Lethal midline granuloma

Lethal midline granuloma, also known as polymorphic reticulosis or midline malignant reticulosis, is a rare and aggressive form of non-Hodgkin lymphoma. It primarily affects the midline facial structures, including the nasal cavity, paranasal sinuses, and palate. This condition is characterized by destructive lesions that can lead to severe disfigurement and, if left untreated, can be fatal.

Symptoms

The symptoms of lethal midline granuloma can vary depending on the extent and location of the lesions. Common symptoms include nasal congestion, epistaxis (nosebleeds), facial pain, and swelling. As the disease progresses, patients may experience difficulty breathing, loss of smell, and ulceration of the nasal mucosa. In some cases, the lesions can extend to the oral cavity, leading to dental problems and palatal perforation.

Causes

The exact cause of lethal midline granuloma is unknown. However, it is believed to be associated with a dysregulated immune response, possibly triggered by viral infections such as Epstein-Barr virus (EBV) or human herpesvirus 8 (HHV-8). Genetic factors and environmental exposures may also play a role in the development of this condition.

Diagnosis

Diagnosing lethal midline granuloma can be challenging due to its rarity and similarity to other conditions. A thorough medical history, physical examination, and imaging studies such as computed tomography (CT) or magnetic resonance imaging (MRI) are often performed to evaluate the extent of the lesions. A biopsy of the affected tissue is necessary to confirm the diagnosis, and immunohistochemical analysis can help differentiate it from other types of lymphoma.

Treatment

The treatment of lethal midline granuloma typically involves a multidisciplinary approach, including chemotherapy, radiation therapy, and surgery. Chemotherapy regimens, such as CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone), are commonly used to target the cancer cells systemically. Radiation therapy is often employed to control local disease and alleviate symptoms. In some cases, surgical intervention may be necessary to remove extensive lesions or reconstruct facial structures.

Prognosis

The prognosis for lethal midline granuloma varies depending on the stage of the disease at the time of diagnosis and the response to treatment. Early detection and aggressive treatment can improve outcomes, but the overall survival rate remains relatively low. The five-year survival rate ranges from 30% to 60%, with a higher likelihood of long-term survival in patients who achieve complete remission.