Primary Esophageal Extranasal NK/T Cell Lymphoma With Biphasic Morphology

Abstract

We report a case of esophageal extranasal NK/T cell lymphoma with biphasic morphologic features revealed by a deep large piecemeal biopsy.A 40-year-old man present with pharyngalgia, dysphagia, recurrent fever, and 5-kg weight loss for 8 months. Endoscopy demonstrated progressing longitudinal ulcers and mucosal bridges along the esophagus. The first and second biopsies obtained superficial mucosa with scattered bland-looking small lymphocytes. A subsequent large piecemeal snare abscission for biopsy showed atypical lymphoid cells infiltrating into the deep lamina propria and muscularis mucosae, whereas the superficial lamina propria was highly edematous with scant small lymphocytes. Immunohistochemical studies confirmed that both underlying atypical cells and superficial small lymphocytes were neoplastic, sharing an identical immunophenotype: positive for CD2, CD3, CD43, CD8, CD56, TIA-1 and granzyme B. Epstein-Barr virus–encoded small RNAs were found in both cells. The histologic findings were diagnostic of primary esophageal extranasal NK/T cell lymphoma. However, the patient developed bone marrow depression during chemotherapy and died of massive cerebral hemorrhage after the first cycle of chemotherapy.Primary esophageal extranodal NK/T cell lymphoma nasal type is extremely rare. We show the biphasic morphology of this disease, which highlights the importance of deep biopsy for accurate diagnosis.

Primary lymphoma of the entire esophagus diagnosed by endoscopic ultrasound-guided fine needle aspiration (EUS-FNA)

“…The GI tract is the most commonly involved extranodal site in non-Hodgkin's lymphoma. [1] Primary GI lymphoma, however, is rare, and the esophagus is an extremely rarely involved site, with less than 30 cases in the literature [2 , 3 , 5 , 6] . They are predominantly B-cell type, with few cases of mucosa-associated lymphoid tissue (MALT) type described, and only one diagnosed by EUS-FNA [7 , 8] .…”

“…They are predominantly B-cell type, with few cases of mucosa-associated lymphoid tissue (MALT) type described, and only one diagnosed by EUS-FNA [7 , 8] . Imaging findings in esophageal lymphoma are unspecific, and vary greatly, including polypoid masses, strictures, ulcers, thickening of the mucosal folds, submucosal nodules, among others [2 , 3] . Given such a diverse spectrum of presentations, histological assessment is mandatory to confirm diagnosis.…”

“…Primary esophageal lymphoma is an extremely rare condition, accounting for <1% of all gastrointestinal (GI) lymphomas. [1] Radiological and endoscopic findings show several morphologic characteristics and are nonspecific, posing diagnostic challenges [2 , 3] , and many of these cases are finally diagnosed surgically. We report a case of an esophageal non-Hodgkin lymphoma involving the whole length of the esophagus.…”

Primary Esophageal Lymphoma: Clinical Experience in Diagnosis and Treatment

“…In this paper, the clinical data of 15 cases of primary esophageal lymphoma were collected and analyzed retrospectively, in which one case was admitted to our institution and 14 cases were reported in the literatures [2][3][4][5][6][7][8][9][10][11][12][13][14]. To improve the diagnosis and treatment of this disease, a systematic analysis was carried out, highlighting the clinical, endoscopic, and pathological features.…”