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.

Imaging findings of granular cell tumours of the head and neck.

AIM: To review the imaging characteristics of granular cell tumours in the head and neck and assess their associations with pathological findings. MATERIALS AND METHODS: Eleven patients (10 [91%] women, mean age 43 years) with histopathologically confirmed granular cell tumours were included in this study. Preoperative imaging studies were performed, including computed tomography (CT), magnetic resonance imaging (MRI), or ultrasound. The location of the tumours, their imaging features, and histopathological findings were analysed. RESULTS: Among the 11 granular cell tumours, four (36%), three (27%), and two (18%) tumours were found in the submucosal layer, subcutaneous layer, and intramuscular area, respectively. On CT, all tumours exhibited homogeneous iso-attenuating enhancement compared with adjacent muscle, and nine out of the 11 tumours (81%) demonstrated well-defined margins. On T2-weighted imaging (T2WI), four out of five tumours (80%) demonstrated iso-signal intensity compared with adjacent muscles, and four tumours (80%) exhibited homogeneous signal intensity. The apparent diffusion coefficient (ADC) values ranged from 0.68-0.81 × 10-3 mm2/s. Histopathological examination revealed densely packed tumour cells with variable amounts of fibrous stroma. CONCLUSION: Granular cell tumours were characterised by well-defined and iso-signals on T2WI and low mean ADC values, and were predominantly located in the submucosal, subcutaneous, or intramuscular areas in middle-aged women. The characteristic locations, demographic characteristics, and imaging findings can help to differentiate granular cell tumours from other soft-tissue tumours in the head and neck.

Congenital Epulis: A Rare Head And Neck Tumor in the Newborn.

Congenital epulis is a rare tumor; when voluminous and ulcerated, appearance can generate concern. Nevertheless, it is a benign lesion that is easily treated with surgery. This is a retrospective study, with the presentation of 2 cases treated by the same team from 2016 to 2020 and a bibliographic review (PubMed and Elsiever). This surgical team is based in Buenos Aires, working in the private medical system. Congenital epulis is an infrequent, benign tumor affecting females predominantly, appearing at birth on the alveolar median ridge of the maxilla. Its pathogenesis is uncertain. Voluminous tumors interfere with feeding, but respiratory interference is exceptional. Histologically, this tumor is similar to the granular cell tumor but has different clinical characteristics and Periodic Acid Schiff stain. Prenatal diagnosis with ecographic or magnetic resonance imaging is ideal; if undiagnosed prenatally, clinical diagnosis is done based on typical features. Surgical excision is the treatment of choice.

Granular cell tumour of the breast: A case report.

INTRODUCTION: Breast granular cell tumour (GCT) is a rare but usually benign lesion. PRESENTATION OF CASE: We report a case of a woman with breast GCT. CONCLUSION: Clinically and radiologically, GCT may mimic breast carcinoma. A conclusive diagnosis is made after a histopathological examination of the lesion. The treatment of choice is surgery.

Clinical outcomes of esophageal granular cell tumors with different endoscopic resection methods.

Esophageal granular cell tumors (GCTs), the second most common subepithelial tumors (SETs) of the esophagus, are potentially malignant with no definite management guidelines available. We retrospectively enrolled 35 patients with endoscopically resected esophageal GCTs between December 2008 and October 2021 and evaluated the clinical outcomes from the various methods performed. Several modified endoscopic mucosal resections (EMRs) were performed for treating esophageal GCTs. Clinical and endoscopic outcomes were evaluated. Mean age of patients was 55.8 ± 8.2, with majority being men (57.1%). Mean tumor size was 7.2 ± 2.6 mm, most (80.0%) were asymptomatic and present in the distal third of the esophagus (77.1%). Endoscopic characteristics predominantly included broad-based (85.7%) and whitish-to-yellowish color changes (97.1%). Endoscopic ultrasound (EUS) of 82.9% of the tumors revealed homogeneous hypoechoic SETs originating from the submucosa. The five endoscopic treatment methods used were: ligation-assisted (77.1%), conventional (8.7%), cap-assisted (5.7%), and underwater (5.7%) EMRs and ESD (2.9%). Mean procedure time was 6.6 ± 2.1 min, and no procedure-associated complications were noted. The en-bloc and complete histologic resection rates were 100% and 94.3%, respectively. No recurrences were noted during follow-up, and no significant differences in the clinical outcomes of the different methods of endoscopic resection were found. Based on tumor characteristics and therapeutic outcomes, modified EMR methods can be effective and safe. However, there were no significant differences in the clinical outcomes of the different methods of endoscopic 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.

Granular cell tumour of the pancreas: a case report and systematic review.

PURPOSE: Granular cell tumours (GCTs) of the pancreas are mostly benign and exceptionally rare, with no unique identifying radiological features. Following a case discussion of a patient with GCT, a comprehensive review of available literature was conducted to identify the common diagnostic features associated with GCT. METHODS: Following a case report identified in our institution, a systematic review was conducted by two authors in accordance with Preferred Reporting Items for Systematic review and Meta-Analysis protocols (PRISMA) guidelines. Databases MEDLINE, EMBASE, Scopus, World of Science, and grey literature were searched on August 2021. Inclusion criteria were histopathology diagnosed granular cell tumour of the pancreas. RESULTS: A 37-year-old male presented with 1 month of abdominal pain and an MRI demonstrating a dilated main pancreatic duct, distal parenchymal atrophy, but no focal lesion. Repeat MRI at 6 months re-demonstrated similar findings and subsequent endoscopic ultrasound was suspicious for main duct IPMN. Following multidisciplinary team discussion, a spleen-preserving distal pancreatectomy was performed. Histopathology demonstrated granular cell tumour with cells diffusely positive for S100 and no malignant transformation. 11 case reports were identified in the literature with diagnosis confirmed on tissue histopathology based on positive immunohistochemical staining for S-100 protein. Eight patients presented with gastrointestinal symptoms with abdominal pain the main presenting complaint (50%). 10 patients underwent CT with portal venous contrast and all underwent endoscopic examination. Imaging findings were similar in five studies for EUS which demonstrated a hypoechoic lesion with homogenous appearance. On non-contrast CT GCT was iso-enhancing, and with portal venous contrast demonstrated hypo-enhancement that gradually enhanced on late phases. Pre-operative diagnosis of pancreatic carcinoma was described in six cases based on imaging and biopsy, resulting in progression to surgical resection. Nine patients were managed surgically and no complications identified on follow-up (6-52 months). CONCLUSION: The currently proposed management pathway includes EUS with biopsy and CT, and surgical resection recommended due to malignancy risk. Improved sample collection with EUS-FNA and microscopic assessment utilising S-100 immunohistochemistry may improve pre-operative diagnosis. Limitations include rare numbers in reported literature and short follow-up not allowing an assessment of GCT's natural history and malignancy risk. Additional cases would expand the current dataset of GCTs of the pancreas, so that surgical resection may be avoided in the future.

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.

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.

Rare case of granular cell tumour at 16 years - A case report.

Rationale: Granular cell tumours (GCTs) of the tongue are a rare, soft tissue pathological entity at young ages. This case report aims to present one such case. Patient Concerns: A 16-year-old female patient sought treatment for a small, slow-growing, painless nodule in the dorsum of the tongue since six months. She underwent successful orthodontic treatment in recent past and is in the retention phase. Diagnosis: Excisional biopsy revealed the lesion to be a GCT under histopathology. Treatment: The patient was treated for an abnormal, small, slow-growing, painless nodule in the dorsum of the tongue. Outcomes: The patient had satisfactory esthetics and early diagnosis. The active intervention dispelled the confusion about the role of trauma and orthodontics appliance in the etiology of GCT in this particular case to the patient. Take-away Lessons: GCT can occur at any age. Early diagnosis and corrective surgery would help to avoid late complications.

Granular cell tumor of the appendix: a case report and literature review.

A granular cell tumor (GCT) is an unusual benign soft tissue tumor that can occur at any age and in any part of the body. GCTs are mostly found in the skin and subcutaneous tissues, bronchi, esophagus, breast tissue, and tongue. A GCT originating in the digestive tract, particularly in the appendix, is relatively rare and usually diagnosed as an incidental finding. We herein describe the first case of abdominal distension and occasional pain secondary to a GCT of the appendix in our hospital. The findings from this case suggest that a GCT of the appendix is a rare entity for which surgical resection is an efficient therapy.

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.

Role of endoscopic ultrasound and endoscopic resection in the diagnosis and treatment of esophageal granular cell tumors.

BACKGROUND AND AIM: Diagnosis and complete resection of esophageal granular cell tumors (GCTs) is an area of concern. However, articles on endoscopic ultrasound (EUS) and endoscopic resection of esophageal granular cell tumors are few. To evaluate the role of endoscopic ultrasound and endoscopic resection in the diagnosis and treatment of esophageal granular cell tumors. METHODS: A retrospective analysis of 15 patients with esophageal granular cell tumors who underwent endoscopic ultrasound examination and endoscopic resection in our hospital was conducted. The clinical data, endoscopic ultrasound images, endoscopic treatment, pathological characteristics, postoperative complications and follow-up status of all patients were evaluated. Ten board-certified endoscopists independently evaluated the white light endoscopic images of the 15 patients (Test 1) and the endoscopic ultrasound images together with white light endoscopic images of the same patient set (Test 2). RESULT: Female patients accounted for 53.4% of the participants. The average age at the time of diagnosis was 49.13 ± 9.31 years old. Ten lesions (66.67%) showed hypoechoic signal, four lesions (26.67%) showed hyperechoic signal and one lesion showed medium signal. The diagnostic accuracy was significantly higher with Test 2(65.3% vs. 92.0%, p < .001). Complete endoscopic resection was performed in all the patients. No complications occurred in any of the patients. No esophageal stenosis, recurrence, or metastases was found in all patients during the follow-up period. CONCLUSION: The endoscopic ultrasound images of esophageal granular cell tumors have certain characteristics that help diagnose esophageal granular cell tumors. Endoscopic resection of esophageal granular cell tumors is an effective, safe and feasible treatment method.

Malignant granular cell tumour at the interventricular septum.

Granular cell tumours are usually benign with a 1-2% incidence of malignancy. They are less sensitive to radiotherapy and chemotherapy and are treated by surgical excision. We report a case of a malignant granular cell tumour located at the interventricular septum.

Isolated Hypoglossal Nerve Palsy as an Early Symptom of a Granular Cell Tumor.

BACKGROUND: Hypoglossal nerve palsy (HNP) is rather common as a neurological disease. However, as an isolated nerve palsy it is an exceedingly rare phenomenon and points at local pathologies along the peripheral course of the nerve. In this communication we report a granular cell tumor (GCT) arising in the submandibular segment of the hypoglossal nerve. CASE-REPORT: Spontaneous isolated HNP was recognized in a female patient. First line MR-imaging identified a clivus-chordoma. However, involvement of the hypoglossal nerve was highly unlikely according to MR-findings. Finally, ultrasonographic investigation revealed a small submandibular mass which, at histological examination, turned out to be a granular cell tumor arising within the hypoglossal nerve. CONCLUSIONS: This is the report of an extremely rare GCT originating within the 12th cranial nerve. The case illustrates that isolated motoric cranial nerve palsy may result from this rare tumor entity. This report also points out the diagnostic value of a simple ultrasonographic investigation to depict pathologic lesions of the submandibular space.

CT and MRI features of extraocular muscle granular cell tumour: preliminary experience in eight cases.

AIM: To characterise the computed tomography (CT) and magnetic resonance imaging (MRI) features, in particular the functional MRI characteristics, of extraocular muscle granular cell tumours (GCTs). MATERIALS AND METHODS: The CT (n=6) and MRI (n=8) features of eight extraocular muscle GCTs cofirmed at histopathology were analysed retrospectively. The imaging findings were evaluated with emphasis on the location, size, margin, shape, extent, bony change, internal architecture, enhancement pattern, and extent of lesions. Based on diffusion-weighted imaging (DWI) and dynamic contrast-enhanced (DCE) MRI, the apparent diffusion coefficient (ADC) value of six lesions and time-intensity curve (TIC) of one lesion were reviewed. RESULTS: Immunohistochemistry revealed strong positivity for S-100 protein and a low Ki-67 index (2-5%) in all cases. Most of the lesions (7/8) were confined to the muscle belly with an ovoid shape. All of the tumours were isodense to cerebral grey matter and showed homogeneously mild enhancement on CT images. All lesions were hypointense to cerebral grey matter on T2-weighted imaging (T2WI) and showed homogeneously marked enhancement on contrast-enhanced T1-weighted imaging (T1WI). All lesions showed a hypo- or isointense signal on DWI images with a high b-value. The mean ADC of six lesions was (0.72 ± 0.14) × 10-3 mm2/s. The TIC of the case examined using DCE-MRI showed a plateau pattern (type II). CONCLUSION: A well-defined oval mass confined to the muscle belly with a hypointense signal on T2WI, homogeneously marked enhancement on contrast-enhanced T1WI, hypo- or isointense signal on DWI, and low ADC value is highly suggestive of a GCT.

A case of esophageal granular cell tumor diagnosed by mucosal incision-assisted biopsy.

For an esophageal submucosal mass suspicious of granular cell tumor (GCT) based on gross appearance and endoscopic ultrasound findings, a sufficient number of biopsy specimens is required for a definite diagnosis using immunohistochemical examination. When the specimen obtained by forceps biopsy is insufficient, endoscopic ultrasound-fine needle aspiration (EUS-FNA) is believed to be an useful alternative. However, it may be difficult to obtain an adequate amount of tumor material using EUS-FNA. Mucosal incision-assisted biopsy (MIAB) is a simple method that can collect larger amounts of specimens. This procedure is helpful for physicians who encounter the problem of obtaining an adequate amount of biopsy material from esophageal tumors suspicious for GCT. We present a case of esophageal GCT that was successfully diagnosed through MIAB.