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Original Report |
1
Department of Diagnostic Radiology, The University of Hong Kong, Rm. 405,
Block K, Queen Mary Hospital, Pokfulam, Hong Kong SAR, China.
2
Department of Medicine, The University of Hong Kong, Queen Mary Hospital,
Pokfulam, Hong Kong SAR, China.
Received April 19, 1999;
accepted after revision September 9, 1999.
Address correspondence to G. C. Ooi.
Abstract
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CONCLUSION. Nasal T-cell/natural killer cell lymphoma is often a locally destructive (T stages 3 and 4) disease, typically presenting with obliteration of the nasal passages and maxillary sinuses. Involvement of the adjacent alveolar bone, hard palate, orbits, and nasopharynx is found in more than 50% of cases and is associated with extensive soft-tissue masses. Presence of bone erosion is suggestive but not diagnostic of the disease.
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(but not CD3 and the
T-cell receptor), and CD56. Genotypically, the T-cell receptor gene in
germline configuration classifies them. T/NK-cell lymphomas can be divided
into three types according to the predominant sites involved. Most cases occur
in the nose and upper aerodigestive system and are referred to as nasal
T/NK-cell lymphoma. Few cases occur in areas other than the nose and are
referred to as nonnasal-type T/NK-cell lymphoma. Rarely, the disease
presents with a leukemia phase and is referred to as T/NK-cell
lymphoma/leukemia [3,
4]. Nasal T/NK-cell lymphomas have been known by various names, including lethal midline granuloma, polymorphic reticulosis, and other more obscure terms such as progressive lethal granulomatous ulceration, malignant granuloma, nonhealing granuloma, and midline malignant reticulosis [5, 6]. All these diseases were destructive lesions involving the nasal cavity, oropharynx, upper palate, and larynx. However, with the advent of immunophenotyping, these lesions were found to be lymphoid in nature and so should now be regarded as nasal lymphoma [7]. Most nasal lymphomas are of T/NK-cell lineage, although some of these lymphomas could be of B- or T-cell lineage [4]. Nevertheless, B- and T-cell lymphomas of the nose are rarely primary tumors and usually represent metastases from other lymph node sites [4]. On the other hand, nasal T/NK-cell lymphomas are predominantly primary tumors [3, 4].
Both nasal and nonnasal T/NK-cell lymphoma pursue an aggressive clinical course but little is known of the imaging features [3, 4]. We have therefore performed this study to evaluate and summarize pertinent imaging features of nasal T/NK-cell lymphoma. The CT and MR imaging findings of nine cases of nasal T/NK-cell lymphoma are described.
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The database search yielded 36 cases of nasal T/NK-cell lymphoma. Of these cases, CT or MR images or both were available for evaluation in only nine cases (six men and three women; mean age ± SD, 56.5 ± 14 years). Few images were available because in most cases chemotherapy was instituted immediately after histologic diagnosis and Ann Arbor staging. Local tumor staging is not mandatory for the Ann Arbor staging system. Eight patients underwent axial or coronal CT examination of the sinonasal area, six with IV contrast medium. One patient underwent unenhanced MR examination of the sinonasal area only, and two patients had both enhanced CT and MR examinations of the sinonasal area. CT examinations were performed on either of two scanners: a 9800 or a HiSpeed Advantage (General Electric Medical Systems, Milwaukee, WI). MR imaging was performed using a 1.5-T system (Signa; General Electric Medical Systems). CT images were obtained in soft-tissue and bone algorithms and filmed in the respective window settings. Both CT and MR images were evaluated for site and extent of disease and for pattern of involvement of adjacent sites. A modified local tumor (T) staging system for nasal cell lymphoma was devised based on previous criteria [8]: T1, tumor confined to the nasal cavity; T2, extension of tumor to the maxillary antra, anterior ethmoidal sinus, or hard palate; T3, extension to the posterior ethmoidal sinus, sphenoidal sinus, orbit, superior alveolar bone, cheeks, or superior buccinator space; and T4, tumor involvement of the inferior alveolar bone, inferior buccinator space, infratemporal fossa, nasopharynx, or cranial fossa.
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Site of Disease
Six patients had disease primarily in the nasal cavity with a soft-tissue
mass (with or without bony erosion) of the nasal septum and turbinates. Of
these, two patients had disease confined solely to the nasal cavity, and four
patients had disease extension into the paranasal sinuses (n = 4),
alveolar bones (n = 2), hard palate (n = 1), and
subcutaneous tissues of the nasal ala, cheek, and buccinator spaces
(n = 3) (Figs.
1A,1B,1C
and 2). In two patients, the
disease site was in the superior nasal cavity with adjacent orbital,
ethmoidal, and maxillary sinus involvement
(Fig. 3). Extensive
infratemporal space and nasopharyngeal involvement was found in the first
patient, a 62-year-old woman (Fig.
3). In the second patient, a 37-year-old man, disease was present
in the hard palate with a nasal component, nasopharynx, and anterosuperior
alveolar bone, with soft-tissue involvement of the buccinator spaces,
infratemporal fossa, and temporalis muscle. No significantly enlarged
parapharyngeal or cervical lymph nodes were noted in any case.
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Bone Involvement
Bone erosion with no osteoblastic (sclerotic) destruction was present in
seven patients. Erosion of the medial maxillary wall was present in all
patients with maxillary antra extension, whereas posterior wall destruction
was seen in only one patient with tumor spread to the infratemporal space and
temporalis muscle (Fig. 3).
Anterior maxillary wall erosion was found in only one of three patients with
tumor extension into the nasal ala, cheek, and lips
(Fig. 2). Orbital floor and
lamina papyracea were destroyed in both patients with orbital and ethmoidal
tumor involvement, a 62-year-old woman and a 40-year-old woman. Both women
also had maxillary antra involvement. Alveolar bone erosion was present in
three patients with extensive soft-tissue infiltration of the buccinator
space. In two of these patients, the adjacent hard palate was also involved
(Fig. 4D).
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Soft-Tissue Involvement
MR imaging was superior to CT in characterizing soft-tissue involvement. In
the three patients with MR images, tumoral tissue appeared isointense to
muscle on T1-weighted sequences, with mild-to-moderate hyperintensity to
muscle on T2-weighted sequences (Fig.
4A,4B,4C,4D).
Mild-to-moderate heterogeneous enhancement occurred in all involved soft
tissues on CT and MR imaging. Fluid retention or mucosal thickening or both
were noted in apparently uninvolved paranasal sinuses of five patients. Fluid
was nonenhancing and markedly hyperintense to muscle on T2-weighted images
(Fig.
4A,4B,4C,4D),
whereas mucosal thickening could be differentiated from tumor by both its
homogenous hyperintensity with respect to muscle on T2-weighted images and its
homogeneous enhancement. In one patient, enhancing inflammatory polypoid
lesions, which were hyperintense to muscle on T2-weighted images, were noted
in both maxillary sinuses (Fig.
4A,4B,4C,4D).
In another patient, hemorrhagic fluid, which appeared hyperdense on CT and
hyperintense and isointense to muscle on T1- and T2-weighted images,
respectively, was noted in a maxillary sinus of a 40-year-old woman.
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Recently, immunophenotype has been shown to significantly influence survival and prognosis of patients with nasal lymphoma [4]. Patients with nasal T/NK-cell lymphoma have the worst prognosis, with a young median age of presentation (50 years), compared with those with B- and T-cell nasal lymphoma [4]. It is not clear whether similar differences can be found in the radiologic features of the three different immunophenotypes of nasal lymphoma because, to our knowledge, no such comparative studies have been performed. However, in two previous series evaluating sinonasal non-Hodgkin's lymphoma, CT evidence of bone destruction was reported in 40% of the cases, despite the presence of disease on both sides of a bony boundary [9, 10]. In comparison, bone erosion in our nasal T/NK-cell patients was present in 78%, the most common sites being the medial maxillary wall, nasal septum, and lamina papyracea. Palatal and alveolar margin erosions were associated with tumor spread to the buccinator space, whereas posterior maxillary wall destruction was found with disease extension into the infratemporal space. Despite marked soft-tissue involvement of the midface region, the anterior maxillary wall was rarely involved. However, compared with sinonasal squamous cell carcinoma, which has been reported to destroy at least one maxillary wall in 85% of cases [11], bone destruction in the cases of nasal T/NK-cell lymphoma we studied was less severe.
Radiologic differential diagnoses of nasal T/NK-cell lymphoma include other causes of sinonasal destruction such as Wegener's granulomatosis, other non-Hodgkin's lymphoma, granulomatous infections, adenoid cystic carcinoma, olfactory neuroblastoma, malignant melanoma, and squamous cell carcinoma. Wegener's granulomatosis is a systemic disorder with involvement of the kidneys and lungs. It is also more localized and rarely, if ever, involves the palate, alveolar bone, or skin. We have already illustrated that bone permeation reported in non-Hodgkin's lymphoma is not a feature consistent with T/NK-cell lymphoma [11]. With granulomatous infections such as allergic aspergillosis, linear interlacing high densities and bone remodeling and thickening are common CT features [12]. Squamous cell carcinoma of the sinuses remains an important radiologic differential diagnosis and may be distinguished from nasal T/NK-cell lymphoma by the presence of more severe bone destruction, lack of enhancement on CT, and a predilection for the maxillary sinuses [11].
In conclusion, the imaging features of nasal T/NK-cell lymphoma are nonspecific, sharing similarities with other aggressive lesions. However, features that may suggest the diagnosis are large soft-tissue masses obliterating the nasal cavity with bone erosion and frequent involvement of the maxillary sinuses. Extensive midface soft-tissue, oropharyngeal, infratemporal, and orbital involvement are common at presentation and are indicative of advanced (T stages 3 and 4) disease. CT and MR imaging are complementary techniques in evaluating the local stage of this aggressive form of lymphoma.
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