Primary retroperitoneal seminoma - embryology, histopathology and treatment particularities.

INTRODUCTION: Retroperitoneal seminoma is a very rare form of cancer, with embryological origin represented by primordial germ cells from the urogenital ridges left behind during the fetal development. Extragenital germ cell tumors can also occur in the mediastinum or the pineal gland. The aim of this paper is to outline the particularities and draw embryological, histopatological and treatment conclusions regarding extragonadal germ cell tumors. PATIENT AND METHODS: A 43-year-old patient without any additional pathology was admitted for anemia of unknown etiology. The clinical examination revealed through deep abdominal palpation a mass in the left flank, and normal testes. Thoraco-abdomino-pelvic computed tomography (CT) scan showed a large retroperitoneal tumor adjacent to the great vessels in the left lumbo-iliac region. The blood work revealed just a low hemoglobin and hematocrit. With the established diagnosis of retroperitoneal tumor, radical surgical removal was decided. During the surgery, we were required to dissect a large solid encapsulated tumor mass from the aorta and the common iliac artery, starting at the renal pedicle all the way to the left iliac bifurcation. The surgical access was obtained through a transperitoneal left subcostal incision prolonged pararectally. Histopathological and immunohistochemical studies revealed a seminoma of the usual type. After the histological findings, the patient's tumor markers were investigated (LDH - lactate dehydrogenase, ßHCG - beta-human chorionic gonadotropin, αFP - alpha-fetoprotein), all values being within normal ranges. In addition, the left testicle was thoroughly reexamined, clinically, through ultrasound and magnetic resonance imaging (MRI) scans, and no abnormalities were observed. After the surgery, the patient followed three courses of chemotherapy (BEP - Bleomycin, Etoposide and Cisplatin). RESULTS: The CT scan done 24 months after surgery found no signs of local or distant tumor recurrence. The patient entered a follow-up schedule consisting of periodical clinical, serological and imagistic evaluations. CONCLUSIONS: Primary retroperitoneal seminoma is a rare entity that must be taken into account when treating a retroperitoneal tumor. It develops out of the urogenital ridge, while the testes are normal. Thorough testicular evaluation (clinical, ultrasound and serum markers) is mandatory in all retroperitoneal tumors. The histopathological analysis is crucial for an accurate diagnosis and a proper management strategy. Through radical surgery and chemotherapy, the patients that are diagnosed prior to massive visceral metastatic dissemination can be cured.

Testicular feminization: complete androgen insensitivity syndrome. Discussions based on a case report.

INTRODUCTION AND OBJECTIVES: Testicular feminization is the syndrome when a male, genetically XY, because of various abnormalities of the X chromosome, is resistant to the actions of the androgen hormones, which in turn stops the forming of the male genitalia and gives a female phenotype. The androgen insensitivity syndrome occurs in one out of 20,000 births and can be incomplete (various sexual ambiguities) or complete (the person appears to be a woman). The aim of this paper is to present the diagnosis and treatment of a case of testicular feminization. PATIENT AND METHODS: A 22-year-old patient is admitted at Gynecology for primary amenorrhea. The clinical examination shows a female phenotype: the breasts are normally developed, but there is no hair in the groins and axillary areas, the labia are small and hypoplastic, the urinary meatus is normally inserted, and the vulva is unpigmented. The gynecological exam reveals that the hymen is present, the vagina has 1.5 cm in length, while the uterus is absent. At Endocrinology, the levels of gonadotropins were measured and found normal (FSH 3.18 mU/mL, LH 15 mU/mL), the progesterone was 5.79 nmol/L, estradiol was 82.39 pmol/L and the testosterone was 4.27 nmol/L. The karyotype was mapped in order to differentiate the androgen insensitivity syndrome from other genetic abnormalities, like the Klinefelter syndrome (46XXY), Turner syndrome (45XO), mixed gonadal dyssynergia (45XO/46XY) or tetragametic chimerism (46XX/46XY). These tests confirmed the suspected diagnosis - testicular feminization (46XY). The pelvic CT scan revealed the lack of uterus and ovaries, hypoplastic vagina, and intra-abdominal prepsoic testes. The testes were removed in order to avoid the malignant risk. We performed laparoscopic bilateral orchiectomy. RESULTS: Surgically, the patient had a simple evolution, being discharged in the second day postoperatory, and estrogen therapy was started from that moment on. Mentally, the patient kept thinking she was a woman, so the decision of telling her the truth was left to the parents. CONCLUSIONS: Testicular feminization is a rare disease that must be diagnosed and treated through close work between gynecologists, endocrinologists, geneticians, urologists, and psychiatrists. Bilateral laparoscopic orchiectomy is the best procedure to remove the intra-abdominal testes, in order to avoid their malignant transformation.