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Year : 2014  |  Volume : 57  |  Issue : 2  |  Page : 323-325
Solitary plasmacytoma of the metacarpal bone in an adolescent

1 Department of Pathology, Regional Cancer Centre, Thiruvananthapuram, Kerala, India
2 Department of Imageology, Regional Cancer Centre, Thiruvananthapuram, Kerala, India

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Date of Web Publication19-Jun-2014


Solitary plasmacytoma of the bone (SPB) is a plasma cell neoplasm that usually presents as a lytic lesion mainly localized within the axial skeleton. The occurrence of SPB in young individuals is exceedingly rare, but has been sporadically reported before. We report a case of SPB involving metacarpal bone in a 16-year-old male with a prior history of trauma at the same site.

Keywords: Metacarpal bone , plasmacytoma ,young adult

How to cite this article:
Jacob PM, Nair RA, Koshy SM, Kattoor J. Solitary plasmacytoma of the metacarpal bone in an adolescent. Indian J Pathol Microbiol 2014;57:323-5

How to cite this URL:
Jacob PM, Nair RA, Koshy SM, Kattoor J. Solitary plasmacytoma of the metacarpal bone in an adolescent. Indian J Pathol Microbiol [serial online] 2014 [cited 2023 Sep 27];57:323-5. Available from:

   Introduction Top

Plasmacytomas are localized neoplasms accounting for about 5-10% of all plasma cell dyscrasias and are characterized by clonal proliferation of plasma cells that display a range of morphologic features ranging from bland and mature to anaplastic forms. [1] They are not associated with anemia, hypercalcemia, renal failure, or bone marrow involvement, features that are usually seen in overt myelomas. They can present as osseous plasmacytomas (solitary plasmacytoma of bone [SPB]) or as extraosseous plasmacytomas (extramedullary) with a lower risk of progression to myeloma in the extraosseous constituent lesions. [1] The clinical course and prognosis of solitary bone plasmacytoma, extramedullary plasmacytoma, multiple solitary plasmacytoma and multiple myeloma differ, leading some to consider these as separate disease entities rather than a spectrum of the same disease. The axial skeleton is the most common location for osseous lesions, while the upper respiratory tract is the most common location for extramedullary lesions. [1] The male to female ratio is 2:1 and the median age at presentation for SPB is 55 years, which is approximately 10 years younger than patients presenting with multiple myeloma. A few cases of plasmacytoma affecting young individuals have been reported before, most of which represent SPB. [2],[3]

   Case REPORT Top

The present case is about a 16-year-old boy (a class XI student) who noticed a swelling in his right thumb after injury with a cricket ball while playing a game of cricket 3 months back. The swelling continued to persist, despite application of local anti-inflammatory agents. The swelling was painless and did not show accompanying discoloration or tenderness [Figure 1]. A subsequent X-ray of his right hand was done and revealed a lytic expansile lesion with a soap bubble appearance in the right metacarpal bone [Figure 2]a and b]. Further work-up, consisted of an magnetic resonance imaging, whole body nuclear scan and biopsy of the lytic lesion. The whole body scan was negative for any other lesions. A tru-cut biopsy was done from the metacarpal bone lesion which revealed sheets of plasmacytoid cells. A panel of immunohistochemical markers comprising leukocyte common antigen, desmin, synaptophysin, MIC-2, CD138, CD56, kappa light chain and lambda light chain were performed on 5-mm, formalin fixed, paraffin-embedded sections and processed using automated immunostaining with the Ventana ultraView DAB detection kit in a Ventana BenchMark XT processor (Ventana Medical Systems, Tucson, AZ). All the immunohistochemistry antibodies were from Dakocytomation, Denmark, RTU except CD56 which was from Novacastra, UK, RTU. The metacarpal bone biopsy revealed sheets of kappa restricted CD138 and CD56 positive plasma cells [Figure 3]a-f]. Epstein-Barr virus (EBV) encoded ribonucleic acid (RNAs) (EBER) by RNA in situ hybridization (Biocare Medical) was done and was negative. The patients blood counts were as follows, hemoglobin = 14.4 g%, total leukocyte count = 9200 cells/mm 3 and platelet count = 245 10 6 /L. Serum calcium was 10.1 mg/dL. His peripheral smear, bone marrow aspirate and trephine were within the normal limits. Urine Bence-Jones protein were negative. Immunoglobulin assay was done, with the following values. Immunoglobulin G = 1798 mg/dL (normal range = 800-1800), IgA = 279 mg/dL (normal range = 90-450), immunoglobulin M = 99 mg/dL (normal range = 60-280). Free kappa lambda light chain ratio was 1.3 (normal range = 0.26-1.65). Human immunodeficiency virus, hepatitis B surface antigen and hepatitis C virus (HCV) were tested for and were negative. Based on the immunohistochemical findings and the clinical picture, a diagnosis of SPB was made. The patient has been taken up for localized radiotherapy.
Figure 1: Clinical picture with swelling at the base of right thumb

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Figure 2: (a) Plain radiograph of the left hand frontal view. An expansile multiloculated lytic lesion with "soap bubble" appearance involving the first metacarpal bone with focal cortical irregularities, no significant adjacent soft-tissue component. (b) Corresponding axial computed tomography sections through the hand at the level of the first metacarpal bone shows the expansile lytic lesion with multiple cortical defects and irregularity with minimal adjacent soft-tissue

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Figure 3: (a) H and E, ×200 section from neoplasm showing sheets of plasma cells. (b) H and E, ×400 plasma cells with abundandant cytoplasm, eccentrically placed nuclei. (c) Tumor cells are CD138
positive. (d) Tumor cells are CD56 positive. (e) Tumor cells show kappa light chain restriction. (f) Tumor cells are lambda negative

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   Discussion Top

The occurrence of a SPB in young individuals is exceedingly rare but has been reported previously. [2],[3] A total of 13 cases of plasmacytoma in young adults have been reported to our knowledge. All these patients, including our reported case were <30 years old at presentation and presented with lytic lesions, mostly in the spine (6 cases), while three patients had tibial lesions, two patients had a rib lesion and one patient had a lesion on the vertex of the head. In SPB, irrespective of age, long bone involvement below the elbow or knee is rare. [2] Plasmacytomas (as with multiple myeloma) are typically seen as well-defined, "punched-out" lytic lesions with or without associated soft-tissue masses, similar in appearance to most metastatic lesions. In some cases, there can be marked erosion, expansion and destruction of bone cortex, sometimes with thick ridging around the periphery, creating a "soap bubble" appearance. It should be considered in the differential diagnosis of expansile lytic lesions with "soap bubble" appearance, in the appropriate clinical setting. Plasmacytoma comes in the radiological differential diagnosis of "bubbly" benign, cystic lytic bone lesions, easily remembered by the mnemonic FEGNOMASHIC which is a list of 14 entities. This mnemonic has been in use for many years and there is no definite claim as to who first coined it. F = Fibrous dysplasia, E = Enchondroma, eosinophilic granuloma, G = Giant cell tumor, N = Non-ossifying fibroma, O = Osteoblastoma, M = Metastasis, multiple myeloma, A = Aneurysmal bone cyst, S = Solitary bone cyst, H = Hyperparathyroidism (brown tumor), I = Infection, C = Chondroblastoma, chondromyxoid fibroma. Interestingly, FEGNOMASHIC is defined in "Funk and Wagners" unabridged dictionary 13 th edition as "one who uses mnemonics." [4]

New insights on etiology

The etiology of plasmacytoma is still unknown and different pathways and stimuli seem to be involved. Recently, several hypothesis has been underlined, including a possible role of viral infection, especially hepatitis C. [5] A few studies have shown that 6.7% plasmacytomas are EBV positive in contrast to >50% of plasmablastic lymphoma associated with EBV. [6],[7] In our case, the patient was HCV negative and EBV negative by EBER. A study by Pasch et al. explores the possibility for a role of preceding trauma. They reviewed all previous reports of plasmacytoma in young adults and found that majority of cases had a history of preceding trauma at the tumor site. The time from the occurrence of the trauma to the onset and the diagnosis of SPB varied from a couple of weeks to 12 years in the reviewed cases. They hypothesized that as trauma can cause enhanced release of cytokine, this can result in increased proliferation of plasma cells and stromal cells in the bone leading onto development of a plasmacytoma. Given that interleukin-6 is a well-known inflammatory cytokine that promotes B-cell proliferation occurring during bone marrow stimulation, the authors postulated that this creates a "feed-forward cytokine amplification loop" in which the tumor environment becomes ideal for plasma cell neoplasm progression. [3],[8] Trauma of any sort can trigger injured cells to secrete these inflammatory cytokines, which can sometimes lead to plasmacytoma development. Zendri et al. in their study have reported a case of a primary cutaneous plasmacytoma of the lower lip in a patient who had recurrent herpes simplex virus infections that were proposed as trauma trigger. [3],[9] Although one can argue that the presence of trauma may represent a circumstantial coincidence with SBP, but given the rarity of such lesions in younger individuals and the identification of a preceding trauma or chronic stimulation in a considerable number of the reported cases, they speculated that trauma may play a role in the pathogenesis of such lesions and may act as the triggering stimulus for plasma cells to proliferate and eventually lead to a clonal infiltrate. Further studies are needed to confirm such a hypothesis. [3]

   Conclusion Top

In summary, we describe a case of SPB in metacarpal bone in a 16-year-old boy. This case is being reported for its rarity.

   References Top

1.McKenna RWKR, Kuehl WM, Grogan TM, Harris NL, Coupland RW. Plasma cell neoplasms. In: Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, et al., editors. WHO Classification of Tumors of Hematopoietic and Lymphoid Tissues. Lyon: IARC; 2008.  Back to cited text no. 1
2.Rago A, Miraglia A, Mecarocci S, Pisanelli GC, Chiappetta C, Di Cristofano C, et al. Solitary plasmacytoma of tibia: A possible correlation with younger age. Leuk Res 2010;34:e181-2.  Back to cited text no. 2
3.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. 3
4.Helms CA. Benign cystic lesions. In: Fundamentals of Skeletal Radiology. Philadelphia: Elsevier; 2005.  Back to cited text no. 4
5.Di Micco P, Niglio A, Torella R, Di Micco B. Solitary plasmacytoma of the jaw occurring in an elderly woman affected by hepatitis C virus infection: A case report. Tumori 2002;88:420-3.  Back to cited text no. 5
6.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;89:1413-20.  Back to cited text no. 6
7.Hummel M, Anagnostopoulos I, Korbjuhn P, Stein H. Epstein-Barr virus in B-cell non-Hodgkin's lymphomas: Unexpected infection patterns and different infection incidence in low- and high-grade types. J Pathol 1995;175:263-71.  Back to cited text no. 7
8.Rutsch S, Neppalli VT, Shin DM, DuBois W, Morse HC 3 rd , Goldschmidt H, et al. IL-6 and MYC collaborate in plasma cell tumor formation in mice. Blood 2010;115:1746-54.  Back to cited text no. 8
9.Zendri E, Venturi C, Ricci R, Giordano G, De Panfilis G. Primary cutaneous plasmacytoma: A role for a triggering stimulus? Clin Exp Dermatol 2005;30:229-31.  Back to cited text no. 9

Correspondence Address:
Rekha A. Nair
Department of Pathology, Regional Cancer Centre, Thiruvananthapuram, Kerala
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.134732

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