Granulomatous lobular mastitis

Other Names: Idiopathic granulomatous lobular mastitis

Granulomatous lobular mastitis is a rare inflammatory disease of the breast. This disease usually affects women of child-bearing age or those who use oral contraceptive medication. It can be confused with breast cancer, so it is often misdiagnosed and proper treatment is delayed.

Cause

The cause of GLM is not known exactly. A various of factors, including microbiology agents, hormonal effect, and immunologic disorder have been suggested of playing an important role in disease cause.

Microbiology agents Coryne bacteria are Gram-positive bacteria and members of the skin flora. They have been found in some patients with GLM recently and been considered to go deeper into the breast tissue via the ductal system.

Hormonal effect GLM is usually detected in women with a history of breast-feeding or birth recently. Gestation, breast-feeding and hyperprolactinemia have also been put forward as a possible pathogenesis of GLM.

Immunologic disorder The association between GLM and erythema nodosum and ankle arthritis have been commonly studied recently. It has been proved that treatment with corticosteroid and immunosuppressants is effective for some patients, which are supported that GLM is a kind of autoimmune disease.

The other factors The other factors when considered about thecause of GLM mainly consist of alpha-1-antitrypsin (AAT), oral contraceptives (OCS) and smoking in general.

Signs and symptoms

It is generally emerged with the clinical symptoms of breast mass, abscess, inflammation and mammary duct fistula. In addition, chronic mass is a very common presentation of the GLM. The mass is hard and oedematous fixed to the skin, accompanied with an axillary node enlargement and nipple retraction occasionally, which is radiological and clinical findings of mimic breast cancer.

Aspiration cytology is characteristic, showing leucocytes, giant cells, epitheloid cells and macrophages. Some patients are diagnosed with the abscess as well, which is a kind of tender breast lump with painful and inflamed overlying skin. The chronic breast abscess can evolve into fistulae following as the disease progresses.

Diagnosis

The diagnosis is made by histopathology with a chronic non-necrotizing granulomatous inflammation in lobules of the breast tissue as the microscopic feature. Differential diagnosis Granulomatous lobular mastitis closely resembles the duct ectasia/periductal mastitis complex (DE/PDM) and tuberculous mastitis in the clinical manifestations and imaging examination.

Treatment

There is no established treatment, but antibiotics, corticosteroids, drainage, excision, and surgical removal of the lesion have been tried with variable success.

Observational management Literature reports that GLM can be self-limiting with 6–12 months natural course to stable no matter what treatment had been taken, and more than half of GLM patients who do not receive any treatment revealed complete remission.

Surgical intervention Surgical intervention is commonly used in present clinical practice, but it should be used with caution for 16%–50% recurrence rate was reported after surgical treatment,27 and this can be related to the scope of surgical resection.

Corticosteroids and immunosuppressive agents Corticosteroids have been used as an conservative management, which have been reported can decrease the mass dimension as primary treament and also could be used in unresectable lesions before surgery.

For resistant and complicated cases, corticosteroids should be administered after excision to prevent GLM recurrence. Prednisone is reported be effective treatment for GLM.

Immunosuppressive agents such as methotrexate can be used as steroid sparing agents for patients with contraindication of corticosteroids or in relapse cases after corticosteroid discontinuation,23, 35 and reports have suggest that low-dose corticosteroids together with methotrexate could be a reasonable option to decrease the side effects of corticosteroids.

NIH genetic and rare disease info

• NIH info on Granulomatous lobular mastitis

Granulomatous lobular mastitis is a rare disease.

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