[A case report of bronchial granular cell tumor].

Granular cell tumor (GCT) is a relatively rare tumor that develops in soft tissues at various sites in the body, and GCT originating in the bronchus is rather rare. Here, we reported a case of primary GCT of the bronchial to improve the understanding of this disease.

Granular cell tumor of soft tissues: MR findings.

Granular cell tumors (GrCT) are histologically unique neoplasms that often manifest as painless solitary nodules located in the dermis or submucosal tissue. In this article we analyze the radiologic features of this type of tumor in five patients by magnetic resonance imaging (MRI), analyzing morphologic and signal characteristics. MR imaging findings were similar to other publications and we demonstrate low ADC values in our series.

Clinicopathological and immunohistochemical analysis of maxillofacial granular cell tumor.

OBJECTIVES: To analyze the clinicopathological features of maxillofacial granular cell tumors (GCT) with the aid of immunohistochemical staining. METHODS: Seven cases of maxillofacial GCT were retrospectively collated, and the microscopic morphology of maxillofacial GCT was analyzed. The expression of S-100, neuron-specific enolase (NSE), SOX-10, CD68, actin, desmin, and Ki-67 in GCT was detected by immunohistochemical staining. The cases were observed in the follow-ups after clinical treatment. RESULTS: All seven GCT tumors lacked envelopes and were poorly defined. Microscopically, the sizes of the tumor cells were large and appeared with inconspicuous cell membranes, forming a syncytium-like appearance. The cytoplasm was filled with characteristic eosinophilic granules. The immunohistochemical results showed that six cases were NSE-positive, five cases were S-100-positive, seven cases were CD68-positive, five cases were SOX-10-positive, one case was actin-positive, and seven cases were desmin-negative. The Ki-67 index did not exceed 5% in all cases. In the follow-up sessions, none of the six cases presented a recurrence. CONCLUSIONS: Maxillofacial GCT has a characteristic histological structure. Immunohistochemical S-100, CD68, and other indicators can assist in diagnosis, and the prognosis is good after clinical resection.

Granular cell tumor of the neurohypophysis presenting as a third ventricle mass.

Granular cell tumors of the neurohypophysis (GCT) are rare benign neoplasms belonging, along with pituicytoma and spindle cell oncocytoma, to the family of TTF1-positive low-grade neoplasms of the posterior pituitary gland. GCT usually present as a solid sellar mass, slowly growing and causing compressive symptoms over time, occasionally with suprasellar extension. They comprise polygonal monomorphous cells with abundant granular cytoplasm, which is ultrastructurally filled with lysosomes. Here we report the case of a GCT presenting as a third ventricle mass, radiologically mimicking chordoid glioma, with aberrant expression of GFAP and Annexin-A, which lends itself as an example of an integrated diagnostic approach to sellar/suprasellar and third ventricle masses.

Esophageal Granular Cell Tumor in Children: A Clinicopathologic Study of 11 Cases and Review of the Literature.

OBJECTIVES: Granular cell tumor (GCT) commonly presents in the subcutaneous tissue and head and neck region, and it is uncommon in the gastrointestinal tract. Experience with esophageal GCTs in the pediatric population is limited, with only 7 cases reported in the literature, 3 with eosinophilic esophagitis (EoE). METHODS: Case information from 11 pediatric patients with GCTs of the esophagus was retrieved. H&E and immunohistochemical slides were reviewed with clinical, endoscopic, and follow-up data from all patients. RESULTS: In total, 7 male and 4 female patients were included, with ages ranging from 3 to 14 years. Indications for esophagogastroduodenoscopy (EGD) included EoE (n = 3), follow-up for Crohn disease, and other nonspecific complaints. Endoscopically, all patients had a single submucosal, firm mass protruding into the lumen, with normal overlying mucosa. The nodules were removed endoscopically in multiple fragments in all cases. Histologically, the tumors showed sheets and trabeculae of cells containing bland nuclei, inconspicuous nucleoli, and abundant pink granular cytoplasm without atypical features. All tumors were immunoreactive for S100, CD68, and SOX10. Follow-up showed that all patients were disease-free (median, 2 years). CONCLUSIONS: We report the largest series of pediatric esophageal GCTs with coincidental association with EoE. These EGD findings are characteristic, and removal by biopsy is both diagnostic and therapeutic.

Benign mesenchymal tumours of the tongue: A report of adult-type rhabdomyoma and granular cell tumour with a review of the literature.

The cytomorphological features of benign mesenchymal tumours of the tongue have rarely been reported. Herein, we present the cytomorphological features of adult-type rhabdomyoma, which occurred in the tongue of a female patient, and granular cell tumour (GCT), which occurred in the tongue of a male patient; both patients were in their mid-50s. The cytological features of the adult-type rhabdomyoma case included large polygonal to ovoid cells with abundant and granular cytoplasm with predominantly peripherally located, uniform, round to oval nuclei and small nucleoli. Cross-striation and crystalline intracytoplasmic structures were not seen. The cytological features of the GCT case included large cells with abundant granular pale cytoplasm, small round nuclei and small distinct nucleoli. The cytological differential diagnoses of these tumours overlap; thus, the cytological findings of the different entities included in their differential diagnoses are discussed.

Granular cell tumors of the urethra.

Granular cell tumors (GCTs) are a rare type of mesenchymal tumors that are histologically derived by Schwann cells and rise within soft tissues such as skin and mucosal surfaces. Differentiation between benign and malignant GCTs is often difficult and relies on their biological behavior and metastatic potential. While there are no standard guidelines for management, upfront surgical resection, whenever feasible, is key as a definitive measure. Systemic therapy is often limited by poor chemosensitivity of these tumors; however, accumulating knowledge of their underlying genomic landscape has opened some opportunities for targeted approaches, for example, the vascular endothelial growth factor tyrosine kinase inhibitor pazopanib, which is already in clinical use for the treatment of many types of advanced soft tissue sarcomas.

Granular cell tumor of the neck treated using a local flap: A case report.

RATIONALE: Granular cell tumors are rare soft tissue neoplasms derived from nerve that can arise in the oral cavity, skin, or gastrointestinal tract. Various hypotheses have proposed that granular cell tumors originate from the nervous system, skeletal muscle, and Schwann cells. PATIENT CONCERNS: A 47-year-old male patient presented with a 5 cm cervical mass. DIAGNOSES: Computed tomography showed a 4 cm-sized homogeneous enhancing mass infiltrating the sternocleidomastoid muscle and even the surrounding skin. INTERVENTIONS: Extensive surgical resection of the tumor including the skin was performed. A submental transposition local flap was used for the wide skin defect. OUTCOMES: Histologic finding showed polygonal granular cells with rich eosinophilic coarse granular cytoplasm without interstitial tissue without mitosis or necrosis. Immunohistochemically, the tumor cells were positive for S100 and CD68, which is consistent with classic granular cell tumors. LESSONS: In microscopic observations, granular cell tumors do not have a defined boundary with surrounding tissues, and they display an infiltrating pattern that can expand to adjacent tissue. As a result, the tumor should be removed with a sufficient margin, including the normal tissues surrounding it. The authors experienced granular cell tumor in the muscle layer of the head and neck. It could be treated without recurrence through extensive surgical resection and local flap.

Rectal Granular Cell Carcinoma Requiring Abdominoperineal Resection.

BACKGROUND: Granular cell tumors, derived from neural crest cells, are rare tumors infrequently located in the colon or rectum. We will discuss a patient with a rectal granular cell tumor invading the anal sphincters requiring an abdominoperineal resection. METHODS: A 56-year-old male, with anal pain, was found to have a perirectal mass. Pathology from ultrasound-guided transrectal biopsy demonstrated low grade granular cell tumor. The patient underwent a laparoscopic abdominoperineal resection with perineum reconstruction. RESULTS: Pathology demonstrated a granular cell tumor of 4.5 centimeters with tumor invasion of the anal sphincters. Surgical margins were free of neoplasm. DISCUSSION: This is the only documented case of a colorectal granular cell tumor that has required an abdominoperineal resection. On histology, it was considered low grade but its behavior was more consistent with a malignant process. Additional research on malignant granular cell tumors is necessary to help improve treatment options, prevent recurrence, and improve overall survival. His medical course will be followed for disease progression or metastasis.

Granular cell tumour of the cavernous sinus: A rare cause of secondary trigeminal neuralgia.

BACKGROUND: Cavernous sinus tumours comprise 0.1-0.2% of all intracranial tumours, and are most commonly meningiomas or schwannomas. Central nervous system and cranial nerve granular cell tumours (GCTs) are extremely rare. We report the tenth case of a GCT arising from a cranial nerve, and the second case reported in a cavernous sinus location, and review the literature. CLINICAL PRESENTATION: A 67-year-old man presented with right sided trigeminal neuralgia. Imaging findings suggested a trigeminal schwannoma and he was treated with CyberKnife radiosurgery. Over a period of 41 months follow up, there was a progression in both symptoms and imaging findings, requiring debulking surgery. Histopathology identified a GCT. CONCLUSIONS: This is the first case of a cranial nerve GCT treated with stereotactic radiosurgery. Trigeminal nerve GCTs are a rare differential in cases of presumed schwannomas.

Dermatofibroma-like dermal non-neural granular cell tumor.

Non-neural granular cell tumor (NNGCT) is a rare tumor with uncertain lineage. It presents as an asymptomatic polypoid or plaque-like lesion, especially on trunk. Because the granular cells are usually strongly reactive with S-100 stain, conventional granular cell tumors (GCTs) are regarded as those of neural or Schwann cell origin. Unlike GCTs, NNGCT is not reactive for S-100 protein and is thought to derive elsewhere, presumably from mesenchymal stem cells. A 20-year-old woman presented with a solitary, dermatofibroma-like, brownish nodule on her right arm. The lesion developed 3 months before presentation without subjective symptoms. Histopathologic examination revealed a grenz zone overlying a poorly circumscribed tumor extending through the reticular dermis. The tumor cells were large and polygonal, and they had numerous eosinophilic small granules in the cytoplasm. Immunohistochemical stains were positive for CD68, vimentin, factor XIIIa, CD10, and cyclin D1. Stains for S-100 protein, neuron-specific enolase, and CD34 were negative. Based on these findings, the lesion was diagnosed as dermal NNGCT.

Combination conventional endoscopy and endoscopic ultrasound can differentiate between esophageal granular cell tumors and leiomyomas.

Esophageal leiomyomas and granular cell tumors (GCTs) are the 2 most common subepithelial tumors found in the esophagus. We attempted to differentiate the 2 tumors using endoscopic findings and endoscopic ultrasound (EUS) features. Between December 2008 and June 2021, a total of 38 esophageal GCTs and 11 esophageal leiomyomas originating from the muscularis mucosa were selected. Clinical characteristics and endoscopic features were retrospectively reviewed. Although esophageal GCTs are mainly located in the lower third of the esophagus (81.6%), esophageal leiomyomas are mainly located in the upper third of the esophagus (45.5%). Broad-based (84.2%, P = .002) and whitish-to-yellowish color changes (97.4%, P < .001) are significant endoscopic features of esophageal GCTs. The echogenicity of esophageal leiomyoma was similar to that of proper muscle echogenicity. However, the echogenicity of esophageal GCTs was hyperechoic compared to that of the proper muscle layer (90.0% vs 9.1%, respectively, P < .001). EUS revealed a clearer hyperechoic epithelial lining in the esophageal leiomyoma than in esophageal GCTs (100% vs 26.7%, respectively, P < .001). The 5 endoscopic factors (location of the lower third, broad base, whitish-to-yellowish color, hyper-echogenic, and unclear demarcated hyperechoic epithelial line) were counted to differentiate esophageal GCTs from esophageal leiomyomas. Tumors with 3 or more endoscopic factors were all esophageal GCTs. The characteristic endoscopic and EUS features of esophageal GCTs were broad-based, whitish-to-yellowish colored subepithelial tumors located in the lower third of the esophagus and hyperechoic tumor with an unclear demarcated hyperechoic epithelial line. A combination of these features can predict esophageal GCTs before endoscopic resection.

Congenital granular cell epulis: 24 new cases with more differences than similarities to granular cell tumor.

Congenital granular cell epulis (CGCE) is a rare tumor of gingiva that is exclusive to newborns, has marked female predominance, and is rarely associated with other abnormalities. Although benign in behavior, CGCE can be lethal by obstruction of respiration and/or deglutition and can require a multidisciplinary team of specialist at birth for survival of an otherwise normal infant. Histologically, CGCE resembles granular cell tumor (GCT), but unlike GCT, which is Schwannian-derived, derivation of CGCE remains an enigma, largely because of its low prevalence. This study presents 24 new cases of CGCE, the largest series since the original description 150 years ago and permits detailed study of homogeneity of cases diagnosed as CGCE as well as detailed comparisons of CGCE with GCT by clinical, morphological, immunohistochemical, and ultrastructural studies. The data show homogeneity within the CGCE cases, more differences than similarities between CGCE and GCT, and no immunohistochemical staining for common placental proteins/hormones in CGCE. The findings support a primitive mesenchymal cell origin, and a progressive degenerative process in CGCE, rather than neoplasia. Prenatal detection of this lesion is important to facilitate adequate preparations for support of these infants during labor and delivery.

Immunohistochemical study of autophagy associated molecules and cell adhesion molecules in canine intracranial granular cell tumors.

Granular cell tumors (GCTs) are characterized by abundant eosinophilic cytoplasmic granules. Based on the hypothesis that canine intracranial GCT is a subtype of meningioma and its cytoplasmic granules are formed through autophagy processes, histopathological and immunohistochemical examination were performed on biopsy samples from 7 cases of canine intracranial GCTs and 15 cases of conventional meningiomas. Histopathologically, 7/7 cases of GCTs involved the meninges; foci of meningothelial-like cells were observed in 3/7 cases; brain invasion was observed in 2/7 cases. Immunohistochemically, neoplastic cells of GCTs were positive for E-cadherin and negative for S100, cytokeratin, CD204, and ß-catenin in 7/7 cases. Neoplastic cells of 15/15 cases of meningiomas were positive for E-cadherin, and negative for S100 and CD204. Immunoreactivity of meningiomas for cytokeratin and ß-catenin was observed in 6/15 cases and 8/15 cases, respectively. Cytoplasmic granules of GCTs were positive for ubiquitin (5/7), p62 (5/7), and LC3 (7/7). Compared to GCTs, the ratios of ubiquitin (6/15) and p62 (3/15) positive cases were lower in meningiomas, and 15/15 cases were negative for LC3. These findings indicate that the biological natures of GCTs including anatomical location, histopathological features and immunoreactivity for E-cadherin are almost in conformity with those of meningiomas. The immunoreactivity for autophagy associated molecules may suggest the possible involvement of autophagy in cytoplasmic granule formation of canine intracranial GCTs.

Granular cell tumor of the pancreas with chronic dilation of the main pancreatic duct.

Although a granular cell tumor (GCT) can originate in various locations, GCT of the pancreas is a rare entity. We herein report a case of GCT of the pancreas with chronic dilation of the main pancreatic duct (MPD). The patient had been referred to our institution for epigastric pain 12 years ago at 50 years old. Multidetector-row computed tomography (MDCT) had shown dilation of the MPD up to 8 mm in diameter from the pancreatic body to the tail. However, she had refused additional workup and been lost to follow-up. She was referred to our institution at 62 years old, as dilation of the MPD had been noted again. Imaging studies showed that the MPD had become dilated up to 33 mm from the pancreatic body to the tail. MDCT showed a hypovascular nodule (6 mm in diameter) in the pancreatic neck, and the nodule was located at the origin of MPD dilation. Pancreatic cancer was considered as a possible differential diagnosis. Surgical treatment was planned for a therapeutic diagnosis, and distal pancreatectomy with splenectomy and lymphadenectomy was performed. The gross findings of the resected specimen revealed a pale-yellow solid tumor measuring 8 mm in diameter at the pancreatic neck. The histopathologic assessment revealed the tumor to be GCT of the pancreas. The characteristics of GCT of the pancreas, such as a tumor causing stenosis and dilation of the MPD frequently and being too small to detect on imaging studies, may lead to a misleading diagnosis of pancreatic cancer.

An update on cutaneous granular cell tumours for dermatologists and dermatopathologists.

Granular cell tumours are rare soft tissue neoplasms, which occur at a wide variety of sites and commonly involve the skin. Distinction between benign and malignant granular cell tumours is important because benign tumours can be fully cured by complete excision, whereas malignant tumours commonly recur and cause fatal metastatic disease. Communication between the dermatologist and pathologist is also important, as pathology may provide false reassurance by evaluating a benign-appearing part of a clinically malignant tumour. The following review summarizes the current literature on the epidemiology, clinical presentation, pathology, radiology, treatment and prognosis of cutaneous granular cell tumours, with a focus on improving diagnosis and management for dermatologists and dermatopathologists.