Lymphoma & related disorders
Mature T/NK cell disorders
Aggressive NK/T cell disorders
Extranodal NK / T cell lymphoma, nasal type


Minor changes: 6 October 2020

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PubMed Search: Extranodal NK/T cell lymphoma nasal type[TI] free full text[SB]

Mario L. Marques-Piubelli, M.D.
Roberto N. Miranda, M.D.
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Cite this page: Marques-Piubelli ML, Torres-Cabala CA, Miranda RN. Extranodal NK / T cell lymphoma, nasal type. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/lymphomanonBnasal.html. Accessed October 28th, 2020.
Definition / general
Essential features
Terminology
  • Synonyms
    • Polymorphic reticulosis
    • Malignant midline reticulosis
    • Lethal midline granuloma
    • Angiocentric T cell lymphoma (Blood 1994;84:1361)
ICD coding
  • ICD-O: 9719 / 3 - extranodal NK / T cell lymphoma, nasal type
  • ICD-10: C86.0 - extranodal NK / T cell lymphoma, nasal type
Sites
Pathophysiology
Etiology
Clinical features
Diagnosis
Radiology description
  • Both CT and MRI are useful for evaluation of extension in nasal lesions (Expert Rev Hematol 2016;9:861)
    • No pathognomonic findings
  • PET / CT is used to identify extranasal lesions (Expert Rev Hematol 2016;9:861)
    • F-18 fluorodeoxyglucose avid
    • Standard uptake value is typically less than aggressive B cell lymphomas
Prognostic factors
Case reports
Treatment
Clinical images

Contributed by Auris Huen, M.D.
Skin lesion

Skin lesion

Microscopic (histologic) description
Microscopic (histologic) images

Contributed by Roberto N. Miranda, M.D. and Carlos A. Torres-Cabala, M.D.
Nasal lesion of ENKTCL

Nasal lesion of ENKTCL

Diffuse infiltrate

Diffuse infiltrate

Cytological atypia

Cytological atypia

EBER positivity

EBER positivity

Angioinvasion in ENKTCL

Angioinvasion in ENKTCL

Atypical infiltrate in skin

Atypical infiltrate in skin


CD3 positivity

CD3 positivity

CD7 positivity

CD7 positivity

CD56 positivity

CD56 positivity

Perforin positivity

Perforin positivity

Hemophagocytosis

Hemophagocytosis

ENKTCL NT in bone marrow

ENKTCL NT in bone marrow


Bone marrow CD3

Bone marrow CD3

Bone marrow EBER

Bone marrow EBER

Skin involvement by PTCL, NOS

Skin involvement by PTCL, NOS

 cell LGL leukemia

Cytological atypia in PTCL, NOS

 cell LGL leukemia

T cell LGL leukemia

CD3 in T cell LGL

CD3 in T cell LGL


CD57 in T cell LGL

CD57 in T cell LGL

TIA1 in T cell LGL

TIA1 in T cell LGL

Skin in EBV+ DLBCL

Skin in EBV+ DLBCL

CD20 in DLBCL

CD20 in DLBCL

EBER in DLBCL

EBER in DLBCL

Necrosis in tuberculosis

Necrosis in tuberculosis


Necrosis in histoplasmosis

Necrosis in histoplasmosis

Histoplasmosis GMS stain

Histoplasmosis GMS stain

Cytology description
Positive stains
Molecular / cytogenetics description
Sample pathology report
  • Left maxillary sinus, biopsy:
    • Extranodal NK / T cell lymphoma, nasal type (see comment)
    • Comment: According to clinical records, patient is a 56 year old Hispanic male with recent clinical history of nasal obstruction, purulent discharge, epistaxis and B type symptoms. In the last 5 months, the patient has received several antibiotic regimens with no improvement of the clinical course. A recent CT of the head and neck showed a 6 cm mass in the left maxillary sinus with invasion of the adjacent bone.
    • The biopsy is adequate and shows respiratory type mucosa with underlying dense and monotonous lymphoid infiltrate with focal vascular invasion. The lymphocytes are of medium size, folded nuclei, indistinct nucleoli and moderate amount of cytoplasm. Frequent mitoses are observed. There is focal ulceration and extensive coagulative necrosis.
    • Immunohistochemical studies are performed and the atypical cells are positive for CD30, CD56, granzyme B and TIA1. Ki67 proliferation index is 60%. In situ hybridization for EBV encoded RNA (EBER) shows reactivity in most tumor cells. The abnormal cells are negative for sCD3, CD5, CD8, CD20, PAX5 as well as for TCRδ and TCRαβ. Acid fast bacilli stain and Gomori methenamine silver stain for fungal organisms are negative.
    • Next generation sequencing demonstrated mutation of STAT3.
    • Polymerase chain reaction for Ig heavy chain (IGH) and T cell receptors gamma (TRG) and beta (TRB) chains revealed a germline pattern.
Differential diagnosis
Board review style question #1
Which of the following is true about extranodal NK / T cell lymphoma, nasal type (ENKTCL)?

  1. Frequently presents as an indolent disease with favorable prognosis
  2. JAK / STAT pathway is usually activated
  3. Most common EBV latency pattern is type III
  4. Necrosis and angiotropism are usually absent
Board review answer #1
B. JAK / STAT pathway is usually activated

Comment Here

Reference: Extranodal NK / T cell lymphoma, nasal type
Board review style question #2
What is the most common immunophenotype of extranodal NK / T cell lymphoma, nasal type?

  1. εCD3+ / sCD3- / CD5- / CD16+ / CD56+ / EBER+
  2. εCD3+ / sCD3+ / CD5- / CD16+ / CD56+ / EBER+
  3. εCD3- / sCD3+ / CD5- / CD16- / CD56- / EBER+
  4. εCD3- / sCD3- / CD5- / CD16- / CD56+ / EBER+
Board review answer #2
A. εCD3+ / sCD3- / CD5- / CD16+ / CD56+ / EBER+

Comment Here

Reference: Extranodal NK / T cell lymphoma, nasal type
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