Malignant Granular Cell Tumor of the Esophagus: A Case Report

Abstract

Introduction: Malignant granular cell tumor (MGCT) of the esophagus is an extremely rare malignancy with a poor prognosis. Literature describing this condition is not sufficient, especially regarding long-term survival. Presentation of Case: A 52-year-old woman presented with dyspnea and slow onset dysphagia. The endoscopy, endoscopic ultrasound (EUS), bronchoscopy, and positron emission tomography (PET)/computed tomography (CT) supported the suspicion of esophageal gastrointestinal stromal tumor (GIST). Open wedge esophagectomy and tracheal resection were performed. The histology proved periodic acid-Schiff (PAS)-positive granules in epithelial cells, hyperchromatic nuclei and the positivity of Protein soluble in 100% ammonium sulfate (S-100), vimentin, neuron-specific enolase, laminin, and myelinic proteins. Local recurrence after 10 months required a two-phase esophagectomy with retrosternal gastroplasty. Bone, liver, and mediastinal metastases occurred 6 months later, with overall survival of 34 months. Discussion: Preoperative histological confirmation is often not reliable. Tracheal invasion increases the perioperative risk and the probability of an unsuccessful resection. Esophagectomy or radical R0 local resection is the only known curative therapy. Repeated resections may increase survival in case of locoregional recurrence. Radiotherapy has a potential for palliative care. Conclusion: Esophageal MGCT requires a detailed presentation including long-term survival. Early surgical removal of intramural esophageal neoplasms with potentially malignant features is highly recommended. Radical and/or repeated esophageal resections are the only known therapies with curative potential.

Bladder malignant granular cell tumor with EP300 gene mutation: a case report and literature review

“…Malignant GCM is extremely rare, and few cases have been reported [7,11,17,23,24]. So far, we know little about the molecular biology and molecular genetics of malignant GCT.…”

“…Most of the clinical manifestations are painless masses in subcutaneous or deep soft tissue. Most of the cases occurred in the extremities, torso, head, neck, and breast [10][11][12][13][14][15][16]. To date, few cases of bladder malignant GCT have been reported [7,17].…”

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

“…Although a few reports indicated the usefulness of a PET-CT scan in distinguishing malignant GCTs from benign ones, it is still unclear whether they are indeed distinguishable by PET-CT. Moreover, there is currently no standard cutoff value of FDG uptake [ 7 , 18 ].…”

“…Stašek et al recommended that GCT, which might have potentially malignant features, should be removed early on, as the prognosis of malignant cases is poor [ 7 ]. Accordingly, the timely diagnosis and resection of GCTs is critical.…”

“…A few reports have indicated that minute specimens obtained via EUS-FNA provided a definite diagnosis of gastrointestinal GCT [3]. However, in most previous studies, a definite diagnosis of GCT was made using the resected tumor itself after endoscopic or surgical resection [4][5][6][7][8]. John et al reported the utility of EUS-FNA, but the final diagnosis of GCT was made using specimens obtained by a computed tomography (CT)-guided core needle biopsy [9].…”