quiz Testicular tumors in the setting of isosexual precocious puberty

[Admin]

First one with correct answer with justification win.

An 8 year old boy presents with a palpable mass in the left testicle associated with testicular enlargement, pubic hair, and clear signs of precocious puberty. You are most suspicious of which of the following testicular tumors in the setting of isosexual precocious puberty

A rhabdomyosarcoma

B Leydig cell tumor

C Sertoli-Leydig cell tumor

D endodermal sinus tumor

E testicular leukemia

[Admin]

correct answer
B Leydig cell tumor

The most common primary testicular neoplasms in boys are testicular germ cell tumors. Endodermal sinus tumor is the most common, but does not present with virilization. In tumors presenting with premature virilization, a Leydig cell tumor secreting testosterone should be suspected. Infiltration of the testes is occasionally the presenting manifestation of leukemia. Rhabdomyosarcoma would typically present as a paratesticular, not a testicular, mass.

Leydig cell tumor of the testis is a rare tumor which, in prepubertal boys, seems to occur on a continuum with Leydig cell hyperplasia. Although pure Leydig cell tumors occasionally occur in the ovary, the majority of pure Leydig cell tumors are found in males, with a bimodal age distribution: usually at 5–10 years of age or in middle-aged adult men (30–60 years). Children typically present with precocious puberty due to excess testosterone secreted by the tumor. Ultrasound and elevated testosterone associated with low FSH and LH are used in diagnosis. Occasionally the tumor is occult on ultrasound and MRI has been described.

Chemotherapy has limited efficacy in Leydig cell tumor of the testis, although Imatinib has been used experimentally in malignant tumors (about 10% of adult tumors are malignant). There is no current role for radiotherapy. The prognosis is generally good as the tumor tends to grow slowly and usually is benign. For malignant tumors with undifferentiated histology, prognosis is poor.

The usual treatment is surgery. Historically, a radical inguinal orchiectomy was recommended. However, as recently described by Mennie et al, there are increasing data in support of simple enucleation with testes-sparing surgery to maintain fertility in children and young adults. Inguinal exploration is used with isolation of the cord, vascular occlusion, and delivery of the testicle into the wound. Biopsy of the tumor with vascular occlusion and simple enucleation of the tumor if not malignant would be acceptable in this situation. In this age group, the tumor is typically unifocal with a low recurrence rate.

[Secundino López Ibarra]

C...Sertoli-Leydig

[Sharon]

C

[mughrabi79]

C

[Manal Dhaiban]

B- Leydig cell tumors

[Basma Waseem]

Leydig cell tumor

[Titolugo]

B Leydig ell tumor - http://pedsurgeryupdate.com/theme01.htm

[Admin]

correct answer
B Leydig cell tumor

The most common primary testicular neoplasms in boys are testicular germ cell tumors. Endodermal sinus tumor is the most common, but does not present with virilization. In tumors presenting with premature virilization, a Leydig cell tumor secreting testosterone should be suspected. Infiltration of the testes is occasionally the presenting manifestation of leukemia. Rhabdomyosarcoma would typically present as a paratesticular, not a testicular, mass.

Leydig cell tumor of the testis is a rare tumor which, in prepubertal boys, seems to occur on a continuum with Leydig cell hyperplasia. Although pure Leydig cell tumors occasionally occur in the ovary, the majority of pure Leydig cell tumors are found in males, with a bimodal age distribution: usually at 5–10 years of age or in middle-aged adult men (30–60 years). Children typically present with precocious puberty due to excess testosterone secreted by the tumor. Ultrasound and elevated testosterone associated with low FSH and LH are used in diagnosis. Occasionally the tumor is occult on ultrasound and MRI has been described.

Chemotherapy has limited efficacy in Leydig cell tumor of the testis, although Imatinib has been used experimentally in malignant tumors (about 10% of adult tumors are malignant). There is no current role for radiotherapy. The prognosis is generally good as the tumor tends to grow slowly and usually is benign. For malignant tumors with undifferentiated histology, prognosis is poor.

The usual treatment is surgery. Historically, a radical inguinal orchiectomy was recommended. However, as recently described by Mennie et al, there are increasing data in support of simple enucleation with testes-sparing surgery to maintain fertility in children and young adults. Inguinal exploration is used with isolation of the cord, vascular occlusion, and delivery of the testicle into the wound. Biopsy of the tumor with vascular occlusion and simple enucleation of the tumor if not malignant would be acceptable in this situation. In this age group, the tumor is typically unifocal with a low recurrence rate.