Extramedullary Solitary Plasmacytoma with Anaplastic Features of the Nasopharynx

Abstract

Extramedullary plasmacytomas (ESPs) are rare forms of plasma cell dyscrasias and usually are seen in the upper aerodigestive tract. ESPs with anaplastic features are extremely rare, and no treatment guidelines exist. We present a 75-year-old gentleman presented with left nasal blockage, and on examination, a polypoid left nasal mass was seen. He was, then, referred to ENT after a CT scan revealed a mass in the left nasopharynx for a biopsy. The preliminary reports suggested a high-grade lymphoma; however, after further testing, it was revealed to be an anaplastic plasma cell neoplasm. PET scan, bone marrow biopsy, serum and urine protein electropheresis, serum immunofixation and light chains were all unremarkable for systemic disease. Differential diagnosis included plasmablastic lymphoma, NK/T cell lymphoma-nasal type, and squamous cell cancers of the head and neck. He was treated with radiation alone given his comorbidities. Given there are no treatment guidelines, we would like to highlight this rare case and discuss different potential management options such as radiation, chemotherapy, surgery, or a combination of different modalities.

Rare case of anaplastic plasmacytoma in the sinonasal tract

“…Diagnostic workup of anaplastic extramedullary plasmacytomas should include a thorough histological evaluation including immunostains, total blood count, bone marrow biopsy, gene rearrangement studies by PCR, and electron microscopic examination 3,20 . Differential diagnoses include plasmablastic lymphoma, anaplastic lymphoma kinase‐positive large B‐cell lymphoma, and extracavitary primary effusion lymphoma 2 . Anaplastic plasmacytomas generally present with a high histologic grade and morphology 2,9 and are composed of large atypical cells with eccentric nuclei, abundant cytoplasm, and increased mitosis 2 .…”

“…Immunofluorescence staining on frozen tissue and immunoperoxidase staining on fixed tissue are suggested techniques to assess for cytoplasmic immunoglobulin, a finding suggestive of lymphocyte malignancy 22,23 . Examples of other pertinent immunostain findings to confirm the diagnosis of anaplastic plasmacytoma include neoplastic cells positive for CD138 and IgA and lack of CD20 and IgG 2 …”

“…3,20 Differential diagnoses include plasmablastic lymphoma, anaplastic lymphoma kinase-positive large B-cell lymphoma, and extracavitary primary effusion lymphoma. 2 Anaplastic plasmacytomas generally present with a high histologic grade and morphology 2,9 and are composed of large atypical cells with eccentric nuclei, abundant cytoplasm, and increased mitosis. 2 Of crucial importance in diagnosis is assessment of cytoplasmic immunoglobulin.…”

“…1 Plasmacytomas rarely express anaplastic features and due to their uncommon nature, there are no treatment guidelines in place. 2 To our knowledge, there have only been three reported cases of anaplastic plasmacytomas of the sinonasal tract in the English literature. We detail the fourth case and provide the first comprehensive review of all sino-nasal anaplastic plasmacytomas.…”

“…They most commonly occur in the upper respiratory tract between the sixth and seventh decades of life 1 . Plasmacytomas rarely express anaplastic features and due to their uncommon nature, there are no treatment guidelines in place 2 . To our knowledge, there have only been three reported cases of anaplastic plasmacytomas of the sinonasal tract in the English literature.…”