Adult-type granulosa cell tumor of the testis: report of a case and review of literature
Meriem Elbachiri1,&, Amina Taleb1, Nora Derrabi2,
Zineb Bouchbika1, Nadia Benchakroun1, Hassan Jouhadi1,
Nezha Tawfiq1, Souha Sahraoui1, Abdellatif Benider1
1Mohamed VI Center of Cancer Treatment, Morocco, 2Service d’Anatomie Pathologique, Chu Ibnou Rochd, Casablanca, Morocco
Meriem Elbachiri, Mohamed VI Center of Cancer Treatment, Morocco
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.
Granulosa cell tumor (GST) belongs to the sex-cord/stromal tumors of the gonads
. Two forms of GST have been recognized, namely, the
typical adult type, and its variation, and the juvenile type .
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
. 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 evidence-based guidelines available
to help clinicians monitor their patients for metastatic disease .
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.
Patient and observation
A 40-year-young man referred to the Mohamed VI center for cancer
treatment in October 2014 with a 1-year history of a painless growing mass
in the left testis without past history of trauma, infection, or lower urinary
tract symptoms. Upon exploration, he reported a history of goiter, diabetes
and scleroderma under treatment. Physical exams revealed a normal right
epididymis and testis, and a 7 cm mass and hydrocele in the left testis,
without hernia. Ultrasonography showed a 8x 10x7cm mass with solid and cystic
components and a large hydrocele. Serum levels of alpha-fetoprotein, beta-hCG,
and LDH were within normal ranges. He underwent inguinal surgical exploration
of the left testis in June 2013, when a large testicular mass was found.
A radical orchidectomy was performed, and the hydrocele fluid was analyzed.
No frozen section biopsy was performed. Histopathological examination showed
a 5.5X5X4cm yellow tumor. Final analysis revealed a granulosa cell tumor
with oval nuclei having a longitudinal nuclear groove (Figure
1, Figure 2, Figure
3), focal invasion of the tunica albuginea and rete testis, and
no invasion of surgical margins, spermatic cord, or epididymis. Cytological
analysis of the hydrocele fluid was negative for tumor cells. Immunohistochemical
tests were positive for inhibine and vimentin, but negative for epithelial
membrane antigen. These findings strongly suggest the diagnosis of a granulosa
cell tumor. His post operative blood tests were within normal limits, and
the post operative abdominal and thoracic CT scans shows a right testis
swollen with presence of nodules on the path of the left spermatic cord
associated with a lomboaortic lymphnodes (Figure
1, Figure 2, Figure
3).the patient underwent 4 cycles of chemotherapy type BEP (bleomycin,
etoposide,cisplatin).The abdominal and thoracic CT scans post chemotherapy
shows the disappearance of the right testicular nodule and the lomboaortic
lymphnodes. No signs of recurrence or metastasis have been identified after
6 months of follow-up.
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 . Granulosa cell tumors
affect mainly white males, usually as a painless testicular mass
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
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 .
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
cells which are embedded in an acellular fibrous stroma 
Immunohistochemically granulosa cell tumor is positive for inhibin, vimentin
and calretinin,negative for epithelial membrane antigen (EMA),
placental alkaline phosphotase,synaptophysin and lymphoid markers.
Yolk sac tumor
(YST) of mixed malignant germ cell tumors can show multiple growth
patterns. However, YST is usually positive for PLAP,cytokeratin
and AFP, albeit it
can also be positive for inhibin . The morphological
features associated with malignant behaviors have not been well
defined. Hanson et al. reviewed all the reported
cases of adult testicular granulosa cell tumors and found that
only size greater
than 5.0 cm reached statistical significance in association with
malignancy. In the present case, even though the tumor was 7 centimeters
we did not find other malignant features, and the clinical behavior
has been benign so far. Mitotic count and tumor necrosis did not
reach statistical significance in predicting the tumor clinical
behavior. More cases
needed to refine the morphological features that may predict for
the clinical behavior of the tumor . Sites of metastases
in male cases include retroperitoneal lymph nodes (most common),
liver, bones, and
the lungs . Initial management is orchidectomy.
Retroperitoneal lymphadenectomy has been additionally performed
in a few cases where metastatic disease was suspected .
Metastatic disease may be managed with chemotherapy (etoposide
alone or in combination with other agents) and adjuvant radiotherapy.
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.
The authors declare no competing interests.
All the authors have read and approved the manuscript.
Figure 1: low power view of granulose cell tumor shows microfollicular pattern of growth
Figure 2: granulosa cell tumor with a solid pattern of growth with call exners bodies (stars)
Figure 3: granulosa cell tumor composed of tumor cells round to ovoid with irregular nuclear membranes and occasional nuclear
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