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Here, we describe the first case of a granular cell tumor (GCT) derived from the brachial nerve. Eleven-year-old neutered female Chihuahua presented to the hospital with a bulge from the left neck to the axilla. The dog had a spherical subcutaneous mass on the cervical subcutis, and cytology hinted at adenocarcinoma or neuroendocrine tumor. However, the origin of the tumor remains unknown. During resection of the mass, bleeding was difficult to control owing to the high blood flow, and tumor removal was extremely difficult. The caudal aspect of the mass was attached to the brachial nerve and had to be removed, along with parts of the nerve fibers. The patient's postoperative course was fair, but it developed paralysis of the left thoracic limb. Pathology revealed that the mass was positive for S100 and vimentin, and GCT was diagnosed. Non-oral GCTs are extremely rare. The clinical diagnosis of GCT is difficult and is often confirmed histopathologically by excision. Although most cases of GCT are benign, they must be recognized as hemorrhagic, indistinct masses that mimic malignancy. Excision carries the risk of hemorrhage and damage to the surrounding tissues to secure margins.

Descrevemos aqui o primeiro caso de um tumor de células granulares (TCG) derivado do nervo braquial. Uma chihuahua castrada de 11 anos de idade deu entrada no hospital com uma protuberância do pescoço esquerdo até a axila. A cadela apresentava uma massa subcutânea esférica no subcutâneo cervical, e a citologia indicava adenocarcinoma ou tumor neuroendócrino. Entretanto, a origem do tumor permanece desconhecida. Durante a ressecção da massa, foi difícil controlar o sangramento devido ao alto fluxo sanguíneo, e a remoção do tumor foi difícil. O aspecto caudal da massa estava ligado ao nervo braquial e teve de ser removido, juntamente com partes das fibras nervosas. A evolução pós-operatória da paciente foi regular, mas ele desenvolveu paralisia do membro torácico esquerdo. O exame anatomopatológico revelou que a massa era positiva para S100 e vimentina, e o TCG foi diagnosticado. Os TCGs não orais são extremamente raros. O diagnóstico clínico do TCG é difícil e geralmente é confirmado histopatologicamente por excisão. Embora a maioria dos casos de TCG seja benigna, eles devem ser reconhecidos como massas hemorrágicas e indistintas que simulam malignidade. A excisão acarreta o risco de hemorragia e danos aos tecidos circundantes para garantir as margens.

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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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To report a case of non-neural granular cell tumor (NN-GCT), an uncommon neoplasm, with only six studies worldwide describing cases involving the oral cavity. Methods: A 26-year-old male patient with an erythematous, firm, polypoid nodule in the floor of the mouth that exhibited areas of ulceration and mild bleeding to the touch. A biopsy was performed to aid in the diagnosis. Results: Based on the histopathological and immunohistochemical results (vimentin +, CD68 +, S100 -), the diagnosis was compatible with S100-negative (primitive polypoid non-neural) granular cell tumor. No recurrence was observed over two years of follow-up. Conclusion: The diagnosis of NN-GCT is extremely challenging because this tumor shares histological and immunophenotypic features with many benign and malignant tumors. Although oral NN-GCT may exhibit unusual and atypical histological features, it has an indolent behavior. Thus, until more cases of oral involvement are reported, complete resection and close follow-up are recommended

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Granular cell tumors are predominantly benign soft tissue tumors originating from Schwann cells, whereas melanocytic nevi are benign proliferations of melanocytes. We present the case of a patient with the presence of both entities located in the cecum and anal canal, respectively, constituting an extremely rare coincidental finding. A 43-year-old woman was evaluated by colonoscopy for iron-deficiency microcytic anemia that had lasted for 1 year. Colonoscopy demonstrated a macular lesion of 0.3 cm with a melanocytic appearance in the anal canal; at the cecum level, a subepithelial, yellowish, and partially mobile firm nodular lesion measuring 1.3 cm was observed. A histopathological study showed a melanocytic nevus in the anal canal and a granular cell tumor in the cecum. This is the first reported case of a patient with the extremely rare coincidental-incidental finding of these 2 entities at the same time.

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Resumen ANTECEDENTES: El tumor de células granulares de la vulva es poco común y de origen neurogénico. Afecta, principalmente, a mujeres entre 60 y 70 años y es más frecuente en la raza negra. CASO CLÍNICO: Paciente de 63 años, con una lesión vulvar indolora y no pruriginosa, en crecimiento. En la exploración se observó una lesión excrecente de 2.5 cm en la región superior del labio mayor izquierdo, dura, vascularizada y con infiltración a 2 cm de profundidad. No se palparon adenopatías sospechosas. Luego del reporte de la biopsia, sugerente de tumor de células granulares, se practicó una escisión completa, con márgenes libres. El estudio inmunohistoquímico se reportó positivo para CD68, S100 y TFE3 lo que confirmó el diagnóstico. Puesto que el índice proliferativo (Ki67) fue inferior al 5% y los márgenes quirúrgicos estaban libres, no se requirió tratamiento adyuvante. La paciente permanece en seguimiento y sin signos de recidiva. CONCLUSIÓN: Si bien los tumores de células granulares de la vulva son poco comunes y casi siempre benignos, deben incluirse en el diagnóstico diferencial de una tumoración vulvar. La inmunohistoquímica es la herramienta más útil para el diagnóstico preciso y su tratamiento de elección es la escisión local amplia, por su tendencia a la recurrencia local.

Abstract BACKGROUND: Granular cell tumor of the vulva is rare and neurogenic in origin. It mainly affects women between 60 and 70 years of age and is more frequent in black women. CLINICAL CASE: A 63-year-old woman with a painless, non-pruritic, growing vulvar lesion. Examination revealed a 2.5 cm excrescent lesion in the upper region of the left labium majus, hard, vascularized and infiltrated to a depth of 2 cm. No suspicious lymph nodes were palpated. After the biopsy report, suggestive of granular cell tumor, complete excision was performed, with free margins. The immunohistochemical study was positive for CD68, S100 and TFE3 which confirming the diagnosis. Since the proliferative index (Ki67) was less than 5% and the surgical margins were clear, adjuvant treatment was not required. The patient remains in follow-up with no signs of recurrence. CONCLUSION: Although granular cell tumors of the vulva are rare and almost always benign, they should be included in the differential diagnosis of a vulvar tumor. Immunohistochemistry is the most useful tool for accurate diagnosis and their treatment of choice is wide local excision because of their tendency for local recurrence.

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INTRODUCTION AND IMPORTANCE: Granular cell tumors (GCT) are rare neoplasms. The most common places are the head and neck. The larynx accounts for 3-10% of cases. PRESENTATION OF CASE: We present the case of a man who consulted our Department of Otorhinolaryngology due to long-term progressive dysphonia without dyspnea or dysphagia. Fibrolaryngoscopy revealed a tumor that affected the left vocal cord. The tumor was surgically resected. The histopathological report revealed a laryngeal GCT. Fourteen months after surgery, the patient had no recurrence. CLINICAL DISCUSSION: Patients with GCT of the larynx typically have persistent hoarseness, stridor, dysphagia and otalgia, but can also be asymptomatic. A definitive diagnosis is provided by histopathology with immunohistochemistry, and the treatment of choice is surgical resection. Unlike the literature, our report concerns a young man. CONCLUSION: Granular cell tumor is a rare tumor of the larynx and should be considered a differential diagnosis in all adult men with long-standing dysphonia.

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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.

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RESUMEN Fundamento: el mioblastoma de células granulares también conocido como tumor de Abrikossoff es una neoplasia benigna de rara frecuencia formada por células de aspecto granular. Objetivo: exponer aspectos clínicos del mioblastoma de células granulares. Presentación del caso: se reportó el caso de un paciente masculino de raza negra, 50 años de edad, que fue remitido de la Atención Primaria con impresión clínica de fibroma lingual, al examen bucal se observó lesión ovoide de 3 cm de diámetro en el dorso de la lengua, asintomática, firme, hipocoloreada, consistencia dura y bordes precisos. Se realizó exéresis mediante biopsia escisional. El diagnóstico histopatológico determinó mioblastoma de células granulares. Conclusiones: tanto las características clínicas como histológicas del tumor de células granulares son muy semejantes a otras neoplasias malignas que se asientan en la lengua como el carcinoma epidermoide por tanto su diagnóstico constituye un reto para el estomatólogo.

ABSTRACT Background: granular cell tumor known as Abrikossoff´s tumor is a benign neoplasm of rare incidence formed by cell of granular aspects. Objective: to expose clinical aspects of granular cell tumor. Case report: a clinical case is reported of a 50 years-old black male patient. He was remitted of Primary Health Service with diagnostic impression of tongue's fibroma, in the oral exam was detected an oval lesion of 3cm of diameter on dorsum of the tongue, asymptomatic, firm consistency, and well defined. The lesion was removed by excision biopsy. The histological-pathological study determined a granular cell tumor. Conclusions: both the clinical and histological characteristics of the granular cell tumor are very similar to other malignant neoplasm that settle on the tongue such as epidermoid cell carcinoma therefore its diagnosis constitutes a challenge for the dentistry.

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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.

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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.

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In rodents, status epilepticus (SE) triggered by chemoconvulsants can differently affect the proliferation and fate of adult-born dentate granule cells (DGCs). It is unknown whether abnormal neurogenesis results from intracellular signaling associated with drug-receptor interaction, paroxysmal activity, or both. To test the contribution of these factors, we systematically compared the effects of kainic acid (KA)- and pilocarpine (PL)-induced SE on the morphology and localization of DGCs generated before or after SE in the ipsi- and contralateral hippocampi of mice. Hippocampal insult was induced by unilateral intrahippocampal (ihpc) administration of KA or PL. We employed conditional doublecortin-dependent expression of the green fluorescent protein (GFP) to label adult-born cells committed to neuronal lineage either one month before (mature DGCs) or seven days after (immature DGCs) SE. Unilateral ihpc administration of KA and PL led to bilateral epileptiform discharges and focal and generalized behavioral seizures. However, drastic granule cell layer (GCL) dispersion occurred only in the ipsilateral side of KA injection, but not in PL-treated animals. Granule cell layer dispersion was accompanied by a significant reduction in neurogenesis after SE in the ipsilateral side of KA-treated animals, while neurogenesis increased in the contralateral side of KA-treated animals and both hippocampi of PL-treated animals. The ratio of ectopic neurons in the ipsilateral hippocampus was higher among immature as compared to mature neurons in the KA model (32.8% vs. 10.0%, respectively), while the occurrence of ectopic neurons in PL-treated animals was lower than 3% among both mature and immature DGCs. Collectively, our results suggest that KA- and PL-induced SE leads to distinct cellular alterations in mature and immature DGCs. We also show different local and secondary effects of KA or PL in the histological organization of the adult DG, suggesting that these unique epilepsy models may be complementary to our understanding of the disease. NEWroscience 2018.

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BACKGROUND: Granular cell tumors (GCTs) are rare neuroectodermal soft tissue neoplasms that mainly affect the skin of the upper limbs and trunks and the oral cavity. GCTs are derived from Schwann cells and, ultrastructurally, their intracytoplasmic granules are considered autophagosomes or autophagolysosomes and are consistent with myelin accumulation. METHODS: In this study, a convenience set of 22 formalin-fixed, paraffin-embedded samples of oral GCTs, all but one sample located at the tongue, was screened for mutations by whole-exome (WES) or targeted sequencing. RESULTS: WES revealed two novel variants in genes of the vacuolar ATPase (V-ATPase) complex: ATP6AP1 frameshift c.746_749del, leading to p.P249Hfs*4, and ATP6V1A non-synonymous c.G868A, leading to p.D290N. Each of these mutations occurred in one case. With regard to the samples that were wild type for these V-ATPase variants, at least two samples presented variants in genes that are part of endosomal/lysosomal/autophagosomal networks including ABCA8, ABCC6, AGAP3, ATG9A, CTSB, DNAJC13, GALC, NPC1, SLC15A3, SLC31A2, and TMEM104. CONCLUSION: Although the mechanisms involved in oral GCT initiation and progression remain unclear, our results suggest that oral GCTs have V-ATPase variants similarly to GCTs from other tissues/organs, and additionally show variants in lysosomes/endosomes/autophagosomal genes.

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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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PURPOSE: Granular cell tumors (GCT) are highly vascularized and adherent to adjacent structures, and so, complete resection represents a challenge. Adjuvant therapy decisions for residual GCTs currently relies on individual clinician decisions due to a paucity of systematic literature data. We present a comprehensive analysis about the impact of adjuvant therapy in reported cases of patients with incomplete GCT resection. METHODS: One database (PubMed) and crossed references were queried for GCT with incomplete resection or biopsy from 1962 to 2020. Literature review was performed according to the PRISMA guidelines. Also, two patients with residual GCT from our institutions are added to the analysis. Data regarding clinical presentation, surgical approach, use of adjuvant therapy, Ki-67 labeling, and follow up assessments were extracted and analyzed from selected publications. RESULTS: Thirty-three studies met the predetermined inclusion criteria and 53 patients were selected (including our two reported cases). The median of age was 49 [IQR, 39-60 years], with a slight male predominance (1.2:1). Among the surgical procedures, seven (13%) were biopsies alone. Adjuvant therapy was used in 18 patients (radiotherapy, 94.5%; chemotherapy, 5.5%) but there is no statistical correlation with adjuvant therapy and the progression of the remnant tumor (p = 0.33). Our institutions' patients did not receive adjuvant therapy and did not show tumor progression on MRI. CONCLUSION: Our systematic literature review suggests there is a limited role for chemo and/or radiotherapy in the management of incomplete GCT resection. It may be reasonable recommending close clinical follow up in patients with incomplete resection.

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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: To review the imaging features of granular cell tumors of the breast (on mammography, ultrasound, and magnetic resonance imaging), establishing a pathological correlation, in order to familiarize radiologists with this entity and make them aware of the differential diagnoses, other than malignancy, of lesions with spiculated margins. MATERIALS AND METHODS: We reviewed the medical records (from a clinical-pathology database and picture archiving and communication system) of five patients with a pathologically confirmed diagnosis of granular cell tumor of the breast, treated at the Portuguese Oncology Institute of Lisbon, in the city of Lisbon, Portugal, between January 2012 and December 2018. RESULTS: All five tumors exhibited imaging features highly suggestive of malignancy (BI-RADS 5 lesions), namely spiculated margins, significant depth, and posterior acoustic shadowing (on ultrasound). One tumor showed a kinetic curve indicative of washout on magnetic resonance imaging, two were adherent to the pectoralis muscle, and one was accompanied by skin retraction. Pathology provided the definitive diagnosis in all cases. CONCLUSION: Granular cell tumors of the breast pose a diagnostic challenge because they can present with clinical and imaging features mimicking malignancy, and the diagnosis is therefore provided by pathology. Radiologists should be familiarized with this entity, so they can be aware of the fact that breast lesions with spiculated margins can be indicative of diagnoses other than malignancy.

OBJETIVO: Rever as características de imagem dos tumores de células granulares da mama (mamográficas, ultrassonográficas e de ressonância magnética), estabelecendo uma correlação anatomopatológica, no intuito de proporcionar aos radiologistas uma familiarização com esta entidade e alertar para outros diagnósticos diferenciais de lesões espiculadas além das malignas. MATERIAIS E MÉTODOS: Consulta dos processos clínicos (base de dados clínica, anatomopatológica e sistema de comunicação e arquivamento de imagens) de doentes seguidos no Instituto Português de Oncologia de Lisboa, com diagnóstico anatomopatológico confirmado de tumor de células granulares da mama, de janeiro de 2012 a dezembro de 2018. RESULTADOS: Todos os tumores exibiram características de imagem altamente sugestivas de malignidade (BI-RADS 5), nomeadamente espiculações, crescimento em profundidade e atenuação posterior (ultrassonografia), um mostrou um perfil cinético com washout na ressonância magnética, dois estavam aderentes ao músculo peitoral e um associava retração cutânea. O diagnóstico definitivo foi anatomopatológico. CONCLUSÃO: Os tumores de células granulares da mama constituem um desafio diagnóstico, pois podem apresentar características clínicas e de imagem que mimetizam malignidade, pelo que o diagnóstico é anatomopatológico. Os radiologistas devem estar familiarizados com esta entidade de forma a considerá-la nos diagnósticos diferenciais de lesões espiculadas, além das lesões malignas.

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Abstract Objective: To review the imaging features of granular cell tumors of the breast (on mammography, ultrasound, and magnetic resonance imaging), establishing a pathological correlation, in order to familiarize radiologists with this entity and make them aware of the differential diagnoses, other than malignancy, of lesions with spiculated margins. Materials and Methods: We reviewed the medical records (from a clinical-pathology database and picture archiving and communication system) of five patients with a pathologically confirmed diagnosis of granular cell tumor of the breast, treated at the Portuguese Oncology Institute of Lisbon, in the city of Lisbon, Portugal, between January 2012 and December 2018. Results: All five tumors exhibited imaging features highly suggestive of malignancy (BI-RADS 5 lesions), namely spiculated margins, significant depth, and posterior acoustic shadowing (on ultrasound). One tumor showed a kinetic curve indicative of washout on magnetic resonance imaging, two were adherent to the pectoralis muscle, and one was accompanied by skin retraction. Pathology provided the definitive diagnosis in all cases. Conclusion: Granular cell tumors of the breast pose a diagnostic challenge because they can present with clinical and imaging features mimicking malignancy, and the diagnosis is therefore provided by pathology. Radiologists should be familiarized with this entity, so they can be aware of the fact that breast lesions with spiculated margins can be indicative of diagnoses other than malignancy.

Resumo Objetivo: Rever as características de imagem dos tumores de células granulares da mama (mamográficas, ultrassonográficas e de ressonância magnética), estabelecendo uma correlação anatomopatológica, no intuito de proporcionar aos radiologistas uma familiarização com esta entidade e alertar para outros diagnósticos diferenciais de lesões espiculadas além das malignas. Materiais e Métodos: Consulta dos processos clínicos (base de dados clínica, anatomopatológica e sistema de comunicação e arquivamento de imagens) de doentes seguidos no Instituto Português de Oncologia de Lisboa, com diagnóstico anatomopatológico confirmado de tumor de células granulares da mama, de janeiro de 2012 a dezembro de 2018. Resultados: Todos os tumores exibiram características de imagem altamente sugestivas de malignidade (BI-RADS 5), nomeadamente espiculações, crescimento em profundidade e atenuação posterior (ultrassonografia), um mostrou um perfil cinético com washout na ressonância magnética, dois estavam aderentes ao músculo peitoral e um associava retração cutânea. O diagnóstico definitivo foi anatomopatológico. Conclusão: Os tumores de células granulares da mama constituem um desafio diagnóstico, pois podem apresentar características clínicas e de imagem que mimetizam malignidade, pelo que o diagnóstico é anatomopatológico. Os radiologistas devem estar familiarizados com esta entidade de forma a considerá-la nos diagnósticos diferenciais de lesões espiculadas, além das lesões malignas.

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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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ABSTRACT The granular cell tumor (GCT) is a neoplasm of connective tissue which rarely occurs in children. This case report aims to describe and discuss the presentation of this tumor in a female neonate. Compared to previous literature, the case report demonstrated a rare form of presentation of this tumor in an unusual age group.

RESUMEN O tumor de células granulares (TCG) é uma neoplasia de tecido conjuntivo que ocorre raramente em crianças. Este relato de caso descreve e discute a apresentação desse tumor em um neonato do sexo feminino. Em comparação com estudos anteriores, o caso reportado demonstrou uma forma rara de apresentação em uma faixa etária não usual.

RESUMO O tumor de células granulares (TCG) é uma neoplasia de tecido conjuntivo que ocorre raramente em crianças. Este relato de caso descreve e discute a apresentação desse tumor em um neonato do sexo feminino. Em comparação com estudos anteriores, o caso reportado demonstrou uma forma rara de apresentação em uma faixa etária não usual.

RESUMO

ABSTRACT A 16-days-old female patient exhibiting an exophytic nodular lesion located at anterior maxilla on the alveolar ridge since birth. The clinical hypothesis of congenital granular cell epulis (CGCE) was established, and the patient underwent excisional biopsy. Microscopically, a sheet-like proliferation of eosinophilic cells with granular cytoplasm was observed in a stroma of vascularized fibrous connective tissue. The immunohistochemical analysis for S100 evidenced the absence of immunostaining. The CGCE hypothesis was confirmed and, after six months, the patient showed no signs of recurrence of the lesion.

RESUMEN Niña de 16 días de edad, presentaba una lesión exofítica de aspecto nodular ubicada en la región de la cresta alveolar maxilar anterior desde su nacimiento. Se estableció la hipótesis clínica de épulis congénito de células granulares, y la paciente se sometió a una biopsia por escisión. Microscópicamente se ha observado una proliferación laminar de células eosinofílicas con citoplasma granular en medio de un estroma de tejido conectivo fibroso vascularizado. El análisis inmunohistoquímica para S100 reveló ausencia de inmunomarcación. La hipótesis de épulis congénito ha sido confirmada, y, después de seis meses, la paciente no mostró señales de recidiva de la lesión.

RESUMO Paciente do sexo feminino, 16 dias de idade, exibiu lesão exofítica de aspecto nodular localizada em região anterior do rebordo alveolar maxilar desde o nascimento. A hipótese clínica de epúlide de células granulares congênita (ECGC) foi estabelecida, e a paciente foi submetida à biópsia excisional. Microscopicamente, observou-se a proliferação em lençol de células eosinofílicas com citoplasma granular em meio a um estroma de tecido conjuntivo fibroso vascularizado. A análise imuno-histoquímica para S100 evidenciou ausência de imunomarcação. A hipótese de ECGC foi confirmada e, após seis meses, a paciente não apresentou sinais de recidiva da lesão.