Treatment and Outcomes for Primary Cutaneous Extramedullary Plasmacytoma: A Case Series

Abstract

Background Primary cutaneous plasmacytoma (pcp) is a rare disease, with few studies to guide therapy. Our

Rare Presentation of Primary Extramedullary Plasmacytoma as Lip Lesion

“…Of the 4% cases, only a few cases have been reported to be associated with lip lesions. In the latest comprehensive literature review by Tsang et al in 2016, there are 5 case reports of primary cutaneous plasmacytomas of the lip [2]. Primary cutaneous plasmacytomas without underlying multiple myeloma is included in the 2005 WHO/EORTC classification among the primary cutaneous marginal zone B-cell lymphoma due to considerable overlapping features [3].…”

“…Currently, there are approximately 50 cases of primary cutaneous plasmacytomas that have been reported in the literature [2]. Due to its rarity, little is known about its treatment.…”

“…The optimal dose for local control is 40–50 Gy in 20 fractions delivered over 4–6 weeks. However, caution should be considered when using radiotherapy as local recurrence or lymph node metastases could develop thereafter [2]. In addition to high dose radiotherapy and fractions, adjuvant chemotherapy may also be considered for lesions with high-grade histology or tumors >5 cm or for patients with multiple lesions.…”

“…They tend to have less local regional or distant dissemination and remained confined to the skin. As a note, large solitary lesions might have a higher risk of progression to metastatic disease, although a size cut-off had not been determined [2]. …”

“…Cases of primary cutaneous plasmacytoma are comprised of 2–4% of extramedullary plasmacytomas. It is unusual to have a primary cutaneous plasmacytoma present as a lip lesion, as only 5 cases have been reported in the literature [2]. The first case reported was by Volk in 1936 [2].…”