Primary central nervous system lymphomas in 72 immunocompetent patients: pathologic findings and clinical correlations. Groupe Ouest Est d'étude des Leucénies et Autres Maladies du Sang (GOELAMS)

We reviewed 72 primary central nervous system lymphomas occurring in immunocompetent patients. The cases were reviewed for clinical data, histology, immunophenotype, bcl-2 and p53 expression, and Epstein-Barr virus association. Follow-up was available for 40 patients included in the Groupe Ouest Est d'étude des Leucénies et Autres Maladies du Sang (GOELAMS) lymphomes cérébraux primitifs (LCP 88) trial. Each diagnosis, requiring a consensus among at least 3 pathologists, was performed according to the recent Revised European-American Lymphoma classification and equivalents in the updated Kiel classification. Tumors were predominantly classified as diffuse large B-cell lymphomas. There were 3 T-cell lymphomas and 1 Hodgkin lymphoma. The proteins bcl-2 and p53 were expressed in 35% and 16% of the tested cases, respectively. Epstein-Barr virus was not found by in situ hybridization except in the case classfied as a cerebral localization of Hodgkin disease. No significant association was found between subtypes, bcl-2 or p53 expression, and patient survival. From the standpoint of their biologic characteristics, primary central nervous system lymphomas are very similar to systemic diffuse large B-cell lymphomas. In contrast to AIDS-related primary central nervous system lymphomas, primary central nervous system lymphomas are rarely associated with Epstein-Barr virus and in immunocompetent patients they express bcl-2 at a relatively low rate.

Cerebral primitive neuroectodermal tumour in a heifer.

A primitive neuroectodermal tumour (PNET) replacing the thalamus was discovered in an 18-month-old Prim'Holstein heifer. Microscopical examination of the tumour showed large sheets of densely packed cells with occasional Homer-Wright and perivascular rosettes. Neoplastic cells were small with ill-defined borders, scant cytoplasm and ovoid, irregularly shaped nuclei. Immunolabelling was positive for vimentin and neuron-specific enolase, in agreement with previous reports of PNETs in human beings and animals. This appears to be the first report of cerebral PNET in cattle.

[Leptomeningeal gliomatosis. 2 clinicopathological cases]. / Gliomatose leptoméningée. Deux cas clinico-pathologiques.

Leptomeningeal gliomatosis is a diffuse glial infiltration of the subarachnoid space. It is primary and very rare when primary astrocytoma arises in the leptomeninges from heterotopic neuroglial tissue; it is secondary and more frequently reported when associated with a medullar or cerebral intraparenchymal astrocytoma and secondary involvement of the leptomeninges. Primary and secondary forms are difficult to differentiate before neuropathological examination. The authors report 2 anatomo-clinical cases of leptomeningeal gliomatosis in adults, with clinical courses of 6 months and 40 days respectively. The initial clinical picture was aseptic chronic or subacute meningitis. Cytologic examinations of the cerebrospinal fluid (CSF) showed moderate lymphocytosis, with elevated protein and low glucose levels, without abnormal cells. On case 2 CT scan and in case 1 spinal MRI isolated diffuse meningeal contrast enhancement was present, without intraparenchymal lesion. The neuropathological study revealed a diffuse astrocytoma glial leptomeningeal tumour with a focal involvement of the central nervous system (spinal cord in one case, temporal lobe in the other). In conclusion, an isolated aseptic lymphocytosis meningitis with meningeal abnormal signal may reveal leptomeningeal gliomatosis. Neuropathological examination can distinguish primary from secondary forms.

[Diffuse sclerosing papillary carcinoma of the thyroid. Report of a case]. / Carcinome papillaire sclérosant diffus de la thyroide. Présentation d'un cas.

The authors report the case of a 12-year-old girl presenting with a diffuse sclerosing papillary carcinoma of the thyroid. The diagnosis was made on a total thyroidectomy specimen three months after thyroid enlargement was detected. This child had previously been treated medically for thyroiditis because of an enlarged thyroid gland with serum antithyroid autoantibodies. A few cases have been described in the literature. Six morphologic findings define this variant of papillary carcinoma: a diffuse growth pattern involving one or both lobes of the thyroid, prominent fibrosis, heavy lymphocytic infiltration with germinal centers, large numbers of psammoma bodies, squamous metaplasia and papillae within cleft-like tissue spaces. Some authors stated that this variant has a poorer prognosis than the usual papillary carcinoma of the thyroid. However, in some series a few patients survive without local recurrence or distant metastasis at a mean follow-up period of more than 10 years. Immunohistochemical studies have shown high accumulation of S-100 protein positive dendritic cells. Such an infiltration has been correlated with a better prognosis.

[Multiple granular cell tumor of the colon]. / Tumeur à cellules granuleuses colique plurifocale.

With reference to a case of multiple colorectal granular cell tumors, the authors briefly review the literature concerning this unusual tumor localization. This case demonstrates the most common appendicular, caecal and rectal forms among the localizations of colonic granular cell tumors: as well as the possible proliferation of many other tumors especially in caecal localizations. However the course of the disease is slow, and at the present time only one case report of malignant colonic TCG has been published.

[Stomach leiomyoblastoma: contribution of cytology. Apropos of a case]. / Léiomyoblastome gastrique: apport de la cytologie. A propos d'un cas.

The authors report the case of a 51-year-old man with gastric leiomyoblastoma, presenting with a history of gastrointestinal bleeding and hemoperitoneum. CAT scan showed a mass measuring 10 x 10 cm arising from the stomach. At laparotomy the mass extended into the gastrosplenic omentum. A splenopancreatectomy was performed and the mass was excised for biopsy. The cytologic findings suggested a mesenchymal tumor; histology demonstrated the diagnosis of leiomyoblastoma. The postoperative course was uneventful. In the literature there is no evidence that intraperitoneal bledding from these tumors worsens survival. The cytopathologic features of leiomyoblastoma are discussed.

[Signet-ring cell adenocarcinoma involving the bladder and the rectum. Apropos of two cases]. / Adénocarcinome à cellules isolées envahissant la vessie et le rectum. Rapport de deux cas.

Two cases of primary signet-ring cell carcinoma involving the urinary bladder, with invasion of the rectum, are presented. Pelvic exenterations were performed, followed by a rapidly fatal outcome (6 and 7.5 months). These cases of signet-ring cell carcinoma of the bladder were reviewed with 33 previously reported cases in the literature. The clinical characteristics, histologic findings, pathogenesis, and effectiveness of aggressive treatment of this rare bladder malignancy are discussed.

[Intraluminal polyp of the common bile duct corresponding to a metastasis of cancer of the kidney]. / Polype intraluminal de la voie biliaire principale correspondant à une métastase de cancer du rein.

The authors report the second case to date of metastatic carcinoma of the common bile-duct from renal cell cancer presenting as an intraluminal polypoid mass. Obstructive jaundice developed in a 55-year old woman 14 years after nephrectomy for renal cell carcinoma. The diagnosis of polypoid tumor of the common bile-duct was established by sonography, endoscopic retrograde cholangiopancreatography and CT-scan. Palliative resection was performed. Postoperative histological examination revealed the resected tumor to be identical to the clear cell type of renal cell carcinoma. The postoperative course was marked by the development of distant metastases 6 months later. Diagnosis and therapeutic features of metastatic malignant biliary obstruction are discussed.

[Peritoneal amyloidosis in Waldenström's macroglobulinemia. X-ray computed tomographic aspects]. / Amylose péritonéale au cours d'une macroglobulinémie de Waldenström. Aspect tomodensitométrique.

A 74 years old man, know to have Waldenström's macroglobulinemia was hospitalized for intestinal obstruction. Upper gastrointestinal barium study revealed an extrinsic stenosis of duodenum. Ultrasonography and abdominal computed tomography revealed a diffuse mesenteric infiltration with medium level echoes and encasing of mesenteric vessels. Histologic examination of biopsies taken at exploratory laparotomy disclosed amyloidosis of the epiploon and mesentery. The review of amyloidosis in macroglobulinemia shows peritoneal amyloidosis to be a very rare event. Sonographic, computed tomographic and pathologic findings of this unusual case are discussed.

[Oncocytic tumors of the thyroid. Diagnostic and therapeutic problems. Report of 17 cases and review of the literature]. / Tumeurs oncocytaires de la thyroïde problèmes diagnostiques et thérapeutiques. Rapport de 17 cas et revue de la littérature.

Histopathologic and clinical follow-up data of 17 patients observed 3-104 months (mean: 38 months) after operation for oncocytic tumors of the thyroid gland are presented. Mean patient age was 50 +/- 15 years. Benign and malignant lesions were respectively 13 and 4. Frozen sections were positive for benign and malignant lesions in respectively 7 and 2 cases. Total thyroidectomy was performed in all cases of malignant lesions and in 7 cases of benign lesion, the latter in order to treat high volume adenomas or associated controlateral lesions. No tumor relapse was observed during the follow-up period (data about 16 patients). A review of the literature indicates that thyroid oncocytic adenoma diagnosis can be trusted and that this tumor is not especially prone to a malignant course with the mode of treatment applied. Thus total thyroidectomy should be recommended for: 1) malignant tumors. 2) and for selected benign adenomas depending on the tumor volume or on associated lesions.

[Cytological study in case of gastric leiomyoblastoma. Review of the literature]. / Examen cytologique en cas de léiomyoblastome gastrique. Revue de la littérature.

The authors describe the fine-needle aspiration cytologic features of leiomyoblastoma. Aspirated tumor cells occur singly, not in clusters. The tumor cells are globular in shape or sometimes have a spindle appearance. The nuclei are round, central or eccentric, with small nucleoli. The cells have well-defined cytoplasmic borders. The cytoplasm is abundant and, to some extent, granular, with sometimes a microvacuolar peripheral pattern. To date five cases of leiomyoblastoma with a cytological study have been published, two of them located to the stomach. A review of the literature is presented.