J Korean Rheum Assoc.  2010 Sep;17(3):289-294.

A Case of NK/T Cell Lymphoma Mimicking Limited Form of Wegener's Granulomatosis

• Park YJ
• Cho CS

Abstract

It is occasionally difficult to distinguish Wegener's granulomatosis (WG) from other diseases including malignancy, tuberculosis, and various types of vasculitis because of the overlapping symptoms and signs. We report on a patient with NK/T cell lymphoma who was treated with a limited form of WG. At his first visit, he presented with left foot drop and recurrent nasal swelling. Necrosis and massive infiltration of inflammatory cells were identified on a nasal tissue biopsy. Sural nerve biopsy findings also showed infiltration of inflammatory cells in both the endoneurium and perivascular area; thus, a diagnosis of a limited form of WG was made. After combination therapy with a glucocorticoid and oral cyclophosphamide was initiated, his condition completely recovered without recurrence for the next 2 years. However, he visited the hospital again for recurrence of nasal swelling. Repeated biopsy of nasal tissues, combined with an immunophenotypic analysis revealed NK/T cell lymphoma. The possibility of NK/T lymphoma should be considered when evaluating a limited type of WG, which shows atypical findings on biopsy as well as recurrent deterioration, as a suboptimal dose of immunosuppressive therapy may mask its expression and lead to a poor prognosis.

Keyword

Limited form of Wegenr's granulomatosis; NK/T cell lymphoma

MeSH Terms

Biopsy
Cyclophosphamide
Foot
Humans
Lymphoma
Masks
Necrosis
Organic Chemicals
Peripheral Nerves
Prognosis
Recurrence
Sural Nerve
Tuberculosis
Vasculitis
Wegener Granulomatosis
Cyclophosphamide
Organic Chemicals

Figure

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  Fig. 1. Initial radiologic examinations and tissue biopsy. (A) Paranasal sinusoid computed tomography scan on admission (3 years ago) shows thickening of the mucoperiosteal lining of the frontal sinus, ethmoid sinus, and maxillary sinuses. Near total opacification of the right maxillary sinus and mucosal thickening of the left maxillary sinus are also seen. (B) A focally dense, mass-like lesion was seen in the anterior nostril area. (arrow) (C) Necrosis and massive infiltration of inflammatory cells were observed on a nasal tissue biopsy. (D) A sural nerve biopsy indicated that inflammatory cells had infiltrated both the endoneurium with axonal degeneration and the perivascular area.

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  Fig. 2. Axial T1 weighted magnetic resonance images show heterogeneously enhancing mass at the anterior nostril area (arrows) and the nasal cavity structure is destroyed. Mucoperiosteal thickening still remained on the frontal, ethmoid, sphenoid, and maxillary sinuses.

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  Fig. 3. Histology of the nasal cavity mass. (A) In highpower fields (hematoxylin- eosin stain, 400) many small lymphocytes infiltrated the lymphoma lesion. The immunophenotype of the tumor cells was positive for CD3, CD56, granzyme B, but negative for CD20.

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