Stesura Seveso Archivio Italiano di Urologia e Andrologia 2014; 86, 4400 CASE REPORT Primary B-cell lymphoblastic lymphoma of the testis Flavia Tombolini 1, Vito Lacetera 1, Guido Gini 2, Debora Capelli 2, Pietro Leoni 2, Rodolfo Montironi 3, Andrea Benedetto Galosi 4, Giovanni Muzzonigro 1 1 Institute of Urology, 2 Institute of Hematology, 3 Institute of Pathological Anatomy and Histopathology, AOU United Hospitals, Polytechnic University of Marche Region, Ancona, Italy; 4 Division of Urology, A. Murri Hospital, Fermo, Italy. . We present a rare case of primary lym- phoblastic B-cell lymphoma of the testis focusing on ultrasonographic and pathological features and clinical implications. Pathological examination revealed primary testicular lymphoblastic B-cell lym- phoma which was treated with adjuvant chemotherapy, including rachicentesis with administration of chemother- apy and with radiotherapy of contralateral testis. Primary testicular lymphoblastic B cell lymphoma is an aggressive disease and it is necessary a multimodal therapy (surgery, chemotherapy and radiotherapy) to prevent metastasis. KEY WORDS: B-cell lymphoblastic lymphoma; Primary testicular lymphoma; Testicular ultrasound. Submitted 3 October 2014; Accepted 31 October 2014 Summary No conflict of interest declared. no history of traumatic events in the inguinal and scrotal region. He underwent surgical correction of left varicocele 12 years before with a complete resolution of the problem. Scrotal ultrasound showed an hypoechoic area with homogeneous aspects in the inferior part of the left testi- cle, size 2.7 x 1.3 cm. Scrotal eco-power-Doppler showed high blood perfusion (Figure 1) and increased vascular- ization of the left epididymis if compared to the right one. Tumoral markers were normal. Patient underwent left orchifunicolectomy and implantation of testicular pros- thesis. Intraoperative pathological evaluation described an extended cellular neoplastic population with mitotic aspects, high nucleocytoplasmic ratio and irregular nucle- us. Definitive pathological report described a creamy white nodular proliferation of 2,8 x 2,5 cm in lower pole of testis; microscopic examination was positive for lym- phoblastic B-cells with intermediate size and irregular nucleus (Figure 2). Immunohistochemical expression pat- tern was: CD45 positive, CD79a-CD10-CD38 positive, Bcl1, Bcl6, CD57, MUM 1 CD99 and CD117 negative; focal areas were CD20, Bcl 2, TdT and CD3 positive (Figu - re 3). The proliferation index, as detected by MIB1 anti- Presented at 19th National Congress SIEUN, Fermo 2014 INTRODUCTION Primary testicular lymphoblastic B-cell lymphoma is a rare tumor, it represents 7% of all testicular cancer (< 2% in men under 50 years of age) and it accounts for 1% of all non-Hodgkin’s lymphomas. It is the most common bilateral testicular tumor, with 10-18% cases of synchro- nous or asynchronous involvement of contralateral testis (1-4). It has no pathognomonic clinical and ultrasono- graphic features and a diagnosis before surgery is uncommon. It needs specific adjuvant chemotherapy and radiotherapy because of the high risk of metastatic process at skin, central nervous system, contralateral testis and other organs. A multidisciplinary care is the key of treatment. CASE REPORT A 39 years old man was referred to our clinic with acute pain at the left testis; a swelling hard mass in the inferior part of the left testicle was palpable; the right testis was normal without any associated symptoms of lower urinary tract infection. After a first clinical evaluation an infective orchiepididymitis was suspected and antibiotics were administered for 2 weeks. He had no comorbidities and Figure 1. High blood perfusion at colour-Doppler examination in the inferior part of testis. DOI: 10.4081/aiua.2014.4.400 Tombolini_Stesura Seveso 16/01/15 12:13 Pagina 400 401Archivio Italiano di Urologia e Andrologia 2014; 86, 4 Primary testicular B-cell lymphoblastic lymphoma body, was 70%. This lesion was classified as a primary tes- ticular lymphoblastic B-cell lymphoma with focal expres- sion of CD3, confined to the testicle. Patient underwent total-body computed tomography (CT), which resulted neg- ative for metastasis. A multidisciplinary discussion with urologists, oncologists, radiation oncologists and hematol- ogists was done. After a negative bone marrow biopsy the patient underwent to 6 cycles of hyper-CVAD chemother- apy (cyclophosphamide, vincristine, doxorubicin, and dexamethasone given as course A, followed by methotrex- ate and cytarabine given as course B), rachicentesis with administration of methotrexate 12 mg and cytarabin 100 mg (2 for each cycle), and radiotherapy of contralateral testis. We proposed also cryoconservation of sperm before starting adjuvant treatment. After 6 months of follow-up the patient is alive and without disease. Discussion is posted in Supplementary Materials in www.aiua.it CONCLUSIONS Primary testicular lymphoma is not associated to pathog- nomonic findings at ultrasound and is not different from other testis neoplasms. Primary testicular lymphoblastic B lymphoma is an aggressive disease and it is necessary a multimodality therapy (surgery, chemotherapy and radiotherapy) to prevent metastasis. REFERENCES 1. Emura A, Kudo S, et al. Testicular malignant lymphoma: imag- ing and diagnosis. Radiat Med. 1996; 14:121-126. 2. Mazzu D, Jeffrey RB, et al. Lymphoma and leukemia involving the testicles: findings on grey scale and colour-Doppler sonography. Am J Roentgenol. 1995; 164:645-647. 3. Ahmad SS, Idris SF, et al. Primary testicular lymphoma. Clin Oncol. 2012; 24:358-65. 4. Cheah CY, Wirth A, Seymour JF. Primary testicular lymphoma. Blood. 2014; 123:486-93. Figure 2. a. Macroscopical aspect of tumor with creamy white nodular proliferation. b. Histological findings: tumor cells penetrate diffusely into testicular tissue with loss of intact seminiferous tubules. Figure 3. c. Histological findings: cells demonstrated enlarged nucleii with little nucleoli and decrement of cytoplasm. d. Hematoxylin and eosin). Immunihistoche mical panel with TdT positive antibodies. Correspondence Flavia Tombolini, MD, Resident in Urology flavia.tombolini@virgilio.it Vito Lacetera, MD vlacetera@gmail.com Giovanni Muzzonigro, MD g.muzzonigro@univpm.it Guido Gini, MD, Hematologist Guido.Gini@ospedaliriuniti.marche.it Debora Capelli, MD, Hematologist Debora.Capelli@ospedaliriuniti.marche.it Pietro Leoni, MD, Hematologist p.leoni@univpm.it Rodolfo Montironi, MD, Pathologist R.Montironi@univpm.it AOU United Hospitals Polytechnic University of Marche Region, Ancona, Italy Andrea B. Galosi, MD (Corresponding Author) galosiab@yahoo.it Division of Urology, Dept. of Surgery, “Augusto Murri” General Hospital Area Vasta 4, Az. Sanitaria Unica Regione Marche 63900 Fermo (FM), Italy Tombolini_Stesura Seveso 16/01/15 12:13 Pagina 401