title: 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.
authors.affiliation: Queen Elizabeth Hospital, Hong Kong Sanatorium, Hong Kong. cheuk_wah@hotmail.com
authors.forename: Wah
authors.initials: W
authors.lastname: Cheuk
authors.forename: Fiona K Y
authors.initials: FK
authors.lastname: Tam
authors.forename: Alice N H
authors.initials: AN
authors.lastname: Chan
authors.forename: Ivy S C
authors.initials: IS
authors.lastname: Luk
authors.forename: Anthony P W
authors.initials: AP
authors.lastname: Yuen
authors.forename: Wai-Kong
authors.initials: WK
authors.lastname: Chan
authors.forename: Terry C W
authors.initials: TC
authors.lastname: Hung
authors.forename: John K C
authors.initials: JK
authors.lastname: Chan
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001706:Biopsy; D005355:Fibrosis; D004854:Herpesvirus 4, Human / Q000302:isolation & purification; D006689:Hodgkin Disease / Q000276:immunology* / Q000473:pathology / Q000821:virology; D006801:Humans; D007154:Immune System Diseases / Q000276:immunology* / Q000473:pathology / Q000628:therapy; D007074:Immunoglobulin G / Q000276:immunology*; D016130:Immunophenotyping; D007249:Inflammation / Q000276:immunology* / Q000473:pathology / Q000628:therapy; D008198:Lymph Nodes / Q000276:immunology; D008206:Lymphatic Diseases / Q000276:immunology; D008214:Lymphocytes / Q000276:immunology; D008297:Male; D008875:Middle Aged; D009333:Neck / Q000473:pathology* / Q000601:surgery; D010950:Plasma Cells / Q000276:immunology; D012598:Sclerosis; D012793:Sialadenitis / Q000276:immunology; D013256:Steroids / Q000627:therapeutic use; D013363:Submandibular Gland / Q000276:immunology; D016896:Treatment Outcome
chemical_list: D007074:Immunoglobulin G; D013256:Steroids