Hematolymphoid proliferations and neoplasia

2025-07-24   Paola Dal Cin, PhD , Judith Ann Ferry, MD 

1.Brigham and Women's Hospital , Harvard Medical School, Boston , MA (USA)
2.Massachusetts General Hospital/ Harvard Medical School

Keywords
extranodal lymphomas , cervical lymph nodes ,non-Hodgkin lymphoma

Classification

Definition

The head and neck region is the second most common site of origin for extranodal lymphomas (following the gastrointestinal tract). Involvement by myeloid sarcoma and histiocytic/dendritic neoplasms is rare. Cervical lymph nodes are a common site for involvement by lymphoma. Waldeyer’s ring is the most common extranodal primary site for non-Hodgkin lymphoma within the head and neck.1 Other relatively common primary sites for lymphoma include salivary glands and ocular adnexa, while primary lymphomas of the thyroid and sinonasal tract are uncommon and primary lymphomas of the oral cavity and larynx are rare. 2-5 Throughout the head and neck, B-cell lymphomas are much more common than T-cell or NK-cell lymphomas; an important exception is the nasal cavity, which is the most common site of origin for extranodal NK/T-cell lymphoma.5 While there is some overlap in histologic and immunophenotypic features between extranodal and nodal lymphomas, there are important differences. For example, Hodgkin lymphoma is rare in extranodal sites, while extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) and extranodal NK/T-cell lymphoma (ENKTL) by definition arise in extranodal sites, and plasmablastic lymphoma is extranodal in the vast majority of cases. In addition, pediatric-type follicular lymphoma (PTFL) most commonly arises in cervical lymph nodes6 while large B-cell lymphoma with IRF4 rearrangement (LBCL-IRF4) most often arises in Waldeyer’s ring.7 Detailed information on lymphomas encountered in the head and neck is available in the WHO 2022 Classification of Tumours of Haematopoietic and Lymphoid tissues.  

Article Bibliography

Reference NumberPubmed IDLast YearTitleAuthors
1114002432001Localized non-Hodgkin's lymphoma of Waldeyer's ring: clinical features, management, and prognosis of 130 adult patients.Ezzat AA et al
2115834022001The clinical presentation of non-hodgkin lymphomas of the major salivary glands.Jaehne M et al
3163081652005Extranodal lymphomas of the head and neck.Vega F et al
4172557612007Lymphoma of the ocular adnexa: A study of 353 cases.Ferry JA et al
5353129792022Update from the 5th Edition of the World Health Organization Classification of Head and Neck Tumors: Hematolymphoid Proliferations and Neoplasia.Ferry JA et al
6228556082012Pediatric-type nodal follicular lymphoma: an indolent clonal proliferation in children and adults with high proliferation index and no BCL2 rearrangement.Louissaint A Jr et al
7311875302019Rare mature B-cell lymphomas in children and adolescents.Woessmann W et al

Citation

Paola Dal Cin, PhD ; Judith Ann Ferry, MD

Hematolymphoid proliferations and neoplasia

Atlas Genet Cytogenet Oncol Haematol. 2025-07-24

Online version: http://atlasgeneticsoncology.org/solid-tumor/209327/hematolymphoid-proliferations-and-neoplasia