Extranodal natural killer/T cell lymphoma, nasal type in the middle cranial fossa

Abstract

Introduction:Extranodal natural killer (NK)/T cell lymphomas, nasal type, are aggressive, non-Hodgkin lymphomas. Extranodal NK/T cell lymphomas, nasal type, involving the central nervous system (CNS) are rare; therefore, delayed diagnosis easily occurs and is associated with a poor prognosis. Early diagnosis and patented systemic chemotherapy are necessary.Case presentation:We present a case of 34-year-old male with facial numbness and diplopia. He was diagnosed with extranodal NK/T cell lymphoma, nasal type, involving the CNS. The tumor, located in the right middle fossa, was subtotally removed, and 3 cycles of systemic chemotherapy were given. He later died of severe neutropenia and infection.Conclusion:NK/T cell lymphomas should be considered to be a potential cause of facial numbness and diplopia. A L-asparaginase-based regimen resulted in reasonable tumor suppression, but adverse effects, including fatal neutropenia, should be carefully considered.

An Unusual Case of Extranodal Natural Killer/T-Cell Lymphoma, Nasal Type Masquerading as Dacryocystitis and Sinusitis

“…2,7 Natural killer/T-cell lymphoma, nasal type presents as rapidly progressive ulceration and necrotic granuloma in the nasal cavity, the Waldeyer's ring, and the upper aerodigestive tract. [1][2][3][4][5]7 Natural killer/Tcell lymphoma, nasal type can invade the paranasal sinus, hard plate, central nervous system, orbits, and facial skin. [1][2][3][4]7 To our knowledge, we report the first case of NNKTL specifically invading and obstructing the nasolacrimal duct to present as persistent dacryocystitis and paranasal sinusitis unresponsive to antibiotics and surgical intervention.…”

“…2,3,8 While there is no standard staging system for NNKTL, F18 fluorodeoxyglucose PET/CT helps with staging using adopted systems like the T-staging system or the Ann Arbor system. 2,5,7 Chemotherapy, combined with radiotherapy either concurrently or sequentially, is the main treatment strategy for NNKTL. 1,3,4 Conventional anthracycline-containing chemotherapy regimens have been replaced by nonanthracycline-containing regimens due to P-glycoproteinassociated resistance.…”

“…1,2,4 Extensive local destruction and invasion has been noted, especially of the paranasal sinuses, hard palate, and central nervous system. [1][2][3][4]7 Invasion of the nasolacrimal duct with dacryocystitis is yet to be reported. Symptoms of NNKTL include nasal obstruction, bloody rhinorrhea, sore throat, necrotizing lesions of the hard palate or nose, hard palate perforation, hoarseness, edema of cheek or orbit, and systemic symptoms such as prolonged fever, night sweats, and weight loss.…”

Natural killer/T-cell lymphoma with intracranial infiltration and Epstein-Barr virus infection: A case report

“…NKTL with eyelid involvement and intracranial infiltration is rare, and the literature indicates that the prognosis is poor[ 2 , 3 ]. Wang et al[ 4 ] reported the case of a 34-year-old man with extranodal nasal-type NKTL involving the middle cranial fossa. He underwent three cycles of chemotherapy and intrathecal injection.…”

Diagnosis, prevention and treatment of central nervous system involvement in peripheral t-cell lymphomas

“…As in nodal lymphomas, PTCL-NOS is the most common T-cell subtype diagnosed in the CNS, followed by ALCL and ENKTCL (Kim et al, 2020;Chiavazza et al, 2018;Menon et al, 2015;Miyata-Takata et al, 2017;Hoang-Xuan et al, 2015). ENKTCL of nasal type commonly occurs in the upper respiratory and digestive tracts, and in spite of its localization and aggressive behavior, it rarely infiltrates the CNS (Yi et al, 2011;Kim et al, 2010;Wang et al, 2018). This may be explained by the frequent use of HD-MTX as part of therapeutic combinations, which should act as CNS prophylaxis (see below) (Yamaguchi et al, 2011).…”