Idiopathic Cervical Fibrosis—A New Member of IgG4-Related Sclerosing Diseases: Report of 4 Cases, 1 Complicated by Composite Lymphoma

Abstract

Idiopathic cervical fibrosis is a rare tumefactive inflammatory-sclerosing lesion involving the soft tissues of the head and neck, and a proportion of patients also have synchronous or metachronous inflammatory fibrosclerosing lesions in other anatomic sites. The latter finding suggests that this entity may represent a member of IgG4-related sclerosing diseases. We report 4 cases to support this postulation. The patients were male adults aged 42 to 89 years, who presented with an infiltrative, firm cervical mass. Two patients also had IgG4-related chronic sclerosing sialadenitis of submandibular gland and lymphadenopathy. Histologically, the cervical soft tissue lesions had ill-defined borders, consisting of coalescent nodular lymphoid aggregates accompanied by a sclerotic stroma. Nerve infiltration, skeletal muscle invasion, and phlebitis were present. There was a significant increase in IgG4 plasma cells (87 to 327 per high-power field, with IgG4/IgG ratio of 63% to 98%). In the soft tissue lesion of 1 patient, there were expansile foci comprising dense sheets of plasma cells and small lymphoid cells that exhibited κ light chain restriction and clonal immunoglobulin gene rearrangement, consistent with supervening extranodal marginal zone lymphoma. The adjacent lymph node from the same patient showed Epstein-Barr virus (EBV)-positive classical Hodgkin lymphoma with typical morphology and immunophenotype (CD30, CD15, PAX5). Thus lymphoma can supervene in the chronic inflammatory background similar to that recently documented for IgG4-related sclerosing disease of the ocular adnexa.

Consensus statement on the pathology of IgG4-related disease

“…6,8,10,12,[15][16][17]27,28,30,32,34,35,49,51,52,[55][56][57][58][59][60][61][62][63] Consensus statement on the pathology of IgG4-related disease V Deshpande et al which is designed to bind to the Fc portion of IgG4 molecules. 7,10,11,31,40,41 Polyclonal antibodies have been also used by some groups. The type of antibody does not have a significant impact on the count of IgG4 þ plasma cells.…”

Systemic immunoglobulin G4 (IgG4) disease and idiopathic orbital inflammation; removing ‘idiopathic’ from the nomenclature?

“…The adjacent lymph node also showed classical Hodgkin lymphoma. 40 In summary, there is mounting evidence suggesting that B-cell lymphomas can supervene on a background of chronic inflammation similar to that recently documented for IgG4-related disease of the ocular adnexa. 23 Also, IgG4-producing cells by themselves might be neoplastic.…”

“…39 Additional disease entities are being discovered to have an IgG4 link. Idiopathic cervical fibrosis, a rare tumefactive inflammatory sclerosing lesion involving the soft tissues of the head and neck, 40 was postulated in 2010. Four cases were reported to demonstrate elevated tissue IgG4 levels although serum samples were not available for analysis.…”

IgG4-related disease and clonal B-cell lymphoid proliferation: Description of two clinical cases and a review of literature

“…В основном это экстранодальные лимфомы из клеток маргинальной зоны (MALT-лимфомы) [13][14][15][16], есть описание 1 фолликулярной лимфомы [14], 1 фолликулярной лимфомы in situ [17], 1 лимфо-мы из малых лимфоцитов [18], несколько случаев В-крупно-клеточной лимфомы [19][20][21] и одна Т-клеточная лимфома [22]. В литературе имеется описание сочетания лимфомы Ходжкина и НХЛ [23]. К сожалению, не во всех случаях проводилось ги-стологическое исследование и ИГХИ для подтверждения диа-гноза IgG4-СЗ.…”

“…Боль-шинство представленных в мировой литературе лимфом отно-сятся к В-клеточным [15-21, 23, 27], описан лишь один случай Т-клеточной лимфомы [22]. В 3 описаниях имелось сочетание различных лимфом: 2 разных MALT-лимфом одновременно [15,27] и MALT-лимфомы и лимфомы Ходжкина [23]. У большин-ства пациентов достигнута ремиссия ЛПЗ после полихимиотера-пии, один пациент умер от ЛПЗ через 4 года [19].…”