A Rare Case of Aggressive Disseminated Nasal-Type Epstein-Barr Virus-Positive Extranodal Natural Killer/T-Cell Lymphoma with Bone Marrow Involvement.

BACKGROUND Nasal-type extranodal natural killer/T-cell lymphoma (ENKL) is an exceedingly rare and aggressive subtype of non-Hodgkin lymphoma. The malignancy has both a high morbidity and mortality and is most commonly discovered in patients with advanced stages of the disease. As a result, early detection and treatment is tantamount to improving survival and minimizing lasting effects. CASE REPORT Herein, we report a case of nasal-type ENKL in a woman with facial pain and associated nasal and eye discharge. We highlight the histopathologic features from nasopharyngeal and bone marrow biopsy, which demonstrated Epstein-Barr virus-positive biomarkers of diffuse and subtle involvement, respectively, with associated chromogenic immunohistochemical staining. We also highlight existing therapy utilizing a combination of chemotherapy with radiation, as well as consolidation therapy, and suggest the need for further research of allogeneic hematopoietic stem cell treatment and the potential of programmed death ligand 1 (PD-L1) inhibition in managing nasal-type ENKL malignancy. CONCLUSIONS Nasal-type ENKL is a rare subtype of non-Hodgkin lymphoma that is infrequently associated with bone marrow involvement. The malignancy has a poor prognosis overall and typically is discovered late in the disease course. Current treatment favors utilization of combined modality therapy. However, previous studies have been inconsistent in determining whether chemotherapy or radiation therapy can be used alone. Additionally, promising results have also been shown with chemokine modulators, including antagonistic drugs that target PD-L1, in refractory and advanced cases.

Multifocal granular cell tumor presenting as an esophageal stricture.

INTRODUCTION: Granular cell tumors are uncommonly found in the gastrointestinal tract with slow progression and are usually benign though they may have propensity for malignant transformation. Initially attributed to neuronal origin through immunohistochemistry, there has been controversy with increasing reports of granular cell tumors of non-neural origin. CASE REPORT: We report a case of multifocal granular cell tumor involving the esophagus and stomach in a young female with history of dysphagia for 9 years with worsening symptoms. She had been managed at another facility with repeated dilations for presumed benign peptic stricture. Radial endosonography (EUS) of the proximal end of stricture showed a posterior submucosal esophageal mass that was heterogeneous and invaded into the muscularis propria. Fine-needle aspiration (FNA) showed large cells with granular cytoplasm along with spindle nuclei. Cells were initially checked for CD117 stain alone and found to be negative. A follow-up CT-guided core needle biopsy revealed similar granular cells that were positive for S-100. She underwent a two-stage transhiatal esophagogastrectomy as the tumor circumferentially involved the cervical esophagus and was adherent to the trachea and recurrent laryngeal nerve bilaterally. At surgery, there were two additional foci palpable in the proximal stomach. DISCUSSION AND CONCLUSION: As these tumors may have potential for malignant transformation and locoregional invasion, they should be considered while evaluating submucosal lesions of the esophagus even in young patients. A large number of granular cell tumors may be missed in the absence of S-100 staining, which should be requested when granular cells are seen on cytology obtained by EUS FNA as this can be a minimally invasive diagnostic modality for these tumors. Other foci should be sought at surgery as they have a propensity for locoregional spread.