Extranodal NK/T-cell lymphoma, nasal type, with extranasal presentation – a case report and a review of the literature

Abstract

Background: Extranodal NK/T-cell lymphoma (ENKTCL), nasal type is a very rare and aggressive non-Hodgkin lymphoma. Most commonly it occurs in the upper aerodigestive tract. But, it can also manifest at locations such as the skin, soft tissue, gastrointestinal tract (GI), lungs, testis, etc. These locations are designated as extranasal ENKTCL. The patients with the latter have often more adverse clinical features and poorer survival rate compared with nasal sites. We present a case of an 83-year-old patient with a primary ENKTCL, nasal type, with extranasal presentation in the right upper eyelid. Material and methods: Materials for the literature review was obtained by a comprehensive search on PubMed, which yielded 82 eligible cases with extranasal ENKTCL. Results: Sixty-eight cases (83 %) were localized as primary ENKTCL in the lungs (17), central nervous system (CNS) ( 14), testis (11), GI-tract ( 7), skin (6), orbit and intraocular tissue (4), pancreas (2), adrenal gland (2), breast (1), etc. 14 cases (17 %) presented as extended or disseminated diseases involving exclusively organs outside the upper aerodigestive tract. There was no systematic pattern of organ involvement in the extended/disseminated ENKTCL. 63 % of the patient with localized extranasal ENKTCL and about 50% of patients with extended/disseminated disease were reported to have died of the disease. Treatment strategies varied with no preferred option. Among the used treatment options were chemotherapy, radiotherapy, surgery, stem cell transplantation alone or in different combinations. Conclusion: ENKTCL is a highly aggressive disease which may present in extranasal areas. Although the tumors respond to both chemotherapy and radiotherapy, durable complete remissions are very rare.

TPX2 Enhanced the Activation of the HGF/ETS-1 Pathway and Increased the Invasion of Endocrine-Independent Prostate Carcinoma Cells

“…The highly aggressive feature of cancerous cells is reflected in the invasive growth of malignant cells that gradually destroy the normal structure of tissues in the primary location and are translocated to another location (the metastatic process) (31,32). During disease progression, tumor cells can break through the restrictions of the extracellular matrix and migrate to other tissues and organs (31,32).…”

“…The highly aggressive feature of cancerous cells is reflected in the invasive growth of malignant cells that gradually destroy the normal structure of tissues in the primary location and are translocated to another location (the metastatic process) (31,32). During disease progression, tumor cells can break through the restrictions of the extracellular matrix and migrate to other tissues and organs (31,32). In these processes, the destruction of the local extracellular matrix by tumor cells and the destruction of the extracellular matrix at the site of metastasis during metastasis are the key steps for its proliferation, metastasis, and invasion (33)(34)(35).…”

Nonhealing Ulcers with Rimming of the Adipose Tissues

“…Both primary and secondary cutaneous ENKTCL-NT have a poor prognosis with 5-year overall survival of 12–61% [ 2 , 5 ] and median survival time from 2 to 15 months [ 4 ], and limited skin involvement portends a better outcome than those with extracutaneous involvement [ 4 , 5 ]. According to the proposed prognostic index of natural killer cell lymphoma (PINK) and PINK with EBV DNA (PINK-E), our patient was stratified as an intermediate-risk group using PINK (1 risk factor) and a low-risk group using PINK-E (1 risk factor), with 3-year overall survival rates of 62% and 55%, respectively [ 6 ].…”

“…Current standard treatment for both skin-limited and extracutaneous ENKTCL-NT is sequential chemoradiotherapy with platinum- or asparaginase-containing chemotherapy, which significantly improves the survival outcomes, especially in the early stages [ 4 , 7 , 10 ]. Furthermore, hematopoietic stem cell transplantation, targeted therapy such as monoclonal antibodies (anti-CD30, anti-CD38), and checkpoint inhibitors, i.e., programmed death 1 inhibitor, pembrolizumab, are viable options in relapsed/refractory condition [ 2 , 5 , 10 , 11 , 12 ].…”

“…Extranodal NK/T-cell lymphoma, nasal type (ENKTCL-NT) is a rare and aggressive EBV-associated non-Hodgkin lymphoma. This entity carries poor response to current treatment, associated with a high relapse rate and mortality [ 1 , 2 ]. Various cutaneous manifestations have been reported, and histopathologic examination can mimic both inflammatory condition and other hematologic malignancies.…”