Fibromuscular dysplasia of the renal artery responsible for renovascular hypertension: a histological presentation based on a series of 102 patients.

BACKGROUND: Fibromuscular dysplasia (FMD) is a rare non-atherosclerotic and non-inflammatory disease in the arterial system. The purpose of the study was a retrospective analysis of FMD in the renal artery. PATIENTS AND METHODS: A total number of 102 patients (mean age: 36.9 years) who suffered from renovascular hypertension underwent a surgical therapy. The operative specimens of the renal arteries were analysed with the lightmicroscop using histological and immunohistochemical methods. RESULTS: 101 patients (99.02%) presented a medial FMD (extensive-medial subtype in 56 patients, 54.9%, subadventitial subtype in 29 patients, 28.4% and combined subtype in 16 patients, 15.7%). In 1 patient (0.98%) an adventitial FMD was found. We observed the following complications: true and dissecting aneurysms (75 patients, 74.5%), arterio-venous fistulae (2 patients, 1.96%) and chronic thrombosis (10 patients, 9.8%). CONCLUSIONS: With the progress in angioplasty, not all patients suffering from FMD undergo a primary surgical therapy and therefore this lesion is less seen in the daily work of the histopathologist.

Plasmodium chabaudi chabaudi chronic malaria and pathologies of the urogenital tract in male and female BALB/c mice.

BALB/c mice of both sexes were infected with a non-virulent strain of Plasmodium chabaudi chabaudi and any pathologies occurring in the urogenital tract and its accessory glands were investigated. Organs and tracts were removed from infected and control mice at 15, 40 and 100 days post-injection, weighed and processed for macroscopical and histological analyses. The relative weights of preputial, clitoral glands and testes were modified in infected mice during the 40 days following infection. The preputial glands show a marked hypotrophy at 15 days post-infection. The bladders of half of the infected female mice and a few infected male mice displayed a conspicuous haemoglobinuria and frequent interstitial cystitis that worsened throughout the experiment. Also, several chronic inflammatory reactions were detected in the prostates, preputial and clitoral glands up to 100 days post-infection. A probable cause of such a divergence in the characteristics of the infection and in the nature of the pathologies identified in infected male and female mice is the interaction between the infection and the immune and endocrine systems of the host. The cause of the pathologies and their consequences on the host condition are discussed.

[Necrotic eosinophilic angiitis with ileal perforation and peritonitis secondary to abdominal angiostrongyliasis]. / Angéite nécrosante à éosinophiles avec perforation iléale et péritonite secondaire à une angiostrongylose abdominale.

INTRODUCTION: Abdominal angiostrongyliasis caused by the filiform nematode Angiostrongylus costaricensis, is an endemic disease in Central and South America. A case of necrotic eosinophilic angeitis with ileum perforation and peritonitis due to abdominal angiostrongyliasis is reported. OBSERVATION: A 32 year-old man, living in a Paris suburb, underwent segmentary resection of the ileum with end to end anastomosis for perforation with generalized peritonitis. The anatomopathological examination revealed eosinophilic necrotic lesions with thrombosis on the borders of the ileum perforation. The discovery of a section of A. costaricensis in the lumen of a nearby muscular artery initiated an epidemiological survey, revealing that the patient had visited French Guyana 2 months earlier. DISCUSSION: Angiostrongylus costaricensis is a nematode parisiting certain forest rodents that become its permanent host. The intermediate hosts are earth molluscs or slugs of the same family. Humans are accidentally infected following ingestion of vegetables infested with L3 larvae or slugs carrying the disease. The clinical symptomatology is unspecific: prolonged fever, anorexia, and right iliac fossa pain with eosinophilia of the blood. Often benign, the progression of abdominal angiostrongylosis is punctuated by complications: occlusive syndrome, generalised peritonitis due to intestinal perforation and mass syndrome. Hemorrhage, infarct, pseudo-tumoural fibrosis and ulcers represent the surgical or macroscopic rearrangements. In the tissue, 4 lesions characterize abdominal angiostrongylosis: eosinophilic necrotic angeitis, foreign body granulomas, eosinophilia in the digestive wall, and the presence of A. costaricensis in the lumen of the vessels. There is presently no medical treatment and surgery is the only therapeutic option.

Primary squamous cell carcinoma of the endometrium: case history, pathologic findings, and discussion.

Primary squamous cell carcinoma of the endometrium (PSCCE) is an exceedingly rare tumor. Rarely are cytological criteria discussed. We report our experience in the cytological diagnosis of a case. A postmenopausal, 64-yr-old woman suffered from pyometria. An endometrial Pap smear displayed some malignant squamous cells. Curettage of the cervix and the uterine cavity only recovered some fragments of atypical squamous epithelium whose origin could not be precisely identified. A hysterectomy with bilateral adnexectomy was decided upon. Pathological study evidenced a primary squamous cell carcinoma in the uterine cavity while the cervix was tumor-free and the lymph nodes were devoid of metastases (pT1, pN0, pM0). The patient died 46 mo PO with multiple pulmonary and renal metastases. The histological feature of PSCCE is identical to that of any tumor of a similar nature, whatever the site, especially the cervix. Confirmation of the primary endometrial nature is only possible on the hysterectomy specimen.

[Visceral lesions in mammals and birds exposed to agents of human cercarial dermatitis]. / Lésions viscérales de mammifères et oiseaux, exposés aux agents de dermatite cercarienne humaine.

Over the past few years, the cercarial dermatitis has become a new problem of public health, obviously linked to the prolonged stay of migrant birds on our territory. This is a skin affection characterized by pruriginous and papulous eruptions caused by penetration of avian bilharzian larvae under the skin. These larvae are emitted by molluscs, mostly limneids. In aquatic birds, especially in migrating Anatidae, these larvae reach the visceral vessels, become adults in a few weeks, lay eggs, then degenerate. Corresponding miracidia contaminate new limneids. Since 1993, the total number of annual cases of cercarial dermatitis has increased from only ten to thousands in France and the affection rages in pools where limneids, migrating water birds and swimmers gather together. Fever, respiratory and/or digestive allergic symptoms appear in some cases. This clinical pattern has encouraged to undertake research on the future of these bilharzian larvae in mammals organism. A preliminary investigation on a rodent model showed that, once the skin barrier had been crossed, the schistosomulae migrated into the lungs of the host; there they survived a week and induced lesions. The goal of this study is to carry on the research, over a longer period, after exposure to cercariae, simultaneously in mammals and birds, with two species of bilharziae present in France. The selected models are the gerbil Meriones unguiculatus for mammals, and the ducks Anas platyrhynchos and Cairina moschata, for birds. 5 M. unguiculatus and 2 A. platyrhynchos were exposed to cercariae emitted by Radix auricularia; 2 gerbils and 5 A. platyryhnchos to larvae of R. peregra, 3 C. moschata to larvae emitted by two species of molluscs: 70-230 from R. auricularia and 330-585 from R. peregra. 5 gerbils died between 2 and 5 weeks after exposure, 2 gerbils sacrificed early, served as control animals for skin manifestations. Eight ducks were sacrificed between 2 and 4 weeks after; the 2 last ones, exposed several times, were sacrificed respectively 7 and 13 weeks after the first exposure. Visceral and skin samples were submitted to histological study. The control gerbils developed skin dermatitis. In ducks, R. auricularia was the vector of Trichobilharzia franki, whose selective dwelling site was the mesentery; R. peregra was the vector of an indeterminate species found in the lungs and nose. This species is called Bilharzia sp. in this study. The ducks, exposed to two kinds of larvae, displayed worms in these two main locations. In gerbils, T. franki induced lesions in the mesenteric veins and the peritoneum. Bilharzia sp. gave rise to lesions in lung arteries, pleura and liver veins. Vascular changes encompassed endothelitis and lymphocytic vasculitis, while serosa displayed mesothelial hyperplasia. The types of lesions observed in gerbils were noticed in ducks, and, according to the species of bilharzia, in the homologous viscera. Additional foreign body granulomas centred on worm's debris or their eggs, and vascular thromboses were present, too. In addition, ducks displayed lesions involving several other viscera including the intestine, the kidneys and the peripheral nerves. These changes were multiple and diffuse in C. moschata exposed to two species of bilharziae. They were observed mainly in mesenteric and intestinal vessels, pulmonary arteries and hepatic veins. In gerbils, the lesions persisted 2 to 5 weeks after exposure, but worms were not identified in the neighbouring tissues near the damaged vessels. In ducks, lesions were important between 2 and 7 weeks after exposure; they co-existed with live or dead worms, sometimes paired, with or without eggs. The hepatic lesions regressed 13 weeks, after exposure. In mammals and birds, young worms could migrate into the same visceral vessels, and stimulating formation of persistent lesions. In individuals exposed to the same cercariae, development of similar lesions would be probable.

Arterial calcifications: morphological aspects and their pathological implications.

Morphological aspects of calcifications are identical whatever their site in the arterial layers: fine granular deposits, plates, rings. Occurrence of complications: fibrosis with foreign body type granuloma, thrombosis, and embolism mainly depend on the site and the amount of calcification. Clinicians should be aware of these complications when performing angioplasty.

Molineus torulosus (Nematoda, Trichostrongylina, Molineoidea) a parasite of Neotropical primates: new morphological and histological data.

Molineus torulosus (Molin, 1861) parasite of Cebus spp. from South America is redescribed in Cebus apella and C. olivecaeus (new host) from French Guyana with emphasis on the synlophe. During the maturation process, the larvae dwelt in the cysts carved alongside the external part of the small intestine. The turn-out of the mature worms and the laid eggs depended on the tissular organisation of cyst walls as the inflammatory process waned and fibrosis progressed to seal the cystic lumen. Adult worms entwine themselves in the cysts, live there permanently as their presence has never been evidenced in the intestinal lumen. They copulated, laid eggs, degenerated and died once entrapped by the fibrotic process. Laid eggs released in the intestinal lumen through a narrow channel ensured the continuation of the developmental cycle. However, erratic migration was possible via the vascular channels surrounding the cysts.

[Multiple jejunal strictures caused by gastric heterotopia]. / Sténoses étagées du jéjunum par hétérotopie gastrique.

A case of gastric heterotopia was discovered incidentally on a jejunal resection specimen in a 42-year-old patient operated for Koenig's syndrome present for 10 years. This anomaly was responsible for seven chronic ulcers with strictures at multiple levels. Gastric heterotopia, especially in the jejunum, is a rare anomaly, except in intestinal duplications and Meckel's diverticulum. The various complications are a direct result of the activity of the gastric glands: hemorrhage, Helicobacter pylori enteritis, perforation, chronic ulcer and obstructive syndrome; malignant adenocarcinomatous degeneration has also been reported.

[Intimal muscular fibrodysplasia responsible for an ischemic gastric ulcer in a patient with a von Recklinghausen's disease: a case report]. / Fibrodysplasie musculaire artérielle intimale responsable d'un ulcère gastrique ischémique chez une patiente ayant une maladie de von Recklinghausen: un cas.

A 78 year-old woman, suffering from a von Recklinghausen's disease sought medical assistance for hematemesis with anemia. This patient had previously experienced an amputation of the right arm for gangrene. Gastric fibroscopy unveiled a deep chronic ulcus developed in the antrum, highly suspect of malignancy. Multiple biopsies of the ulcer showed mainly interstitial gastritis. The persistence of the hematemesis imposed a subtotal gastrectomy. Pathological examination of the operative specimen evidenced an ischemic ulcer caused by arterial intimal muscular fibrodysplasia with associated neurofibromatosis in the neighboring sub-mucosal layer. This case report highlights the frequent association of phacomatosis especially von Recklinghausen's disease, with vascular lesions whose clinical expression mainly depends on the involved vascular area.

[Horton's disease and aortic aneurysm: coincidence or causality? 5 cases]. / Maladie de Horton et anévrisme aortique : coïncidence ou association? Cinq observations.

Five inflammatory aortopathies were disclosed 3 to 16 years after inaugural giant cell arteritis. Three patients were symptomatic: one aneurysm of the subrenal abdominal aorta discovered at work-up for an inferior arteriopathy, one thoraco-abdominal aneurysm with a "fissuration" episode, one calcified thoraco-abdominal aortopathy suggesting dissection. In these three cases, there was a severe inflammatory syndrome with asthenia, fever, elevated erythrocyte sedimentation rate and a large inflammatory crown around the aortopathy. In the two asymptomatic cases, the diagnosis was made during the follow-up of Horton's disease, in one patient with active disease, the other late after the initial episode. Two aneurysms required surgical cure, with resection-prosthesis of the thoraco-abdominal aneurysm and revascularization of the digestive and renal arteries. In the 4 active cases, corticosteroid therapy cured the inflammatory process both on the basis of laboratory results and the involution of the periaortic crown and, in one case, the total regression of ureteral compression causing pyeloureteral dilatation on the left. The diagnosis of giant cell arteritis was confirmed histologically in the two operated cases. Extra-cervical localizations of aortic aneurysm of dissection in patients with giant cell arteritis is not a fortuitous coincidence but an association as demonstrated by the Mayo Clinic epidemiology. On the basis of these reported cases and data in the literature, the practical conclusions are: in case of aorta involvement, particularly with inflammation in subjects under 50, giant cell arteritis should be entertained as a possible diagnosis; in patients with giant cell arteritis, follow-up should include yearly thoracic radiograms to search for thoracic aorta involvement and Doppler and ultrasound explorations to identify any abdomino-iliac lesions. This protocol is required to avoid the life-threatening complications of dissection or rupture of an aortic aneurysm.

Drastic reduction of a filarial infection in eosinophilic interleukin-5 transgenic mice.

In order to establish the role of eosinophils in destroying parasites, transgenic mice have been used in experimental helminthiases but not in filariasis. Litomosoides sigmodontis offers a good opportunity for this study because it is the only filarial species that completes its life cycle in mice. Its development was compared in transgenic CBA/Ca mice overexpressing interleukin-5 (IL-5) and in wild-type mice following subcutaneous inoculation of 40 infective larvae. An acceleration of larval growth was observed in the IL-5 transgenic mice. However, the recovery rate of adult worms was considerably reduced in these mice, as evidenced 2 months postinoculation (p.i.). The reduction occurs between days 10 and 30 p.i. in the coelomic cavities. As early as day 10, spherical aggregates of eosinophils and macrophages are seen attached on live developing larvae (always similarly localized on the worm) in both wild-type and transgenic mice. However, on day 60 p.i., granulomas were found in the transgenic mice only, probably because of the higher density of eosinophils. Furthermore, on day 30 p.i., young filariae are seen trapped in granulomas, some of them surrounded by Splendore-Hoeppli deposits, which illustrates the release of the major basic protein by eosinophils. The high protection rate obtained (65%) is similar to that observed previously in BALB/c mice following vaccination with irradiated larvae. Both protocols have a common factor, the high production of IL-5 and eosinophilia. However, protection occurs later in primary infected transgenic mice because specific antibodies are not yet present at the time of challenge.

Parasitology and immunology of mice vaccinated with irradiated Litomosoides sigmodontis larvae.

This study was performed with Litomosoides sigmodontis, the only filarial species which can develop from the infective larvae to the patent phase in immunocompetent laboratory BALB/c mice. Parasitological features and immune responses were analysed up to 3 months before and after challenge inoculation, by comparing 4 groups of mice: vaccinated challenged, challenged only, vaccinated only, and naive mice. Male larvae were very susceptible to irradiation and only female irradiated larvae survived in vivo. Protection, assessed by a lower recovery rate, was confirmed and was established within the first 2 days of challenge. This early reduction of the recovery rate in vaccinated challenged mice was determined by their immune status prior to the challenge inoculation. This was characterized by high specific IgM and IgG subclass (IgG1, IgG2a and IgG3) levels, high specific IL-5 secretion from spleen cells in vitro and a high density of eosinophils in the subcutaneous connective tissue. Six h after the challenge inoculation, most tissue eosinophils were degranulated in vaccinated challenged mice. Thus, in the protocol of vaccination described, protection appeared mainly to result from the stimulation of a Th2 type response and eosinophils seemed to be the main effectors for the increased killing of infective larvae in vaccinated challenged mice. Two months after challenge inoculation, the percentage of microfilaraemic mice was lower in vaccinated challenged mice as a consequence of this overall reduction in the worm load. In both vaccinated challenged and challenged only groups, the in vitro splenocyte proliferative capacity was reduced in microfilaraemic mice.

[Pathology of gestational trophoblastic tumors]. / Pathologie des tumeurs trophoblastiques gestationnelles.

Gestational trophoblastic tumours result from an abnormal proliferation of different types of trophoblasts. The morphological pattern, together with the immunohistochemical aspect, the cytogenetic data and the clinical profile, helps identify each pathological entity. Hydatiform moles represent malformed placentas caused by genetic aberrations of the villous trophoblast. A complete hydatiform mole displays an hydropic degeneration of all the chorionic villi with a more or less marked proliferation of trophoblasts. A partial hydatiform mole is made up of molar vesicles interspersed with normal chorionic villi. In an invasive hydatiform mole or chorioma destruens, molar vesicles penetrate the myometrium giving rise to a mass distorting the uterine wall. A choriocarcinoma is a malignant proliferation of atypical villous trophoblasts without villi formation. Necrosis, haemorrhage, vascular invasion and distant metastases strongly compromise its outcome. A trophoblastic implantation site tumor, clearly less frequent, results from a proliferation of extravillous trophoblasts, particular for their secretion of human placental lactogen hormone (hPL). This tumour, exceptionally malignant, should be differentiated from the exaggerated placental site and its variants. Except for the placental site trophoblastic tumour, and whatever the outcome (benign or malignant), all gestational trophoblastic tumours secrete the beta-subunit of the chorionic gonadotropic hormone (beta-hCG) more or less abundantly. The serum or urinary level of this unit is proportional to the tumour volume and represents a fundamental basis for the follow-up of these tumours. Multidisciplinary care of high-risk cases allows us to cure the disease, and helps the patient recover her reproductive uterine function.

[Arterial fibromuscular dysplasia unveiled by a traumatic false aneurysm: a case report]. / Fibroplastie musculaire artérielle révélée par un faux anévrisme traumatique: un cas.

An inadvertent puncture of the brachio-cubital artery during an intravenous perfusion provoked a false aneurysm in a 59-year-old woman. The angiographic check-up disclosed a "pile of dishes" pattern on the humeral artery. Pathological examination of the resected false aneurysm confirmed the presence of an extensive medial fibromuscular dysplasia distant from the rupture area. This clinical case underlines the importance of a systematic study of all traumatic vascular specimens in order to find any possible occult disease responsible for the weakening of the vessel (J Mal Vasc 1999; 24: 377-380).

Takayasu's arteritis and fibromuscular dysplasia as causes of acquired atypical coarctation of the aorta: retrospective analysis of seven cases.

Coarctation of the aorta usually occurs in the aortic isthmus: however, 2% of all coarctation is localized at atypical sites such as the aortic arch and the thoracic and/or abdominal aorta. Causal therapy involves vascular surgery, and during the procedure, biopsies of the involved vessels should be taken to establish the etiology. This study involved the retrospective analysis of clinical and histopathological findings for seven patients who underwent vascular surgical procedures (age range, 10-37 years; male/female ratio 3:4). Histopathological analysis of specimens revealed two pathological processes taking place with different localization in the aortic wall as the cause of the atypical coarctation of the aorta: (1) fibrosis/scarification in the tunica media and adventitia compatible with chronic lesions of Takayasu's arteritis (four cases); (2) fibromuscular dysplasia in the tunica media (three cases).

Bladder tissue biopsies in spinal cord injured patients: histopathologic aspects of 61 cases.

It was the aim of this histopathologic study to examine and compare results of bladder tissue biopsies in spinal cord injured (SCI) patients. The study group consisted of 61 SCI patients who received treatment at the Swiss Paraplegic Centre in Nottwil, near Lucerne, Switzerland. The mean age of the study group was 41 years (range, 17-73) and the mean duration of spinal cord lesion was 10 years and 4 months (range, 5 months to 44 years). The male:female ratio was 57:4. Bladder infections had occurred in each patient one to six times per year (median, 3.2). All samples were taken from the trigone of the bladder during endoscopic urologic procedures with a flexible cystoscope. Histopathologic analysis showed abnormal alterations of bladder tissue in 56 SCI patients (91.8%). Forty-six SCI patients (75.4%) had a chronic type and 10 SCI patients (16.4%) a subacute type of inflammation. Normal bladder tissue was found in five cases (8.2%). Further observation revealed the presence of fibrosis (34.4%), edema (9.8%), and lymphoid hyperplasia (6.6%). A t-test for independent samples showed a lack of significant correlation between the number of clinical bladder infections per year, the duration of injury, the neurologic level of the spinal cord lesion, and histopathologic types of infections.

[Guidelines for the evaluation of internal quality control of smears for screening of uterine cancer in France in the structures of Pathologic Anatomy and Cytology. French Association for Quality Assurance in Pathologic Anatomy and Cytology (AFAQAP)--Commission for cervical smears]. / Recommandations pour l'évaluation de qualité interne des frottis de dépistage du cancer du col utérin en France dans les structures d'Anatomie et Cytologie Pathologiques. Association Française pour l'Assurance de Qualité en Anatomie et Cytologie Pathologiques (AFAQAP)-Commission Frottis du col utérin.

A national organized mass-screening effective programme is the only way to reduce the risk of cervical cancer, if properly organized and correlated with a system of Quality Assurance. Since 1900, an Association for Quality Assurance was created by the French pathologists, named "AFAQAP". These pathologists thus demonstrated their interest in this kind of action that should be effective if women and clinicians are also implied. The pathologists have concluded the first part of their programme with these French guidelines for internal quality control of pap smears.