Pediatric Granular Cell Tumor Research Articles

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

Pediatric granular cell tumors (GCT) involving the gastrointestinal tract (GIT) are rare with limited case report/series reported to date. Multicenter retrospective study of pediatric GIT GCT. 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. 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.

42081 Pediatric granular cell tumors: A case series with emphasis on the dermal non-neural variant

42081 Pediatric granular cell tumors: A case series with emphasis on the dermal non-neural variant

Lip Granular Cell Tumor (GCT) in a Pediatric Patient: Critical Literature Review on Pediatric Intraoral GCT and Acquired and Congenital Intraoral Non-Neural GCT.

Granular cell tumor (GCT) is an uncommon benign neoplasm derived from Schwann cells, frequently affecting the oral cavity, skin, and gastrointestinal tract. On microscopy, pseudocarcinomatous squamous hyperplasia (PSH) and perineural involvement are potential diagnostic pitfalls. GCT should be differentiated from non-neural GCT (NN-GCT). A 13-year-old male patient was referred presenting a nodular lesion on the upper lip several months ago. After excisional biopsy, microscopy revealed GCT without PSH but presenting multifocal perineural involvement. By immunohistochemistry, ALK was negative, whereas Rb and INI1 expression was intact. Moreover, with few intraoral NN-GCTs being assessed, recent studies suggest that acquired dermal NN-GCT subgroup seems to correspond to ALK-rearranged variants of epithelioid fibrous histiocytoma. Accordingly, further research on this topic is strongly encouraged.

Pediatric granular cell tumor in the posterior wall of the larynx extending to the trachea.

Granular cell tumor (GCT) is a slow-growing benign neoplasm that can be found in any organ. Pediatric laryngotracheal GCT is rare. We experienced a 6-year-old boy suffering from a barking cough and symptoms of stridor and croup for one month. Head and neck computed tomography revealed a protruding mass that occluded 60% of the airway lumen. Under the impression of hemangioma or papilloma, excision revealed a submucosal non-encapsulated mass. Histologically, the mass was composed of sheets of large polyhedralshaped tumor cells containing plump eosinophilic granular cytoplasm and centrally placed, small, bland-appearing nuclei. The tumor cells were positive for S-100 protein, and voluminous eosinophilic cytoplasm was stained by diastase-resistant periodic acid-Schiff. The present report describes a unique case of a huge pediatric laryngeal GCT extending to the subglottic trachea. We also review the clinical course of pediatric laryngotracheal GCT and emphasize the importance of diagnosing GCT in children.

Pediatric Granular Cell Tumors: A Report of Four Cases and a Review of the Literature

Granular cell tumors (GCTs) are soft tissue lesions that can involve the female genital tract, including the vulva. Pediatric management decisions are often extrapolated from adult patients, as gynecologic GCTs are rare in patients under 18 years of age. This study aims to inform management of pediatric gynecologic GCT by presenting four new patients and a review of existing literature.

Eosinophilic Esophagitis and Esophageal Granular Cell Tumor: An Unexpected Association.

Esophageal granular cell tumors (GCTs) are rare. Eosinophilic esophagitis (EoE) is an immune-mediated disease characterized by esophageal eosinophilia despite proton pump inhibitor (PPI) therapy. Given that GCTs occur at sites of scarring and inflammation, we sought to determine the prevalence of EoE in patients with esophageal GCTs. Our center's pathology database was searched for GCT specimens from 1995 to 2014. Slides were blindly rereviewed. GCTs were scored for atypical cytological features. Presence and number of eosinophils in the tumor and the surrounding esophageal epithelium and any EoE features were recorded. Medical records were reviewed. From >30,000 esophageal cases, 23 esophageal GCTs were identified, with 18 available for review (16 adult, 2 pediatric). Median patient age was 38.7 years. Four adults had esophageal intraepithelial eosinophilia (peak 38 to 68 eosinophils/high power field [HPF]); 2 confirmed to have EoE, 1 with PPI-responsive esophageal eosinophilia, and 1 had not received PPI therapy. Both pediatric cases had confirmed EoE (peak 24 and 34 eosinophils/HPF). In total, 12/18 GCTs had intratumoral eosinophilia (peak 1 to 16 eosinophils/HPF). All 6 cases with esophageal eosinophilia had intratumoral eosinophilia. Two GCTs displayed atypical cytologic features. Esophageal eosinophilia was present in 25% of adult and 100% of pediatric GCTs, the majority confirmed to have EoE. Overall, 67% of cases had intratumoral eosinophilia and 2 had atypical features. On the basis of these findings, we propose evaluating surrounding tissue for eosinophilia when esophageal GCT is diagnosed, and adding GCT as a potential complication of untreated EoE. Research for an immunologic link between EoE and esophageal GCTs is needed.

Pediatric Granular Cell Tumor of the Breast: A Case Report and Review of the Literature.

Objective. Granular cell tumors arise from neurogenic mesenchymal stem cells and can occur anywhere throughout the body. They rarely present as breast masses and should be included in the differential diagnosis of pediatric breast neoplasms. We report a rare presentation of a pediatric breast granular cell tumor and a review of the literature. Participant. A 15-year-old female presented with an enlarging breast mass. She underwent ultrasound imaging and excisional biopsy, which revealed a granular cell tumor. Granular cell tumors of the breast are difficult to diagnose using ultrasound and mammography due to numerous similarities to other breast masses. Histopathologic staining best differentiates breast granular cell tumors from other breast masses with their positive staining for S100, CD68, and neurospecific enolase. Conclusion. Although rare, granular cell tumors of the breast should be considered as a possible diagnosis for pediatric breast masses to allow for proper management and follow-up for these patients. Although rare, these tumors do have malignant potential necessitating a correct and timely diagnosis.

A Case of Pediatric Subglottic Granular Cell Tumor

We report a rare pediatric case of granular cell tumor (GCT) arising from the subglottis. A 10-year-old boy seen for hoarseness was found in flexible fiberscopic examination to have a submucosal mass below the left vocal cord. The tumor was completely removed in direct laryngoscopy under general anesthesia. Histologically, the tumor showed the proliferation of large cells with abundant eosinophilic granular cytoplasm and oval nuclei and a surface entirely covered by stratified squamous epithelium. Immunohistochemically, S-100 protein was positive. Although a polypoid mass was seen 3 months after surgery, reexcision confirmed it to be postoperative granulation eliminating the possibility of recurrence. He remains free of recurrence 29 months after surgery. The number of pediatric laryngeal GCT reported appears to be increasing, possibly due to progress in indirect laryngoscopic devices.

Tumor de células granulares da laringe na infância: relato de caso

O tumor de células granulares (TCG) é uma neoplasia incomum, de evolução lenta, na maioria dos casos de caráter benigno e que pode acometer qualquer órgão do corpo. Entre as hipóteses que tentam explicar sua origem, a teoria da gênese neural apresenta embasamento sólido e é a mais aceita atualmente. O TCG é mais comum na raça negra, entre a 4ª e 5ª décadas de vida, acometendo com maior freqüência a região da cabeça e pescoço. A localização laríngea é rara, e quando ocorre é mais comum na porção posterior. É muito raro em crianças em geral acomete a porção anterior da subglote, podendo estender-se para a glote. O sintoma predominante é a rouquidão, podendo ocorrer disfagia, dor, tosse, hemoptise, e estridor. Macroscopicamente o TCG se manifesta como nódulo de pequeno tamanho, firme, séssil ou pediculado, não-ulcerado, de coloração clara, e usualmente bem circunscrito, porém sem cápsula. À microscopia, as granulações citoplasmáticas são características, apresentando positividade para a imunoperoxidase S100 e para a enolase neurônio-específica. O tratamento do TCG laríngeo consiste na exérese cirúrgica. Neste trabalho descrevemos um caso pediátrico de TCG laríngeo e sua evolução clínica após a remoção cirúrgica, alertando para o diagnóstico do TCG na população pediátrica. Foi realizada revisão de literatura abrangendo as características clínicas e histopatológicas do TCG, assim como as formas atuais de tratamento.

Pediatric granular cell tumor of the tracheobronchial tree

Granular cell tumors are lesions of neural crest origin with a predilection for the skin and soft tissue. Granular cell tumors of the tracheobronchial tree are exceedingly rare in the pediatric population, with only 5 reported cases. All 5 of these lesions presented within the cervical trachea. We report a large granular cell tumor in the posterior mediastinum, densely adherent to the intrathoracic tracheal carina.