Extranodal NK-T-cell lymphoma overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ramyar Ghandriz MD[2] Sowminya Arikapudi, M.B,B.S. [3]
Overview
Extranodal NK-T-cell lymphoma, nasal type, can develop in either T cells or natural killer cells, but most often in the NK cells. Natural killer cells are a type of lymphocyte that are closely related to T cells and attack foreign cells. Extranodal NK-T-cell lymphoma may be classified according to WHO into 2 subtypes: NK cell-derived neoplasms, namely, aggressive NK cell leukemia and extra nodal NK-T-cell lymphoma, nasal type. Based on the organ involvement, extranodal NK-T-cell lymphoma may be classified into extranodal NK-T-cell lymphoma, nasal type and extranodal NK-T-cell lymphoma, extra nasal type. On gross pathology, angiocentric and angiodestructive pattern of growth with associated geographical necrosis and ulceration are characteristic findings of extranodal NK-T-cell lymphoma. On microscopic histopathological analysis, medium sized tumor cells and polymorphic infiltrate of nonneoplastic inflammatory cells are characteristic findings of extranodal NK-T-cell lymphoma.There are no established causes for extranodal NK-T-cell lymphoma. Extranodal NK-T-cell lymphoma must be differentiated from other diseases such as anaplastic large cell lymphoma, non specific inflammatory process, enteropathy associated T cell lymphoma, and peripheral T cell lymphoma.Patients of all age groups may develop extranodal NK-T-cell lymphoma. Males are more commonly affected with extranodal NK-T-cell lymphoma than females. The majority of extranodal NK-T-cell lymphoma cases are reported in Asian, Central-American, and South-American countries.There are no established risk factors for extranodal NK-T-cell lymphoma. According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for extranodal NK-T-cell lymphoma.If left untreated, patients with extranodal NK-T-cell lymphoma, nasal type may progress to develop proptosis and hard palate perforation. Common complications of extranodal NK-T-cell lymphoma include hepatosplenomegaly and pancytopenia. Depending on the extent of the tumor at the time of diagnosis, the prognosis may vary. Prognosis is generally regarded as poor.[1][2]There is no established system for the staging of extranodal NK-T-cell lymphoma. A T-staging system, originally designed for sinonasal B-cell lymphoma has been adopted to overcome this problem by taking into account the extent of local tumor involvement.The most common symptoms of extranodal NK-T-cell lymphoma include fever, weight loss, skin rash, night sweats, protrusion of eye, swelling of the face, discharge from the nose, nose bleeds, blockage of the nasal passages, chest pain, abdominal pain, bone pain, and painless swelling in the neck, axilla, groin, thorax, and abdomen.Common physical examination findings of extranodal NK-T-cell lymphoma include fever, rash, ulcer, proptosis, midfacial destructive lesions, epistaxis, nasal obstruction due to mass, chest tenderness, abdomen tenderness, bone tenderness, peripheral lymphadenopathy, and central lymphadenopathy.Laboratory tests for extranodal NK-T-cell lymphoma include complete blood count (CBC), blood chemistry studies, cytogenetic analysis, flow cytometry, immunohistochemistry, and immunophenotyping.Lymph node or extranodal tissue biopsy is diagnostic of extranodal NK-T-cell lymphoma. CT scan may be helpful in the diagnosis of extranodal NK-T-cell lymphoma. Computerized tomography (CT) may be performed to detect metastasis and assess the local extent of extranodal NK-T-cell lymphoma.MRI may be helpful in the diagnosis of extranodal NK-T-cell lymphoma.Magnetic resonance imaging (MRI) may be performed to better define local soft tissue and bony involvement of extranodal NK-T-cell lymphoma. [2]PET scan may be helpful in the diagnosis of extranodal NK-T-cell lymphoma.Other diagnostic studies for extranodal NK-T-cell lymphoma include PCR, bone marrow aspiration, bone marrow biopsy, and in situ hybridization.The predominant therapy for extranodal NK-T-cell lymphoma is radiation therapy. Adjunctive chemotherapy and stem cell transplant may be required.
Classification
Extranodal NK-T-cell lymphoma may be classified according to WHO into 2 subtypes: NK cell-derived neoplasms, namely, aggressive NK cell leukemia and extra nodal NK-T-cell lymphoma, nasal type. Based on the organ involvement, extranodal NK-T-cell lymphoma may be classified into extranodal NK-T-cell lymphoma, nasal type and extranodal NK-T-cell lymphoma, extra nasal type.
Pathophysiology
Extra nodal NK-T-cell lymphoma, nasal type, can develop in either T cells or natural killer cells, but most often in the NK cells. Natural killer cells are a type of lymphocyte that are closely related to T cells and attack foreign cells. On gross pathology, angiocentric and angiodestructive pattern of growth with associated geographical necrosis and ulceration are characteristic findings of extranodal NK-T-cell lymphoma. On microscopic histopathological analysis, medium sized tumor cells and polymorphic infiltrate of nonneoplastic inflammatory cells are characteristic findings of extranodal NK-T-cell lymphoma.
Causes
There are no established causes for extranodal NK-T-cell lymphoma.
Differential Diagnosis
Extranodal NK-T-cell lymphoma must be differentiated from other diseases such as anaplastic large cell lymphoma, non specific inflammatory process, enteropathy associated T cell lymphoma, and peripheral T cell lymphoma.
Epidemiology and Demographics
Patients of all age groups may develop extranodal NK-T-cell lymphoma. Males are more commonly affected with extranodal NK-T-cell lymphoma than females. The majority of extranodal NK-T-cell lymphoma cases are reported in Asian, Central-American, and South-American countries.
Risk Factors
There are no established risk factors for extranodal NK-T-cell lymphoma.
Screening
According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for extranodal NK-T-cell lymphoma.
Natural History, Prognosis, and Complications
If left untreated, patients with extranodal NK-T-cell lymphoma, nasal type may progress to develop proptosis and hard palate perforation. Common complications of extranodal NK-T-cell lymphoma include hepatosplenomegaly and pancytopenia. Depending on the extent of the tumor at the time of diagnosis, the prognosis may vary. Prognosis is generally regarded as poor.
Diagnosis
Staging
There is no established system for the staging of extranodal NK-T-cell lymphoma. A T-staging system, originally designed for sinonasal B-cell lymphoma has been adopted to overcome this problem by taking into account the extent of local tumor involvement.
Symptoms
The most common symptoms of extranodal NK-T-cell lymphoma include fever, weight loss, skin rash, night sweats, protrusion of eye, swelling of the face, discharge from the nose, nose bleeds, blockage of the nasal passages, chest pain, abdominal pain, bone pain, and painless swelling in the neck, axilla, groin, thorax, and abdomen.
Physical Examination
Common physical examination findings of extranodal NK-T-cell lymphoma include fever, rash, ulcer, proptosis, midfacial destructive lesions, epistaxis, nasal obstruction due to mass, chest tenderness, abdomen tenderness, bone tenderness, peripheral lymphadenopathy, and central lymphadenopathy.
Laboratory tests
Laboratory tests for extranodal NK-T-cell lymphoma include complete blood count (CBC), blood chemistry studies, cytogenetic analysis, flow cytometry, immunohistochemistry, and immunophenotyping.
Biopsy
Lymph node or extranodal tissue biopsy is diagnostic of extranodal NK-T-cell lymphoma.
CT
CT scan may be helpful in the diagnosis of extranodal NK-T-cell lymphoma. Computerized tomography (CT) may be performed to detect metastasis and assess the local extent of extranodal NK-T-cell lymphoma.
MRI
MRI may be helpful in the diagnosis of extranodal NK-T-cell lymphoma.Magnetic resonance imaging (MRI) may be performed to better define local soft tissue and bony involvement of extranodal NK-T-cell lymphoma.
Other Imaging Studies
PET scan may be helpful in the diagnosis of extranodal NK-T-cell lymphoma.
Other Diagnostic Studies
Other diagnostic studies for extranodal NK-T-cell lymphoma include PCR, bone marrow aspiration, bone marrow biopsy, and in situ hybridization.
Treatment
Medical Therapy
The predominant therapy for extranodal NK-T-cell lymphoma is radiation therapy. Adjunctive chemotherapy and stem cell transplant may be required.
References
- ↑ Extranodal NK-/T-cell lymphoma, nasal type. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd530f/. Accessed on February 04, 2016
- ↑ 2.0 2.1 Extranodal Natural-Killer/T-Cell Lymphoma, Nasal Type. Hindawi Publishing Corporation. http://www.hindawi.com/journals/ah/2010/627401/. Accessed on February 18, 2016