Primary yolk sac tumor of the endometrium: a case report and review of the literature Roberto Rossi, MD; Daniela Stacchiotti, MD; Maria Grazia Bernardini, MD; Giovanna Calvieri, MD; Roberto Lo Voi, MD
n September 2004, a 30-year-old woman was admitted with abnormal vaginal bleeding that had been ongoing for 2 months. A transvaginal ultrasound showed that the uterine cavity was completely occupied by an oval-shaped tumor, which was hyperechoic, 55 ⫻ 37 mm in size, highly vascularized, and showed aspects of neoangiogenesis. Resistive index (RI) was 0.20. Smear cytology was performed and showed that the tumor was a cervical adenocarcinoma. Hysteroscopy showed polypoid formations in the cervical canal and uterine cavity. A biopsy revealed that the tumor was of germ cell origin. Abdominal and pelvic computerized scanning showed a uterine mass mimicking a myoma. No enlarged lymph nodes were present. Alpha-fetoprotein (AFP) levels were 1.762 ng/mL (reference value: 0-20 ng/mL). A test for the beta-subunit of human chorionic gonadotropin (B-hcG) was negative and Ca-125 was borderline (36 U/mL). After consulting the limited existing literature,1-3 the case was discussed with the pathologist and oncologist. The patient was informed of the recommended treatment, and she gave her consent. A simple total hysterectomy was performed. Bilateral adnexa were preserved. Pelvic paraaortic lymph nodes were not dissected.2,3 The removed uterus measured 11 ⫻ 5.5 ⫻ 5.6 cm and weighed 170 g. Sectioning of the uterus revealed a tumor with rounded margins. The tumor was yellowish-white
From the Obstetric and Gynecologic Unit, Mazzoni Hospital, Ascoli Piceno, Italy. Received Oct. 17, 2010; revised Nov. 28, 2010; accepted Dec. 7, 2010. Reprints: Roberto Rossi, MD, Via Giuseppe di Vittorio, 52-60131 Ancona, Italy. email@example.com 0002-9378/free © 2011 Mosby, Inc. All rights reserved. doi: 10.1016/j.ajog.2010.12.014
We describe a rare case of a primary yolk sac tumor of the endometrium treated with a simple total hysterectomy without bilateral salpingo-oophorectomy and 3 cycles of the bleomycin, etoposide and cisplatin regimen. Key words: endodermal sinus tumor, germ cells tumor, yolk sac tumor
with a central hemorrhagic area, extended from the region around the isthmus to the uterine body. The tumor was infiltrating the entire wall of the myometrium and had infiltrated the cervical stroma, reaching 1.5 mm from the outer surface. The tumor had the typical microscopic features of an endodermal sinus tumor in that it had reticular and solid growth patterns, and 47 SchillerDuval bodies were present. The neoplastic cells and hyaline globules stained positively for AFP. The morphologic and immunohistochemical pattern was consistent with a primary yolk sac tumor (YST) of the endometrium (Figure). Five days after the total hysterectomy, the patient was discharged and the levels of AFP were 759.5 ng/mL. Postoperative adjuvant therapy for the YST included 3 cycles of the bleomycin, etoposide, and cisplatin (BEP) regimen. Before the third cycle of chemotherapy, AFP levels were normal. The patient is still alive and has remained free of disease for more than 6 years after the completion of the therapy.
Metastases also occur via the blood and the lymphatic system. Because of their rarity, there has been no systematic study on the surgical management of extragonadal YSTs with a large number of cases. In 1993, Fujita et al6 reported 41 patients with endodermal sinus tumor of the ovary treated with conservative surgery, followed by chemotherapy with 5- and 10-year survival rates of around 80%. AFP determinations are useful in the diagnosis to monitor the results of therapy and for detecting metastases and recurrence after therapy. In our case, a preoperative abdominal-pelvic computed tomographic scan did not show pathologies of either the ovaries or involvement of retroperitoneal lymph nodes. The inspection of the abdomen confirmed the absence of metastatic lesions. Given the age of the patient, the proven efficacy of chemotherapy in this type of malignancy, and the ability to monitor the development of cancer with the determination of AFP, we decided to perform a conservative therapy.
Comment Of all the genital tumors, YSTs are relatively uncommon and they are mostly discovered in infants and adolescents (median age, 19 years).4 Although they typically arise from the gonads, YSTs have already been reported in many extragonadal sites. Ten to 15% of the cases may arise in a variety of midline extragonadal sites that mostly display an axial distribution pattern, such as the mediastinum, the pineal region, and sacrococcygeal region and the female reproductive tract.5 These neoplasms metastasize early and invade surrounding structures.
Conclusion Extraovarian pelvic YSTs are rare, with only 9 documented previously.7 Primary YSTs arising in the endometrium are an even more rare malignant neoplasm because, to our knowledge, this is the sixth documented case and the first treated with a simple total hysterectomy without bilateral salpingo-oophorectomy. Because YSTs occur more often in young women, we believe that preservation of ovarian function is important when possible. We also believe that a greater number of cases are necessary to determine the most adequate and appropriate treatment. f
APRIL 2011 American Journal of Obstetrics & Gynecology
Case Report FIGURE
Primary yolk sac tumor
A, Neoplasm 65 ⫻ 55 ⫻ 15 mm, yellowish-white 1 with central hemorrhagic area. B, ¡M SchillerDuval bodies. Rossi. Primary yolk sac tumor of the endometrium. Am J Obstet Gynecol 2011.
American Journal of Obstetrics & Gynecology APRIL 2011
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