Dr Kiran Jagtiani1, Dr Kanchan Dholam2, Dr Gurkaran Preet Singh3, Dr Sandeep Gurav4, Dr Nimisha Manjrekar
2Ex-Professor and HOD,
Department of Dental and Prosthetic Surgery
Tata Memorial Hospital, Parel, Mumbai, India.
Head and neck region is the second most common anatomical site for Non-Hodgkin’s lymphomas. Among them extranodal NK/ T-cell lymphoma, nasal and aggressive type are relatively newly recognised distinct clinico-pathological entities in the WHO classification. The nasal type is predominantly located close to the nose and upper aero-digestive tract, often showing midline anatomical predilection, and palatal perforation. Intra-orally it presents as swelling on the hard palate that precedes formation of a deep necrotic ulcer which may enlarge and lead to formation of oronasal fistula. This predisposes the patient to hyper-nasal speech, leakage of fluid, tooth mobility, difficulty in eating. The present article reports a rare case of nasal type NK/T-cell lymphoma and its multidisciplinary management, role of prosthetic rehabilitation as well as importance of evaluation of patient satisfaction with the obturator prosthesis using the obturator functioning scale. The objective is to highlight that timely management of this disease and its oral implication with a well-functioning obturator prosthesis can positively contribute to overall improved quality of life of the patient.
Keywords: NK/T cell lymphoma, Obturator functional scale, Palatal defect, Quality of Life.
Citations: Jagtiani K, Dholam K, Singh G, Gurav S. Nasal NK/T cell lymphoma- A Literature Review and Case Report of Prosthetic Management. J Prosthodont Dent Mater 2021;2(2)11-16.
Palatal lesions presenting in the form of an unremitting, slowly progressive ulcer causing erosion of nasal, paranasal sinuses and destruction of soft tissue always raise concerns over the diverse differential diagnosis. These can result from a myriad of neoplastic, autoimmune, infectious and traumatic aetiologies. The first description of extensive lesions of the nose and oral cavity dates back to 1897 by McBride under the title “case of rapid destruction of the Nose and Face”. Ten cases of midfacial destructive lesions were reported by Stewart in 1922 which became to be known as Stewart syndrome or Stewart granuloma. Later, the term “lethal midline granuloma” was suggested by Williams in 1949 to describe these an destructive lesions of the oral and nasal cavity with varying involvement of other local and distant sites. Histopathologically, angiocentric growth pattern with zonal necrosis is seen thus the term “angiocentric T-cell lymphoma” was proposed in the REAL Classification (Revised European American Classification of Lymphoid Neoplasm).6 Further immunophenotyping has allowed better characterization of these cases as rare, aggressive form of mature T-cell and Natural Killer Lymphoma as classified by 2016 WHO classification.
In general, lymphomas may arise in lymph nodes or any organ, either by spread from lymphatic sites or as a manifestation of primary extra nodal disease. They account for 3-5% of all malignant tumors; and are classified as: Hodgkin’s and Non-Hodgkin’s Lymphoma (NHL). The NHL variant often presents outside the lymph nodes at sites such as the gastrointestinal system, head and neck region. Extranodal NK/ T-cell lymphoma (ENKTL) has two pathological variants: Nasal type and aggressive NK-cell lymphoma. The nasal type is predominantly located close to the nose and upper aero-digestive tract, often showing midline anatomical predilection and palatal perforation. It has a higher propensity for occurrence in South-Asia and males (M-F, 2.5:1) in the age group of 19 to 80 years. Initial signs and symptoms include headache, nasal obstruction, foul discharge that masquerade sinusitis. As the disease progresses fever, night sweats, weight loss, lymphadenopathy may occur. Intraorally it presents as swelling on the hard palate that precedes formation of a deep necrotic ulcer which may enlarge and lead to formation of oronasal fistula.12,13 This predisposes the patient to hypernasal speech, leakage of solids / fluids and tooth mobility.
The cornerstone for definitive diagnosis of NK/T cell lymphoma is the identification of atypical lymphoid cells and expression of surface CD3-, cytoplasmic CD3epsilon+, CD56 and cytotoxic granule-associated protein which are phenotypic markers of immune cells.14 Because of extensive necrosis and reactive inflammatory changes, superficial biopsies are often inconclusive.15 ENKTL is strongly associated with EBV positivity. Also, high lactate dehydrogenase level may indicate some form of tissue damage. If left untreated, the disease may progress and lead to systemic dissemination. This underlines the importance of early diagnosis, timely referral and appropriate management with a multi-disciplinary approach.16-19 Owing to the aggressive nature and guarded prognosis of the disease, surgical reconstructive procedures for closing the intra-oral defect are pretermitted and prosthodontic management is sought to enhance the quality of life. Prosthodontic rehabilitation with an obturator prosthesis and its impact on the quality of life as well as psychosocial adaption of patients can be subjectively evaluated by using obturator functioning scale (OFS).20 This article presents a rare case of nasal type NK/T-cell lymphoma and its multidisciplinary management, role of prosthetic rehabilitation as well as importance of evaluation of patient satisfaction with the obturator prosthesis using the obturator functioning scale.
A 42-year-old male presented at a tertiary care centre with left sided headache, facial pain, bilateral swelling in the neck, ulceration on the palate causing nasal regurgitation. Patient had undergone septoplasty 2 months back and developed palatal ulceration 7 days post-operatively. Other systemic symptoms included fever, night sweats and history of weight loss of about 5kg in 2 months. The patient underwent routine haematological testing which revealed low haemoglobin levels, left shift in white blood cells count, high platelet count.
A PET CECT scan (18-F FDG) revealed hypermetabolic bilateral soft tissue mass in the nasal cavity and soft palate with erosion of nasal septum, hard palate that extended laterally into the left maxillary sinus with erosion of medial wall. Posteriorly the mass extended into the left retropharyngeal and left para-pharyngeal space. Discrete nodes were noted bilaterally at level IB, level II, level III, level IV and left level V, largest in right IB, measuring 20 x 21mm. Rest of the scan appeared unremarkable and showed physiological tracer uptake. On bacterial and fungal culture, no pathogen growth was observed after 5 days of incubation. Immunophenotypic tests demonstrated the presence of T-cellassociated markers CD2, CD7, CD45RO, and CD56. The diagnosis of high-grade NHL of peripheral Tcell immunophenotype and in view of clinical findings, a possibility of nasal type NK/T-cell lymphoma was established. The patient was started on multidrug chemotherapy under the S.M.I.L.E protocol by the oncologists followed by radiation therapy; 50-54 Gy respecting the organ at risk (OAR) doses after 4 weeks. On completion of which, near complete regression of the cervical node with low grade metabolic activity was noted. The patient was then referred to the dental department with the request to address nasal regurgitation of liquids.
Oral examination revealed exposed necrotic bone with white slough on the midline of hard palate and a separate defect involving some part of hard and soft palate. Both defects were separated by a thin band of palatal mucosa. The defect measured approximately 5 x 5 cm, not involving the dental alveolus or teeth but confined to the palate only. An obturator prosthesis was fabricated in a conventional manner to close the oronasal fistula which also served as a carrier for topical antifungal medication (liposomal topical ketoconazole gel). On follow up relining was done once within the 1st week of use to address the changes in the palatal mucosa. Subjective assessment of patient satisfaction with respect to functions such as speech, swallowing, the comfort of the prosthesis was done using the obturator functioning scale, twice i.e., 1 week and 1 year after obturator insertion. (Figure 1, 2 ,3)
Head and neck region is the second most common anatomical site for non-hodgkin’s lymphoma. Among them extranodal NK/ T-cell lymphoma, nasal and aggressive type are relatively newly recognised distinct clinico-pathological entity in the WHO classification. The clinical picture is often misleading due to its resemblance to commonly occurring pathologies such as; fungal infections, tertiary syphilis, rhinoscleroma, wegener’s granulomatosis, necrotising sialometaplasia, cocaine abuse. This often contributes to delayed diagnosis, advancement in the stage, secondary infections, and guarded prognosis which contraindicate surgical intervention. Currently, the treatment modalities for stage I/II NK/T-cell lymphoma (Ann-Arbor staging) include radiotherapy (RT), chemotherapy (CT), or their combination. Whereas, for the stage III/IV ENKTL, CT remains the primary treatment option. Special regimes such as SMILE protocol (Dexamethasone, Methotrexate, Ifosfamide, L-asparaginase, Etoposide) and DeVic protocol (Dexamethasone, Etoposide, Ifosfamide, Carboplatin) have been designed for the tumor control, concurrent with radiotherapy.
There is also a trend to use “sandwich” protocols, with earlier RT after an initial two to three cycles of chemotherapy (with or without early PET reassessment), followed by further “consolidation” cycles of chemotherapy. 19 Overall, patients with ENKTCL have a cumulative probability of survival at 5 years ranging from 37.9% to 45.3%.11 There is a vast amount of literature available on ENKTL; epidemiology, aetiological factors, pathogenesis, diagnosis and management strategies (CT, RT, both) but not much has been documented on oral implications and their prosthodontic management.
Orofacial rehabilitation using obturator protheses is an appropriate treatment modality in cases with acquired palatal defects susceptible to recurrence.16 The benefit of using an obturator prosthesis over autogenous tissue reconstruction is that it simplifies oncological surveillance, maintains the integrity of oral and nasal compartments, restores deglutition and speech, thereby reducing functional impairment and psychological trauma.25 To measure the obturator prosthesis functioning and patient’s adjustment, in terms of psychological, family, sexual and social acceptance subjectively, the obturator functioning scale (OFS) was utilized. This scale was developed by Kornblith et al18 in 1996 at Memorial Sloan Kettering Cancer Center. It consists of 15 questions where all items are rated on a 5-point Likert scale ranging from 1-5, where rating 1 – not at all, 2- a little difficult, 3- somewhat difficult, 4- very much difficult, 5- extremely difficult. Total score ranges from 15-75 i.e., 15 (absolutely no problems with the obturator) to 75 (very severe problems with the obturator). Higher score reflects greater difficulty in obturator functioning. In this case report, patient’s satisfaction with the existing obturator prosthesis was evaluated using the Obturator Functioning scale (OFS).
Upon 1 week follow-up, the patient’s OFS score was 34 on a total scale of 75. As the patient had an intra-oral defect, aesthetic concerns were minimal to none. Since, the obturator covered most of the hard palate, hypernasality of speech was absent, eating and drinking had improved significantly but patient reported slight change in voice, difficulty in talking in public and avoidance of social events due to uneased pronunciation of words. Due to the imposed restrictions related to ongoing Covid-19 pandemic, telephonic consultation was done for subsequent follow-up. The patient’s OFS score after 1-year of using the obturator prosthesis was 26 which indicated better adjustment in the following item scores: change in voice, avoidance of family and social events, understandability of speech, and ndifficulty pronouncing words. This report signifies that timely management of this disease and a wellfunctioning obturator prosthesis can positively contribute to overall improved quality of life of the patient.
Prompt diagnosis, timely referrals, multidisciplinary coordination and comprehensive management are important considerations in the overall prognosis of Nasal type NK/T-cell lymphoma. This article attempts to highlight role of dental specialist in appropriate oral rehabilitation. Prosthetic rehabilitation of oro-nasal fistula with obturator prosthesis is an acceptable treatment option to restore functional and esthetic requirements of the maxillary defect. For subjective assessment, the obturator functioning scale serves as a valuable tool in assessment of patient satisfaction.
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