Urology Annals
About UA | Search | Ahead of print | Current Issue | Archives | Instructions | Online submissionLogin 
Urology Annals
  Editorial Board | Subscribe | Advertise | Contact
Users Online: 688   Home Print this page  Email this page Small font size Default font size Increase font size

Table of Contents
Year : 2015  |  Volume : 7  |  Issue : 4  |  Page : 530-533  

Plasmacytoma of the testis in a patient with relapsed and refractory multiple myeloma: Case report and review of the literature

1 Department of Surgery, University of Melbourne, Austin Hospital, Melbourne, Australia
2 Department of Pathology, Austin Hospital, Melbourne, Australia
3 Department of Cancer Medicine, Peter MacCallum Cancer Centre; Department of Oncology, Sir Peter MacCallum, University of Melbourne, Melbourne, Australia
4 Department of Oncology, Sir Peter MacCallum, University of Melbourne; Department of Radiation Oncology and Cancer Imaging, Peter MacCallum Cancer Centre, Melbourne, Australia
5 Department of Surgery, University of Melbourne, Austin Hospital; Olivia Newton-John Cancer Research Department, Austin Hospital; Department of Cancer Surgery, Peter MacCallum Cancer Centre, Australia

Date of Submission22-Feb-2015
Date of Acceptance10-Mar-2015
Date of Web Publication14-Oct-2015

Correspondence Address:
Munad Khan
Department of Surgery, Level 6, ONJ Building, Austin Hospital, 145 Studley Road, VIC 3084
Login to access the Email id

DOI: 10.4103/0974-7796.164844

PMID: 26692682

Rights and Permissions

Testicular plasmacytoma, whether occurring as a primary lesion or as a reflection of underlying multiple myeloma (MM), is a rare disease. We report the case of a 38-year-old male with multiply relapsed MM, who was found to have a testicular plasmacytoma. He presented with a gradually enlarging scrotal mass. Following orchidectomy, pathologic examination of the specimen demonstrated a plasmacytoma. In the context of active MM, the specimen was also sent for cytogenetic analysis but this was unhelpful in guiding a chemotherapy regime, which still continues at time of reporting. Although a rare lesion, there remains no definitive treatment protocol for the management of testicular plasmacytoma representing an extramedullary manifestation of MM.

Keywords: Extramedullary plasmacytoma, multiple myeloma, plasmacytoma, testis

How to cite this article:
Khan M, Rajarubendra N, Azer S, Skene A, Harrison SJ, Campbell B, Lawrentschuk N. Plasmacytoma of the testis in a patient with relapsed and refractory multiple myeloma: Case report and review of the literature. Urol Ann 2015;7:530-3

How to cite this URL:
Khan M, Rajarubendra N, Azer S, Skene A, Harrison SJ, Campbell B, Lawrentschuk N. Plasmacytoma of the testis in a patient with relapsed and refractory multiple myeloma: Case report and review of the literature. Urol Ann [serial online] 2015 [cited 2020 Oct 1];7:530-3. Available from: http://www.urologyannals.com/text.asp?2015/7/4/530/164844

   Introduction Top

Primary plasmacytomas are relatively uncommon lesions at diagnosis that may present as solitary lesions in bone (solitary bony plasmacytoma [SBP] or within soft tissues extramedullary plasmacytoma [EP]), but occur more frequently as a late manifestation of advanced heavily pretreated multiple myeloma (MM). Multiple solitary plasmacytomas are relatively rare and occur in up to 5% of patients with an apparently solitary plasmacytoma. Primary SBP and EP have been classified as distinct clinical entities within the plasma cell dyscrasia family by both the World Health Organization and International Myeloma Working Group [1],[2] and have a relatively good prognosis with local radiotherapy alone with 50–72% of SBP and 0–36% EP 10 years progression to myeloma.[3],[4],[5],[6],[7],[8],[9],[10],[11],[12],[13] Regardless of the association with underlying MM, plasmacytoma of the testis is very uncommon.[14],[15] Seventy-one cases of testicular plasmacytoma have been published up to 2008.[16] The majority of these present as extramedullary manifestations of MM (eMM). When dealing with testicular plasmacytoma, the distinction between primary testicular EP and eMM is important given the differences in prognosis and treatment pathways.[17]

We report here the case of a 38-year-old male with testicular plasmacytoma on a background of aggressive MM with numerous extramedullary localizations.

   Case Report Top

A 38-year-old Indian male presented with a gradually increasing lump in his right hemiscrotum. He had a history of multiply relapsed MM, manifesting as multiple plasmacytomas with minimal marrow infiltration, initially treated with thalidomide based induction therapy, tandem autologous, followed by reduced intensity allogeneic transplantation and multiple lines of salvage therapy with novel agents (including lenalidomide, pomalidomide and bortezomib) and sequential hemibody radiotherapy. Otherwise, his medical history was unremarkable and did not have any environmental or developmental risk factors. He did not complain of any other genitourinary symptoms although at the time of presentation had multiple soft tissue nodules reflecting active MM. Examination confirmed a mass in the right testis. This was noted to be metabolically active on a fluorodeoxyglucose positron emission tomography study, which incidentally demonstrated multiple avid lymph nodes [Figure 1]. Given his extensive burden of disease, several treatment options were discussed with the patient but in the end a right inguinal orchidectomy and lymph node dissection was performed to provide cytoreductive benefit along with the opportunity to run cytogenetic tests to guide further management.
Figure 1: Axial positron emission tomography/computed tomography image demonstrates an avid region in the right testicle

Click here to view

Macroscopically, the right testis was 68 mm × 35 mm × 35 mm and weighed 53 g. Normal testicular tissue was replaced by lobulated tan and hemorrhagic tumor components [Figure 2]. Microscopically, there were visible sheets of cells with plasmacytoid morphology [Figure 3]. The tumor appeared to infiltrate the epididymis but did not extend into the tunica vaginalis or proximal spermatic cord. The inguinal lymph node was also infiltrated with plasmacytoid tumor cells, with evidence of extra-nodal extension.
Figure 2: Macroscopic specimen showing a grossly enlarged testicle without breach of the tunica vaginalis or proximal spermatic cord

Click here to view
Figure 3: H and E staining demonstrating the tumor comprises a diffuse proliferation of atypical plasmacytoid cells, with abundant mitoses and apoptosis. (a) Low power (×40); (b) high power (×400)

Click here to view

Unfortunately, cytogenetic analysis failed but fluorescencein situ hybridization (FISH) was performed in conjunction with cytoplasmic immunoglobulin staining to positively identify plasma cells for analysis. There was no molecular cytogenetic evidence of the high risk (4;14), the t (14;16) or deletion of TP53 (17p13) abnormalities. However, FISH suggested an increased ploidy level in the plasma cell myeloma clone with four IGH (14q32), three FGFR3 (14p16), three MAF (16q32) and 4 chromosome 17 centromere signals per cell in the majority (79–100%) of cells scored.

   Discussion Top

Testicular plasmacytoma, whether occurring as a primary EP or an eMM, is a rare clinical entity. As a proportion of all primary and secondary testicular tumors, plasmacytoma is estimated to have an incidence between 0.03% and 0.1%.[14],[18] Even in the context of MM alone, the proportion of extramedullary manifestations in the testis ranges between 0.6% and 2.7%.[14] Given its rarity, it is important that all cases of testicular plasmacytoma are reported, to not only improve the understanding of eMM and EP involving the testis, but also to better understand the distinctions between EP and eMM in general.

Although rare, the underlying cause of a testicular plasmacytoma has prognostic implications. The far more common [19] secondary extramedullary spread into the testis from MM usually indicates aggressive disease and a poor clinical prognosis.[20] In contrast, primary EP of the testis appears to have a better prognosis, which is in keeping with EP at other sites.[21] However, given the small absolute number of cases of primary testicular EP reported, the overall prognosis needs to be confirmed by larger cohort studies.[13]

In light of the prognostic differences, it follows that treatment protocols for EP and eMM would differ. Although treatment options for plasma cell neoplasms are constantly evolving, systemic therapy (with or without local radiotherapy to a dominant testicular mass) is usually necessary in the case of eMM, whereas primary testicular EP should be treated by local therapies such as radiotherapy or resection. The treatment of unifocal testicular relapse of MM is less clear, but extended periods of remission from local therapy alone have been reported.[22] However, as with other solitary EP, having primary testicular EP does not preclude disease progression to other plasma cell neoplasms and there are multiple case reports of primary EP progressing to MM but given the small numbers the exact rate is unknown.[13],[23] The timeline for this progression can extend as far as several years and as such, these patients need to be closely monitored given the risk of developing subsequent generalized MM.[19],[23],[24]

Finally, despite the small incidence, eMM involving the testis may have a physiological basis. Testis involvement in myeloma and other hematological malignancies has been hypothesized to be partly attributable to the testes being a sanctuary site.[22] As has been noted by Rosenberg, this would be particularly important in the case of a solitary plasmacytoma in the testis without evidence of other myelomatous spread throughout the body. As an overall proportion, however, the absolute number of secondary solitary testicular plasmacytoma occurring as a manifestation of MM relapse is small [Figure 4]. The other interesting finding from the graph below is that although there were 14 cases of primary testicular plasmacytoma, seven of these went on to develop MM later and a further three were associated with plasmacytoma in other organs, making isolated primary testicular plasmacytoma very rare indeed.
Figure 4: Testicular plasmacytomas in the literature and their underlying diagnosis[13],[14],[16],[18],[19],[21],[22],[25],[26],[27],[28],[29],[30],[31],[32],[33],[34],[35],[36],[37],[38],[39],[40],[41],[42],[43],[44],[45],[46],[47],[48]

Click here to view

However, even in cases like ours, where a patient presents with a testicular mass in the context of active systemic disease following extensive treatment cytogenetic testing from such testicular masses may assist in delineating clonal evolution of the underlying myeloma.

   Conclusion Top

Testicular plasmacytoma is a very uncommon lesion. It appears to occur more commonly in the context of widespread MM in the context of aggressive end-stage disease rather than as a primary solitary clinical entity. Even when occurring in isolation, it remains important to screen and subsequently monitor patients with MM. Although it continues to carry a poor prognosis, cases of testicular plasmacytoma should be reported to facilitate the creation of a definitive treatment protocol.

Source of Support:


Conflict of Interest:


   References Top

International Myeloma Working Group. Criteria for the classification of monoclonal gammopathies, multiple myeloma and related disorders: A report of the International Myeloma Working Group. Br J Haematol 2003;121:749-57.  Back to cited text no. 1
Jaffe E, Harris N, Stein H, Varidman J. World Health Organization Classification of Tumours, Pathology and Genetics, Tumours of Haematopoietic and Lymphoid Tissues. Lyon: IARC Press; 2001.  Back to cited text no. 2
Ozsahin M, Tsang RW, Poortmans P, Belkacémi Y, Bolla M, Dinçbas FO, et al. Outcomes and patterns of failure in solitary plasmacytoma: A multicenter Rare Cancer Network study of 258 patients. Int J Radiat Oncol Biol Phys 2006;64:210-7.  Back to cited text no. 3
Katodritou E, Terpos E, Symeonidis AS, Pouli A, Kelaidi C, Kyrtsonis MC, et al. Clinical features, outcome, and prognostic factors for survival and evolution to multiple myeloma of solitary plasmacytomas: A report of the Greek myeloma study group in 97 patients. Am J Hematol 2014;89:803-8.  Back to cited text no. 4
Dagan R, Morris CG, Kirwan J, Mendenhall WM. Solitary plasmacytoma. Am J Clin Oncol 2009;32:612-7.  Back to cited text no. 5
Suh YG, Suh CO, Kim JS, Kim SJ, Pyun HO, Cho J. Radiotherapy for solitary plasmacytoma of bone and soft tissue: Outcomes and prognostic factors. Ann Hematol 2012;91:1785-93.  Back to cited text no. 6
Strojan P, Soba E, Lamovec J, Munda A. Extramedullary plasmacytoma: Clinical and histopathologic study. Int J Radiat Oncol Biol Phys 2002;53:692-701.  Back to cited text no. 7
Sasaki R, Yasuda K, Abe E, Uchida N, Kawashima M, Uno T, et al. Multi-institutional analysis of solitary extramedullary plasmacytoma of the head and neck treated with curative radiotherapy. Int J Radiat Oncol Biol Phys 2012;82:626-34.  Back to cited text no. 8
Reed V, Shah J, Medeiros LJ, Ha CS, Mazloom A, Weber DM, et al. Solitary plasmacytomas: Outcome and prognostic factors after definitive radiation therapy. Cancer 2011;117:4468-74.  Back to cited text no. 9
Creach KM, Foote RL, Neben-Wittich MA, Kyle RA. Radiotherapy for extramedullary plasmacytoma of the head and neck. Int J Radiat Oncol Biol Phys 2009;73:789-94.  Back to cited text no. 10
Chao MW, Gibbs P, Wirth A, Quong G, Guiney MJ, Liew KH. Radiotherapy in the management of solitary extramedullary plasmacytoma. Intern Med J 2005;35:211-5.  Back to cited text no. 11
Bachar G, Goldstein D, Brown D, Tsang R, Lockwood G, Perez-Ordonez B, et al. Solitary extramedullary plasmacytoma of the head and neck – Long-term outcome analysis of 68 cases. Head Neck 2008;30:1012-9.  Back to cited text no. 12
Anghel G, Petti N, Remotti D, Ruscio C, Blandino F, Majolino I. Testicular plasmacytoma: Report of a case and review of the literature. Am J Hematol 2002;71:98-104.  Back to cited text no. 13
Chica G, Johnson DE, Ayala AG. Plasmacytoma of testis presenting as primary testicular tumor. Urology 1978;11:90-2.  Back to cited text no. 14
Dores GM, Landgren O, McGlynn KA, Curtis RE, Linet MS, Devesa SS. Plasmacytoma of bone, extramedullary plasmacytoma, and multiple myeloma: Incidence and survival in the United States, 1992-2004. Br J Haematol 2009;144:86-94.  Back to cited text no. 15
Wang YM, Li FY, Luo JD, Li J, Xie LP, Yang GS. Testicular plasmacytoma: A case report and review of the literature. Chin Med J (Engl) 2008;121:956-8.  Back to cited text no. 16
Kremer M, Ott G, Nathrath M, Specht K, Stecker K, Alexiou C, et al. Primary extramedullary plasmacytoma and multiple myeloma: Phenotypic differences revealed by immunohistochemical analysis. J Pathol 2005;205:92-101.  Back to cited text no. 17
Levin HS, Mostofi FK. Symptomatic plasmacytoma of the testis. Cancer 1970;25:1193-203.  Back to cited text no. 18
Ferry JA, Young RH, Scully RE. Testicular and epididymal plasmacytoma: A report of 7 cases, including three that were the initial manifestation of plasma cell myeloma. Am J Surg Pathol 1997;21:590-8.  Back to cited text no. 19
Bataille R, Harousseau JL. Multiple myeloma. N Engl J Med 1997;336:1657-64.  Back to cited text no. 20
Yokogi H, Igawa M, Ishibe T, Maruyama R, Nagaoka S. Secondary plasmacytoma of the testis: Report of a case. Int Urol Nephrol 1996;28:559-62.  Back to cited text no. 21
Rosenberg S, Shapur N, Gofrit O, Or R. Plasmacytoma of the testis in a patient with previous multiple myeloma: Is the testis a sanctuary site? J Clin Oncol 2010;28:e456-8.  Back to cited text no. 22
Walker FB, Bluth EI, Kenney A, Beckman EN. Plasmacytoma of the testis. J Ultrasound Med 2005;24:1721-5.  Back to cited text no. 23
Castagna M, Gaeta P, Cecchi M, Pagni GL, Pingitore R. Bilateral synchronous testicular involvement in multiple myeloma. Case report and review of the literature. Tumori 1997;83:768-71.  Back to cited text no. 24
Hathaway AR. Incidental discovery of a testicular plasmacytoma at initial presentation of multiple myeloma. Case Rep Hematol 2013;2013:752921.  Back to cited text no. 25
Pow Sang M, Astigueta JC, Abad M, Sánchez J, León J. Testicular plasmacytoma as presentation of multiple myeloma: Case report and review of the literature. Arch Esp Urol 2013;66:242-8.  Back to cited text no. 26
Ahnach M, Marouan S, Rachid M, Madani A, Quessar A, Benchekroun S, et al. Extramedullary plasmocytoma relapsing at differents sites: An unusual presentation. Pan Afr Med J 2013;14:34.  Back to cited text no. 27
Takahashi M, Ito K, Sato K, Yoshii T, Umeda S, Shimazaki H, et al. Primary bilateral testicular plasmacytoma: A case report. Hinyokika Kiyo 2011;57:653-6.  Back to cited text no. 28
Berrondo C, Gorman TE, Yap RL. Primary plasmacytoma of the testicle: A case report. J Med Case Rep 2011;5:494.  Back to cited text no. 29
Valentini CG, Bozzoli V, Fianchi L, Voso MT, Di Paolantonio G, Criscuolo M, et al. Primary plasma cell leukemia followed by testicular plasmacytoma. Int J Hematol 2011;93:224-7.  Back to cited text no. 30
Matsuda Y, Kato R, Miyao N, Sato S, Konishi Y, Kon S. A case of extramedullary plasmacytoma of testis converted to multiple myeloma. Hinyokika Kiyo 2010;56:593-5.  Back to cited text no. 31
Turk HM, Komurcu S, Ozet A, Kuzhan O, Günhan O. An unusual presentation of extramedullary plasmacytoma in testis and review of the literature. Med Oncol 2010;27:1378-80.  Back to cited text no. 32
Hassen-Troudi F, Mege-Lechevallier F, Colombel M, Scoazec JY. Testicular localisation of a plasmocytoma. Ann Pathol 2009;29:32-5.  Back to cited text no. 33
Kamisawa H, Akita H, Naiki T, Kato T, Okamura T. Testicular metastasis from an extramedullary plasmacytoma. Int J Clin Oncol 2008;13:464-6.  Back to cited text no. 34
Shafqat A, Yum MN, Abanour R, Ganjoo KN. Unusual locations of involvement by malignancies: Case 1. Testicular plasmacytoma. J Clin Oncol 2003;21:3368-9.  Back to cited text no. 35
Hou TY, Dai MS, Kao WY. Testicular plasmacytoma with bone dissemination without medullary plasmacytosis. Ann Hematol 2003;82:518-20.  Back to cited text no. 36
Wellwood J, Taylor K, Wright S, Bentley M, Hill G, Phan H, et al. Relapsed plasma cell leukaemia presenting with aggressive extramedullary plasmacytomas in the meninges and testis – A missed opportunity for induction CNS prophylaxis. Leuk Lymphoma 2002;43:893-6.  Back to cited text no. 37
de Rose AF, Giglio M, Naselli A, Truini M, Carmignani G. Case report: Testicular secondary localization of a multiple myeloma. Int Urol Nephrol 2002;33:101-2.  Back to cited text no. 38
Fernandez LA, Couban S, Sy R, Miller R. An unusual presentation of extramedullary plasmacytoma occurring sequentially in the testis, subcutaneous tissue, and heart. Am J Hematol 2001;67:194-6.  Back to cited text no. 39
Suzuki K, Shioji Y, Morita T, Tokue A. Primary testicular plasmacytoma with hydrocele of the testis. Int J Urol 2001;8:139-40.  Back to cited text no. 40
Bude RO. Testicular plasmacytoma: Appearance on gray-scale and power Doppler sonography. J Clin Ultrasound 1999;27:345-6.  Back to cited text no. 41
Kahr WH, Al-Homadhi A, Meharchand J, Bailey DJ, Stewart AK. Testicular plasmacytoma following chemical orchiectomy: Potential role of hypogonadism in myeloma proliferation. Leuk Lymphoma 1998;28:437-42.  Back to cited text no. 42
Turhal N, Henehan MD, Kaplan KL. Multiple myeloma: A patient with unusual features including intracranial and meningeal involvement, testicular involvement, organomegaly, and plasma cell leukemia. Am J Hematol 1998;57:51-6.  Back to cited text no. 43
White J, Chan YF. Solitary testicular plasmacytoma. Br J Urol 1995;75:107-8.  Back to cited text no. 44
Kang MJ, Kim TY, Bang YJ, Heo DS, Park S, Kim BK, et al. Multiple myeloma presenting as a testicular mass. Korean J Intern Med 1994;9:39-42.  Back to cited text no. 45
Croft GV, Albertyn LE. Sonographic appearance of plasmacytoma of the testis. Australas Radiol 1992;36:265-7.  Back to cited text no. 46
Benson CB, Deligdish CK, Loughlin KR. Sonographic detection of testicular plasmacytoma. J Clin Ultrasound 1987;15:490-3.  Back to cited text no. 47
Steinberg D. Plasmacytoma of the testis. Report of a case. Cancer 1975;36:1470-2.  Back to cited text no. 48


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


    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Access Statistics
    Email Alert *
    Add to My List *
* Registration required (free)  

  In this article
   Case Report
    Article Figures

 Article Access Statistics
    PDF Downloaded255    
    Comments [Add]    

Recommend this journal