Pulmonary granular cell tumors: a clinicopathological analysis of five cases / 中华病理学杂志

Objective: To investigate the clinicopathological features of pulmonary granular cell tumors (pGCTs) and to improve the diagnostic accuracy of the tumor. Methods: A total of 5 pGCTs were diagnosed from February 2016 to January 2022 at Shanghai Pulmonary Hospital, Tongji University School of Medicine and Fudan University Shanghai Cancer Center, China. Immunohistochemical staining, and analysis of the clinicopathological characteristics were performed. Results: The average age of the pGCTs patients was 46 years (ranging from 24 to 54 years), with 3 females and 2 males. One case occurred in the bronchus with multiple nodules in the lung, 2 cases occurred in the bronchial opening, and 2 cases were solitary nodules in the lung. The maximum diameter of the tumors ranged from 12 to 15 mm (mean size 14 mm). Microscopically, the tumor showed infiltrative growth and consisted of round, oval or polygonal cells. Abundant eosinophilic cytoplasm was noted, and the nucleoli were prominent. None of the 5 cases showed any mitosis or necrosis. Immunohistochemical and histochemical study showed positive staining for S-100 (5/5), SOX10 (5/5), Vimentin (5/5), TFE3 (4/5), PAS (3/5), and amylase-digested-PAS (3/5), while 4 cases were negative for CD68. TFE3 FISH analyses on 2 cases showed that no signal abnormality was detected in these 2 cases. The average proliferation index of Ki-67 was 2.2% (range 0-5%). There was no recurrence in 4 cases of pGCTs with a follow-up time ranging from 2 months to 60 months. Conclusions: pGCTs are very rare tumors, most likely originating from Schwann cells. Immunohistochemical staining is the conventional diagnostic tool for pGCTs diagnosis. Recognition of this entity is essential for pathologists to avoid misdiagnosis and unnecessary treatments.

Clinicopathological characteristics and differential diagnosis of 6 cases of congenital granular cell tumor / 中华口腔医学杂志

To provide references for the diagnosis and treatment of congenital granular cell tumor (CGCT), by comprehensive analysis of the clinical data, histopathological and immunohistochemical results. Patients with CGCT were involede, from March 2015 to November 2020, at the Department of Oral and Maxillofacial Surgery of the First Affiliated Hospital of Zhengzhou University. A total of 6 children, aged 3-16 days, 1 male and 5 female, 5 maxillary and 1 mandibular, with maximum tumor diameter of 6-70 mm, were included. The lesions of CGCT were single and connected to the alveolar ridge by a pedicle. The surface of the tumor was covered with a vascular network, and two cases had ulcers on the surface of the tumor. All 6 cases had the tumor removed surgically and there was no recurrence or metastasis in the follow-up visit. Although CGCT is rare, it is a benign tumor and generally does not recur or metastasize after surgery, and has a good prognosis. The prenatal imaging, clinical manifestations after delivery, pathological characteristics and immunohistochemical analyses may provide reference for early diagnosis and treatment of CGCT.

S100 negative granular cell tumor of the oral cavity: dermoscopy and surgical approach

Abstract: We report the case of a 47-year-old male patient with S100 negative granular cell tumor of the oral cavity, focusing on dermoscopic features as well as surgical approach, not previously reported in the literature. The study contributes to the literature on dermoscopy and surgical treatment for this tumor and provides a practical approach to differentiating non-neural granular cell tumors and granular cell tumors.

Granular cell tumor of anal border / Tumor de células granulares de borda anal

The objective of this report is to describe a case of granular cell tumor of the anal border and to review the most relevant topics of the literature on the subject. Ours is a female patient, 57 years old, with an asymptomatic nodule in the anal border for 2 years. Surgical excision was performed, with a histopathological diagnosis of granular cell tumor. The first description of this tumor was carried out in 1926 by Abrikossoff. The techniques of immunohistochemistry and electron microscopy allowed us to determine its origin in Schwann cells. These are rare tumors, most often diagnosed between the 4th and 6th decade of life and, in general, are benign formations - only 2% of them are malignant. These tumors can occur in any part of the body, although they are more common in the oral mucosa, dermis and subcutaneous tissue. The treatment solely by surgery has a curative effect, and its recurrence is unusual. The location in the anal/perianal area occurs even more rarely, and we found only 48 cases previously described in the literature.

O objetivo deste relato é descrever um caso de tumor de células granulares de borda anal e revisar os tópicos mais relevantes da literatura acerca do tema. Trata-se de uma paciente do sexo feminino, 57 anos, com histórico de nódulo na borda anal assintomático há 2 anos. Foi realizada ressecção cirúrgica da lesão, com diagnóstico histopatológico de tumor de células granulares. A primeira descrição deste tumor foi em 1926 por Abrikossoff. As técnicas de imunohistoquímica e de microscopia eletrônica permitiram determinar a sua origem nas células de Schwann. São tumores raros, mais frequentes entre a 4ª e 6ª década de vida e, no geral, benignos, apenas 2% são malignos. Podem ocorrer em qualquer parte do corpo, embora sejam mais comuns na mucosa bucal, derme e tecido celular subcutâneo. O tratamento cirúrgico isolado é curativo e a recorrência incomum. A localização no ânus/canal anal/perianal é ainda mais rara, sendo encontrados apenas 48 casos previamente descritos na literatura.

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.

Tumor colorrectal de células granulares: presentación de dos casos y revisión de la literatura / Granular cell of colorectal tumor: presentation of two cases and review of the literature

Los tumores de células granulares (Abrikossoff) son tumores estromales benignos que se manifiestan más frecuentemente en la cabeza y cuello. La ubicación colorrectal es menos frecuente. Presentamos 2 casos de ubicación colorrectal. En el primer caso se trató de un tumor ubicado en el recto bajo, a 4 cm del margen anal, que se resecó mediante técnica transanal. El siguiente caso se ubicó en el ciego y, ante la falta de exéresis endoscópica, se resecó mediante hemicolectomía derecha laparoscópica. Ambos tuvieron diagnóstico histopatológico de Tumor de Células Granulares (TCG) confirmado por inmunohistoquímica. Los TCG son tumores generalmente benignos con características inequívocas en el estudio histológico (abundante citoplasma eosinófilo, núcleos pequeños, uniformes y redondos, sin mitosis evidentes) e inmunohistoquímico (PAS y proteína S-100 positivas). Aparecen entre la 4ª y 6ª década en cualquier parte del organismo. En el tubo digestivo aparecen más frecuentemente en el esófago. Debe sospecharse su presencia ante la aparición de un nódulo submucoso sólido, menor de 2 cm, y generalmente único.

Granular cell tumors (Abrikossoff) are benign stromal tumors that usually appear in the head and neck. Colorectal location is less frequent. We present two clinical cases in this location. The first patient presented with a tumor located in the lower rectum, 4 cm from the anal verge, which was resected via local excision. The second case was located in the cecum, and it was resected by laparoscopic right colectomy. Both cases had histopathology diagnosis of Granular cell tumors (GCT) confirmed by immunohistochemistry. GCT are usually benign tumors with unequivocally features in histological analysis (abundant-eosinophilic cytoplasm, small nuclei, round and uniform, without evident mitoses) and immunohistochemichal staining (PAS and S-100 protein positive). The age of presentation is around 4th and 6th decades at any part of the body. In the digestive tract they grow more frequently at the esophagus. Diagnosis should be suspected when facing a unique, solid, less than 2 cm submucosal nodule.

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.

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.

Tumor de células granulares da laringe na infância: relato de caso / Granular cell tumor of the larynx in children: a case report

O tumor de células granulares (TCG) é uma neoplasia incomum, de evolução lenta, na maioria dos casos de caráter benigno e que pode acometer qualquer órgão do corpo. Entre as hipóteses que tentam explicar sua origem, a teoria da gênese neural apresenta embasamento sólido e é a mais aceita atualmente. O TCG é mais comum na raça negra, entre a 4ª e 5ª décadas de vida, acometendo com maior freqüência a região da cabeça e pescoço. A localização laríngea é rara, e quando ocorre é mais comum na porção posterior. É muito raro em crianças em geral acomete a porção anterior da subglote, podendo estender-se para a glote. O sintoma predominante é a rouquidão, podendo ocorrer disfagia, dor, tosse, hemoptise, e estridor. Macroscopicamente o TCG se manifesta como nódulo de pequeno tamanho, firme, séssil ou pediculado, não-ulcerado, de coloração clara, e usualmente bem circunscrito, porém sem cápsula. À microscopia, as granulações citoplasmáticas são características, apresentando positividade para a imunoperoxidase S100 e para a enolase neurônio-específica. O tratamento do TCG laríngeo consiste na exérese cirúrgica. Neste trabalho descrevemos um caso pediátrico de TCG laríngeo e sua evolução clínica após a remoção cirúrgica, alertando para o diagnóstico do TCG na população pediátrica. Foi realizada revisão de literatura abrangendo as características clínicas e histopatológicas do TCG, assim como as formas atuais de tratamento.

The granular cell tumor (GCT) is an uncommon neoplasm, with slow progression, usually benign, that can be found in any organ. The most common region for GCT involvement is in the head and neck. Laryngeal involvement is uncommon and accounts for 6 to 10 percent of all cases reported. Among the major theories of origin and based on the strongest evidence, the most accepted one is that the tumor stems from neuronal tissue. The GCT has a higher incidence in African-descendent patients, and most commonly in their 4th and 6th decades of life. The posterior larynx is the most common laryngeal site. Pediatric laryngeal GCT is rare, anterior subglottis involvement has been described and extensive glottic involvement may occur. Affected patients typically present with hoarseness, dysphagia, cough, haemoptysis, stridor and pain. The GCT presents as a small, firm nodule, sessile or polypoid, with intact mucosa, well outlined but not encapsulated. Cytoplasm granules are typically seen under light microscopy, and the cells are positive for S100 immunoperoxidase and neuron-specific enolase. Treatment of laryngeal GCT is based on surgical excision. This paper describes a pediatric patient with GCT and its clinical course before and after surgical treatment, stressing the importance of GCT diagnosis in the pediatric population. We review clinical course, pathology characteristics 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...

Tumor de células granulares / Granular cell tumour

Presentamos el caso clínico de una mujer de 54 años que consulta por una gran tumoración infraclavicular de dos años de evolución. Los resultados de la punción-aspiración de la lesión son compatibles con un tumor de células granulares. La lesión es extirpada y el estudio histológico confirma el diagnóstico. Realizamos una revisión sobre el origen del tumor, su biología, histología, diagnóstico y tratamiento.

The case reported is a 54 years-old woman with a great mass below the clavicle for two years. The results of fine-needle aspiration biopsy were compatible with granular cell tumour. The mass was removed and the histopathological study confirmed the diagnosis. The present report reviews some aspects about the origin, biology, histology, diagnosis and treatment of this tumour.

Tumor maligno de células granulares: evaluación clínica-patológica de un caso / Malignant granular cell tumor: evaluation clinical-pathological of a case

Describir el caso de un tumor maligno de células granulares haciendo especial énfasis en los hallazgos clínico-patológicos e inmunohistoquímicos. Paciente femenino de 62 años de edad con tumor en muslo derecho. Se practicó extirpación quirúrgica y se observó tumor de 3,8 cm x 2,5 cm. El análisis histopatológico evidenció un tumor de células granulares con características malignas. El estudio inmunohistoquímico mostró tinción positiva para la proteína S-100 y la vimentina. Los tumores malignos de células granulares son neoplasias extremadamente raras, estimándose entre 1 por ciento - 2 por ciento todos los tumores de células granulares. La adecuada correlación clínico-patológica es necesaria para alcanzar un diagnóstico final

Tumor de Abrikossoff gingival: reporte de un caso / Abrikossof gingival tumor: a case report

El tumor de las células granulosas en una rara entidad; existen numerosas teorías sobre su origen que aún están bajo polémica, aunque la más aceptada es la neurogénica, que asimila su procedencia como neuroectodérmico. Se detectó un paciente en consulta con cuadro clínico similar al de esta patología con localización en encía superior, región de premolares. La lesión se mostraba submucosa, elevada, no dolorosa y de aspecto áspero.Tras realizar la exéresis y practicar la biopsia, se corroboró el diagnóstico de un mioblastoma de células granulosas o tumor de Abrikossoff(AU)

Granular cell tumors is a rare entity. There is a number of theories on their origin under discussion, but the most accepted is the neurogenic theory that covers the neuroectodermal origin of the tumor. A female patient was detected in outpatient service, who presented with a clinical picture similar to that of this pathology and located in upper gingiva, premolar region. The lesion looked submucosal, raised, painless and rough. After exeresis and biopsy, the diagnosis of granular cell myoblastoma or Abrikossof tumor was confirmed(AU)

Ressecção endoscópica de tumor de células granulares do esôfafo / Endoscopic removal of granular cell tumor of the esophagus

O tumor de células granulares (TCG), também chamado de tumor de Abrikossoff (TA), é uma lesão benigna, de origem controversa, encontrada mais frequentemente na língua e pele. Aproximadamente 5 a 11 por cento destas lesões são encontradas no trato digestivo e o esôfago é o local mais acometido. Pode ser assintomático ou evoluir com disfagia de intensidade variada. Apresenta-se um caso com sintoma de disfagia leve em que a ultra-sonografia endoscópica mostrou uma massa hipoecóica na submucosa sem extensão à muscular própria. Optou-se pela ressecção endoscópica devido à sintomatologia do paciente, crescimento recente das lesões e aspecto ultra-sonográfico. A lesão foi ressecada com sucesso e sem complicações

Mioblatoma de células granulosas: presentación de un caso clínico / Granular cell myoblastoma: report of a clinical case

El tumor de las células granulosas en una rara entidad; existen numerosas teorías sobre su origen que aún están bajo polémica, aunque la más aceptada es la neurogénica que asimila su procedencia como neuroectodérmico. Se detectó un paciente en consulta con cuadro clínico similar al de esta patología con localización en el dorso de la lengua. La lesión se mostraba submucosa, elevada, no dolorosa y de aspecto áspero. El caso fue remitido a la atención secundaria de cirugía del Instituto Nacional de Oncología y Radiobiología (INOR) donde, tras realizar la operación y practicar la biopsia, se corroboró el diagnóstico de un mioblastoma de células granulosas(AU)

The tumor of granular cells is a rare entity. There are numerous theories about its origin that are still polemic, though the most accepted is the neurogenic one that assimilates its source as neuroectodermic .A patient with a clinical picture similar to that of this pathology with localization on the dorsum of the tongue was observed at the physician's office. The lesion was submucous, elevated, unpainful and with a rough aspect. The case was referred to the secondary surgery care service of the National Institute of Oncology and Radiobiology (INOR, in Spanish), where the patient was operated on and a biopsy was performed that confirmed the diagnosis of granular cell myoblastoma(AU)

Tumor de células granulares endobrônquico: relato de um caso diagnosticado por biópsia endoscópica / Endobronchial granular cell tumor: report of a case diagnosed by endoscopic biopsy

Tumor de células granulares (TCG) é um termo descritivo para um tumor com histologia distintiva, perfil imunohistoquímico característico e achados ultra-estruturais peculiares. Tem distribuição topográfica ampla e sua localização nas vias aéreas é considerada incomum. Os autores relatam o caso de uma mulher de 40 anos com tosse produtiva e febre há dois meses e exame físico normal. A tomografia computadorizada evidenciou espessamento de parede do brônquio intermediário e a broncoscopia mostrou, nesse nível, hiperemia e elevação da mucosa endobrônquica. Os exames histopatológicos e imunohistoquímico dos tecidos deste local diagnosticaram TCG. O objetivo do presente relato é chamar a atenção para a possibilidade de diagnóstico desse tumor em pequenas biópsias endoscópicas