Case report of a Sertoli cell tumor with unique presentation including fevers, weight loss, and leukocytosis in a 13-year-old male

Abstract

Sertoli cell tumors are a rare type of sex-cord stromal tumor. We present a case of a thirteen-year-old male presenting with 2–3 months of fevers and twenty-pound weight loss. Evaluation revealed leukocytosis, anemia, elevated systemic inflammatory markers and a negative infectious disease evaluation. An evaluation for malignancy revealed an incidental testicular mass. A radical orchiectomy lead to prompt resolution of fevers, leukocytosis, and elevated systemic inflammatory markers. Final pathology was consistent with a Sertoli cell tumor. To our knowledge, this is the first reported case of a Sertoli cell tumor presenting with fever, leukocytosis, and weight loss.

1 Introduction

Testis cancer is relatively rare accounting for about 1 % of all tumors. A small fraction of testicular tumors are sex cord-stromal tumors (SCSTs) encompassing Leydig, Sertoli, granulosa, tumors in the fibroma-thecoma group, mixed and unclassified sex-cord stromal tumors. We present a case of a 13-year-old male presenting with daily fevers and weight loss found to have a testicular mass whose symptoms resolved with radical orchiectomy with a diagnosis of Sertoli cell tumor (SCT).

2 Case presentation

A thirteen-year-old Hispanic male with no relevant medical history presented with 2–3 months of daily fevers and twenty-pound weight loss to 5th percentile for age. Serum blood markers showed leukocytosis to 50.3 thousand/mm ³ , thrombocytopenia to 488 thousand/mm ³ , and hemoglobin of 10.6 g/dL. Systemic inflammatory markers were elevated including ferritin (2017.4 ng/mL), ESR (78 mm/hr), and CRP (24.3 mg/dL). A broad infectious work-up was initiated; however, urine cultures, blood cultures, and anti-organism antibodies were negative. A quantiferon tuberculosis test was indeterminate and patient was initiated on appropriate therapy. After three weeks, the patient’s symptoms were unchanged prompting a malignancy work-up for a paraneoplastic syndrome. Scrotal ultrasound identified a 3.2 cm intraparenchymal mass ( Fig. 1 ). Computed tomography was negative for metastases. Serum tumor markers including alpha-fetoprotein, beta-human chorionic gonadotropin, and lactate dehydrogenase were negative. Physical exam was consistent with a firm right testicle. After multi-disciplinary discussion and consent from family, the patient underwent a right radical orchiectomy. Postoperative course was uncomplicated with prompt fever and leukocytosis resolution and weight gain (12 pound weight gain to 19th percentile for age by 1 month postoperatively). Inflammatory markers declined rapidly postoperatively and were normal within 2 weeks. Pathology revealed a pT2 testis tumor most consistent with a Sertoli cell tumor ( Figs. 2 and 3 ). Staining for steroidogenic factor 1, transcription factor binding to IGHM enhancer 3, and inhibin were supportive of Sertoli cell tumor diagnosis. The absence of nuclear beta-catenin, prominent granulocytic infiltrate, and peripheral lymphoid aggregates were consistent with large cell calcifying subtype of Sertoli cell tumor (LCCSCT) but calcifications were not seen. At one year follow-up, there was no evidence of metastatic disease and markers were negative. He remains on surveillance protocol.