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Introduction: The frequency of amyloidosis is not well known in France. We compiled a register of amyloidosis diagnosed from 1995 to 1999 in the University Hospital of Rennes., Patients and Methods: This retrospective study was performed between 01 January 1995 and 31 december 1999. Diagnosis was assessed on positivity of red Congo by anatomopathology. Immunohistochemistry allowed the definition the type of amyloidosis. Clinical data, staging and outcome of patients were analysed., Results: Forty-three amyloidosis were diagnosed (27 women, 16 men) with an incidence of 8,6 new cases per year. Mean age was 63.7 years. Five diagnosis were realised in 1995, six in 1996, six in 1997, 12 in 1998, 14 in 1999. Twenty amyloidosis were AL type (46.5%), seven AA (16.3%), 1 beta2 microglobulin type, 15 (35%) remained of undetermined type. Thirty-three amyloidosis (77%) were systemic, 10 were localized to one organ (23%). When diagnosis was made, biopsies concerned affected organs in 86% of the cases, accessory sites (labial salivary glands, bone marrow) in only 14% of the cases. Twenty-five patients died (58%). Two deaths were treatment-related, 16 to amyloidois, seven patients died of another complaint., Conclusion: Increased incidence of amyloidosis needs to be confirmed. We emphasize the importance of immunohistochemical typing on frozen samples, the value of accessory biopsies and the need for complete extension staging.

Primary small cell carcinoma of the urinary bladder is an uncommon tumor, compared to the frequency of urothelial tumors. Fifty percent of cases are combined with a non endocrine carcinomatous component. We report six new cases of this tumor, three of pure, and three associated with an urothelial carcinoma. Diagnosis is easy established by the immunohistochemical study which show the neuro-endocrine differentiation of these aggressive tumors. Pathologist needs to look for a neuro-endocrine part in all bladder cancer, as its presence modify the treatment. Chemotherapy is used in these cancers, due to their high metastatic power. Places of radical surgery and radiotherapy need to be specified.

We report a case of nested cell carcinoma, an uncommon transitional tumor. These tumors are composed of regular cuboidal transitional cells forming small nests with minimal cytologic atypia. Despite the benign course, this tumor resembling proliferation of Brunn's nests or inverted papilloma, must be considered as an aggressive transitional tumor. Thus, morphologic criteria are needed to make the diagnosis. Because of its aggressive behaviour, the surgical therapy depends on the tumor's infiltration.

In the 80s it was established that atherosclerotic renal artery stenosis (ARAS) is a leading cause of renal insufficiency and that this condition ranks among the rare etiologies of chronic renal failure amenable to improvement or stabilization particularly in the white. Nephroangiosclerosis (NAS) is an increasing cause of ESRD in the western countries, especially in blacks. Epidemiological data on the vascular nephropathies leading to ESRD are still rare. In this study, we compare annual incidence of ESRD due to ARAS and NAS during two five-year periods: period A = 1982-1986, period B = 1992-1996. The region of the survey comprised 410,664 inhabitants (99.6% of Caucasians), of whom 100,230 were aged over 60 years. Diagnosis of ARAS required arteriography and that of NAS a renal biopsy. Undetermined vascular nephropathy was diagnosed when ESRD patients had had previously no arteriography or no histological examination. Major results were as follow (A vs B, incidence = n/million inhabitants): 1) Increasing incidence of ESRD due to all causes: 76 vs 95 per million, mean age at ESRD 56 vs 62 yrs, percentage of patients over 65 yrs 28 to 59% (p < 0.001). 2) Increasing incidence of ESRD due to vascular nephropathies: 5.5 vs 27.5 per million (p < 0.0001) in general population and 22 vs 110 per million (p < 0.0001) in population aged over 60 years, mean age at ESRD 68 vs 73 yrs. 3) Increasing incidence of different types of ischemic renal diseases leading to ESRD: ARAS 2.5 vs 15 per million (p < 0.0001) in general population and 10 vs 60 per million (p < 0.001) in those aged over 60 yrs, mean age 69 vs 74 yrs, NAS: 1 vs 8 and 4 vs 32 per million (p < 0.001), mean age 67 vs 72 yrs, undetermined VN 0.5 vs 2.5 and 2 vs 10 per million, 65 vs 73 yrs. Our study demonstrates that ischemic renal diseases 1) have become the most frequent causes of ESRD (27% of all patients and 43% of those aged over 6C years) in the Caucasian elderly. 2) are the only cause of increasing incidence of ESRD in this French region.

We report a case of intravascular malignant lymphomatosis observed in a 71 year-old male and characterised by the presence of a proteinuria in relation to the specific intraglomerular localisation. This malignant lymphoma, usually of the B phenotype, is rare and affects predominantly the central nervous system and the skin. Neoplastic cells home selectively to endothelium. Histological renal infiltration is frequent but a glomerular localisation, with proteinuria, is rare. The mechanism whereby lymphocytes home to endothelium cells is unclear but it could be related to the expression of lymphocyte-endothelium adhesion molecules. When present the nephrotic syndrome is associated with minimal change disease.

Non hemochromatotic liver siderosis often present an heterogeneous iron distribution, but histological scoring of iron overload are validated only in case of genetic hemochromatosis, where iron is homogeneously distributed. The aim of this work was to study the improvement of histological scoring by introducing a coefficient of heterogeneity in cases of heterogeneous liver siderosis. Thus, on 254 liver biopsies with siderosis were determined: i) the histologic total iron score (TIS) as previously described; ii) the coefficient of heterogeneity, leading to corrected TIS (corTIS); iii) the liver iron content (LIC). Liver siderosis was homogeneously distributed in 160 biopsies and heterogeneously distributed in 94. Correlation between histological scoring and LIC, in the group with heterogeneous liver siderosis, was improved by the use of the coefficient of heterogeneity. This coefficient leads to accurately quantify all liver siderosis.

We present a case of a primary pericardic thymoma, revealed by a pericardic effusion. This ectopic localisation is very unusual; neoplastic pericarditis are usually due to metastasis of carcinomas or lymphomas. Sometimes, they are secondary to pericardic invasion by mediastinal malignant thymomas or a metastasis of thymomas. Our patient presented a single pericardic localisation of thymoma without any other site. We discuss ectopic localisations of thymic tissue and thymomas and the diagnostic usefulness of the MIC 2 antibody.

From March 1972 to 1st of January 1996, 804 kidney, liver and pancreas transplants were performed in 690 patients. 39 post transplant cancers occurred in 27 patients. 21 skin tumors (15 squamous cell carcinoma, 4 basocellular carcinomas, 1 squamous carcinoma, 1 melanoma), 4 squamous cancers (anus, esophagus, tongue, and parotid), 4 post-transplant lymphoproliferative disorders, 1 non-Hodgkin's lymphoma T, 4 gynecological tumors (breast, ovarian peritoneal carcinomatosis, 2 uterine cervix cancers), 4 miscellaneous tumors (larynx, right colon, brain, prostate, own kidney). 14 patients died (14/27: 52%). Post transplant de novo cancers are a major risk in transplant patients These cancers are mainly represented by skin tumors and lymphomas. Skin cancers are mainly spinocellular and occur in the areas which are exposed to the sun. Post transplant lymphoproliferative disorders are very close to Burkitt's lymphoma, they produced B lymphocyte proliferation due to the reduction of the control of T lymphocytes and induced by Epstein-Barr virus. They can disappear with the diminution of immunosuppression.

Hyperplastic polyps are the most common type of colorectal polyps. They are usually multiple and localized in the rectosigmoid area. They are easily recognized by their small size (< 10 mm), sessile form, smooth-surface and translucid appearance. Typically, these polyps have no malignant potential. We report the case of a 68-year-old man who had multiple rectosigmoid polyps, some of them having macroscopic features of adenomatous polyps. Because of the occurrence of bleeding after endoscopic polypectomy of two polyps corresponding to adenomas at examination, because of the multiplicity of these polyps and their localization in diverticulosis, a left colectomy followed by coloanal anastomosis was performed. Histologic examination of the surgical specimen revealed the hyperplastic nature of all polyps with, in two of them, a focus of adenomatous tissue.

A case of hepatic mesenchymal hamartoma, found after sudden onset clinical and biological cholestasis, is reported in a 18-year-old man. Abdominal ultrasound examination and computed tomography showed a intrahepatic cystic tumor. The diagnosis of hepatic mesenchymal hamartoma was made by the pathological examination of the resected specimen. This rare tumor is found in most cases in children less than 2 years old. Thirteen cases in adulthood were already reported, five of them in Japanese patients. Our case is peculiar because no hepatic tumor was shown by ultrasound examination, 2 years before, the large size of cysts and presence of smooth muscle fibers in the wall.

This retrospective study reports the clinical results obtained in a series of 150 heart transplantations performed from October 1986 to January 1995. Overall actuarial survival rate, including operative mortality, was 71% and 57% at 1 and 5 years respectively. Despite a certain level of morbidity, the spectacular functional results in this series are emphasized.

The authors report a case of laryngeal ulceration due to cytomegalovirus occurring in a 60-year-old man one month after heart transplant. The recipient was initially CMV seronegative and the donor positive. No preventive treatment was administered. One month after the transplantation, search for CMV in blood and urine became positive and a seroconversion occurred. Laryngeal biopsy showed an inflammatory reaction with cells containing atypical inclusions and positivities with anti-CMV antibody. Usual intranuclear eosinophilic inclusions of classic CMV infection were absent. The authors stress the unusual site of the lesions in this primo-infection and the atypical aspect of the inclusions. Such atypical inclusions which have already been reported in immunosuppressed people make the diagnosis more difficult.

The authors report two cases of adenocarcinoma arising from Brünner's glands. The diagnosis was made on histological, histochemical and lectin-histochemical grounds. Brünner's glands carcinoma cells were alike and located very close to normal Brünner's glands. Brünner's glands carcinoma cells contained neutral glycoproteins and were positive for Concanavalin A.

Between January 1st, 1976 and December 31st, 1990, histological diagnosis of primary glomerular diseases (PGD) was made in 480 patients born and living at the time of diagnosis in a region of France comprising 410,664 inhabitants, of whom 390,574 were aged from 10 to 80 years. The prevalence of PGD during a 70-year exposure to risk (10-80 years of age) was evaluated to 5.7 in 1000 (7.6 in 1000 males and 3,8 in 1000 females). The most common PGD was IgA nephropathy with a prevalence of 1.9 in 1000 (3.3 in 1000 males, 1 in 1000 females). The annual incidence of the disease was evaluated separately for 3 consecutive 5-year periods: period A (1976-80), period B (1981-85), and period C (1986-90). Within each of these 3 periods the number of patients with PGD was 179, 170 and 131 respectively, and annual incidence was 9.3, 8.8 and 6.7 in 100,000. The incidence of IgA nephropathy remained the same throughout the 3 periods: 2.6, 3.1 and 2.5 in 100,000. The incidence of membranoproliferative glomerulonephritis decreased from 1981 onward (0.9, 0.5 and 0.15/100,000). Acute streptococcal glomerulonephritis virtually disappeared during periods B and C. Lipoid nephrosis was less frequent in period C and idiopathic proliferative glomerulonephritis with crescents slightly increased (0.3, 0.3 and 0.6 in 100,000). There was no significant difference between the 3 periods regarding the incidence of other PGD. Incidence of IgA nephropathy was 3 to 4-fold higher in the adult aged from 20 to 60 years than in the elderly. In contrast, membranous nephropathy was 3 fold more frequent in the elderly than in the adult. Therefore only some histopathological forms have a different incidence according to age, but the major information furnished by this study is that the risk of occurrence of a PGD is similar in the population living in the area, whatever the age group (10-19 years: 6.4/10(5) inhabitants, 20-39: 7.1/10(5), 40-59: 8.4/10(5), 60-79: 8.4/10(5)). Finally, we confirm that the most common PGD going to end stage renal disease was IgA nephropathy, particularly under 60 years of age (0.8/10(5)). In contrast, membranous nephropathy was a less frequent cause of ESRD (0.2/10(5)).

Intraductal mucin-producing tumors of the pancreas are rare, recently described tumors; they have a better prognosis than usual ductal carcinomas of the pancreas. We report two cases of villous tumor of the main pancreatic duct and one case of mucinous ductal ectasia, with histochemical and immunohistochemical study.

The authors report a case of congenital epulis in the lower gum of a new born female. This rare benign tumor of the newborn is histologically characterized by the granular aspect of its cells. It occurs more frequently in females than in males. Differential diagnosis mainly concerns Abrikossoff tumor whose histogenesis and evolution are different. The distinctive features of epulis are the presence of a fusiform cell component and the negativity of the granular cells for PS100.

Desmoid tumor are rare connective tissue tumors currently considered as sarcoma of low grade malignancy. They are most often encountered in young women of child-bearing years. Abdominal localisation is the most frequent site. The main aim of treatment is to avoid recurrences. We report a case of desmoid tumor developing in the thoracic cage in a patient who had been operated on six years earlier for an epidermoid carcinoma of bronchus.

The authors studied the diagnostic interest of cell cultures in thyroid tumors. They cultivated 10 tumours (4 papillary carcinomas, 5 medullary carcinomas and 1 sarcoma-looking tumour). In 7 cases, cultures yielded nothing more than pathologic examination. Nonetheless the study allowed to detect a double secretion (calcitonin and thyroglobulin) in 2 medullary carcinomas which turned out to be mixed proliferations. It also detected a production of thyroglobulin by the sarcoma. They think that cell cultures may have a diagnostic interest besides pathologic examination, allowing precise study of secretory features of the tumors.

Cystadenomas and cystadenocarcinomas are the most common cystic tumors of the pancreas. Their inner lining contains neuroendocrine cells that may, in rare cases, produce a systemic syndrome. Similarly, digestive or pancreatic endocrine tumors have been reported in association with cystic tumors of the pancreas. We report a case of cystadenocarcinoma of the pancreas head, associated with a serous cystadenoma and an endocrine tumor of the body of the pancreas. In a 70 year old patient, jaundiced due to obstruction of the extra-hepatic bile ducts, the abdominal ultrasound and CT scan showed a hypoechogenic hypodense lesion of the pancreas head resulting in an enlargement of the common bile duct and pancreatic duct. Echoendoscopy confirmed the diagnosis and gave additional information; it showed that the cephalic lesion was heterogeneous, with anechogene spaces separated by coarse echogenic septae associated to a solid contingent. Also detected by echoendoscopy was a cystic lesion containing thin septae and a contiguous hypoechogenic nodule of the body of the pancreas. Histology of the specimen following total pancreatectomy revealed a cephalic cystadenocarcinoma associated with a serous cystadenoma and a non-secreting endocrine tumor of the body region. This observation highlights the usefulness of echoendoscopy in the work-up of cystic lesions of the pancreas; indeed, the tumors of the body of the pancreas were not detected by others methods. Furthermore, there are reports in literature on endocrine secretion syndromes secondary to digestive endocrine tumors or neuroendocrine cells in the lining of cystic tumors of the pancreas.

The authors report two cases of thoracic actinomycosis with contrasting presentations, just as the diagnosis was obtained by different tests underlying the varied aspects of this disease. Indeed this infectious pathology does infrequently mimic tuberculosis or lung cancer with similar clinical and radiological appearances. The particular characteristic of the bacteriological culture and the quasi specific character of the pale, yellow grains on histology are recalled, as well as the different treatments necessary to ensure a cure in 90% of cases.

Carcinoma in situ, dysplasia, prostatic intra-epithelial neoplasia, duct-acinar dysplasia and large-acinar hyperplasia are various terms describing more or less identical forms of prostatic epithelial atypia. The precancerous nature of these lesions can be demonstrated by: morphological and functional similarities with carcinoma, a younger age than that of carcinoma, a higher incidence in cancerous prostates, an identical zonal distribution and a significant progression of high-grade lesions towards carcinoma. These hypoechoic lesions can be detected or monitored by transrectal ultrasonography. They also secrete PSA at levels intermediate between those of benign prostate and adenocarcinoma. Because of the occasional risk of malignant transformation and a frequent association with carcinoma, these lesions should be regularly monitored by digital rectal examination, PSA assays and possibly by ultrasound-guided biopsies.

Villous neoplasms of the main pancreatic duct are uncommon. Two cases of neoplasm of the main cephalic pancreatic duct in 61- and 42-year-old patients presenting with long standing (10 and 12 years) history of abdominal pain are reported. In both cases, duodenal fistula was present and mucus was observed by endoscopy at the fistula and major papilla levels. Endoscopic retrograde pancreatography showed a stricture of the main pancreatic duct in the pancreatic head. In one case, with incomplete stricture, pancreatic ducts disclosed typical features of chronic obstructive pancreatitis and contained mucus casts. Histologic examination of total and proximal duodenopancreatectomy showed a villous neoplastic pattern with focal malignant changes within the main pancreatic duct. The adjacent pancreatic tissue showed signs of stromal invasion without lymph node or nervous infiltration. Glandular parenchyma was atrophic in the pancreatic body and tail, with extensive fibrosis, and the pancreatic duct depicted signs of nonpapillary hyperplasia. Histochemical study disclosed a predominant sialomucin secretion by villous adenoma and sulfomucin secretion by epithelial cells lining the accessory or main caudal pancreatic ducts. These results lead us to suggest a possible relationship between villous adenoma of ducts and pancreatic adenocarcinoma.

Monoclonal antibodies (AcM) have opened up new perspectives in the study of cancer. The production of murine AcM has been well described in the past. The characterisation of the antigen which they recognise and their in vitro and in vivo biological properties has led to their use in diagnosis and in due course to therapeutic possibilities. The specificity of AcM "vis a vis" certain molecules or tumour associated antigens enables their utilisation for diagnosis and their widespread use for the labelling of tumour markers. AcM may in difficult cases be more accurate in specifying the histological type of a bronchopulmonary or pleural tumour; by coupling the antibody to a radio isotope and injecting it into the circulation. This enables the detection of tumours in vivo by immunoscintigraphy. The numerous problems linked to the technique and above to biology of AcM limits its clinical value in assessing the degree of extension either locally or regionally of bronchopulmonary tumours. The therapeutic applications are at this stage experimental and above all studies have shown the limits of AcM used alone or coupled to a radio isotope, a toxin or a drug. The limits of the technique are linked to numerous factors, such as accessibility of the antigen site, the heterogeneity of tumour markers and non specific fixation and immunogenicity of the AcM. The experimental clinical stage should be continued in order to define the most effective utilisation of AcM in therapy which will doubtless be complementary to other forms of treatment.

Gastroduodenal duplication is a rare congenital entity. Preoperative diagnosis is difficult because symptoms are usually non specific and can mimick pancreatitis. We report 2 cases of gastric and duodenal cystic duplication in adults presenting as pancreatitis. Chronic pancreatitis was observed on endoscopic retrograde pancreatography and confirmed at laparotomy in both cases. Pancreatitis was probably related to alcoholism in one case, but might have been secondary to duplication in the other. In both cases, duplication was misinterpreted initially as a pseudocystic complication of pancreatitis until laparotomy was performed. These 2 cases lead us to discuss the relationship between duplication and pancreatitis.

A Wolff-Parkinson-White syndrome was observed during acute rejection in a patient who had undergone orthotopic cardiac transplantation. The sometimes intermittent nature of this syndrome could explain its postoperative appearance in this patient; the relationship with the episode of rejection is discussed.

Fourteen cases of anti-GBM antibody-induced RPGN were evaluated retrospectively in terms of renal function improvement and therapeutic risks. Nine men and 5 women (mean age: 55.3 years) were observed over a 9 year period; in three patients, hemoptysis was associated with renal disease (Goodpasture's syndrome). Most of these patients had received combinations of steroid therapy (ST), immunosuppressive drugs (IS) and plasma exchanges (PE). Age, duration of symptoms prior to diagnosis, initial renal function, therapeutic modalities and complications were assessed according to renal outcome: 9 patients (group A, "non-responders") remained on dialysis irrespective of the treatment administered; 5 patients (group B, "responders") recovered renal function. Complications, especially infections, were twice as frequent in group A. Two of the 4 recorded deaths were related to the disease or the treatment. Analysis of clinical and pathological values at the time of entry into the study for both groups indicated that oliguria/anuria, serum creatinine greater than 500 mumol/l and greater than 50% crescents, when associated, were factors predictive of poor renal outcome; in these patients, dialysis may be required except in cases of pulmonary hemorrhage. In all other patients, treatment with ST, IS and PE is recommended. Active hemoptysis necessitates pulse steroids or PE; if absent, further tests (carbon monoxide uptake, bronchoalveolar lavage, lung biopsy) are indicated before use of aggressive therapy.

Diffuse gastric antral vascular ectasia (GAVE) is a new disease entity corresponding to peculiar acquired vascular malformations. In 1984 Jabbari et al. coined the term "Watermelon Stomach" for this lesion. We report here in a new case particular by its association with systemic sclerosis in a 66 year-old female. The diagnosis of GAVE was suggested upon endoscopic and angiographic findings. Atrophic fundic gastritis associated with achlorhydria and hypergastrinemia was also noted. Antrectomy was performed with success. Pathologic examination and morphometric analysis of the antrum specimen revealed the characteristic features of GAVE: 1) increase of the mean cross sectional area of mucosal vessel lumen and increase of the mean percentage of specimen area occupied by vessels; 2) presence of intravascular fibrin thrombi and 3) fibromuscular hyperplasia of the lamina propria.

Two cases of paralysis of the etxernal popliteal sciatic nerve are described. They are secondary to the development of a pseudo-cyst containing mucoid matter within the connective tissue sheath of the nerve itself. The authors describe the pathological anatomy of these lesions and discuss their aetiology, favouring the theory of mucoid degeneration of the connective tissues of the nerve sheath. They give brief indication of the clinical symptoms and emphasize the need for early but simplified surgery, ruling out any nerve resection.

The frequency of the association between extramembranous glomerulopathy and a visceral tumour suggests the responsibility of various antigen-antibody systems related to malignancy, at the origin of the nephropathy. In a number of special cases, the role of these systems has been more precisely demonstrated. The cases reported here emphasise the need to seek a visceral tumour when the onset of extramembranous glomerulitis occurs after the age of 40 years, in particular since the onset of proteinuria may precede the first clinical signs of the tumour by several months. The presence of polynuclear cells in the glomerular capillary loops, associated with extramembranous deposits, is an additional element of orientation in the direction of neoplastic aetiology.

Twenty-five observations (children and adults) of glomerulo-nephritis secondary to Schoenlein-Henoch purpura were studied retrospectively. Histological studies done early in the course of the disease-less than one year after the first attack-enabled us to made, in function of the evolution, clinical and pathological correlations using the method of Levy et al. The intensity of the clinical signs at the time of biopsy appeared proportional to the degree of cellular proliferation whether it was endo or extracapillary form; it is much more difficult to define when mesangial proliferation predominates or in the absence of proliferation. In addition to the usual immunopathological abnormalities, this study found that IgE plasma concentration was frequently elevated. Thus, IgE may play a role in the pathogenesis of this systemic disease.

The authors report a case of a 35 year old man with of congestive cardiac failure. Echocardiography showed diffuse hypokinetic wall motion with moderate parietal hypertrophy without dilatation. Post-mortem examination showed intramyocardial deposits of light chains identical to those observed in "in vivo" renal and liver biopsies. This rarely described disease has a poor prognosis. It is characterised by polyvisceral infiltrations of light chain monoclonal immunoglobulins. Renal disease is usually the main problem progressing rapidly to renal failure. Of the extra renal localisations, cardiac involvement would appear to be common and preoccupying in itself. Monoclonal plasmocytic proliferation is observed in all cases, the majority but not all being malignant (myeloma). The incidence of this condition is probably underestimated and may pass undetected if immunofluorescent techniques are not used. Myelomatous light chain cardiac disease could therefore be more common than amyloidosis with which it presents a number of common features.

A retrospective and prospective study of 58 patients with IgA glomerulonephritis was undertaken between the 1st April 1975 and the 1st May 1982, to determine the clinical, biological and histological features associated with the onset of renal failure and a poor prognosis. At the end of this study the patients were classified into 4 groups according to the clinical stage of their disease: a) normal, b) minor urinary abnormalities, c) active disease and d) terminal renal failure. The renal biopsies were reviewed and an index of histological activity defined. The significant features associated with progression of the disease to renal failure were: persistent proteinuria, a transient nephrotic syndrome, hypertension, diffuse proliferative glomerular changes with epithelial crescent formation, tubulo-interstitial changes and vascular lesions on renal biopsy. When confronted with the clinical outcome, the histological results of the first renal biopsy performed when the disease was first diagnosed and quantified in an index of activity, had a good predictive value.

With reference to a case of Hodgkin's granuloma of the frontal lobe, a review of the literature has enabled 8 similar cases of intra-cerebral localisation of this disease to be reclassified. Their mechanism is discussed on the basis of neuropathological data and in particular their development in situ. Excision of the lesion appears to be the treatment of choice.