Juvenile granulosa tumor of the ovary: Report of two cases

Juvenile granulosa tumours are malignant tumours belonging to the group of sexual cord and stroma tumours, they are rare and represent less than 5% of ovarian tumours in children and adolescents, with a maximum frequency of between 0 and 10 years [1]. They have the particularity of being in the majority of secretory and hyperoestrogenic cases, and are considered to be tumours with low malignant potential. The ideal treatment for these tumours is surgical: adnexectomy [1-2].


Introduction
Juvenile granulosa tumours are malignant tumours belonging to the group of sexual cord and stroma tumours, they are rare and represent less than 5% of ovarian tumours in children and adolescents, with a maximum frequency of between 0 and 10 years [1]. They have the particularity of being in the majority of secretory and hyperoestrogenic cases, and are considered to be tumours with low malignant potential.
The ideal treatment for these tumours is surgical: adnexectomy left ovarian cyst, a cystic formation of another nature cannot be ruled out ( Figure 2). Tumor markers (Alpha-fetoprotein and beta HCG) were normal. Surgical exploration found a huge tumor at the left ovary (Figures 3,4) of 25×20 cm, with serum effusion. The girl has benefi ted from an ovarictomy with normal excisional borders on histological examination. The anatomo-pathological study completed by immunolabeling was returned in favour of a juvenile tumor of the granulosa.
The postoperative consequences were simple. The patient received periodic clinical and radiological monitoring through chest x-rays and abdominal-pelvic ultrasound. This follow-up was without particularities. In a multidisciplinary staff it was decided that the patient would not require chemotherapy or radiotherapy.

Case report 2
An 11-year-old girl with no particular pathological history had been suffering for about 2 months from pelvic, paroxysmal, moderate intensity pain associated with an increase in abdominal volume without altering her general state of health.
The abdominal examination found a painless abdominalpelvic mass measuring approximately 30 ×20 cm in diameter, of hard consistency, poorly limited, mobile with respect to the superfi cial plane, and fi xed with respect to the deep plane, with collateral venous circulation, without signs of hypersecretion and an undepressed umbilicus.
She had an ultrasound scan outside the UHC, which found a mainly echogenic mass, compatible with a supravesical cystic mass, without intraperitoneal effusion or deep adenopathies, and without liver damage. An abdominal-pelvic CT scan found this right lateral-uterine mass of 32×21 cm, tissue, semi-solid,     and Gs, anomaly in gene expression in gonadal determination [4]. Juvenile granulosa tumours are manifested by a tumor syndrome: painful abdominal distention related to tumor size [5].
Sometimes, the pain is acute, resulting from the twisting of the ovary and their hemorrhagic nature. Exceptionally, granulosa tumours may appear in the form of a rupture table with hemoperitoneum due to their hemorrhagic nature [1][2][3][4][5].
An endocrine syndrome is linked to the secretory functions of these tumours: early isosexual pseudo-puberty in young girls in case of estrogenic secretion; hirsutism, clitoral hypertrophy in case of androgenic secretion [6]. In the case of our patient, we found the abdominal-pelvic mass that was isolated, with no signs of hypersecretion.
Ultrasound is the most commonly used complementary examination for the exploration of ovarian tumours; it confi rms the clinical examination data, links the pelvic mass to its ovarian origin, determines its semiological characteristics and evaluates the degree of abdominal-pelvic extension of the tumour. Ultrasound may reveal a large echogenic mass, or a cystic mass with partitions, achieving a multilocular aspect, but the unilocular aspect is also found, or it may appear of a pure solid homogeneous or heterogeneous nature [5].
CT has a detection rate that is sometimes lower than that of ultrasound, in the diagnosis of presumptive ovarian tumours. It could be justifi ed in view of the large size of the pelvic tumour, which poses the problem of its primary site and its relationship with neighbouring anatomical structures [7]. The information provided by the MRI does not appear to be superior to that of a pelvic ultrasound performed under excellent technical conditions by an experienced ultrasound technician [7]. The ultrasound and CT scan performed on our patient identifi ed the adnexal origin of a huge multi-partitioned cystic tumor ( Figures 1,2). Estradiol is measured in early pseudo-puberty; it can be used as a tumor marker, while inhibin is currently a good marker specifi c for granulosa tumors [5].
The tumor is usually unilateral (97%) and measures on average 12.5 cm. Its surface is smooth, solid, cystic or a combination of both. It is characterized by dense ranges of cells with non-incised, hyperchromatic and often mitosis nuclei. Rare immature follicles secreting mucus are observed.
The ideal treatment for these tumours is surgical: adnexectomy. Chemotherapy is offered in case of recurrence in addition to surgical resumption, and must use at least one anthracycline [9,10]. Radiation therapy has not been shown to be effective [9,10].
The poor prognostic factors are: large size, ascites and capsular rupture [10].

Conclusion
Granulosa tumours are rare tumours of the ovary with an anatomo-pathological diagnosis. Several cellular and molecular alterations could be involved in the development of these tumours. Treatment typically involves initial surgery, which consists of an oophorectomy or salpingo-oophorectomy, and the prognosis is generally favourable.