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  Table of Contents    
LETTERS TO EDITOR  
Year : 2018  |  Volume : 61  |  Issue : 1  |  Page : 158-159
Primary orbital plasmacytoma: An unusual presentation in an adolescent


1 Department of Immunopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
2 Department of Histopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
3 Department of Neurosurgery, Postgraduate Institute of Medical Education and Research, Chandigarh, India

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Date of Web Publication22-Mar-2018
 

How to cite this article:
Sharma S, Kaur R, Bal A, Mohindra S, Aggarwal R. Primary orbital plasmacytoma: An unusual presentation in an adolescent. Indian J Pathol Microbiol 2018;61:158-9

How to cite this URL:
Sharma S, Kaur R, Bal A, Mohindra S, Aggarwal R. Primary orbital plasmacytoma: An unusual presentation in an adolescent. Indian J Pathol Microbiol [serial online] 2018 [cited 2019 Dec 8];61:158-9. Available from: http://www.ijpmonline.org/text.asp?2018/61/1/158/228170




Editor,

We read with great interest the article by Misra et al.[1] and would like to share an interesting case of primary orbital plasmacytoma occurring in young age. Solitary plasmacytomas (SP) constitute 5%–10% of all plasma cell dyscrasias, mean age at presentation being 50–58 years. Isolated orbital involvement without systemic manifestations is rare.[2] We present a case of SP of orbit in a young boy with prior trauma being the possible triggering factor.

A 19-year-old boy presented with a rapidly progressive mass in the superolateral part of orbit involving right frontal region. The patient reported a history of trauma at the same site 1½ years back. Magnetic resonance imaging was performed which revealed a heterogeneous nonencapsulated mass of altered signal intensity in the lateral wall of right orbit, sparing the lacrimal gland and molding the orbital structures with intracranial extension to right frontal region. The previous biopsy performed was reported as sarcoma. The mass recurred a year later, postexcision. An incisional biopsy performed in our institute showed diffuse sheets of plasmacytoid cells with eccentric nucleus and abundant cytoplasm [Figure 1]. On immunohistochemistry, the tumor cells were positive for CD38, CD138 and showed kappa light chain restriction. Vimentin, desmin, and myogenin were negative. A diagnosis of plasmacytoma was rendered. Serum protein electrophoresis exhibited a prominent “M band” in gamma region. Immunofixation electrophoresis was reported as monoclonal gammopathy, IgG kappa [Figure 2]. Urine protein electrophoresis did not reveal any abnormal proteins. The hematological indices, serum calcium, uric acid, and renal function tests were within normal limits. Bone marrow aspirate revealed 6% plasma cells with no plasma cell aggregates on biopsy. Skeletal survey was normal. Overall findings were of solitary extramedullary plasmacytoma. The orbital mass was excised and the patient was planned for 40 Gy of external beam radiotherapy.
Figure 1: Histopathologic examination and immunohistochemical findings. (a) Diffuse sheets of tumor cells (H and E, ×40). (b) Higher magnification showing tumor cells with eccentric nuclei and eosinophilic cytoplasm (H and E, ×400). (c) Tumor cells positive for CD138, kappa light chain (d), and negative for lambda light chain (e) and vimentin (f)

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Figure 2: (a) Serum protein electrophoresis with a prominent M band in gamma region (highlighted by arrow). (b) Immunofixation electrophoresis confirming monoclonal gammopathy, IgG kappa

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SP are clonal proliferations of plasma cells manifesting as localized growth in the absence of a systemic plasma cell disorder. Orbital plasmacytomas are rare with only a handful of cases reported in literature, and presentation at <20 years of age is extremely rare.[3] The mean survival in SP is 8.3 years whereas that in multiple myeloma (MM) is only 20 months.[4] Hence, systemic involvement needs to be excluded in a case of suspected plasmacytoma, and a long-term follow-up is required to check for progression. Radiotherapy is the treatment of choice.

The preceding history of trauma in the index case is intriguing. Pasch et al. studied 13 cases of SP in young individuals, of which 8 had a history of trauma. Trauma is postulated to trigger the injured cells to secrete inflammatory cytokines, especially interleukin-6 which could promote progression to plasma cell neoplasm.[5]

To summarize, a case of solitary orbital plasmacytoma is being reported due to its rarity in an adolescent. Plasmacytoma should be kept in the differential diagnosis of orbital mass even in young age. A preceding trauma plausibly plays a role in the pathogenesis. A long-term follow-up is warranted as some of these cases may eventually progress to MM.

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

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Misra A, Bakhshi S, Kumar R, Chopra A. Pediatric plasmablastic lymphoma: Diagnostic and therapeutic dilemma. Indian J Pathol Microbiol 2017;60:303-4.  Back to cited text no. 1
[PUBMED]  [Full text]  
2.
Chattapadhyay S, Saha A, Mukherjee A, Azam M. Extramedullary plasmacytoma of the orbit: A rare case. Clin Cancer Invest J 2013;2:163-5.  Back to cited text no. 2
    
3.
Brannan PA, Cetinkaya A, Kim AS, Palkovacs EM. Solitary orbital plasmacytoma associated with chronic hepatitis C: A case report. Orbit 2009;28:71-3.  Back to cited text no. 3
    
4.
Soutar R, Lucraft H, Jackson G, Reece A, Bird J, Low E, et al. Guidelines on the diagnosis and management of solitary plasmacytoma of bone and solitary extramedullary plasmacytoma. Br J Haematol 2004;124:717-26.  Back to cited text no. 4
    
5.
Pasch W, Zhao X, Rezk SA. Solitary plasmacytoma of the bone involving young individuals, is there a role for preceding trauma? Int J Clin Exp Pathol 2012;5:463-7.  Back to cited text no. 5
    

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Correspondence Address:
Ritu Aggarwal
Department of Immunopathology, Postgraduate Institute of Medical Education and Research, Research Block-A, 4th Floor, Chandigarh - 160 012
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/IJPM.IJPM_378_17

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