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BACKGROUND: Granular Cell Tumor (GCT) is an uncommon benign lesion in the oral cavity whose pathogenesis remains poorly understood. Due to their infrequent occurrence and similarity to other oral lesions, they are often forgotten during the initial clinical diagnosis. Therefore, understanding its prevalence, clinical and pathological characteristics is crucial for an accurate diagnosis and adequate management. METHODS: All cases diagnosed as GCTs in six Brazilian and Argentinian oral diagnostic centers were re-evaluated by HE staining, and clinical, demographic, and histopathological data were collected and evaluated. RESULTS: The series comprised 45 female (73.8%) and 16 male (26.2%), with a 2.8:1 female-to-male ratio and a mean age of 35.3 ± 16.9 years (range: 7-77 years). Most cases occurred on the tongue (n = 49; 81.6%) and presented clinically as asymptomatic papules or nodules (n = 50; 89.3%) with a normochromic (n = 25; 45.5%) or yellowish (n = 11; 20.0%) coloration and sizes ranging from 0.2 to 3.0 cm (mean ± SD: 1.40 ± 0.75 cm). Morphologically, most tumors were characterized by a poorly delimited proliferation (n = 52; 88.1%) of cells typically rounded to polygonal containing abundant, eosinophilic, finely granular cytoplasm. Pustulo-ovoid bodies of Milian were identified in all lesions (n = 61; 100%). Entrapment of skeletal striated muscle (n = 44; 72.1%) and nerve fibers (n = 42; 68.9%) were common findings. Pseudoepitheliomatous hyperplasia (PEH) was observed in 23 cases (39.0%). In only 27.7% of cases (n = 13) there was agreement between the clinical and histopathological diagnosis. Outcome information was available from 16 patients (26.2%), with clinical follow-up ranging from 4 to 36 months (mean 13.3 months), and none developed local recurrence. CONCLUSIONS: The clinical and histopathological features of GCTs were consistent with those described in previous studies. In general, these lesions have a predilection for the lateral region of the tongue in adult women. It is essential to consider GCTs in the differential diagnosis of yellow or normochromic papules and nodules in the oral cavity. Histopathological evaluation is essential for the definitive diagnosis and the prognosis is excellent.

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Lesões neurais são incomuns na região de cabeça e pescoço, especialmente na cavidade oral, representa n d o o principal grupo de lesões da cavidade oral com células fusiformes. O diagnóstico destas lesões pode ser desafiador já que podem compartilhar características clínicas e microscópicas similares , podendo ter natureza reacional ou neoplásica. O objetivo deste trabalho foi avaliar as características clínicas e microscópicas das lesões neurais diagnosticadas no Laboratório de Patologia Oral da Faculdade de Odontologia da Universidade Federal do R io d e Janeiro Brasil no período entre 1971 até 20 2 1 Os casos foram obtido s após a revisão dos arquivos da instituição e o diagnóstico final de cada caso foi confirmado pela análise microscópica . A s características clínicas e microscópicas de cada caso foram apresentadas de maneira descritiva. Cento e setenta e um a lesões neurais foram identificadas . As lesões apresentaram se principalmente como nódulos normocrômicos, assintomáticos, de consistência firme e superfície lisa, localizados na língua de mulheres com m é dia de idade de 43, 5 anos Os di agnósticos mais comuns foram hamartoma neurovascular 30,4 tumor de cé lulas granulares (1 7 %), neuroma traumático ( 1 6,4 %)%), seguidos de placa neurogênica subgemal (1 2,9 %), neuroma solitário circunscrito ( 9,9 %)%), schwannoma 7,6 e neurofibroma 5, 8 O presente e stud o provavelmente representa uma das maior es casuística s de lesões neurais da cavidade oral disponível na literatura. (AU)

Neural lesions ar e uncommon in the head and neck region, parti cularly in the oral cavity, representing the most common group of oral spindle cell lesions of the oral cavity. The diagnosis of neural lesions may be ch a llenging since it may share cli nical and micros copical fe atures, showing reactive or neoplastic nature. The aim of the present study was to evaluate the clinical and microscopical features of neural lesions diagnosed in the Oral P athology Labora tory of th e School of Dentistry of Federa l U niversity of Rio d e Jan eiro Brazil in a period between 1971 and 20 21 . The cases were obtained from the review of the files of this institution and the final diagnosis were confirmed after microsc opical evaluati on O ne hu n dred and seventy one neural lesions were identified . The lesions presented preferably as asymptomatic and normochromic nodules of tender consistency and smooth surface, located in the tongue of women with median age of 4 3, 5 year s The most com mo n diagnose s were neurovascular hamartoma 30,4 granular cell tumor (1 7 %)%), traumatic neuroma 1 6,4 and followed by subgemmal neurogenous plaque 1 2,9 %), circumscribed solitary neuroma 9,9 %)%), schwannoma ( 7,6 and neurofibroma ( 5, 8 %)%). The present study likely re p resent s one of the largest series of neural lesions of the oral cavity of the literature. (AU)

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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)

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OBJECTIVE: Granular cell tumour (GCT) is a benign neoplasm that originates from Schwann cells. Within the oral cavity, it usually appears as a lingual nodule and especially amongst female adults. Histologically, GCT shows a proliferation of polygonal cells with eosinophilic granular cytoplasm, which can be associated with a pseudoepitheliomatous hyperplasia (PEH). In this study, we analyse the main clinicopathological data of intraoral GCT and we compare our results with previous studies. MATERIAL AND METHODS: We have studied a series of 56 cases of oral GCT in Spain and Brazil, and we have conducted a systematic review in PubMed, Web of Knowledge and Scopus databases, using the keywords: "granular cell tumour" and oral. RESULTS: In our series, GCT appeared as an asymptomatic benign tumour that is more frequent in women and in the tongue. PEH was observed in 32% of the lesions. In the review, we collected 282 cases of oral GCT with a similar clinical profile; seven patients had multiple lesions, and 33% of the cases presented PEH. No cases of malignant oral GCT have been described to date. GCT is an uncommon oral benign neoplasm, mainly unique and asymptomatic, derived from Schwann cells. CONCLUSIONS: Although the etiopathogenesis of this oral tumour is unknown, its characteristics suggest that it could have a reactive nature. Conducting a complete clinicopathological study in all intraoral GCT is fundamental in order to dismiss other entities, including oral carcinoma.

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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.

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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.

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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.

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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.

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BACKGROUND: Granular cell tumor is a rare benign tumor that can present a pseudoepitheliomatous hyperplasia of the covering epithelium. This lesion is not encapsulated and can be characterized by a pseudo invasive growth pattern, represented by the tumoral cells that infiltrate between adjacent connective tissue elements. Diagnostic difficulties may arise because histopathological features of the pronounced pseudoepitheliomatous hyperplasia can be confused with a well-differentiated oral squamous cell carcinoma. The aim of this case report is to demonstrate the role of an immunohistochemical panel in the diagnosis of a granular cell tumor in the tongue with clinical and microscopic features resembling an oral squamous cell carcinoma. CASE PRESENTATION: A 44-year-old white man with a history of heavy smoking and alcohol abuse presented an ulcerated nodular lesion in the dorsum of the tongue. The lesion was asymptomatic with fast growth. The clinical diagnosis was an oral squamous cell carcinoma. An incisional biopsy was performed and the ensuing histopathological analysis showed a pseudoepitheliomatous hyperplasia in the overlying epithelium mimicking the invasion of epithelial tumor cells into the connective tissue as in an oral squamous cell carcinoma. Immunohistochemical antibodies (S-100, vimentin, CD68, p53, Ki-67, E-cadherin, collagen IV and cytokeratin AE1/AE3) were used to characterize molecular aspects of the lesion. Strong staining of S-100 protein, CD68, vimentin, E-cadherin and low proliferative activity observed with Ki-67 expression confirmed the diagnosis of a granular cell tumor. The patient was submitted to surgical excision of the whole lesion. At a 12-month check-up, there was no evidence of recurrence. CONCLUSION: This case report showed that the immunohistochemical profile was helpful in determining the clinical behavior of the tumor and establishing the final diagnosis with appropriate treatment.

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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)

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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.

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Granular cell tumor (GCT) is an uncommon benign soft tissue neoplasm, which usually presents as a solitary nodule, appearing especially in the tongue. There are few cases of multiple oral GCT in the English-language literature, with only three cases reported in the last 20 years. This report describes a case of two oral GCT in a young patient and provides a literature review from 1995 to 2015. Clinical characteristics of the reviewed cases were retrieved and compared with the present case. Exceptionally, the current case was the only one that occurred in an adolescent and solely affected the oral cavity. Besides the oral cavity, the other cases also observed GCT lesions in the skin. Although rare, it is important to know multiple oral GCT clinical and histopathological characteristics so they can be correctly diagnosed, treated and followed up.

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Granular cell tumour is a rare tumour of neural origin usually located on the face and the neck. The biological behaviour is usually benign. However, certain clinical and histopathological features should alert physicians to a malignant behaviour. This case report describes the occurrence of a granular cell tumour in the inguinal area that resembled a malignant tumour. The histopathological study revealed typical features of granular cell tumour and an extension study confirmed the absence of metastasis. This case highlights the importance of considering this disorder in the differential diagnosis of ulcerated nodules and of managing atypical granular cell tumor appropriately.

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Granular cell tumour is a rare tumour of neural origin usually located on the face and the neck. The biological behaviour is usually benign. However, certain clinical and histopathological features should alert physicians to a malignant behaviour. This case report describes the occurrence of a granular cell tumour in the inguinal area that resembled a malignant tumour. The histopathological study revealed typical features of granular cell tumour and an extension study confirmed the absence of metastasis. This case highlights the importance of considering this disorder in the differential diagnosis of ulcerated nodules and of managing atypical granular cell tumor appropriately.

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Granular cell tumor is a rare benign neoplasm of neural origin. We report the case of a female patient, 27 years old presenting a brown-red nodule in the right arm, which pathological examination showed to be formed by polygonal cells with eosinophilic granular cytoplasm and immunohistochemistry positive for S100 protein and CD68. Granular cell tumor is usually solitary and in half the cases located in the head and neck areas, 30% of these in the tongue. It is most frequent between the third and fifth decades of life in women and people of African-American ethnicity. Its origination is controversial, including the possible origins in muscle, fibroblasts, neural crest, neural sheath or histiocytes. The positivity for S-100 and CD68 suggest the neural origin.

O tumor de células granulares é uma neoplasia benigna rara, de origem neural. Relatamos caso de paciente feminina, 27 anos, com nódulo de superfície acastanhada no braço direito, cujo exame anatomopatológico evidenciou densa proliferação de células, com amplo citoplasma contendo grânulos eosinofílicos, e imuno-histoquímica positiva para proteínas S100 e CD68. O tumor de células granulares é geralmente solitário e, em metade dos casos, localiza-se em cabeça e pescoço, dos quais 23% na língua. É mais frequente entre a terceira e a quinta décadas de vida, em mulheres e pessoas de etnia negra. A positividade para S-100 e CD68 favorece origem neural.

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