Download Follicular Dendritic Cell Sarcoma of Tonsil

CASE REPORT
Follicular Dendritic Cell Sarcoma of Tonsil
Zahid Suhail, Mohammad Ayub Musani, Salman Afaq, Abbas Zafar* and Syed Khalid Ahmed Ashrafi
ABSTRACT
Follicular dendritic cell sarcoma is a very rare entity. So far only 12 cases have been reported world wide with involvement
of tonsil. We present a new case of follicurlar dendritic cell sarcoma of the tonsil in a 52 years old woman with no evidence
of neck node involvement. She had undergone diagnostic tonsillectomy due to unilateral tonsillar enlargement. The final
diagnosis after histological review and immunohistochemical stains was follicular dendritic cell sarcoma. Bone scan
showed no metastasis. One year follow-up after postoperative chemotherapy showed no evidence of local or regional
recurrance. Follicular dendritic cell sarcoma is a rare and underdiagnosed neoplasm. It should be included in the
differential diagosis of any tonsillar mass in adults. Follicular dendritic cell markers such as SR-100, CD21 and/or CD35
are essential for the diagnosis.
Key words:
Follicular dendritic cell. Sarcoma. Tonsil.
INTRODUCTION
Follicular dendritic cells like T-lymphocytes, B-lymphocytes,
macrophages and N.K cells are among the accessory
cells in the immune system.1 They are present in the
germinal centres of lymphoid follicles in the spleen and
lymph nodes.2 They have numerous dendritic
cytoplasmic process. They bear Fc receptors for Ig G so
they can trap antigen bound to antibodies and present
them to B cells. The cells express positivity towards
CD21, CD 35 and S-100 monoclonal antibodies.
Follicular dendritic cell sarcoma (FDCS) is unusual and
its extranodal origin is extremely rare.3,4 Seventy one
cases have been reported in the English language
medical literature, of which 25 cases were extranodal,
occurring in the head and neck region1 Twelve cases of
tonsillar FDCS have been reported.1
We report a new case of FDCS of tonsil in a 52 years
old woman.
CASE REPORT
the head and neck area , ultrasound of abdomen and
Mountoux test were all unremarkable. Diagnostic
tonsillectomy was performed and a specimen of size 2.5x2
cm was sent for histopathology. Preliminary diagnosis
was suggestive of "non-epithelial malignant neoplasm”.
Histological sections revealed proliferation of spindled to
ovoid cells. The tumour cells had eosinophilic cytoplasm
with indistinct cell borders. The nuclei were elongated
with vesicular to finely dispersed chromatin (Figure 1).
There was mild to moderate nuclear atypia and
pleomorphism. Mitotic activity was enhanced. In some
areas lymphocytes were intermixed with neoplastic
cells. Overlying stratified squamous epithelium was
unremarkable. The sections were stained with a pannel
of monoclonal antibodies using "Envison system". The
tumor cells expressed CD21 and S-100 protien. CD6 B
was equivocal on additional immunohistochemical
stain.
The final diagnosis was FDCS with negative margins of
resection. Skeletal scintography was found negative for
metastatic involvement of the skeleton. Chemotherapy
The patient was a 52-year-old woman with no
cormorbidity. She presented to the out patient
department with complaints of a swelling in the throat
and dysphagia for the last few weeks. There was no
history of fever, otalgia or trismus. On clinical
examination , the right tonsil was found to be enlarged
and indurated with a normal mucus membrane covering.
The base line investigations were normal. CT scan of
Department of ENT, Karachi Medical and Dental College and
Abbasi Shaheed Hospital, Karachi.
* Department of ENT, Zaiuddin Medical University, Karachi
Correspondence: Dr. Mohammad Ayub Musani, D-44, Block-H,
North Nazimabad, Karachi.
E-mail: [email protected]
Received June 02, 2009; accepted September 29, 2009.
Journal of the College of Physicians and Surgeons Pakistan 2010, Vol. 20 (1): 55-56
Figure 1: Slide showing microscopic picture of FDCS. Section reveal sheets
of spindled to ovoid large cells with slightly pale eosinophilic cytoplasm.
55
Zahid Suhail, Mohammad Ayub Musani, Salman Afaq, Abbas Zafar and Syed Khalid Ahmed Ashrafi
(Doxorubicin and Ifosfamide) was given with
consultation of the oncologist. The patient was free of
disease one year after the end of the treatment.
dissection was not done as CT showed no evidence of
cervical node metastasis. In the 12 reported cases of
tonsillar FDCS, only 3 patients had lymph node
metastasis but 5 patients underwent neck dissection.10
DISCUSSION
The role of postoperative adjuvant treatment is also
debatable. Some authors suggest systemic radiotherapy
in all the resected cases,7 while others reserve this
option in neck node metastasis, cases with positive
resected margins, tumours with adverse pathological
features and in recurrent cases.8 A one year follow-up in
this case showed no evidence of regional recurrence.
Aydin et al. reported the case of a 76 years old female of
FDCS of tonsil who was disease free at 4 years follow
up.4 Ludwig reported the case of a 47 years old female
who had 3 recurrences within a period of 11 years and
the recurrences showed a more aggressive FDCS
behaviour than was initially assessed.5 Due to the
limited number of cases reported with short follow-up, a
general consensus about the significance of neck
dissection, prognostic role of adjuvant therapy and
frequency of recurrence has not been established.
FDC sarcoma is a rare tumour that derives from the
dendritic cells of lymphoid follicles.5 Follicular dendritic
cells are non-lymphoid, non-phagocytic, accessory cells
in the immune system that are essential for antigen
presentation and germinal centre regulation.6 Lymph
nodes are the sites most commonly involved by FDC
sarcoma and its extranodal origin is extremely rare.4,6
However, it may arise at a variety of extranodal sites
including the oral cavity, tonsils, gastroi and liver,
because of the presence of dendritic cells there.6-9
Only a few cases of FDCS of the head and neck region
have been reported.5 It occurs most frequently in adults
aged between 30 and 50 years, with no gender
predilection.1 The maximum age was noted to be 77
years in a woman, who was previously misdiagnosed as
squamous cell carcinoma of the tonsil.10
REFERENCES
Histologically, tumour cells present with abundant
eosinophilic cytoplasm, hyperchromic and pleomorhic
nuclei and prominent neucleoli.6 Immunohistochemically, tumour cells were strongly and diffusely
positive for follicular dendritic cells markers CD21,
CD35, CD1a, S100 protien.1,6 These markers are,
therefore, essential for correct diagnosis.
The extranodal tumours, therefore, can easily be
misdiagnosed as FDC markers and are not routinely
been used in the immunohistochemical study of poorly
differentiated tumours.10 Differential diagnosis is to be
made from several tumours, including undifferentiated
carcinoma, squamous cells carcinoma, malignant
melanoma, large cell lymphoma, menengioma, thymoma,
malignant fibrous histiocytoma, peripheral never sheath
tumour, angiofibrosarcoma and inflamatory pseudotumours.7,8,10
Treatment modalites include surgery followed by
radiotherapy or chemotherapy or an adjuvant chemoradiotheropy. However, surgery is the primary treatment
and a wide resection is recommended when the
diagnosis has been established pre-operatively in cases
with postoperative positive margins and in recurrent
cases.1 Neck dissection in a clinically negative neck
examination is controversial and there is no consensus
opinion described in the previously reported cases.1
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However, neck dissection is recommended in cases with
evidence of cervical node metastasis. In this case, neck
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Journal of the College of Physicians and Surgeons Pakistan 2010, Vol. 20 (1): 55-56