Granulosa cell-tumor of the testis: a case report of a very rare tumor and revision of the literature
Objective
To present a very rare case of a patient with an adult type of GCT of the testis, treated with testis sparing surgery.
Materials and Methods
We describe a case of a 32 years old patient, who was admitted to our department with suspicion of tumor in the left testis. The ultrasonography showed a 15 mm hypo-echoic, vascularised lesion in the upper pole of the left testis. The patient had no history of cryptorchidism and his past medical history was not significant, including only a spontaneous pneumothorax, when he was 18 years old. The patient denied decreased libido or erectile disfunction in the months before. Alpha-fetoprotein (alfa-FP), human chorionic gonadotropin (HCG) and lactate dehydrogenase (LDH) were normal. During the operation a frozen section was request as usual in our department, before to perform a radical treatment. The lesion was little, intra-parenchymal and simple to remove, without infiltration of the surrounding tissue. The pathologist suspected a GCT and the surgeon decided to perform a testis sparing surgery, waiting for the definitive histology.
Results
The postoperative course was uneventful and the patient was released from the hospital the day after the operation without complications. The definitive histology confirmed the previous report of adult-type GCT; the lesion measured 1.5 cm and showed to be not infiltrating. There was no evidence of angio-invasion or necrosis. The tumor was composed of clusters of cells with scanty cytoplasm in a predominantly micro-follicular pattern; very few mitosis could be seen. The immunohistochemical study showed positivity for calretinin, inhibin, CD-99 and beta-catenin while chromogranin, cytokeratin and melan-A were negative. A computed tomography showed absence of lymph node enlargement or distant metastases. The patient underwent regular follow up without any other surgery: an ultrasound of the testicles was done every 3 months; 6 months after surgery an abdominal ultrasound and a chest X-ray were performed and after one year the patient has well done and shows no signs of residual disease on ultrasound and computed tomography.
Discussions
Granulosa cell tumors (GCT) of the testis belong to the sex-cord stromal tumors and represent 4-5% of all testicular tumors (1). They were described for the first time in 1952 and they can be divided in two distinct groups, the juvenile type and the adult type. Although the juvenile-type represents only 1-4% of pre-pubertal testicular tumors, it’s the most common testis neoplasm in the first 6 months of life (2). The adult type is instead extremely rare and occurs at any age after puberty with only a limited number of reported cases to date: after a review of the literature we have found only 51 published cases, mostly isolated as case report (1, 2, 3). Unlike the juvenile type, which is typically benign, 20-25% of the adult type has been reported to be malignant with a metastatic potential even after 10 years: the retroperitoneal lymph nodes are the most common metastatic regions but liver, lung and bone metastases have been also described. Gynecomastia, erectile disfunction and decreased libido may be present in 20-25% of the cases, due to hormonal or chromosomal abnormalities. Because of his low incidence and the lack of data, the prognosis is not really known and consequently it’s almost difficult to decide what surgery to perform and if a radical treatment is really needed. Testis-sparing surgery could be offer in selected cases by small, intra-parenchymal lesions, if frozen sections are suspicious for a GCT, waiting for the definitive histologic diagnosis: by angio-invasion, necrosis, infiltrating margins and severe nuclear atypia or elevated mitotic count (4) a delayed radical orchiectomy could be performed although no cut-off for the tumor size is offered in the guide lines. A personalized follow-up is required.
Conclusion
The adult-type GCT is a rare entity, which can be malignant in 20-25% of the patients. The reported case showed, that testis sparing surgery is safe in very selected cases, if frozen sections are available and if the definitive histology shows no risk factors.
Reference
1) Giulianelli R, Mirabile G, Vincenti G, Pellegrino F, Soda G. A very rare case of adult-type granulosa cell tumor. Arch Ital Urol Androl 2015; 87 (1): 98-99
2) Mohapatra A, Potretzke AM, Knight BA, Han M, Figenshau RS. Metastatic Granulosa Cell Tumor of the Testis: Clinical Presentation and Management. Case Report in Urology 2016: 1-4.
3) Al Alao O, Gul T, Al-Ani A, Bozom A, Al-Jalham K. Adult-type granulosa cell tumor of the testis: report of a case and review of the literature. Arab J Urol 2016; 14: 44-49
4) Cornejo KM, Young RH. Adult granulosa cell tumors of the testis: a report of 32 cases. AJSP 2014; 38 (9): 1242-1250