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1.
J Clin Pathol ; 47(10): 959-61, 1994 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-7962613

RESUMO

Glandular tumours involving the middle ear and the mastoid are rare, and distinguishing between adenoma and adenocarcinoma is difficult. Two distinct histopathological patterns are accepted. While their clinical presentation differs, both require primary surgical treatment and both have a high rate of local recurrence. The papillary form takes a more aggressive course and wider regional spread. This pattern occurs predominantly in women. Its microscopic appearance and clinical course have been extensively described by Heffner who considered it as "low-grade adenocarcinoma of probable endolymphatic sac origin". A few cases have been associated with von Hippel-Lindau disease. The case of a 32 year old black woman is described. It is suggested that papillary adenocarcinoma of the endolymphatic sac should be considered in the spectrum of neoplasms seen in von Hippel-Lindau disease.


Assuntos
Adenocarcinoma Papilar/complicações , Saco Endolinfático , Doença de von Hippel-Lindau/complicações , Adenocarcinoma Papilar/patologia , Adulto , Feminino , Humanos , Doenças Vestibulares/complicações , Doenças Vestibulares/patologia , Doença de von Hippel-Lindau/patologia
2.
Ann Pathol ; 16(4): 271-5, 1996 Sep.
Artigo em Francês | MEDLINE | ID: mdl-9172616

RESUMO

Glandular tumors involving the mastoid and the middle ear are rare, and distinguishing between adenoma and adenocarcinoma remains difficult. Among these latter lesions, two distinct patterns are accepted. One of them, the papillary form takes a more aggressive course with wider regional spread and must be separated from the other type, the middle ear carcinoma. Its microscopic appearance and clinical course have been extensively described by Heffner who considered it as "low-grade adenocarcinoma of probable endolymphatic sac origin". A few cases have been associated with von Hippel-Lindau disease. Three cases of papillary adenocarcinoma of endolymphatic sac origin are reported. One concerned an isolated tumor, the two others were associated with von Hippel-Lindau disease. Their clinical, pathological and immunohistochemical data are presented.


Assuntos
Adenocarcinoma Papilar/patologia , Saco Endolinfático/patologia , Imuno-Histoquímica , Doenças Vestibulares/patologia , Adenocarcinoma Papilar/química , Adulto , Idoso , Saco Endolinfático/química , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Doenças Vestibulares/metabolismo
4.
Acta Neuropathol ; 89(1): 99-104, 1995.
Artigo em Inglês | MEDLINE | ID: mdl-7709738

RESUMO

We describe a case of a desmoplastic brain tumor which was initially resected from the right fronto-temporal region in a 2 year-old boy. This nodular, calcified tumor was vascularized by the internal carotid artery and the middle meningeal artery branches. Grossly, it contained several mucoid cysts. Light microscopy showed cords or nests of small cuboidal cells surrounded by a loose connective tissue and desmoplastic areas containing fibers and spindle cells. The cuboidal cells expressed epithelial, neuronal and neuroendocrine markers. Some foci of spindle cells showed glial differentiation. The tumor recurred 16 months later and displayed some characteristics of the small cell neuroepithelial component, mitoses being conspicuous. Electron microscopy revealed undifferentiated clear cells, some containing neurosecretory granules. Karyotyping demonstrated the following formula: < 15 > 46, t(8;11) (q13; q11). The chromosome 11 breakpoint was different from that described in Ewing's sarcoma. This isolated translocation has not been previously reported to our knowledge. These unusual features lead us to report this case and to discuss its pathogenesis.


Assuntos
Neoplasias Encefálicas/imunologia , Anticorpos , Astrócitos/ultraestrutura , Encéfalo/patologia , Neoplasias Encefálicas/genética , Neoplasias Encefálicas/ultraestrutura , Pré-Escolar , Epitélio/ultraestrutura , Humanos , Imuno-Histoquímica , Imageamento por Ressonância Magnética , Masculino
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