Idiopathic cervical fibrosis--a new member of IgG4-related sclerosing diseases: report of 4 cases, 1 complicated by composite lymphoma

Am J Surg Pathol. 2010 Nov;34(11):1678-85. doi: 10.1097/PAS.0b013e3181f12c85.

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.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • Biopsy
  • Fibrosis
  • Herpesvirus 4, Human / isolation & purification
  • Hodgkin Disease / immunology*
  • Hodgkin Disease / pathology
  • Hodgkin Disease / virology
  • Humans
  • Immune System Diseases / immunology*
  • Immune System Diseases / pathology
  • Immune System Diseases / therapy
  • Immunoglobulin G / immunology*
  • Immunophenotyping
  • Inflammation / immunology*
  • Inflammation / pathology
  • Inflammation / therapy
  • Lymph Nodes / immunology
  • Lymphatic Diseases / immunology
  • Lymphocytes / immunology
  • Male
  • Middle Aged
  • Neck / pathology*
  • Neck / surgery
  • Plasma Cells / immunology
  • Sclerosis
  • Sialadenitis / immunology
  • Steroids / therapeutic use
  • Submandibular Gland / immunology
  • Treatment Outcome

Substances

  • Immunoglobulin G
  • Steroids