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Year : 2017  |  Volume : 13  |  Issue : 6  |  Page : 1078-1079

Extramedullary sarcomatoid variant of plasmablastic plasmacytoma

Department of Oncopathology, Cancer Institute (W.I.A), Chennai, Tamil Nadu, India

Date of Web Publication13-Dec-2017

Correspondence Address:
Dr. Urmila Majhi
Department of Oncopathology, Cancer Institute (W.I.A), Adyar, Chennai, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0973-1482.183196

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How to cite this article:
Majhi U, Sundersingh S, Murhekar K, Radha K. Extramedullary sarcomatoid variant of plasmablastic plasmacytoma. J Can Res Ther 2017;13:1078-9

How to cite this URL:
Majhi U, Sundersingh S, Murhekar K, Radha K. Extramedullary sarcomatoid variant of plasmablastic plasmacytoma. J Can Res Ther [serial online] 2017 [cited 2020 Aug 3];13:1078-9. Available from: http://www.cancerjournal.net/text.asp?2017/13/6/1078/183196


A 69-year-old male patient presented with dysphasia along with multiple rib fractures and a 4-cm globular swelling in the lateral aspect of the left scapula. Positron emission tomography-computed tomography scan showed irregular soft-tissue thickening in esophagus at the level of D2–D4 vertebrae, involvement of bilateral ribs, left scapula, left clavicle, C1, C2, C8, L3, L5 vertebrae, sacrum, left femur, subpleural nodular opacity of left lung, and cirrhotic liver with hypodense lesions in the left lobe [Figure 1]. Bone scan showed multiple hot spots involving bilateral ribs, left scapula, left clavicle, L1 vertebra, sacrum, and left femur. Routine hemogram as well as serum and urine protein electrophoresis did not show any abnormality. Bone marrow aspiration and biopsy were normal.
Figure 1: Positron emission tomography-computed tomography and bone scan showing multiple hot spots involving bilateral ribs, left scapula, sacrum, left femur, vertebrae. Intraluminal growth in esophagus is also well made out

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The biopsy from esophageal lesion suggested a poorly differentiated malignant tumor with a sarcomatoid appearance. The cells were round to plump, spindle-shaped and were seen in sheets and bundles with round to fusiform vesicular nuclei with prominent nucleoli and increased mitotic activity [Figure 2]a. Immunohistochemistry (IHC) revealed strong positivity for vimentin [Figure 3]d, epithelial membrane antigen (EMA) [Figure 2]d, CD-99 [Figure 3]a, CD-10 [Figure 3]b, and CD-56 [Figure 3]c and moderate positivity to CD-138 [Figure 3]f, leukocyte common antigen, and cyclin-D1 [Figure 2]c. Ki-67 index was 80% [Figure 3]e. Tumor cells were negative for pan-keratin, high molecular weight cytokeratin, C-Kit, smooth muscle actin, desmin, melan-A, HMB-45, CD-21, CD-23, CD-20, CD-79a, BCL-2, BCL-6, CD-3, CD-15, CD-30, AlK-1, placental alkaline phosphatase, CD-68, lateral plate mesoderm, CD-31, CD-34, CK 5/6, and Epstein–Barr virus (EBV)-encoded RNA (EBER). Fine needle aspiration cytology of left scapular swelling revealed large pleomorphic cells with plasmacytoid appearance [Figure 2]b and [Figure 2]e. The histology, cytology, and IHC were suggestive of sarcomatoid variant of plasmablastic plasmacytoma.
Figure 2: (a) Microphotograph of esophageal biopsy showing plump round, oval to spindle cells in bundles and sheets. Increased mitotic activity is seen (M) (H and E, ×40). (b and e) Fine needle aspiration cytology left scapular swelling showing large pleomorphic round oval to fusiform cells along with mono-, bi- - to multi-nucleated tumor giant cells with eccentric nuclei with a plasmacytoid appearance. Mitotic activity is increased (H and E, ×40). (c) Tumor cells showing nuclear positivity for cyclin D1 (IHC, ×20). (d) Round, oval to spindle cells showing strong cytoplasmic positivity for epithelial membrane antigen (IHC, ×20)

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Figure 3: (a) Tumor cells showing strong positivity for CD-99 (IHC, ×40). (b) Tumor cells showing strong positivity for CD-10 (IHC, ×40). (c) Tumor cells showing strong positivity for CD-56 (IHC, ×40). (d) Tumor cells showing moderate positivity for vimentin (IHC, ×20) (e) Tumor cells showing strong nuclear positivity for Ki-67 (IHC, ×20). (f) Tumor cells showing moderate positivity for CD-138 (IHC, ×20)

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Sarcomatoid tumors in mucosal sites are usually thought to be sarcomas, sarcomatoid carcinomas, and melanomas. Other rare tumors include anaplastic large cell lymphoma and dendritic cell sarcomas. Rarely, sarcomatoid plasma cell tumors are considered under differential diagnosis.[1],[2] In our patient, the markers for carcinomas, different types of sarcomas, melanomas, and dendritic cell tumors were negative. Plasmablastic lymphoma was a close differential diagnosis as the tumor cells were positive for CD-138, EMA, CD-56, cyclin D1, CD-10, CD-99, and C-kit.[3]

Highly aggressive plasmablastic plasmacytomas can contain mainly plasmablasts and resemble plasmablastic lymphoma. Plasmablastic lymphoma can show weak or absent expression of conventional B-cell markers and positive for CD-138. Other markers such as CD-99, CD-56, CD10, and C-kit can be aberrantly expressed by both types of tumors.[3] EBV infection is much more strongly associated with plasmablastic lymphoma than with plasma cell neoplasms.[3] However, EBV-associated plasmacytomas are also reported.[4],[5] Latent membrane protein was negative. EBER was also negative. As the patient had multiple lytic lesions of bones and the patient was seronegative, diagnosis of sarcomatoid plasmacytoma was favored over plasmablastic lymphoma.

It is postulated that there could be a genetic overlap between plasmablastic plasmacytoma and plasmablastic lymphoma. These two neoplasms may be different manifestations of malignancies with a common derivation from B-cells at a later stage of B-cell maturation. There may be a common genetic feature that imparts a plasmablastic morphology and an aggressive clinical course in these tumors.[3]

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There are no conflicts of interest.=

 > References Top

Lum D, Wong KP. Sarcomatoid plasmacytoma: A diagnosis not often considered. Pathology 2006;38:593-6.  Back to cited text no. 1
Gauer IC, Hagen V, Merz H. A new sarcomatoid variant of multiple myeloma in a 20-year-old male. Ann Hematol 2004;83:251-2.  Back to cited text no. 2
Vega F, Chang CC, Medeiros LJ, Udden MM, Cho-Vega JH, Lau CC, et al. Plasmablastic lymphomas and plasmablastic plasma cell myelomas have nearly identical immunophenotypic profiles. Mod Pathol 2005;18:806-15.  Back to cited text no. 3
Scarberry K, Jegalian A, Valent J, Wood HM. Solitary extramedullary plasmacytoma of the penis. Urol Ann 2014;6:242-3.  Back to cited text no. 4
[PUBMED]  [Full text]  
Yan B, Tan SY, Yau EX, Ng SB, Petersson F. EBV-positive plasmacytoma of the submandibular gland – Report of a rare case with molecular genetic characterization. Head Neck Pathol 2011;5:389-94.  Back to cited text no. 5


  [Figure 1], [Figure 2], [Figure 3]


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