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Year : 2012  |  Volume : 55  |  Issue : 1  |  Page : 104-106
Plasmablastic extramedullary plasmacytoma

Department of Oral and Maxillofacial Pathology, Tamil Nadu Government Dental College and Hospital, Chennai, Tamil Nadu, India

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Date of Web Publication11-Apr-2012


Extramedullary plasmactyoma is the solitary, soft tissue form of plasma cell neoplasm but lack the defining features of medullary or multiple myeloma. The diagnosis is difficult to make in routine practice setting due to the morphological and immunohistochemical overlap with plasmablastic lymphoma. We report a case of plasmablastic extramedullary plasmacytoma in a 52-year-old in the mandibular lingual gingiva and discuss its differential from plasmablastic lymphoma. The gingival mass regressed with primary radiotherapy.

Keywords: Extramedullary, gingiva, lymphoma, plasmablastic, plasmacytoma

How to cite this article:
Ponniah I, Rajan S. Plasmablastic extramedullary plasmacytoma. Indian J Pathol Microbiol 2012;55:104-6

How to cite this URL:
Ponniah I, Rajan S. Plasmablastic extramedullary plasmacytoma. Indian J Pathol Microbiol [serial online] 2012 [cited 2022 Nov 28];55:104-6. Available from:

   Introduction Top

Plasmactyoma exists in three clinical forms, viz., multiple myeloma (MM), medullary plasmacytoma (MP), and extramedullary plasmacytoma (EMP). [1] The latter is a localized collection of tumor cells in the soft tissue with plasma cell differentiation, but without evidence of the former two or lymphoma. [1] The adjective "plasmablastic" is used when there are more than 30% of plasmablasts in routine sections. [2] We report a case of plasmablastic EMP in the gingiva of a 52-year-old male and discuss its primary differential diagnosis.

   Case Report Top

A 52-year-old apparently healthy male presented with a painless, gingival mass of 3 months duration. His past medical history was not significant. He did not give any history of fever, night sweats, chills, or weight loss. On examination, a palpable cervical lymph node was noted on the right side of the neck. Intra-orally, a reddish, smooth surfaced, soft tissue mass involving the gingival/alveolar complex in relation to the right second mandibular molar, edentulous third molar, and retromolar region was noted [Figure 1]. There was no tooth mobility or bleeding on provocation. Panoramic and lateral skull radiographs revealed no bone lesion.
Figure 1: The clinical appearance of the lesion at presentation

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An incisional biopsy was performed under the impression of squamous cell carcinoma, which microscopically showed sheets of medium to large-sized cells with variable cytoplasm, eccentric nuclei with dense to light chromatin, variable perinuclear hof, single prominent nucleoli, binucleated cells, and occasional mitotic figures. The description indicates varying levels of cellular maturity [Figure 2]. The microscopical differential diagnosis included plasmablastic lymphoma (PBL) and plasmacytoma. Fine needle aspiration cytology of the cervical lymph node suggested lymphoma.
Figure 2: Neoplastic plasma cells with varying degrees of maturation with plasmacytes (arrow), intermediate form and plasmablasts (arrow head) in A, and plasmablasts in B (H and E; ×240)

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Complete blood count and biochemical values were within normal limits. Serum and urine electrophoresis for gammoglobulins and Bence-Jones protein were negative. Enzyme-linked immunosorbent assay for HIV was negative. No abnormalities were detected on skeletal survey. Plain chest radiograph and abdominal computed tomography scan were normal. No abnormalities were detected on bone-marrow examination.

A panel of commercially available immunohistochemical markers [Table 1] was used to characterize the lesion as to whether it was PBL or EMP, but the immunoprofile of the current case is consistent with EMP [Figure 3] and is also not inconsistent with PBL. The slides were interpreted by three pathologists, with and without immunohistochemistry, but no specific diagnosis as to PBL or EMP was rendered by each of them. Therefore, the diagnosis of EMP was based on the clinical and laboratory parameters in conjunction with morphological and immunohistochemcial features. The patient was treated initially by radiotherapy (40 Gy in 20 fractions over 4 weeks) followed by conditioning chemotherapy with oral mephalan plus thalidomide (six 4-week cycle). Follow-up showed regression of the oral lesion [Figure 4]. No changes were noted on laboratory investigation.
Figure 3: (a) Positive reaction with CD 138. (b) Positive reaction with Lambda. (c) Negative reaction with Kappa. (d)-(f) Positive reaction with CD 43, Ki-67, and CD 45 (IHC, ×400)

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Figure 4: Resolution of oral lesion after radiotherapy

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   Discussion Top

EMP is rare in the head and neck region, with an incidence of EMP to MM as 1:80 per year. [3] Within the head and neck region, EMP very rarely occurs on the gingiva [4] and about 75-85% are found in the upper aerodigestive tract with sites of predilection in the nasal cavity, paranasal sinuses, and nasopharynx. [3] Men are affected more frequently than women with peaks in the sixth to eighth decade of life. The disease may present as a localized mass, with lymph node involvement being not uncommon at the time of diagnosis. [3]

Histologically, the presence of neoplastic proliferation with features of mature plasma cells can establish a morphological diagnosis of EMP, but the diagnosis will be complicated when cytological variants coexist within the tumor infiltrates. This is especially true with PBL, which share similar features of monomorphic population of large cells (plasmablastic or immunoblastic) characterized by more or less eccentrically placed nucleus, little chromatin and single prominent centrally located nucleoli, or multiple peripherally located small nucleoli together with the presence of mature plasma cells. [5],[6] However, the presence of cells with obvious plasmacytic differentiation is not typical of PBL, [7],[8] but is the defining feature of EMP [Figure 2]. [5],[8]

The immunohistochemical panel used in the present case [Table 1] and Figure 3] may well exclude other tumors characterized by plasma cell cytology, but is unlikely to establish a diagnosis when the differential is PBL and EMP as both share identical immunophenotypes. [5],[8] The low Ki-67 proliferation index (20-25%) is consistent with EMP compared to higher expression in PBL (more than 80%). [7],[9] Though in situ hybridization for EBER was not done in the present case, the reliance on EBER status to resolve the diagnosis of a case in routine practice is conflicting, [2],[7],[8] especially in non-HIV patients. [9]
Table 1: Shows the panel of immunohistochemical markers employed

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The foregoing indicates that distinction between the two competing diagnoses may be best resolved when morphology is supplemented with clinical parameters such as aggressiveness, gingival presentation, and HIV status. [5],[6],[7],[9],[10] Interestingly, Kane et al. [7] is of the view that "plasmablastic EMP presenting as a gingival mass is more of a theoretical possibility rather than a practical problem." Although this is likely to be true in PBL in HIV positive patients, oral involvement is considered uncommon in non-HIV patients. [9] Moreover, PBL with light chain restrictions are considered as plasmablastic EMP. [6]

The lymph node involvement as noted in the present case has been reported to develop in 8% of EMP but has no prognostic implications. [3] Radiotherapy is the mainstay of treatment as it is a radiosensitive tumor. The optimal radiation dose adopted is in the range of 40-60 Gy given over a period of 4-6 weeks. [1] Good remission of the disease was achieved in the present case with primary radiotherapy. It has been reported that the type of light chain restriction has prognostic significance with better outcome when it is lambda light chain restriction, [3] a finding also found in the present case. [3]

In conclusion, we report a rare case of plasmablastic EMP in the gingiva with light chain restrictions that can be distinguished from PBL if subtle attention is paid to the morphological and clinical detail.

   References Top

1.Alexiou C, Kau RJ, Dietzfelbinger H, Kremer M, Spiess JC, Arnold W. Extramedullary plasmacytoma: Tumor occurrence and therapeutic concepts. Cancer 1999;85:2305-14.  Back to cited text no. 1
2.Chang ST, Liao YL, Lu CL, Chuang SS, Li CY. Plasmablastic cytomorphologic features in plasma cell neoplasms in immunocompetent patients are significantly associated with EBV. Am J Clin Pathol 2007;128:339-44.  Back to cited text no. 2
3.Susnerwala SS, Shanks JH, Banerjee SS, Scarffe JH, Farrington WT, Slevin NJ. Extramedullary plasmacytoma of the head and neck region: Clinicopathological correlation in 25 cases. Br J Cancer 1997;75;921-7.  Back to cited text no. 3
4.Moshref M, Mashhadi-Abbass F, Sargolzaei S, Nafarzadeh S. Extramedullary plasmacytoma of the gingiva. Arch Iranian Med 2007;10:91-3.  Back to cited text no. 4
5.Colomo L, Loong F, Rives S, Pittaiuga S, Martinez A, Lopez-Guillermo A, et al. Diffuse large B-cell lymphomas with plasmablastic differentiation represent a heterogenous group of disease entities. Am J Surg Pathol 2004;28:736-47.  Back to cited text no. 5
6.Boy SC, van Heerden MB, Raubenheimer EJ, van Heerden WF. Plasmablastic lymphomas with light chain restriction - plasmablastic extramedullary plasmacytomas? J Oral Pathol Med 2010;39:435-9.  Back to cited text no. 6
7.Kane S, Khurana A, Parulkar G, Shet T, Prabhash K, Nair R, et al. Minimum diagnostic criteria for plasmablastic lymphoma of oral/sinonasal region encountered in a tertiary cancer hospital of a developing country. J Oral Pathol Med 2009;38:138-44.  Back to cited text no. 7
8.Vega F, Chang CC, Medeiros LJ, Udden MM, Cho-Vega JH, Lau CC, et al. Plasmablastic lymphomas and plasma cell myeloma have nearly identical immunophenotypic profiles. Mod Pathol 2005;18:806-15.  Back to cited text no. 8
9.Castillo JJ, Winer ES, Stachurski D, Perez K, Jabbour M, Milani C, et al. Clinical and pathological differences between human immunodeficiency virus-positive and human immunodeficiency virus-negative patients with plasmablastic lymphoma. Leuk Lymphoma 2010;51:2047-53.  Back to cited text no. 9
10.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;29:1633-41.  Back to cited text no. 10

Correspondence Address:
Irulandy Ponniah
Department of Oral and Maxillofacial Pathology, Tamil Nadu Government Dental College and Hospital, Chennai - 600 003, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.94874

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

  [Table 1]

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