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idiopathic cervical fibrosis

Monday 15 November 2010

Definition: Idiopathic cervical fibrosis is a rare tumefactive inflammatory-sclerosing lesion involving the soft tissues of the head and neck.

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.

Synopsis

- 42 to 89 years
- an infiltrative, firm cervical mass
- IgG4-related chronic sclerosing sialadenitis of submandibular gland and lymphadenopathy
- ill-defined borders
- coalescent nodular lymphoid aggregates
- sclerotic stroma
- nerve infiltration
- skeletal muscle invasion
- phlebitis
- significant increase in IgG4 plasma cells (87 to 327 per high-power field, with IgG4/IgG ratio of 63% to 98%)

See also

- IgG4-related sclerosing diseases

References

- Idiopathic cervical fibrosis—a new member of igg4-related sclerosing diseases: report of 4 cases, 1 complicated by composite lymphoma. Cheuk W, Tam FK, Chan AN, Luk IS, Yuen AP, Chan WK, Hung TC, Chan JK. Am J Surg Pathol. 2010 Nov;34(11):1678-85. PMID: 20871392