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Rare midfacial necrotizing lesion characterized by destructive, mucosal lesions of the upper aero-digestive tract.
- Also referred to as polymorphic reticulosis, midline malignant reticulosis, Stewart’s granuloma
Malignant lymphomas of the sinonasal region and nasopharynx are majorly of non-Hodgkin’s lymphomas (NHL) and fall either into NK/T cell type, B cell type or peripheral T cell type. The most common of the so called “nasal type” in which the nasal cavity is the prototype site of involvement are the extranodal NK/T cell lymphomas.
Clinical features
Macroscopically the lesions usually look like necrotic granulomas and are characterized by ulceration and destruction of the nose and paranasal sinuses with erosion of soft tissues, bone and cartilage of the region. The patients show an aggressive and lethal course with rapid destruction of the nose and face (midline), therefore the term “lethal midline granuloma”.
This disease occurs around the fourth decade and the male to female ratio is 8:1 to 2:1. The major symptoms are nasal stuffiness with/without nasal discharge. Oral/nasal ulcer with conjunctivitis may also occur. Perforation of the nasal septum with mutilation of the surrounding tissues eventually occurs.
Diagnosis
The nonspecificity of symptoms obscures the correct diagnosis and is responsible for the delay in treatment which can be detrimental as this grave disease calls for urgent intervention.
Management
Untreated, this disease has a very high mortality reaching almost 100% due to septicemia, perforation into blood vessels or penetration into brain leading to abscess.