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Case report

Nasal NK/T-cell lymphoma: A tragic case

L. Taali ∗ , M. Abou-Elfadl , M. Fassih , M. Mahtar
Service d’ORL et de Chirurgie Cervico-faciale, Hôpital 20-Août-1953, Casablanca, Morocco

a r t i c l e i n f o a b s t r a c t

Keywords: Introduction: Nasal NK/T-cell lymphoma is a rare clinicopathological entity, formerly called midline lethal
NK/T-cell lymphoma granuloma. Following progress in histology and the routine use of immunohistochemistry, nasal NK/T-cell
Rapid progression lymphoma was recognized as a distinct entity by WHO in 2001.
Delayed diagnosis
Case report: The authors report the case of a 22-year-old, insulin-dependent diabetic woman, who pre-
Poor prognosis
sented with mid-facial inflammatory swelling following facial trauma, initially diagnosed and treated as
cellulitis of the face. The subsequent course was rapidly progressive and fatal, with the development of
midline destructive disease. Histological examinations concluded on NK/T-cell lymphoma.
Discussion: The various differential diagnoses of NK/T-cell lymphoma include gangrenous cellulitis, inva-
sive mycotic rhinosinusitis, Wegener’s granulomatosis, actinomycosis, and facial T-cell lymphoma. The
clinical presentation of this case was atypical, resulting in delayed diagnosis and treatment. Treatment
is based on radiotherapy and chemotherapy, but the prognosis remains very poor even when treatment
is rapidly initiated.
© 2016 Published by Elsevier Masson SAS.

1. Introduction

Nasal NK/T-cell lymphoma is a rare clinical entity, character-

ized by rapidly progressive midline destructive disease with no
tendency to healing. The pathogenesis of this disease remains
unknown, but various hypotheses include an association with
Epstein-Barr virus (EBV) infection. The prognosis of nasal NK/T-cell
lymphoma remains very poor despite treatment.
In the light of a case of a nasal NK/T-cell lymphoma in a 22-year-
old woman, we discuss the clinical characteristics, clinical course,
modalities of diagnosis and treatment and prognosis.

2. Case report

A 22-year-old woman with insulin-dependent diabetes pre-

sented with midline facial inflammatory swelling following facial
trauma with a nasal site of impact (aggression), initially diagnosed
and treated as cellulitis of the face by parenteral triple combination
antibiotic therapy comprising amoxicillin-clavulanic acid 3 g/day,
metronidazole 1.5 g/day and gentamicin 160 mg/day.
She presented a rapidly progressive course with the appearance,
48 hours after admission, of midline destructive disease involving
∗ Corresponding author at: Boîte postale 9619 Moulay Rchid, Casablanca,
Morocco. Tel.: +212642492699.
E-mail address: dr.taali.@gmail.com (L. Taali).
Fig. 1. Intraoperative appearance of debridement of necrotic tissues.
the upper lip, nostrils, and nasal septum, with impaired conscious-
ness and poor glycaemic control. Debridement of necrotic tissues
was performed (Fig. 1) with histopathological, bacteriological, and
mycological examination. Centrifugal extension of the necrosis
was observed with subsequent involvement of the cheeks, fore-
head, palate, and nasal pyramid. No cervical lymphadenopathy,
respiratory disorders, or haematuria were observed on clinical
examination.

http://dx.doi.org/10.1016/j.anorl.2016.08.006
1879-7296/© 2016 Published by Elsevier Masson SAS.

Please cite this article in press as: Taali L, et al. Nasal NK/T-cell lymphoma: A tragic case. European Annals of Otorhinolaryngology, Head
and Neck diseases (2016), http://dx.doi.org/10.1016/j.anorl.2016.08.006
G Model
ANORL-592; No. of Pages 2 ARTICLE IN PRESS
2 L. Taali et al. / European Annals of Otorhinolaryngology, Head and Neck diseases xxx (2016) xxx–xxx
The laboratory work-up revealed normal C-reactive pro-
tein and erythrocyte sedimentation rate, mild leukocytosis with
11,500/mm3 white blood cells, severe hypokalaemia (potassium:
1.5 mmol/L) and hyperglycaemia (glucose: 3.6 g/L).
Several diagnoses were proposed at this stage: gangrenous cel-
lulitis, invasive mycotic rhinosinusitis, Wegener’s granulomatosis,
actinomycosis, or facial T-cell lymphoma. Three days after admis-
sion, the patient died in a context of septicaemia and multi-organ
failure.
The final histological examination concluded on aggressive
lymphoma (diffuse, atypical perivascular lymphoid infiltrate with
images of angiocentrism and angio-invasion, presence of large
areas of ischaemic necrosis) and immunohistochemistry concluded
on CD2+, CD3+, CD56+, and EBV+ nasal NK/T-cell lymphoma.
3. Discussion
Nasal NK/T-cell lymphoma is a rare clinical entity with a rapidly
fatal outcome, constituting a diagnostic and therapeutic emer-
gency. First described by Stewart in 1933 [1,2], nasal NK/T-cell
lymphoma is characterized by necrosis starting in the nasal cavity
with centrifugal extension to mid-facial bones.
The nasal cavities and sinuses are the anatomical sites most
commonly involved (70%), but nasal lymphoma can also arise in
Waldeyer’s ring (38%), the oral cavity (14%), larynx, hypopharynx
(10%), mandible or cheek [2].
NK/T-cell lymphoma can occur at any age, but the mean age at
diagnosis ranges between 40 and 50 years with a sex ratio of 2 to 4.5
depending on the series [2,3]. It is associated with a marked geo-
graphical and ethnic predilection, essentially affecting Asian and
Latin American populations [1].
The pathogenesis remains unknown, but a genetic predisposi-
tion has been reported (p53 and c-kit gene mutations) [2] as well
as an association with EBV infection [1–3].
At the onset of the disease, the patient presents nonspecific nasal
symptoms (blocked nose, rhinorrhoea, epistaxis) and endonasal
examination may reveal necrotic ulcers or perforated septum. At
the full-blown phase, the nasal pyramid presents signs of destruc-
tion evolving towards total necrosis with ulceration essentially
involving the nasal septum and inferior turbinate, but which can
also involve all adjacent bones and cartilages [1]. Regional lym-
phadenopathy is described in 15% to 25% of cases [1–3].
Systemic signs such as fever, weight loss, night sweats and
anaemia are rarely reported and are due to secretion of cytokines
by tumour cells.
A discordance is observed between the extent of tissue destruc-
tion and inconstant laboratory signs of inflammation [1].
CT scan and magnetic resonance imaging can visualize the
tumour, its extension to adjacent structures, the extent of bone
destruction, and can distinguish between neoplastic and inflam-
matory lesions, facilitating the differential diagnosis between
neoplastic disease and inflammatory or infectious disease and are
also useful for pretreatment and post-treatment evaluation.
Histopathological examination of the resection specimen con-
firms the diagnosis by demonstrating lymphoid cells associated
Please cite this article in press as: Taali L, et al. Nasal NK/T-cell lymphoma: A tragic case. European Annals of Otorhinolaryngology, Head
and Neck diseases (2016), http://dx.doi.org/10.1016/j.anorl.2016.08.006
with plasma cells, polymorphonuclear neutrophils and histiocytes
presenting an angiocentric pattern, as well as areas of coagula-
tion necrosis, with no signs of vasculitis. Immunohistochemistry
specifies the T-cell lymphocyte phenotype and specific markers of
natural killer (NK) cells: CD2+, CD56+, with or without EBV+.
Treatment is difficult and complex and depends on the stage of
the disease. Some authors consider that surgery is ineffective and
may even lead to deterioration of the lesions by inducing rapid pro-
gression of the disease [2]. Some teams use anthracycline-based
combination chemotherapy followed by external beam radiothe-
rapy for patients under the age of 60 years and chemotherapy
without anthracycline in older patients [3,4]. Other authors recom-
mend exclusive radiotherapy for less advanced stages with a dose
of 52 Gray, which allows complete remission in 40 to 80% of cases
with survival rates ranging between 40% and 59% [2]. Radiothe-
rapy after failure of chemotherapy may improve the prognosis [5].
Immunotherapy could be a treatment of the future. Studies on p53
expression are currently underway to elucidate the carcinogenesis
of nasal NK/T-cell lymphoma and to develop therapeutic and prog-
nostic approaches to these aggressive lymphomas. Interferon has
not been shown to be effective [2,4].
NK/T-cell lymphoma has a poor prognosis, with a 5-year overall
survival rate between 10 and 45% depending on the series [2].
There are very few published cases of sudden onset of inflam-
matory swelling of the face, subsequently resulting in rapidly
progressive centrifugal necrosis involving all of the mid-facial soft
tissues. Our patient died within one week. The nasal trauma occur-
ring in a clinical context of immunodepression caused by diabetes
may have played a role in the fulminant clinical presentation and
rapid progression of the disease.
4. Conclusion
This case report illustrates the fact that mid-facial inflamma-
tory and necrotic disease is often incorrectly diagnosed as infection.
Nasal NK/T-cell lymphoma is characterized by polymorphic clin-
ical features, and diagnostic difficulties due to the rapid onset
and progression of the disease. Only histopathological examination
completed by immunohistochemistry can establish the diagnosis.
Disclosure of interest
The authors declare that they have no competing interest.
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