Tumor de células granulares de laringe en el niño: Caso clínico / Granular cell tumor of the larynx in the child: Case report

Los tumores de laringe son poco frecuentes en los niños. Representan el 2% de las anomalías laríngeas. El 98% son benignos; la papilomatosis respiratoria recurrente y el hemangioma son los más comunes. El tumor de células granulares, también llamado tumor de Abrikossoff, es una neoplasia benigna inusual y, más aún, en la localización laríngea. Las manifestaciones clínicas dependen del tamaño y la ubicación de la masa tumoral; el síntoma de presentación principal es la disfonía. El diagnóstico de certeza lo proporciona la biopsia. El tratamiento de elección es la cirugía. Se presenta a una niña de 9 años con disfonía y disnea de esfuerzo debidas a un tumor de células granulares laríngeo y se destaca la importancia de considerar la evaluación endoscópica de la vía aérea en todo niño con disfonía progresiva o persistente a fin de determinar la lesión causal.

Laryngeal tumors are uncommon in children, accounting only for 2% of the laryngeal anomalies. Ninety-eight percent are benign; the most frequent ones are recurrent respiratory papillomatosis and haemangioma. Granular cell tumor, also called Abrikossoff tumor, is an unusual benign neoplasm, especially in the larynx. Clinical manifestations depend on the size and location of the tumor. Dysphonia is the main presenting symptom. The diagnosis is confirmed by the biopsy. The treatment of choice is surgery. We present a 9-year-old girl with dysphonia and exertion dyspnea due to a granular cell tumor of the larynx, and we emphasize the importance of considering the endoscopic evaluation of the airway in every child with progressive or persistent dysphonia in order to determine the etiology.

Tumeur d'Abrikossoff de l'estomac : une localisation rarissime

La tumeur d'Abrikossoff ou tumeur à cellules granuleuses est une lésion rare, souvent unique et bénigne. L'âge de son diagnostic habituellement est compris entre 40 et 60 ans. Elle prédomine légèrement chez les femmes, notamment de race noire. Elle est de siège ubiquitaire et préférentiellement cervico-céphalique. La langue reste la localisation la plus fréquente avec 28% des cas. Les autres localisations sont beaucoup moins fréquentes. Nous rapportons le premier cas documenté au Congo Brazzaville, de localisation gastrique, suivi d'une revue de la littérature

Tumor de células granulares bifocal em mucosa jugal / Tumor de células granulosas bifocal en mucosa bucal / Granulous cell bifocal tumor in the oral mucosa

O tumor de células granulares é uma lesão incomum que apresenta predileção pela cavidade oral, por apresentar baixa taxa de recidiva, o tratamento de escolha é a excisão cirúrgica simples. O objetivo caracterizar um caso de tumor de células granulares bifocal em mucosa jugal e explanar características clínicas e histopatológicas acerca da lesão. Relata-se o caso de uma paciente de 60 anos apresentando dois pequenos nódulos de superfície lisa em mucosa jugal, consistência fibroelástica e coloração levemente amarelada. Foi realizada a excisão cirúrgica das duas lesões a partir de uma biópsia excisional, onde microscopicamente foi observada uma neoplasia de células granulares arranjadas em ilhas, chegando ao diagnóstico de Tumor de células granulares. O diagnóstico final da lesão foi obtido a partir do exame histopatológico, visto que, a aparência clínica da lesão é inespecífica, sendo de extrema importância a realização da biópsia excisional. A paciente continua em acompanhamento há 6 meses e não demonstra recidiva da lesão(AU)

The granulosa cell tumor constitutes a rare disease that predominates in the tongue and has a low rate of recurrence, simply by surgical removal. The objective is to characterize a case of granulosa cells bifocal tumor in the oral mucosa with some clinical and histopathologic features to this injury. A 60-year-old patient presented with two small nodules in the smooth surface, consistency and slightly yellowish fibroelastic. These lesions were removed by excisional biopsy. From the microscopic point of view, it was demonstrated that there was a neoplasia with granule cells in some areas and the diagnosis was granular cell tumor. The definitive diagnosis of the lesion is obtained by histopathology, as the clinical appearance of the lesion is nonspecific, so it is very important to perform the excisional biopsy. The patient remains under follow-up for six months and shows no recurrence(AU)

El tumor de células granulosas es una enfermedad rara que predomina en la lengua y tiene una baja tasa de recurrencia; se trata mediante la extirpación quirúrgica simple. El próposito es caracterizar un caso de tumor de células granulosas bifocal en la mucosa bucal con algunas características clínicas y histopatológicas de esta lesión. Se presenta un paciente de 60 años con dos pequeños nódulos de superficie lisa, consistencia fibroelástica y ligeramente amarillento. Estas lesiones fueron extirpadas por biopsia excisional. Desde el punto de vista microscópico se demostró una neoplasia con células granulares en islas y se llegó al diagnóstico de tumor de células granulares. El diagnóstico definitivo de la lesión se obtiene de la histopatología, pues el aspecto clínico de la lesión es inespecífica, por lo que resulta muy importante la realización de la biopsia excisional. El paciente continúa bajo seguimiento durante 6 meses y no muestra la recurrencia(AU)

Granular Cell Epulis of a Newborn Male Child.

Congenital granular cell epulis is an uncommon benign tumor arising from alveolar ridges of the newborn sometimes interfering with mouth closure and feeding and when multiple the tumor may cause respiratory obstruction. It is mostly composed of nests of cells with granular cytoplasm set in a prominent vasculature. Here we report a case of CGCE observed in a newborn male due to its rare occurrence, with a brief review of literature pertaining to its histogenesis and differentiation from its adult counterpart.

Clinical and histopathological features of orbital granular cell tumor: case report / Características clínicas e histopatológicas de tumor de células granulares da órbita: relato de caso

A 53 year-old woman presented with a slowly progressive, painless proptosis OS. Computed tomography disclosed a round, homogeneous, well-delimited lesion in the inferior-temporal orbit. The tumor was composed of round cells with eosinophilic granular cytoplasm. Some of the cells had larger eosinophilic granules surrounded by a clear halo; known as pustulo-ovoid bodies of Milian or Bangle bodies. The diagnosis of a granular cell tumor was then established and confirmed by immunohistochemistry. Granular cell tumors are uncommon benign soft tissue neoplasms that have a predilection for the head and neck region. Awareness of the typical histopathological features is crucial for the correct diagnosis.

Mulher de 53 anos apresentou proptose lentamente progressiva no olho esquerdo. Tomografia computadorizada mostrou uma lesão na região temporal inferior da órbita esquerda, bem delimitada, arredondada, homogênea. O tumor era composto de células com citoplasma granular eosinofilico. Algumas das células possuíam grandes grânulos eosinofílicos circundados por um halo claro, conhecidos como corpos ovoides-pustulares de Milian or corpos de Bangle. O diagnóstico de tumor de células granulares foi estabelecido, confirmado pela imuno-histoquímica. Tumor de células granulares são neoplasias incomuns com predileção da região da cabeça e pescoço. O conhecimento das características histopatológicas típicas são cruciais para o correto diagnóstico.

Tumor cutáneo de células granulares en la infancia: presentación de un caso / Childhood cutaneous granular cell tumor: case report

El tumor de células granulares (TCG) es una neoplasia benigna de diferenciación distintiva a la microscopia de luz caracterizada por la presencia de células con citoplasma eosinofílicoabundante y granular. Los informes en niños son extremadamente raros. Se presenta el caso de un paciente de sexo masculino, de 11años de edad, con una lesión nodular de seis meses de evolución, que aumentó progresivamente de tamaño, asintomática, localizada en la espalda. A la exploración se evidencia un nódulo oval, de 1,5 cm de diámetro, móvil, no adherido ni doloroso. El estudio histológico muestra lesión tumoral no encapsulada, formada por células de gran tamaño, de abundante citoplasma ocupado por gránulos eosinofílicos y núcleos de localización central. El estudio inmunohistoquímico señala positividad para S100. Se indica como tratamiento la resección quirúrgica local conservadora. Destacamos la necesidad de biopsiar nódulos solitarios ulcerados persistentes en el tiempo, para tener certeza diagnóstica y resaltamos la importancia de la histopatología, ya que el diagnóstico clínico es difícil y casi nunca se piensa en él, salvo que la lesión se presente en la lengua.

Ictericia obstructiva secundaria a tumor de células granulares de la vía biliar extrahepática / Obstuctive jaundice secondary to extrahepatic bile ducts granular cell tumor

Bile ducts granular cell tumor is a rare entity. Of neural origin, mostly benign, may, however, present mimicking malignancy. We report a 32 years old female presenting with painless jaundice and extrahepatic bile ducts stenosis confirmed with MRC. Extrahepatic bile ducts resection is performed. Reconstruction involves four independent ducts to a Roux en Y enteric loop. She has a good postoperative outcome, with no evidence of complications nor recurrence at 17 months of follow up.

El tumor de células granulares en la vía biliar es una neoplasia rara de origen neural, en su mayoría benigna y cuya presentación puede sugerir patología maligna. Objetivo: Se presenta el caso clínico, características anatomopatológicas, manejo y evolución de una paciente joven que se presenta con ictericia obstructiva por estenosis subcarinal biliar. Paciente y Método: Paciente 32 años, sexo femenino, con ictericia, coluria y prurito. Diagnóstico de estenosis biliar y dilatación de vía biliar intrahepática se confirma con colangiorresonancia magnética. Se realiza resección de vía biliar extrahepática desde supracarinal que incluye vía biliar distal. Reconstitución bilioentérica a Y de Roux que involucra cuatro conductos intrahepáticos. Evoluciona en forma satisfactoria en el postoperatorio. El seguimiento alejado a 17 meses revela una satisfactoria condición de la paciente, sin signos de complicación o recidiva. Conclusión: El manejo por un equipo de experiencia multidisciplinario nos permitió ayudar a una paciente con rara patología, benigna en lo histológico, pero que puede representar un gran desafío técnico.

Tumor de células granulares acometendo a órbita em uma criança / Granular cell tumor with orbital involvement in a child

Os autores relatam um raro caso de tumor de células granulares no músculo reto medial de um menino de sete anos de idade. São descritos os achados clínicos, histológicos e radiológicos do caso, bem como uma breve revisão da literatura.

The authors report a rare case of granular cell tumor in the left medial rectus muscle of a seven-year-old boy. Clinical, pathologic and radiologic findings of the present case are described and a brief literature review is undertaken.

Gingival Granular Cell Lesion-Congenital Epulis.

Gingival granular cell tumour or congenital epulis is a rare benign soft tissue tumour that occurs mostly over lateral alveolar ridge of maxilla. The lesion behaves in a benign manner with spontaneous regression and without any recurrence or metastasis. We hereby report a case of congenital epulis in a 5 weeks old, baby girl who presented with single sessile mass over right side of maxillary alveolar ridge since birth; and want to emphasize that although rare this entity should always be considered in differential diagnosis of neonatal lesions of the oral cavity.

Congenital epulis.

Congenital epulis of the newborn is a very rare and unique tumor first described in 1871 by Neuman. It has a female predilection. It is a tumor with no tendency to recur after excision. The histogenesis of the lesion is unknown, but it is believed to be of mesenchymal origin. We report a 2-day-old female with tumor mass on the anterior mandibular alveolar ridge, which demonstrated immunoreactivity for vimentin, S-100 and neuron-specific enolase; thus, suggesting a similar histogenesis with granular cell tumor.

Tumor de células granulares de esófago / Tumor of granular cells of esophagus

Los tumores de células granulares son lesiones raras y asintomáticas, generalmente un hallazgo incidental en la endoscopia alta o baja. Fueron descritos por primera vez en 1926 por Abrikossoff. Las localizaciones más frecuentes son la mucosa bucal, la dermis y el tejido celular subcutáneo, la mayor parte de estos tumores tienen una naturaleza benigna. Presentamos el caso clínico de una mujer, de 44 años con historia de pirosis desde hace aproximadamente 1 año, se observa por endoscopia superior lesión elevada de 8 mm en tercio distal de esófago y se confirma por estudio histológico el diagnóstico de tumor de celulas granulares. El tratamiento de elección de esta lesión es la polipectomía endoscópica. A pesar de que el potencial maligno es escaso se sugiere seguimiento estrecho clínico y endoscópico

Granular cells tumors are rare and asymptomatic lesions and by general, it is an incidental finding en high or low endoscopy. They were described for the first time by Abrikossoff in 1926. The more frequent locations are the buccal mucosa, dermis and subcutaneous cellular tissue, most of these tumors has a benign origin. This is the case of a woman aged 44 with a pyrosis history from a year ago; by high endoscopy it is noted a 8mm lesion distal to esophagus and confirmed by histological study of granular cells tumor. Elective treatment of this lesion is the endoscopic polypectomy. Despite that the malign potential is low; we suggested a close clinical and endoscopic follow-up

Granular Cell Tumor of the Descending Colon Treated by Endoscopic Mucosal Resection: A Case Report and Review of the Literature

Although colorectal granular cell tumors (GCTs) are rare, their incidental finding has increased as the use of diagnostic colonoscopy has become more common. Here we describe the case of a 41-yr-old man with a GCT in the descending colon that was detected after a screening colonoscopy. Endoscopic examination revealed a yellowish submucosal tumor, 13x12 mm in diameter, in the descending colon. Endoscopic mucosal resection (EMR) followed by histological examination revealed that the tumor was composed of plump histiocyte-like cells with an abundant granular eosinophilic cytoplasm and small round nuclei. The tumor cells expressed S-100 protein and stained with periodic acid-Schiff, but were negative for desmin and cytokeratin. The resected tumor was diagnosed as a GCT. Colonoscopists should consider the possibility of GCT in the differential diagnosis of yellowish submucosal tumors of the colon. In such patients, EMR seems to be a feasible and safe approach for diagnosis and treatment.

Tumor de células granulares: relato de caso em uma localização incomum / Granular cell tumor: case report in an uncommon location

O tumor de células granulares é uma neoplasia benigna relativamente rara que pode ocorrer em qualquer parte do corpo, sendo que na cavidade oral a língua é o local mais comumente afetado. Geralmente são nodulares, únicos, bem delimitados, assintomáticos, de crescimento lento, mais freqüente no gênero feminino durante a quarta e sexta décadas de vida. Histologicamente é caracterizado por uma proliferação de células poligonais de aspecto granular que se agrupam em ninhos, cordões ou lençol e apresentam uma forte marcação imunoistoquímicamente para a proteína S-100, podendo também estar presente uma hiperplasia pseudo-epiteliomatosa. O tratamento de escolha é a sua completa remoção cirúrgica e o prognóstico em geral é bom, com raras recidivas, entretanto, casos de agressividade local e transformação maligna são relatados na literatura. O objetivo deste trabalho foi relatar um caso clínico de tumorde células granulares localizado em um sítio incomum, mostrando seu aspecto clínico, histopatológico, imunoistoquímico e formas de tratamento.

Granular cell tumor is a relatively rare, benign neoplasia that can occur in any part of the body, and in the oral cavity, the tongue is the location most commonly affected. They are generally nodular, single, well delimited, asymptomatic, slow growing, and most frequent in women during the fourth and sixth decades of life. Histologically it is characterized by a proliferation of polygonal cells with a granular appearance, which group themselves in nests, cords or sheets and present a strong immunohistochemical marker for S-100 protein, and a pseudoepitheliomatous hyperplasia can also be present. The treatment of choice is its complete surgical removal and in general, the prognosis is good, with rare recurrences, however cases of local aggressiveness and malign transformation have been related in the literature. The aim of this article was to relate a clinical case of granular cell tumor located in an uncommon site, showing its clinical, histopathologic and immunohistochemical aspects and forms of treatment.

Tumores mesenquimático del esófago / Mesenchymal tumor of the esophagus

Antecedentes: la mayoría de los tumores esofágicos son adenocarcinomas o carcinomas epidermoides. Los leiomiomas, aunque infrecuentes, son los tumores benignos más usuales. Pero también existen otros de muy ocasional presentación(estromales, de células granulares, schwannomas y otras neoplasias más raras aún), que se diagnostican con certeza desde que existe la inmunomarcación para identificar sus características anatomopatológicas. Objetivo: Comunicar la experiencia de los autores en relación al diagnóstico y tratamiento. Diseño: Evaluación retrospectiva. Lugar de aplicación: instituto oncológico universitario y hospital general. Población: 19 pacientes portadores de tumores mesenquimáticos primarios del esófago. Método: evaluación de manifestaciones clínicas, procedimientos diagnósticos, abordajes terapéuticos y evolución de los pacientes. Materiales: Entre 1982 y 2005, se operaron 243 tumores esofágicos observados. Dos eran melanomas, 1 tumos epitelial de colisión y 19 tumores mesenquimáticos. De estos últimos, 13 eran leiomiomas, 2 estromales benignos, 2 leiomiosarcomas, 1 schwannoma y 1 tumor de células granulares. Con la excepción de 1 caso cuyo tumor se descubrió incidentalmente, todos los demás se manifestaron por disfagia leve o moderada. El tumor de células granulares era sésil, 1 leiomioma (asociado a leiomiosarcoma independiente) era pedulado. Todos los demás fueron intramurales. Dos lesiones radicaban en área yustacardial, 1 en tercio superior y los demás en el tercio medio. Los procedimientos operatorios fueron: 1) 2 esofagectomías debido a leiomiosarcomas y otra por schwannoma, efectuada fuera de nuestro hospital, 2) 11 enucleaciones extramucosas realizadas a través de las siguientes vías de abordaje: 8 toracotomías, 1 laparotomia y 2 toracoscopias. Se llevaron a cabo 2 resecciones endoscópicas por 1 leiomioma y 1 tumor de células granulares. Resultados: No hubo mortalidad operatoria...

Cytodiagnosis of granular cell tumor: a case report.

A young male aged 28 years presented with a subcutaneous nodule in the left arm. Fine needle aspiration diagnosis was benign granular cell tumor which was confirmed by histopathology. Since it is an uncommon tumor, of controversial histogenesis, it is being reported here.

A Case of Granular Cell Tumor of the Trachea

A 20-year-old man presented to our outpatient clinic with hemoptysis, cough, and pleuritic chest pain. His chest radiograph and pulmonary function tests (PFT) were normal. A bronchoscopy showed a small yellowish patch with a regular surface. A direct bronchoscopic biopsy was performed. The pathologic findings showed a benign granular cell tumor. The respiratory symptoms resolved after biopsying the tumor. On follow?up, there were no signs of recurrence of the granular cell tumor after a period of 24 months.

Granular cell tumour of the ampulla of Vater.

Granular cell tumour (GCT) is a rare soft tissue neoplasm commonly encountered in the head and neck region, skin and subcutaneous tissue. GCT of the biliary system is most commonly reported in African-American females and usually presents as abdominal pain and obstructive jaundice. The neoplasm constitutes less than 10% of all benign tumours of the extra-hepatic biliary tree. No case of GCT involving the ampulla of Vater has been reported in the literature to date. We report a case of benign GCT involving the ampullary region in a 44-year-old Ethiopian male. Preoperative diagnosis was available on ERCP and deep biopsy. The patient was managed by debulking resection and biliary-enteric bypass and is symptom-free with no evidence of tumour progression after a follow-up of one year.

Tumor de células granulares da mama: relato de um caso / Granular cells tumor of the breast: report of a case

Relatamos um raro caso de tumor de células granulares da mama, que pode simular um carcinoma tanto clinicamente quanto radiologicamente. Trata-se de uma paciente de 57 anos de idade, sem antecedentes familiares, apresentando nódulo palpável no quadrante inferior externo da mama direita, de consistência endurecida e contorno espiculado, de tamanho mamográfico de 14mm, BI-RADS V. Realizou-se biópsia excisional que diagnosticou tumor de células granulares. Microscopicamente, evidenciaram-se típicas células de citoplasma granular positivas para proteína S-100. Tal tumor é freqüentemente localizado na língua, podendo ocorrer em diversos órgãos. Na mama, aparece sobretudo em mulheres de idade mediana, pré-menopáusicas, localizando-se preferencialmente no quadrante superior interno. Geralmente apresenta bordas mal definidas com características infiltrativas