Epignathus teratoma: report of three cases with a review of the literature.

Three cases of epignathus teratoma associated with other midline anomalies are reported. The first case involved Pierre Robin sequence and a bifid tongue. The second case was characterized by two teratomas, a meningoencephalocele, and a cleft lip and nose. The third case had Pierre Robin sequence associated with duplication of the pituitary gland and hypoplasia of the corpus callosum.

[Granular cell tumor and congenital epulis. Histochemical and immunohistochemical of 58 cases]. / Tumeur à cellules granuleuses et épulis congénitale. Etude histochimique et immunohistochimique de 58 cas.

56 cases of granular cell tumour and 2 cases of granular cell congenital epulis have been studied morphologically, histochemically and immunohistochemically. The subject of this study is to evaluate the diagnosis interest of the PAS stain, determine the positivity of 4 antibodies (vimentin, NSE, PS100, NK1/C3) and confirm or not the neuroectodermic origin. The study showed that the microscopic findings are often sufficient. When the diagnosis is less evident, the PAS stain is not discriminant, but we can use the immunohistochemistry study: we confirmed the positivity of the 4 antibodies, but PS100 and NK1/C3 are the most constant. Only the 2 cases of granular cell congenital epulis always show negative immunoreactivity with S100 protein. About the histogenesis, our study confirm the neurogenic origin, but the hypothesis of a non neoplastic nature is raised.

[Long term results of esophageal epidermoid cancers in complete remission after preoperative chemo-radiotherapy]. / Devenir des cancers épidermoïdes de l'oesophage en réponse complète après chimio-radiothérapie pré-opératoire.

INTRODUCTION: Improvements of the results of combined chemoradiotherapy (CRT) in esophageal cancer has led several groups to adopt a non surgical attitude specially in case of complete response (endoscopy +/- biopsy). Few information are available about the follow-up of these patients. We studied long-term results of 35 patients who underwent resection after complete response to preoperative chemoradiotherapy. PATIENTS AND METHODS: 161 patients with resecable carcinoma of the thoracic esophagus have received the same protocol of CRT (cisplatin 80 mg/m2, radiation therapy split course: 37.5 Gy) all patients were followed every for 4 months (no lost of view). RESULTS: Complete response (endoscopy and biopsy) was obtained for 35 patients (21.7%), 19 of them (54%) had pathologic complete response (PCR) (no tumor in the specimen), 16 have microscopic foci of residual tumor (46%). The overall 5-year survival rate was 49.8% for the whole group (median survival 64 months), 70% for the group without tumor in the specimen, 48% for the group with microscopic foci of residual tumor (NS). CONCLUSIONS: One half of the complete response (endoscopy + biopsy) have not a pathologic complete response (microscopic foci of residual tumor in the specimen). The 49.8% of five year survival suggests a benefit from esophagectomy for complete response after combined chemoradiotherapy.

[Intra-masseter metastasis of a gastric adenocarcinoma]. / Métastase intra-massétérine d'un adénocarcinome gastrique.

We report a case of metastasis in the masseter muscle secondary to stomach carcinoma. Second neoplastic invasion in skeletal muscle is an exceptional phenomenon. And this frequency is paradoxical against corporeal muscular mass. Immune hypothesis is conjure up for explain this fact. Symptoms are not specific and the diagnosis is not often recognized. However, systematic postmortem investigation is positive in one per cent of the cases. Consequently, every painful and nodular induration involved in carcinoma supervision must do perform a systematic biopsy.

Mucin gene expression in biliary epithelial cells.

BACKGROUND/AIMS: In recent years considerable advances have been made in our knowledge of human mucin genes. Although analysis of their genomic organization is still in progress, the pattern of their expression in different human mucosae is now fairly well established. However, little is known about their expression in the biliary tree. In this study we determined the pattern of expression of the different human mucin genes in gallbladder biliary epithelial cells, intrahepatic bile ducts and liver. METHODS: Two complementary methods were used: Northern-blot and in situ hybridization analyses. The experiments were performed with eight probes corresponding to MUC1, MUC2, MUC3, MUC4, MUC5AC, MUC5B, MUC6 and MUC7. RESULTS: Our results revealed a strong mRNA expression of MUC3, MUC6 and MUC5B, a weak expression of MUC1, MUC5AC and MUC2, and no expression of MUC4 and MUC7. Surprisingly, MUC3, which was the gene which was most expressed in the biliary tree, was also found in hepatocytes, suggesting another function for the MUC3 protein than that of a secreted mucin. CONCLUSIONS: We conclude that MUC3, MUC6 and MUC5B were the main mucin genes expressed in biliary epithelial cells.

[Total pancreatectomy for diffuse and malignant mucinous ductal ectasia of the pancreas. Apropos of 2 cases]. / Pancréatectomie totale pour ectasie canalaire mucineuse diffuse et dégénérée du pancréas. Apropos de deux observations.

Two cases of mucinous duct ectasia are reported. Epithelial changes were spread along the pancreatic duct and ductal ectasia was diffuse. Multi focal infiltrating carcinoma was observed in one case, microinvasive carcinoma located in the head of the pancreas was observed. Morphologic features by computed tomography, endoscopic ultrasonography pancreatography were useful for preoperative diagnosis of mucinous pancreatic duct ectasia. In one case, malignancy was suggested by measurement of tumor markers in cystic fluid aspirated during percutaneous pancreatography. Total duodenopancreatectomy was performed in both cases. The two patients were alive and disease-free with a follow-up in both cases of 18 and 36 months.

[Metaplastic breast cancer with chondroid differentiation. Report of three cases]. / Carcinome mammaire métaplasique avec différenciation chondroïde. A propos de trois cas.

The authors report three cases of breast carcinoma with chondroid metaplasia. These three tumors were characterized by direct transition between the carcinomatous and the mesenchymal component. Immunohistochemical study revealed epithelial antigens within the mesenchymal appearing component. Pitfalls in the diagnosis and in the classification of metaplastic carcinomas are reviewed.

Systemic vascular smooth muscle cell impairment in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy.

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is characterized by a cerebral non-atherosclerotic, nonamyloid angiopathy mainly affecting the small arteries penetrating the white matter. In the brain vessels of two patients with CADASIL, abnormal patches of granular osmiophilic material have recently been described. Here we report the observation of similar granular osmiophilic material within the vessel walls of muscle and skin biopsies from a 54-year-old woman belonging to a CADASIL family, who suffered from subcortical dementia with leukoencephalopathy demonstrated on neuroimaging. Postmortem examination disclosed changes of the vessel walls in all the organs chiefly leading to cerebral lesions. Ultrastructural study showed destruction of the vascular smooth muscle cells (VSMC) and the granular osmiophilic material already found in muscle and skin biopsies in this patient. Both changes were found all along the arterial tree. The findings of this study indicate that CADASIL is a systemic vascular disease involving arterial VSMC and that the lesions are different in each organ and vessel wall, depending on their fine structure. Moreover, it emphasizes that skin and muscle biopsies might be useful for diagnosis of and research into CADASIL.