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IMAGES IN ONCOLOGY
Year : 2021  |  Volume : 58  |  Issue : 1  |  Page : 145-146
 

A young boy with stridor and bilateral Bell's palsy


1 Department of Haematology, Vardhman Mahavir Medical College, and Safdarjung Hospital, Delhi, India
2 Consultant Pathologist, COE Histopathology, Mumbai, Maharashtra, India

Date of Submission24-Apr-2020
Date of Decision03-May-2020
Date of Acceptance22-Jan-2021
Date of Web Publication24-Mar-2021

Correspondence Address:
Ankur Jain
Department of Haematology, Vardhman Mahavir Medical College, and Safdarjung Hospital, Delhi
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijc.IJC_385_20

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How to cite this article:
Jain A, Sharma M, Mehta J. A young boy with stridor and bilateral Bell's palsy. Indian J Cancer 2021;58:145-6

How to cite this URL:
Jain A, Sharma M, Mehta J. A young boy with stridor and bilateral Bell's palsy. Indian J Cancer [serial online] 2021 [cited 2021 Apr 13];58:145-6. Available from: https://www.indianjcancer.com/text.asp?2021/58/1/145/311853




An 18-year-old boy presented to our hospital with stridor. He had inability to close both eyes for 15 days. Examination showed bilaterally tonsillomegaly and a nasopharyngeal mass (NPM) extending into oropharynx causing the airway compromise [Figure 1]a. Bilateral Bell's phenomenon was observed. An emergency tracheostomy was performed. Blood investigations were: hemoglobin 150 g/L, white cell count 7 × 109/L, and platelets 238 × 109/L. computerised tomography (CT) scan of the neck revealed a large (69 × 40 × 92 mm) heterogeneously enhancing mass lesion involving the nasopharynx and extending down upto the oropharynx causing the airway compromise [Figure 1]b. The lesion was 18FDG-avid on positron emission tomography-computerised tomography (PET-CT) scan with areas of necrosis [Figure 1]c. No mediastinal mass was discerned on PET-CT scan [Figure 1]b. Biopsy of the NPM revealed sheets of medium-sized lymphoid cells with blastic nuclear chromatin. Immunohistochemistry showed diffuse immunoreactivity for CD3 (membranous) and TdT in the neoplastic cells, and negativity for CD56, CD16, and in-situ-hybridization-Epstein Barr virus early RNA (EBER ISH), consistent with the diagnosis of T-lymphoblastic lymphoma (LBL) [Figure 1]d,[Figure 1]e,[Figure 1]f. Bone marrow aspiration showed 40% blasts, immunoreactive for CD2, CD3, CD4, CD5, CD7, CD8, TdT, CD38, HLA-DR, and TCR-α/β, and negative for CD1a. Cerebrospinal fluid analysis revealed malignant cells. A diagnosis of T-LBL/leukemia with central nervous system (CNS) involvement was made. Treatment with BFM-95 protocol resulted in complete remission of T-LBL/leukemia, and patient is currently in maintenance phase. Among lymphoma involving the nasopharynx, diffuse large B-cell lymphoma is the commonest subtype (70–80%), followed by T-cell lymphoma, especially extranodal NK/T-cell lymphoma, nasal type.[1] T-LBL accounts for only 2.4–7% of all nasopharyngeal lymphoma cases.[1],[2] T-LBL/leukemia is an aggressive neoplasm of T-lymphoblasts encountered most frequently in adolescent and young adults. Large mediastinal mass with/without superior vena cava syndrome is the commonest clinical presentation. CNS involvement at presentation is rare (3–9%).[3] Extranodal presentation of T-LBL/leukemia without a concomitant mediastinal mass is uncommon.[3] Pediatric acute lymphoblastic leukemia-based treatments impart an excellent prognosis.[3]
Figure 1: (a) Clinical photograph showing bilateral tonsillomegaly. (b) Positron emission tomography-computerised tomography (PET-CT) scan (left) showing a large nasopharyngeal mass (yellow arrow) and absence of mediastinal mass (blue arrow), and CT image (right) showing nasopharyngeal mass (c) intensely 18FDG-avid on PET-CT scan. (d) Microphotograph of the nasopharyngeal mass (H&E, 20×) showing blastoid cells, (e) immunoreactive for TdT (40×), and (f) CD3 (40×)

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Declaration of patient consent

The authors state that a written and informed consent was obtained from the patient prior to publication.

Compliance with ethical standards

The article has not been submitted elsewhere for consideration of publication. The article complies with the ethical standards by Declaration of Helsinki.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Laskar S, Muckaden MA, Bahl G, de S, Nair R, Gupta S, et al. Primary non-Hodgkin's lymphoma of the nasopharynx: Prognostic factors and outcome of 113 Indian patients. Leuk Lymphoma 2006;47:2132-9.  Back to cited text no. 1
    
2.
Miyagi Maeshima A, Taniguchi H, Makita S, Kitahara H, Miyamoto K, Fukuhara S, et al. Histopathological characteristics of lymphomas in the upper aerodigestive tract. A single-institute study in Japan. J Clin Exp Hematop 2015;55:7-11.  Back to cited text no. 2
    
3.
Lepretre S, Graux C, Touzart A, Macintyre E, Boissel N. Adult T-type lymphoblastic lymphoma: Treatment advances and prognostic indicators. Exp Hematol 2017;51:7-16.  Back to cited text no. 3
    


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