A rare case of cutaneous granular cell tumor with unusual Melan-A expression in a child.

Granular cell tumors (GCTs) are rare, indolent neoplasms classically characterized by eosinophilic granular cytoplasm, infiltrations of polygonal cells in the collagenous stroma, and pustulo-ovoid bodies of Milian. We describe a case of a 10-year-old female presenting with a GCT of the upper arm, remarkable for positive Melan-A expression without additional melanocytic features. The differentiation between granular cells versus melanocytic neoplasms carries significant implications for clinical management, and such diagnoses should be considered carefully in the setting of unusual immunophenotypes.

Gastrointestinal Tract Granular Cell Tumor in the Pediatric Population: A Multicenter Experience.

BACKGROUND: Pediatric granular cell tumors (GCT) involving the gastrointestinal tract (GIT) are rare with limited case report/series reported to date. METHODS: Multicenter retrospective study of pediatric GIT GCT. RESULTS: A total of 10 cases were included in the study with a median age of 13.5 years (range: 7-18 years) and were predominantly female patients (60%). In half of the patients no significant medical history was present with the remaining 5 having Crohn disease (10%), eosinophilic esophagitis (EoE) (10%), Crohn disease and EoE (10%), growth hormone deficiency (10%), and aplasia cutis congenita (10%). The GCT median size was 1.3 cm (range: 1-1.6 cm) and were more commonly located in the esophagus (70%) followed by the stomach (20%) and rectum (10%). Most of the cases showed round/polygonal tumor cells with abundant granular cytoplasm, and none of the cases had nuclear atypia, increased mitotic activity, or tumor cell necrosis. None of our cases received specific therapy for GCT other than clinical follow-up, and none of the patients had evidence of local recurrence or metastatic disease. CONCLUSION: We present our multicenter experience with GIT GCT, all cases had a benign course. Interestingly, 4 of the esophageal GCT cases (including 2 patients with EoE) showed an eosinophil-rich esophagitis in the underlying mucosa.

Simultaneous granular cell tumor and seminoma in the descended testis of a cryptorchid rabbit.

Testicular tumors are rarely reported in rabbits. In this case study, a 4-year-old Holland lop rabbit, previously diagnosed with unilateral cryptorchidism, was presented because of enlargement of the descended testis. The rabbit was clinically normal. Following unilateral orchiectomy and scrotal ablation, histopathological analysis revealed 2 distinct types of testicular tumor in the descended testis: a granular cell tumor and a seminoma. To the best of the author's knowledge, this is the first documented report of simultaneous testicular tumors in the testis of a rabbit with unilateral cryptorchidism.

Tumeur à cellules granulaires et séminome simultanés dans le testicule descendu d'un lapin cryptorchideLes tumeurs testiculaires sont rarement rapportées chez le lapin. Dans cette étude de cas, un lapin Holland Lop de 4 ans, précédemment diagnostiqué avec une cryptorchidie unilatérale, a été présenté en raison d'une hypertrophie du testicule descendu. Le lapin était cliniquement normal. Après orchidectomie unilatérale et ablation scrotale, l'analyse histopathologique a révélé 2 types distincts de tumeur testiculaire dans le testicule descendu : une tumeur à cellules granuleuses et un séminome. À la connaissance de l'auteur, il s'agit du premier rapport documenté de tumeurs testiculaires simultanées dans le testicule d'un lapin atteint de cryptorchidie unilatérale.(Traduit par Dr Serge Messier).

Primary Intraosseous Granular Cell Tumor of the Sphenoid and Central Skull Base in a Pediatric Patient.

BACKGROUND: Granular cell tumors occur in all ages and many anatomic sites. In the craniofacial region, they typically arise in soft tissue, not bone. We present a primary intra-osseous granular cell tumor of the sphenoid and central skull base arising in a 12- year- old girl. CASE REPORT: A 12-year-old female with sickle cell disease and Jeavons syndrome presented with seizures. Imaging and partial resection revealed an expansile benign granular cell tumor (GCT) involving the sphenoid body, pterygoid process, and central skull base. The disease has remained stable after 36-month follow up. DISCUSSION: GCT primarily involving the osseous sphenoid/skull base has not been previously reported in a child. Although mostly benign, some are aggressive, with malignant transformation in 1-2%. Surgery is the mainstay of treatment, but in the skull base this may be limited by adjacent critical structures. Decision-making is guided by anatomic extent, histology, and clinical behavior.

Unique Case of Rare Non-Neural Granular Cell Tumor of the Rectus Abdominis Muscle.

Background and Objectives: Our report contributes a unique case of a non-neural GCT occurring in an unusual location, with its development during pregnancy adding to its rarity. Materials and Methods: Granular cell tumors (GCTs), also known as Abrikossoff's tumors, are rare neoplasms of Schwann cell origin with predominantly benign behavior. We present a case of a 29-year-old female with a non-neural variant of a GCT discovered incidentally during a cesarean section, situated on the posterior surface of the rectus abdominis muscle. Results: Histologically, the tumor exhibited features consistent with a benign non-neural GCT, confirmed through an immunohistochemical analysis. Despite the atypical presentation and challenging surgical removal due to prior scarring, the patient experienced no postoperative complications and showed no signs of recurrence during follow-up. Conclusions: This case highlights the importance of considering GCTs in differential diagnoses, particularly in unusual anatomical locations, and underscores the favorable prognosis associated with timely surgical intervention.

[Breast granular cell tumor]. / Zernisto-kletochnaya opukhol' molochnoi zhelezy.

The report of the biopsy diagnosis of the granular cell tumor with rare localization in the breast is given. Currently, the tumor is considered a neoplasm of neuroectodermal origin. Differential diagnostic criteria for the tumor are positive expression in cytoplasm of protein S-100, absence of expression of epithelial antigens, histiocytic antigens, oncoproteins, estrogens and progesterone receptors, PAS-positive reaction of intracellular granules. With ultrasound examination and mammography, a tumor always initially assessed as cancer or calcification.

Cytological diagnosis of cutaneous granular cell tumor: Rare tumor with rare presentation.

ABSTRACT: Granular cell tumors (GCTs) are uncommon soft tissue tumors, which are difficult to diagnose merely by clinical examination. Fine-needle aspiration cytology (FNAC), being an effective first-line investigation, plays a significant role in the preoperative diagnosis of GCT. However, the tumor can mimic certain other lesions; hence, a cytopathologist needs to be aware of its characteristic morphology. We report here a case of GCT, presented as a subcutaneous nodule in the first finger web. A differential diagnosis of lipoma/neurofibroma was made clinically. FNAC was done and showed characteristic features of granular cell tumor along with intranuclear inclusions and subsequently, it was confirmed on histopathology.

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

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.

From prenatal diagnosis to surgical treatment: two case reports of congenital granular cell epulis.

Herein, we detail a multidisciplinary approach and sequential treatment for two infants with congenital granular cell epulis (CGCE). Ultrasonic examinations at 34 weeks of gestation revealed prominent oral masses in both fetuses. To devise a carefully considered treatment strategy, a comprehensive multidisciplinary consultation including oral and maxillofacial surgeons, pediatricians, obstetricians, and anesthesiologists was convened. Following cesarean sections, the lesions were successfully removed, measuring approximately 30 × 15 mm and 30 × 20 mm in size, respectively. Immunohistochemical analysis showed that vimentin was positive, S-100 protein was negative, and NSE protein and CD68 protein were negative. These findings underscore the importance of prenatal diagnosis of congenital granular cell epulis for the effective management of these rare benign conditions.

Granular cell tumour of the breast.

Granular cell tumours (GCT) of the breast have similar clinical and radiological features to breast carcinomas. We present a case of a female patient with a tender, palpable lump, and associated skin changes. Imaging of the lesion was suspicious of malignancy. Initial histological examination showed uniform sheets of polygonal cells with abundant granular cytoplasm, and follow-up immunohistochemistry showed strongly positive staining of tumour cells with S100 and CD68, confirming the diagnosis of GCT. Wide local excision with complete resection margins was performed as a curative treatment for this lesion. This case report highlights the importance of considering GCTs in the differential diagnoses of breast lesions suspicious of malignancy and emphasises the necessity of accurate diagnosis of GCT for proper treatment.

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.

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.

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)

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.

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.