Lethal midline granuloma
A 35 year old male presented with swelling in the right side of neck since two months. This was followed by pain and swelling on the dorsum of nose since one and a half months. The pain radiated to right half of the face and head. Patient also gave a two week history of stuffiness in the right nostril and bilateral nasal mucopurulent discharge. There was also a three day history of fever.\nOn examination, there was a 5 × 4 cm swelling on the right side of dorsum of nose and nasolabial fold. The area was inflamed, erythematous and tender []. A crust covered mass was seen filling the right sided nasal cavity. There was no history of epistaxis, anosmia, post nasal drip, oral bleed or cough. Right sided cervical and submandibular lymphadenopathy was observed.\nAll routine hematological and biochemical investigations were normal. HIV, HBsAg and VDRL were negative. A contrast enhanced computed tomogram (CECT) of the paransal sinuses showed right sided superficial medial canthus related right sided nasolabial disease with metastatic right sided cervical and submandibular lymphadenopathy. Nasal endoscopic smears were positive for Pseudomonas aeruginosa. FNAC of right cervical lymph nodes showed atypical cells against a necrotic background. A biopsy was taken from the mass and sent to National Institute of Pathology for histopathological examination. A diffuse lymphomatous infiltrate having an angiocentric and angiodestructive pattern was seen. The infiltrate was seen destroying the hair follicles []. Extensive areas of necrosis were also seen []. Plasma cells, histiocytes and eosinophils were present admixed with atypical lymphocytes []. Immunohistochemistry was performed and the lymphomatous infiltrate was found to be positive for LCA [], CD56 [] and CD3 []. Infiltrate was negative for CD20. No neutrophilic microabscesses or granuloma was seen. However fungal stains, Periodic Acid Schiff (PAS) were done to exclude fungal disease. Based on these findings a diagnosis of extranodal NK cell lymphoma was made. The patient was referred to the oncology department for further management and he received four cycles of multidrug chemotherapy (CHOP regimen). This was followed by external beam irradiation (36Gy). At six months follow-up the mass had slightly reduced in size, there was relief from episodes of epistaxis and nasal discharge had stopped.