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 Table of Contents  
Year : 2015  |  Volume : 40  |  Issue : 3  |  Page : 150-152

Plasmablastic lymphoma developing in thyroid: a rare entity in an immunocompetent individual

1 Department of Laboratory Medicine, Basavatarakam Indo American Cancer Hospital and Research Institute, Hyderabad, Telangana, Andhra Pradesh, India
2 Department of Medical Oncology, Basavatarakam Indo American Cancer Hospital and Research Institute, Hyderabad, Telangana, Andhra Pradesh, India

Date of Submission05-May-2015
Date of Acceptance15-May-2015
Date of Web Publication8-Sep-2015

Correspondence Address:
Faiq Ahmed
Department of Laboratory Medicine, Basavatarakam Indo American Cancer Hospital and Research Institute, Road No. 10, Banjara Hills, Hyderabad - 500 034, Telangana, Andhra Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1110-1067.164743

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A case of plasmablastic lymphoma that has not been described previously in the thyroid is presented with its clinicopathological features and the diagnostic difficulties encountered. A detailed histopathology in conjunction with immunohistochemistry is recommended for appropriate diagnosis and management in the setting of HIV-negative status. Dealing with a case presenting primarily as a thyroid mass can be challenging in the scenario of a CD20-negative immunoprofile.

Keywords: immunocompetent, non-Hodgkin′s lymphoma, plasmablastic lymphoma, thyroid

How to cite this article:
Ahmed F, Mundada MC, Murthy SS, Rajappa SJ. Plasmablastic lymphoma developing in thyroid: a rare entity in an immunocompetent individual. Egypt J Haematol 2015;40:150-2

How to cite this URL:
Ahmed F, Mundada MC, Murthy SS, Rajappa SJ. Plasmablastic lymphoma developing in thyroid: a rare entity in an immunocompetent individual. Egypt J Haematol [serial online] 2015 [cited 2022 Aug 19];40:150-2. Available from: http://www.ehj.eg.net/text.asp?2015/40/3/150/164743

  Introduction Top

Plasmablastic lymphoma (PBL) is a rare non-Hodgkin's lymphoma in which the proliferating B-cells has switched its phenotype to the plasma cell gene expression profile. Evidences on cases of PBL in the thyroid are lacking. Although in the HIV setting the preferred site of PBL is the oral cavity, in the non-HIV setting, various extraoral sites have been described [1] . This is the first case of PBL involving the thyroid, as an unusual presentation with diagnostic features and difficulties encountered in arriving at a diagnosis of such a rare entity, that will be discussed.

  Case history Top

A 61-year-old man presented to our surgical outpatient department with a swelling in the midline neck of 3 months' duration. He also had a history of dysphagia and change in voice of 1-month duration. His medical history was not significant and there was a lack of any obvious immunosuppression. Physical examination indicated a large hard mass ~10 × 10 cm involving the thyroid with tiny small level IV cervical lymph nodes. MRI of the neck indicated a large lobulated isointense mass on T1W and a hyperintense mass on T2W images, measuring 11 × 8.2 × 7.4 cm, involving left lobe of the thyroid and the isthmus. This mass was causing a compression effect, with a mild shift of the trachea to the right side ([Figure 1]). The larynx was normal. Multiple small cervical lymph nodes were identified at the level IV region and the largest lymph node measured 1.8 cm. Fine-needle aspiration cytology performed from the thyroid raised the suspicion of lymphoma. Enzyme-linked immunosorbent assay for HIV was negative. A trucut biopsy under guidance was performed. The biopsy showed a diffuse lesion of large cells with a syncytial pattern and entrapped thyroid follicles. These cells were large, with round, central to peripherally placed nuclei, with single prominent nucleoli and few cells with coarse chromatin and smaller one to two nucleoli. Apoptosis and focal necrosis were noted ([Figure 2]a and b).
Figure 1 MRI showing involvement of plasmablastic lymphoma in the left lobe and isthmus of the thyroid.

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Figure 2 (a) Entrapped thyroid follicles amidst the lesion (×40). (b) Cells with immunoblastic and plasmablastic morphology (×40).

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In view of the morphologic diagnosis of a high-grade lymphoma, an initial panel of markers comprising of CD20, CD3, CD5, CD10, BCL6, and Ki67 was requested for. All the immunohistochemistry were negative and the tumor showed a high Ki67 proliferative index (90%). The negativity of specific B and T cell markers ([Figure 3]) raised a possibility of poorly differentiated carcinoma and anaplastic large cell lymphoma in our differentials. A secondary panel comprising of B lineage markers CD79a, Pax5, along with markers of anaplastic large cell lymphoma (CD30, ALK1), epithelial markers such as PCK, the thyroid transcription factor 1 marker for medullary carcinoma (calcitonin), along with leukocyte common antigen (LCA) was ordered. Except for faint reactivity to LCA, the neoplastic cells were negative for the entire battery of markers. Additional markers with CD138 showed strong positivity, along with MUM1 showing diffuse strong nuclear positivity, and CD56 was found to be negative ([Figure 4]a and b). Epstein-Barr virus (EBV) performed by rapid in-situ hybridization was negative. Staging and work-up with marrow evaluation indicated uninvolved marrow. Serum protein electrophoresis was normal, without any paraproteinemia. Serum albumin levels were normal (4.3 g/ml). On the basis of the clinical, morphological, and immunoprofile, the diagnosis of PBL was made.
Figure 3 CD20 negativity in neoplastic cells (×40).

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Figure 4 (a) CD138 strongly positive in neoplastic cells (×40). (b) MUM1 strong and diffuse positivity in neoplastic cells (×40).

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  Discussion Top

PBL is a rare and distinct B-cell neoplasm where the large cells have downregulated the B-cell differentiation program and express plasma cell markers. PBL accounts for 2.6% of non-Hodgkin's lymphoma in HIV-related neoplasm [2] , but is also increasingly being reported in immunocompetent individuals at unusual sites such as the lungs, stomach, cervical lymph nodes, nasal cavity, jejunum, anorectal junction, and larynx [3],[4] . Previous studies have described 80 cases of HIV-negative PBL, with 75% subset of cases occurring in an immunocompetent host [1] . The median age reported was 57 years, and with advancing age, there is a decrease in immune-surveillance, which could be a contributing pathogenetic mechanism for the plausible cause of this disease [5] .

PBL can present a significant diagnostic challenge on morphologic and immunohistochemical grounds. The important criterion in the diagnosis of these lymphoma is strong positivity of CD138, CD38, VSCD38, and MUM1 and weak/absent expression of CD20 and CD79a [6] . Our case showed weak LCA reactivity with strong CD138 and MUM1 expression. In the thyroid, the morphology and CD20-negative immunoprofile can be mistaken for poorly differentiated carcinoma, which is more frequent than the unusual PBL. In contrast, the other differential diagnosis that has considerable overlap both morphologically and phenotypically is extramedullary plasmablastic myeloma. Differentiating this from PBL is very important as the management of both these entities varies. Distinction requires evaluation of clinical findings such as serum elecrophoresis, Bence-Jones proteins, and bone lytic lesions, which are more common in extramedullary plasmablastic myeloma than PBL. Immunohistochemistry with CD56 is found to be specific in plasma cell neoplasm compared with PBL as reported by a study of Colomo et al. [7] who described CD56 positivity in one of 18 cases of PBL. Our patient also lacked expression of CD56.

PBL are usually associated with latent EBV, encountered in 60-75% of cases [8] . EBV infection can be detected by fluorescence in-situ hybridization for EBV-RNA or PCR. However, immunohistochemistry for LMP1 has been described to be less sensitive in detecting latent EBV infection [9] . EBV testing performed in this case by rapid in-situ hybridization was negative. Castillo and Reagan [10] have described EBV positivity in 82 and 46% of the HIV-positive and HIV-negative cases, respectively, by the in-situ hybridization technique. HIV-negative patients with PBL are known to have worse prognosis than their HIV-positive counterparts [1] . There are no definitive uniform guidelines for the treatment of such cases and the treatment regime varies on the basis of the clinician's discretion. Our patient was started on cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP regimen); he showed a dramatic response and the patient is on follow-up.

  Conclusion Top

To our knowledge, this is the first case of PBL being reported in the thyroid that warrants awareness in a CD20-negative setting.

  Acknowledgements Top

The authors would like to thank Mr Sambasiva Reddy for his technical support.

Conflicts of interest

There are no conflicts of interest.

  References Top

Castillo JJ, Winer ES, Stachurski D, Perez K, Jabbour M, Milanic C, et al. HIV negative plasmablastic lymphoma: not in the mouth. Clin Lymphoma Myeloma Leuk 2011; 11 :185-189.  Back to cited text no. 1
Folk GS, Abbondanzo SL, Childers EL, Foss RD. Plasmablastic lymphoma: a clinicopathologic correlation. Ann Diagn Pathol 2006; 10 :8-12.  Back to cited text no. 2
Brahmania M, Sylwesterowic T, Leitch H. Plasmablastic lymphoma in the ano-rectal junction presenting in an immunocompetent man: a case report. J Med Case Rep 2011; 5 :168.  Back to cited text no. 3
Stephenson K, Peer S, Govender D, Fagan JJ. Plasmablastic lymphoma of the larynx: report of two cases. J Laryngol Otol 2013; 127 :96-99.  Back to cited text no. 4
Liu JJ, Zhang L, Ayala E, Field T, Ochoa-Bayona JL, Perez L, et al. Human immunodeficiency virus (HIV)-negative plasmablastic lymphoma: a single institutional experience and literature review. Leuk Res 2011; 35 :1571-1577.  Back to cited text no. 5
Thakral C, Thomas L, Gajra A, Hutchisom RE, Ravizzini GC, Vajpayee N. Plasmablastic lymphoma in an immunocompetent patient. J Clin Oncol 2009; 27 :e78-e81.  Back to cited text no. 6
Colomo L, Loong F, Rives S, Pitaluga S, Martinez A, Lopez-Guillermo A, et al. Diffuse large B-cell lymphoma with plasmablastic differentiation represents a heterogenous group of disease entities. Am J Surg Pathol 2004; 28 :736-747.  Back to cited text no. 7
Delecluse HJ, Anagnostopoulos I, Dallenbach F, Hummel M, Marafioti T, Schneider U, et al. Plasmablastic lymphomas of the oral cavity: a new entity associated with the human immunodeficiency virus infection. Blood 1997; 89 :1413-1420.  Back to cited text no. 8
Dong HY, Scadden DT, de Leval L, Tang Z, Isaacson PG, Harris NL. Plasmablastic lymphoma in HIV-positive patients: an aggressive Epstein-Barr virus-associated extramedullary plasmacytic neoplasm. Am J Surg Pathol 2005; 29 :1633-1641.  Back to cited text no. 9
Castillo JJ, Reagan JL. Plasmablastic lymphoma: a systematic review. Scientific World Journal 2011; 11 :687-696.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

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