Search

Background. Esophageal squamous cell papillomas are rare epithelial lesions with malignant potential, diagnosed by upper gastrointestinal endoscopy. Their prevalence is from 0.01% to 0.43%. Objective. Description of rare esophageal squamous papilloma diagnosed during upper gastrointestinal endoscopy. Material and methods. A 38-year-old asymptomatic female patient underwent an upper gastrointestinal endoscopy. A growth described as an "esophageal polyp" was detected. The biopsy was performed and specimens were sent for histopathological study. Results. The final diagnosis of esophageal squamous papilloma was made due to histopathological study. Because of the malignant potential of these lesions, once diagnosed, early treatment is essential to prevent progression to esophageal carcinoma. Conclusions. Esophageal squamous papillomas are increasing in prevalence and have been shown to be potentially malignant. This argues the relevance of the problem and the need for widespread immunizations to control human papillomavirus infection.

espanolLa esclerodermia sistemica juvenil es una rara patologia cronica del tejido conectivo, cuyos sintomas comienzan, en promedio, a los 11 anos de edad. Tiene una alta morbilidad y mortalidad, y es una enfermedad sobre la que no existen consensos de trata-miento, por lo que se convierte en un reto para los especialistas que la manejan. Este trabajo describe un caso de esclerodermia juvenil sistemica difusa en una nina de un ano, diagnosticada y tratada de forma multidisciplinaria en el Hospital Pediatrico «Baca Ortiz» de Quito, Ecuador. Destaca la importancia de mantener una alta sospecha, para llegar a un diagnostico precoz, y realizar tratamientos farmacologicos y no farmacologicos para estabilizar la actividad de la enfermedad y mejorar la calidad de vida de las personas afectadas por esta enfermedad EnglishJuvenile systemic scleroderma is a rare chronic pathology of connective tissue, whose symptoms begin on average at age 11. It has a high morbidity and mortality, being a disease of which there is no consensus of treatment, so it becomes a challenge for the specialists who manage it. This paper describes a case of diffuse systemic juvenile cutaneous scleroderma in a one-year-old girl, diagnosed and treated in a multidisciplinary manner at the «Baca Ortiz» Children’s Hospital in Quito, Ecuador, which highlights the importance of maintaining a high level of suspicion, allowing early diagnosis, and carrying out pharmacological and non-pharmacological treatments to stabilize the activity of the disease and improve the quality of life of people affected by this disease.

espanolLa enfermedad de Rosai Dorfman es una entidad clinica sumamente rara caracterizadapor una proliferacion histiocitica diseminada. Usualmente afecta a adultos jovenes, presentandose conadenopatias multiples por lo que constituye un diagnostico diferencial de varias entidades infecciosas ylinfoproliferativas. A continuacion, presentamos un caso de esta enfermedad y una revision sobre el tema. Descripcion del casoUn paciente masculino de 32 anos presento adenomegalias generalizadas de forma recurrente acompanadasde fiebre, rash cutaneo episodico y toma del estado general. Al examen fisico las adenopatias tienden afocalizarse en la region supraclavicular y axilar. Se realizo estudios de laboratorio los cuales no son relevantessalvo por niveles exagerados de IgG contra toxoplasma. En tomografia se encontro proliferacion ganglionardiseminada a nivel cervical, toracico, axilar y abdominal. La biopsia de un ganglio supraclavicular derechodemostro la presencia de un infiltrado histiocitico con inmunohistoquimica positiva para S100, hallazgocompatible con la Enfermedad de Rosai Dorfman. ConclusionesLa enfermedad de Rosai Dorfman es una entidad poco estudiada en cualquier medio que corresponde a undiagnostico interesante en pacientes jovenes con adenomegalias generalizadas, por lo que su presencia debeser siempre considerada en casos atipicos o recurrentes como el expuesto en esta revision. EnglishRosai Dorfman’s disease is an extremely rare clinical entity characterized by a disseminatedhistiocytic proliferation. It usually affects young adults, presenting with multiple lymphadenopathy, whichconstitutes a differential diagnosis of several infectious and lymphoproliferative entities. Next, we presenta case of this disease and a review on the subject. Description of the caseA 32-year-old male patient presented recurrent generalized lymphadenopathy accompanied by fever,episodic skin rash and malaise. At examination he had several adenopathies located on the supraclavicular and axillary region. Laboratory studies were conducted which were not relevant except for exaggeratedlevels of IgG against toxoplasma. In tomography disseminated lymph node proliferation was found at thecervical, thoracic, axillary and abdominal levels. A biopsy of the right supraclavicular ganglion showed thepresence of a histiocytic infiltrate with positive immunohistochemistry for S100, a finding compatible withRosai Dorfman’s disease. ConclusionsRosai Dorfman disease is an often ignored entity which constitutes an interesting differential diagnosis inyoung patients with generalized adenomegalies, so its presence should always be suspected in any atypicalor recurrent cases such as the one presented in this review.

A gastrointestinal stromal tumor (GIST) with signet ring cell features is a rare variant of epithelioid GIST. The current case demonstrates a 35-year-old woman with a 22.0 cm stomach mass. Tomography-guided core biopsy of the mass showed an undifferentiated tumor with abundant signet ring cells in a myxoid background. A preliminary diagnosis of adenocarcinoma was considered based on histomorphologic features; however, by immunohistochemistry studies the tumor cells were negative for cytokeratins and intensely positive for CD117/c-kit and CD34. Therefore a diagnosis of GIST with signet ring-like cells features was rendered. Making a diagnosis in a small biopsy specimen is always challenging, due to the variable histomorphological features of these tumors.