Blue Rubber Bleb Nevus Syndrome Diagnosed Prenatally as an Epignathus.

The authors present a clinical report of the giant fetal tumor protruding from the oral cavity diagnosed sonographically at 32 weeks of gestation as an epignathus. After delivery, tumor proved to be a presentation of the blue rubber bleb nevus syndrome. To the best of our knowledge, the literature offers no reports on similar cases.

Congenital dermoid cyst of the tongue.

True dermoid cysts of the oral cavity are rare, usually presenting as midline swellings in the floor of the mouth and occasionally elsewhere in the oral cavity. This report describes the diagnosis and management of a congenital dermoid cyst of the lateral aspect of the body of the tongue in a 10-year-old girl. The theories of the pathogenesis of this cyst during embryogenesis of the tongue are also reviewed.

[Ectopic lingual thyroid. Apropos of 3 cases]. / La thyroïde ectopique linguale. A propos de trois cas.

Lingual thyroid is a rare cause of upper airway obstruction, dysphagia or hypothyroidism symptoms. This report describes three cases of lingual thyroid arising in women. One was in pregnancy and developed a lingual goiter and hemorrhages with hypothyroidy. The second case have been diagnosed because of a dysphagia and the third was asymptomatic and have been diagnosed during physical examination for cervical nodes. Diagnosis and possible therapeutic options are discussed regarding these three cases. Surgical therapy is appropriate for patients with clinical signs of upper airway obstruction or when malignant degeneration is suspected. Without of clinical sign, substitutive therapy with thyroid hormone allows the stabilization or the regression of the ectopic thyroid.

Adenocarcinoma de base de lengua de origen tiroideo / Adenocarcinoma of base of tongue with thyroideal origin

Describimos el caso de un paciente de 49 años de edad que desarrolló un adenocarcinoma de base de lengua originado en tejido tiroideo, es de hacer notar y esto es lo que da al caso características excepcionales, que el enfermo tenía su glandula tiroides sana y ubicada normalmente en la base del cuello. El origen de este carcinoma debió haber estado en el tejido tiroideo, que en más del 10 por ciento de la población existe en los alrededores del foramen coecum en el vértice de la "V" lingual de la base de la lengua. El enfermo fue tratado por nosotros quirurgicamente en junio de 1981, en el Hospital de Clínicas de Montevideo, el (número de Registro de historia clínica 468.216) en 3 actos quirúrgicos: El primero en junio de 1981 traqueotomía y biopsia de la tumoración por la vía transoral (faringotomía transoral) según técnica de Sorensen, la anatomía patológica de esta primera biopsia nos informó de la existencia de un adenocarcinoma. El segundo acto quirúrgico fue realizado para el tratamiento en vista de la resección quirúrgica de la lesión, que no tenía adenopatías en el cuello y que se abordó por la vía mediana de Trotter (faringotomía mediana trans-labio-mandibulo-lingual) en esta oportunidad el estudio completo anatomopatológico mostró la existencia de un adenocarcinoma de origen tiroideo en el cual se pudieron apreciar folículos recordando la morfología tiroidea; los estudios histoquímicos posteriores confirman el diagnóstico de tumor originado en tejido tiroideo. La glándula tiroide del paciente era normal. En 1982 se constata en un control de rutina recidiva de la lesión pero sobre el borde izquierdo de la base de la lengua, se procedió al abordaje de la lesión por faringotomía lateral transmaxilar izquierda (tercer acto quirurgico). El paciente está libre de lesión en enero de 1988 (fig. 8) y lo sigue estando en diciembre de 1994. Realizamos control periódico del paciente que deberemos observar aún durante años

Embryogenesis of enterocystomas-enteric duplication cysts of the tongue.

Enteric duplication cysts of the tongue are unusual lesions that may be confused with dermoid cysts, hemangiomas, lingual thyroid remnants, ranulas, and cystic hygromas. Two cases of lingual enteric duplications are reported in a 5-year-old boy and a 4-month-old boy. In the first case the cyst was lined by gastric-type epithelium, and in the second by colonic-type epithelium. Theories of pathogenesis of enteric duplications include development from small epithelial inclusions trapped during fusion of primordial tissues, from incomplete coalescence of lacunas that form between epithelial cells of the solid core of the developing gut, from persistence of epithelial buds within the wall of the bowel, or from nests of trapped entodermal cells. However, each of these theories presents problems with respect to lingual enteric cysts, as possible trapping of epithelium by fusing primordia does not explain the presence of heterotopic mucosa, and the tongue does not develop in the same manner as the hollow viscera. It becomes apparent therefore that enteric duplication cysts are a heterogeneous group of lesions that share some morphologic features, but perhaps not the same pathogenesis.

Lingual thyroid and associated epiglottitis.

A relatively rare genetic anomaly, lingual thyroid has a reported incidence of 1:100,000. Histologically, it may consist of normal or fetal-type tissue, and may show lymphoid infiltration. Differential diagnosis should include consideration of various benign and malignant diseases. We review diagnostic procedures as well as medical and surgical management, and present an illustrative case of lingual thyroid with associated epiglottitis.

[Dermoid cyst of the tongue]. / Le kyste dermoïde de la langue.

An epidermoid cyst of the tongue with sinus tract is discussed. Its interest comes from the fact that histologic sections show sinus tract wall and epidermoïd cyst wall made with the same major characteristics of skin structures. Both have therefore the same embryologic origin. Adequate treatment is surgical excision.

Enterocystoma of the head and neck.

Enterocystomas of the head and neck are exceedingly rare lesions; only 21 cases have been reported previously. We describe a case of a newborn infant with an enterocystoma of the anterior tongue. All of the previously reported cases and the three theories of embryogenesis are reviewed. Diagnostic evaluation includes computerized axial tomography and radioiodine thyroid scan, particularly if the tongue is involved. Complete surgical excision is the only appropriate therapy and is curative.