Second Lymphomas and Other Malignant Neoplasms in Patients With Mycosis Fungoides and Sézary Syndrome

Abstract

Setting: Nine population-based US cancer registries that constitute the Surveillance, Epidemiology, and End Results Program (SEER-9), and Stanford University referral center cohort of patients with cutaneous lymphoma.Patients: Patients with mycosis fungoides or Sézary syndrome from the SEER-9 registry diagnosed and followed up from 1984 through 2001 and from the Stanford University cohort diagnosed and followed up from 1973 through 2001.Main Outcome Measures: Relative risk was estimated using the standardized incidence ratio (SIR). The expected cancer incidence for both cohorts was calculated using age-, sex-, race-, and calendar year-specific SEER-9 incidence rates for the general population. Nonmelanoma skin cancers were excluded because these cancers are not routinely reported by the SEER database. Results: In the SEER-9 cohort (n = 1798), there were 197 second instances of cancer (SIR=1.32; 95% confidence interval [CI], 1.15-1.52) at all sites. Significantly elevated risk (PϽ.01) was observed for Hodgkin disease (6 cases; SIR = 17.14; 95% CI, 6.25-37.26) and non-Hodgkin lymphoma (27 cases; SIR=5.08; 95% CI, 3.34-7.38). Elevated risk (PϽ.05) was also observed for melanoma (10 cases; SIR = 2.60; 95% CI, 1.25-4.79), and urinary cancer (21 cases; SIR=1.74; 95% CI, 1.08-2.66). In the Stanford University cohort (n=429), there were 37 second instances of cancer (SIR=1.04; 95% CI, 0.76-1.44). Elevated risk (PϽ.01) was observed for Hodgkin disease (3 cases; SIR = 27.27; 95% CI, 5.35-77.54). Elevated risk (PϽ.05) was also observed for biliary cancer (2 cases; SIR = 11.76; 95% CI, 1.51-42.02). Conclusion: Updated SEER (population based) and Stanford (clinic based) data confirm the generalizability of earlier findings of increased risk of lymphoma in patients with mycosis fungoides or Sézary syndrome.

Revisions to the staging and classification of mycosis fungoides and Sézary syndrome: a proposal of the International Society for Cutaneous Lymphomas (ISCL) and the cutaneous lymphoma task force of the European Organization of Research and Treatment of Cancer (EORTC)

“…Visceral abnormalities seen radiologically in MF could be secondary to either another unrelated cancer (second malignancies are not uncommon in MF/SS [86][87][88] or to an infectious disorder and not to MF/SS.…”

Cytologic evaluation of lymphadenopathy associated with mycosis fungoides and Sezary syndrome

“…Our findings are complementary to those of others who have shown that patients with MF/SS are at increased risk for developing a second primary lymphoma, particularly classic Hodgkin lymphoma. 34 It is uncertain why patients with MF/SS are at increased risk of developing a second primary lymphoma. In any event, recognition of the association of MF/SS and development of other lymphomas is essential when evaluating FNAB specimens, especially when allocating the limited sample obtained by FNAB for various ancillary studies such as flow cytometry, immunohistochemistry, and molecular studies.…”

Mycosis Fungoides and Head and Neck Malignancies

“…Patients with advanced disease may develop visceral dissemination, and the most commonly involved organs are the lungs, spleen, liver, gastrointestinal tract, kidneys, thyroid gland, pancreas, bone marrow, and heart [16,29]. MF has also been described in the head and neck in advanced stages with reports of spread to the larynx, oral cavity, oropharynx, hypopharynx, cervical esophagus, paranasal sinuses, and the aerodigestive tract [3,8,15,16,22]. If peripheral blood involvement is present, patients are considered to have Sezary syndrome (SS) [29].…”