Lethal Midline Granuloma, also known as Lethal Midline Granuloma syndrome or malignant midline reticulosis, is a rare malignant disorder characterized by the progressive destruction of the midline structures of the upper respiratory tract, including the nose, nasal passages, palate, and pharynx.
Patients with Lethal Midline Granuloma typically present with symptoms such as nasal congestion, bloody discharge, ulceration of the nasal and oral mucosa, as well as craniofacial pain and swelling. As the disease progresses, it may involve nearby structures such as the orbit, the central nervous system, and the skin, leading to blindness, facial deformities, and neurological deficits.
Histologically, Lethal Midline Granuloma is characterized by the infiltration of large malignant cells, primarily lymphocytes and histiocytes, within the affected tissues. The exact cause of this disorder remains unclear; however, it is thought to arise from a malfunction of the immune system that triggers an uncontrolled immune response against an unknown antigen, leading to tissue destruction.
The rarity and aggressiveness of Lethal Midline Granuloma pose significant diagnostic and therapeutic challenges. Treatment usually involves a multimodal approach, including chemotherapy, radiation therapy, and occasionally, surgical intervention. Unfortunately, even with aggressive management, the prognosis of Lethal Midline Granuloma is often poor, with a high mortality rate.
