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

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