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.

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.

Multiple oral and cerebral relapses of a Granular cell tumor (Abrikossoff Tumor) in a young girl affected by congenital dyserythropoietic anemia type II.

CASE REPORT: A 14-year-old girl presented with 1 cm large whitened lesion on the ventral surface of the tongue, appeared from 1 month. Past history showed congenital dyserythropoietic anemia type II. The lesion was excised and microscopic and immunohistochemical analyses were compatible with benign Abrikossoff tumor. Total body MRI was negative. After six months the patient presented a second tongue lesion and four months later another large painful lesion in the soft palate, with the same istological diagnosis. In addition, she had other multiple lesions: two apperead at pharyngeal level (not biopsied) that remain stable over time, and one at the pituitary gland. CONCLUSION: Granular cell tumors, with or without multiple lesions, are rare in children. About 50% of cases involve the head and neck region, with the tongue being the most affected site. Therapy is based on the surgical excision of the lesions; however some tumor forms, although their histological aspect of benignity, often have an important infiltrative power, making the therapeutic approach difficult, as in our case.

A 55-Year-Old Woman With Frequent Pulmonary Exacerbations and Endobronchial Lesions.

CASE PRESENTATION: A 55-year-old woman with COPD, heart failure with preserved ejection fraction (congestive heart failure), diabetes mellitus, and hypertension presented with baseline dyspnea at rest that had worsened over the last week. She reported associated runny nose, congestion, and cough productive of green sputum. She smoked six cigarettes per day and denied alcohol, drugs, or occupational exposure. She was admitted and initiated on treatment for acute exacerbation of COPD; however, her condition did not improve with steroid, ceftriaxone, and nebulized albuterol and budesonide treatments. She had been diagnosed with asthma and COPD without ever undergoing pulmonary function testing. She presented 11 times to the ED with six hospital admissions in the last 1.5 years for worsening dyspnea at rest, wheezing, and lower extremity edema deemed secondary to exacerbation of her COPD or congestive heart failure. She reported medication compliance, which included fluticasone-vilanterol, tiotropium bromide, and furosemide. She repeatedly demonstrated mild vascular congestion on imaging without hyperinflation, a normal to mildly elevated brain natriuretic peptide (<10 to 200 pg/mL), and dyspnea without hypoxia. She was treated normally for both COPD and congestive heart failure exacerbations simultaneously with methylprednisolone, albuterol, and furosemide with rapid improvement over the course of 1 to 2 days. No significant improvement was noted with steroid therapy, despite receiving them as an inpatient and outpatient. At the time of discharge, her symptoms would be at her baseline.

Laryngeal granular cell tumour: a very rare diagnosis for a child presenting with hoarse voice in the UK.

We present a rare case of a paediatric laryngeal granular cell tumour (GCT) of the vocal cord. GCTs are rare clinical entities, thought to arise from Schwann cells. There are only a handful of paediatric laryngeal GCTs in the literature, and therefore, little is known regarding their natural history or preferred management strategies. In this case report, we discuss the incidence, aetiopathogenesis, presentation and management of a laryngeal GCT in an 11-year-old girl, who presented with dysphonia. We believe that this case will aid otolaryngologists in the management of paediatric patients with the common dysphonia.

Granular Cell Tumor of the Pituitary Presenting with Major Intraventricular Hemorrhage.

Granular cell tumors of the pituitary gland are rare, slow-growing lesions arising from the neurohypophysis or pituitary stalk. We describe an extremely rare presentation of a pituitary granular cell tumor mimicking an anterior communicating artery aneurysmal rupture with ventricular hemorrhage. The patient was admitted in a comatose state and underwent urgent bilateral external ventricular drainage. Further diagnostic investigation revealed a sellar tumoral mass with suprasellar extension. No vascular anomalies, hormonal abnormalities, or visual disturbances were observed. Macroscopic complete resection without neurologic impairment was obtained via a right pterional approach. Posthemorrhagic hydrocephalus necessitated ventriculoperitoneal shunt placement, and hormonal substitution for panhypopituitarism was provided. The 5-year follow-up examination showed no tumor recurrence. The clinical course of these benign World Health Organization grade I lesions will normally correspond to nonsecreting pituitary adenomas with an insidious development of visual disturbances, hypopituitarism, or hydrocephalus. Sudden onset with potential catastrophic intratumoral and intraventricular hemorrhage is very uncommon.

Giant Granular Cell Tumor of the Cervical Spinal Cord Resected via Anterior Corpectomy with Reconstruction: Technical Note and Review of Literature.

BACKGROUND: Granular cell tumors (GCT) are rare soft tissue neoplasms with a nerve sheath origin, most often found in female adult populations. When these tumors arise in the central nervous system, they most commonly appear intradurally in the thoracic or lumbar spine. GCT malignancy rates vary and recurrence rates can be relatively high, thereby necessitating complete resection. CASE DESCRIPTION: We present an exceedingly rare case of an intradural, extramedullary GCT originating in the anterior cervical spine of a male pediatric patient who presented with progressive neck pain and gait instability. CONCLUSIONS: The patient underwent an anterior C7 corpectomy for resection of the tumor, followed by stabilization and fusion, and recovered without neurologic deficit. A literature review of spinal GCTs is provided.

Tumors of the Neurohypophysis: One Unit's Experience and Literature Review.

OBJECTIVE: To evaluate and understand the clinical behavior and radiologic correlates of tumors originating from the posterior pituitary gland. To review the management strategy for these rare tumors and add to the limited existing literature. METHODS: Retrospective review of 8 cases (5 pituicytomas, 2 spindle cell oncocytomas, and 1 granular cell tumor) managed at our institution between 2004 and 2019. The patients' clinical course, histologic features, and radiologic findings were reviewed. Their management and long-term follow-up is presented and compared with the literature. RESULTS: Long-term follow-up ranged from 1 to 9 years. There was 1 recurrence in a patient with spindle cell oncocytoma, and this was treated with radiotherapy. The endoscopically managed cases resulted in complete tumor excision with no recurrence. CONCLUSIONS: Epidemiologic data on primary tumors of the neurohypophysis is limited because of the rarity of these tumors. This study adds to the literature that these tumors behave as World Health Organization grade I tumors, although close follow-up is recommended as a few cases have shown recurrence. The endoscopic approach resulted in better gross total tumor resection rate in this series.

18F-FDG PET/CT and MRI of a Mediastinal Malignant Granular Cell Tumor With Associated Recurrent Pericarditis.

We report a rare case of a mediastinal malignant granular cell tumor in a 41-year-old man presenting with dyspnea, retrosternal chest pain, and recurrent pericardial effusion under treatment with corticosteroids. Because of recurrent episodes, further investigations with chest MRI and F-FDG PET/CT revealed a large infiltrating and strongly hypermetabolic mass in the mediastinum with pericarditis. Histopathology and immunohistochemical analysis showed large cells with greater pleomorphism and eosinophilic granular cytoplasm with positive staining for S100 protein and CD68 as well as high Ki67 proliferative index (15%) confirming the diagnosis of a malignant granular cell tumor.

Granular cell tumor of the pancreas diagnosed by endoscopic ultrasound-guided fine-needle aspiration.

A 68-year-old woman was referred to our hospital for the treatment of bile duct stone, pancreatic tumor, and pancreatic cysts. First, bile duct stone was removed using endoscopic retrograde cholangiopancreatography. By abdominal contrast-enhanced computed tomography, a 12-mm diameter tumor was found in the pancreatic body. The tumor was isodense compared with the surrounding pancreatic parenchyma in the non-contrast phase and poorly enhanced in the arterial phase; it exhibited gradual enhancement from the portal vein phase to the late phase. Numerous pancreatic cysts were also observed by contrast-enhanced computed tomography. By magnetic resonance imaging, the tumor was hypointense in T1-weighted images, isointense in T2-weighted images, and hyperintense in diffusion-weighted images. By magnetic resonance cholangiopancreatography, the main pancreatic duct was not dilated, and pancreatic cysts communicated with the main pancreatic duct. The pancreatic cysts were diagnosed as branch-type intraductal papillary mucinous neoplasm. Histopathologic assessment of the specimens obtained by endoscopic ultrasound-guided fine-needle aspiration revealed the tumor as benign pancreatic granular cell tumor. The patient was followed up without surgical resection. On contrast-enhanced computed tomography at 6 months after admission, the tumor did not show any changes in diameter or characteristics.

A Statistically Significant Association Between Esophageal Granular Cell Tumors and Eosinophilic Esophagitis: A 16-year Analysis at Two Large Hospitals of 167,434 EGDs.

BACKGROUND: Esophageal granular cell tumor (eGCT) is a rare, usually benign, neoplasm of neuroectodermic origin. Eosinophilic esophagitis (EoE) is a relatively uncommon, immune-mediated, chronic disease. Both diseases commonly present with dysphagia. One case has been reported of simultaneous occurrence of both diseases. AIMS: To determine the association between diseases. METHODS: The present study was an IRB-approved, retrospective review of esophagogastroduodenoscopies (EGDs) with esophageal biopsies from two large hospitals, 1999-2014. RESULTS: Among 29,235 EGDs with esophageal biopsies for 16 years (167,434 total EGDs), 16 patients had pathologically diagnosed eGCT, and 1225 patients had pathologically diagnosed EoE. Five (31%) of 16 patients with eGCT had concomitant EoE (p = 0.001, OR 10.43, 95% ORCI 3.16-32.44, Fisher's exact test). Patients with simultaneous eGCT and EoE were young (mean age = 33.6 ± 12.9 years). Three were female. Dysphagia was presenting symptom in 4 (80%) of patients. Three had asthma. All five patients had > 20 eosinophils/hpf in esophageal biopsy specimens. Three patients had endoscopic esophageal abnormalities suggesting EoE. Four patients were treated with a PPI (before and after diagnosis of EoE), and 2 patients underwent six-food-elimination diet with partial symptomatic improvement. The eGCTs averaged 13.4 ± 4.2 mm in maximal diameter and were located in upper-2, middle-2, and lower esophagus-2 (1 patient had 2 eGCTs). eGCTs were endoscopically resected-3 patients, and monitored-2 patients. Surveillance endoscopies revealed no recurrence or growth of eGCTs after resection (mean follow-up = 4.6 years). CONCLUSIONS: This novel report of 5 patients with simultaneous EoE and eGCT adds to one, previously published case and suggests these two diseases are associated, and have a common pathophysiologic link, despite apparently different pathogenesis. Large, prospective, endoscopic and pathologic studies are warranted to further investigate this association.