Gynecomastia Secondary to Choriocarcinoma In A Man With Persistant Mullerian Duct
Subgroup: Volume 3, Issue 3, Summer
Date: September 2005
Type: Case Report
Start Page: 140
End Page: 142
- R Aboutorabi Endocrine Research Center, Mashhad University of Medical Sciences, Mashhad, Iran
- B Feiz zadeh Endocrine Research Center, Mashhad University of Medical Sciences, Mashhad, Iran
- R Rajabian Endocrine Research Center, Mashhad University of Medical Sciences, Mashhad, Iran
City, Province: Mashhad,
Persistent mullerian duct is a rare syndrome secondary to anti-mullerian hormone deficiency. Here we describe the case of a phenotypically fertile male who presented with bilateral tender gynecomastia. Other than a left undescendent testicle, which was palpable in the inguinalcanal, he had normal external gentalia. Physical examination revealed an abdominal mass, palpable in the supra pubic area. The ectopic testicle showed endometrial tissue. Abdominal mass proved to be choriocarcinoma. Later he developed distant metastasis to the lung and paraortic area
Keywords: Persistent mullerian duct;Choriocarcinoma;Gynecomastia
Persistent Mullerian duct syndrome (PMDS) is a rare form of internal male pseudohermsphroditism caused by failure of the fetal testis to produce Mullerian inhibiting substance or failure of the tissues to respond to this hormone. Patients usually present in childhood with an inguinal hernia or cryptorchidism. As with undescended testes, theses gonads are at an increased risk of malignant tansformation. Gynecomastia1-3 is a rare presentation of this syndrome.4,5 We describe a case of phenotypically fertile male who presented with bilateral gynecomastia.
Mr. H.A, a 30-year old farmer, was admitted because of bilateral tender gynecomastia and decreased libido in the last 2 months. He has two children. He had an undesendent left testicle, palpable in the left inguinal canal (12 X 30 mm). His right testicle was normal. He also had a palpable firm mass below the umbilicus. Sonographic studies revealed normal right testicle (35X 16 mm), maldecendent left testicle (30 X 12 mm), pelvic mass of 70 X 120 mm (lobulated and mixed echo with central necrosis and a pressure effect on the bladder). An abdominal CT scan confirmed these findings. Laboratory study results showed testosterone of 11.4 n mol/l (N: 1035), LH 12.5 (N: 1.5-18), FSH 0.03 (N: up to 20) mIU/ml, Estradiol 240 pg/ml (N: <90), β HCG was 58 and 195 mIU/ml on 2 occasions (N: 0-5); α-Feto protein (AFP) was 1.3 (N: <4), FBS 75 mg/dl, WBC 13100, Hb 13.2, and ESR 21. Chest radiography showed 2 ill-defined nodes in the right and left lungs. In the first surgical operation, the ectopic testicle was removed. This showed a testis of 1.5×2×3.5 centimeter with a 3cm long spermatic cord. Histology revealed fibro muscular tissue with edema, dilated vessels and hemorrhage consistent with endometrial tissue (Fig. 1). After 10 days he underwent a second surgery and biopsy of the abdominal mass. 6-biopsy specimens confirmed hemorrhagic and necrotic mass with cytotrophoblasts and syncytiotrophoblasts with hyperchromatic nuclei and atypia, all charac-characteristic of choriocarcinoma (Fig. 2). Following surgery he underwent chemotherapy. He
was under control for 8 months he was admitted again for lung metastasis and paraaortic lynphadenopathy. A second course of chemotherapy was planned; we do not have further follow up of this patient.
Fig 1. Endometrial struma and glands in ectopic testicle, Х 40.
Fig 2. Biopsy of abdomial mass showing choriocarcinoma cells with necrosis, Х 400.
Gynecomastia secondary to choriocarcinoma 141
teristic of choriocarcinoma (Fig. 2). Following surgery he underwent chemotherapy. He was under control for 8 months he was admitted again for lung metastasis and paraaortic lynphadenopathy. A second course of chemotherapy was planned; we do not have further follow up of this patient.
Gynecomastia is the most common manifestation of estrogen excess in men.1-3 Tenderness may be present in a third of patients. Enlargement is usually central and symmetric, although occasionally it is eccentric. Idiopathic and drug-induced gynecomastia is usually unilateral; however, in pubertal and hormonal cases, the changes are often bilateral.4 Pathologic gynecomastia and decreased libido may be due to testosterone deficiency, increased estrogen production, or increased conversion of androgens to estrogens. The pathological conditions associated with gynecomastia include congenital anorchia, Klinefelter syndrome, testicular feminization, hermaphroditism, adrenal carcinoma, liver disorders, and malnutrition and choriocarci 1,4,5noma. Our patient had persistent mullerian duct syndrome which is secondary to anti-mullerian hormone deficiency.6 This patient was phenotypically male, fertile, and presented with gynecomastia; he was found to have an ectopic testis that contained uterine tissue. He also had an abdominal mass, found to be choriocarcinoma originating from the ectopic testicle. In this patient, gynecomastia is secondry to increased ß-HCG and estrogen that is possibly due to conversion from testosterone.5,7In patients with unrecognised intraabdominal testis, nonseminomatous germ-cell tumors are less common than pure seminomas.8,9 20 cases of testicular neoplasia with PMDS have been reported; . About 10% of testicular tumours arise from an undescended testis,9 and the relative risk of testicular cancer in cryptorchidism is 7·5%.10 The location of the undescended testis also affects the risk of developing a tumor, in that the higher the position of the undescended testis, the greater the risk of developing a malignant tumor.11 The overall incidence of malignant transformation in the gonads of patients with PMDS is 18%, which is similar to the rate in abdominal testes in otherwise healthy males.12 We suggest that any case of gynecomastia be evaluated for secondary causes and congenital malformations.
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- Giri SK, Berney D, O'Driscoll J, Drumm J, Flood HD, Gupta RK. Choriocarcinoma with teratoma arising from an intra-abdominal testis in patient with persistent Mullerian duct syndrome. Lancet Oncol. 2004; 5(7): 451-2.
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