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Angiocentric T-cell Lymphoma

Definition

Angiocentric T-cell lymphoma, nasal natural killer (NK)/T-cell lymphoma, malignant granuloma, or midline lethal granuloma is a rare aggressive form of lymphoma characteristically involving the midline structures of the palate and nasal cavity.

Etiology

The etiology is unknown, although the Epstein-Barr virus is frequently associated with this form of lymphoma.

Main Clinical Features

•Prodromal signs and symptoms, e.g., epistaxis, pain, and nasal stuffiness

•Swelling, nonhealing ulceration, and necrosis of the palate, alveolar processes, retromolar pads and the nasal cavity are common, causing destruction and perforation of the palate, nasal septum and bones

•Secondary infections and hemorrhage usually complicate the course of the disease

Diagnosis

The clinical diagnosis should be confirmed by biopsy and histopathologic examination and immunohistochemical evaluation.

Differential Diagnosis

•Wegener granulomatosis

•Non-Hodgkin lymphoma

•Squamous cell carcinoma

•Necrotizing sialadenometaplasia

•Mucormycosis

•Other multiple mycoses

•Leprosy

•Syphilitic gumma

Treatment

Suggested Therapies

•High-dose radiation therapy is the treatment of choice, in particular, of localized early disease. The lesion usually responds well to this treatment and recurrences are rare.

•Chemotherapy alone or in combination with radiation must be used for aggressive and disseminated disease. The prognosis of this form is unfavorable.

References

Koch M, Blatterspiel GJ, Niedobitek G, Konstantinidis J. Angiocentric T/NK cell lymphoma: A special clinical-pathological entity of lethal midline granuloma. A case report. Laryngorhinoolologie 2001;80:410–415.

Lee PY, Freeman NJ, Khorsand J, et al. Angiocentric T-cell lymphoma presenting as lethal midline granuloma. Int J Dermatol 1997;36:419–427.

Mosqueda-Taylor A, Meneses-Garcia A, Zarate-Osorno A. et al. Angiocentric lymphomas of the palate: Clinico-pathological consideration in 12 cases J Oral Pathol Med 1997;26:93–97.

Torre V, Bucolo S, Galletti B, Cavallari V. Midfacial granuloma syndrome or an inflammatory non-specific disease? J Oral Pathol Med 2001;30:190–192.

Vidal E, Dean A, Alamillos F, et al. Lethal midline granuloma in a human immunodeficiency virus-infected patient. Am J Med 2001;111:244–245.