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Juvenile granulosa cell tumour is a similar but histologically distinct rare tumour. It too occurs in both the ovary and testis. It too occurs in both the ovary and testis. In the testis it is extremely rare, and has not been reported to be malignant. [ 5 ]
A very rare tumour producing both ovarian (granulosa and/or theca) and testicular (Sertoli and/or Leydig) cells or tissues. Typically it consists of adult-type granulosa cells and Sertoli cells, [4] [5] but it has been reported with juvenile-type granulosa cells. [6] It has been reported to occur in the ovary usually, rarely in the testis. [7]
Granulosa cell tumors are the most common sex-cord stromal tumors, making up 70% of cases, and are divided into two histologic subtypes: adult granulosa cell tumors, which develop in women over 50, and juvenile granulosa tumors, which develop before puberty or before the age of 30.
Brenner tumor. Sex cord-stromal tumor Ovarian fibroma: 1.5%: 0%: Spindle-shaped fibroblastic cells and abundant collagen. [11] Adult granulosa cell tumor: 1%: Almost 100%: Small, bland, cuboidal to polygonal cells in various patterns. [12] Other sex cord-stromal tumors 1%: Others include mainly juvenile granulosa cell tumor, thecoma and ...
They are pathognomonic for granulosa cell tumors. Histologically, these tumors consists of monotonous islands of granulosa cells with "coffee-bean" nuclei. That same nuclear groove appearance noted in Brenner tumour , an epithelial-stromal ovarian tumor distinguishable by nests of transitional epithelial cells (urothelial) with longitudinal ...
Juvenile granulosa cell tumour has also been associated with the disease. [9] One case study indicates that this is due a mesodermal dysplasia in the long bones resulting in ovarian cancer. [10] The incidence of a secondary chondrosarcoma in Ollier disease is most commonly approximated at 25–30% with some projections even as high as 50%.
Two studies show that Akt1 is involved in Juvenile Granulosa Cell tumors (JGCT). In-frame duplications in the pleckstrin-homology domain (PHD) of the protein were found in more than 60% of JGCTs occurring in girls under 15 years of age. The JGCTs without duplications carried point mutations affecting highly conserved residues.
The embryological origin of granulosa cells remains controversial. In the 1970s, evidence emerged that the first cells to make contact with the oogonia were of mesonephric origin. It was suggested that mesonephric cells already closely associated with the oogonia proliferated throughout development to form the granulosa cell layer.