Published case study – primary NK/T-Cell Lymphoma with metastasis to the penis carries rapid progression and poor outcomes

In BMC Urology on 6^th September 2017, Xiaotian Wang from Shengjing Hospital of China Medical University, Shenyang, Liaoning, China, et al. published a case report of a male, 58-year-old patient with diffuse swelling, as well as sclerosis and ischemic changes in the glans and anterior segment, of the penis.

Key Highlights

Clinical History

• No bloody or purulent discharge; no fever, night sweats, or weight loss
• Vital signs were within normal limits
• No lymphadenopathy palpated in the neck or supraclavicular region
• Visual examination of genitals manifested a diffused enlargement of the penis with local ulcer and necrotic tissue in the glans
• WBC count = 8,100/mm3; Hemoglobin = 12.5g/dL; platelet count = 276,000/mm³; liver function, kidney function, and serum LDH levels were within normal limits
• EBV IgG was positive in the serum, EBV DNA determination was negative in the blood, and C-reactive protein = 22.9mg/mL
• Pelvic enhanced MRI identified space-occupying lesions in the forward part of the penis, urethral orifice stenosis, and cavernosal swelling
• Bone marrow biopsy showed lymphoma cell infiltration ≤2%

Histological Features

• Microscopic findings identified infiltration of medium-sized cells with irregular nuclei, inconspicuous nucleoli, and many apoptotic bodies
• Lymphoma cells exhibited notable angioinfiltrative growth pattern with homocentric arrangement around small arteries, and coagulative necrosis was also observed

Immunohistochemistry

• Positive for: CD3, CD56, vimentin, LCA, TIA-1, and granzyme B
• Negative for: cytokeratin, CD20, BCL2, BCL6, and ALK
• In situ hybridization found EBV encoded RNA in most atypical cells

Detailed History

• Patient had nasal obstruction for >10 years
• PET-CT found high-FDG metabolic mass in the nasal cavity and the nasopharyngeal cavity; biopsied with sinoscopy
• Diffuse infiltration of small to medium-sized lymphoid cells with angio-destructive growth pattern and apoptotic bodies
• Positive for: CD3, CD56, EBV encoded RNA
• Negative for: CD20 and granzyme B
• Diagnosis of penile metastasis from Extranodal Nasal-Type NK/T-Cell Lymphoma was made

Treatment and Outcomes

• Patient consented to chemotherapy and subsequent radio-therapy and ASCT 20 days after neoplasm resection
• Cyclophosphamide, doxorubicin, vincristine, prednisone, and L-asparaginase (CHOP-L) planned to be administered every 3 weeks, with six cycles in total
• Third chemotherapy cycle delayed due to ascites, fever, and grade 4 myelosuppression
• Subsequently developed tachypnea, abdominal pain, fever, and diarrhea
• On CT, Lymphoma was refractory to chemotherapy; metastases identified in adrenal gland, liver, spleen, and lymph nodes
• Patient died of disease 1 month later, approximately 4 months after diagnosis

The authors stated that the case they present of a primary NK/T-Cell Lymphoma with metastasis to the penis is extremely rare but holds significant clinical value. They noted that this case widens the spectrum of differential diagnosis of penile tumors and emphasizes the value of detailed history and comprehensive work-up. The group concluded that this malignant disease carries rapid progression and poor outcomes, and so healthcare professionals need to cooperate and strive to design and test more effective therapeutic strategies.

Abstract

BACKGROUND: Extranodal 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 PRESENTATION: One 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.

CONCLUSION: This 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.