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Original Report |
1
Department of Diagnostic Radiology and Organ Imaging, Faculty of Medicine, The
Chinese University of Hong Kong, Prince of Wales Hospital, Shatin, New
Territories, Hong Kong SAR, China.
2
Department of Clinical Oncology, Faculty of Medicine, The Chinese University
of Hong Kong, Prince of Wales Hospital, Shatin, New Territories, Hong Kong
SAR, China.
Received March 19, 1999;
accepted after revision June 25, 1999.
Address correspondence to A. D. King.
Abstract
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CONCLUSION. Nasal T-cell/natural killer cell lymphoma frequently exhibits diffuse invasion of the nasal cavity with necrosis, midline destruction, and extension into the nasopharynx. These features may be seen in both early- and late-stage disease.
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Endoscopic examination of the nasal cavity, nasopharynx, oropharynx, hypopharynx, and upper aerodigestive tract was performed on all patients. Histologic evaluation and immunohistochemical studies were performed on the biopsy specimens obtained from the tumor. The diagnosis of nasal T/NK cell lymphoma was based on the criteria described by Jaffe et al. [7]. All patients were staged according to the Ann Arbor system [9] with modification for extranodal lymphoma. Clinical staging procedures included complete history and physical examination; complete blood count; blood chemistry studies; serum lactate dehydrogenase level; plain chest radiograph; CT scans of the head, neck, abdomen, and pelvis; and bone marrow aspiration and biopsy. CT of the thorax was performed only after a patient's chest radiograph showed abnormalities. None of the patients underwent a staging laparotomy or lymphangiography.
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There was involvement of both sides of the nasal cavity in five patients and unilateral involvement in two. In the 12 sides of the nasal cavities with disease, tumor was small and localized to one region (n = 2), moderate and localized to more than one region (n = 2), or involved the entire nasal cavity (n = 8). Tumor volume ranged from 5 to 50 cm3 with a mean of 18 cm3. Sites of extension outside the nasal cavity included tumor extension into the nasopharynx (n = 4), oropharynx (n = 1), paranasal sinuses (n = 3), and palate (n = 1). Of the seven patients, three tumors showed areas of necrosis. There was destruction of the nasal turbinates (n = 4), nasal septum (n = 4), and hard palate (n = 1). There was no regional lymphadenopathy The stage of disease was IE, single extranodal organ or site (n = 6); and stage IVE, diffuse or disseminated involvement of one or more distant extranodal sites with or without associated lymph node involvement (n = 1). The one patient with stage IVE disease had disseminated disease to the bone marrow. Large-volume tumors involving both sides of the nasal cavity with extranasal extension, necrosis, and destruction of the nasal turbinates and bone were found in both stages IE and IVE.
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In this study, the nasal T/NK cell lymphomas were of intermediate signal intensity on T1- and T2-weighted images (Figs. 1 and 2). The intermediate signal is common for many malignant tumors of the head and neck and contrasts to the very hyperintense signal intensity on T2-weighted images that is found almost exclusively in benign membrane and mucosal diseases. Nasal T/NK cell lymphomas in this study showed a predilection for diffuse invasion of the nasal cavity (Fig. 1), often involving both sides. Even in the smaller volume lymphomas there was a tendency to spread as a diffuse thin sheet of tumor along the walls of the nasal cavity to envelop the nasal turbinates and nasal septum (Fig. 2). In larger volume disease a more discernible mass was seen with destruction of the underlying nasal turbinates (Figs. 3 and 4). Midline destruction of the nasal septum occurred in half of the patients (Fig. 4) and involved the palate in one patient. Destruction of the lateral wall of the nasal cavity was not seen, although in one patient the lymphoma caused bowing of the nasal wall into the maxillary antrum. Tumor necrosis, which is a typical histologic feature of nasal T/NK cell lymphoma [7], was shown on MR imaging in all but one of the patients with a tumor volume of greater than 15 cm3 (Figs. 3 and 4).
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The T/NK cell lymphomas were confined to the nasal cavity in two patients. The remaining five patients had tumor extensions outside the nasal cavity, most frequently into the nasopharynx. In two of these five patients, a small volume of tumor extended just beyond the nasal cavity into one wall of the nasopharynx. Two patients showed invasion of a wider area of the nasopharynx, and in the fifth patient the lymphoma extended inferiorly into the oropharynx. Invasion of the paranasal sinuses was less common and of smaller volume when compared with those invading into the nasopharynx and oropharynx. Tumor extension superiorly through the cribriform plate into the cranium was not seen. Regional nodal disease was not identified in this study. This was to be expected because nodal involvement in nasal T/NK cell lymphoma is rare and more commonly associated with the B-cell lymphomas [10].
The diagnosis of nasal T/NK-cell lymphoma requires histologic evidence. MR features alone cannot reliably distinguish this tumor from other nasal cavity tumors such as squamous cell carcinoma, minor salivary gland tumor, esthesioneuroblastoma, rhabdomyosarcoma, and lymphoepithelioma. In particular, the aggressive carcinomas, such as adenocarcinoma and undifferentiated carcinoma, can show a similar low to intermediate signal intensity on T1- and T2-weighted images [14] and they are frequently accompanied by bone destruction [15]. Nonneoplastic conditions including Wegener's granulomatosis, sarcoidosis, cocaine abuse, and infections such as leprosy, syphilis, tuberculosis, and fungus may also cause midfacial destruction. However, the differential diagnosis relies more on clinical and laboratory findings than radiologic findings.
In accordance with the literature, most of the patients with nasal NK/T cell lymphomas in this study presented at an early stage with nearly 90% classified as stage IE [5]. Disseminated disease carries a poorer prognosis and was present in one patient (stage IVE). The patient with disseminated disease showed a large-volume necrotic bilateral nasal cavity tumor extending into the paranasal sinuses and nasopharynx with extensive destructive changes in the nasal septum and palate. However, large-volume disease accompanied by necrosis, bone destruction, or extranasal disease was also found in patients with early stage I disease. This limited sample suggests that there may be a disparity between the severity of the radiologic findings and the stage of disease. Further studies will be required to determine the correlation between the extent of tumor, degree of tissue destruction, and tumor volume revealed by MR imaging and the various clinical characteristics, disease stage, and, ultimately, clinical outcome.
In conclusion, patients with nasal T/NK cell lymphoma frequently present with only localized disease. The MR imaging appearance is nonspecific and diagnosis requires histologic confirmation. However, the predilection for T/NK cell lymphoma to cause a diffuse necrotic mass that spreads to the nasopharynx with destruction of the midline structures—the nasal turbinates, nasal septum and palate—are features that in an Asian patient should suggest the diagnosis of nasal T/NK cell lymphoma.
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