Oral Granular Cell Tumor Research Articles

Oral granular cell tumor: a collaborative clinicopathological study of 61 cases.

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. 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. 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 3.0cm (mean ± SD: 1.40 ± 0.75cm). 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. 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.

A CASE REPORT OF MULTIPLE GRANULAR CELL TUMORS OF TONGUE WITH REVIEW OF LITERATURE.

Also called as Abrikossoff's tumor, the granular cell tumor (GCT) can occur in any part of the body, most of them occur in the head and neck especially in the tongue, cheek and palate. Almost three fourth of the lesions seen in head and neck are present over the tongue, oral mucosa or hard palate. Both benign and malignant lesions have been reported; although occurrence of malignancy is rare, comprising of 2% of all granular cell tumors. Here we present a case report of granular cell tumor of tongue at two different sites in a 17-year-old girl with a brief review of literature on granular cell tumors. Although oral GCTs exhibit a benign behavior they may demonstrate a wide variety of features and architectural patterns. Rather than a true neoplasm, GCTs could be regarded as lesions that reect a local metabolic or reactive change.

Granular Cell Tumor: A Clinical Study of 81 Patients

BackgroundAlthough a granular cell tumor (GCT) usually develops on the skin or oral mucosa, it has been described in many other organs. GCT typically presents as a solitary tumor, but multiple lesions can occur. It has also been described in association with other diseases. ObjectiveTo describe the clinical characteristics of cutaneous and oral mucosal GCTs and explore potential associations with other diseases. Material and methodsRetrospective study of patients diagnosed with GCT at our hospital between 1995 and 2019. The following information was collected from the patients’ medical records: age, sex, number of GCTs, location, diameter, time to diagnosis, tentative clinical diagnosis, surgical margin status, recurrence, follow-up time, and associated diseases. ResultsWe detected 89 cutaneous or oral mucosal GCTs in 81 patients (43 women, 38 men) with a mean age of 40.21 years. The mean tumor diameter was 1.34 cm. Five of the 81 patients (6.2%) had multiple GCTs, including noncutaneous tumors. Patients with multiple GCTs were on average younger than those with a single tumor (P = .004). There was only a single case of local recurrence and no cases of distant metastasis. None of the patients had associated diseases. ConclusionsMost GCTs are benign and local recurrence is uncommon, even in patients with positive margins. Nevertheless, the possibility of multiple tumors affecting the skin, oral mucosa, or internal organs should be borne in mind, especially in young patients.

Whole-exome sequencing reveals novel vacuolar ATPase genes' variants and variants in genes involved in lysosomal biology and autophagosomal formation in oral granular cell tumors.

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. 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. 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. 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.

Tumor de células granulares. Estudio clínico de 81 pacientes

IntroductionAlthough a granular cell tumor (GCT) usually develops on the skin or oral mucosa, it has been described in many other organs. GCT typically presents as a solitary tumor, but multiple lesions can occur. It has also been described in association with other diseases. ObjectivesTo describe the clinical characteristics of cutaneous and oral mucosal GCTs and explore potential associations with other diseases. Material and methodsRetrospective study of patients diagnosed with GCT at our hospital between 1995 and 2019. The following information was collected from the patients’ medical records: age, sex, number of GCTs, location, diameter, time to diagnosis, tentative clinical diagnosis, surgical margin status, recurrence, follow-up time, and associated diseases. ResultsWe detected 89 cutaneous or oral mucosal GCTs in 81 patients (43 women, 38 men) with a mean age of 40.21 years. The mean tumor diameter was 1.34cm. Five of the 81 patients (6.2%) had multiple GCTs, including noncutaneous tumors. Patients with multiple GCTs were on average younger than those with a single tumor (P=.004). There was only a single case of local recurrence and no cases of distant metastasis. None of the patients had associated diseases. ConclusionsMost GCTs are benign and local recurrence is uncommon, even in patients with positive margins. Nevertheless, the possibility of multiple tumors affecting the skin, oral mucosa, or internal organs should be borne in mind, especially in young patients.

Oral granular cell tumour: A multicentric study of 56 cases and a systematic review.

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. 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. 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. 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.

Clinical and histopathological study of 19 cases of oral granular cell tumor, especially focused on vascular invasion

Clinical and histopathological study of 19 cases of oral granular cell tumor, especially focused on vascular invasion

GRANULAR CELL TUMOR S-100 NEGATIVE VARIANT

Granular cell tumor is a benign mesenchymal lesion that occurs more often in the oral cavity and skin. Its cell origin is controversial, including muscle, fibroblasts, neural crest, neural sheath and histiocytes. Granular cell tumors composed of larger and polygonal cells with abundant eosinophilic granular cytoplasm. Classic granular cell tumor shows positive immunostaining for S-100 and vimentin, and in some cases, positive CD68 staining is also reported positive. However, three cases of S100 negative granular cell tumor in oral mucosa and several cases in skin have also been reported in the literature recently. Therefore, the purpose of this study is to report a case of oral S100 negative granular cell tumor to increase the awareness of this entity. A 79-year-old female patient presented with a small asymptomatic, circumscribed mass lesion in the posterior lateral aspect of the palatal torus. Histologically, the specimen was covered by keratinized stratified squamous epithelium showing no pseudoepitheliomatous hyperplasia. The lamina propria consisted of fibrous connective tissue with chronic inflammation and cluster or sheet of large polygonal cells with granular cytoplasm. Immunohistochemistry showed that the granular cells stain positive for CD68 but negative for S100. Currently there is no difference in recommended treatment or in prognosis of S100 negative from S100 positive granular cell tumor. However, it is important to be aware of this rare variant of granular cell tumor for stablishing correct diagnosis.

Oral neural tumors: Clinicopathologic analysis of 157 cases and review of the literature.

BackgroundOral neural tumors (ONTs) are rare lesions and represent reactive or neoplastic proliferations of nerve sheath cells. The purpose of the present study is to report the clinical, demographic and histopathologic features of 157 ONTs diagnosed in a single Oral Pathology Department and review the pertinent literature.Material and Methods157 cases of ONTs diagnosed during a 44-year period were retrospectively collected and the diagnosis was reconfirmed by studying representative haematoxylin and eosin stained tissue sections. The patients’ gender and age, as well as the main clinical features of the lesions, were retrieved from the biopsy submission forms.ResultsThe 157 ONTs represented approximately 0.4% of 35,590 biopsies accessioned during the study period. They affected 71 male and 86 female patients with a mean age of 38.4±18.8 years. They mainly appeared as asymptomatic nodules of normal or white colour on the tongue, lip mucosa and hard palate. The most common ONT was granular cell tumour (38.9%) followed by neurofibroma (19.7%), schwannoma (15.9%), traumatic neuroma (15.9%), palisaded encapsulated neuroma (8.3%) and nerve sheath myxoma (1.3%).ConclusionsThis study confirmed the rarity of ONTs. Their clinical characteristics mimic other oral lesions; thus, microscopic examination is the only mean to arrive at a definitive diagnosis. Key words:Oral neural tumors; oral neural neoplasms; neurofibroma; oral neurofibroma; schwannoma; oral schwannoma; neurilemmoma; oral neurilemmoma; granular cell tumor; oral granular cell tumor; traumatic neuroma; oral traumatic neuroma; palisaded encapsulated neuroma; oral palisaded encapsulated neuroma.

Oral granular cell tumor: Report of case series and a brief review of the literature

Background/Aim: The present analysis focuses on examining a case series of eight patients diagnosed with a granular cell tumor located in the oral cavity. Case series: The patients’ clinical states were thoroughly studied, along with the histopathological and immunohistochemical examinations findings. Their surgical treatment and postoperative course are also within the scope of this analysis. Numerous histogenesis theories and the appropriate tumor treatment are mentioned within the article being always in accordance with the relative literature. Conclusions: Oral granular cell tumor is a benign oral disease of possible neural origin commonly located on the tongue. Surgical excision is the treatment of choice. In any case, histological and immunohistochemical examination confirm both the clinical diagnosis and the differential diagnosis between oral squamous cell carcinoma.

Lack of Utility of Cytokeratins in Differentiating Pseudocarcinomatous Hyperplasia of Granular Cell Tumors from Oral Squamous Cell Carcinoma.

Granular cell tumor (GCT) of the oral cavity is a benign lesion. Half of oral GCTs demonstrate pseudocarcinomatous hyperplasia (PCH) of the mucosa which can mimic invasive islands of oral squamous cell carcinoma (SCC). Such similarity can be confusing when diagnosing or evaluating the two conditions, potentially leading to misdiagnosis or misclassification. Indeed, several misdiagnosed cases of oral GCT have been reported in the literature as OSCC or malignant oral GCT that resulted in unnecessary aggressive treatment for the affected patients. The aim of this study was to investigate if the cytokeratin pattern of the PCH can help in differentiating GCT from oral SCC. To distinguish between these two entities, we examined 12 patient specimens of oral GCT-PCH and oral SCC histologically and via immunohistochemistry (IHC) for CK13, CK17 and P75. The results suggest that the cytokeratin profile of PCH is similar to that of oral SCC. Therefore, consideration of IHC findings for epithelial markers alone may lead to erroneous diagnosis; thus, the presence of the granular tumor underneath the PCH and its immunopositivity for P75 or other neural definition markers can be essential to identify the underlying tumor and exclude oral SCC. Finally we recommend more studies on the molecular biology of PCH to understand how it can mimic oral SCC histologically without harboring its malignant phenotype clinically, which could have significant translational potential for understanding invasive oral SCC.

Extra-tongue oral granular cell tumor: Histological and immunohistochemical aspect.

BackgroundGranular cell tumor (GCT) is an uncommon benign tumor founded in any part of the body but mainly in the tongue. Extra-tongue oral granular cell tumor (ETOGCT) is rare with few cases reported. Here we describe seven cases of oral GCT located in sites other then the tongue and discuss histopathological and immunohistochemical differences between differential diagnoses.Material and MethodsWe retrieved all cases diagnosed with oral granular cell tumor, from the Oral Pathology Service at the School of Dentistry/ University of São Paulo, and excluded the ones sited in the tongue. Immunohistochemical staining anti-S100 was also performed.ResultsThe presented cases of Extra-tongue Oral Granular Cell Tumor (ETOGT) are composed by granular cells with intimately association with the adjacent tissue. Atypia and mitoses were not seen, and in most cases, the typical pseudoepitheliomatous hyperplasia was not observed.ConclusionsThe importance of an adequate attention is to avoid misdiagnoses, since ETOGT is rare and the tricking histopathological findings could induce to it. All the cases can be differentiated from the tumors that has a granular cell proliferation through a morphological analysis and when needed, immunohistochemistry stain. Key words:Abrikossoff`s tumor, granular cell tumor, oral cavity.

Granular cell tumour of the tongue in a 17‐year‐old orthodontic patient: a case report

AbstractTo describe the clinical presentation of a granular cell tumour (GCT) in an orthodontic patient, as well as discuss its aetiology and treatment of choice. We present a case of GCT of the tongue in an otherwise healthy 17‐year‐old male patient along with a brief review of literature on GCTs. The lesion was surgically excised and orthodontic treatment was successfully finalised. Clinically, GCTs are indistinguishable from other benign connective tissue and neural tissue neoplasms and may be found in any site, with cases commonly involving the gastrointestinal system, breast and lung. However, over 50% of cases involve the head and neck, with the tongue being the most frequently involved site (65–85% of oral GCTs). GCTs demonstrate a close anatomical relationship with peripheral nerve fibres and demonstrate the presence of myelin and axon‐like structures thus lending credence to their neural origin. The treatment of choice for GCTs is conservative surgical excision. Because GCTs present with a potential for recurrence, follow‐up is recommended. While the primary focus of orthodontic treatment is the position of the teeth within the orofacial complex, the health and wellness of the patient and his/her surrounding oral tissues always take precedence. This case demonstrates the importance of routine physical examination of the intraoral and extraoral tissues during routine orthodontic care.

Evidence for loss of heterozygosity (LOH) at chromosomes 9p and 17p in oral granular cell tumors: a pilot study

The objective of this study was to assess loss of heterozygosity (LOH) in tumor suppressor gene loci in oral granular cell tumors (GCTs). We assessed LOH in 8 samples of oral GCT using polymorphic microsatellite markers at chromosome regions 3p, 9p, 11q, and 17p, flanking areas close to tumor suppressor genes. We further performed immunohistochemistry to detect the p53 and Ki-67 proteins and associated these expressions with the molecular results. Five samples showed LOH in 3 markers at chromosomes 9p and 17p (markers P53, AFM238WF2 and D9S162) with fraction of allelic loss of 42.8% for each of these markers. No LOH was identified in any other chromosome. LOH was not associated with the immunohistochemical expression of p53 and Ki-67. The present study shows LOH at chromosomes 9p and 17p in oral GCTs.

Case Series: Two Cases of an Atypical Presentation of Oral Granular Cell Tumour

This paper describes two cases of oral granular cell tumours with an atypical clinical presentation; both are in females aged between 45 and 63 years of age. Granular cell tumours are unusual soft tissue neoplasm of neural or Schwann cell origin. Oral GCTs usually present clinically as pink or yellow small sessile lesions. GCTs are usually benign in nature; however they can present in a malignant form in fewer than 2% of cases. In benign cases treatment is surgical and usually curative with extremely low recurrence rates.

The neural histogenetic origin of the oral granular cell tumor: An immunohistochemical evidence

Granular cell tumor (GCT) is a rare neoplasm that can appear in any site of the body, but most are located intraorally. Its histogenetic origin remains unclear. This report analyzes the immunoprofile of 15 cases of granular cell tumors, occurring in 13 women and 2 men and the lesions were located on the tongue or upper lip. Patient age ranged from 7 to 52. The patients demographic data and the cytological and architectural features of the lesions were analyzed in oral GCTs (n=15). The lesions were also submitted to a panel of immunohistochemical stains with antibodies against S-100, p75, NSE, CD-68, Ki-67, Synaptofisin, HHF-35, SMA, EMA, Chromogranin, Progesterone, Androgen and Estrogen. Among the fifteen cases analyzed, the most common location was the tongue (84.6%). Histologically, the tumors exhibited cellular proliferation composed mainly by polygonal cells presenting an abundant granular eosinophilic cytoplasm. The nuclei were central, and the cell membranes were moderately clear. No mitotic figures were observed. The immunohistochemical analysis showed positivity in all cases for S-100, p75, NSE and CD-68, and no immunoreactivity for Ki-67, Synaptofisin, HHF-35, SMA, EMA, Chromogranin, Progesterone, Androgen and Estrogen. The immunoprofile of granular cell tumors showed nerve sheath differentiation--lending support to their neural origin--and helping to establish a differential diagnosis between this lesion and other oral granular cell tumors, whether benign or malignant.

Oral granular cell tumor: a study of twelve cases in a Brazilian population

Objectives: Analyze clinic and epidemiological aspects of all Oral Granular cell tumor (GCT) during a 5-year period in a Brazilian population. Study design: clinical and treatment outcome data of patients with oral GCT seen at the Division of Stomatology, School of Dentistry, Federal University of Ceara, Brazil, between January 2001 and December 2005 were reviewed. Results: during the analyzed period, 6231 oral lesions were collected from files of different anatomo-pathology laboratories situated in the state of Ceara, Brazil. Of all cases, only 12 of GCT were diagnosed. The lesion was more common in female patients, with a mean age of 40.8 years, occurring mainly in tongue. Conclusion: Oral GCT is a rare and infrequent lesion in this population, and affects more female in the third, fourth a fifth decade of life. Clinical significance: Although it is a rare occurrence in the oral cavity, the clinician must know about its occurrence and should include GCT in the differential diagnose of certain lesions especially when situated in tongue.

Granular cell tumor of the oral cavity: updated immunohistochemical profile

Granular cell tumor (GCT) is a benign lesion that occurs at different body sites with preponderance to the oral cavity. It is generally believed to be of schwann cell/neural cell origin. We used a large panel of both traditional and recently developed antibodies in an attempt to trace the origin of GCTs on the basis of their immunoprofile. The patients' demographic data and the cytological and architectural features of the lesions were analyzed in a large series of oral GCTs (n = 68). Forty-two lesions were also submitted to a panel of immunohistochemical stains with antibodies against S-100, CD-68 (KP-1 and PG-M1), vimentin, calretinin, NKI/C3, PGP9.5, p75/NGFR and inhibin-alpha. The tongue was the most common location of oral GCTs (81%). The granular cells demonstrated a wide array of cytological features in terms of cell shape and position of the nucleus. In addition, the lesions showed different architectural patterns, including 'infiltration' with satellite nodules. Interestingly, no recurrences were reported, even in lesions that were not completely excised. Granular cells were usually found to be strongly and diffusely positive for p75, vimentin, calretinin and NKI/C3, inhibin-alpha, PGP9.5, and S-100. Immunoreactivity of the granular cells to a broad panel of antibodies that characterize different tissues does not confirm any particular cell type for the histogenetic origin of GCTs. Furthermore, GCTs could be regarded as lesions that reflect a local metabolic or reactive change rather than a true neoplasm.

Multiple congenital oral granular cell tumours in a newborn black female: a case report

IntroductionCongenital oral granular cell tumour of the newborn is an uncommon benign tumour of uncertain origin. The typical clinical appearance is of a single nodule occurring on the anterior maxillary ridge. In 10% of cases there are multiple lesions. The occurrence of congenital epulis in non-Caucasians is rare.Case presentationTwo firm pedunculated nodular lesions were noticed in the mouth of a 3-day-old black female: one on the anterior maxillary ridge and the other further posteriorly in the midline of the palate. Both lesions were excised when the baby was nine days old. Microscopic examination of the lesions showed densely packed round to oval cells with abundant granular eosinophilic cytoplasm and uniform nuclei. The diagnosis was congenital granular cell tumour.ConclusionCongenital oral granular cell tumour occurs almost exclusively in Caucasian newborns but also rarely in black infants. The parents should be assured of the benign nature and the simple treatment of the condition.

Oral granular cell tumour of the lip in an adult patient

Oral granular cell tumour is a rare soft tissue tumour of mesenchymal origin. The most frequently affected site in the oral cavity is the tongue, followed by the floor of mouth, and buccal mucosa. In paediatric patients, 25% of cases have been reported to occur in the lip, but this presentation in adults is extremely rare. We report a case of oral granular cell tumour in a 35 year-old female, located in the lower lip. Histopathological examination revealed eosinophilic granular cells which stained positively for S-100 protein; a finding supportive of a neural origin. A history of trauma was elicited in this case, and the lesion was treated with surgical excision.