Generic selectors
Exact matches only
Search in title
Search in content
Post Type Selectors
Filter by Categories
15th National Conference of the IAOMFP, Chennai, 2006
Abstracts from current literature
Acne in India: Guidelines for management - IAA Consensus Document
Art & Psychiatry
Association Activities
Association Notes
Award Article
Book Review
Brief Report
Case Analysis
Case Letter
Case Letters
Case Notes
Case Report
Case Reports
Clinical and Laboratory Investigations
Clinical Article
Clinical Studies
Clinical Study
Conference Oration
Conference Summary
Continuing Medical Education
Cosmetic Dermatology
Current Best Evidence
Current Issue
Current View
Derma Quest
Dermato Surgery
Dermatosurgery Specials
Dispensing Pearl
Do you know?
Drug Dialogues
Editor Speaks
Editorial Remarks
Editorial Report
Editorial Report - 2007
Editorial report for 2004-2005
Fourth All India Conference Programme
From Our Book Shelf
From the Desk of Chief Editor
Get Set for Net
Get set for the net
Guest Article
Guest Editorial
How I Manage?
IADVL Announcement
IADVL Announcements
IJDVL Awards
IJDVL Awards 2018
IJDVL Awards 2019
IJDVL Awards 2020
IJDVL International Awards 2018
Images in Clinical Practice
In Memorium
Inaugural Address
Knowledge From World Contemporaries
Leprosy Section
Letter in Response to Previous Publication
Letter to Editor
Letter to the Editor
Letter to the Editor - Case Letter
Letter to the Editor - Letter in Response to Published Article
Letter to the Editor - Observation Letter
Letter to the Editor - Study Letter
Letter to the Editor - Therapy Letter
Letter to the Editor: Articles in Response to Previously Published Articles
Letters in Response to Previous Publication
Letters to the Editor
Letters to the Editor - Letter in Response to Previously Published Articles
Letters to the Editor: Case Letters
Letters to the Editor: Letters in Response to Previously Published Articles
Medicolegal Window
Miscellaneous Letter
Net Case
Net case report
Net Image
Net Letter
Net Quiz
Net Study
New Preparations
News & Views
Observation Letter
Observation Letters
Original Article
Original Contributions
Pattern of Skin Diseases
Pediatric Dermatology
Pediatric Rounds
Presedential Address
Presidential Address
Presidents Remarks
Report of chief editor
Report of Hon : Treasurer IADVL
Report of Hon. General Secretary IADVL
Research Methdology
Research Methodology
Resident page
Resident's Page
Resident’s Page
Residents' Corner
Residents' Corner
Residents' Page
Review Article
Review Articles
Revision Corner
Self Assessment Programme
Seminar: Chronic Arsenicosis in India
Seminar: HIV Infection
Short Communication
Short Communications
Short Report
Special Article
Specialty Interface
Study Letter
Study Letters
Symposium - Contact Dermatitis
Symposium - Lasers
Symposium - Pediatric Dermatoses
Symposium - Psoriasis
Symposium - Vesicobullous Disorders
Symposium Aesthetic Surgery
Symposium Dermatopathology
Symposium-Hair Disorders
Symposium-Nails Part I
Symposium-Nails-Part II
Systematic Review and Meta-Analysis
Systematic Reviews and Meta-analyses
Systematic Reviews and Meta-analysis
Therapeutic Guideline-IADVL
Therapeutic Guidelines
Therapeutic Guidelines - IADVL
Therapy Letter
Therapy Letters
View Point
What’s new in Dermatology
View/Download PDF

Translate this page into:

Case Report
doi: 10.4103/0378-6323.39701
PMID: 18388376

Nasal NK/T cell lymphoma presenting as a lethal midline granuloma

Vandana Mehta1 , C Balachandran1 , Sudha Bhat2 , V Geetha2 , Donald Fernandes3
1 Department of Skin and STD, Kasturba Medical College, Manipal, India
2 Department of Pathology, Kasturba Medical College, Manipal, India
3 Department of Radiotherapy, Kasturba Medical College, Manipal, India

Correspondence Address:
Vandana Mehta
Department of Skin and STD, Kasturba Medical College, Manipal - 576 104, Karnataka
How to cite this article:
Mehta V, Balachandran C, Bhat S, Geetha V, Fernandes D. Nasal NK/T cell lymphoma presenting as a lethal midline granuloma. Indian J Dermatol Venereol Leprol 2008;74:145-147
Copyright: (C)2008 Indian Journal of Dermatology, Venereology, and Leprology


Nasal NK/T cell lymphomas are aggressive, locally destructive, midfacial, necrotizing lesions. The nonspecific clinical symptoms constitute a major stumbling block in the early diagnosis and management of these lymphomas. We report here a case of probable nasal NK/T cell lymphoma in an apparently healthy male that progressed rapidly in a short span of time and was managed subsequently with chemotherapy and external beam irradiation with which the lesion regressed.
Keywords: Lymphoma, Midline granuloma, Nasal
Figure 3: Dense atypical lymphocytic infiltrate in the dermis, (H and E, 400)
Figure 3: Dense atypical lymphocytic infiltrate in the dermis, (H and E, 400)
Figure 2: Marked progression of the plaque on the face
Figure 2: Marked progression of the plaque on the face
Figure 1: Erythematous, infiltrated plaque on the face
Figure 1: Erythematous, infiltrated plaque on the face


Lethal midline granuloma also known as ′Stewart′s granuloma′ or ′polymorphic reticulosis′ refers to the presence and sequelae of a destructive lesion in the upper respiratory tract. According to literature, the majority of lethal midline granulomas are nasal NK/T cell lymphomas. [1] Nasal NK/T cell lymphomas are aggressive, locally destructive, midfacial, necrotizing lesions. Most of them were initially diagnosed as lethal midline granuloma, a term that is slowly being phased out. The nonspecific clinical symptoms constitute a major stumbling block in the early diagnosis and management of these lymphomas. Thus, we need to be aware of the uncommon presentations of this lymphoma. We report here a case of nasal NK/T cell lymphoma in an apparently healthy male that progressed rapidly in a short span of time.

Case Report

A 74 year-old healthy male presented with eight month-old complaints of erythema and swelling involving the nose and upper lip. Erythema started insidiously over the inner canthus of the right eye, which slowly progressed to involve the nose, upper lip and malar area and was later associated with swelling. There was no history of nasal stuffiness; however, the patient complained of blood-stained nasal discharge. There was no history of photosensitivity, hypopigmented, hypnoanesthetic skin lesions, sensory or motor weakness or any systemic symptoms such as fever and night sweats. Cutaneous examination revealed diffuse erythema on the nose, malar area and upper lip extending up to the eyes [Figure - 1]. Phymatoid enlargement of the nose was seen with bilaterally widened nasal cavities. A single oral ulcer with mild slough was also present on the upper labial mucosa. There was no lymphadenopathy.

All the routine hematological and biochemical investigations such as hemogram, urinalysis, fasting and postmeal blood sugar, liver function tests, renal function tests were normal except for a raised ESR and absolute eosinophil count. HIV-ELISA and HBs-Ag tests were negative. Nasal endoscopic smears for fungi and mycobacteria were negative. A CT scan of the osteomeatal complex and X-ray of paranasal sinuses showed only a mild haziness in the maxillary sinuses. Skin biopsy was inconclusive with only numerous proliferating capillaries dissecting the collagen bundles with a perivascular lymphoplasmacytic infiltrate. Tissue biopsy for fungal and AFB cultures was negative. Due to the increase in absolute eosinophil counts and ESR, the patient was discharged after being prescribed a three-week course of antibiotics and diethylcarbamazine. One month later, the patient presented with a sudden onset of a nodular, erythematous, vascular lesion completely occluding the left nostril with edema and erythema of the right upper lip [Figure - 2].

A repeat biopsy at this stage showed a mixed cellular infiltrate of eosinophils, lymphocytes, plasma cells and histiocytes. A diffuse dense infiltrate of atypical small and large lymphocytes with pleomorphism was present in the dermis [Figure - 3] and subcutis extending between the collagen bundles in rows, columns and cords. The abnormal cells were also seen infiltrating the skeletal muscle. Immunohistochemistry (IHC) was positive for T cell markers, i.e. , CD3 and CD45RO but was negative for a B cell marker, i.e. , CD20. IHC for an NK cell marker, i.e. , CD56 was unavailable; in spite of this, the biological behaviour of NK-positive and T-positive nasal extranodal lymphomas is similar and hence, collectively termed as NK/T cell lymphomas. Thus, based on the clinical picture and histopathology, we came to a presumptive diagnosis of extranodal nasal NK/T cell lymphoma as the IHC for an NK cell marker (CD56) was not performed. Accordingly, our patient was referred to oncology for the initiation of field radiotherapy and chemotherapy where he was given injection methotrexate 50 mg I.V. for 12 weeks along with external beam irradiation (total 34 Gy units). During his three months′ stay in the hospital. there was considerable improvement in the nasal and facial lesions.


Mature or peripheral NK/T cell lymphomas account for only 10-15% of non-Hodgkin′s lymphomas. Amongst them, the most common and well-characterized ones are the ′nasal′ and ′nasal type′ NK/T cell lymphomas. They are currently referred to as angiocentric lymphomas in the Revised European American Lymphoma (REAL) classification or as nasal NK/T cell lymphoma by the World Health Organization-European Organization for Research and Treatment of Cancer (WHO-EORTC) classification. [2] Their characteristic histological feature is an angiocentric/angiodestructive growth pattern with zonal necrosis. Both nasal and nasal type NK/T cell lymphomas reflect specific characteristics of NK cells. [3] NK cells are cytolytic cells which are active against tumor cells and cells infected with bacteria and viruses without prior sensitization. They develop in the bone marrow from a bipotential progenitor capable of differentiation into T and NK cells. Phenotypically, NK cells appear commonly as small lymphocytes with azurophilic granules and immunophenotypically, they express the characteristic marker CD56. Due to a common ontogeny with T cells, NK cells also express some T cell markers. Nasal NK/T cell lymphomas have a predilection for the nasal cavity and the upper aerodigestive tract although the skin and soft tissues may be involved. They are rare and are mainly seen in Asia and Latin America. Elderly males in the age group of fifty years and above are affected. The most common presentation is a chronic nasal obstruction or a purulent rhinorrhea. [4] Systemic symptoms such as fever and weight loss are absent or are observed only in advanced cases. The skin is the most common site of involvement after the nasal cavity or the nasopharynx and skin involvement may be a primary or secondary manifestation of the disease. Approximately 10-20% of the nasal lymphomas have skin involvement. [5],[6] ′Nasal type′ lymphomas show the same histological features as nasal NK/T cell lymphomas but arise from extranasal sites such as the skin, gastrointestinal tract, testis, kidney, eyes and the orbit. They occur more commonly in females. Epstein Barr virus RNA is present in 80-100% of nasal NK/T cell lymphoma cases and less often (15-40%) in nasal type NK/Tcell lymphoma cases. [7] Our patient also presented with a long history of erythematous skin lesions and blood-stained nasal discharge. IHC in this case confirmed a probable NK/T cell lineage of the tumor by demonstrating presence of CD3 and CD45RO markers and the absence of CD20 marker. Zhong et al. reported that most upper aerodigestive tract NK/T cell lymphomas were genotypically of NK cell origin and only a few belonged to T cell lineage. [7]

Nasal NK/T cell lymphoma follows an aggressive and rapid downhill course in Asians with death occurring due to relapse or systemic spread in 50% of the cases. [8] Multidrug chemotherapy followed by involved field radiotherapy appears to be the most effective treatment approach.

Mendenhall WM, Olivier KR, Lynch JW, Mendenhall NP. Lehtal midline granuloma-nasal natural killer/T cell lymphoma. Am J Clin Oncol 2006;29:202-6.
[Google Scholar]
Jaffe ES. Clasification of natural killer (NK) cell and NK like T cell malignancies. Blood 1996;87;1207-10.
[Google Scholar]
Kato N, Yasukawa K, Onozuka T, Kikuta H. Nasal and nasal type T/NK cell lymphoma with cutaneous involvement. J Am Acad Dermatol 1999;40;850-6.
[Google Scholar]
Gourin CG, Johnson JT, Selvaggi K. Nasal T cell lymphoma: Case report and review of diagnostic features. Ear Nose Throat J 2001;80:458-60.
[Google Scholar]
Reddy RR, Singh G, Prathima KJ, Harendra Kumar ML. CD3 positive extranodal T cell lymphoma of nasal type with skin involvement. Indian J Dermatol Venereol Leprol 2006;72:215-7.
[Google Scholar]
Patel V, Mahajan S, Kharkar V, Khopkar U. Nasal extranodal NK/T cell lymphoma presenting as a perforated palatal ulcer: A diagnostic challenge. Indian J Dermatol Venereol Leprol 2006;72:218-21.
[Google Scholar]
Zhong BN, Zhang XH, Li M. Study of the pathology, immunophenotype, etiology and genetic markers of NK/T cell lymphoma. Zhonghua Xue Ye Xue Za Zhi 2003;24:505-9.
[Google Scholar]
Cleary KR, Batsakis JG. Sinonasal lymphomas. Ann Otol Rhinol Laryngol 1994;103:911-4.
[Google Scholar]

Fulltext Views

PDF downloads
Show Sections