basaloid) in the sublinguals (3) and parotid glands (1); alpha 1-AT was found in 6 cases and alpha 1-Ach in 17 cases. The immunohistochemical localization of Ly and La was usually confined to luminal tumor cells of tubulo-ductal structures, irrespective of the pathologic types. Positive staining for alpha 1-AT and alpha 1-Ach appeared in tumor cells of cribriform, tubular and solid ACC. Tumor cells with positive La staining coincided with a positive reaction to alpha 1-AT and alpha 1-Ach, and tumor cells with alpha 1-AT positive deposition were also positive for alpha 1-Ach. The contents of pseudocysts in the cribriform pattern showed a positive reaction to La, alpha 1-AT, and alpha 1-Ach. Of the 28 cases of ACC, positive expressions for Ly, La, alpha 1-AT and alpha 1-Ach were found with a high frequency of alpha 1-Ach staining (17 in 28 cases were positive). In sublingual ACC (8), 7 cases were positive for immunohistochemical reactions. Co-expression or simultaneous expression for Ly, La, alpha 1-AT, and alpha 1-Ach in ACC suggest that tumor cells are protected from proteolysis or degradation.

Acinic cell carcinomas of salivary glands histoprognosis. Value of NORs stained with AgNOR technique and examined with semi-automatic image analysis.

Twenty one patients surgically treated for acinar cell salivary gland carcinomas were studied retrospectively. Four cases were excluded from the results because of an inadequate follow-up time (less than 5 years). The rest was classified into two groups: eleven patients with a favorable outcome were still alive without any recurrence after a mean follow-up period greater than 10 years; in six other patients, recurrences and metastases occurred followed by death in four cases. Previously for such tumors a high degree of histological undifferentiation usually gave rise to a poor prognosis, although some exceptions were noted. Thus, in an attempt to find a more reliable prognosis criterion, we evaluated by semi-quantitative image analysis nucleolar organizer regions by means of AgNOR count and mean area in all these cases. Our results significantly correlated with the clinical prognosis: a high count (more than 3) and a small mean area were always found in tumors with an unfavorable outcome whereas lower counts and larger mean areas were found in tumors with a favourable clinical course. Thus, this method showed promise as establishing the prognosis of acinar cell tumours.

Origin of malignant centrofacial granulomas: surface markers and gene rearrangement of malignant cells.

Malignant centrofacial granuloma (MCFG) is a clinical entity characterized by a relentless ulceration of the upper airway involving the nose, palate, and face, without any demonstrable etiology. The origin of 11 cases were analyzed with the help of cell-surface immunostaining in all and with T-cell receptor gene (TCR) rearrangement in 3. The results show that most of the cases of MCFG are in fact T-cell lymphomas with cell-surface antigens (CD2, CD7, CD3) consistent with either early or mature T lymphocytes. However, some cases exhibit B-lymphoid (CD19, CD20) or histiomonocytic (CD13, CD14) lineage-specific markers. In conclusion, despite its remarkable clinical unity, MCFG is a heterogeneous group of neoplastic diseases, most but not all of which may be classified as T-cell lymphoma.

Degenerative aortic regurgitation.

In view of the growing incidence of degenerative origin among the aetiologies of aortic regurgitation, we felt it interesting to report our experience of the surgical treatment of such patients. Out of 313 patients operated on for isolated chronic aortic regurgitation between 1974 and 1989, 102 (32.6%) had aortic regurgitation of degenerative origin and form the basis of this study. The patients were divided into group I (n = 48) without aneurysm of the ascending aorta and group II (54 patients) with anulo-aortic ectasia. The first group was further subdivided according to the diameter of the ascending aorta: in group Ia (23 patients) the aorta was normal with a root diameter of less than 40 mm, while in group Ib (25 patients) the aorta was enlarged (root diameter between 40 and 54 mm). There was no statistically significant difference between the pre-operative and operative parameters of patients in groups Ia and Ib. All underwent aortic valve replacement without associated surgery of the ascending aorta. During a mean follow-up of 58 months, five patients from group Ib had aortic dissection and in another three, an ascending aorta aneurysm developed. Reoperation was performed in six cases. No such complications occurred in group Ia. At 6 years, the proportion of patients free from complications related to the ascending aorta was 100% in group Ia vs 63 +/- 6% in group Ib (P less than 0.05). In group II, all the patients underwent aortic valve replacement. The modalities of repair of the ascending aorta differed with time.(ABSTRACT TRUNCATED AT 250 WORDS)

Aetiology of surgically treated mitral regurgitation.

The aetiologies of mitral regurgitation were reviewed in 336 patients operated on between 1970 and 1990. The relative frequency of rheumatic fever has decreased and the most common cause is now primary mitral valve prolapse (PMVP) which accounts for 60% of all patients. The increasing age of the surgical population seems to be one of the main factors of this change. Conservative surgical repair according to Carpentier's technique is now performed in 60% of all patients with mitral regurgitation and 80% of cases of PMVP. The aetiology of PMVP is still under discussion. Surgical description seems to indicate the existence of two different morphological aspects with significantly different rates and localizations of chordae ruptures, but our study failed to demonstrate any other anatomical, clinical or histological differences between the two groups.

[Severe pulmonary artery involvement of Takayasu arteritis. 3 cases and review of the literature]. / Formes artérielles pulmonaires graves de l'artérite de Takayasu. trois observations et revue de la littérature.

Three cases of pulmonary arterial disease were identified out of a cohort of 75 cases of Takayasu's disease. In the first case, pulmonary hypertension considered to be idiopathic caused massive haemoptysis and the death of a 48 year old Spanish woman; autopsy revealed characteristic stenoses of the common carotid arteries. In the second case, haemoptysis led to pneumonectomy in a 23 year old West Indian woman with a diagnosis of agenesis of the right pulmonary artery. Disease of the supraaortic vessels occurred several months later and a retrospective diagnosis of Takayasu's disease of the pulmonary artery was made. In the third case, systematic pulmonary angiography in a 41 year old French woman referred for Takayasu's disease of the supraaortic arteries showed severe stenosis of the right pulmonary artery. She developed severe haemoptysis four years later which led to an attempted balloon angioplasty of the right pulmonary artery and embolisation of a branch of the right coronary artery thought to be the cause of the haemoptysis. This was complicated by posterior wall myocardial infarction but the haemoptysis did not recur thereafter. These three cases and a review of the literature show that the pulmonary lesions of Takayasu's disease occur mostly on the main right pulmonary artery but they may be more distal and involve the pulmonary arterioles. A coronaro-broncho-pulmonary collateral circulation may develop distal to the stenosed segments. The main complications are pulmonary hypertension and massive haemoptysis. Surgery is possible in cases of stenosis of the right main pulmonary artery. The potential role of endoluminal angioplasty is discussed.

[Dystrophic aortic insufficiency: influence of dilatation of the ascending aorta on secondary outcome]. / Insuffisance aortique dystrophique: influence de la dilatation de l'aorte ascendante sur l'évolution secondaire.

This cooperative study recensed 89 cases of patients operated for aortic regurgitation in whom the dystrophic process was confirmed on anatomical (thin, translucent valves without symphysis, multilation or sclerosis) and histological criteria (mucinous infiltration). They included 81 men (91%) and the average age was 52 +/- 14 years. The valvular degeneration was part of a generalised dystrophy of elastic tissue in 8 patients (6 "formes frustes" of Marfan's syndrome, 2 Lobstein's syndrome). The patients were divided into 2 groups according to the diameter of the ascending aorta measured by echocardiography and/or aortography. In Group 1 (n = 40), the aorta was not dilated (diameter less than 40 mm) whereas in Group 2 (n = 49), the diameter of the ascending aorta was dilated (40-55 mm) but not aneurysmal (loss of parallelism of the aortic walls). The two groups were comparable before surgery with respect to age, sex, functional class, degree of left ventricular dilatation, left ventricular ejection fraction and presence of associated coronary disease). There were no differences in the operative parameters but aortic parietal biopsy (n = 35) revealed clear signs of cystic medianecrosis more often in Group 2 than in Group 1 (14/25 versus 1/10, p less than 0.05). No operative procedure was performed on the ascending aorta during aortic valve replacement. One operative death occurred in each group. After an average follow-up of 4 years, there was a higher mortality in Group 2: the actuarial 7 year survival rate being 74% in Group 1 and 54% in Group 2. This was explained by a higher incidence of ascending aortic complications. Only 1 patient in Group 1 developed an aneurysm of the ascending aorta requiring reoperation compared to 14 patients in Group 2, 8 of whom were reoperated (p less than 0.01). These results suggest that non-aneurysmal dilatation of the ascending aorta in patients with dystrophic aortic regurgitation is a poor prognostic factor because of the high incidence of secondary aortic parietal complications (aneurysm, dissection).

[Myocardial involvement in 2 women, carriers of Duchenne de Boulogne muscular dystrophy]. / L'atteinte myocardique chez deux femmes transmettrices de la maladie de Duchenne de Boulogne.

Duchenne muscular dystrophy is the commonest genetic muscular disease. The prognosis, which depends on cardiac involvement, is poor. In boys, this takes the form of a hypokinetic cardiomyopathy particularly affecting the postero-lateral wall of the left ventricle which then dilates. The recent identification of the gene which transmits the disease on the X chromosome and of the coded protein, dystrophin, has improved our understanding of the disease. We report two cases of isolated cardiac involvement in two female carriers of the disease, classically thought to be unaffected. They presented with apparently idiopathic dilated cardiomyopathies. These cases show that Duchenne muscular dystrophy is an original example of a genetically determined diffuse muscular disease with cardiac involvement.