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Wegener's Granulomatosis Presenting as
Nasal Polyp
Amn Gupta, Subhash Bhardwaj
granulomatosis (\VG) is characterised by aseptic granulomatous innammatioll and
",,,ilili, \\ hich classically affects kidneys, upper and lower respiratory tract Although majority of
\Jtu.:nls present \\ ith symptoms of head and neck region, rarely this region is the sole site of
lohclllcnL The interpretation of biopsy for the diagnosis of this disease needs precision, We report
,"" \\ho presented clin ically as nasal polyp, However. the microscopy confinned the diagnosis of
granuloma. Vascul it is, Midline granuloma.
.,. Granulomatosis (WG) is a rare disease,
is not known (I), Most of the
'Me ,b,)le "0 years of age and mostly present
of upper and lower respiratory tract
organs involved are eyes, ear and
salivary glands, but the incidence is
I), The characteristic microscopic features
It'lle necrotizing granulomas of upper and
Il'" respiratory tracL
Ih-olnecrotizing vascul itis of medium and small
bldisease in the form of focal and diffuse
IL !ingglomerulonephritis,
Some patients of WG who do nOl manifest the ful/
triad are labelled as "Limited" WG (1), Aboul 15% of
the patients show involvement of respiratory tract only
and present with rhinorrhea, bloody or purulent nasal
discharge, epistaxis. anosmia, mucosal ulcers and sinus
pain, Very rarely, swelling or mass is observed (2),
Case Report
A ten years old boy presented with epistaxis, pain ana
mild swelling left side of nose of one month duration,
There was also history of foul smelling yell?wish
discharge with difficulty in breathing with no significant
past history, General physical examination was
unremarkable, Per speculum examination revealed a
_lvIII.en! of Pathology, Govt. Medical College, Jammu-ISO 001 (J&K) India,
""rlu': Dr Arun Gupta. Demonstrator. Department of Pathology. Govt. Medical College. Jammu-180 00 i (J&K) Indi'l.
l> no 1999
____________, ~ . d K SCIENCE
polypoidal ulcerated mass in posterior part of left nostril
extcnding to nasopharynx. It was covered with whitish
A provisional diagnosis ofchronic inflammatory polyp
"as made "ith a differential diagnosis of
rhabdomyosarcoma. Multiple biopsies were taken.
X-I'ay chest, rOlltine urine analysis, renal function
tests and ultrasound of abdomen were within normal
The CT scan of the nasopharynx and nasal cavity
,Illmcd a mass in left nasal cavity destroying the medial
and anterolateral wall of left maxillary sinus and antrum.
'I he pterygoid plate was intact. The mass was seen
c\tending to left cheek (Fig. I).
Fig. I. CT SClin sho\\ing mass in left nasal cavity e).tending
into Idt check.
Pathological Findings
The microscopic examination revealed fragments lined
partly by respiratory epithelium with underlying tissue
exhibiting focal areas of fibrinoid necrosis as well as
necrosis of vessel walls (Fig. 2, 3). Granulomatous
inflaminh!ion with giant cells was seen around many
vessel; (fig. 4). There was no evidence of malignancy.
No atypical lymphoid cells were seen. Other granuloma
producing conditions were excluded by special stains.
Fig. 2. I.uw poner view showing tiny
necrosis (100),).
Fig. 4. High power view showing granulomatous destructiol
of vessel wall with giant cell reaction (400x).
Vol. I No.2, April-June I ~
aN _
/It], aM nJJI))JnI:
(non-healing) granuloma. Head Neck Surg 1979;
I : 213-222.
estimating the activity of disease. Major antibody is
directed against proteinase-3, a neutrophilic serine
protease which can be seen on indirect
immunoflourescence in the cytoplasm of neutrophils.
ANCA is positive in 67% patients with active limited
disease and approaches 96% if disease is generalized.
The test is helpful is differentiating other conditions
mimicking WG clinically (2, 5).
1993;24: 160-t66.
Fien Berg R, Mark El, Goodman Mel. 01. Correlation of
ANCA with extrarenal histopathology of Wegener's
Granulomatosis and related forms of vasculitis. Hum Palhol
BatsakisJG. Mid facial necrotizing diseases. Ann Orol Ellinol
Laryngol t982 : t5 : 597-6 t I.
Wegener's granulomatosis. Vascular System. Diseases ofthe
blood, heart and lungs. Anderson's pathology. Mosby
company, Vol. I. 10th Ed. 1996: 1397-1445.
Kenneth 0 Devaney, MD, Willium 0 et. al. Interpretation of
head and neck biopsies in Wegener's Granulomalosis.
Am J Surg Path 1990: 14 (6) : 555-564.
Colby TV, Tanzelaar HD, Specks U el. of. Nasal biopsy
in Wegener's granulomatosis. Hum Palhol 1991 .
22: tOI-t05,
Untreated, the course of disease is malignant and 80%
patients die within one year. Immunosupression with
cyclophosphamide improves survival in 90% patients. TI c
presence of immune complex in glomeruli and vessel wal'
and granulomas in upper respiratory tract and lungs
suggest 'some form of hypersensitivity reaction possibly
to an inhaled infectious or other environmental agent.
Highest relapse rate has been seen in Staph aureus nasal
carriers. The reason for this is unknown (1-3).
ner"s Granulomatosis is a c1inico-pathological
hlCh requires fulfillmcnt of both clinical and
icalcrileria to establ ish the diagnosis. All patients
demonstrate the classical triad of disease. Also all
d) llotdemonstrate classical pathological triad
!llma!OUS inflammation, necrosis and vasculitis
ead and neck biopsies. a criteria for diagnosis
,uggested and is applied where infectious causes
idlme granulomatous diseases are excluded (2).
alllhree major pathological criteria should be
in isolated case of head and neck involvement
ilsdemollstratioll, multiple sections to the extent
plion ofwhole ofthe block may beconsidered.
strams should be done in all cases. Major entities
are to be distinguished pathologically from WG
egranulomatous infections, lymphomatoid
alosis, idiopathic midline granuloma, sarcoidosis
'Ign body reaction (2-4). The organisms which
el) 10 be encountered in head and neck regions are
e IIhich cause blastomycosis, histoplasmosis,
Ichosisand tuberculosis (2, 4, 6).
II).ANCA should be performed for diagnosis and'
IIG. the patients have raised ESR, anemIa,
)losis and mild hypergammaglobulinemia (lg A),
Increase in rheumatoid factor and positive anti-
hilic cytoplasmic antibodies-ANCA (I). In our
the involvement of kidneys and lungs was not seen.
or malignant cell was seen on microscopic
ination. No special stain for fungus or
ll. 2. Apnl-Jullc 1999