A 42-year-old Caucasian male was admitted to our Hospital referring a 4-month history of gradually increasing upper left quadrant pain. His medical history included bladder neck sclerosis, cholecystectomy, diabetes insipidus, hypogonadotropic hypogonadism, allergic asthma, and a smoking history until 10 months before this admission. The medical examination did not show any relevant features and, therefore, the subsequent diagnostic work-up continued with abdominal US.
Abdominal US showed multiple hypoechoic splenic and hepatic lesions ranging from 5 to 27 mm and, therefore, the patient underwent total body CT scan for a correct lesions characterization and staging. CT scan demonstrated bilateral hilar lymphadenopathies in the chest, pulmonary perilymphatic micronodules, enlarged retroperitoneal lymph nodes, and confirmed the hepatic and splenic lesions (Figures 1A,B). The first diagnostic hypothesis was the lymphoma and a 18F-Fludeoxyglucose (18F-FDG) PET/CT was performed, showing increased 18F-FDG uptake (standardized uptake value (SUV) max=17 at the chest hilar lymphadenopathies level) in all the lesions revealed on CT (Figure 1C). Moreover, a focal uptake was detected in left testis (Figure 1D). A brain MRI excluded central nervous system involvement. Lymphopenia was the only abnormal blood cell count value. Alpha-fetoprotein (AFP), Human chorionic gonadotropin (HCG), Aspartate aminotransferase (AST), Alkaline phosphatase (ALP) were within their normal ranges, while alanine aminotransferase (ALT) (55 U/L), gamma-glutamyl transferase (GGT) (60 U/L), C-reactive protein (CRP) (0.82 mg/dL) and Erythrocyte sedimentation rate (ESR) (20 mm) were mildly elevated. Moreover, Angiotensin Converting Enzyme (ACE) levels were elevated (95 U/L). He had no history or signs of tuberculosis (QuantiFERON -TB Gold Plus test was negative).
These findings raised the suspicion of lymphoma or sarcoidosis. An endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) was performed on the chest hilar lymphadenopathies, showing non-caseating granulomas, consistent with sarcoidosis. Stains for acid-fast bacilli and polymerase chain reaction (PCR) in specimens were negative. The final diagnosis was sarcoidosis with multiorgan involvement. A testicular US evaluation was carried out using a Canon-Toshiba Aplio 500TM (Otawara, Kanto, Japan) with a high frequency (4-14 MHz) linear transducer. The US demonstrated, using the B-mode evaluation, a hypoechoic lesion of 20 mm with ill-defined margins in the left testis (Figure 2A) corresponding to the lesion identified on PET-CT; moreover, differently from the latter technique, the US identified a smaller and well-shaped hypoechoic lesion also in the right testis (6 mm). The Color Doppler demonstrated the presence of vascular flow within the lesions (Figure 2B). Therefore, it was decided to perform CEUS, conducted with the administration of 4.8 ml of second-generation contrast media (SonoVue , Bracco, Milano, Italy) followed by 10 mL of 0.9% saline solution. Both the testicular lesions demonstrated the same pattern on CEUS. In particular, during the arterial phase, the masses showed a hypovascular appearance as compared to the surrounding testicular tissue (Figure 2C), maintaining the hypo-enhancement in the late phase. This CEUS pattern was not typical of the most frequent testicular tumors such as seminomas, which usually are arterialized appearing hyperechoic on CEUS. Finally, the imaging diagnosis was testicular sarcoidosis, based principally on bilateral involvement of testes on CEUS and on the CEUS pattern (hypoenhancement). Testicular sarcoid involvement was confirmed by surgical biopsy of both testicular masses that demonstrated non-caseating granulomas.
The patient was treated with corticosteroids and then with a second-line therapy (methotrexate), thus achieving a reduction of the SUVmax in all of the sarcoid lesions (SUVmax = 2.6 at the testicular level) at 6- and 12-month PET/CT follow-up.
According to our review (Table 1), from 2004 until now we have identified 20 cases (including the present one) of testicular sarcoidosis. Finally, to date, the total number of histologically proven testicular sarcoidosis published in literature account for 48 cases.