Adult-type granulosa cell tumor of the testis: report of a case and review of literature

Granulosa cell tumors is classified into juvenile and adult types and comprise less than 5% of ovarian tumors in women and are much rarer in men which only 45 have been previously reported. We report here a 40-year young man with a left testicular adult type granulosa cell tumor. The tumor measured 5.5X5X4cm; Immunohistochemical stains showed the tumor diffusely positive for inhibin and vimentin. Post operative CT scans shows a lomboaortic lymphnodes treated by four cycles of chemotherapy type BEP (bleomycin, etoposide, cisplatin). The thoraco abdominal CT scans post chemotherapy shows the disappearance of the right testicular nodule and the lomboaortic lymphnodes. 2 years after treatment, the patient is alive and well with no signs of recurrence. Our report highlights one more case of this very rare tumor of the testis, which is quite problematic In terms of prognosis and management, and for this reason seems to have attracted the interest of many researchers recently.


Introduction
Granulosa cell tumor (GST) belongs to the sex-cord/stromal tumors of the gonads [1]. Two forms of GST have been recognized, namely, the typical adult type, and its variation, and the juvenile type [1]. Adult testicular granulosa cell tumors are extremely rare account for 1.6-6% of adult testicular tumors and occur somewhat more frequently in children and the clinical behavior of adult type granulosa cell tumors is difficult to predict [2]. Presently, these tumors appear to be slow-growing neoplasm with the potential to metastasize to distant sites years after initial diagnosis. Men who have undergone orchidectomy require extended follow-up because of the delayed metastatic potential of these tumors. Follow-up can be somewhat difficult, however, because there are no evidencebased guidelines available to help clinicians monitor their patients for metastatic disease [3]. Factors predictive of malignancy have yet to be well defined due to the very limited number of cases. We report an original case of adult type granulosa cell tumor in the testis reported for the first time at Mohamed VI center for cancer treatment in Casablanca for the first time and briefly review the previously published literature so as to improve the quality of management of this affection.

Discussion
Described for the first time in 1952, granulosa cell tumors are derived from epithelial elements of the sex cord, and they can be divided in juvenile or adult types [4]. Granulosa cell tumors affect mainly white males, usually as a painless testicular mass [5,6] Gynecomastia is present in 25% of cases, due to hormonal abnormalities such as estrogen hypersecretion, or chromosomal abnormalities [4,7]. Granulosa cell tumors affect both the ovaries and the testis, Only 29 cases of testicular adult type granulosa cell tumor have been previously reported [3,6]. The diagnosis of sex cord-stromal tumors is mostly based on microscopic, morphologic features [5]. Morphological diagnosis is based primarily on the typical morphology of the granulosa cells with their coffeebean like, angulated and grooved nuclei. Macrofollicles ought to be present, the presence of the Call Exner bodies makes correct diagnosis easier; however, they are not always found and thus, are not indispensable to diagnosis [8]. The very rare fibrothecoma of the testis can be also mistaken for undifferentiated sex cord tumor since it manifests the same immunoreactivity as all other stromal tumors.
This tumor, however, is composed of highly monotone spindle cells which are embedded in an acellular fibrous stroma [9] Immunohistochemically granulosa cell tumor is positive for inhibin,  [12]. Sites of metastases in male cases include retroperitoneal lymph nodes (most common), liver, bones, and the lungs [13]. Initial management is orchidectomy. Retroperitoneal lymphadenectomy has been additionally performed in a few cases where metastatic disease was suspected [13]. Metastatic disease may be managed with chemotherapy (etoposide alone or in combination with other agents) and adjuvant radiotherapy.

Conclusion
Our report highlights one more case of this very rare tumor of the testis, which is quite problematic In terms of prognosis and management, and for this reason seems to have attracted the interest of many researchers recently. It's necessary to identify prognostic factors that can reliably predict tumor behavior and to optimize methods of diagnosis and treatment .Long-term follow-up with a sufficient number of cases is recommended to define optimal treatment options since recurrence of the disease may appear late in the clinical course.

Competing interests
The authors declare no competing interests.

Authors' contributions
All the authors have read and approved the manuscript.