Nasal type of Natural Killer (NK)/T cell lymphoma manifests in the nasal cavity. Approximately 95% of them are associated with EBV(Ebstein Barr Virus) with a strong predilection for the Asian population. It has certain systemic and localized symptoms which aid in diagnosis of the condition. However, the histological criteria is pivotal in confirming the diagnosis as well as aiding in confirming the association of EBV. Nasal type of NK/T cell lymphoma has a guarded prognosis. Treatment plan include radiotherapy with concurrent chemotherapy. Despite all this, the 5-year survival rate ranges from 15-75%. A 35 years old male presented with an ulcerative nasal lesion. Diagnosed as nasal type of NK/T cell lymphoma via a tissue biopsy, it was managed by chemo-radiotherapy leading to complete resolution of symptoms and disease free on his follow-up 6 months later

Ikram, Mubasher

Junaid, Montasir

Kazi, Maliha

English

eCommons@AKU

eCommons@AKUSection of Otolaryngology, Head & Neck Surgery Department of SurgeryMarch 2014Nasal T cell lymphoma: a rare entityMaliha KaziMubasher IkramAga Khan University, mubasher.ikram@aku.eduMontasir JunaidFollow this and additional works at: https://ecommons.aku.edu/pakistan_fhs_mc_surg_otolaryngol_head_neckPart of the Otolaryngology Commons, and the Surgery CommonsRecommended CitationKazi, M., Ikram, M., Junaid, M. (2014). Nasal T cell lymphoma: a rare entity. Journal of the College of Physicians and Surgeons Pakistan,24 Suppl 1, S50-S1.Available at: https://ecommons.aku.edu/pakistan_fhs_mc_surg_otolaryngol_head_neck/77S50 Journal of the College of Physicians and Surgeons Pakistan 2014, Vol. 24 (Special Supplement 1): S50-S51INTRODUCTIONThe nasal type of NK/T (natural killer/T) cell lymphomais a rare but a slowly progressive destructive entity. Theterm nasal type of NK/T cell lymphoma was firstintroduced by Williams in 1949. With time, it came to beknown by multiple synonyms including Stewart'sgranuloma, polymorphic reticulosis and other moreobscure terms like progressive lethal granulomatousulceration, malignant granuloma, midline granuloma,non-healing granuloma, and midline malignant reticu-losis.1 Nasal type of NK/T cell lymphoma occurs mostcommonly in the fourth decade of life, with a femalepreponderance (2:1 - 8:1).Described below is the case of a middle aged malepresenting with nasal type of NK/T cell lymphoma andsubsequent management.CASE REPORTA 35 years old male, with no prior co-morbid conditions,presented with a necrotic defect over the left ala of noseand bridge. He had initially developed the lesion 3months prior that had been un-responsive to multipleantibiotic therapies. He subsequently underwent abiopsy for it, but since he was travelling abroad, he leftbefore receiving the final biopsy report and hencereceived no treatment for the same. As a result, thenecrotic area continued to increase in size and sooninvolved the dorsum, eroding the septum, with indurationaround the malar area and inferior orbital region on theleft side (Figure 1).Upon presentation to our department, he already had adisfigured nose, with almost complete necrosis of thelateral alar wall. A small part of the cartilaginous nasalseptum remained which was visible through the necroticarea and blackened. On examination of the oral cavity,the hard palate had blackened mucosa with a fistulouscommunication in the nasal cavity. Patient denied anyhistory of fever or myalgias.The patient was admitted, started on intravenousantibiotics and his baseline investigations includingcomplete blood picture, electrolytes, creatinine andblood sugars were carried out, which came out normal.Computed tomography (CT) scan of the para nasalsinuses was performed; it showed the disease to besuperficial, not involving the sinuses, and localized to themidface. There was no evidence of bony destruction.A biopsy under local anaesthesia was performed atbedside. It showed dense lymphocytic infiltration withangiocentric pattern of growth, characteristic of nasaltype of NK/T cell lymphoma. Further immunochemistryperformed was positive for CD56.The gentleman was then referred to radiation andmedicine oncology. He received chemo-radiationtherapy, with radiation of 50Gy (2 Grays/fraction) givenin 5 weeks and 3 cycles of chemotherapy (CHOPregimen:cyclophosphamide, hydroxy doxorubicin,vincristine, prednisolone ). It led to gradual but completeresolution of the disease (Figure 2).He remained well after completing his treatment and onregular follow ups-of 6 months he was disease free. Hehad a 3 cm x 3 cm sized defect along the left lateral wallof his nose. He was advised to get treated by a plasticsurgeon for the muco-cutaneous defect of his nose forwhich he opted to receive treatment at local hospital inhis city.CASE REPORTNasal T Cell Lymphoma: A Rare EntityMaliha Kazi1, Mubasher Ikram1 and Montasir Junaid2ABSTRACTNasal type of Natural Killer (NK)/T cell lymphoma manifests in the nasal cavity. Approximately 95% of them are associatedwith EBV(Ebstein Barr Virus) with a strong predilection for the Asian population. It has certain systemic and localizedsymptoms which aid in diagnosis of the condition. However, the histological criteria is pivotal in confirming the diagnosisas well as aiding in confirming the association of EBV. Nasal type of NK/T cell lymphoma has a guarded prognosis.Treatment plan include radiotherapy with concurrent chemotherapy. Despite all this, the 5-year survival rate ranges from15 - 75%. A 35 years old male presented with an ulcerative nasal lesion. Diagnosed as nasal type of NK/T cell lymphomavia a tissue biopsy, it was managed by chemo-radiotherapy leading to complete resolution of symptoms and disease freeon his follow-up 6 months later.Key Words: Nasal type of NK/T cell lymphoma.   Nasal ulcer.   Midface destruction.   Radiation therapy.   Chemotherapy.   CHOP regimen.1 Department of Otolaryngology, Head and Neck Surgery,The Aga Khan University Hospital, Karachi.2 Department of ENT- Head and Neck Surgery, Jinnah Medicaland Dental College, Karachi.Correspondence: Dr. Maliha Kazi, B-15, Pak Tameer Plaza,Block-14, Gulshan-e-Iqbal, Karachi.E-mail: maliha_kazi@hotmail.comReceived: January 16, 2012;   Accepted: March 18, 2013.Nasal T cell lymphomaDISCUSSIONNasal type NK / T cell lymphoma was first recognizedmore than a century ago, in 1897 by McBride. Hereported a case of rapid destruction of face and noseand facial swelling.2 The condition gained popularity in1922, when Stewart described a case series of 10patients with similar mid-face destructive lesions.1Rarely reported in all races, it is more common inMongoloids, Indonesians and Latin Americans.3 Despitethe fact that the etiology is unknown, approximately 95%of the cases are associated with Epstein Barr Virus(EBV).4Diagnosis of nasal type of NK/T cell lymphoma is basedon history, clinical examination along with pathologicalfindings. Symptoms may be general or with head andneck manifestations. Constitutional symptoms like fever,malaise, chills, rhinorrhea and weight loss usuallyprecede the midface destruction, none of which wereapparent in this patient. Other symptoms includediplopia, proptosis and cranial nerve paralysis due toinvolvement of local structures. Histopathology showsinvasion and destruction of blood vessels along withsuperimposed bacterial infection. The histologicalcriteria considered in confirming the diagnosis includes:angiocentric infiltration with or without necrosis, extranodal infiltration into the surrounding tissues, immuno-phenotype of CD56 or CD3 as well as association withEBV.5 T/NK cell lymphoma with an association to EBV isresistant to conventional chemotherapy and has animmensely aggressive clinical course.6 EBV test wasperformed in our patient after his initial diagnosis, whichturned out to be negative.Studies have suggested imaging as non-specific andonly beneficial to see the extent of destruction.6 It has aseries of prodromal symptoms of persistent rhinorrhea,stuffiness and symptoms of sinusitis. In its active phasethe nasal discharge becomes purulent with progressivedestruction of nasal framework and mid-face. It all endswith gross mutilation as well as systemic metastasis.On radiological images, nasal T / NK cell lymphoma hasto be distinguished from a number of different entitiescausing sinonasal destruction such as Wegener'sgranulomatosis, other non-Hodgkin's lymphoma, granu-lomatous infections, adenoid cystic carcinoma, olfactoryneuroblastoma, malignant melanoma, and squamouscell carcinoma.4 Although, it leads to bony erosion of thepalate, alveolar bones and orbit, which are easily pickedup on computed tomographic scans, the fact remainsthat these findings also correlate to a malignant neo-plasm. Magnetic resonance imaging is helpful in evalu-ation of the disease extent but again it is non-specific.7Untreated, nasal type of NK/T cell lymphoma is fatal.Maeda et al. have reported that one year survival rate of50% and 5 years survival rate of 23% for an untreatedcondition.8 Chemotherapy (CHOP regimen) withradiation therapy is the proposed treatment option.Despite radiation and chemotherapy the overall survivalrate remains low.9Discrepancies persist in the rates of local control ratesusing radiation therapy as the modality in differentseries. The favoured dose of radiation is at least 50 Gy.Studies have shown the 5-year overall survival afterradiation therapy to be around 15 - 75%.10Nasal type of NK/T cell lymphoma is a slow growingcondition, following a waxing and waning course. Itfollows a downhill course in case of relapse or systemicspread. Good cure and overall survival rates have beenobserved, once diagnosed early and treated promptly.REFERENCES1. Mehta V, Bhat S. Nasal NK/T cell lymphoma presenting as alethal midline granuloma. Indian J Dermatol Venereol Leprol2008; 74:145-7.2. Aozasa K, Zaki MA. Epidemiology and pathogenesis of nasalNK/T-Cell lymphoma: a mini-review. Sci World J 2011; 11:422-8.3. Aozasa K, Takakuwa T. Nasal NK/T-cell lymphoma:epidemiology and pathogenesis. Int J Hematol 2008; 87:110-7.4. Semenzato G, Marino F, Zambello R. State of the art in naturalkiller cell malignancies. Int J Lab Hemat 2012; 34:117-28.5. Razif KS. Nasal NK/T cell lymphoma mimicking an iatrogeniclateral nasal wall infection: a diagnostic dilemma. Med JMalaysia 2011; 66:160-1.6. Lohrmann C, Uhl M, Warnatz K, Kotter E, Ghanem N, LangerM. Sinonasal computed tomography in patients with Wegener'sGranulomatosis. J Comput Assist Tomogr 2006; 30:122-5. 7. Oul CH, Chen CC, Ling JC. Nasal NK/T-cell lymphoma:computed tomography and magnetic resonance imagingfindings. J Chin Med Assoc 2007; 70:207-12.8. Maeda H, Aozasa K, Tsujimura T, Sasaki R, Yoshida J,Matsunaga T. Malignant lymphomas and related conditionsinvolving nasal cavity and paranasal sinuses; a clinico-pathologic study of forty two cases with emphasis onprognostic actors. Eur J Surg Oncol 1988; 14:9-15.9. Advani R, Kohrt H. Extranodal natural killer/T-cell lymphoma:current concepts in biology and treatment. Leuk Lymphoma2009; 50:1773-84.10. Sakata K, Hareyama M, Ohuchi A, Sido M, Nagakura H, MoritaK, et al. Treatment of lethal midline granuloma type nasal T-celllymphoma. Acta Oncol 1997; 36:307-11.Figure 1: Figure showing necroticedges of the lesion with loss of theseptum.Figure 2: Small defect caused dueto the destructive effect of thelesion, requiring a local flap.Journal of the College of Physicians and Surgeons Pakistan 2014, Vol. 24 (Special Supplement 1): S50-S51 S51

Nasal T cell lymphoma: a rare entity

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Nasal T cell lymphoma: a rare entity

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