Global Journal of

Otolaryngology
ISSN 2474-7556

Case Report Glob J Otolaryngol

Volume 14 Issue 4 - April 2018
Copyright © All rights are reserved by Cristina Otilia Laza
DOI: 10.19080/GJO.2018.14.555893

Lethal Midline Granuloma-Stewart Nasal

Nk/T-Cell Lymphoma-0ur Experience
Cristina Otilia Laza*, Cristian Afternie and Dan Mihail
ENT Department, Scju Sf Apostol Andrei Constanta, Romania
Submission: March 06, 2018; Published: April 10, 2018
*Corresponding author: Cristina Otilia Laza, ENT Department, Scju Sf Apostol Andrei Constanta, Romania, Email:

Abstract

Lethal midline granuloma or Stewart granuloma now recognized as a Naso facial NK/T-cell lymphoma is a rare clinical entity characterized
by destruction and mutilation of the naso facial area. It has an unknown etiology, often affect face, nose, para-nasal sinuses, palate, oral cavity
and surrounding structures. Because clinical manifestations are often variable and non specific, and the majority of the clinicians are not aware
of such a disease this diagnosis is not simple, plus most of the patient are diagnose and treat wrong by a number of doctor loosing time. Plus
till the correct diagnosis. Wait for the results of biopsy, IHC, is a long way which lead to a delay in proper treatment the purpose of our article is
to present 2 cases of lymphoma TK/NK with in a period of 15 years between them with the difference in possibilities of diagnosis, treatment,
etiology, prognosis. Every case was a real challenge I can say a real battle to establish the final diagnosis and to collaborate with someone with
experience in the treatment at this horrible disease. Because is very rare (my professor never encounter a patient with destructive midline
granuloma in 40 years of work), a lot, s of doctors with different specialities ask for my opinion, because of my “large experience”. These require
not only clinical feeling but a lot of informations, which means intense study.
Keywords: Lethal midline granuloma; Lymphoma; Tk/Nk; Mutilation; Protocol of treatment

Introduction as nasal type extra-nodal NK/T-cell lymphoma. It is common in

Midline destructive lesions of the face were first described in
Asian origin whereas rare in western population. It is difficult
1897, and later a variety of terms were proposed. Angiocentric
to diagnose because of the wide array of similar pathology and
lymphoma, malignant granuloma, malignant midline reticulosis,
nonspecific symptoms. It occurs around the 4th decade and
and polymorphic reticulosis. Previously known as lethal midline
male to female ratio is 8:1 to 2:1. Common sites for sinonasal
granuloma or STEWART granuloma, was first introduced in the
NK/T-cell lymphomas are: nasal cavities the maxillary sinus,
large category of granulomatous diseases like Wegener, because
nasopharynx, or pharynx, tonsils palate, oral cavity, and hypo
of the histopathology-chronic inflammatory granulomatous
pharynx Figure 1.
destructive process, but a lots of chronic infections like
Tuberculosis, Leprosis, Mucormycosis, and tumors have a
similar aspect, but the treatment implicate chimio, radiotherapy
after all other possibilities were excluded. This tumor is
responsible for the classical destruction of the mid face. In the
past, death resulted from inter-current infection of disseminated
lymphoma due to a failure of diagnosis or insufficiently.
Aggressive oncologic treatment. Recently, similar appearances
of aggressive mid facial destruction caused by Cocaine abuse
have been reported in which tests for granulomatous diseases
and lymphoma have been negative. Todays mid facial necrotizing
disease is a naso facial natural killer (NK)/T-cell lymphoma, Figure 1: Coronal (left) and axial (right) CT scans of the sinus
characterized by granulomatous lesion with destruction of the reveal severe pansinusitis with abnormal nasopharyngeal
thickening, right facial edema and right temporal bone
upper aero digestive tract. Naso facial definitively categorized in
pacification.
the World Health Organization lymphoma classification system

Glob J Otolaryngol 14(4): GJO.MS.ID.555893 (2018) 001

 Global Journal of Otolaryngology

Clinical manifestations vary according to the site being h) Primary cutaneous anaplastic large cell lymphoma
affected. The common clinical findings to all such lesions are the
i) Peripheral T-cell lymphoma
progression of an ulceration or vegetative process to destruction
of naso facial region, resulting in functional and cosmetic j) Angioimmunoblastic T-cell lymphoma
deformity. Natural killer (NK) cell lymphoma is a in an extra nodal
k) Anaplastic large-cell lymphoma
non Hodgkin lymphoma most of them (90%) are of B cell origin.
In the past, without immune-hystochemistry it was impossible T-cell proliferations of uncertain malignant potential include
to describe just using morphologic parameters, without cell the following:
markers what type of non Hodgkin lymphoma is Advances in
a) Lymphomatoid papulosis
tumor cell biology lead to a better classification of lymphomas
(WHO). Controversy still exists if NK-cell lymphoma represents b) Hodgkin lymphoma
the presence of a true NK cell or merely the presence of a T cell c) Histiocytic and dendritic cell neoplasms
with abnormal cell markers is under debate but identification of
more specific cell markers of NK and T cells will likely resolve d) Mastocytosis
this controversy in the future [1-5].
Pathophysiology
World Health Organization classification of lymphomas Extranodal NKTCL outside the lymph nodes manifests in
a) Precursor B-lymphoblastic leukemia/lymphoma the nasal cavity. Patients with this type tend to have earlier
disease (stage I) and is associated with Epstein-Barr virus
b) Chronic lymphocytic leukemia/small lymphocytic (EBV), irrespective of the ethnicity of the patient, but the
lymphoma exact mechanism of malignant transformation via EBV is not
c) Lymphoplasmacytic lymphoma clear till now. Extranodal nasal type NKTCL demonstrates a
predilection for the nasopharynx, palate, skin, soft tissues,
d) Plasma cell myeloma orbit, gastrointestinal (GI) tract, and testes. Secondary lymph
e) Extraosseous plasmacytoma nodes may be involved in some cases; a disseminated leukemia
is even possible. Lymphomas that manifest outside of the nose
f) Extranodal marginal zone B-cell lymphoma of mucosa- have a strong association with EBV in Asian patients, but not in
associated lymphoid tissue (MALT) whites. The pattern of involvement of the extranasal sites has
g) Follicular lymphoma been hypothesized to be related to the marker CD56-neural
cell adhesion molecule. The skin is the most common site of
h) Mantle cell lymphoma
dissemination in Etiology Figure 2.
i) Diffuse large B-cell lymphoma

j) Intravascular large B-cell lymphoma

B-cell proliferations of uncertain malignant potential include

the following:

a) Lymphomatoid granulomatosis

b) Posttransplant lymph proliferative disorder,

polymorphic

T-cell and NK-cell neoplasms include the following:

Figure 2: Coronal (left) and axial (right) CT scans of the sinus
a) Precursor T-lymphoblastic leukemia/lymphoma reveal severe pansinusitis with abnormal nasopharyngeal
thickening, right facial edema and right temporal bone
b) Blastic NK-cell lymphoma pacification.

c) Adult T-cell leukemia/lymphoma Risk factors

d) Extranodal NKTCL, nasal type a) Inherited immunodeficiencies Klinefelter, Chédiak

Higashi, Ataxia Telangiectasia, Wiskott-Aldrich syndrome, and
e) Subcutaneous panniculitislike T-cell lymphoma common variable immunodeficiency disease
f) Mycosis fungoides b) Acquired immunodeficiency diseases Iatrogenic-
g) Sézary syndrome corticoizi, post-transplant, HIV-1 infection, acquired
Hypogammaglobulinemia

How to cite this article: Cristina O L, Cristian A, D Mihail. Lethal Midline Granuloma-Stewart Nasal Nk/T-Cell Lymphoma-0ur Experience. Glob J Oto 2018;
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 Global Journal of Otolaryngology

c) Autoimmune disease Sjögreen syndrome, Celiac Sprue, History

Rheumatoid Arthritis, and Systemic Lupus Erythematosus
General manifestations of natural killer (NK)/T-cell
d) Chemical or drug exposures Phenytoin, Dioxin, lymphoma (NKTCL) include weight loss, fever, fatigue, night
Phenoxyl herbicides, irradiation, and prior chemotherapy or sweats, bone pain. This disorder is associated with aggressive
radiotherapy destruction of middle of face. This disease has classically been
divided into three stages:
e) Association with infectious agents EBV, human T-cell
leukemia/lymphoma virus-I, and ataxia Telangiectasia syndrome a) Prodromal Stage: Nasal Obstruction and Rhinorrhea.
May last few years.
NK-TC lymphoma is very rare in white populations. In
Western populations, the prevalence of nasal lymphomas is b) Active Stage: Areas of necrosis develop in and around
estimated at 0.17-1.5% of all non Hodgkin lymphoma, 45% of the nasal cavity associated with purulent nasal discharge,
which are thought to be NK-/T-cell in origin. Worldwide is a rare. crusting and tissue loss. There is also progressive destruction
Disease but it is much more prevalent in Asia, Central and South of the nasal framework, palate, upper lip extending in to the
America. Patients with NKTC-lymphoma commonly present in pharynx. Orbit and skull base also could be involved.
their sixth decade of life, but the disease has been seen in both
c) Terminal Stage: This stage is characterized by
geriatric and pediatric populations. Men are more commonly
severe mutilation of the face, exhaustion and eventual death.
affected with the disease than women, with a male-to-female
Systemic metastasis is also seen in this stage. Head and neck
ratio 3:1 [6-10].
manifestations include the following:
Prognosis
Symptoms facial pain and swelling
Compared with other subtypes of lymphoma found in
I. Ocular symptoms
the head and neck region, TK-NK lymphoma respond bad at
traditional chemo-radiotherapy so the mortality is much higher a) Diplopia
,only 6% of the patients respond complete at the treatment, 45%
b) Decreased visual acuity
survive after 2 years, the rest survive maximum 12,5 month.
Presence of a multidrug resistance (P-glycoprotein–positive) c) Orbital swelling
phenotype CD56 is thought to facilitate tumor cell dissemination
II. Nasosinusal
by virtue of its binding properties, and low rates of survival.
NKTC lymphoma recurs locally much more frequent (31.4%) a) Nasal obstruction
than do either lymphomas presenting in the nasopharynx. The
b) Purulent nasal discharge
recurrences in the cervical nodes are very low (2.4%) compared
with B-cell lymphomas (14.3%). Hemophagocytic syndrome- c) Epistaxis
fever, pancytopenia, hemophagocytic Histiocytic in the bone
d) Fetid crusts
marrow, and rapid liver function deterioration, are much
more common in NKTCLs. EBC virus is consider implicated in e) Cacosmia
etiology so circulating EBV-DNA levels could serve as a valuable
f) Anemia
biomarker of tumor load for accurate classification of early-
stage disease Figure 3. III. Otologic

a) Impaired hearing

b) Otalgia

IV. Oropharyngeal

a) Odynophagia,

b) Dysphasia,

c) Velopharyngeal incompetence

d) Trismus

e) Halitosis

V. Laryngeal
Figure 3: CT scan axial view destructive process of the nasal
pyramid with destruction of the nasal septum and turbinates. a) Dyspnea

How to cite this article: Cristina O L, Cristian A, D Mihail. Lethal Midline Granuloma-Stewart Nasal Nk/T-Cell Lymphoma-0ur Experience. Glob J Oto 2018;
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14(4): 555893. DOI: 10.19080/GJO.2018.14.555893
 Global Journal of Otolaryngology

b) Hoarseness t) Ulcerations on the epiglottis

VI. Pulmonary manifestations u) Vocal cords Neck

a) Cough v) Adenopaty

b) Breathing restriction Pulmonary findings include the following:

VII. Gastrointestinal (GI) a) Bronchitis

a) pain GI findings include the following:

b) abdominal fullness a) Hepato splenomegaly

c) early satiety b) Abdominal mass

d) perforation c) A testicular mass

e) hemorrhage Differential Diagnosis

f) Testicular pain may also be reported, as may non Infection (bacterial, fungal, or parasitic), Tuberculosis
healing sores on the skin. lupus, Leprosy, Leishmaniosis, Mucormycosis Rhinoscleroma.
Inflammatory disease (Sarcoidosis, Wegener granulomatosis,
g) Physical Examination
Systemic Lupus Erythematosus, or Polyarteritis nodosa).
General findings on physical examination include the Neoplastic processes (squamous or bas cell carcinoma,
following: melanoma, esthesio neuroblastoma, adenoid cystic carcinoma,
adenocarcinoma, fibrosarcoma, rhabdomyosarcoma, plasma cell
a) Ulcerations involving the skin
myeloma, or an additional sinonasal lymphoma). Cocaine abuse,
b) Pallor Trauma (gunshots, bites )with saddle nose deformity, mutilation
giant cell granuloma, cholesterol granuloma and lobular
Head and neck findings include the following:
capillary hemangioma, Non Hodgkin Lymphomas polymorphic
a) Cranial neuropathies reticulosis Figure 4.

b) Trigeminal neuralgia

c) VI CN palsy

d) Diplopia

e) Ocular

f) Visual impairment

g) Orbital mass

h) Proptosis

i) Inspection

j) Facial swelling and erythema (midline facial features)

k) Deformation of the nasal pyramid

Figure 4: CT scan contrast pansinusitis, fetid purulent discharge
l) Anterior rhino’s copy/posterior rhinos copy necrosis, destruction of the inf turbinate nasal septum.

m) Septal perforation Laboratory Studies

n) Sinusitis a) Test for inflammation PCR, RF

o) Nasal mass Otoscopy b) Complete blood count (CBC) anemia or lymphocytopenia

p) Serous otitis media Bucopharyngoscopy c) Liver function tests, Elevated LDH levels have been
associated with poorer prognoses
q) Ulcerations of the palate
d) Renal function tests
r) Tonsil
e) Uric acid and calcium levels
s) Laryngoscopy

How to cite this article: Cristina O L, Cristian A, D Mihail. Lethal Midline Granuloma-Stewart Nasal Nk/T-Cell Lymphoma-0ur Experience. Glob J Oto 2018;
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 Global Journal of Otolaryngology

f) Epstein-Barr virus (EBV) titers and associated with glomerulonephritis. Antineutrophil

cytoplasmic antibodies (c-ANCA) are present in patients
g) panca, canca
with Wegener’s granulomatosis, which is useful in diagnosis.
h) RBW, VDRL Immuno phenotypical tests demonstrate characteristic cell
markers for NK-TK lymphoma CD2+, cytoplasmic CD3e+, and
i) IDR ,la PPD, Quanti FERON test, RX thorax
CD56+. Other markers are CD7, CD30, CD43, CD45RO, HLA-DR,
j) Bacteriology, mycology of the nasal discharge to interleukin (IL)-2 receptor, Lumbar puncture when intrathecal
exclude tuberculosis, mycosis chemotherapy is indicated. Bone marrow aspiration in case of
invasion with pancytopenia, hemophagocytic syndrome-fever,
k) r X lung
hemophagocytic Histiocytic. Genetic tests find EBV in most
l) CT scan cranium, neck is important for importance and tumor cells .The virus is present in a clonal episomal form.
extent of destructive process Various genetic abnormalities have been identified in patients
with NKTCLs, but no specific chromosomal translocations Figure
m) CT scan thorax, abdomen-metastasis Magnetic
5.
resonance imaging (MRI) of the head is performed in cases
of suspected skull base invasion intracranial extension. Staging Ann Arbor classification:

Imaging Studies a. Stage I: Involvement in a single lymph node region or

a) ENT complete exam single extra lymphatic site

b) Inspection b. Stage II: Involvement of 2 or more lymph node regions

on the same side of diaphragm, localized contiguous involvement
c) palpation of only one extra lymphatic site and lymph node region
d) face c. Stage III: Involvement of lymph node regions on both
e) neck sides of diaphragm; may include spleen

f) Rhinoscopy ant/posterior d. Stage IV: Disseminated involvement of one or more

extra lymphatic organs with or without lymph node involvement.
g) rigid endoscopy When added to any of these stage numbers, the letter E denotes
extra lymphatic sites, and the letter B indicates the presence of B
symptoms (i.e., fever, night sweats, or weight loss).

Treatment
Because NK/T-cell lymphoma of the head and neck is an
extremely rare malignancy, there is not in function a protocol
standard for the treatment but should be discuss in a mixed
team including planning \with hematologists, oncologists,
and radiation oncologists. Currently the most recommended
treatment includes CHOP (Cyclophosphamide, doxorubicin,
vincristine, prednisone) chemotherapy in conjunction with
Figure 5: Examination of the face edema, congestion of the left
radiotherapy. The combination of treatments has yielded 5-year
eye both eyelids ,edema of the conjunctiva , chemosis ,position
in the orbit ,mobility normal with the patient consent. survival rates ranging from 20% to 80%, unfortunately, with a
high rate of relapse rate, Also they have a very a high degree of
Flexible nasopharyngoscopy with direct laryngoscopy should resistance to standard therapy. For these patients, alternative
be performed to characterize the extent of the lesion. Biopsy of strategies have been investigated with some success. High-
the primary site-anatomo-morphology Biopsies showed diffuse dose chemotherapy with or without total-body irradiation,
infiltrates of pleomorphic large lymphoid cells and atypical followed by Autologous stem cell rescue, has been used for
small lymphoid cells with frequent mitotic figures, admixed patients with relapsing disease. More recently, new treatments
with a large number of inflammatory cells such as granulocytes, using the SMILE protocol (dexamethasone, methotrexate,
macrophages, and plasma cells. Extensive ischemic necrosis ifosfamide, l-asparaginase, and etoposide) show promising
was the common characteristic of these lesions. Angiocentric results but adverse effects, including significant myelotoxicity,
and/or angioinvasive infiltrates were also prominent However, suggest more research is needed to further develop this
histologic confirmation is indispensable. Angio destruction and promising protocol. Treatment regimen for early stage nasal
necrosis are characteristic for TK-NK lymphoma. It is important NKTL that included radiotherapy combined with infusion intra-
to distinguish LMG-NTL from Wegener’s granulomatosis which arterial, through the superficial temporal artery, of ifosfamide,
is characterized by necrotizing granulomas and vasculitis,

How to cite this article: Cristina O L, Cristian A, D Mihail. Lethal Midline Granuloma-Stewart Nasal Nk/T-Cell Lymphoma-0ur Experience. Glob J Oto 2018;
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14(4): 555893. DOI: 10.19080/GJO.2018.14.555893
 Global Journal of Otolaryngology

carboplatin, methotrexate, peplomycin, and etoposide. Surgical In the same time I order cultures for fungi like Aspergillus,
management of patients with NKTK-Lymphoma is limited Candidiasis, Mucor. Also I run a battery of blood test to appreciate
to biopsy, stabilization of the airway if necessary, debunking the general status of the patient others for the differential
of disease in case of hemorrhage-ligation of the ECA ,IMA diagnosis, in fact in that time it was an excluding diagnosis with.
,cauterization ,laser Reconstruction in case of positive stable Wegener granulomatosis=PANCA, CANCA, biopsy histopathology,
response Patients refractory to treatment or with high-risk can IHC Tuberculosis-RX thorax, ex BK direct, cultures, Protein
benefit from hematopoietic stem cell transplantation. Because of chain reaction, Quanti FERON test Tertiary syphilis guma
the relatively high mortality, low response rate, and high relapse RBW, VDRL , biopsy histopathology, IHC Leprosy biopsy,B.
rate after definitive treatment, patients should be regularly Hansae, cultures Rhinoscleroma Klebsiella rhinos cleromatis,
monitored by an otolaryngologist Figure 6 [11-20]. biopsy histopathology ,IHC Cancers biopsy histopathology,
IHC Mucormicosis cultures, biopsy histopathology, IHCCT,
MRI scans axial, coronal view revealed pansinusitis, necrosis
of the intersinuso nasal wall destruction of the inf turbinate
perforation of the nasal septum, the extension of the process
also endocranial extension. Finally we send the patient on TG
.MURES on Oncological Clinic , where my diagnosis was confirm
They report the case and it was the single patient with Lethal
Midline Granuloma in Europe. They use an aggressive protocol
of chemo-radiotherapy but without response .He return without
the nasal pyramid, with the hole in his mid face, with high fever,
fetid nasal discharge [21-25].

The evolution was negative with fast enlargement of the

hole, day by day, starting to involve the orbits than the skull
Figure 6: CT scan of paranasal sinus with coronal cut showing base. The patient accuses excruciated pain-trigeminal neuralgia,
destruction of dorsum of nose along with mucosal hypertrophy
in maxillary sinus and ethmoidal sinus. headache, vomiting and then starts to bleed from the large hole.
At that stage he was extremely aggressive and refuse help, trying
Case Report to isolate and refusing to eat .In the end he ask for my help so
In my career I had the chance to diagnose 2 patients with I admit him in our Clinic. First to stop the bleeding I try with
TK/NK lymphoma. sponges like Merocel, Tahocom, than classic with gauzes soaked
in an ointment with antibiotic but finally I ligate on that side
Case Nr 1
ECA. Medical treatment consist in MER, plasma, vitamin, sol.
My first patient was a young man, 16 years ago, in a period glucosis, amino acids, lipids, antialgics, antibiotics but when
when Lethal Midline Granuloma was considered a granulomatous hemorrhage starts on the other side, he refuse everything, he
disease like Wegener. At the first examination it was a young man wants to die at home. Much, more lately, 4 years ago I had the
30 years old pale, spreading a fetid smell around him. He accuses chance to diagnose a 67 year old lady with TK/NK lymphoma in
nasal obstruction nasal purulent discharge, crusts, epistaxis, and the left maxillary sinus. She was admitted in the clinic for nasal
headache, and fever, asthenia, lost in weight. All the symptoms left complete obstruction, purulent nasal discharge severe facial
start 7 month before and he describe me all the treatments and pain including the eye also inflammation, edema of eye lids,
surgical procedures suffered anterior. With the nasal pyramid fever, night sweets, asthenia. All the accuses starts 4 weeks ago,
still in place, his face was still symmetric but pain on pressure with progressive aggravation Examination of the face edema,
on both canine fossa and frontal area was severe. On anterior congestion of the left eye both eyelids, edema of the conjunctiva,
Rhinoscopy on the inferior and lateral walls I saw intense red chemosis, position in the orbit, mobility normal aspect of
granulations covered with fetid crusts, a very large ulceration anterior orbital cellulitis The pain was severe in the canine fossa,
on the nasal septum with complete absence of the cartilage and suborbital point and in the internal angle of the eye. Clinical
bone, just superior a rest of bone from perpendicular plate of the aspect was similar with a complicated maxilla ethmoidal chronic
ethmoid, green, without mucosa and periosteum. In rest pituitary suppurated sinusitis with tendencies for complications edema
mucosa presents inflammation, granulations and necrotic areas .congestion of the suborbital area with extension in to the orbit-
on the turbinate Thinking that only a very severe, rare disease, anterior cellulitis superficial. There was no lymphadenopathy.
can explain such an important destruction, I order direct, exam There was no history of nasal bleeding, post nasal drip or cough.
and cultures for aerobic, anaerobic germs also special mediums Routine blood test did not reveal any relevant findings, urine
of cultures for fungi. Mycobacterium species, tuberculosis, bovis, analysis, liver function tests, renal function test were normal
aviarum, Mycobacterium leprae, Klebsiella rhioscleromatis except raised ESR and PCR. CT scan studies with the same

How to cite this article: Cristina O L, Cristian A, D Mihail. Lethal Midline Granuloma-Stewart Nasal Nk/T-Cell Lymphoma-0ur Experience. Glob J Oto 2018;
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14(4): 555893. DOI: 10.19080/GJO.2018.14.555893
 Global Journal of Otolaryngology

aspect like a fungus ball “extensive sinusitis. The usual lab test the response to the treatment but unfortunately she died after
were normal so in emergency under general anesthesia- we first session of chemotherapy, because of severe neurotoxin
start with the surgical treatment a combination of FESS and complication [31-35].
an external procedure for the radical ethmoidectomy type de
Discussion
Lima using a trans maxillary approach type CALDWELLLUC.
We send to the lab secretions directly from the sinuses for i. Nasal NK/T cell lymphomas are aggressive, locally
bacteriology, mycology, BK direct, also the material collected at destructive, characterized by a granulomatous necrotic process
the anatomopathology the histopathological examination was arising in the nasal cavity or sinuses and extending to the
showing a diffuse Lymphomatoid infiltrates with angiocentric midfacial area with centrifugal destruction of the nasal bone.
and angiodestructive pattern chronic granulomatous nonspecific
ii. It was also called as polymorphic reticulosis, mid
inflammations. Extensive area of necrosis with infiltration of
line malignant reticulosis, Stewart’s granuloma and NK/T-cell
plasma cells, histiocytes and eosinophils are present along with
lymphoma.
atypical lymphocytes [26-30].
iii. Extranodal NK-T-cell lymphoma accounts for 5–10%
Even we use the best antibiotics, antifungal drugs iv,
of all non-Hodgkin lymphoma, with most cases reported in East
antialgics everyday ,we clean and wash out the cavity with saline
Asia (China, Korea, Hong Kong, and Japan) . The incidence of
and a local antibiotics ,she complains of more severe pain ,the
NK-T-cell lymphoma in the United States is around 0.036 per
soft tissues of the face continue to be even more inflamed and
10 000 people and it has higher prevalence in Hispanic whites
a small area of osteitis with a small hole appear in the palatine
and Asian/Pacific Islanders and lowest among blacks. but the
bone in the sinuses area All other destructive chronic processes
EBV infection rate in blacks is very high (92.2%) which may
were excluded, also no causes for immunosuppressant-post-
contribute to the high prevalence of NK/T-cell lymphoma.
transplant ,other autoimmune disorders LED ,WG,RP, no DZ,
hepatic or renal failure we exclude also a possible Mucormicosis. iv. The nonspecific clinical symptom is a major obstacle in
Because of the palatine hole we ask the opinion of a colleague from early diagnosis and management of this lesion.
oro maxillo facial department, but there were no odontogenic
v. Gross appearance of the lesion is usually looking like
causes our patient use a total prosthesis dentures superior and
necrotic granulomas with ulcerations and destruction of nose
inferior, gingiva, oral cavity were normal. Serology was positive
and sinuses with destruction of soft tissue, cartilage and bone.
for EBV IgG antibody but negative for EBV IgM antibody. Human
There is usually aggressive and lethal progression with rapid
T-lymph tropic virus I/II (HTLV) and HIV antibodies both were
destruction of nasofacial area. Nasal septal perforation with
negative. Pan CT did not reveal any other involved lymph nodes
mutilation of the surrounding tissues often occurs. The most
or organs, bone marrow aspiration was negative for lymphoma
common symptoms are nasal stuffiness with or without nasal
involvement or leukemia but showed phagocytosis of nucleated
discharge. Ulcerations at nasal cavity or oral cavity along with
cells by macrophages).
conjunctivitis may occur.
The single way was to ask for a specific immunohistochemistry
vi. The diagnosis of mid facial NK/T-cell lymphoma is
for TK, NK cells. Possible only in Bucharest. My colleagues
based on the histopathological picture, immunophenotype of
from that hospital were very optimistic because they inspect
the atypical cells and the analysis of T-cell receptor genes. The
the cavity with an endoscope and were clean and they believe
surface of the affected site is associated with crusting and necrotic
in an OMF examination for them it was necrosis induced by
tissue, so the diagnosis of NK/T-cell lymphoma is extremely
bisphosphonates. In reality our patient never uses in her life
difficult by taking only punch biopsy, excisional biopsy or deep
such a medication. Finally after long discussions they agreed
biopsy is often essential for the diagnosis of this disease. The
with the Immunohistochemistry (IHC) study showed abnormal
characteristic histopathological picture in NK/T cell lymphoma
lymphocytes of CD56, CD3.so we finally diagnose an lymphoma
shows angiocentric and angiodestructive growth pattern with
TK/NK stage I-EB no lymphadenopathy, no bone marrow, and
zonal necrosis. Immunohistochemical study shows positive
other organ involvement. The patient was referred immediately
CD3, CD43, CD45RO, CD20 and CD57 demonstrates the a typical
to IOB Institute Oncologic Bucharest ‘’Profesor doctor Alexandru
lymphoid cells have T-cell phenotype. Radiological findings in
Trestioreanu ‘’for further treatment and she was proposed to
CT scan and MRI are not distinctive for other malignant lesions,
receive a multidrug chemotherapy of four cycle’s protocol (CHOP
typically showing irregular margins, bone destruction and
regimen).followed by external beam irradiation (36Gy). During
heterogenous contrast enhancement.
chemotherapy, her general status continues to deteriorate,
because mielotoxicity with neutropenia with spiking fever. vii. Nasal NK/T cell lymphoma is an aggressive lesion with
She start a complicated treatment that include antibiotics= rapid evolution and high mortality if not treated timely. The high
meropenem and vancomycin, antifungal=fluconazole iv.and an mortality is due to septicemia, invasion into blood vessels or into
antiviral Vallacyclovire drugs to cov I was very curious about brain leading to abscess formation.

How to cite this article: Cristina O L, Cristian A, D Mihail. Lethal Midline Granuloma-Stewart Nasal Nk/T-Cell Lymphoma-0ur Experience. Glob J Oto 2018;
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14(4): 555893. DOI: 10.19080/GJO.2018.14.555893
 Global Journal of Otolaryngology

viii. Treatment of sinonasal NK/T-Cell lymphoma is 5. Lan SK, Lin CW, Ho HC, Lee MS, Tzeng JE, et al. (2008) Penile metastasis
combined chemotherapy in different protocols ex. CHOP or secondary to nasal NK/T-cell lymphoma. Urology 72(5): 1014-1015.

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7. Jaffe ES, Chan JK, Su IJ, Frizzera G, Mori S, et al. Report of the workshop
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Surg Pathol 20(1): 103-111.
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How to cite this article: Cristina O L, Cristian A, D Mihail. Lethal Midline Granuloma-Stewart Nasal Nk/T-Cell Lymphoma-0ur Experience. Glob J Oto 2018;
009
14(4): 555893. DOI: 10.19080/GJO.2018.14.555893