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    Pathology – Research and Practice 211 (2015) 88–91

    Contents lists available at ScienceDirect

    Pathology – Research and Practice

     journal homepage: www.elsevier .com/ locate /prp

    Teaching cases

    Follicular dendritic cell sarcoma of the tonsil

    Monique Vorspracha,∗, Thomas Kalinskib, Ulrich Vorwerkc

    a Department of Pneumology,Otto-von-Guericke University, Magdeburg, Germanyb Department of Pathology,Otto-von-Guericke University, Magdeburg,Germanyc Department of Otorhinolaryngology,Otto-von-Guericke University, Magdeburg, Germany

    a r t i c l e i n f o

     Article history:

    Received 8 April 2014

    Received in revised form 8 August 2014Accepted 16 September 2014

    Keywords:

    Follicular dendritic cell sarcoma

    Tonsil

    Immunohistochemistry

    FDCS

    a b s t r a c t

    Follicular dendritic cell sarcoma (FDCS) is a rare neoplasm that occurs extranodally and nodally. The

    following case report describes a 24-year-old male patient who suffered from FDCS of  the tonsil. He

    presented at the ENT Department of the University Hospital Magdeburg with throat pain that had lasted

    for 3 months. There were neither B symptoms nor abnormal fatigue. An extended tonsillectomy was

    performed. The morphological and immunohistochemical findings confirmed the diagnosis of FDCS.

    FDCS should be considered as an important differential diagnosis in spindle cell tumors of the tonsil.

    © 2014 Elsevier GmbH. All rights reserved.

    Introduction

    Clinical history

    A 24-year-oldmalepatientwas admittedto theUniversityClinicof Magdeburg with a complaint of throat pain which, according

    to him, had lasted for almost 3 months. B-symptoms or abnormal

    fatigue were not reported by the patient. Before admission to the

    hospital, he had visited an otolaryngologist who noticed an asym-

    metry of the tonsils, and performed a biopsy of the left tonsil. The

    histopathological examination of the biopsy revealed a follicular

    dendritic cell sarcoma. At the time of the admission to the hos-

    pital, intraoral examination showed a massive swelling of the left

    tonsil (Fig. 1). This tonsil was found to be smooth, and exhibited

    easy luxation. No necrotic or ulcerated areas were observed. The

    tonsil of the opposite side showed signs of chronic tonsillitis and

    appearedscarred. Inaddition, theleft tympanicmembrane showed

    a retraction pocket and was atrophic. Neck examination revealed

    two lymph nodes of approximately 1cm in size on both sides of 

    the neck in region II. The results of a general ear nose and throat

    examination were normal without any relevant comorbidity.

    Diagnostics

    Ultrasonography revealed a lymphadenitis that was more

    severe on the left side of the neck than on the right side. A tumor

    ∗ Corresponding author. Tel.: +49391 67 15421.

    E-mail address:[email protected](M. Vorsprach).

    mass of approximately 24mm in diameter with a homogenous

    structure and low echogenocity (Fig. 2) was found in the left ton-

    sillar region.

    The MRI scan showed a mass of 25mm×20mm in the area of 

    theleft tonsil, whichlimits the spaceof the left oropharynx (Fig. 3).The native 18-fluoro-desoxy-d-glucose-positron-emission-

    tomography-computed-tomography (F-18-FDG-PET CT) scan

    showed only a slight increase of the glucose metabolism in the

    area of the left tonsil. No sign of metastasis was found in other

    parts of the body; however, their presence could not be ruled out

    with absolute certainty.

    Therapy

    Extended laser-tonsillectomy was performed with a CO2-laser

    on the left tonsil, where the tumor was located, under general

    anesthesia. Themargins were free of tumor. Furthermore, conven-

    tional tonsillectomy was also performed on the opposite side, as

    requested by the patient.

    Moreover, the patient was treated with cefuroxime for 10 days

    aftertheoperation.Adjuvantradiochemistryorneckdissectionwas

    not performed. Nevertheless, the patient was requested to return

    to theclinic for furtherregularexaminations.Untilnow,no residual

    tumor mass has been detected.

    General information on the disease

    Follicular dendritic cells (FDCs) are non-phagocytic, non-

    lymphoid antigen-presenting cells of the accessory immune

    system. These cells of mesenchymal origin are capable of  

    http://dx.doi.org/10.1016/j.prp.2014.09.015

    0344-0338/© 2014 Elsevier GmbH. All rights reserved.

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    M. Vorsprachet al./ Pathology – Research andPractice 211 (2015) 88–91 89

    Fig. 1. Intraoral examination with thetumor of theleft tonsil.

    capturing, processing and presenting antigens and immune com-plexes to T-cells and B-cells in order to maintain the humoral

    immune response. They are part of the dendritic cell group, which

    can be divided into four main categories found in lymphatic tissue:

    interdigitating dendritic, indeterminate, Langerhans and follicular

    dendritic cells [6]. FDCs are mainly found in the germinal centers

    of primary and secondary follicles of lymph nodes, the spleen or

    mucosa associated lymphatic tissue (MALT) within the network of 

    other local cells.

    The first case of a FDC sarcoma was described by Lennert [8].

    Then, in 1986, Monda et al. reported the first case of primary neo-

    plasm of FDCs in the lymph node [9]. To date, only a few cases

    of FDC sarcoma in the tonsil have been described [13]. Few extra

    nodal FDCS were reported by Youens and Waugh [14], and in the

    pharyngeal or abdominal region [12].

    Fig. 2. Ultrasonographyof theleft tonsildepictingthe tumor mass of 24mm diam-

    eter.

    Fig. 3. MRI scan of the head with the tumor of the left tonsil.

    Material andmethods

    Histology

    Aftercompletetonsillectomy,thetissueofthelefttonsilwascol-

    lectedandpreservedin buffered formalin (10%). Subsequently, the

    tissuewasdehydrated and embedded in paraffin. Sections (3.0m

    thick) fromformalin-fixed, paraffin-embedded tissue werestained

    withhematoxylinandeosin(HE). Light microscopy wasperformed

    using an Axioplan microscope (Zeiss, Oberkochen, Germany) and

    a Hamamatsu Nanozoomer (Hamamatsu Photonics, Hamamatsu,

     Japan).

    Immunohistochemistry

    Sections(3.0mthick) fromformalin-fixed,paraffin-embedded

    tissue were incubated with a panel of standard antibodies against

    cytokeratins (MNF116, AE1/AE3), epithelial membrane antigen

    (EMA), vimentin, sm-actin, desmin, S-100 protein, HMB45, leuko-

    cyte common antigen (LCA), KiM4p, CD23, and ki-67 using a

    Benchmark XT (Ventana Medical Systems, Tucson, USA). The reac-

    tions were visualized using DAB detection.

    Results

    Morphology and immunophenotype

    The tumor presented as a well-defined mass in an otherwise

    regularlystructuredtonsil (Fig.4). Thespindledtumorcellsformed

    fasciclesand storiformpatterns. Thenuclei showeddispersedchro-

    matin and small nucleoli without significant cytological atypia.

    Only a few mitoses were observed (4/10 HPF). Small lymphocytes

    werescatteredacross thetumor. Thesurgicalmarginsweretumor-

    free.

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    90 M. Vorsprachet al./ Pathology – Research andPractice 211 (2015) 88–91

    Fig. 4. Histopathological and immunohistological findings after tonsillectomy. (A) Left tonsil with a well-defined tumor mass. (B) Spindled tumor cells forming fascicles or

    storiformpatterns.(C) Highermagnificationshowingnuclei ofthe tumorcellswith dispersedchromatinand smallnucleoli,and scatteredsmalllymphocytes.(D) Immunostain

    demonstratingpositivity for Vimentin. (E) Immunostain demonstrating positivity for KiM4p.

    Immunohistochemically, the tumor cells demonstrated strong

    positivity for Vimentin and KiM4p, and partially a slight positivity

    for EMA. The tumor cells were negative for cytokeratins (MNF116,

    AE1/AE3), sm-actin, desmin, S-100 protein,HMB45, LCA,andCD23.

    The ki-67 labeling index was about 10%.

    The findings confirmed the diagnosis of a follicular dendritic

    sarcoma (FDCS).

    Discussion

    FDCS is an unusual disease that mainly occurs in middle-aged

    males and females between 30 and 50 years [7,10,11]. The FDCS

    rarelyoccursextranodally in the head andneck region. This type of 

    tumor is usually found as a painless lymph adenopathy in lymph

    nodesof theneck,axilla andmediastinum.Nevertheless,FDCSmust

    beconsideredanimportantdifferentialdiagnosis ofa tonsillarmass

    and, in addition to thehistopathological examination, immunohis-

    tochemical methods should be used for diagnostics to confirm the

    diagnosis.

    Typical characteristics of FDCS in histological examinations are

    oval to spindle shaped cells with eosinophilic cytoplasm arrangedin fascicles or sheets with a storiform growth pattern, which leads

    to the diagnosis of sarcoma. Due to the typical characterization of 

    small lymphocytes, lymphoma might be misdiagnosed [5].

    The importance of a precise diagnosis was emphasized by

    Duan et al. in their overview of 36 cases of FDCS, in which they

    showed that 21 cases had initially been misdiagnosed [4] before

    immunohistochemistryrevealedFDCS. Importantdifferential diag-

    noses areextracranialmeningioma,peripheral nerve sheathtumor,

    malignant melanoma, ectopic thymoma, metastatic carcinoma,

    malignant fibrous histiocytoma, large cell lymphoma, and inter-

    stitial reticulum cell sarcoma [1,3].

    In immunohistochemistry, FDCS are positive for CD21, CD35,

    KiM4p, and KiFDC1p, and are variably positive for vimentin, S-

    100 protein, CD68 and specific muscle actin [2,5,7]. In our case,

    thehistologicalexaminationsshowed typical spindle-shaped cells.

    Immunohistochemically, the reactions using antibodies against

    vimentin and Ki-M4p were positive and confirmed the diagnosis.

    The role of adjuvant therapy is a debated topic in the literature.

    Chan et al. [2] consider systemic radiotherapy as the treatment

    of choice, while other authors such as Perez-Ordonez and Rosai

    [10,13] prefer this therapy as an option for the treatment of lymph

    node metastasis of theneck region, positive margins or recurrenceof the tumor.

    In 2006, a case report of a 76-year-old woman was presented

    by Aydin et al. [1]. This patient, who suffered from an FDCS of the

    tonsil, underwent surgery and adjuvant radiotherapy treatment.

    She was recurrence-free after 4 years of follow up.

    Inour case,we performeda wide excision of the tumor without

    any further therapy such as neck dissection or adjuvant radiother-

    apy or chemotherapy.

    We decided to use this strategy because according to our find-

    ings, metastases or suspect lymph nodes were neither found in

    the neck region nor in the rest of the body. Considering the fact

    that FDCS is a malignancy with a high risk of local recurrence, we

    asked thepatient topresent forregular follow-ups inourclinic.We

    believe that any adjuvant therapy at this stage will reduce the lifequality of the patient. In case of tumor recurrence or metastasis,

    we still have the options to perform a neck dissection or adjuvant

    radiotherapy or chemotherapy. We will reconsider our strategy in

    order to find the best treatment for the patient.

    References

    [1] E. Aydin, L.N. Ozluoglu, B. Demirhan, U. Arikan, Follicular dendritic cell sar-coma of the tonsil: case report, Eur. Arch. Otorhinolaryngol. 263 (12) (2006)1155–1157.

    [2]  J.K. Chan, C.D. Fletcher, S.J. Nayler, K. Cooper, Follicular dendritic cell sarcoma.Clinicopathologicanalysis of 17 cases suggesting a malignant potential higherthan currently recognized, Cancer79 (2) (1997) 294–313.

    [3] H. Domínguez-Malagón, A.M. Cano-Valdez, A. Mosqueda-Taylor, O. Hes, Fol-

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