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.

Tumor de células granulares en región axilar: a propósito de un caso y revisión de la literatura / Tumor of granular cells in the axillar region: purpose of a case and literature review

Resumen El tumor de células granulares es una neoplasia infrecuente, de comportamiento biológico benigno. Por lo general, se presenta entre la cuarta y sexta década de vida como una lesión solitaria, de crecimiento lento y buen pronóstico. Su histogénesis es probablemente de origen neural siendo positivo para S-100 y Enolasa Neuronal Especifica. Se muestra un caso con una localización inusual en la región axilar, las dificultades para alcanzar el diagnóstico puesto que puede confundirse con otras neoplasias, y los elementos clínicos esenciales de este tipo de tumor.

Abstract Granular cell tumor is a rare neoplasm with benign behavior. It usually occurs in the fourth to sixth decade of life as a solitary, slow growing lesion with a good prognosis. Its histogenesis is probably of neural origin, being positive for S-100 and Neuron-Specific Enolase. We demonstrate an unusual location in the axillary region, the obstacles to reaching the diagnosis since it can be confused with other malignancies, and the essential elements for clinically suspecting benign lesions of this type.

Case for diagnosis. Dorsal nodule in a 10-year-old male

Abstract Granular cell tumors (GCTs) are rare soft-tissue neoplasms. GCT immunohistochemistry is positive for S-100, NSE, and CD68. This report describes the case of a 10-year-old male who presented with a dorsal nodule. A biopsy revealed aggregates and sheets of large epithelioid and spindle cells. The cells had abundant eosinophilic granular cytoplasm. Immunohistochemical analysis was positive for CD68, NKI/C3, and synaptophysin; weakly positive for NSE; and negative for S-100, SOX10, HMB45, Melan A, cytokeratin, SMA, EMA, and CD163. The Ki-67 index was less than 1%. A diagnosis of an S-100 negative, cutaneous, benign GCT was determined.

Tumor de célula granular em cavidade oral: relato de caso / Granular cell tumor in oral cavity: case report

Introdução: O tumor de células granulares (TCG) é uma neoplasia benigna de tecido mole incomum que acomete principalmente mulheres na quarta e sexta década de vida. A lesão possui predileção pela região de cabeça e pescoço, sendo a superfície dorsal da língua o local de maior acometimento. Clinicamente, apresenta-se como um nódulo submucoso firme, de coloração semelhante a mucosa ou levemente amarelada, de crescimento lento e assintomático. O objetivo deste trabalho é relatar um caso de TCG apresentando suas características clínicas, histopatológicas bem como os possíveis diagnósticos diferenciais. Relato de caso: Paciente sexo feminino, 46 anos de idade, apresentando nódulo localizado no bordo lateral posterior da língua. Ao exame intraoral foi possível observar tumefação firme à palpação, com coloração amarelada, medindo aproximadamente 1cm no seu maior diâmetro. Baseado na hipótese diagnóstica de lipoma, foi realizada uma biópsia excisional. A análise histopatológica revelou características morfológicas compatíveis com o TCG. Considerações finais: O Cirurgião-Dentista deve conhecer os diagnósticos diferenciais clínicos e histopatológicos, uma vez que a conduta terapêutica pode ser distinta em alguns casos... (AU)

Introduction: Granular cell tumor (GCT) is a benign neoplasm of uncommon soft tissue that mainly affects women in the fourth and sixth of life. The lesion has a predilection for the head and neck region, and the dorsal surface of the tongue is the site of major involvement. Clinically, it presents as a firm, mucosal or slightly yellowish, slowly growing, asymptomatic submucosal nodule. The objective of this work is to report a case of GCT presenting its clinical, histopathological and possible differential diagnoses. Case report: A 46-year-old female patient with a nodule located on the posterior lateral border of the tongue. On intraoral examination, it was found on a table, with a yellowish color, measuring approximately 1cm in its largest diameter. Based on the diagnostic hypothesis of lipoma, an excisional biopsy was performed. A histopathological analysis revealed morphological characteristics compatible with GCT. Final considerations: The Dentist should know the differential clinical and histopathological diagnoses, since the therapeutic behavior may be different in some cases... (AU)

Pediatric Granular Cell Tumor of the Breast: An uncommon neoplasm in an uncommon site and age group

Granular cell tumor (GCT) is a rare soft tissue neoplasm of Schwann cell origin. Most cases occur in adults; however, the precise incidence is unknown in children. GCT is usually a slow-growing, painless tumor involving the skin and soft tissues that is mostly located in the head and neck region, especially the tongue. The breast is one of the least common sites involved by GCT. This paper presents a 3-year-old girl who presented with a soft to firm, ill-defined swelling on the right breast with painful ulceration of the overlying skin. Fine needle aspiration rendered an initial diagnosis of fibrocystic change accompanied by apocrine metaplasia. Histologic evaluation of the excised breast mass revealed a benign granular cell tumor. Although rare, GCT of the breast should be included in the differential diagnosis for breast masses in pediatric patients. Proper diagnosis and timely management of this tumor are essential because of its malignant potential (<2% of cases) and high rate of local recurrence if not properly excised.

Tumor de células granulares em criança: relato de caso / Granular cell tumor in a child: Case report

Introdução: O Tumor de células granulares é uma lesão, que foi descrita pela primeira vez em 1926, por Abrikosoff, ocorrendo geralmente entre a segunda e a quinta década de vida, predominantemente em mulheres negras, sendo raro o acometimento em crianças. A característica clínica é de uma lesão séssil, não sangrante, coloração da mucosa, indolor, tamanho aproximado de 1,0 cm, firme à palpação. Relato de caso: Este trabalho tem por objetivo apresentar um relato de caso de um tumor de células granulares localizado no dorso da língua de uma criança. Foi realizado excisão cirúrgica. O paciente segue em acompanhamento de 3 anos, sem recidiva. Consideracões finais: É importante relatar que o TCG é uma lesão incomum em crianças. Apresenta-se como um tumor benigno, sendo raro o aparecimento da variante maligna. Recidiva é incomum. A remoção cirúrgica é o tratamento indicado... (AU)

Introduction: Granular cell tumor is an injury that was described for the first time in 1926 by Abrikosoff, a histologically similar tumor with skeletal muscle fibers. It is suggested that this lesion originates from the skeletal muscles, this type of lesion usually occurs between the second and fifth decade of life, occurs predominantly in women and blacks, and is uncommon in children, presents as a sessile, non-bleeding, painless mucosal color lesion, approximately 1.0 cm in size, firm the palpation and the skin covering the lesion remain intact without ulcerations. Case report: Thus, this work aims to present a case report of a surgical excision of a granular cell tumor located on the back of a child's tongue after incisional biopsy and with the histopathological result confirming that it was a granular cell tumor. Surgical excision was performed under general anesthesia, where it goes through 3 years of follow-up without relapse. Final considerations: It is important to report that GCT is an uncommon injury in children. It presents as a benign tumor, being rare the appearance of the malignant variant. Relapse is uncommon. Surgical removal is the indicated treatment... (AU)

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.

Ameloblastoma with distinctive granular cell pattern: an 8 case study

Ameloblastoma with granular cell pattern (AGC) is classified as an unusual histological subtype of solid/multicystic ameloblastoma, characterized by granular changes in stellate-like cells located within the inner portion of the epithelial follicles. Studies have revealed that lysosomal overload causes cytoplasmic granularity; however, the mechanism involved remains poorly understood. Here we report on eight cases of granular cell ameloblastoma, in the posterior region of the mandible. The age of the patients included in this case series ranged from 35 -64 years old and 87.5% of cases occurred in non-Caucasians, with a slight gender predilection for men (62.5%). There was no evidence of recurrence, and the majority of the cases were treated with surgical resection (87.5%). All tumors displayed histopathological features consistent with the diagnosis of ameloblastoma with granular cell pattern.

Tumor de células granulosas del esófago / Granulosa cell tumor in the esophagus

Los tumores de células granulosas afectan raramente al esófago. Presentamos el caso de tumores de células granulosas del esófago y revisar la literatura. Se presenta una paciente de 21 años de edad y piel negra con síntomas de reflujo gastroesofágico. La endoscopia digestiva demostró un tumor de 2 cm que protruía hacia la luz del órgano, localizado en el tercio inferior, con mucosa sana. El esofagograma corroboró el hallazgo en la porción abdominal del órgano y la biopsia endoscópica informó que se trataba de tumores de células granulosas. El acceso videolaparoscópico fue convertido a cirugía convencional debido a la abertura mucosa y a que quedaba un pequeño fragmento del tumor. El periodo posoperatorio transcurrió sin complicaciones. Un año después de la operación no existía evidencia de recidiva. Los tumores de células granulosas son raros en el esófago. Los tumores pequeños (< 1 cm) pueden ser tratados conservadoramente, mientras que los de mayor volumen deben ser resecados mediante diferentes técnicas: endoscópicas y cirugía de mínimo acceso o convencional(AU)

Granulosa cell tumors rarely affect the esophagus. We present the case of granulosa cell tumors of the esophagus and literature review. A 21-year-old patient of black skin presents with symptoms of gastroesophageal reflux. The digestive endoscopy showed a 2-cm tumor that protruded towards the organ lumen, located in the lower third, with healthy mucosa. The esophagogram corroborated the finding in the abdominal portion of the organ, and the endoscopic biopsy reported that there were granulosa cell tumors. Videolaparoscopic access was converted to conventional surgery due to the mucosal opening and because a small fragment of the tumor was remaining. The postoperative period had no complications. One year after the operation, there was no evidence of recurrence. Granulosa cell tumors are rare in the esophagus. Small tumors (smaller than 1 cm) can be treated conservatively, while larger tumors should be resected using different techniques: endoscopic and minimal access or conventional surgery(AU)

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.

Tumor de células granulares de la mama. Presentación de un caso / Granular cell tumor of the breast. Case presentation

El tumor de células granulares (TCG) es una neoplasia muy infrecuente y usualmente benigna, que afecta preferentemente a la lengua.En sólo un 6% por ciento de los casos publicados ha sido primario de la mama. Desde el punto de vista clínico e imagenológico, se plantea el diagnóstico diferencial con un carcinoma mamario invasor. Por ende, el examen anatomopatológico de muestras por punción o quirúrgicas, es crucial para establecer el diagnóstico e intentar subclasificarlo para predecir su conducta biológica. Se presenta el caso de una mujer chilena de 54 años con un TCG de la mama izquierda:(AU)

Granular cell tumor (GCT) is an uncommon and usually benign neoplasm that mostly occurs on the tongue. In only 6 percent of all published cases, GCT has been primary of the breast. From a clinical and radiological point of view, a differential diagnosis with an invasive breast carcinomais considered. Therefore, the anatomopathological examination of core biopsie or surgical samples is crucial to establish a diagnosis and tryto subclassify it in order to predict its biological behavior. We present the case of a 54-year-old chilean woman with a GCT in her left breast.(AU)

Newly Arising Contralateral Granular Cell Tumor after Wide Excision of Large Breast Granular Cell Tumor: A Case Report

Granular cell tumor (GCT) of the breast is a rare neoplasm that is usually benign in nature. The results of physical examination and ultrasonographic findings of GCT of the breast often mimic those of other malignancies. A 46-year-old woman underwent wide excision for removal of a large mass in the left breast (about 10 cm in diameter). The pathologic finding of the excised mass was benign GCT. A year after initial surgery, recurrence of the left breast mass was found along with a newly developed mass in the contralateral right breast, which was excised. In this study, we describe the case of a newly developed GCT in the contralateral breast 1 year after excision of a larger breast GCT.

Tumor de células granulares del esófago: reporte de un caso / Esophageal granular cell tumor: Case report

Introducción: el tumor de células granulares del esófago (TCG) es una neoplasia rara y su diagnóstico preciso se basa en el examen histopatológico. Con el incremento de la endoscopia como medida de tamizaje se ha visto un leve aumento en la incidencia, por lo que debe tenerse en cuenta como diagnóstico diferencial en el momento de abordar una lesión subepitelial. Metodología: presentación de un caso clínico con TCG cuya endoscopia de vías digestivas altas (EVDA) muestra una lesión subepitelial en el tercio distal del esófago, y que debido a las características histopatológicas, clínicas y ecosonográficas se decide seguimiento y manejo expectante. Conclusiones: es importante el conocimiento de las características, comportamientos y estrategias de manejo del TCG, puesmuchos son asintomáticos y estables en el seguimiento, por lo que no necesitan tratamientos agresivos. Por el riesgo de malignidad, es importante su control riguroso.

Introduction: Esophageal granular cell tumors (GCTs) are rare. Their precise diagnosis is based on histopathological examination of the specimen. However, owing to the use of endoscopy as a screening tool the incidence of these lesions has presently mildly increased and must be considered as a differential diagnosis of subepithelial lesions. Methodology: A case is presented of a GCT as a subepithelial lesion in the distal part of the esophagus found by esophagogastroduodenoscopy (EGD). Conservative management and follow-up was decided due to the histopathological, clinical and ultrasound features of the lesion. Conclusions: Knowledge regarding GCTs´i characteristics, behavior and management is important for many are asymptomatic and remain clinically stable during follow-up, requiring no aggressive treatment. A rigorous follow-up is recommended due to its malignant potential.

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

Congenital Non-Neural Granular Cell Tumor Mimicking Nevus Lipomatosus Superficialis

A 4-month-old infant presented with asymptomatic soft nodules on his right forearm, which had developed since birth. On the suspicion of nevus lipomatosus superficialis (NLS), biopsy was performed. Histopathologic findings showed monomorphic polygonal cells with abundant granular cytoplasm. Immunohistochemical stains for CD68 and vimentin were strongly positive, but were negative for S-100 protein. Based on the pathologic findings, the patient was diagnosed as non-neural granular cell tumor (NN-GCT). GCT can be divided into conventional and non-neural GCT by immunoreactivity for S-100 protein. NN-GCT is typically manifested as a well-circumscribed, papulo-nodular dermal mass, and is known to occur in a younger group than does in conventional GCT, but is rare among children. To our knowledge, there have been no case reports of NN-GCT which appeared at birth and presented as grouped nodules. Therefore, we report this interesting case of congenital NN-GCT clinically mimicking NLS.

A Case of Granular Cell Tumor of the Clitoris in a Postmenopausal Woman

Granular cell tumor (GCT) is a rare soft tissue tumor that derived from Schwann cells. Most are benign, less than 2% are malignant and, in the malignant cases, the prognosis of survival is poor. Most of these tumors are less than 3 cm in size, and they are more common in black women. The disease usually occurs in the 40s to the 60s and occurs after menopause, but there are few cases reported in adolescence. A 45-year-old woman visited the outpatient clinic with a solid mass that developed 2 years ago and present to date on the left side of the clitoris. After complete resection, pathologic examination proved to be a granular cell. The patient has been living without recurrence for one year. We report the first case of the GCT of the clitoris in postmenopausal woman in Korea with a brief review of the literature.

A Granular Cell Tumor of the Rectum: A Case Report and Review of the Literature

A granular cell tumor (GCT) is an uncommon mesenchymal lesion that rarely occurs in the colon and the rectum. We describe the case of 51-year-old man with a 2-cm-sized rectal GCT 10 cm above the anal verge that was incidentally detected after a screening colonoscopy. Preoperative radiologic studies demonstrated a suspicious submucosal rectal mass with mesorectal fat infiltration, but without circumferential resection margin threatening, extramural vessel invasion, and regional lymph-node enlargement. The tumor was resected by using a transanal endoscopic operation (TEO) without immediate postoperative complications. The final pathology revealed that the tumor consisted of a GCT that had invaded the subserosa with clear margins. It had no other risk factors for malignancy according to Fanburg-Smith criteria. We systematically reviewed the English literature by using PubMed and Google Scholar. This report may be the first documented case in the literature to describe a TEO for a GCT that had invaded the subserosa in the rectum.

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)