Virchows Arch (2002) 441:154–158 DOI 10.1007/s00428-001-0579-4
O R I G I N A L A RT I C L E
Takuya Aoki · Hajime Okita · Hidekazu Kayano Hideki Orikasa · Kentaro Watanabe · Brian P. Eyden Kazuto Yamazaki
Anaplastic plasmacytoma with malignant pleural effusion lacking evidence of monoclonal gammopathy Received: 12 October 2001 / Accepted: 24 October 2001 / Published online: 12 January 2002 © Springer-Verlag 2002
Abstract A case of plasmacytoma of the pleural cavity is reported with massive malignant pleural effusion, which, most unusually, lacked monoclonal gammopathy, thereby making it difficult to distinguish from lymphoma. The pleural tumor and pleural effusion contained large mononuclear lymphoma-like cells with distinct nucleoli. Immunohistochemistry revealed neither lymphoma markers nor clonal cytoplasmic nor cell surface immunoglobulins. Tumor cells were stained with vimentin and the plasma cell markers, VS38c, CD138 (syndecan-1), and MUM1 antibodies. Bone marrow contained small amounts of tumor consisting of similar cells. Electron microscopy showed well developed rough endoplasmic reticulum and peripherally positioned nuclei with euchromatin. Flow cytometry of bone marrow revealed a minimal involvement of CD38-positive cells. Chromosomal analysis of marrow cells revealed a complex abnormal karyotype. A polymerase chain reaction demonstrated clonal re-arrangement of the immunoglobulin heavychain gene. The overall results indicate a clonal expansion of tumor cells with primitive plasma cell differentiation with the highly unusual feature of absent monotypic immunoglobulin. The study illustrates the need for a comprehensive array of techniques to distinguish such T. Aoki · K. Watanabe Department of Internal Medicine, Saiseikai Central Hospital, Tokyo, Japan H. Okita · H. Kayano · H. Orikasa · K. Yamazaki (✉) Department of Pathology, Saiseikai Central Hospital, 1-4-17 Mita, Minatoku, Tokyo 108-0073, Japan e-mail:
[email protected] Tel.: +81-3-34518211, Fax: +81-3-34516102 H. Okita Department of Pathology, School of Medicine, Keio University, Tokyo, Japan H. Kayano Department of Pathology, Saitama Medical School, Saitama, Japan B.P. Eyden Department of Histopathology, Christie Hospital NHS Trust, Manchester, UK
rare non-synthesizing and non-secretory plasmacytomas from lymphoma. Keywords Plasmacytoma · Pleural effusion · Differential diagnosis · Lymphoma
Introduction The diagnosis of anaplastic plasmacytoma with massive pleural effusion and its distinction from lymphoma can be difficult, partly because primary pulmonary plasmacytoma is very rare, with only 28 cases in the literature [4, 6, 7], but also because anaplastic plasmacytoma may show reduced plasma-cell characteristics and may therefore be confused with lymphoma, particularly of largecell and effusion type. Poorly differentiated (anaplastic) plasmacytoma should be ruled out when cells of a pleural effusion show a large-cell appearance but lack lymphoid, epithelial, and mesothelial markers. We describe a very rare case of primary pulmonary (pleural) nonsecreting but also non-synthesizing anaplastic plasmacytoma whose distinction from lymphoma depended on multiple laboratory investigations.
Clinical history An 81-year-old man with right back pain for 3 months was found to have pulmonary fibrosis and a vague intrapulmonary mass of approximately 1.5 cm in diameter and a sequestered pleural effusion pattern in the right lower lung following computed tomography (CT). Physical examination showed only decreased respiratory sound on the right lower chest field. There was no lymphadenopathy. White cells were morphologically normal, and the count was 7.4×109/l with 55% neutrophils and 32% lymphocytes and hemoglobin at 13.2 g/l. Investigations for Epstein-Barr virus (EBV), herpes simplex virus, and human immunodeficiency virus (HIV) were negative, and the patient was not, therefore, considered to be immunocompromised. Neither monoclonal gammopathy in serum nor Bence-Jones protein in urine was demonstrable. Normal immunoglobulins were not suppressed. Chest X-ray and CT showed a sequestered pleural effusion (Fig. 1) without punched out bone lesions. Pleurocentesis revealed an exudate pleural effusion, which
155 Japan) in an automatic thermal cycler (PJ9700, Applied Biosystems, Foster, Calif.). The PCR products were fractionated using electrophoresis on 1.5% low-melting point agarose gel containing ethidium bromide and visualized under ultraviolet (UV) light. Monoclonal PCR products corresponding to the expected size (250 base pairs) were gel-purified and subjected to nucleotide sequencing using the Big Dye Terminator cycle sequencing kit (Applied Biosystems) and an automated sequencer (ABI Prism 377 Genetic analyzer, Applied Biosystems) according to the manufacturer’s protocol. We compared sequence data with germline sequences of IgH (VH, D, and JH) genes that have been deposited at the DDBJ/EMBL/Genbank database using the basic local alignment search tool of the National Center for Biotechnology Information [13].
Results
Fig. 1 Chest X-ray showing a vague intrapulmonary mass (arrow) and a sequestered pleural effusion pattern in the right lower lung was found to contain 7035 IU/l (normal, less than approximately 60% serum level, i.e.,
