Isolated Sarcoidosis of the Tonsil:
Case Report and Literature Review
A
B
MBA ,
B
BA ,
A
MD
Saral Mehra, MD
Catherine Chang
Ashutosh Kacker
AWeill Cornell Medical College, Department of Otorhinolaryngology, New York, New York
BColumbia University Medical Center, Department of Otolaryngology, New York, New York
Objectives
Case Report
Discussion
1.To present only the eighth published
case of sarcoidosis presenting as
unilateral tonsil hypertrophy; and even
more rare, to present sarcoidosis limited
only to the tonsil in the absence of a
history of acute or chronic tonsillitis.
A 25 year old African American woman presented to her internist with one month of
noticing her uvula was being pushed to one side beginning after a self-limited common
cold. This progressed to pain and mild odynophagia without fevers or chills. She also
noted that her right tonsil was enlarged as compared to the left. She was sent for an MRI
and referred to an otolaryngologist by her primary medical doctor for further evaluation.
We report a new case of unilateral tonsillar enlargement and elevated
ACE level as the only manifestations of sarcoidosis. Only seven authors
have previously published cases of sarcoidosis presenting as tonsillar
asymmetry.2,6-11
2.To review the existing literature as it
relates to evaluation, management, and
follow-up of this finding including the
association with malignancy.
Abstract
She had no significant medical history, including no history of tonsil infections. She
occasionally smoked and reported drinking a a small amount of alcohol socially. She took
no medications and has no family history of malignancy.
Preoperative laboratory values showed normal CBC, basic metabolic panel, and LFT
except for borderline low platelets (148) and elevated AST (50 U/L, normal 15 to 41 U/L)
and ALT (83 U/L, normal 14 to 54 U/L). Imaging is shown below.
Study Design: Case report and literature
review.
The patient was taken to operating room for tonsil biopsy which showed non-necrotizing
granulomas within a lymphoid infiltrate just deep to squamous epithelial surface lining of
tonsil which was consistent with sarcoidosis.
Methods: A case is presented with
appropriate history, laboratory values,
imaging results, and pathology slides.
Then the literature on the topic of
sarcoidosis of the tonsil is systematically
reviewed.
Follow-up evaluation showed an elevated angiotensin converting enzyme (ACE) level of
80 Units/Liter (normal 9 to 67 U/L). Chest x-ray was within normal limits without hilar
lymphadenopathy. The remainder of malignancy workup and review of systems was
negative. The patient was placed on steroids. One year follow-up revealed no further
evidence of sarcoidosis manifestations.
Results: A 25 year old black woman with
no history of acute or chronic tonsillitis
presented with unilateral tonsil hypertrophy
and had biopsy showing non-necrotizing
granulomas consistent with sarcoidosis.
She had elevated angiotensin converting
enzyme (ACE), but no other systemic signs
or symptoms at diagnosis or upon follow-up
evaluation.
Conclusions: This case and literature
review support the finding of sarcoidosis
limited to and presenting as asymmetric
tonsillar hypertrophy. However, it is still
important that such patients receive
appropriate work-up, referral, and follow-up
for development of systemic manifestations
of sarcoid and to be vigilant for any signs or
symptoms of malignancy.
Introduction
Sarcoidosis is a multi-systemic disease
of unknown etiology characterized by
the presence of noncaseating
granulomas composed mainly of
epithelioid cells with the presence of
Langhan’s giant cells and histiocytes.1,2
Manifestations of sarcoidosis in the head
and neck are present in 10 to 15 % of
patients with sarcoidosis, and may
include cervical adenopathy, enlarged
lacrimal glands, uveitis, orbital mass,
nasopharyngeal lesions, or bilateral
parotid enlargement.3,4 Tonsillar
manifestations are rare, comprising less
than 0.03% of cases.5
Contact
Ashutosh Kacker, MD
Weil Cornell Medical College
Department of Otorhinolaryngology
ask9001@med.cornell.edu
A
Granulomatous tonsillar enlargement can be the result of numerous
etiologies, such as Crohn’s disease, fungal infection, tuberculosis,
lymphoma, squamous cell carcinoma, venereal disease, toxoplasmosis,
berylliosis, foreign body reactions, and secondary responses to lymphoid
tissue draining carcinoma.2,12,13 Sarcoid-like granulomas can also be a
manifestation of neoplastic disease such as Hodgkin’s and non Hodgkin’s
lymphoma.14,15,16 These causes must be excluded prior to making a
diagnosis of sarcoidosis.
Some believe that development of sarcoidosis is a cellular immune
response to excessive antigen stimulation resulting from an exaggerated
immune response to the stimuli of chronic tonsillitis. 11,13 However, the
patient in this case report, had an elevated ACE level and no history of
chronic tonsillitis, thereby supporting the finding of sarcoidosis as a mild
self-limited finding isolated to the tonsil. It will, however, be important to
follow this patient for any subsequent systemic manifestations of systemic
sarcoidosis.
A number of studies have shown a relationship between sarcoidosis and
malignancy. Some authors have suggested the existence of a
sarcoidosis-lymphoma syndrome in which the chronic active type of
sarcoidosis appears to be responsible for an increased risk of malignant
transformation of lymphoid cells.14,17,18 As described by Brickner, there
are three main features of sarcoidosis-lymphoma syndrome: 1) lymphoid
malignancy occurs after a preceding history of sarcoid; 2) the median
age of onset of sarcoidosis is 10 years above that of unselected patients
with sarcoid; 3) Hodgkin’s disease occurs more frequently than other
types of lymphoma. Following patients for any signs or symptoms of
malignancy will be important.
B
Conclusion
C
D
Figure 1. A, B, C. Axial T1 postpost-contrast fat suppressed MRI images showing right tonsil enlargement
enlargement (arrow), right cervical node (arrowhead), and retropharyngeal
retropharyngeal nodes.
Coronal T1 postpost-contrast fat suppressed MRI images showing right tonsil enlargement.
enlargement.
D.
Figure 1. A, B, C. Axial T1 postpost-contrast fat suppressed MRI images showing right tonsil enlargement
enlargement (arrow),
right cervical node (arrowhead), and retropharyngeal nodes. D. Coronal T1 postpost-contrast fat suppressed MRI
images showing right tonsil enlargement.
Figure 2. A and B. LowLow-power view of specimen showing nonnon-necrotizing granulomas (arrows) within a
lymphoid infiltrate just deep to squamous epithelial surface lining
lining of tonsil. C. HighHigh-power view of single
nonnon-necrotizing granuloma with epitheloid histiocytes with carrot shaped
shaped nuclei. D. GMS stain showing the
absence of microorganisms. E. Special stain for CD20 showing
showing presence of B lymphocytes. F. Special
stains for CD3 showing presence of T lymphocytes.
Figure 2. A and B. LowLow- power view of specimen showing nonnon- necrotizing granulomas (arrows) within a lymphoid infiltrate just
just deep to squamous epithelial surface lining of
tonsil. C. HighHigh- power view of single nonnon- necrotizing granuloma (arrow) with epitheloid histiocytes with carrot
carrot shaped nuclei (arrow(arrow- head). D. GMS stain showing the
absence of microorganisms. E. Special stain for CD20 showing
showing presence of B lymphocytes. F. Special stains for CD3 showing
showing presence of T lymphocytes.
This case and literature review supports the finding of sarcoidosis limited
to and presenting as asymmetric tonsillar hypertrophy. However, it is still
important that the patient receive appropriate work-up, referral, and
follow-up for development of systemic manifestations of sarcoidosis and
to be vigilant for any signs or symptoms of malignancy.
References
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