Biomedical subjects
Publications and source records attributed to B Gorguet.
Immunoperoxidase using monoclonal antibodies may be applied to paraffin sections previously stained. The precise nature of cells found by routine staining may thus be investigated. It also enables, with small biopsies, immunohistochemical identification of a tissue zone lost by serial sectioning.
The clinical and pathological features of 14 cases of Merkel cell carcinoma are reported. They commonly arise in the skin of elderly patients, particularly on the face and pelvis. They have a loco-regional aggressivity (nodal metastases in 4 cases) but some patients die with disseminated metastases (2 cases). These tumors are composed of round cells with scanty cytoplasm, arranged in solid or trabecular sheets. Mitotic figures are usually numerous. The ultrastructural study reveal secretory granules and paranuclear collection of intermediate filaments. Immunohistochemical phenotype is highly characteristic: cytoplasmic diffuse positivity with an anti-neuron-specific enolase polyclonal antibody; polar and/or diffuse positivity with anti-cytokeratin, anti-epithelial membrane antigen and anti-S100 protein monoclonal antibodies; polar positivity with an anti-neurofilament monoclonal antibody. The negativity with an anti-common leucocyte antigen monoclonal antibody is helpful to differentiate Merkel cell carcinoma from cutaneous malignant lymphoma.
Sanchez has recently described a new entity characterized by the presence of papules bilaterally located on the aural pinnae. The four presented cases concerned women. Clinical picture of the disease is, without any doubt, unusual but "hyalin" masses are non-specific since they are similar to amyloidosis on account of thioflavine T positivity and microfilamentous aspect in electron microscopy. We reported at the Meeting of the French Dermatological Society (Toulouse, November 10, 1981) a very similar case under the name of "Hyalinose micro-papuleuse de la conque des oreilles avec élimination trans-épidermique". In the present paper we report this personal case, and we point out some original findings: pigmentary changes, trans-epidermal elimination of the hyalinized findings material and presence of an inflammatory process.
Multinucleate giant cells resembling Warthin-Finkeldey cells have been described in various lymphoid disorders. These Warthin-Finkeldey-like cells (WFLC) with as many as 50 nuclei are of three main types: reticular, lymphocyte and intermediary. In reactive lymphoid proliferations (34 cases) WFLC were mainly observed inside germinal centres and to a lesser extent in the interfollicular zones. In neoplastic lymphoid proliferations (33 cases) WFLC were most commonly found in the lymphocytic predominance type of Hodgkin's disease (16/25 cases). All non-Hodgkin's lymphomas (13 cases) in which WFLC were detected proved to be of low grade malignancy (lymphocytic: one case, lymphoplasmacytic-plasmacytoid: six cases; and centroblastic-centrocytic, six cases). They were also found in two cases of angioimmunoblastic lymphadenopathy. Immunoperoxidase and electron microscopic studies could not elucidate the exact histogenesis of these cells, but it is assumed that they are associated with B cell proliferations.
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The authors report a case of glucagonoma in a 52 years old man presenting a migratory necrolytic erythema. By conjugated means of arteriography and splenoportography with plasma glucagon assays the tumour was localized in the tail of the pancreas. Surgical excision was easy but hepatic metastases revealed the malignant nature of the tumor. This glucagonoma has been investigated by several approaches including electron microscopy, immunocytochemistry and radioimmunological techniques. The tumor contained scattered glucagon and pancreatic polypeptide immunoreactive cells; insuline, glucagon, somatostatin, pancreatic polypeptide, gastrin and VIP antisera gave negative results. Ultrastructurally, these cells showed atypical secretory granules different from A granules of the normal glucagon cell. Radio immunological determinations carried out after gel permeation chromatography of plasma revealed high molecular weight (4 000, 9 000, 14 000) immunoreactive glucagon peptides. They have been thought to be proglucagon forms which did not react with specific antiglucagon sera used in cytological studies. Reported data are consistent with the classification of this tumor in the category of glucagonoma with the "glucagonoma syndrome".
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The authors report 9 cases with pseudo-lymphomatous lesions associated with dysimmunitary features. They discuss the correlations between these cases and similar entities, for instance angio-immunoblastic lymphadenopathy (LAID). It seems that all these anatomoclinical syndromes could be referred to as dysimmunitary and pseudolymphomatous adenopathies (ADPL). The first type--ADPL type I--rich in immunoblasts and plasmocytes is defined. LAID is the most common form. The term "dysimmunitary" reflects not only the biological disturbances which accompany these lesions, but also their possible physiopathological mechanism, type I ADPL being apparently due to problem chiefly affecting humoral immunity.
Besides the dysimmunitary and pseudo-lymphomatous adenopathies rich in immunoblasts and plasmocytes (ADPL type I) five cases showed similar clinical and biological data but with frequently otorhinolaryngologic location. The lesions are characterized by important structural changes and abundant epithelioid cells are comparable to Lukes' type III immunoblastic lymphadenopathies and to Lennert's lymphoepithelioid lymphomas. They must be distinguished from Hodgkin's granulomas which are rich in epithelioid cells. They are perhaps due to a disturbancy bearing mainly on the cellular immunity.
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BACKGROUND: Brooke-Spiegler syndrome is an association of multiple trichoepitheliomas and cylindromas, sometimes accompanied by other adnexal tumors. CASE REPORT: A 44-year-old woman with trichoepitheliomas involving the naso-genal and mental areas associated with cylindromas and spiradenomas on the forehead and pretragal regions creating a turban effect. Other complete or diassociated syndromes were found in family members. No neoplastic tumor was identified. DISCUSSION: Brooke-Spiegler syndrome is an hereditary disease with autosomal dominant transmission. Both benign and malignant neoplasias can be associated. The concomitant existence of different tumors could be helpful in understanding the pathophysiology. There is some debate about the exact origin of the trichoepitheliomas, cylindromas and spiradenomas. Several single-cause theories have been put forward but remain to be confirmed as the genetic anomalies identified for trichoepitheliomas and cylindromas map to different sites. Patients with Brooke-Spiegler syndrome should be explored for malignant neoplasia. A family study is indicated.