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Complete androgen insensitivity syndrome with a large gonadal serous papillary cystadenofibroma.

Ozdemir O, Sari ME, Akmut E, Selimova V, Unal T, Atalay CR - J Hum Reprod Sci (2014)

Bottom Line: We performed bilateral gonadectomy.The pathology showed testicular tissue in the right inguinal mass and a serous papillary cystadenofibroma in the left one.Gonadectomy should be performed right after puberty to prevent the risk of malignancy development in the testes.

View Article: PubMed Central - PubMed

Affiliation: Department of Gynecology and Obstetrics, Ankara Numune Education and Research Hospital, Ankara, Turkey.

ABSTRACT
We present a patient with complete androgen insensitivity syndrome (CAIS) diagnosed with a serous papillary cystadenofibroma. A 41-year-old married female with a mass in the left inguinal region and a history of primary amenorrhea. A bulging mass of 13.7 cm × 8 cm × 12.4 cm in the left inguinal region extending from the hip joint to the level of labia majus, and a 3.2 cm × 2.8 cm mass in her right inguinal region were found by ultrasonography and magnetic resonance imaging. We performed bilateral gonadectomy. The pathology showed testicular tissue in the right inguinal mass and a serous papillary cystadenofibroma in the left one. CAIS is an infrequent clinical entity, occurrence of serous papillary cystadenofibroma is even rarer in this syndrome serous cystadenofibroma should come to mind in patients with a huge inguinal mass. Gonadectomy should be performed right after puberty to prevent the risk of malignancy development in the testes.

No MeSH data available.


Related in: MedlinePlus

A bulging mass of 15 cm × 10 cm on the left side of inguinal canal
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Figure 1: A bulging mass of 15 cm × 10 cm on the left side of inguinal canal

Mentions: A 41-year-old married female applied to our clinic with a left inguinal mass. The patient had been primary amenorrheic, but had not consulted a doctor. Clinical examination showed a bulging mass of 15 cm × 10 cm on the lateral side of the left inguinal canal and another 3 cm × 4 cm mass in the right inguinal region [Figure 1]. The vulva and perineum were normal in appearance, the vagina was 6 cm long but ended blind, pubic and axillary hair was sparse; however, the patient had normal breast development as well as a normal female phenotype. The abdominal ultrasonography (USG) and magnetic resonance imaging revealed the absence of the uterus and ovaries. They also showed a 13.7 cm × 8 cm × 12.4 cm polypoid cystic mass with a regular surface in the left inguinal region extending from the hip joint to the level of labia majus, and a solid 3.2 cm × 2.8 cm mass in the right inguinal region. In hormonal analysis, follicle stimulating hormone was found to be 9.68 mIU/mL, luteinizing hormone was 15.74 mIU/mL, estradiol was 56.07 pg/mL, and testosterone was 1.83 ng/mL. The tumor markers including carcinoembryonic antigen, CA125, alpha-fetoprotein, lactate dehydrogenase, and alkaline phosphatase were within normal ranges. The chromosome test revealed a 46, XY karyotype. The diagnosis of CAIS was made based on these findings.


Complete androgen insensitivity syndrome with a large gonadal serous papillary cystadenofibroma.

Ozdemir O, Sari ME, Akmut E, Selimova V, Unal T, Atalay CR - J Hum Reprod Sci (2014)

A bulging mass of 15 cm × 10 cm on the left side of inguinal canal
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4150143&req=5

Figure 1: A bulging mass of 15 cm × 10 cm on the left side of inguinal canal
Mentions: A 41-year-old married female applied to our clinic with a left inguinal mass. The patient had been primary amenorrheic, but had not consulted a doctor. Clinical examination showed a bulging mass of 15 cm × 10 cm on the lateral side of the left inguinal canal and another 3 cm × 4 cm mass in the right inguinal region [Figure 1]. The vulva and perineum were normal in appearance, the vagina was 6 cm long but ended blind, pubic and axillary hair was sparse; however, the patient had normal breast development as well as a normal female phenotype. The abdominal ultrasonography (USG) and magnetic resonance imaging revealed the absence of the uterus and ovaries. They also showed a 13.7 cm × 8 cm × 12.4 cm polypoid cystic mass with a regular surface in the left inguinal region extending from the hip joint to the level of labia majus, and a solid 3.2 cm × 2.8 cm mass in the right inguinal region. In hormonal analysis, follicle stimulating hormone was found to be 9.68 mIU/mL, luteinizing hormone was 15.74 mIU/mL, estradiol was 56.07 pg/mL, and testosterone was 1.83 ng/mL. The tumor markers including carcinoembryonic antigen, CA125, alpha-fetoprotein, lactate dehydrogenase, and alkaline phosphatase were within normal ranges. The chromosome test revealed a 46, XY karyotype. The diagnosis of CAIS was made based on these findings.

Bottom Line: We performed bilateral gonadectomy.The pathology showed testicular tissue in the right inguinal mass and a serous papillary cystadenofibroma in the left one.Gonadectomy should be performed right after puberty to prevent the risk of malignancy development in the testes.

View Article: PubMed Central - PubMed

Affiliation: Department of Gynecology and Obstetrics, Ankara Numune Education and Research Hospital, Ankara, Turkey.

ABSTRACT
We present a patient with complete androgen insensitivity syndrome (CAIS) diagnosed with a serous papillary cystadenofibroma. A 41-year-old married female with a mass in the left inguinal region and a history of primary amenorrhea. A bulging mass of 13.7 cm × 8 cm × 12.4 cm in the left inguinal region extending from the hip joint to the level of labia majus, and a 3.2 cm × 2.8 cm mass in her right inguinal region were found by ultrasonography and magnetic resonance imaging. We performed bilateral gonadectomy. The pathology showed testicular tissue in the right inguinal mass and a serous papillary cystadenofibroma in the left one. CAIS is an infrequent clinical entity, occurrence of serous papillary cystadenofibroma is even rarer in this syndrome serous cystadenofibroma should come to mind in patients with a huge inguinal mass. Gonadectomy should be performed right after puberty to prevent the risk of malignancy development in the testes.

No MeSH data available.


Related in: MedlinePlus