Enterogenous cysts are rare, benign lesions involving the spinal canal or the cerebellopontine angle. Typically they present with compression of the spinal cord or cranial nerves. They are usually 1 cm or less in size and are typically lined by columnar mucin-producing epithelium resembling enteric epithelium or columnar ciliated epithelium resembling respiratory epithelium. The case records of the Division of Neuropathology at Indiana University were reviewed for cystic lesions of the spinal canal and cranial cavity for a 26-year period. Seven surgically resected enterogenous cysts were identified. The original slides were studied. Additional sections were stained with mucicarmine, and immunohistochemical stains including thyroid transcription factor 1, epithelial membrane antigen, and cytokeratin 5/6 (CK5/6) were performed. Electron microscopy was performed in 1 case. The cysts ranged in size from 4 to 10 mm. One had stratified cuboidal epithelium, 1 had ciliated columnar and stratified squamous epithelium, 1 had columnar mucinous epithelium, and 4 had ciliated columnar epithelium. Epithelial membrane antigen and CK5/6 positivity were observed in all cases. Strong CK5/6 positivity was seen in the basal cells, with little or no immunoreactivity in the ciliated cells. Mucicarmine positivity was observed in 4 cases, and thyroid transcription factor 1 positivity was observed in 2 cases. Electron microscopy demonstrated well-developed stereocilia, distinct basal cells, and a thin basement membrane. The histopathologic, ultrastructural, and immunologic findings in these lesions are similar to those present in the bronchial epithelium, supporting the hypothesis of endodermal origin for these lesions.
[Show abstract][Hide abstract] ABSTRACT: RESUMEN El quiste enterógeno o entérico es una lesión benigna muy poco frecuente, que afecta principalmente la médula espinal y el ángulo ponto-cerebeloso. Se caracteriza por signos secundarios de compresión a los nervios craneales. Son lesiones quísticas que miden menos de 1 cm de diámetro. Están revestidos por una sola capa de células columnares muco-productoras. El epitelio se semeja al epitelio entérico o al respiratorio. Se comunica el caso de una hombre de 58 años de edad que inició con cefaleas occipitales, náuseas, vértigo y posteriormente alteraciones visuales. La resonancia magnética y la TAC craneal mostraron lesión quística en el ángulo pontocerebeloso que sugería neurocisticercosis quística. Recibió tratamiento con albendazol (30 mg/kg) y esteroides sin mejoría clínica, por lo que se sometió a cirugía con resección total de la lesión mediante un abordaje retromastoideo. El paciente tuvo mejoría clínica importante después de la cirugía. Histológicamente se diagnosticó como quiste entérico y las tinciones de AA, PAS y mucicarmín fueron positivas. Por inmunohistoquímica fue positivo para antígeno de membrana epitelial, citoqueratinas 8 y 5/6. La histología, inmunohistoquímica y microscopia electrónica del quiste entérico son similares al epitelio bronquial, por lo que se sugiere que las lesiones sean de tipo endodérmico. Alteraciones embrio-Palabras clave: quiste entérico, lesiones quísticas cerebrales. ABSTRACT Enterogenous cysts are rare, benign lesions involving the spinal canal and the cerebellopontine angle. Usually they present compression of the spinal cord or cranial nerves. These lesions are 1 cm or less in size and are usually lined by columnar mucin-producing epithelium resembling enteric epithelium or columnar ciliated epithelium resembling respiratory epithelium. We report the case of a 58-year-old male with occipital cephalea, visual disturbance and vertigo. MRI and CAT imaging showed a cerebellopontine angle enhancement suggested cyst neurocisticercosis. He was treated with albendazol (30 mg/kg) and steroids by two years without clinical improvement. Near total ex-cision of the cyst was carried out through the retro mastoid route. Patient made a good postoperative recovery. Histologically corresponded to an enteric cyst, was stained with AA, PAS and mucicarmine positive; and immunohistochemical stains including: epithelial membrane similar to those present in the bronchial epithelium, supporting the hypothesis of endodermal origin for these lesions. Cord malformations derive from defects in these early embryonic stages, and are collectively called spinal dysraphisms.
[Show abstract][Hide abstract] ABSTRACT: Mucus-secreting adenomas or acinar adenoma of the choroid plexus are very rare. We report the case of a 79-year-old male with a 3-year history of occipital headaches with vomiting, ataxia and cerebellar signs. He was first seen due to difficulty while walking. He was admitted to the hospital with significant tumor expansion and clinical deterioration. CT and MRI revealed obstructive hydrocephalus secondary to a large fourth ventricular cyst mass, which enhanced markedly on contrast administration. Pathological findings were consistent with an acinar choroid plexus adenoma. The tumor was attached to the ependymal lining and was strongly adhered to the walls and floor of the IV ventricle. Post-operative bleeding complicated partial removal of this tumor. The patient died 6 h after surgery.
Journal of Neuro-Oncology 07/2007; 83(2):191-7. DOI:10.1007/s11060-006-9304-0 · 3.07 Impact Factor
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