|Year : 2015 | Volume
| Issue : 4 | Page : 923-924
Primary extramedullary plasmacytoma of ovary: Report of a rare neoplasm
Santosh Kumar Mondal1, Shankha Chatterjee2, Saikat Mandal2, Debasish Bhattacharjee1
1 Department of Pathology, B.S. Medical College, Bankura, West Bengal, India
2 Department of Pathology, Medical College, Kolkata, West Bengal, India
|Date of Web Publication||15-Feb-2016|
Santosh Kumar Mondal
"Teenkanya Complex", Flat 1B, Block B, 204 R N Guha Road, Dumdum, Kolkata - 700 028, West Bengal
Source of Support: None, Conflict of Interest: None
Primary extramedullary plasmacytoma is a rare plasma cell neoplasm. Extramedullary plasmacytomas are most commonly found in head and neck region, but it can occur at other sites occasionally. Involvement of ovary by this tumor is exceedingly rare. Here, we report a case of primary ovarian plasmacytoma in a 47-year-old woman. The p atient presented with a lower abdominal pain and a left ovarian mass (12 cm × 10 cm) was detected during the ultrasonographic examination. The patient underwent hysterectomy with bilateral salpingo-oophorectomy. Subsequent histopathologic, immunohistochemistry, bone marrow examination, and other relevant examinations established the diagnosis of primary ovarian plasmacytoma. The patient did not receive the postoperative chemotherapy and 6 months follow-up was uneventful.
Keywords: Ovary, plasmacytoma, primary neoplasm
|How to cite this article:|
Mondal SK, Chatterjee S, Mandal S, Bhattacharjee D. Primary extramedullary plasmacytoma of ovary: Report of a rare neoplasm. J Can Res Ther 2015;11:923-4
| > Introduction|| |
Plasmacytomas are malignant tumors of plasma cells; characterized by the clonal proliferation of plasma cells which produces monoclonal immunoglobulins. Plasmacytomas can be subclassified as an osseous disease or an extraosseous/extramedullary tumor.  Extramedullary plasmacytomas (EMP) comprises 3-5% of all plasma cell tumors.  EMP typically arises from the muscle, fat, mucosal surfaces; common sites include head and neck region, respiratory and digestive tracts with >80% situated above the diaphragm.  Other common sites are a lymph node, skin, liver, kidney, and very rarely ovary.
EMP typically affects the middle aged persons (median 55-60 years) with a male: female ratio of 3:1. Confirmed risk factors for EMP remain unknown; however, prior radiation exposure has been suggested. These patients have a higher rate of progression to multiple myeloma/disseminated disease and they require close monitoring after appropriate treatment. 
| > Case report|| |
A 47-year-old woman presented with lower abdominal pain for the last 3 months. Subsequent ultrasonography (USG) revealed a large left ovarian cyst measuring 12 cm × 10 cm. Routine hematological and biochemical reports were within the normal limits. Chest X-ray was also unremarkable. The patient underwent hysterectomy with bilateral salpingo-oophorectomy.
Grossly, uterus and cervix measured 10.5 cm × 5.5 cm × 4 cm. Left ovarian cyst measured 10 cm × 9.5 cm × 6 cm. Cut section revealed the hypertrophic myometrium. On cut opening the ovarian mass, thin yellowish colored fluid came out. Inner wall showed multiple tiny nodules and striations. The maximum thickness of cyst wall is 2.1 cm. The necrotic material was seen within the thickened portion of cyst wall [Figure 1]a].
|Figure 1: (a) Gross examination of left ovarian cyst wall showing multiple tiny nodules and striation. (b) High power view is showing plasma cells arranged in sheets (H and E, ×400). (c) Oil immersion view is showing plasma cells with multiple grape such as cytoplasmic vacuoles or mott cell, marked by arrows (H and E, ×1000). (d) Low power view of CD138 immunomarker staining showing the diffuse cytoplasmic membrane positivity (×100)|
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Microscopy revealed dense infiltration of plasma cell into cyst wall. The plasma cells were arranged in diffuse sheets. Few reactive lymphocytes were seen interspersed between the plasma cells. Many binucleated and a few multinucleated plasma cells were noted. Occasional plasma cells were noted and it is filled with cytoplasmic vacuoles [Figure 1]b and c].
Cervix was unremarkable and endometrium showed simple hyperplasia. Based on the morphological features, a provisional diagnosis of plasmacytoma was given and immunohistochemistry (IHC) was suggested for the confirmatory diagnosis. IHC showed a plasmacytoid neoplasm positive for CD138, CD38, and leukocyte common antigen (CD45) [Figure 1]d]. The tumor cells were negative for cytokeratin, inhibin, synaptophysin, Desmin, and CD99.
Bone marrow examination and peripheral blood did not show the increased plasma cells. Serum/urinary M protein or urinary Bence Jones protein was not detected. Thus, a confirmatory diagnosis of primary EMP of the ovary was established.
No adjuvant chemotherapy or radiotherapy was given. Six months follow-up was uneventful and the patient continues to do well without the evidence of recurrence.
| > Discussion|| |
Multiple myeloma primarily involves the bone marrow, is a systemic monoclonal plasma cell neoplasm. EMP is rare, and almost 90% cases occur in head and neck, where they typically exhibit a close relationship to mucus membranes. EMP seldom involves the female reproductive organs, even more rarely in the ovaries. 
To differentiate between EMP and multiple myeloma, a bone marrow examination is necessary which should have <10% plasma cells with no dyscrasia and normal skeletal survey in EMP. 
EMP usually do not exhibit serum M protein or Bence Jones light chains in urine, unlike multiple myeloma. However, EMP may show M protein in upto 25% cases. But in our case, neither M protein nor Bence Jones protein was present.
Shakuntala et al. reported an ovarian plasmacytoma which presented as large (15 cm × 15 cm) right adnexal mass. USG showed a solid heterogenous mass with increased vascularity on color doppler.  They also reviewed nine reported cases of ovarian plasmacytoma, all of them were either solid cystic or solid mass excepting the case reported by Zhong et al. 
Our case was also presented as a cyst which is an unusual presentation. Most of the cases, ovarian EMP was seen in elderly persons. Only a child (12 years) have had this disease till date. 
Most patients with ovarian EMP are treated with surgery with/without adjuvant therapy (chemotherapy or radiotherapy). In case of solitary pulmonary EMP, surgical resection is the best choice.  However, in case of diffuse pulmonary involvement, chemotherapy (melphalan with prednisolone) is the treatment of choice.  In 25% of such case, combined chemotherapy and radiotherapy proved to be useful. But the long-term results of single chemotherapy or radiotherapy have been significantly worsen.
Recently, three novel agents have been introduced in the management of multiple myeloma: Thalidomide, lenalidomide, and bortezomib.  Zhong suggested the postoperative treatment with combined bortezomib and dexamethasone, etoposide, cyclophosphamide, and cisplatin chemotherapy to control the disease progression. ,
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| > References|| |
Schor AP, Moraes MP, Bisson FW, Bisson MA, Luiz OM, Bacchi CE. Primary plasmacytoma of the cervix in a 21-year-old female patient. Int J Gynecol Pathol 2010;29:290-3.
Ujiie H, Okada D, Nakajima Y, Yoshino N, Akiyama H. A case of primary solitary pulmonary plasmacytoma. Ann Thorac Cardiovasc Surg 2012;18:239-42.
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.
Zhong YP, Zhang JJ, Huang XN. Multiple myeloma with rupture of ovarian plasmacytoma. Chin Med J (Engl) 2012;125:2948-50.
Shakuntala P, Praveen S, Shankaranand B, Rajshekar K, Umadevi K, Bafna U. A rare case of plasmacytoma of the ovary: A case report and literature review. Ecancermedicalscience 2013;7:288.
Andze G, Pagbe JJ, Tchokoteu PF. Solitary extraovarian plasmacytoma. J Chir 1993;130:137-40.
Niitsu N, Kohri M, Hayama M, Nakamine H, Nakamura N, Bessho M, et al.
Primary pulmonary plasmacytoma involving bilateral lungs and marked hypergammaglobulinemia: Differentiation from extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue. Leuk Res 2005;29:1361-4.
Richardson PG, Barlogie B, Berenson J, Singhal S, Jagannath S, Irwin D, et al.
A phase 2 study of bortezomib in relapsed, refractory myeloma. N Engl J Med 2003;348:2609-17.