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.

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

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

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

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

Granular cell 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.

Case for diagnosis

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.

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.

Case for diagnosis / Caso para diagnostico

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.

Case for diagnosis / Caso para diagnóstico

Granular cell tumor (Abrikossoff's tumor) is a rare benign disease that preferentially affects the cervicofacial segment. It is usually a solitary nodule that may ulcerate and present pearly infiltration on the borders, while keeping a clean background and a hyperchromic halo. This paper describes the case of an ulcerated granular cell tumor on an unusual location, which reinforces the necessity of including this tumor in the differential diagnosis of nodular-ulcerative skin lesions.

O tumor de células granulares (tumor de Abrikossoff) é uma doença rara, benigna, que acomete preferencialmente o segmento cérvico facial. Geralmente é uma lesão única, nodular, que pode ulcerar e apresentar bordas infiltradas e peroláceas, com fundo limpo e halo hipercrômico. Esse trabalho descreve um caso de tumor de células granulares ulcerado e de localização pouco usual reforçando a necessidade de se incluir esse tumor no diagnóstico diferencial das lesões nódulo-ulceradas da pele.

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.

Granular cell tumor on perianal region: a case report

Granular cell tumor [GCT] was first described by Abrikossoff in 1926. GCT is a rarely seen soft tissue tumor and is generally benign. While the tumor can be seen in all parts of the body it is generally located on the head and neck region, and especially on the tongue. GCT is rarely seen in the anal-perianal region. In accordance with literature this case was reported because it was thought to be the 27[th] anal-perianal located GCT case. In this case report, approximately 0,5-1 cm pedunculated polypoid lesion was determined in the perianal region during the physical examination of a 23 year old female patient who applied with palpable mass complaint in the perianal region. Lesion in the patient was totally excited with healthy skin-subcutaneous tissue under local anesthesia. A benign granular cell tumor was detected in the histopathological examination. Positive staining was monitored immunohistochemically with S-100 and neuron specific enolase [NSE]. GCT is a rarely seen tumor in the anal-perianal region and its malign transformation rate is very low. Even lesions seen in the perianal region have clinically a benign appearance, a histopathological examination should be conducted and also GCT should be kept in mind during diagnosis. Malign-benign separation of these lesions is difficult so histopathological examination should be conducted with great care. Large local excision in the treatment provides curative treatment. But for those presenting malign transformation further examination must be performed for metastasis. After the treatment local recurrence and metastasis should be considered carefully. Prognosis of metastatic disease is very bad

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

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

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

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

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

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

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

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

Isolated intra-oral granular cell tumor: report of two cases and review of the literature

Granular cell tumor (GCT) is a relatively uncommon lesion occurring in almost any part of the body, including the orofacial region. The tongue and the buccal mucosa are common intra oral sites. Although aggressive and malignant variants of this neoplasm have been described, most GCTs are benign. The histogenesis of the lesion still remains unknown. However, histochemical and ultra-structural studies propose the origin of the lesion from Schwann cells, striated muscle, mesenchymal cells, histiocytes and epithelial cells. The tumor generally occurs in middle-aged or older adults. The lesion is typically seen as an uninflamed asymptomatic mass measuring about two cm in diameter with reddish surface coloring. As most granular cell tumors are benign, surgical excision of the lesion is the treatment of choice. In this study, 2 oral GCT cases are presented, corresponding to 2 male patients, with a mean age of 27 years and a mean time of evolution of the lesions of 8 months. Both lesions were located on the tongue. Differential diagnoses include fibrous hyperplasia, minor salivary gland tumor, condyloma acuminatum and neurilemmoma. In all the cases a resection with safety margins of the lesions was carried out under local anesthesia. The samples were fixed and processed for histopathological study. The main clinical pathology and diagnostic features of this neoplasm are reviewed and discussed.

O tumor de celulas granulares (TCG) e uma lesao relativamente incomum que pode acometer qualquer parte do corpo, incluindo a regiao buco-maxilo-facial. A lingua e a mucosa bucal sao localizacoes intraorais comuns. Embora casos de transformacoes malignas e agressividade terem sido descritos, a maioria dos TCG sao benignos. A histogenese desta lesao continua desconhecida. No entanto estudos de imunohistoquimica e ultraestruturais sugerem que a lesao se origina das celulas de schwann, musculatura estriada, celulas mesenquimais, histiocitos e celulas epiteliais. O tumor geralmente ocorre na meia idade ou em pacientes idosos. A lesao e tipicamente vista como uma massa assintomatica nao inflamatoria que mede cerca de dois centimetros de diametro com uma superficie de coloracao avermelhada. A maioria dos tumores de celulas granulares sao benignos cuja excisao cirurgica e o tratamento de escolha. Neste estudo, 2 casos orais de TCG sao apresentados sendo ambos em pacientes do sexo masculino, com media de idade de 27 anos e tempo medio de evolucao da lesao de 8 meses. A localizacao de ambas as lesoes foi o dorso da lingual. O dignostico diferencial incluiu a hiperplasia fibrosa, tumor de glandulas salivares menores, condiloma aculminado e neiurilenoma. Em todos os casos a resseccao com margens de seguranca foi realizado sob anesthesia local. As amostras foram fixadas e processadas para o estudo histopatologicos. As principais caracteristicas clinicopatologicas e de disgnostico foram revisadas e discutidas.

Tumor odontogênico de células granulares: [revisão] / Granular cell odontogenic tumor: [review]

Introdução: os tumores odontogênicos de células granulares são neoplasias raras que normalmente se manifestam clinicamente de maneira benigna. O tumor ocorre em uma ampla faixa etária com uma predileção pela região de caninos a molares mandibulares. Objetivo: apresentar uma revisão da literatura do tumor odontogênico de células granulares. Conclusões: o tumor não apresenta comportamento biológico agressivo, porém pode ocorrer recorrência. O tratamento é conservador, sendo a curetagem e a enucleação os procedimentos de eleição.

Introduction: the granular cell odontogenic tumors are an uncommon neoplasm with benign clinical behavior. The lesion occurs over a wide age range with a typically predilection for the mandibular canine and molar regions. Aim: present a granular cell odontogenic tumor literature review. Conclusions: the tumor don’t present an aggressive biological behavior although recurrences can occur. The lesion is treated by conservative surgery w ith curettage and enucleation as election procedures.