Disease related to IgG4 and retro orbital MALT Lymphoma. A rare disease

Abstract

IgG4-related disease (IgG4-RD) is a chronic benign neoplastic process, clinically heterogeneous, of unknown etiology and low occurrence, with specific histologic and immunocytochemical characteristics, predominantly affecting adults. Exceptionally, it may progress to lymphoma.

Objective: To report a pediatric patient with IgG4-RD and emphasize the importance of long-term follow-up, given its potential evolution towards malignancy.

Clinical Case: We present a 6-year-old girl who presented with a one-year history of asymmetric bilateral enlargement of cervical lymph nodes and salivary glands, which progressed rapidly over the past two months with no associated symptoms. Malignant neoplasms were ruled out, and spontaneous regression occurred. Seven years later, she presented bilateral cervical lymphadenomegaly with left predominance, parotid swelling, and left eyelid edema. Histopathological and immunohistochemical analysis of the lymph node biopsy and serum IgG4 levels confirmed the diagnosis of IgG4-RD. Treatment with oral corticosteroids followed by methotrexate was started, with positive response over 2 years. However, during methotrexate therapy, lymph node, glandular, and eyelid involvement recurred, with left proptosis. The MRI and biopsy of the retrobulbar mass revealed a mucosa-associated lymphoid tissue lymphoma, which was treated with radiotherapy and chemotherapy until remission.

Conclusion: Long-standing head and neck tumor processes may consist of benign proliferative diseases, such as IgG4-RD, which may occasionally progress to lymphoma.