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Extranodal natural killer/T-cell lymphoma, nasal type, in a 50-year-old black man
A 50-year-old black man initially presented to his ear, nose and throat specialist with a 2-month history of sinusitis that was not relieved by antibiotic therapy. His symptoms at the time included nasal swelling and congestion; he reported no fevers, night sweats or chills.
Several weeks passed. During this time the patient was prescribed a 2-week course of sulfamethoxazole and trimethoprim (Bactrim, Mutual) and ciprofloxacin. Despite antibiotic therapy, the patient’s symptoms worsened and now included a foul-smelling bloody discharge from the left nostril and worsening congestion.
On physical examination, the patient was afebrile, in no acute distress, Karnofsky performance status of 90, with significant skin swelling and firmness over the bilateral paranasal areas. Nasal examination demonstrated yellowish-bloody discharge draining from the left nostril.
The pharynx appeared normal; no masses were noted in the neck. There was no palpable lymphadenopathy in the cervical, supraclavicular or axillary regions.
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A CT scan of the sinuses was performed, demonstrating a soft tissue mass within the left nasal cavity extending anteriorly to involve the left nasal vestibule, surrounding the frontal process of the maxillary sinus. A biopsy was conducted revealing extranodal natural killer/T-Cell lymphoma, nasal type, positive for CD3, CD45, granzyme B, and weak focally positive CD56, with a proliferation rate of approximately 30% to 50%.
Further workup included a PET scan and cerebrospinal fluid analysis. The PET showed a soft tissue mass involving bilateral nasal cavity measuring 5.4 cm × 2.6 cm with positive uptake with an standardized uptake value of 26.4, in addition to avid uptake in a single left cervical lymph node. Cerebrospinal fluid analysis was negative. The patient’s clinical staging was stage IIe. What follows is a brief discussion on the management of early-stage nasal natural killer/T-cell lymphoma.
Which of the following statements about extranodal natural killer/T-cell lymphoma is not correct?
A)It is characterized by CD56 and cytoplasmic CD3 expression, a germline configuration of the T-cell receptor gene, and a strong association with Epstein-Barr virus.
B)These tumors tend to have an indolent growth pattern, formerly known as latent angiogenic lymphoma.
C)Radiation therapy followed by, or combined with, chemotherapy has been demonstrated as the optimal initial treatment in multiple recent retrospective reviews.
D)Although primary nasal lymphomas are uncommon, they show predilections for people of Asian, Latin and South American descent.
Correct Answer: B
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Courtesy of L Dad, MD |
Case Discussion
Extranodal natural killer (NK)/ T-cell lymphoma, nasal type, (formerly known as angiocentric lymphoproliferative lesions), recently was recognized by WHO as a distinct entity of malignant lymphoma.
This type of lymphoma often demonstrates an angiocentric and angiodestructive growth pattern. Primary nasal lymphomas are uncommon and show predilections for people of Asian, Latin and South American descent. There is also a strong association with Epstein-Barr virus. This lymphoma is characterized by expression of NK cell marker CD56 and lack of expression of surface CD3, but presence of cytoplasmic CD3.
The optimal treatment for this unique group of patients is under debate. However, the response to radiotherapy generally is so rapid and dramatic that the delivery of radiotherapy has become accepted as the preferred treatment of choice for localized disease. Given the rarity of this tumor, we are limited to retrospective literature reviews.
Furthermore, most information in the literature has reported no significant clinical benefit with the addition of chemotherapy to radiation therapy. Historically, the 5-year DFS and OS range from 30% to 40%. Several clinical factors have been reported to have prognostic significance in nasal NK/T-cell lymphomas, including stage, B symptom, age and bulk.
Based on the largest experiences, the recommended target volume includes all macroscopic lesions, the paranasal sinuses, the nasopharynx, nasal cavity and any adjacent lymph node groups. Improved local control rates have been observed with doses delivering 50 Gy or more to the target volume. The consensus appears to be that total doses higher than that generally recommended for non-Hodgkin’s lymphoma may be needed for controlling this aggressive disease. In this case, our plan was to deliver 60 Gy to the gross tumor volume.
The sequence of therapy has been evaluated in the retrospective setting, with improved outcome with respect to locoregional control and OS noted in patients receiving radiotherapy upfront, followed by consolidative chemotherapy. The only phase 2 randomized clinical trial in this cohort of patients evaluating the role of induction chemotherapy with semustine did not demonstrate improvement in response, PFS or OS. The reasons for resistance to chemotherapy and the general aggressive nature of this tumor type has not been elucidated; however, delivery of therapy to largely necrotic angio-destructed tissue, and expression of multidrug resistance phenotype have been implicated.
In conclusion, primary nasal NK/T-cell lymphomas demonstrates overall poor outcome, and lack of survival benefit with systemic agents. Given the rarity of this tumor, it is difficult to attain prospective-randomized data to help guide development of optimal treatment approaches. However, this should not preclude major centers from cooperative research to promote the evaluation of novel experimental treatment paradigms in a prospective setting.
Luqman Dad, MD, is a resident physician in the department of radiation medicine at Roswell Park Cancer Institute Buffalo, N.Y.
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