Abstract
Primary cutaneous plasmacytoma (pcp) is a rare disease, with few studies to guide therapy. Our primary study objective was to define treatments used for pcp; a secondary objective was to describe outcomes of patients, including disease recurrence and death. An institutional cancer registry was used to identify cases for retrospective chart review. In a systematic review, treatments for, and outcomes of, all known cases of pcp were described. Three eligible cases identified at our institution; each patient had a solitary pcp. The systematic review identified 66 patients. Radiotherapy was the most commonly used primary treatment modality (31% of all patients; 42% for patients with solitary lesions), followed by surgery (28% of all patients; 36% for patients with solitary lesions). Median survival for all patients was 10.4 years [95% ci: 4.3 years to not reached], with a trend toward a decreased risk of death with solitary lesions compared with multiple lesions (hazard ratio: 0.37; 95% ci: 0.13 to 1.08; p = 0.059). For patients with solitary lesions, the median and recurrence-free survivals were, respectively, 17.0 years (95% ci: 1.7 years to not reached) and 11.0 years (95% ci: 2 years to not reached); for patients with multiple lesions, they were 4.3 years (95% ci: 1.3 to not reached) and 1.4 years (95% ci: 0.6 years to not reached). Disease recurrence, including progression to multiple myeloma, was the most common cause of death. Compared with patients having multiple pcp lesions, those presenting with a single pcp lesion might experience longer overall survival. Local therapy (radiation or surgery) is a reasonable curative treatment for a solitary pcp lesion.
Highlights
Primary cutaneous plasmacytoma is a rare diagnosis, with fewer than 50 cases estimated to be have been reported and compiled in the literature[1,2,3]
Compared with patients having multiple pcp lesions, those presenting with a single pcp lesion might experience longer overall survival
Cutaneous deposits of plasma cells are more commonly seen in the context of multiple myeloma, where such deposits are associated with poor prognosis[4,5]
Summary
Primary cutaneous plasmacytoma (pcp) is a rare diagnosis, with fewer than 50 cases estimated to be have been reported and compiled in the literature[1,2,3]. As well, compared with osseous plasmacytomas, soft-tissue plasmacytomas are associated with a lower rate of progression to multiple myeloma[7]. Extramedullary plasmacytomas are associated with good clinical outcomes, patients with pcp can develop local recurrence, distant skin recurrence, or systemic progression to multiple myeloma[1,2]. Primary cutaneous plasmacytoma (pcp) is a rare disease, with few studies to guide therapy. Our primary study objective was to define treatments used for pcp; a secondary objective was to describe outcomes of patients, including disease recurrence and death
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