Abstract

Multiple myeloma is a plasma cell tumor that homes to and expands in the bone marrow and that, despite the new available drugs, remains incurable. Extramedullary plasmacytoma is a not frequent manifestation during the natural history of multiple myeloma and is frequently associated with plasma cell bone marrow infiltration. The most common locations for an EMP include the gastrointestinal tract, pleura, testis, skin, peritoneum, liver, endocrine glands, and lymph nodes. Primary involvement of the gallbladder fossa is exceedingly rare. In this report, we describe a patient with multiple myeloma who achieved a clinical and serological remission after autologous transplant but progressed rapidly at extramedullary site mimicking a second cancer (i.e., pancreatic or biliary cancer). In this case, the extramedullary localization was refractory to standard therapy, differently from bone marrow localization, but responded to lymphoma-like therapy. In this patient (i) the particular site of developing plasmacytoma is the gallbladder fossa, (ii) the timing of onset of this neoplasm is immediately after autologous transplant, and (iii) its disjunction from primary myeloma is that it appears in clinical and serological remission phase which may be confounding during the diagnostic approach simulating a different tumor (solid tumor).

Highlights

  • Extramedullary plasmacytomas (EMP) represent a rare manifestation in the course of multiple myeloma (MM) [1]

  • The clonal evolution theory has been refined to include the concepts of cancer stem cell and intermediate subclones with cancer stemness properties, the importance of genomic instability, the role of epigenetics, and the impact of cancer microenvironment on clonal selection [7, 8]

  • The Darwinian behavioural characteristics of cancer stem cells are applicable to MM

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Summary

Introduction

Extramedullary plasmacytomas (EMP) represent a rare manifestation in the course of multiple myeloma (MM) [1]. Extramedullary progression of MM has consistently been associated with a poorer disease prognosis [3]. This poorer prognosis is not clearly related to the type or intensity of prior treatments and cannot always be explained by occurrence of this progression in very advanced disease stages. There is increasing evidence that extramedullary relapse is associated with secondary changes in the myeloma clone, aggressive disease progression, poor prognostic histological and biological factors (plasmablastic morphology, higher proliferative index, and p53 expression), and treatment resistance [4, 5]. It has been reported that extramedullary progression or relapse is often associated with the “escape” phenomenon of the monoclonal component [6]

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