TY - JOUR
T1 - Extranodal NK/T-cell lymphoma, nasal type presenting as primary intestinal lymphoma vs intestinal T-cell lymphoma
T2 - A borderline diagnostic category in the revised WHO classification
AU - Beltrán, Brady E.
AU - Marques-Piubelli, Mario L.
AU - Quiñones, M. Pilar
AU - Cotrina, Esther
AU - Palomino, Eugenio A.
AU - Morales, Johnny
AU - Ramos, Wilder
AU - Sotomayor, Eduardo M.
AU - Chavez, Julio C.
AU - Castillo, Jorge J.
AU - Miranda, Roberto N.
N1 - Publisher Copyright:
© 2021 The Authors
PY - 2021/9
Y1 - 2021/9
N2 - Extranodal NK/T-cell lymphoma, nasal type, is an unusual Epstein-Barr Virus (EBV)-related lymphoma that carries a poor prognosis. Most cases affect upper aerodigestive tract, and secondarily may extend to skin, lungs, testis, and other sites. Extranodal NK/T-cell lymphoma, nasal type, presenting primarily as intestinal lymphoma is rare, and not recognized as a distinct entity and neither is considered as a special subtype of intestinal lymphoma of T-cell lineage. Here, we describe a 36-year-woman who presented with abdominal pain and acute-onset diarrhea. Her clinical course was complicated by intestinal perforation and peritonitis. She died one month later of sepsis. Pathologic examination revealed extranodal NK/T-cell lymphoma, nasal type, confined to the intestine. Immunophenotypically, the neoplastic cells expressed CD3 and Granzyme B, and were positive for EBV-encoded RNA (EBER) by in situ hybridization. From the practical point of view, we highlight the clinical, pathologic and immunophenotypic features that can be useful in the differential diagnosis from other intestinal lymphomas of T-cell lineage.
AB - Extranodal NK/T-cell lymphoma, nasal type, is an unusual Epstein-Barr Virus (EBV)-related lymphoma that carries a poor prognosis. Most cases affect upper aerodigestive tract, and secondarily may extend to skin, lungs, testis, and other sites. Extranodal NK/T-cell lymphoma, nasal type, presenting primarily as intestinal lymphoma is rare, and not recognized as a distinct entity and neither is considered as a special subtype of intestinal lymphoma of T-cell lineage. Here, we describe a 36-year-woman who presented with abdominal pain and acute-onset diarrhea. Her clinical course was complicated by intestinal perforation and peritonitis. She died one month later of sepsis. Pathologic examination revealed extranodal NK/T-cell lymphoma, nasal type, confined to the intestine. Immunophenotypically, the neoplastic cells expressed CD3 and Granzyme B, and were positive for EBV-encoded RNA (EBER) by in situ hybridization. From the practical point of view, we highlight the clinical, pathologic and immunophenotypic features that can be useful in the differential diagnosis from other intestinal lymphomas of T-cell lineage.
KW - EBV
KW - Intestinal lymphoma
KW - NK/T-cell
KW - Peripheral T-cell lymphoma
KW - T-cell lymphoma
UR - http://www.scopus.com/inward/record.url?scp=85108261427&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85108261427&partnerID=8YFLogxK
U2 - 10.1016/j.ehpc.2021.200534
DO - 10.1016/j.ehpc.2021.200534
M3 - Article
AN - SCOPUS:85108261427
SN - 2214-3300
VL - 25
JO - Human Pathology: Case Reports
JF - Human Pathology: Case Reports
M1 - 200534
ER -