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.

Treatment modalities and outcomes of granular cell tumors and spindle cell oncocytomas of the pituitary gland: an analysis of two national cancer databases.

BACKGROUND: Spindle cell oncocytomas (SCO) and granular cell tumors (GCT) are rare primary pituitary neoplasms; the optimal treatment paradigms for these lesions are unknown and largely unexplored. Thus, using national registries, we analyze the epidemiology, management patterns, and surgical outcomes of SCOs and GCTs. METHODS: The National Cancer Database (NCDB; years 2003-2017) and the Surveillance, Epidemiology, and End Results Program (SEER; years 2004-2018) were queried for patients with pituitary SCOs or GCTs. Incidence, extent of surgical resection, and rate of postoperative radiation use for subtotally resected lesions comprised the primary outcomes of interest. All-cause mortality was also analyzed via time-to-event Kaplan-Meier curves. RESULTS: SCOs and GCTs have an annual incidence of 0.017 and 0.023 per 1,000,000, respectively. They comprise 0.1% of the benign pituitary tumors registered in NCDB. A total of 112,241 benign pituitary tumors were identified in NCDB during the study period, of which 83 (0.07%) were SCOs and 59 (0.05%) were GCTs. Median age at diagnosis was 55 years, 44% were females, and median maximal tumor diameter at presentation was 2.1 cm. Gross total resection was achieved in 54% patients. Ten patients (7%) had postoperative radiation. Comparing patients with GCTs versus SCOs, the former were more likely to be younger at diagnosis (48.0 vs. 59.0, respectively; p < 0.01) and female (59% vs. 34%, p = 0.01). GCTs and SCOs did not differ in terms of size at diagnoses (median maximal diameter: 1.9 cm vs. 2.2 cm, respectively; p = 0.59) or gross total resection rates (62% vs. 49%, p = 0.32). After matching SCOs and GCTs with pituitary adenomas on age, sex, and tumor size, the former were less likely to undergo gross total resection (53% vs. 72%; p = 0.03). Patients with SCOs and GCTs had a shorter overall survival when compared to patients with pituitary adenomas (p < 0.01) and a higher rate of thirty-day mortality (3.1% vs 0.0%; p = 0.013). CONCLUSION: SCOs and GCTs are rare pituitary tumors, and their management entails particular challenges. Gross total resection is often not possible, and adjuvant radiation might be employed following subtotal resection.

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.

Granular Cell Tumor (Abrikossoff's Tumor) of the Vulva in Childhood.

Granular cell tumors (GCTs) (Abrikossoff's tumors) are rare neoplasms derived from Schwann cells. Immunohistochemistry remains the most useful instrument for diagnosing GCTs. Complete surgical excision has been demonstrated to be curative for benign lesions. However, long-term follow-up in these patients is strongly recommended.

[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.

Incidental multifocal granular cell tumor in the setting of chronic gastritis discovered following gastric sleeve operation: a case report and brief review of the literature.

Granular cell tumors are uncommon neoplasms of Schwannian origin that can present nearly anywhere in the body, but are most commonly seen in the head and neck region. While the majority of these tumors are benign, a subset can behave aggressively and even have metastatic potential. We are reporting a case of a 35-year-old female with a history morbid obesity presenting for bariatric surgery (sleeve gastrectomy). Upon receiving the specimen in pathology, nodular vaguely defined lesions were identified in the gastric wall. Representative sections were submitted for microscopic examination, which revealed the incidental finding of a multifocal granular cell tumor with a background of chronic gastritis. The patient has not had any regional recurrences or metastasis in the past 2 years following the procedure.

Granular cell tumor of the brachial nerve in a dog: A case report.

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.

Oral Granular Cell Tumor: A Case Report with Emphasis on Pseudoepitheliomatous Hyperplasia in Oral Lesions.

A granular cell tumor (GCT) is an unusual benign mesenchymal neoplasm with Schwann cells origin. The most common site is the dorsum of the tongue. It has a striking tendency to occur in females and is more frequent in adult patients. GCT typically shows an asymptomatic, slow-growing, single nodule. Histopathologically, it reveals a proliferation of polygonal cells with granular cytoplasm penetrating the adjacent muscles. In some cases, the overlying epithelium demonstrates pseudoepitheliomatous hyperplasia (PEH), which can complicate its precise diagnosis and may mimic squamous cell carcinoma (SCC). This paper presents a 58-year-old woman with a chief complaint of painless mass on the dorsal of the tongue for two years. The lesion was pink and circumscribed with firm consistency measuring 1×1cm. The surface of the lesion was intact. Microscopic examination demonstrated unencapsulated sheets of large, polygonal cells with abundant eosinophilic, granular cytoplasm, and vesicular nuclei. The overlying epithelium showed florid PEH and keratin pearl formation. S100 protein was positive diffusely. The diagnosis of oral GCT was made. Though GCT is a non-aggressive lesion, it may be confused with SCC due to florid PEH and keratin pearl formation. Although PEH is a neglected topic among oral pathologists, it is of great importance in the field of research. Diagnosis can sometimes be problematic because they mimic other lesions. The pathogenesis of PEH is still uncertain. Therefore, familiarity with these characteristics and determining the cause of the PEH leads to correct treatment. This article intends to raise the insight of oral pathologists about PEH in oral lesions.

Case Report of a Benign Granular Cell Tumor Resembling Breast Carcinoma in a Young Woman: A Diagnostic Challenge.

Introduction: Granular cell tumors (GCTs) are uncommon, low-grade Schwann cell tumors found in the skin, soft tissue, and mucosal surfaces of the oral, gastrointestinal, and respiratory tracts. One in 1,000 breast cancer cases is GCT. Just 1-2% of GCTs are malignant GCTs. Case Presentation: This case report presents the clinical details and outcomes of a 34-year-old woman with a main concern of a palpable mass and pain in her right breast. In the clinical examination, we found a 1.5 × 2-cm palpable mass in her right breast with no axillary lymph node detection. The primary diagnosis was a benign GCT mimicking carcinoma of the breast. Upon evaluation, the mass was confirmed to be a benign GCT through pathology. The patient underwent breast-conserving surgery and sentinel lymph node dissection at the Cancer Research Center of Shahid Beheshti University of Medical Sciences on November 30, 2022. The surgical margins were found to be free of tumors, and there was no involvement of skin or axillary lymph nodes. The patient had a positive postoperative outcome, with no complications observed. Conclusion: The case highlights the importance of accurate diagnosis and appropriate surgical planning to avoid invasive procedures and unnecessary radical surgeries in cases of benign GCT mimicking carcinoma of the breast.

Breast granular cell tumor: A case report and review of literature.

Granular cell tumor (GCT) is a rare neoplasm. Its diagnosis is based on imaging and pathological findings. There are only a few reported cases of GCT of the breast (GCTB) in the literature. We present a case of a female patient diagnosed with GCTB and perform a review on the prevalence, diagnosis, histology, treatment, and prognosis.

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.

Metastatic Granular Cell Tumor: A Rare Entity.

Granular cell tumor, which is thought to recapitulate a Schwann cell phenotype, is a very rare neoplasm that belongs to soft tissue tumors. It can be classified as benign, atypical or malignant, based on specific histological criteria, with the majority of cases exhibiting an indolent behavior. Its biology and clinical course are poorly understood and its optimal management is yet to be defined, given the rarity of cases. Here we describe an atypical granular cell tumor in the upper middle back skin that evolved after a thirty-year indolent period. Despite complete surgical removal, the patient experienced a recurrence, both local and in the lungs, following an aggressive clinical course. Data on management of metastatic disease are extremely scarce, comprised exclusively of case reports. Therefore, we administered to the patient systemic therapy according to soft tissue sarcoma guidelines, which led to disease progression, with fatal outcome. In conclusion, recurrent and/or metastatic granular cell tumor is a rare disease that can be life-threatening, for which response to different therapies is unknown. The biologic behavior of atypical and malignant granular cell tumor is quite different from its benign counterpart, evoking soft tissue sarcomas, and its diagnosis should alert clinicians. The role of adjuvant chemotherapy and radiation therapy in this setting should be explored, to limit disease recurrence.

A Case of a Granular Cell Tumor Arising in a Patient with Long-segment Barrett's Esophagus.

Esophageal cell tumors are rare. Esophagogastroduodenoscopy performed on a 48-year-old woman revealed an elevated esophageal lesion and the presence of long-segment Barrett's esophagus. Endoscopic ultrasonography showed a 15 mm homogeneous hypoechoic tumor extending from the lamina propria mucosa to the submucosa. Pathological examination of the biopsy tissue revealed a sheet-like cluster of histiocytoid cells with an abundant eosinophilic granular cytoplasm. Immunohistochemical examination revealed S-100 (+) and CD68 (+), thus suggesting the diagnosis of a granular cell tumor. The tumor was resected by endoscopic submucosal dissection. Pathologically, the background mucosa was Barrett's mucosa. This is the first reported case of an esophageal granular cell tumor in long-segment Barrett's esophagus.

Granular Cell Tumor Mimicking Breast Carcinoma: A Report of Two Cases.

Granular cell tumor (GCT) of the breast is a rare neoplasm that can mimic the clinical and radiological features of breast carcinoma. This paper presents two case reports - a rare male case and a more common female case - to underline the diagnostic challenges posed by GCT in the breast. The male patient was initially suspected of having a breast tumor based on mammography and ultrasound findings. The female patient also exhibited radiological signs suggestive of breast cancer. In both cases, the mammograms showed irregular lesions, while ultrasounds revealed solid masses with posterior shadowing and echogenic halos, mimicking carcinoma. Dynamic contrast-enhanced magnetic resonance imaging (MRI) suggested benign patterns in both cases, but only histopathologic examination post-core needle biopsy confirmed the diagnosis of GCT. These cases highlight the variability of GCT imaging presentations and the potential for misdiagnosis as breast carcinoma. The tumors exhibited distinct histopathological features, such as large polygonal cells with granular eosinophilic cytoplasm and S100 protein, differentiating them from breast carcinoma. However, imaging alone proved insufficient for diagnosis, emphasizing the need for histopathologic confirmation. The report discusses the importance of including GCT in differential diagnoses and utilizing core needle biopsy for accurate evaluation. Both cases had no recurrence during follow-up after wide resection, indicating a favorable prognosis for GCT when properly managed.

Gastric granular cell tumor: A case report and literature review.

Granular cell tumors (GCTs) are neoplasms of uncertain histopathological etiology and therefore there are no universally accepted treatment strategies. GCTs are characterized by abundant eosinophilic granules. Since they are predominantly located in the skin and subcutaneous tissues, gastric GCTs are exceedingly rare. The present study documents the case of a 52-year-old man who visited the Gastroenterology Clinic of the People's Hospital of Putuo District (Zhoushan, China) due to upper abdominal fullness. Endoscopic ultrasonography revealed a well-defined hypoechoic nodule in the submucosal layer of the stomach body. The lesion was completely excised using endoscopic submucosal dissection and the patient made a full postoperative recovery. Immunohistochemistry showed positivity for S100 and CD68, with CD34 expression surrounding the tumor cells. At telephone follow-up until May 2024, the patient's fullness and discomfort were noted to be relieved. The characteristics of the CD34 expression pattern may serve as a novel basis for the pathological diagnosis of gastric GCTs. Endoscopic resection is a feasible option for gastric GCTs smaller than 2 cm.

Melan-A-Positive Granular Cell Tumor With Extensive Intraneural and Perineural Spread: A Rare Case Report.

Cutaneous granular cell tumors (GCTs) are rare tumors that typically exhibit benign clinical behavior and are likely of Schwann cell origin. Some histologic and immunohistochemical variants of GCTs may present challenges due to infiltrative growth patterns, perineural invasion, and expression of Melan-A. In this case report, we present a 27-year-old male who had previously been diagnosed with a typical GCT on the back a few years ago. The current biopsy from the proximal palm demonstrated a cytologically similar tumor with extensive perineural spread and notable positivity for Melan-A. Although uncommon, these features are consistent with the histological appearances of GCTs. The current views on the histogenesis of GCTs, clinical associations, differential diagnosis with melanoma, and histological criteria for malignant GCTs are discussed. A panel of immunohistochemical stains, including Inhibin-α and preferentially expressed antigen in melanoma (PRAME), is proposed for use in rare instances of Melan-A-positive GCTs.

Beyond Rarity: Insights Into the Diagnosis and Histopathology of Breast Granular Cell Tumors.

Breast granular cell tumors, which are benign and rare tumors of the breast, pose a diagnostic challenge due to their rarity and nuanced clinical presentations. This article explores a unique case of a 41-year-old female with a biopsy-confirmed granular cell tumor, shedding light on the intricacies involved in diagnosis. Rooted in a neuroectodermal origin, particularly Schwann cells, these tumors demand a multidimensional diagnostic approach for accurate identification. Despite their predominantly benign nature, malignant variants exist, necessitating a thorough histomorphology examination, supported by immunohistochemistry, for precise classification. This article contributes to our understanding of breast pathology and emphasizes the pivotal role of histopathology in unraveling complexities associated with granular cell tumors, reaffirming the importance of a comprehensive diagnostic approach.

Collision Tumors of the Colon and Peritoneum: Signet-Ring Cell Carcinoma and Granular Cell Tumor.

Collision tumors, although rare, characterized by two distinctive (morphological, as well immunohistochemical) and spatially independent tumor components at the same location, are always puzzling for clinicians, pathologists, and patients because they do not fit into the usual approaches, being neither diagnostic nor therapeutic. Reviewing the specialized literature, to date, collision tumors have been reported in multiple locations such as the skin, esophagus, stomach, intestine, liver, kidney, bladder, adrenal gland, or thyroid. We report a case of coexistence at the same site of a malignant tumor of the ascending colon and a benign tumor emerging from the peritoneal lining, initially thought by the surgeon to be right-sided serosal carcinomatosis. But histopathological examination reveals that those multiple serosal nodules were benign granular cell tumors that have collided with highly aggressive transparietal signet-ring colon carcinoma. These results put the patient's prognosis and therapeutic strategy in a different light than the clinical and intraoperative evaluation.