| Abstract|| |
The follicular variant of peripheral T-cell lymphoma, not otherwise specified, is very rare. Primary epiglottic follicular variant of peripheral T-cell lymphoma is extremely rare in clinical practice. Here, we report the first case of a follicular variant of peripheral T-cell lymphoma not otherwise specified in a 44-year-old Chinese man, who presented with a tumor in the middle of the epiglottis tongue surface. Microscopically, the tumor had a vague nodular growth pattern and the morphology of the nodules was different from each other at low power. Atypical lymphoid cells were medium to large in size and had round nuclei, with an irregular nuclear membrane, distinct nucleoli, and rapid mitotic activity. Plasma cells were found surrounding the nodules. The tumor cells were positive for follicular helper T-cell markers (CD10, PD-1, CXCL13, and BCL-6). The EBER was negative by in situ hybridization. Polymerase chain reaction-based analysis showed monoclonal rearrangements of TCRβ, TCRγ, and polyclonal rearrangements of IgH, IgK, and IgL. The clinical and imaging features and the prognostic factors of FV PTCL-NOS remain poorly understood. Thus, investigation of more cases and longer follow-up is necessary to understand the disease and to identify the best treatment to improve prognosis.
Keywords: Epiglottis, follicular T-helper cells, follicular variant PTCL
|How to cite this article:|
Kong J, Zhong L, Gao X, Qi W, Zhang L, Lin Z. Primary epiglottic follicular variant of peripheral T-cell lymphoma. Indian J Pathol Microbiol 2019;62:586-8
|How to cite this URL:|
Kong J, Zhong L, Gao X, Qi W, Zhang L, Lin Z. Primary epiglottic follicular variant of peripheral T-cell lymphoma. Indian J Pathol Microbiol [serial online] 2019 [cited 2020 Jun 4];62:586-8. Available from: http://www.ijpmonline.org/text.asp?2019/62/4/586/269089
| Introduction|| |
According to the World Health Organization (WHO) classification in 2008, the variant of peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS), is termed “follicular variant” (FV). In the present study, we report the first case of FV PTCL-NOS in a 44-year-old-man involving the epiglottis, and focus on the clinical, histopathologic, and immunophenotyping characteristics of this case.
| Case Report|| |
A 44-year-old man complained of an ambiguous swallowing foreign body sensation for 2 months. Laryngoscopy examination confirmed the presence of a tumor in the epiglottis tongue surface, and the epiglottis structure had disappeared. Computed tomography revealed a tumor mass in the supraglottic portion involving the uvula, which measured 23 × 21 mm [Figure 1]a. Fluorine-18 fluorodeoxyglucose positron emission tomography scanning indicated the increased uptake of FDG located in the epiglottis [Figure 1]b.
|Figure 1: (a) Computed tomography revealed an irregular tumor mass in the supraglottic portion involving the uvula. (b) 18F-fluorodeoxy glucose positron emission tomography (FDG-PET) shows the epiglottis|
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Microscopically, the tumor beneath the squamous epithelium showed a vague nodular growth pattern and the morphology of the nodules were different from each other at low power. The tumor cells were medium-to-large in size and had round nuclei with an irregular nuclear membrane, distinct nucleoli, and mitotic figures [Figure 2]. Plasma cells surrounded the nodules.
|Figure 2: Morphological features of FV PTCL-NOS. (a) The tumor beneath the squamous epithelium has a vague nodular growth pattern and the nodule morphology is different at low power (magnification 40×). (b and c) Atypical lymphoid cells are medium-to-large in size and have round nuclei with an irregular nuclear membrane, and distinct nucleoli. Plasma cells surrounded the nodules (magnification 100×, 200×). (d) Higher magnification of such clusters shows they contain medium-to-large cells with a pale cytoplasm and irregular clear nuclei (magnification 400×)|
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Immunohistochemistry demonstrated a meshwork of CD21-positive follicular dendritic cells (FDCs). Most tumor cells expressed programmed death-1 (PD-1), Bcl-6, CD10. CXCL-13 was located in cytoplasm in a few tumor cells [Figure 3]. Tumor cells were also positive for Bcl-2, CD2, CD3, CD4, CD5, CD30, CD43, and CD99, and negative for CD1α, CD7, CD8, CD79α, CD34, CD56, MUM-1, TdT, granzymeB, Cyclin D1, and ALK. CD20 and PAX-5 were negative in tumor cells but were expressed in B-cells in the nodules. EBER was negative by in situ hybridization. About 40% of cells express the cell proliferation marker Ki-67. Polymerase chain reaction (PCR) analysis of lymphocyte clonality showed monoclonal rearrangements of T-cell receptor β (TCR) and TCRγ.
|Figure 3: Immunophenotype of FV PTCL-NOS. (a) CD21 highlights the extensive dendritic cell network. Most tumor cells expressed (b) CD3, (c) CD10, (d) BCL-6, (e) PD-1, (f) CXCL-13 was located in cytoplasm in a few tumor cells (a-d, magnification 100×; e and f, magnification 200×)|
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On the basis of the histopathological and immunohistochemical examinations, a diagnosis of FV PTCL-NOS was made. Based on this, the patient underwent six cycles of cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP). Currently, he is alive with no tumor recurrence at 42 months follow-up.
| Discussion|| |
PTCL-NOS accounts for about 25% of all PTCL, which represent over 15% of all lymphomas. PTCL-NOS is usually found in males, with a median age of 55--60 years, and has a higher prevalence in adults. In the lymph node, FV PTCL-NOS shows paracortical or diffuse infiltrates with effacement of the normal architecture and inflammatory background frequently observed. Patients often have B symptoms, and generalized lymphadenopathy and extranodal involvement is common, with the skin and gastrointestinal tract representing the most commonly affected sites. FV PTCL-NOS is a rare morphology variant of PTCL-NOS presenting in the lymph nodes, especially the primary form, which is extranodal., The present case is the first case of FV PTCL-NOS involving the epiglottis.
Rüdiger et al. reported the first case of F-PTCL, and further studies have described some similarities between F-PTCL and AITL other than their origin. Different with AITL, PTCL-NOS usually lacks a follicular T-helper phenotype with the exception of the follicular variant and proliferation is usually high. Tumor cells in FV PTCL-NOS and AITL were reported to originate from the TFH with immunohistochemical expressions of CD10, Bcl-6, PD-1, and CXCL13. In studies of FV PTCL-NOS, most cases had the same pathologic feature with AITL, although diffuse effacement of the architecture was infrequent., Huang et al. reported that patient with FV PTCL-NOS at first biopsy at diagnosis relapsed as AITL. This suggests that at least some FV PTCL-NOS cases can present with the pathologic features of AITL. However, Ortiz--Muchotrigo considered that this is a different condition from AITL.
Hu et al. also reported that monoclonal rearrangement of the TCR genes as detected in 85% (47/55) of the FV PTCL-NOS cases assessed.
Patients with stage I--II disease are rare, and radiation therapy is suitable for individual clinical treatment. Ikonomou et al. reported that FV PTCL-NOS behaved as an intermediate aggressive lymphoma, with multiple relapses and rapid progression of the disease after chemotherapy. It was reported that patients with CHOP treatment had an overall response rate greater than 60%, and many patients had a complete or partial remission. However, FV PTCL-NOS relapses are frequent and the 5-year OS is approximately 20--30%. Dupuis et al. reported that approximately 30% cases of FV PTCL-NOS were found Epstein--Barr virus (EBV) cases, which might be associated with prognosis.
The clinical and imaging features and the prognostic factors of FV PTCL-NOS remain poorly understood. Thus, larger numbers of cases and longer follow-up is necessary to understand the disease and to identify the best treatment to improve prognosis.
Ethics approval and consent to participate
This study was approved by the Human Research Ethics Committee of the First Affiliated Hospital of Dalian Medical University.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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Department of Pathology, The First Affiliated Hospital of Dalian Medical University, 222 Zhongshan Road, Dalian - 116011, Liaoning
Department of Pathology, Yanbian University Medical College, 977 Gongyuan Road, Yanji 133002, Jilin
Source of Support: None, Conflict of Interest: None
[Figure 1], [Figure 2], [Figure 3]