Which testicular tumour produces feminizing sympto-ms: (AIIMS May 2009)
|C||Lydig cell tumour|
|D||Sertoli cell tumour|
Gonadal Stromal Tumors
a. Gonadal sex cord stromal tumors are the most common nongerminal testicular tumors in children).
b. These benign non-GCTs are more common in children than adults.
c. They arise from a common mesenchymal stem cell that can differentiate toward Leydig cells, Sertoli cells, granulosa cells, or a combination of these cells.
d. An interesting feature of these tumors is their ability to secrete hormones.
Leydig Cell Tumor
a. Leydig cell tumor is the most common of the sex cord tumors, with a peak incidence at the age of 4 to 5 years.
b. Leydig cells produce testosterone, and production of the hormone by the tumor can result in precocious puberty
c. The differential diagnosis of precocious puberty includes pituitary lesions, Leydig cell hyperplasia, large cell Sertoli cell tumors, and hyperplastic testicular nodules that develop in boys with poorly controlled congenital adrenal hyperplasia (CAH)
d. Children with Leydig cell hyperplasia have normal levels of urinary 17-ketosteroids. Testicular nodules tend to occur bilaterally in CAH patients, but there have been reports of bilateral Leydig cell tumors.
e. A family history of CAH is helpful in making the diagnosis.
f. Urinary ketosteroids are elevated in patients with 21-hydroxylase deficiency, and serum levels of 17-hydroxyprogesterone are elevated.
g. Inguinal orchiectomy has been performed most often for Leydig cell tumors.
h. Testis-sparing surgery with enucleation of the tumor has now been reported in a number of children.
i. Malignancy has not been reported in leydig cell tumors in children.
Sertoli Cell Tumor
b. This tumor develops at an earlier age than Leydig cell tumors, with the usual manifestation being a painless testicular mass.
c. These tumors are not as metabolically active as Leydig cell tumors, but gynecomastia has been reported .
d. Large cell Sertoli cell tumors have been noted with increased frequency in patients with Peutz-Jeghers syndrome and the Carney complex.