Hodgkin lymphoma involving extranodal sites in head and neck: report of twenty-nine cases and review of three-hundred and fifty-seven cases

Abstract

Introduction: Hodgkin lymphoma (HL) involving the Waldeyer's ring (WR) and other extranodal head and neck sites are rare. We report our experience and PubMed literature review.

Methods: Retrospective single institution cohort study using lymphoma data base and PubMed literature search using twenty-six various search terms.

Results: Twenty-nine patients were treated in our institution (1975-2018). Male:Female 22:7, median age at diagnosis 33 years (15-64), stages I-II:III-IV 25:4. Sites were nasopharynx (10), tonsil (9), parotid (5), mandible (2) and others (3). 20/29 patients received radiation therapy, 22/29 received chemotherapy. Ten years overall-survival and progression-free survival are 92% and 66% respectively. PubMed search showed 8766 citations and identified 357 patients including our patients. Male:Female 199:131, median age 45 years (5-89). Stages I-II in 286 (80%). Involvement was nasopharynx 109 (30.5%), tonsil 67 (18.8%), parotid 58 (16.2%), thyroid 45 (12.6%), adenoid 10 (2.8%), mandible 10 (2.8%) and others in 58 (16.2%). Pathology: mixed cellularity 99 (27.7%), nodular sclerosis 88 (24.6%), nodular lymphocyte-predominant 56 (15.7%), lymphocyte rich 25 (7%), classical-HL-not otherwise specified 16 (4.5%) and lymphocyte depleted 7 (2%) patients. Treatment details are available for 233 patients; 165 (46%) received radiation therapy, 137 (38%) chemotherapy. Complete remission in 208 (58%), progressive disease 14 (4%), no information 135 (38%). Overall, treatment failure in 54 (15%). Thirty (8.4%) have died; 21 disease related. KM overall-survival at 5 and 10 years was 88.5% and 77.6% respectively.

Conclusion: This largest report showed that HL involving extranodal head and neck sites is not very uncommon and has excellent prognosis.

Keywords: Hodgkin lymphoma; Waldeyer’s ring Hodgkin lymphoma; extranodal Hodgkin lymphoma; extranodal Hodgkin lymphoma and outcome; extranodal nodular lymphocyte predominant Hodgkin lymphoma; head and neck Hodgkin lymphoma; nasopharynx Hodgkin lymphoma; tonsillar Hodgkin lymphoma.