Multifocal primary cutaneous extranodal NK/T lymphoma nasal type

Abstract

Nasal type extranodal NK/T-cell lymphoma is a distinct entity according to the World Health Organization classification. Although 60% to 90% of patients with this disease present with a destructive mass in the midline facial tissues, it may also primarily or secondarily involve extranasal sites, like the skin. We report the case of a 77-year-old patient that came to our department with erythematous plaques of the right leg and eczematous lesions of the trunk. These lesions were biopsied and the patient was diagnosed with extranodal NK/T-cell lymphoma, nasal type. He was treated with multi-agent systemic chemotherapy but died 5 months after diagnosis. This case highlights the rarity and variability of cutaneous features of this disease and its aggressive course and poor prognosis.

Primary Cutaneous Extranodal NK/T-Cell Lymphoma Mimicking a Large B-Cell Lymphoma (Leg Type)

“…This unusual clinically presentation may lead to misdiagnosis, including large B cell lymphoma of leg type and other cutaneous malignancies (like squamous cell carcinoma). This presentation of ENTCL resembling a primary cutaneous large B-cell lymphoma of leg type is extremely rare [1,[4][5][6]10].…”

“…The most frequently observed cutaneous lesions are usually subcutaneous nodules, erythematous papules, infiltrative plaques. They are often ulcerated, commonly located on the trunk or extremities [5][6][7][8]. They also can mimic cellulitis, panniculitis, fasciitis and facial swelling [1,9].…”

Nasal-type extranodal T-cell/NK lymphoma in association with hemophagocytic syndrome

“… 2 , 5 It typically affects the nasal and paranasal regions, but it can also affect the lungs, gastrointestinal tract, testicles, kidneys, pancreas, central nervous system and skin, 2 - 6 the latter being the second most commonly involved area after the nasal cavity. 2 , 3 Cutaneous lesions can also be similar to those seen in mycosis fungoides. 3 Enlarged lymph nodes can be seen, particularly in patients with nasal involvement, and they can be inflammatory or related to the tumor.…”

“… 2 , 3 Cutaneous lesions can also be similar to those seen in mycosis fungoides. 3 Enlarged lymph nodes can be seen, particularly in patients with nasal involvement, and they can be inflammatory or related to the tumor. 1 …”

“…The association of the pathogenesis of the tumor with Epstein-Barr virus (EBV) infection is well established in the literature. 1 - 3 On the histopathology, extranodal NK/T-cell lymphoma presents with a mixed cellular infiltrate, with atypical lymphocytes, plasma cells, eosinophils and histiocytes, besides the common angiocentric and angiodestruction features. 1 , 2 , 4 , 5 Immunohistochemistry is positive for CD45Ro, CD3, CD7 and CD56, besides Eber and cytotoxicity markers such as TIA-1 and granzyme B.…”