Lethal midline granuloma

Lethal midline granuloma is a rare and aggressive condition characterized by the progressive destruction of the tissues in the midline structures of the face, including the nose, sinuses, and palate. This condition is often associated with non-Hodgkin lymphoma, particularly the extranodal NK/T-cell lymphoma, nasal type.

Presentation

Patients with lethal midline granuloma typically present with symptoms such as nasal obstruction, chronic sinusitis, and ulceration of the nasal septum. As the disease progresses, it can lead to severe facial disfigurement, including the destruction of the nasal bridge and palate, resulting in oronasal fistulas.

Pathophysiology

The exact cause of lethal midline granuloma is not well understood, but it is believed to be related to an abnormal immune response. The condition is characterized by the presence of atypical lymphoid cells, which are often positive for Epstein-Barr virus (EBV). These cells infiltrate and destroy the tissues in the midline structures of the face.

Diagnosis

Diagnosis of lethal midline granuloma involves a combination of clinical evaluation, imaging studies, and biopsy. Computed tomography (CT) and magnetic resonance imaging (MRI) can help assess the extent of tissue destruction. A biopsy of the affected tissue is essential to confirm the diagnosis and to differentiate it from other conditions such as Wegener's granulomatosis and sarcoidosis.

Treatment

The treatment of lethal midline granuloma typically involves a combination of radiotherapy and chemotherapy. Early diagnosis and treatment are crucial to prevent extensive tissue destruction and to improve the prognosis. In some cases, surgical intervention may be necessary to repair the damaged structures and to restore function.

Prognosis

The prognosis for patients with lethal midline granuloma varies depending on the stage at which the disease is diagnosed and the response to treatment. Early-stage disease has a better prognosis, while advanced disease with extensive tissue destruction is associated with a poorer outcome.

Related Pages

• Non-Hodgkin lymphoma
• Extranodal NK/T-cell lymphoma, nasal type
• Epstein-Barr virus
• Computed tomography
• Magnetic resonance imaging
• Wegener's granulomatosis
• Sarcoidosis
• Radiotherapy
• Chemotherapy