An unusual presentation of NK/T-cell lymphoma, nasal-type in the United States




Abstract


Introduction


NK/T-cell lymphoma (NKCL), nasal-type is rare in the United States, representing only 1.5% of non-Hodgkin lymphomas. Classically, patients initially present with nasal obstruction (70%), caused by invasion of the localized lesion into the sinuses and nasal cavities. Initial presentation with persistent sore throat and odynophagia due to oropharyngeal tumor extension is rare, and thus, is often overlooked as viral or bacterial pharyngitis. By studying a case of NKTCL nasal type, we emphasize the need to apply high clinical suspicion for NKTCL, nasal type for early diagnosis and improved survival.


Methods


A case report of a rare presentation of NKTCL, nasal-type is discussed. A literature review is provided to define clinical signs crucial for early diagnosis, appropriate work-up, and expedient treatment of this aggressive, rapidly progressive malignancy.


Results


In the present case, a 25 year-old healthy male presented with a 2-week history of sore throat and odynophagia. On exam, the patient had an ulcerative lesion of the soft palate, an enlarged uvula, and tonsillar exudate with tender submandibular lymphadenopathy. After the patient failed to respond to antibiotic therapy for presumptive pharyngitis, a biopsy of the oropharyngeal tissue was completed, which identified necrotizing sialometaplasia. High clinical suspicion led to repeat deep-tissue biopsy, where a final diagnosis of NKTCL, nasal type was made. The patient then began definitive treatment with chemotherapy and radiation.


Conclusions


High clinical suspicion is key to early diagnosis and improved survival of NKTCL, nasal-type. Otolaryngologists who encounter prolonged, complicated cases of pharyngitis or necrotizing sialometaplasia should consider a diagnosis of NKTCL, nasal-type, in order to prevent rapid disease progression.



Introduction


Extranodal NK-cell/T-cell lymphoma (NKTCL), nasal type, is a rare extranodal lymphoma of NK-cell or T-cell origin that most commonly affects immunocompetent middle-aged men of Asian, Native American, or Central/South American decent . It is almost always associated with Ebstein–Barr virus (EBV). As a result, EBV viral load is intimately tied to prognosis, clinical course, and disease relapse . NKTCL account for nearly 6–7% of all non-Hodgkin’s lymphoma (NHL) in certain geographic areas such as Southeast Asia . In the United States, however, the incidence is lower at 1.5% of NHL and usually seen in individuals of South Asian or Latin American descent .


NKTCL, nasal type most frequently affects the nasal cavity and upper aerodigestive tract, particularly in extranodal sites, which make diagnosis difficult and often mistaken for reactive disease processes. Within these regions, NKTCL causes extensive destruction of the cartilages and surrounding soft tissues as a result of inflammatory processes and angiodestructive behavior, which leads to widespread necrosis . Disease within the nasal cavity has a better prognosis and can be curative with radiation therapy alone . Unfortunately, while nasal disease may be cured with radiotherapy 85% of the time, the relapse rate is high at 25% . When disease has spread to adjacent sites, however, it can compromise local function and clinical response to treatment falls greatly . Early diagnosis and treatment of this aggressive disease are thus crucial.


A review of the current literature demonstrates several cases of NKTCL, nasal type in high prevalence nations, but none presenting with sore throat and dysphagia in the United States. We present one case of NKTCL, nasal type in a 25 year-old previously healthy male, which was originally identified as necrotizing sialometaplasia. In this case, high clinical suspicion led to repeat deep-tissue biopsy and a correct diagnosis. We highlight key clinical features that help make early diagnosis and administer early treatment.





Case report


A 25-year-old male immigrant from Mexico with history of non-Hodgkin’s lymphoma treated with chemotherapy presented with a 2-week history of sore throat in December of 2012. He also reports an ulcerative lesion of the left soft palate with some mucosal sloughing. He had no fevers, cough, night sweats, or weight loss. Prior to this, the patient had been seen at an outside clinic and was prescribed pain medication. Physical examination revealed slight edema and erythema of the uvula, tonsils and soft palate. The uvula remained at midline with asymmetric elevation of and yellow mucous on the left soft palate. There was tender bilateral submandibular lymphadenopathy. Complete blood count showed normal levels of white blood cells and neutrophils, but decreased hemoglobin (11.7 g/dL) and hematocrit (35.9%). The patient was diagnosed with pharyngitis, prescribed augmentin, and asked to follow-up in 1 week.


At his 1-week follow-up, the patient reported no improvement in symptoms. He denied fevers, respiratory distress, voice changes, or neck pain. Physical examination showed an enlarged uvula, large ulcer of the left soft palate, and exudate over the uvula and right tonsillar fossa, as shown in Fig. 1 . No lymphadenopathy could be appreciated. Biopsies of the midline soft palate and left soft palate were obtained. The patient was switched to clindamycin and asked to return after 1 week for pathology results.




Fig. 1


Patient with NK/T-cell lymphoma, nasal-type of the soft palate and tonsils.


Pathology results showed acute and chronic sialadenitis with sialometaplasia, lymphoplasmacytic infiltrate within the midline soft palate, and a lymphoplasmacytic infiltrate of the left soft palate. A pathological diagnosis of necrotizing sialometaplasia was reported. However, given the high clinical suspicion, repeated biopsies were taken at the affected regions. Repeat biopsy showed infiltration by extranodal NK/T-cell lymphoma, nasal type and geographic zones of tumor necrosis. EBV viral copies were not present on quantitative EBV PCR. Immunohistochemical stains of the midline soft palate showed variably-sized atypical lymphoid cells that were CD2 +, CD3 +, CD8 +, CD16 weakly +, and CD56 weakly +. Immunohistochemical stains of the left soft palate showed CD3 +, CD8 +, and CD4 + atypical lymphoid cells.


With a confirmed pathological diagnosis, the patient was transferred to the Hematology/Oncology Service 22 days after initial presentation of his condition. He was advised to undergo radiotherapy with concurrent chemotherapy involving dexamethasone, etoposide, ifosfamide, and carboplatin, given his aggressive and rapidly progressing disease. One week later, the Otolaryngology service was consulted for shortness of breath and worsening of the oropharyngeal mass. On physical examination, the left soft palate mass had enlarged and there was some edema of the epiglottis, arytenoids, and posterior oropharynx with narrowing of the airway. To protect the airway, an elective tracheostomy prior to radiation for airway protection was advised. The patient successfully completed treatment and was transferred to an outside hospital for supportive care.





Case report


A 25-year-old male immigrant from Mexico with history of non-Hodgkin’s lymphoma treated with chemotherapy presented with a 2-week history of sore throat in December of 2012. He also reports an ulcerative lesion of the left soft palate with some mucosal sloughing. He had no fevers, cough, night sweats, or weight loss. Prior to this, the patient had been seen at an outside clinic and was prescribed pain medication. Physical examination revealed slight edema and erythema of the uvula, tonsils and soft palate. The uvula remained at midline with asymmetric elevation of and yellow mucous on the left soft palate. There was tender bilateral submandibular lymphadenopathy. Complete blood count showed normal levels of white blood cells and neutrophils, but decreased hemoglobin (11.7 g/dL) and hematocrit (35.9%). The patient was diagnosed with pharyngitis, prescribed augmentin, and asked to follow-up in 1 week.


At his 1-week follow-up, the patient reported no improvement in symptoms. He denied fevers, respiratory distress, voice changes, or neck pain. Physical examination showed an enlarged uvula, large ulcer of the left soft palate, and exudate over the uvula and right tonsillar fossa, as shown in Fig. 1 . No lymphadenopathy could be appreciated. Biopsies of the midline soft palate and left soft palate were obtained. The patient was switched to clindamycin and asked to return after 1 week for pathology results.


Aug 23, 2017 | Posted by in OTOLARYNGOLOGY | Comments Off on An unusual presentation of NK/T-cell lymphoma, nasal-type in the United States

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