Midline non healing granuloma of nose

Synonyms:

1. Lethal midline granuloma
2. Stewart’s granuloma
3. Polymorphic reticulosis
4. T / NK cell lymphoma

This tumor is responsible for the classical destruction of the midface

Classification:

1. Generalized lymphoma involving the Sinonasal tract
2. Lymphomas of waldayer’s ring extending to the nasopharynx
3. Peripheral – Sinonasal lymphoma the classic T cell lymphoma

Age / Sex distribution: T/NK cell lymphoma may occur at any age from the 1^st – 9^th decade. Male preponderance has been documented.

This disorder is associated with aggressive destruction of middle of face. This disease has classically been divided into three stages:

1. Prodromal stage: This stage could last for many years with the patient complaining of persistent nasal obstruction and rhinorrhoea.
2. Period of activity: Areas of necrosis develop on and around the nasal cavity associated with purulent nasal discharge, crusting and tissue loss. There is also progressive destruction of the nasal framework, palate, upper lip extending into the pharynx. Orbit and skull base also could be involved.
3. Terminal stage: This stage is characterized by gross mutilation of the face, exhaustion and eventual death. Systemic metastasis is also seen in this stage

Histopathology: Nasal T cell lymphoma presents a diagnostic problem. Atypical cell infiltrates are found dispersed in necrotic areas. Immuno histochemistry using a panel of monoclonal antibodies against T cell differentiation antigen should be applied as 80% of peripheral T cell lymphomas show aberrant phenotypes.

Histologically, the infiltrates are polymorphic and atypical cells. These atypical cells are atypical T lymphocytes. Thrombosis and necrosis are common findings. Granulomas and giant cells are not present in this condition.

Treatment: These patients are initially treated with low dose irradiation. Now a complete course of radical radiotherapy of 55 Gy with wide field coverage including nose, sinuses and palate is administered. Chemotherapy is reserved for high grade tumors.