Primary Central Nervous System Extranodal NK/T Cell Lymphoma, Nasal Type, with Antecedent Hemophagocytic Syndrome in a Child

Abstract

Primary central nervous system (CNS) extranodal natural killer (NK)/T-cell lymphoma, nasal type (NKTCL), is an exceedingly uncommon entity. Here, we present a case of CNS NKTCL that manifested initially as hemophagocytic syndrome 4 months earlier in a 13-year-old girl. Histological examination revealed the cerebellum mass was composed of large-sized and atypical tumor cells, with an angiocentric and angiodestructive growth pattern and prominent necrosis. The tumor cells exhibited marked pleomorphism with conspicuous nucleoli and prominent mitotic activity. Immunohistochemical staining showed the tumor cells were positive for CD45, CD2, CD3ε, CD30, CD43, CD56, and granzyme B. Epstein-Barr virus--encoded ribonucleic acid was expressed in almost all of the nuclei of the lymphoma cells. The T-cell receptor γ chain gene rearrangement study showed no evidence of a clonal rearrangement. The patient was treated with etoposide and dexamethasone and died a few days after the operation. As far as we know, this case is the 1st pediatric and female patient of primary CNS NKTCL with antecedent hemophagocytic syndrome, which highlights the clinical data and is helpful for the diagnosis of this tumor.

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

“…[ 1 , 2 ] Extranodal NK/T cell lymphomas are commonly located in the upper respiratory and digestive tracts, such as the nasal cavity, and sometimes occur in the skin or gastrointestinal (GI) tract, but rarely invade the central nervous system (CNS). [ 3 ] Only a few cases of a NK/T cell lymphoma invading the CNS have been reported; [ 4 – 6 ] here, we reported a case of an extranodal NK/T cell lymphoma in the right middle cranial fossa, with diplopia and facial numbness as the initial presenting symptoms.…”

Successful Treatment of Primary CNS Extranodal NK/T-Cell Lymphoma with Surgery and Chemotherapy Combined with Sintilimab: A Case Report and Literature Review

“…than extracranial NK/TCL. [6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24] PCNSLs are often accompanied by nonspecific clinical symptoms such as dizziness, headache, vomiting, and blurred vision. Our patient presented with loss of visual acuity and weakness of the limbs, which were consistent with the nonspecific symptoms of primary CNS lymphoma.…”

“…collected from the English literature and summarized. [6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24] These patients were pathologically diagnosed as primary CNS ENK/TCL, excluding secondary and invasive NK cell leukemia. According to previously reported cases, overall survival in patients with PCNS ENK/TCL is not high.…”

“…To our knowledge, only 23 cases of PCNS ENK/TCL have been reported in the English literature. [6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24] To date, the understanding of this tumor is not deep enough. Herein, we present a case of PCNS ENK/TCL in a 50year-old immunocompetent Chinese female and review of the literature.…”

An Unusual Case of EBV-Negative Primary CNS Lymphoma of Natural Killer/T-Cell Lineage

“…The diagnoses were established either on biopsy or by the detection of malignant cells in the cerebrospinal fluid. 12 - 25 …”