AMER ICA N JOURNAL OF OT OLARYNGOLOGY –HE A D A N D N EC K ME D IC IN E AN D S U R G ER Y 3 6 ( 2 01 5 ) 8 0 –8 3
Available online at www.sciencedirect.com
ScienceDirect www.elsevier.com/locate/amjoto
Case reports
An unusual presentation of NK/T-cell lymphoma, nasal-type in the United States Sarah M. Kidwai, B.S.⁎, Arjun K. Parasher, M.D., Fred Y. Lin, M.D. Department of Otolaryngology-Head and Neck Surgery, Icahn School of Medicine at Mount Sinai, New York, NY, USA
ARTI CLE I NFO
A BS TRACT
Article history:
Introduction: NK/T-cell lymphoma (NKCL), nasal-type is rare in the United States,
Received 3 July 2014
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, nasaltype, in order to prevent rapid disease progression. © 2015 Elsevier Inc. All rights reserved.
1.
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
[1]. 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 [2]. NKTCL account for nearly 6–7% of all non-Hodgkin's lymphoma (NHL) in certain geographic areas such as Southeast Asia [3,4]. In the United States, however, the incidence is lower at 1.5% of NHL
⁎ Corresponding author at: Department of Otolaryngology-Head and Neck Surgery, Icahn School of Medicine at Mount Sinai, New York, NY, USA. E-mail address: [email protected] (S.M. Kidwai). http://dx.doi.org/10.1016/j.amjoto.2014.07.012 0196-0709/© 2015 Elsevier Inc. All rights reserved.
AMER ICA N JOURNAL OF OT OLARYNGOLOGY –H EA D A N D N EC K ME D I CIN E AN D SUR G E RY 3 6 ( 2 01 5 ) 8 0 –8 3
and usually seen in individuals of South Asian or Latin American descent [5]. 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 [6]. Disease within the nasal cavity has a better prognosis and can be curative with radiation therapy alone [7]. Unfortunately, while nasal disease may be cured with radiotherapy 85% of the time, the relapse rate is high at 25% [8,9]. When disease has spread to adjacent sites, however, it can compromise local function and clinical response to treatment falls greatly [5]. 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 yearold 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.
2.
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. 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
81
Fig. 1 – Patient with NK/T-cell lymphoma, nasal-type of the soft palate and tonsils.
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.
Table 1 – Cases of NKTCL, nasal type, involving the palate and sinuses reported in nations other then the United States. Study
Location
Number of cases reported
Gualco et al. [10] Ikeda et al. [11] Nikoloaos et al. [12] Reinartz et al. [13] Meng et al. [14] Chaudhary et al. [15] Ramanathan et al. [16] Kanitsap et al. [17]
Brazil Japan Greece The Netherlands China India Malaysia Thailand
97 5 1 2 1 1 42 1
82
AMER ICA N JOURNAL OF OT OLARYNGOLOGY –HE A D A N D N EC K ME D IC IN E AN D S U R G ER Y 3 6 ( 2 01 5 ) 8 0 –8 3
Table 2 – Cases of NKTCL, nasal type, in the United States. Study
Location
Number of cases presented Presenting symptoms
Healey [18] Wood et al. [19] Gaarl et al. [20]
New Orleans, LA Dallas, TX Los Angeles, CA
1 1 12
Prouix et al. [21] Li et al. [22]
Boston, MA Houston, TX
8 63
3.
Discussion
Extranodal NK/T-cell lymphoma, nasal type is rare in the United States, and usually arises in patients endemic to East Asia, South and Central America. In our review of the literature, there are many reports of NKTCL of the palate and sinuses in nations other than the United States. These cases are presented in Table 1. In the United States, where the incidence of NKTCL is much lower, fewer cases have been reported. These are presented in Table 2. All of these cases presented with sinonasal symptoms or facial swelling. In all of these case reports in the United States, none presented with persistent sore throat, as in the case described here. Patients usually present with nasal obstruction, bleeding, pain, or local swelling. It can produce ulcerative, destructive lesions within extranodal sites. While the nasal cavity is the typical site of origin, as outlined by our review of the literature, it can present with involvement of other midline structures within the upper aerodigestive tract, including the palate and nasopharynx. Often, it is associated with the Ebstein–Barr virus (EBV). Histologically, extranodal NKTCL, nasal type, is characterized by angiocentric and angiodestructive growth and by tumor cells that vary in size. In addition, an inflammatory cell infiltrate of plasma cells, histiocytes, and eosinophils is present [23]. Immunophenotypically, the tumor cells express CD2, CD3, and CD56. In some cases, the cells can lack CD56 and express CD8+ T cell antigens [23]. This case highlights the aggressive and rapidly progressive nature of this disease. The course of NKTCL, nasal type is aggressive with a 5-year overall survival ranging from 25 to 50% [24]. In addition, the neoplasms can be associated with hemophagocytic syndrome, which is clinically characterized by fever, hepatosplenomegaly, pancytopenia, and laboratory evidence of hemolysis. Given this, we highlight key clinical features that allow practitioners to make the correct diagnosis. While nasal obstruction, nasal discharge, facial swelling, and other nonspecific sinonasal symptoms have been recognized as presenting symptoms of NKTCL, nasal type, other less common signs and symptoms are often overlooked. Sore throat and dysphagia, for example, while known as presenting symptoms of NKTCL, nasal type, are often missed and treated as viral and bacterial pharyngitis, leading to delay of diagnosis and treatment that increases morbidity and mortality. In this case, the patient did not respond to initial courses of antibiotics and initial biopsy showed chronic sialadenitis with sialometaplasia. It was only heightened suspicions that
3-cm nasal mass Facial swelling, fever, unintentional weight loss Eleven patients presented with nasal masses, with some having ulceration, septal deviation or destruction, and/or involvement of contralateral nasal cavity or adjacent sinuses; one presented with isolated paranasal sinus lesion Nasal obstruction and localized soft-tissue swelling Nonspecific sinonasal symptoms, such as nasal obstruction, nasal discharge, or facial swelling
led to a repeat deep-tissue biopsy and diagnosis of NKTCL, nasal type, rather than conservative therapy and prolonged follow-up for necrotizing sialometaplasia, which would have led to worsening condition and rapid progression of the patient's NKTCL. The patient then began treatment of his diagnosed NKTCL weeks after his initial presentation. For cases of persistent sore throat and dysphagia, a diagnosis of NKTCL, nasal type should be considered, especially in individuals of Native American, Central, or South American descent. Suspicious lesions and clinical features should prompt practitioners to initiate work-up to rule out NKTCL, nasal type. In addition, when a diagnosis of necrotizing sialometaplasia is considered, NKTCL, nasal type should also be considered, as they can often present similarly. As in this case, deep-tissue biopsies to ensure accurate diagnosis are crucial.
4.
Conclusion
As evident by our review of the literature, NK/T-cell lymphomas, nasal type, are rare in the United States. Furthermore, cases that present with persistent sore throat have not been reported in the United States. Thus, there is a need for a high index of suspicion for early diagnosis of NK/T-Cell lymphomas.
REFERENCES
[1] Roschewski M, Wilson WH. EBV-associated lymphomas in adults. Best Pract Res Clin Haematol 2012;25(1):75–89. [2] Suzuki R, Yamaguchi M, Izutsu K, et al. Prospective measurement of Epstein–Barr virus–DNA in plasma and peripheral blood mononuclear cells of extranodal NK/T-cell lymphoma, nasal type. Blood 2011;118:6018–22. [3] Hahn JS, Lee ST, Min YH, et al. Therapeutic outcome of Epstein–Barr virus positive T/NK cell lymphoma in the upper aerodigestive tract. Yonsei Med J 2002;43(2):175–82. [4] Van Prooyen KS, Eloy P, Delos M, et al. Sinonasal lymphomas: case report. Acta Otorhinolaryngol Belg 2000;54(1):45–51. [5] Frierson HF, Mills SE, Innes DJ. Non-Hodgkin's lymphomas of the sinonasal region: histologic subtypes and their clinicopathologic features. Am J Clin Pathol 1984;81:721–7. [6] Al-Hakeem DA, Fedele S, Carlos R, et al. Extranodal NK/T-cell lymphoma, nasal type. Oral Oncol 2007;43:4–14. [7] Jaccard A, Hermine O. Extranodal natural killer/T-cell lymphoma: advances in the management. Curr Opin Oncol 2011;23:429–35.
AMER ICA N JOURNAL OF OT OLARYNGOLOGY –H EA D A N D N EC K ME D I CIN E AN D SUR G E RY 3 6 ( 2 01 5 ) 8 0 –8 3
[8] Liang R. Diagnosis and management of primary nasal lymphoma of T-cell or NK-cell origin. Clin Lymphoma 2000;1 (1):33–7 [discussion 38]. [9] Kim GE, Cho JH, Yang WI, et al. Angiocentric lymphoma of the head and neck: patterns of systemic failure after radiation treatment. J Clin Oncol 2000;18:54–63. [10] Gualco G, Domeny-Duarte P, Chioate L, et al. Clinicopathologic and molecular features of 122 Brazilian cases of nodal and extranodal NK/T-cell lymphoma, nasal type, with EBV subtyping analysis. Am J Surg Pathol 2011;35: 1195–203. [11] Ikeda T, Kanaya T, Matsuda A, et al. Clinicopathologic study of non-Hodgkin lymphoma in sinonasal and hard palate regions in 15 Japanese cases. J Otorhinolaryngol Relat Spec 2005;67:23–9. [12] Nikolaos N, Grigorios P, Konstantinos K, et al. Extranodal nasal-type NK/T-cell lymphoma of the palate and paranasal sinuses. Am J Case Rep 2012;13:79–85. [13] Reinartz SM, Schot LJ, Riedl RG, et al. Presentation of two cases of nasal type NK/T-cell lymphoma. Eur Arch Otorhinolaryngol 2007;264:39–43. [14] Meng W, Zhou Y, Zhang H, et al. Nasal-type NK/T-cell lymphoma with palatal ulcer as the earliest clinical manifestation: a case report with literature review. Pathol Oncol Res 2010;16:133–7.
83
[15] Chaudhary SV, Karnik ND, Sabnis GR, et al. Extranodal NK/T-cell lymphoma presenting as palatal perforation with oronasal fistula. J Assoc Physicians India 2011;59:112–4. [16] Ramanathan A, Mahmoud HA, Hui LP, et al. Oral extranodal non-Hodgkin's lymphoma: series of forty two cases in Malaysia. Asian Pac J Cancer Prev 2014;15:1633–7. [17] Kanitsap N, Warnnissorn N. NK/T-cell lymphoma, nasal type with sinonasal mass and palatal ulcer: a clinical case report and review of treatment. J Med Assoc Thai 2010;93:S294–8. [18] Healey JL. Case study interpretation—New Orleans: case 3. Clin Cytom 2013;84B:346–9. [19] Wood PB, Parkikh SR, Krause JR. Extranodal NK/T-cell lymphoma, nasal type. Proc (Baylor Univ Med Cent) 2011;24:251–4. [20] Gaarl K, Sun N, Hernandex A, et al. Sinonasal NK/T-cell Lymphomas in the United States. 2000;24:1511–7. [21] Prouix G, Caudra-Garcia I, Ferry J, et al. Lymphoma of the nasal cavity and paranasal sinuses. Am J Clin Oncol 2003;26:6–11. [22] Li S, Feng X, Li T, et al. Extranodal NK/T-cell lymphoma, nasal type: a report of 73 cases at MD Anderson Cancer Center. Am J Surg Pathol 2013;37:14–23. [23] Hasserjian RP, Harris NL. NK-cell lymphomas and leukemias: a spectrum of tumors with variable manifestations and immunophenotype. Am J Clin Pathol 2007;127:860–8. [24] Kwong YL. Natural killer-cell malignancies: diagnosis and treatment. Leukemia 2005;19(12):2186–94.
Available online at www.sciencedirect.com
ScienceDirect www.elsevier.com/locate/amjoto
Case reports
An unusual presentation of NK/T-cell lymphoma, nasal-type in the United States Sarah M. Kidwai, B.S.⁎, Arjun K. Parasher, M.D., Fred Y. Lin, M.D. Department of Otolaryngology-Head and Neck Surgery, Icahn School of Medicine at Mount Sinai, New York, NY, USA
ARTI CLE I NFO
A BS TRACT
Article history:
Introduction: NK/T-cell lymphoma (NKCL), nasal-type is rare in the United States,
Received 3 July 2014
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, nasaltype, in order to prevent rapid disease progression. © 2015 Elsevier Inc. All rights reserved.
1.
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
[1]. 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 [2]. NKTCL account for nearly 6–7% of all non-Hodgkin's lymphoma (NHL) in certain geographic areas such as Southeast Asia [3,4]. In the United States, however, the incidence is lower at 1.5% of NHL
⁎ Corresponding author at: Department of Otolaryngology-Head and Neck Surgery, Icahn School of Medicine at Mount Sinai, New York, NY, USA. E-mail address: [email protected] (S.M. Kidwai). http://dx.doi.org/10.1016/j.amjoto.2014.07.012 0196-0709/© 2015 Elsevier Inc. All rights reserved.
AMER ICA N JOURNAL OF OT OLARYNGOLOGY –H EA D A N D N EC K ME D I CIN E AN D SUR G E RY 3 6 ( 2 01 5 ) 8 0 –8 3
and usually seen in individuals of South Asian or Latin American descent [5]. 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 [6]. Disease within the nasal cavity has a better prognosis and can be curative with radiation therapy alone [7]. Unfortunately, while nasal disease may be cured with radiotherapy 85% of the time, the relapse rate is high at 25% [8,9]. When disease has spread to adjacent sites, however, it can compromise local function and clinical response to treatment falls greatly [5]. 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 yearold 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.
2.
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. 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
81
Fig. 1 – Patient with NK/T-cell lymphoma, nasal-type of the soft palate and tonsils.
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.
Table 1 – Cases of NKTCL, nasal type, involving the palate and sinuses reported in nations other then the United States. Study
Location
Number of cases reported
Gualco et al. [10] Ikeda et al. [11] Nikoloaos et al. [12] Reinartz et al. [13] Meng et al. [14] Chaudhary et al. [15] Ramanathan et al. [16] Kanitsap et al. [17]
Brazil Japan Greece The Netherlands China India Malaysia Thailand
97 5 1 2 1 1 42 1
82
AMER ICA N JOURNAL OF OT OLARYNGOLOGY –HE A D A N D N EC K ME D IC IN E AN D S U R G ER Y 3 6 ( 2 01 5 ) 8 0 –8 3
Table 2 – Cases of NKTCL, nasal type, in the United States. Study
Location
Number of cases presented Presenting symptoms
Healey [18] Wood et al. [19] Gaarl et al. [20]
New Orleans, LA Dallas, TX Los Angeles, CA
1 1 12
Prouix et al. [21] Li et al. [22]
Boston, MA Houston, TX
8 63
3.
Discussion
Extranodal NK/T-cell lymphoma, nasal type is rare in the United States, and usually arises in patients endemic to East Asia, South and Central America. In our review of the literature, there are many reports of NKTCL of the palate and sinuses in nations other than the United States. These cases are presented in Table 1. In the United States, where the incidence of NKTCL is much lower, fewer cases have been reported. These are presented in Table 2. All of these cases presented with sinonasal symptoms or facial swelling. In all of these case reports in the United States, none presented with persistent sore throat, as in the case described here. Patients usually present with nasal obstruction, bleeding, pain, or local swelling. It can produce ulcerative, destructive lesions within extranodal sites. While the nasal cavity is the typical site of origin, as outlined by our review of the literature, it can present with involvement of other midline structures within the upper aerodigestive tract, including the palate and nasopharynx. Often, it is associated with the Ebstein–Barr virus (EBV). Histologically, extranodal NKTCL, nasal type, is characterized by angiocentric and angiodestructive growth and by tumor cells that vary in size. In addition, an inflammatory cell infiltrate of plasma cells, histiocytes, and eosinophils is present [23]. Immunophenotypically, the tumor cells express CD2, CD3, and CD56. In some cases, the cells can lack CD56 and express CD8+ T cell antigens [23]. This case highlights the aggressive and rapidly progressive nature of this disease. The course of NKTCL, nasal type is aggressive with a 5-year overall survival ranging from 25 to 50% [24]. In addition, the neoplasms can be associated with hemophagocytic syndrome, which is clinically characterized by fever, hepatosplenomegaly, pancytopenia, and laboratory evidence of hemolysis. Given this, we highlight key clinical features that allow practitioners to make the correct diagnosis. While nasal obstruction, nasal discharge, facial swelling, and other nonspecific sinonasal symptoms have been recognized as presenting symptoms of NKTCL, nasal type, other less common signs and symptoms are often overlooked. Sore throat and dysphagia, for example, while known as presenting symptoms of NKTCL, nasal type, are often missed and treated as viral and bacterial pharyngitis, leading to delay of diagnosis and treatment that increases morbidity and mortality. In this case, the patient did not respond to initial courses of antibiotics and initial biopsy showed chronic sialadenitis with sialometaplasia. It was only heightened suspicions that
3-cm nasal mass Facial swelling, fever, unintentional weight loss Eleven patients presented with nasal masses, with some having ulceration, septal deviation or destruction, and/or involvement of contralateral nasal cavity or adjacent sinuses; one presented with isolated paranasal sinus lesion Nasal obstruction and localized soft-tissue swelling Nonspecific sinonasal symptoms, such as nasal obstruction, nasal discharge, or facial swelling
led to a repeat deep-tissue biopsy and diagnosis of NKTCL, nasal type, rather than conservative therapy and prolonged follow-up for necrotizing sialometaplasia, which would have led to worsening condition and rapid progression of the patient's NKTCL. The patient then began treatment of his diagnosed NKTCL weeks after his initial presentation. For cases of persistent sore throat and dysphagia, a diagnosis of NKTCL, nasal type should be considered, especially in individuals of Native American, Central, or South American descent. Suspicious lesions and clinical features should prompt practitioners to initiate work-up to rule out NKTCL, nasal type. In addition, when a diagnosis of necrotizing sialometaplasia is considered, NKTCL, nasal type should also be considered, as they can often present similarly. As in this case, deep-tissue biopsies to ensure accurate diagnosis are crucial.
4.
Conclusion
As evident by our review of the literature, NK/T-cell lymphomas, nasal type, are rare in the United States. Furthermore, cases that present with persistent sore throat have not been reported in the United States. Thus, there is a need for a high index of suspicion for early diagnosis of NK/T-Cell lymphomas.
REFERENCES
[1] Roschewski M, Wilson WH. EBV-associated lymphomas in adults. Best Pract Res Clin Haematol 2012;25(1):75–89. [2] Suzuki R, Yamaguchi M, Izutsu K, et al. Prospective measurement of Epstein–Barr virus–DNA in plasma and peripheral blood mononuclear cells of extranodal NK/T-cell lymphoma, nasal type. Blood 2011;118:6018–22. [3] Hahn JS, Lee ST, Min YH, et al. Therapeutic outcome of Epstein–Barr virus positive T/NK cell lymphoma in the upper aerodigestive tract. Yonsei Med J 2002;43(2):175–82. [4] Van Prooyen KS, Eloy P, Delos M, et al. Sinonasal lymphomas: case report. Acta Otorhinolaryngol Belg 2000;54(1):45–51. [5] Frierson HF, Mills SE, Innes DJ. Non-Hodgkin's lymphomas of the sinonasal region: histologic subtypes and their clinicopathologic features. Am J Clin Pathol 1984;81:721–7. [6] Al-Hakeem DA, Fedele S, Carlos R, et al. Extranodal NK/T-cell lymphoma, nasal type. Oral Oncol 2007;43:4–14. [7] Jaccard A, Hermine O. Extranodal natural killer/T-cell lymphoma: advances in the management. Curr Opin Oncol 2011;23:429–35.
AMER ICA N JOURNAL OF OT OLARYNGOLOGY –H EA D A N D N EC K ME D I CIN E AN D SUR G E RY 3 6 ( 2 01 5 ) 8 0 –8 3
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