Abstract

Lymphomatoid granulomatosis (LYG) is a rare Epstein-Barr virus (EBV) driven angiocentric and angiodestructive lympho-proliferative disorder predominantly involving extranodal sites; lymph nodes are rarely involved. We present an 80-year-old man with high-grade lymphomatoid granulomatosis with isolated cervical nodal involvement. He suffered from IgA monoglonal gammopathy and nephropathy for several years. He presented with fluctuating cervical lymphadenopathy for a few months. Repeated fine needle aspiration cytology (FNAC) was misleading, with necrosis and reactive lymphoid cells only, suggesting an infective process resulting in the patient receiving antibiotic treatment repeatedly. Flow cytometry was inconclusive. Excision biopsy of an enlarged submandibular lymph node demonstrated extensively necrotic lymph node and adjacent adipose tissue with a rim of viable lymphoid tissue. In the viable areas there was a polymorphic infiltrate of lympho-histiocytic cells and a significant population of large atypical cells with prominent and occasionally multiple nucleoli and mitoses. The blood vessels within necrotic areas and viable zones showed variable necrosis and transmural and periadventitial infiltrate of tumour cells. Immunohistochemical examination was diagnostic of grade 3 lymphomatoid granulomatosis. This case report highlights challenges faced by pathologists in accurate diagnosis of the condition as FNAC and flow cytometry are inconclusive. An emphasis on differential diagnosis, histological diagnostic features, treatment and prognosis is included.

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