Isolated relapse of a lymphoblastic leukemia T cell precursor in the epididymis

Abstract

A 41-year-old man who had otherwise asymptomatic right scrotal swelling presented to our urology clinic. He had been diagnosed with T cell precursor lymphoblastic leukemia/lymphoma 2 years previously. On examination, his right epididymis was enlarged. A regular, homogeneous, slightly hypoechoic solid mass was observed at the right caput epididymis. This testicular mass measured approximately 7×11×12 mm. Leukemia and lymphoma appear in a variety of locations throughout the body, but an isolated relapse involving the epididymis is rare. Epididymectomy was performed which naturally removed the patient’s right-sided sperm duct system. Especially in the younger age group epididymal masses can lead to fertility problems.

Introduction

Acute lymphoblastic leukemia (ALL) is one of the frequently observed malignancies, and it is encountered as β-cell (85%), and T-cell ALL (15%).^[1] T-cell acute lymphoblastic leukemia (T-ALL) emerges as a result of supranormal proliferation of immature T cells, and constitutes 10–15% of pediatric, and 25% of the adult cases with ALL. T-cell precursor lymphoblastic leukemia is an aggressive type of ALL. It carries high risk for treatment failure. It is treated with chemotherapy protocols used for ALL.^[2]

Case representation

A 41-year-old male patient was followed up in the hematology clinics. He had undergone bone marrow biopsy 2 years ago, and diagnosed as T-cell precursor lymphoblastic leukemia.

He received chemotherapy, and 12 sessions of radiotherapy, and presented to our clinic during his follow-ups because of painless scrotal swelling. Physical examination revealed left testis, and epididymidis with normal dimensions, and location. In the right epididymis, a painless swelling which can be separated from the testis with distinct contours was detected on palpation. On scrotal ultrasonograms dimensions of the right (18×27×49 ml;12.1 mL), and the left (34×30×48; 18 mL) testes were determined. Besides, a slightly hypoechoic, solid mass with dimensions of 7×11×12 mm and smooth contours was detected in the right epididymis. On Doppler ultrasonographic examination hypervascularity was found on the periphery of the lesion. Its appearance was consistent with leukemic infiltration of the right epididymis. Levels of beta-HCG, alpha-fetoprotein, and lactate dehidrogenase were within normal limits.

After informed consent of the patient was obtained, through inguinal incision the surgical field was explored. Excisional biopsy material sent to the histopathological examination during the operation was consistent with leukemic infiltration, and epididymectomy was performed (Figure 1). Excisional epididymidal pathology was reported as T-cell precursor lymphoblastic leukemia with strong CD8, weak CD5, and negative CD20, CD79a expressions (Figure 2a, b). The mass stained strongly with Ki-67 and terminal deoxynucleotidyl transferase (TdT). During the postoperative period of 12 months, any recurrence was not detected in the patient who received chemotherapy.

Figure 1.

Epididymectomy material

Figure 2a.

Epididymal leukemic infiltration (HE ×200)

Discussion

Extramedullary histopathological recurrence rates secondary to ALL in the genitourinary system have been reported as 5–8 percent. Effective application of chemotherapy has reduced testicular relapse down to 0–3 percent.^[3–5] Isolated relapse of ALL in the epididymidis is extremely rare. As reported in the literature, patients with isolated relapse of ALL in the epididymis present with painful scrotal emergency similar to epididymitis or testicular torsion or as is the case with our patient, painless swelling resembling a tumoral mass can be observed.^[5–7]

Mazzu et al.^[8] observed that hypervascularity was associated with every case of testicular leukemic or lymphoblastic infiltration Diffuse hypervascularity detected on ultrasonograms supports leukemic or lymphoblastic infiltration.

In similar conditions which may emerge in the adulthood, as a point to remember, surgical intervention may adversely affect fertility. In patients with epididymal painless mass lesions detected in cases with a history of leukemia, and lymphoma, the probability of relapse should be taken into consideration.

Figure 2b.

Leukemic cells stained with terminal deoxynucleotidyl transferase (TdT)