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The diagnosis and management of NK/T-cell lymphomas

View Article: PubMed Central - PubMed

ABSTRACT

Extranodal natural killer (NK)/T-cell lymphoma is an aggressive malignancy of putative NK-cell origin, with a minority deriving from the T-cell lineage. Pathologically, the malignancy occurs in two forms, extranodal NK/T-cell lymphoma, nasal type; and aggressive NK-cell leukaemia. Lymphoma occur most commonly (80%) in the nose and upper aerodigestive tract, less commonly (20%) in non-nasal areas (skin, gastrointestinal tract, testis, salivary gland), and rarely as disseminated disease with a leukemic phase. Genetic analysis showed mutations of genes involved in the JAK/STAT pathway, RNA assembly, epigenetic regulation, and tumor suppression. In initial clinical evaluation, positron emission tomography computed tomography, and quantification of plasma EBV DNA are mandatory as they are useful for response monitoring and prognostication. In stage I/II diseases, combined chemotherapy and radiotherapy (sequentially or concurrently) is the best approach. Conventional anthracycline-containing regimens are ineffective and should be replaced by non-anthracycline-containing regimens, preferably including L-asparaginase. Radiotherapy alone is associated with high systemic relapse rates and should be avoided. In stage III/IV diseases, non-anthracycline-regimens-containing L-asparaginase are the standard. In relapsed/refractory cases, blockade of the programmed death protein 1 has recently shown promising results with high response rates. In the era of effective non-anthracycline-containing regimens, autologous haematopoietic stem cell transplantation (HSCT) has not been shown to be beneficial. However, allogeneic HSCT may be considered for high-risk or advanced-stage patients in remission or relapsed/refractory patients responding to salvage therapy. Prognostic models taking into account presentation, interim, and end-of-treatment parameters are useful in triaging patients to different treatment strategies.

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Positron emission tomography computed tomography of NK/T-cell lymphomas. a Nasal lesion that shows avidity for 18F-fluorodeoxyglucose. b Disseminated disease. Note the large nasal tumor (arrow) and multiple hypermetabolic lesions in other anatomical sites
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Fig2: Positron emission tomography computed tomography of NK/T-cell lymphomas. a Nasal lesion that shows avidity for 18F-fluorodeoxyglucose. b Disseminated disease. Note the large nasal tumor (arrow) and multiple hypermetabolic lesions in other anatomical sites

Mentions: Nasal NK/T-cell lymphomas show a geographic predilection for Asian and South American populations, and are uncommon in other countries [6]. Its incidences in Southeast Asian and Central/South American countries were 5.2 and 3%, respectively, as compared with just 0.3% in North American and European countries [32]. The lymphoma is locally invasive. Because of angiodestruction, the lymphoma destroys midline facial structures, often manifesting as hard palate perforation (Fig. 1d) [33]. Other facial structures may also be involved, including the orbit, salivary glands, and paranasal sinuses. In non-nasal NK/T-cell lymphoma, commonly involved primary sites include the skin [34], gastrointestinal tract [35, 36], and testis [37]. Occasional involvement of rare sites including the muscle [38] and uterus [39] had also been reported. NK/T-cell lymphomas are predominantly extranodal, and primary nodal presentation is exceptionally rare [40]. It must be noted that when NK/T-cell lymphomas appear to present primarily in non-nasal sites, F18 florodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) is needed to exclude an occult nasal primary (practically all NK/T-cell lymphomas are FDG-avid [41, 42]) (Fig. 2a, b). If nasal involvement is found, these “non-nasal” cases should be regarded as nasal lymphomas with systemic spread [43]. Hence, a strict definition of non-nasal NK/T-cell lymphoma requires exclusion of a nasal primary on PET/CT.Fig. 2


The diagnosis and management of NK/T-cell lymphomas
Positron emission tomography computed tomography of NK/T-cell lymphomas. a Nasal lesion that shows avidity for 18F-fluorodeoxyglucose. b Disseminated disease. Note the large nasal tumor (arrow) and multiple hypermetabolic lesions in other anatomical sites
© Copyright Policy - OpenAccess
Related In: Results  -  Collection

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Show All Figures
getmorefigures.php?uid=PMC5391564&req=5

Fig2: Positron emission tomography computed tomography of NK/T-cell lymphomas. a Nasal lesion that shows avidity for 18F-fluorodeoxyglucose. b Disseminated disease. Note the large nasal tumor (arrow) and multiple hypermetabolic lesions in other anatomical sites
Mentions: Nasal NK/T-cell lymphomas show a geographic predilection for Asian and South American populations, and are uncommon in other countries [6]. Its incidences in Southeast Asian and Central/South American countries were 5.2 and 3%, respectively, as compared with just 0.3% in North American and European countries [32]. The lymphoma is locally invasive. Because of angiodestruction, the lymphoma destroys midline facial structures, often manifesting as hard palate perforation (Fig. 1d) [33]. Other facial structures may also be involved, including the orbit, salivary glands, and paranasal sinuses. In non-nasal NK/T-cell lymphoma, commonly involved primary sites include the skin [34], gastrointestinal tract [35, 36], and testis [37]. Occasional involvement of rare sites including the muscle [38] and uterus [39] had also been reported. NK/T-cell lymphomas are predominantly extranodal, and primary nodal presentation is exceptionally rare [40]. It must be noted that when NK/T-cell lymphomas appear to present primarily in non-nasal sites, F18 florodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) is needed to exclude an occult nasal primary (practically all NK/T-cell lymphomas are FDG-avid [41, 42]) (Fig. 2a, b). If nasal involvement is found, these “non-nasal” cases should be regarded as nasal lymphomas with systemic spread [43]. Hence, a strict definition of non-nasal NK/T-cell lymphoma requires exclusion of a nasal primary on PET/CT.Fig. 2

View Article: PubMed Central - PubMed

ABSTRACT

Extranodal natural killer (NK)/T-cell lymphoma is an aggressive malignancy of putative NK-cell origin, with a minority deriving from the T-cell lineage. Pathologically, the malignancy occurs in two forms, extranodal NK/T-cell lymphoma, nasal type; and aggressive NK-cell leukaemia. Lymphoma occur most commonly (80%) in the nose and upper aerodigestive tract, less commonly (20%) in non-nasal areas (skin, gastrointestinal tract, testis, salivary gland), and rarely as disseminated disease with a leukemic phase. Genetic analysis showed mutations of genes involved in the JAK/STAT pathway, RNA assembly, epigenetic regulation, and tumor suppression. In initial clinical evaluation, positron emission tomography computed tomography, and quantification of plasma EBV DNA are mandatory as they are useful for response monitoring and prognostication. In stage I/II diseases, combined chemotherapy and radiotherapy (sequentially or concurrently) is the best approach. Conventional anthracycline-containing regimens are ineffective and should be replaced by non-anthracycline-containing regimens, preferably including L-asparaginase. Radiotherapy alone is associated with high systemic relapse rates and should be avoided. In stage III/IV diseases, non-anthracycline-regimens-containing L-asparaginase are the standard. In relapsed/refractory cases, blockade of the programmed death protein 1 has recently shown promising results with high response rates. In the era of effective non-anthracycline-containing regimens, autologous haematopoietic stem cell transplantation (HSCT) has not been shown to be beneficial. However, allogeneic HSCT may be considered for high-risk or advanced-stage patients in remission or relapsed/refractory patients responding to salvage therapy. Prognostic models taking into account presentation, interim, and end-of-treatment parameters are useful in triaging patients to different treatment strategies.

No MeSH data available.


Related in: MedlinePlus