Idiopathic cervical fibrosis

Definition

Idiopathic cervical fibrosis is an infllammatory-sclerosing lesion in the soft tissues of the head and neck, a member of the group of IgG4-related sclerosing conditions.

Synonyms

Sclerosing cervicitis, cervical fibrosclerosis

Epidemiology

This condition is most common in the fifth to seventh decades of life with a male predominance.

Clinical features

Presentation is with a neck mass. Patients may also have sclerosing sialadenitis and lymphadenopathy or more distant IgG4-related sclerosing conditions.

Histopathology

The lesion has ill-defined margins. It comprises nodular lymphoid aggregates and a hypocellular collagenised sclerotic stroma. There is infiltration into nerves and skeletal muscle. A phlebitis is present. IgG4-positive plasma cells are a conspicuous component . Some cases are associated with an extranodal marginal zone lymphoma or classical Hodgkin lymphoma.

Immunohistochemistry

Many of the lymphocytes are CD3+ T-cells. CD20+ B-cells tend to be confined to lymphoid follicles. Plasma cells are polytypic and from 45% to 100% express IgG4.

Differential diagnosis

lymphoma
fibromatosis: shows a spindle cell proliferation and lacks IgG4+ plasma cells
inflammatory myofibroblastic tumour

Management

There is often a dramatic response to steroids.

References

1 Cheuk W, Tam FK, Chan AN, Luk IS, Yuen AP, Chan WK, et al. 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.