Annular Erythematous Patches as the Presenting Sign of Extranodal Natural Killer/T-Cell Lymphoma

Baykal, Can; Ekinci, Algün Polat; Sarı, Serkan; Topkarcı, Zeynep; Demir, Özgür; Büyükbabanı, Nesimi

doi:10.4274/tjh.2016.0071

Cited by 4 publications

(2 citation statements)

References 9 publications

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“…Bone marrow involvement occurs in <10% of patients. Distant metastasis of NK/T-cell lymphoma is sporadically reported, with the most commonly involved site including skin, testis, gastrointestinal tract, eyes, lungs, adrenal glands, breast, and brain [ 10 , 11 ]. ENKTL with penis involvement is very rare, with only one case reported.…”

Section: Discussionmentioning

confidence: 99%

Extranodal nasal-type natural killer/T-cell lymphoma with penile involvement: a case report and review of the literature

Wang¹,

Gong²,

Li³

et al. 2017

BMC Urol

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BackgroundExtranodal natural killer/T-cell lymphoma (ENKTL) usually presents as a localized disease in the nasal cavity; extension to the male genitourinary system is very rare and has been characterized only recently. Most cases present with predominantly extranodal involvement, advanced stage disease, highly aggressive course, and strong association with Epstein-Barr virus (EBV). While metastasis is common in ENKTLs, the penis is rarely involved in both nasal and non-nasal ENKTLs and only one report was published to date.Case presentationOne patient with NK/T-cell lymphoma, presented initially with a penile mass, is reported. The 58-year-old man who presented with progressive painless penile swelling underwent penectomy for penile tumor. Histologically, the glans and foreskin revealed neoplastic infiltration of medium-sized lymphoma cells expressing CD56, CD3, granzyme-B, and labeled for EBV-encoded RNA in situ hybridization. Findings were consistent with NK/T-cell lymphoma. By detailed history, we learned that the patient had nasal obstruction for more than 10 years. Nasopharyngeal involvement was screened with PET-CT; ENKTL was diagnosed after a nasopharyngeal biopsy. The final diagnosis was primary nasal NK/T-cell lymphoma, with metastasis to the penis. Additional sites of disease appeared soon afterward (adrenal gland, liver, spleen and lymph nodes). The patient died within 4 months.ConclusionThis study suggested that penile NK/T-cell lymphoma tends to disseminate early and pursues an aggressive course. It is imperative to distinguish nasal NK/T lymphoma from other types of tumors, because the prognosis and treatment differ significantly for secondary metastases.

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Section: Discussionmentioning

confidence: 99%

Extranodal nasal-type natural killer/T-cell lymphoma with penile involvement: a case report and review of the literature

Wang¹,

Gong²,

Li³

et al. 2017

BMC Urol

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“…In this case, the patient presented with diffuse erythema on the face and ulcers on the right leg, rather than as masses or nodules on the nose and in the nasal cavity. Baykal et al 11 had reported 2 cases of ENKTL with erythematous patches on the trunk as presenting manifestation. In the 2 cases, the main clinical manifestations were edematous erythema and scales on the trunk.…”

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Erythematous Patch and Ulcerations on the Right Leg: Challenge

Zhang

Zheng

2023

The American Journal of Dermatopathology

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ANSWERExtranodal NK/T-cell lymphoma. DISCUSSIONExtranodal NK/T-cell lymphoma (ENKTL) is an extremely rare non-Hodgkin lymphoma with a strong association with the Epstein-Barr virus and an unfavorable prognosis. 1 The incidence of ENKTL is the highest in East Asia and Latin America. 2 This lymphoma mainly occurs in those aged 40-50 years without gender predilection. 3 In recent years, research on the pathogenesis of this lymphoma have found that the onset of ENKTL is related to the Epstein-Barr virus proteins and their genetic variants. 4,5 In 2020, research on 700 Asian patients with ENKTL found that the increased risk of ENKTL is associated with variants at HLA-DPB1. 6 ENKTL can affect the nasal cavity, skin, stomach, intestines, upper respiratory tract, and other extranodal sites. 7 Due to the presentation of this disease at several extranodal sites, the qualifier "nasal type" has been dropped from its initial name in the latest World Health Organization classification. 8 Primary cutaneous ENKTL represents approximately less than 1% of cutaneous lymphomas. 9 The lesions of primary cutaneous ENKTL are often found as masses or nodules with ulcers in the nasal cavity. The tumor gradually affects the surrounding tissues and eventually damages the facial structure. In addition, fever, fatigue, and weight loss are common systemic symptoms. 10

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Annular skin tumors and lymphoproliferative diseases

Baykal

Ekinci²

2023

Clinics in Dermatology

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Annular Erythematous Patches as the Presenting Sign of Extranodal Natural Killer/T-Cell Lymphoma

Cited by 4 publications

References 9 publications

Extranodal nasal-type natural killer/T-cell lymphoma with penile involvement: a case report and review of the literature

Extranodal nasal-type natural killer/T-cell lymphoma with penile involvement: a case report and review of the literature

Erythematous Patch and Ulcerations on the Right Leg: Challenge

Annular skin tumors and lymphoproliferative diseases

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