Nasal T-cell lymphoma: Case report and review of diagnostic features.
A 73-year-old man was referred to us for evaluation of extensive nasal crusting and progressive erosion of the nasal midline structures. Clinical examination suggested that the patient had a T-cell lymphoma, a suspicion that was confirmed on immunohistochemical analysis. The patient was treated with combination chemo- and radiotherapy and exhibited a marked response. At the 14-month followup, he remained disease-free.
Nasal T-cell lymphomas are aggressive, locally destructive midfacial necrotizing lesions. It is now recognized that the vast majority of cases of lethal midline granuloma represent nasal T-cell lymphomas. [1-5] In this article, we describe one such case in an elderly man, and we briefly review some of the more salient features of the diagnosis of this disease.
A white 73-year-old man was evaluated at an outside institution for an 18-month history of nasal obstruction and chronic rhinorrhea. He was found to have nasal polyposis and a septal perforation, and he underwent endoscopic sinus surgery with polypectomy and septal button placement. Postoperatively, he failed to improve. He exhibited extensive nasal crusting and progressive erosion of the nasal midline structures. Results of biopsy analysis of the involved nasal mucosa were inconclusive. He was referred to us for further evaluation.
Our examination revealed an extensive amount of friable, granular tissue along the floor of the nose and lateral nasal wall bilaterally (figure 1). A large septal perforation encompassed the anterior half of the septum. A fistula tract extended from the floor of the nose to the gingivobuccal sulcus under the upper lip, and there was a separate midline ulcer of the hard palate (figure 2).
Biopsy specimens taken from the intranasal and sublabial margins of the fistula were consistent with nasal T-cell lymphoma. Microscopic examination revealed a dense lymphocytic infiltrate, dissection of collagen bundles, and angioinvasion. Immunohistochemistry was strongly positive for the T-cell-associated markers CD2 and CD7 and for the natural killer cell marker CD56. Flow cytometry revealed a predominant population of T cells. Terminal repeat analysis by Southern blot for Epstein-Barr virus was positive.
The patient was treated with 5,000 cGy of external beam radiation, followed by six cycles of CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) chemotherapy. He demonstrated a marked clinical response.
On followup examination at 14 months, the oronasal fistula had closed. Nasal examination revealed a foreshortening of the columella secondary to fibrosis, with contracture (figure 3). The patient developed osteoradionecrosis of the hard palate at the site of his previous ulceration because of inadequate soft-tissue coverage (figure 4). There was no evidence of local recurrence or residual disease.
Nonspecific nasal symptoms often predate the appearance of mucosal ulceration and tissue necrosis by 1 year or more. [6,7] The ambiguous nature of these symptoms can result in a delay in diagnosis. The initial symptom in most cases is nasal obstruction; purulent rhinorrhea is the second most common sign. [3,6,7] The nasal mucosa is usually pale, friable, and granular and is often accompanied by purulence or crusting. Oronasal fistulas frequently occur as a result of mucosal ulceration and palate necrosis. 
Nasal septal perforation has been reported in 40% of cases of nasal T-cell lymphoma.  Systemic symptoms such as fever and weight loss are not typically noted except in advanced cases. [4,6] Systemic dissemination at the time of the initial evaluation is seen in fewer than 10% of patients. When it is present, it usually occurs in extranodal sites. [2,3]
Histologically, representative biopsies demonstrate a mixed cellular infiltrate, which consists of plasma cells, eosinophils, histiocytes, atypical lymphoid cells, neutrophils, and macrophages. Angiocentricity and angioinvasion are present, along with the resultant marked tissue ischemia and necrosis. The diagnosis depends on the identification of atypical lymphoid cells. Because of extensive necrosis and reactive inflammatory changes, superficial biopsies are often inconclusive.  Immunohistochemistry and flow cytometry typically demonstrate the presence of the T-cell-associated markers CD2, CD7, CD45RO, and CD43.  The lymphoma cells express the natural killer cell marker CD56, but not the natural killer cell markers CD16 and CD57.  Evidence of Epstein-Barr virus has been found in all nasal T-cell lymphomas to date, suggesting that the virus plays a causative role in the pathogenesis of these lesions." [1,3,6,7] Phenotypically, nasal lymphomas differ from lymphomas that arise in the paranasal sinuse s and in Waldeyer's ring; the latter tumors are predominantly B cell lymphomas. [1,2,5]
Nasal T-cell lymphomas respond well to local radiation therapy. Even so, death from this disease occurs in 50% of patients as a result of distant extranodal spread or relapses that occur outside the treatment field. 1,3,6,7] Treatment with chemotherapy alone as a primary modality has not been shown to confer a survival advantage. [6,9] The use of chemotherapy for salvage following radiation therapy has been disappointing to date. [7,10] Multiagent chemotherapy in combination with radiation therapy as the initial treatment approach is now recommended in an attempt to control the primary lesion as well as to prevent early dissemination." [1,10]
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(3.) Weiss LM, Arber DA, Strickler JG. Nasal T-cell lymphoma. Ann Oncol 1994:5(Suppl 1):S39-S42.
(4.) Sakata K, Hareyama M, Ohuchi A, et al. Treatment of lethal midline granuloma type nasal T-cell lymphoma. Acta Oncol 1997;36:307-l1.
(5.) Ratech H, Burke JS, Blayney DW, et al. A clinicopathologic study of malignant lymphomas of the nose, paranasal sinuses, and hard palate, including cases of lethal midline granuloma. Cancer 1989;64:2525-31.
(6.) Hartig G, Montone K, Wasik M, et al. Nasal T-cell lymphoma and the lethal midline granuloma syndrome. Otolaryngol Head Neck Surg 1996;114:653-6.
(7.) Davison SP, Habermann TM, Strickler JG, et al. Nasal and nasopharyngeal angiocentric T-cell lymphomas. Laryngoscope (1996;106:139-43.
(8.) Batsakis JG. Midfacial necrotizing diseases. Ann Otol Rhinol Laryngol 1982;91:541-2.
(9.) Sobrevilla-Calvo P, Meneses A, Alfaro P. et al. Radiotherapy compared to chemotherapy as initial treatment of angiocentric centrofacial lymphoma (polymorphic reticulosis). Acta Oncol (1993;32:69-72.
(10.) Aviles A, Rodriguez L, Guzman R, et al. Angiocentric T-cell lymphoma of the nose, paranasal sinuses and hard palate. Hematol Oncol 1992; 10: 141-7.
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|Comment:||Nasal T-cell lymphoma: Case report and review of diagnostic features.|
|Publication:||Ear, Nose and Throat Journal|
|Article Type:||Brief Article|
|Date:||Jul 1, 2001|
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