Severe systemic cytomegalovirus infections in patients with steroid-refractory ulcerative colitis treated by an oral microemulsion form of cyclosporine: report of two cases.

CMV reactivation is frequently observed in acute flares of ulcerative colitis (UC), particularly those which do not respond to intravenous steroids. Several recent series have suggested that, in most cases, CMV reactivation does not lead to severe complications and resolves spontaneously with the UC flare and discontinuation of immunosuppression. In the present paper, we describe two patients with active UC who developed a severe systemic CMV infection during a treatment with an oral microemulsion form of cyclosporine. This is of concern, particularly in a context of increasing use of immunosuppressive drugs in UC. We propose a prophylactic and curative approach to decrease morbidity related to CMV infection in active UC.

Vestigial retrorectal cystic tumors in adults: a review of 30 cases.

OBJECTIVES: Retrorectal tumors are uncommon. This is a report on a series of vestigial retrorectal cystic tumors in adults that were surgically removed at two medical centers in France. We also assessed the significance of imaging and, in particular, magnetic resonance imaging (MRI) in the management of these lesions. METHODS: The medical files of adult patients operated on for vestigial retrorectal cystic tumors over the past 15 years were retrospectively studied and, in particular, the radiological studies, the treatment and the histopathology. RESULTS: Thirty patients underwent surgery for vestigial retrorectal cystic tumor. A preoperative diagnosis was possible in almost all cases. The surgical procedure was justified by preoperative imaging and included the transanal approach (three cases), posterior approach (23 cases), anterior approach (two cases) and combined posterior and anterior approach (two cases). Imaging can identify a multilobular lesion that may require the use of a coccygeal approach to achieve complete resection. During a mean follow-up of 3.2 years (range 0.5-15 years), two patients had a local recurrence (successfully surgically removed), and a further two patients were lost to follow-up. Only one lesion was malignant. CONCLUSIONS: Preoperative imaging, and especially magnetic resonance imaging, enables both a specific diagnosis and the selection of the optimal surgical procedure for the treatment of vestigial retrorectal cystic tumors in adults.

[A false peritoneal carcinomatosis]. / Une pseudocarcinose péritonéale.

Abdominal actinomycosis is an unusual peritoneal disease due to actinomyces, which are Gram-positive bacillus bacteria. Those saprophytes pathogens of digestive tract and genital mucous, can appear accidentally in the peritoneum cavity, in tumor form, simulating a neoplasic disease. We report the case of a 43-year old woman, with abdominal actinomycosis, revealed by feverish bowel obstruction. During laparotomy, several nodes were removed. Their histologic study permitted the diagnosis. Long-term antibiotics treatment allowed patient recovery.

Medullary thyroid microcarcinoma: a clinicopathologic retrospective study of 38 patients with no prior familial disease.

Thirty-eight patients (25 women, 13 men; mean age, 57.8 [32 to 91]) showing one or more medullary thyroid microcarcinomas (ie, < 1 cm), with no prior MEN II or medullary thyroid carcinoma history in their family, were reviewed. Follow-up was available for 29 patients (mean, 53.6 months [1 to 147]). 21 patients (72.4%) are alive and free of disease, four patients (13.8%) died during follow-up without disease, 2 patients are alive with disease (local recurrence and persistent hypercalcitoninemia) after 80 and 99 months, respectively, and 2 patients died of disease after 24 and 46 months. Most tumors were incidental pathological findings (19 of 38) or were discovered by systematic blood calcitonin measurement for a nodular thyroid disease (15 of 38). Only the four patients who had an unfavorable outcome were symptomatic cases (palpable micro-MTC, diarrhea, cervical lymph node metastasis and pulmonary metastatic disease). The two patients with metastatic disease at diagnosis died during follow-up. In univariate analysis, a symptomatic medullary thyroid carcinoma was a strong predictor of an unfavourable outcome (p < .00008), as were the preoperative calcitonin level (P = .007) and an elevated postoperative calcitonin level (P = .004). Among 30 histopathological criteria, only the presence of amyloid correlated with an unfavorable outcome (P = .018).

Medullary thyroid carcinoma: search for histological predictors of survival (109 proband cases analysis).

A group of 13 pathologists belonging to the French Calcitonin Tumor Study Group (GETC: Groupe d'Etude des Tumeurs à Calcitonine) examined the histological slides and medical records of 109 proband cases of medullary thyroid carcinoma (MTC) diagnosed on clinical features. The cases belonged to the various forms of the disease (80 sporadic and 29 familial MTC). The aim of the study was to detect histological predictors for survival by comparing morphological data from patients killed by the disease versus the others. Twenty-seven histological parameters were considered, including cellular heterogeneity, shape of the cells, and cytoplasmic characteristics. Other parameters such as sex, age, and phenotype of the disease were also studied. First, predictive parameters of interest on survival function were selected by univariate analysis (Mantel-Cox test). Then, the extracted parameters were tested in a multifactorial analysis using the Cox's forward stepping proportional hazard model. Five parameters were significantly associated with a lower survival function: presence of necrosis in the tumor (P = .001), squamous pattern (P = .002), age over 45 years (P = .004), presence of oxyphil cells in the tumor and absence of cells with intermediate cytoplasm (P = .025), less than 50% of calcitonin immunoreactive cells in the tumor (P = .04).

Systemic cat scratch disease: hepatic and splenic involvement about 3 pediatric cases.

Numerous diseases can lead to multinodular lesions of liver and spleen; surgical biopsy can be required for the etiologic diagnosis. Among these diseases, systemic cat scratch disease has been recently described. Macroscopical appearance of the lesions is evocative and must be known by surgeons. Three children with systemic cat scratch disease involving liver and spleen are reported.

[Histamine-producing neuroendocrine tumor of the ampulla of Vater]. / Tumeur neuro-endocrine de l'ampoule de Vater sécrétant de l'histamine.

Neuroendocrine tumors of the ampulla of Vater are rare (less than 100 cases reported). We report here a new case characterized by histamine secretion, a hitherto unreported feature. Clinical presentation is similar to that of other tumors of the ampulla of Vater. In our observation, the patient had noticed urticaria on the right forearm for several months. Tumor of the ampulla was confirmed by endoscopic ultrasonography, while neuroendocrine characterization was assessed on biopsies after endoscopic sphincterotomy. Histamine concentration in blood was the only elevated neuroendocrine marker and returned to normal after surgical resection. Histology showed a neuroendocrine tumor with extension to lymph nodes. On immunohistochemical analysis, production of histamine was confirmed, and the diagnosis of mastocytoma was eliminated. In view of the literature, neuroendocrine tumors of the ampulla of Vater are associated with a good prognosis (5 year-survival rate: 90%) despite early lymph node involvement.

[Rathke's cleft cyst]. / Kystes de la poche de Rathke.

Rathke's cleft cyst (RCC) are frequent benign cystic sellar lesions. Most RCC are small, intrasellar and asymptomatic. Larger cysts may compress adjacent structures and rarely become symptomatic. Diagnosis is strongly suggested at MRI by the presence of a midline non-enhancing lesion located exactly between the anterior and posterior lobes of the pituitary gland. Even if its signal is variable and related to intracystic protein concentration, it must be homogeneous with no fluid-fluid level. Once a diagnosis of RCC made, routine MR and clinical follow-up is sufficient for incidental asymptomatic cysts whereas the rare symptomatic lesions are neurosurgically resected.

[Hemothorax disclosing hemorrhagic necrosis of a pheochromocytoma: circumstance for detection of multiple endocrine neoplasia type IIa. Detection of 5 familial cases]. / Hémothorax révélateur d'une nécrose hémorragique d'un phéochromocytome: circonstance de découverte d'une néoplasie endocrine multiple de type II a. Dépistage de cinq cas familiaux.

Whilst investigating 26 members of the same family, we discovered 5 cases of multiple endocrine neoplasia type II a. The present report demonstrates the diagnostic value of basal plasma thyrocalcitonin (TCT) assays, before and after stimulation with pentagastrin, and of plasma carcinoembryonic antigen (CEA) assays. Some of the clinical features encountered were novel--e.g. in one patient the phaeochromocytoma was revealed by a haemothorax with cardiovascular collapse--and others were peculiar; thus, in 4 cases the medullary thyroid carcinoma (MTC) was less than 2 cm in diameter and without lymph node or visceral metastases, even in patients aged 87, 59 and 56. More classically, MTC, always multifocal, was clinically silent, as were the two cases of phaeochromocytoma and hyperparathyroidism. Phaeochromocytomas were difficult to diagnose. Ultrasonic tomography did not prove very helpful and the disease was transmitted as an autosomal dominant trait. Finally, MTC secreted a variety of substances (TCT, CEA, beta-endorphin, somatostatin), and HLA A2-B15 antigens were found in 3 patients.

[Systemic amyloidosis in the elderly: diagnostic value of the test of subcutaneous abdominal fat and the labial salivary glands. Prospective study in 100 aged patients]. / L'amylose systémique du sujet âgé: valeur diagnostique de l'examen de la graisse sous-cutanée abdominale et des glandes salivaires accessoires. Etude prospective chez 100 patients âgés.

Primary and secondary amyloidosis are not uncommon in aging but the diagnosis is rarely made on account of the risk of bleeding in the site of biopsies and the difficulty to distinguish senile from systemic amyloidosis on the biopsy samples. We have studied the frequency of amyloid deposition in the abdominal fat aspirate (AFA), the labial salivary gland (LSG), the temporal arteries (four cases), bone marrow (two cases), digestive tract (four cases) in 100 elderly patients (aged 80 or greater). AFA was positive in 15 percent of the patients and LSG in 5%; both samples were positive in 4%. Four cases of systemic amyloidosis were found (two of the AL and two of the AA type). Sensitivity of AFA was 75%, specificity was 87% and the positive predictive value was 20%. The values were respectively 100%, 99%, 100% for LSG. In 11 patients whose AFA biopsies samples were singly positive, amyloid deposits were found in temporal arteries in four of four cases. We conclude that AFA is too sensitive for the diagnosis of systemic amyloidosis in aging. The responsibility of senile amyloid deposition on AFA should require further investigations. LSG biopsies seem to be a more reliable test for the diagnosis of primary and secondary amyloidosis in elderly.

[Diagnosis of amyloidosis in the elderly: value of the aspiration of the abdominal subcutaneous fat. Prospective study apropos of 100 cases]. / Diagnostic de l'amylose du sujet âgé: intérêt de l'aspiration de la graisse sous-cutanée abdominale. Etude prospective à propos de 100 cas.

Study of the presence of amyloid deposits on subcutaneous fat aspirates was carried out in 100 patients hospitalized in a geriatric unit. The test was positive in 15%, and in 4% of them, amyloid deposits were also seen on accessory salivary glands, confirmed by immunohistochemical staining. These results indicate that sub-cutaneous fat aspirate may be a useful test for an early diagnosis of amyloidosis.

[Solid cell nests and thyroid pathologies. Retrospective study of 1,390 thyroids]. / <> et pathologies thyroïdiennes. Etude retrospective de 1,390 thyroïdes.

Solid Cell Nests (SCN) of the thyroid gland were considered as embryonic remnants or as squamous metaplasia of follicular epithelium. In a retrospective study of 1 390 thyroids, SCN were found in 9.7 % of thyroids (16 % men and 8 % women, chi(2) : p <0, 01). The number of SCN rises with increasing number of sections examined. No statistical differences were found with age or thyroid disorders and presence of SCN, excluding the metaplastic theory. SCN contained in 38 % of cases, neuroendocrine cells (calcitonin and/or calcitonin gene related peptide, chromogranin A) and thyroglobulin cells was observed in 78.3 % of SCN. The different cell types of SCN fitted with the ultimobranchial remnant theory. Thus, some rare carcinomas (like mixed medullary and vesicular carcinoma or mucoepidermoid carcinoma) might originate from SCN.

[Amyloid goiter. Report of two cases]. / Le goitre amyloïde. A propos de deux observations.

We report two cases of amyloid goiter which clinical appearance had evoked a neoplastic lesion. The diagnosis was made by histologic examination. The nature of the amyloid precursor, determined by immunohistochemistry, has allowed to evoke primitive amyloidosis in one case and to attribute amyloid goiter to renal insufficiency in the second case.

[Recurrence of hyperparathyroidism following a parathyroid autograft. Hyperplasia or carcinoma?]. / Récidive d'un hyperparathyroïdisme sur autogreffe parathyroïdienne. Hyperplasie ou carcinome?

The authors report a case in which hyperparathyroidism recurred after total parathyroidectomy and autogenous parathyroid graft into the fore-arm musculature, in a patient with chronic renal failure. Light and electron microscopic studies of the grafted tissue show a severe, nodular hyperplasia. This case report illustrates the rapid "tumour-like" growth of the hyperplastic parathyroid grafted tissue; according to some authors, the hyperplastic parathyroid tissue displays an increased propensity to become malignant.

[112 cases of sporadic and genetically determined pheochromocytoma: a comparative pathologic study]. / 112 cas de phéochromocytomes sporadiques et génétiquement déterminés: Etude anatomo-pathologique comparative.

The aim of this study was to compare 64 genetically determined pheochromocytomas (PH) (49 MEN IIa, 3 MEN IIb, 6 Von Recklinghausen diseases, 1 von Hippel-Lindau disease, 5 familial pheochromocytomas) and 48 sporadic PH. Genetically determined PH were more often observed among men and more frequently bilateral and multicentric than sporadic PH. Sporadic tumors had more often adrenal capsular invasion, necrosis and pseudocysts. Genetically determined PH were more differentiated with an insular pattern, hyaline globules and a higher percentage of polyhedric cells. Sporadic tumors were less differentiated with more frequently a diffuse pattern and small cells. Adrenal medullar hyperplasia was significantly associated with genetically determined PH. Adrenal cortical hyperplasia was not associated with a particular type of PH. The PS100 and chromogranin immunodetection was equivalent in both groups.

[Small intestinal stromal tumors with skenoid fibers. Clinicopathological study of three cases]. / Trois observations de tumeurs stromales de l'intestin grêle à fibres en écheveau.

Gastrointestinal stromal tumours (Gist) are mesenchymal tumour with uncertain prognosis occurring in the gastrointestinal tract wall. For clinicians, these tumours raise two problems: to establish the diagnosis and to determinate the future behaviour for the choice officient therapeutics. For the diagnosis the new marker c-KIT is useful. A new treatment with an inhibitor of c-KIT has given encouraging results. currently there is no consensus on specific cut-points to distinguish as low or high risk (i.e., malignant) Gist. For metastases-free Gist, the prominent histopronostic markers are size, mitotic index and localization of the tumour. The small intestine Gist have the reputation to be more aggressive than in other localization. Skenoid fibers in small intestine Gist could be a marker of good prognostic. The authors reported three cases of small intestine Gist with skenoid fibers. The discussion point out the significance of this particular morphological aspect.

[Congenital microcoria: a case report with histopathological study]. / La microcorie congénitale: une observation avec étude histopathologique.

The authors report a case in which congenital microcoria was associated with glaucoma; a complete histopathological study was performed and the relation between microcoria and glaucoma was considered. The association with extra-ocular abnormalities, particularly renal lesions is noticed.

[A little known cause of persistent fever and granulomatous hepatitis in children: cat scratch disease]. / Une cause peu connue de fièvre prolongée et d'hépatite granulomateuse chez l'enfant: la maladie des griffes du chat.

BACKGROUND: Extensive hepatic and splenic involvement in cat-scratch disease has rarely been reported. CASE REPORT: A 2 1/2 year-old boy suffered for 2 weeks from high-grade fever, abdominal pain and alteration of his general condition. Ultrasonography revealed multiple hypoechogenic nodules in liver and spleen. The CT scan also showed hypodense lesions. An open liver biopsy was performed 5 weeks after the onset of illness. Histopathology of a resected nodule demonstrated neutrophilic granulomatous inflammation with central abscess formation. Recent cat exposure, suppurated epitrochlear lymph node 15 days before admission were also consistent with cat-scratch disease which was confirmed by elevated anti-Rochalimaea antibody titers. Follow-up showed complete resolution of all hepatic and splenic lesions within 6 months and emergence of splenic calcifications. CONCLUSION: Cat-scratch disease should be considered in the diagnosis of fever of unknown origin and hepatosplenic abscesses in children.

[Intrathyroidal parathyroid adenoma. Study of a case]. / L'adénome parathyroidien en position intrathyroidienne: à propos d'un cas clinique.

Intrathyroidal parathyroid adenoma is an infrequent lesion which can be explained by abnormalities during embryonic migration of the parathyroid glands. That abnormality can be a cause of failed cervical exploration for primary hyperparathyroidism. From a case report and literature data, we propose an exploration processes to search those ectopic parathyroid adenoma.