Abstract
Background: Primary cutaneous B-cell lymphoma (PCBCL) refers to B-lymphocyte derived lymphoma that develops in the skin without any extracutaneous involvement at the time of diagnosis. Primary cutaneous follicle center lymphoma (PCFCL), primary cutaneous marginal zone lymphoma (PCMZL), and primary cutaneous large B-cell lymphoma (PCLBCL) are the three major entities of PCBCL under the World Health Organization/European Organization for Research and Treatment of Cancer (WHO/EORTC) classification of cutaneous lymphomas. Current guidelines recommend obtaining staging Positron emission tomography/computed tomography (PET/CT) scan or CT scan for all PCBCL, and bone marrow biopsy for at least PCLBCL-leg type variant. However, evidence supporting these recommendations, especially radiological imaging, is lacking.
Methods: Data including demographics, white blood cell (WBC) count at diagnosis, lactate dehydrogenase (LDH) at diagnosis, and results of staging CT-scan, PET/CT-scan, single-photon emission computed tomography scan (SPECT-scan), and bone marrow biopsy were collected through chart review on all patients seen at Roswell Park Cancer Institute between 2001-2016 who presented with a skin lesion and had a biopsy diagnostic of B-cell lymphoma. Patients without any radiological imaging at diagnosis and those diagnosed of diffuse large B-cell lymphoma (DLBCL), marginal zone lymphoma (MZL), or follicular lymphoma (FL) prior to cutaneous manifestation were excluded.
Results: 67 patients met criteria for this study of whom 97% were Caucasian and 60% were male. Cutaneous biopsies noted follicle center cell histology (16 patients; 24%), marginal zone histology (32 patients; 48%), or large B-cell histology (19 patients; 29%). Staging CT-scan, functional imaging (PET/CT-scan or SPECT-scan), and bone marrow biopsy were performed for 59 (88%), 48 (72%), and 36 (54%) patients respectively (distribution across B-cell lymphomas shown in Figure 1). Radiological imaging studies were over-interpreted in 13 patients. Radiological imaging upstaged diagnosis in 13 patients (8 DLBCL, 3 MZL, 2 FL) while bone marrow biopsy alone upstaged diagnosis in only 1 patient (DLBCL). Together, work-up upstaged diagnosis in patients with cutaneous high-grade lymphoma (large B-cell lymphoma) significantly more than it did for cutaneous low-grade lymphoma (follicle center cell and marginal zone lymphoma) histology (47% vs. 10%; p=0.0018). Presence of B-symptoms correlated with systemic disease (0 patients with PCBCL vs. 4 patients with systemic disease; p=0.0013). However, age (p=0.059), gender (p=0.5418), WBC (p=0.6676), and LDH (p=0.1736) had no correlation with systemic disease.
Conclusion: Our single center retrospective analysis showed that staging work-up including radiological imaging (CT-scan or functional imaging like PET/CT-scan) and bone marrow biopsy upstaged the diagnosis in a small minority (10%) of low-grade cutaneous B-cell lymphomas. However, nearly half (47%) of those with cutaneous large B-cell lymphoma histology were found to have systemic disease upon staging. Given the aggressive disease course of large B-cell lymphomas, staging through radiological imaging and bone marrow biopsy should be pursued as currently recommended. However, for low-grade B-cell lymphomas, where even observation is a reasonable management option in selected stage IV patients, staging radiological imaging and bone marrow biopsies could be avoided unless dictated by clinical judgment.
Figure 1 Staging radiologic imaging and bone marrow biopsies (BM bx) performed in patients with cutaneous B-cell lymphoma Figure 1. Staging radiologic imaging and bone marrow biopsies (BM bx) performed in patients with cutaneous B-cell lymphoma
Disclosures
No relevant conflicts of interest to declare.
Two cases of uveitis masquerade syndrome caused by bilateral intraocular large B-cell lymphoma