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Ovarian Endodermal Sinus Tumor in a Postmenopausal Woman
Gynecologic Oncology 82, 392–394 (2001) doi:10.1006/gyno.2001.6286, available online at http://www.idealibrary.com on
CASE REPORT Ovarian Endodermal Sinus Tumor in a Postmenopausal Woman Chien Oh, M.D.,* Ady Kendler, M.D., Ph.D.,† and Enrique Hernandez, M.D.* ,† *Department of Obstetrics, Gynecology, and Reproductive Sciences, and †Department of Pathology and Laboratory Medicine, Temple University School of Medicine, 3401 N. Broad Street, Philadelphia, Pennsylvania 19140 Received March 23, 2001; published online July 10, 2001
women. The median age of patients with ovarian endodermal sinus tumor in one series is 19 years [3]. In this series, approximately 23% of these patients were prepubertal and the oldest woman was 45 years old. Several cases of endodermal sinus tumor in the perimenopausal and menopausal age groups have been reported. The oldest women that we could find described in the literature with pure endodermal sinus tumor were two patients in their sixties. One tumor originated from the ovary [4], and the other was of extraovarian origin [5]. We report what we believe to be the oldest woman to be diagnosed with a pure endodermal sinus tumor of the ovary.
Background. Ovarian endodermal sinus tumor is rare in postmenopausal women. Case. We report the case of a 75-year-old woman with a pure endodermal sinus tumor of the ovary. Conclusion. We believe this to be the oldest patient reported with an ovarian endodermal sinus tumor. The histogenesis of this entity in older patients may be different than when it occurs in young women. © 2001 Academic Press Key Words: ovary; neoplasm; endodermal sinus tumor; yolk sac tumor; postmenopausal.
INTRODUCTION CASE REPORT The endodermal sinus tumor, the second most common form of malignant germ cell tumor, is believed to arise from germ cells that differentiate into the extraembryonal yolk sac. Endodermal sinus tumors have been reported not only in the ovary, but also in extragonadal regions such as the mediastinum [1] and pineal body [2]. Histologically, multiple patterns may be seen in a single tumor including microcystic and myxoid, polyvesicular vitelline, glandular, and hepatoid morphologies. Characteristic papillary structures (Schiller–Duvall bodies) may also be seen. Intracellular and extracellular hyaline droplets that represent deposits of ␣-fetoprotein (AFP) can be identified throughout the tumor. Serum AFP is the ideal tumor marker for following the patient’s disease status and is extremely useful in detecting recurrences. Germ cell tumors are relatively uncommon, accounting for 15–20% of all ovarian tumors, and only 2–3% of germ cell tumors are malignant. Dysgerminoma and endodermal sinus tumors account for the majority of the malignant germ cell tumors. Specifically, endodermal sinus (also known as yolk sac) tumors account for approximately 20 –25% of the malignant germ cell tumors. Patients with endodermal sinus tumors present with a rapidly enlarging pelvic mass and abdominal pain. Most patients present in their second and third decades of life although there have been case reports of malignant germ cell tumors occurring in infancy and in postmenopausal 0090-8258/01 $35.00 Copyright © 2001 by Academic Press All rights of reproduction in any form reserved.
392
A 75-year-old multiparous woman presented to her primary gynecologist with complaints of lower abdominal pain and urinary frequency for 1 month. Physical examination was significant for a 15-cm mass in the left lower abdominal quadrant. Pelvic ultrasound revealed a 16 ⫻ 11 ⫻ 13 cm pelvic mass. It was unclear whether the mass was of uterine or ovarian origin. Her CA-125 was slightly elevated at 100 units/ ml. A computerized tomogram of the abdomen confirmed the ultrasound findings and also identified a 3.3-cm precaval lymph node. A total abdominal hysterectomy, bilateral salpingo-oophorectomy, sigmoid colectomy with colorectal anastomosis, omentectomy, and pelvic node biopsy was performed. The right ovary measured 15 ⫻ 12 ⫻ 8 cm and weighed 605 g. The external surface of the ovary showed smooth, pink–tan areas along with firm, rough, yellow– brown areas. Cut-section showed two cystic cavities containing yellow– brown fluid, an area of pink, spongy, and cystic soft tissue, and firm yellow– gray tissue. Microscopic examination of the ovarian tumor showed characteristics consistent with an endodermal sinus (yolk sac) tumor. A multicystic polyvitelline pattern (Fig. 1), solid and hepatoid areas, and malignant glandular structures floating within abundant myxoid stroma (Fig. 2), as well as secondary structures consistent with Schiller–Duvall bodies,
CASE REPORT
FIG. 1.
393
The multicystic polyvitelline pattern of the endodermal sinus tumor is demonstrated in this microphotograph (HE, original magnification ⫻100).
were seen. The tumor cells were strongly immunopositive for low- and high-molecular-weight keratins (AE1/AE3 and cam 5.2) and negative for OC-125. Scattered glands were immunopositive for secreted AFP. Immunostain for stromal ele-
FIG. 2.
ments (myoglobin) was negative. No other germ cell elements were seen. No elements consistent with an epithelial surface ovarian cancer or with endometriosis were present. Metastases at multiple sites were present throughout the patient’s abdom-
The myxomatous pattern of the endodermal sinus tumor is shown (HE, original magnification ⫻100).
394
OH, KENDLER, AND HERNANDEZ
inal cavity, and her disease was staged as IIIC endodermal sinus tumor of the right ovary. Serum AFP levels were obtained after the diagnosis was established and found to be 17,318 ng/ml. Four weeks after the surgery, the patient was started on a chemotherapeutic regimen of etoposide and cisplatin and received a total of three courses. AFP levels dropped rapidly to 12.3 ng/ml after the third course. Despite the laboratory response, the patient expired 4 months after the surgery of cardiac tamponade secondary to a malignant pericardial effusion.
postmenopausal. A case of an 82-year-old woman with an ovarian mucinous tumor with a yolk sac component has also been reported [10]. These cases, as well as others occuring in younger women in association with endometriosis, are believed to originate from somatic, mesodermal cells and not germ cells. We believe that our patient is the oldest woman documented with a pure endodermal sinus tumor of the ovary. After extensive sampling of the tumor, we found no areas of primary surface carcinoma or endometriosis. REFERENCES
DISCUSSION The occurrence of endodermal sinus tumor in postmenopausal women is relatively rare. A Medline search of the medical literature published in English produced four cases of pure endodermal sinus tumor in women over the age of 50 [4 –7]. A few more cases of mixed tumor in which some of the microscopic findings were consistent with endodermal sinus tumor in postmenopausal women were also found. Talerman [8] makes reference to two cases of endodermal sinus tumor in postmenopausal women but does not describe them in detail. He states that although the histogenesis of the endodermal sinus tumor in a postmenopausal woman is unclear, “a likely explanation is that it originates from the surface epithelium by a process of neoplastic dedifferentiation or transformation and therefore the histogenesis is totally different from that of germ cell neoplasms.” Nogales et al. [9] reported six cases of ovarian endometrioid tumors with yolk sac components. Four of the six patients were
1. Truong LD. Endodermal sinus tumor of the mediastinum: a report of seven cases and review of literature. Cancer 1986;58:130 –9. 2. Bestle J. Extragonadal endodermal sinus tumors originating in the region of the pineal gland. Acta Pathol Microbiol Scand 1968;74:214 –22. 3. Kurman RJ, Norris HJ. Endodermal sinus tumor of the ovary: a clinical and pathologic analysis of 71 cases. Cancer 1976;38:2404 –19. 4. Kinoshita K. A 62-year-old woman with endodermal sinus tumor of the ovary. Am J Obstet Gynecol 1990;172:760 –2. 5. Ferracini R, Gardini G, Lanzanova G, Lorenzini P. Endodermal sinus tumor in a 63-year-old female. Pathologica 1979;71:885–7. 6. Pliskow S. Endodermal sinus tumor of the ovary: review of 10 cases. South Med J 1993;86:187–9. 7. Brown JR, Green JD. Yolk sac carcinoma. South Med J 1976;69:728 –31. 8. Talerman A. In: Kurman RB, editor. Blaustein’s pathology of the female genital tract, 4th ed. New York:Springer-Verlag, 1994:868. 9. Nogales FF, Bergerson C, Carvia RE, Alvaro T, Fulwood HR. Ovarian endometrioid tumors with yolk sac tumor component, an unusual form of ovarian neoplasm. Am J Surg Pathol 1996;20:1056 – 66. 10. Mazur MT, Talbot WH Jr, Talerman A. Endodermal sinus tumor and mucinous cystadenofibroma of the ovary: occurrence in an 82-year-old woman. Cancer 1988;62:2011–5.
CASE REPORT Ovarian Endodermal Sinus Tumor in a Postmenopausal Woman Chien Oh, M.D.,* Ady Kendler, M.D., Ph.D.,† and Enrique Hernandez, M.D.* ,† *Department of Obstetrics, Gynecology, and Reproductive Sciences, and †Department of Pathology and Laboratory Medicine, Temple University School of Medicine, 3401 N. Broad Street, Philadelphia, Pennsylvania 19140 Received March 23, 2001; published online July 10, 2001
women. The median age of patients with ovarian endodermal sinus tumor in one series is 19 years [3]. In this series, approximately 23% of these patients were prepubertal and the oldest woman was 45 years old. Several cases of endodermal sinus tumor in the perimenopausal and menopausal age groups have been reported. The oldest women that we could find described in the literature with pure endodermal sinus tumor were two patients in their sixties. One tumor originated from the ovary [4], and the other was of extraovarian origin [5]. We report what we believe to be the oldest woman to be diagnosed with a pure endodermal sinus tumor of the ovary.
Background. Ovarian endodermal sinus tumor is rare in postmenopausal women. Case. We report the case of a 75-year-old woman with a pure endodermal sinus tumor of the ovary. Conclusion. We believe this to be the oldest patient reported with an ovarian endodermal sinus tumor. The histogenesis of this entity in older patients may be different than when it occurs in young women. © 2001 Academic Press Key Words: ovary; neoplasm; endodermal sinus tumor; yolk sac tumor; postmenopausal.
INTRODUCTION CASE REPORT The endodermal sinus tumor, the second most common form of malignant germ cell tumor, is believed to arise from germ cells that differentiate into the extraembryonal yolk sac. Endodermal sinus tumors have been reported not only in the ovary, but also in extragonadal regions such as the mediastinum [1] and pineal body [2]. Histologically, multiple patterns may be seen in a single tumor including microcystic and myxoid, polyvesicular vitelline, glandular, and hepatoid morphologies. Characteristic papillary structures (Schiller–Duvall bodies) may also be seen. Intracellular and extracellular hyaline droplets that represent deposits of ␣-fetoprotein (AFP) can be identified throughout the tumor. Serum AFP is the ideal tumor marker for following the patient’s disease status and is extremely useful in detecting recurrences. Germ cell tumors are relatively uncommon, accounting for 15–20% of all ovarian tumors, and only 2–3% of germ cell tumors are malignant. Dysgerminoma and endodermal sinus tumors account for the majority of the malignant germ cell tumors. Specifically, endodermal sinus (also known as yolk sac) tumors account for approximately 20 –25% of the malignant germ cell tumors. Patients with endodermal sinus tumors present with a rapidly enlarging pelvic mass and abdominal pain. Most patients present in their second and third decades of life although there have been case reports of malignant germ cell tumors occurring in infancy and in postmenopausal 0090-8258/01 $35.00 Copyright © 2001 by Academic Press All rights of reproduction in any form reserved.
392
A 75-year-old multiparous woman presented to her primary gynecologist with complaints of lower abdominal pain and urinary frequency for 1 month. Physical examination was significant for a 15-cm mass in the left lower abdominal quadrant. Pelvic ultrasound revealed a 16 ⫻ 11 ⫻ 13 cm pelvic mass. It was unclear whether the mass was of uterine or ovarian origin. Her CA-125 was slightly elevated at 100 units/ ml. A computerized tomogram of the abdomen confirmed the ultrasound findings and also identified a 3.3-cm precaval lymph node. A total abdominal hysterectomy, bilateral salpingo-oophorectomy, sigmoid colectomy with colorectal anastomosis, omentectomy, and pelvic node biopsy was performed. The right ovary measured 15 ⫻ 12 ⫻ 8 cm and weighed 605 g. The external surface of the ovary showed smooth, pink–tan areas along with firm, rough, yellow– brown areas. Cut-section showed two cystic cavities containing yellow– brown fluid, an area of pink, spongy, and cystic soft tissue, and firm yellow– gray tissue. Microscopic examination of the ovarian tumor showed characteristics consistent with an endodermal sinus (yolk sac) tumor. A multicystic polyvitelline pattern (Fig. 1), solid and hepatoid areas, and malignant glandular structures floating within abundant myxoid stroma (Fig. 2), as well as secondary structures consistent with Schiller–Duvall bodies,
CASE REPORT
FIG. 1.
393
The multicystic polyvitelline pattern of the endodermal sinus tumor is demonstrated in this microphotograph (HE, original magnification ⫻100).
were seen. The tumor cells were strongly immunopositive for low- and high-molecular-weight keratins (AE1/AE3 and cam 5.2) and negative for OC-125. Scattered glands were immunopositive for secreted AFP. Immunostain for stromal ele-
FIG. 2.
ments (myoglobin) was negative. No other germ cell elements were seen. No elements consistent with an epithelial surface ovarian cancer or with endometriosis were present. Metastases at multiple sites were present throughout the patient’s abdom-
The myxomatous pattern of the endodermal sinus tumor is shown (HE, original magnification ⫻100).
394
OH, KENDLER, AND HERNANDEZ
inal cavity, and her disease was staged as IIIC endodermal sinus tumor of the right ovary. Serum AFP levels were obtained after the diagnosis was established and found to be 17,318 ng/ml. Four weeks after the surgery, the patient was started on a chemotherapeutic regimen of etoposide and cisplatin and received a total of three courses. AFP levels dropped rapidly to 12.3 ng/ml after the third course. Despite the laboratory response, the patient expired 4 months after the surgery of cardiac tamponade secondary to a malignant pericardial effusion.
postmenopausal. A case of an 82-year-old woman with an ovarian mucinous tumor with a yolk sac component has also been reported [10]. These cases, as well as others occuring in younger women in association with endometriosis, are believed to originate from somatic, mesodermal cells and not germ cells. We believe that our patient is the oldest woman documented with a pure endodermal sinus tumor of the ovary. After extensive sampling of the tumor, we found no areas of primary surface carcinoma or endometriosis. REFERENCES
DISCUSSION The occurrence of endodermal sinus tumor in postmenopausal women is relatively rare. A Medline search of the medical literature published in English produced four cases of pure endodermal sinus tumor in women over the age of 50 [4 –7]. A few more cases of mixed tumor in which some of the microscopic findings were consistent with endodermal sinus tumor in postmenopausal women were also found. Talerman [8] makes reference to two cases of endodermal sinus tumor in postmenopausal women but does not describe them in detail. He states that although the histogenesis of the endodermal sinus tumor in a postmenopausal woman is unclear, “a likely explanation is that it originates from the surface epithelium by a process of neoplastic dedifferentiation or transformation and therefore the histogenesis is totally different from that of germ cell neoplasms.” Nogales et al. [9] reported six cases of ovarian endometrioid tumors with yolk sac components. Four of the six patients were
1. Truong LD. Endodermal sinus tumor of the mediastinum: a report of seven cases and review of literature. Cancer 1986;58:130 –9. 2. Bestle J. Extragonadal endodermal sinus tumors originating in the region of the pineal gland. Acta Pathol Microbiol Scand 1968;74:214 –22. 3. Kurman RJ, Norris HJ. Endodermal sinus tumor of the ovary: a clinical and pathologic analysis of 71 cases. Cancer 1976;38:2404 –19. 4. Kinoshita K. A 62-year-old woman with endodermal sinus tumor of the ovary. Am J Obstet Gynecol 1990;172:760 –2. 5. Ferracini R, Gardini G, Lanzanova G, Lorenzini P. Endodermal sinus tumor in a 63-year-old female. Pathologica 1979;71:885–7. 6. Pliskow S. Endodermal sinus tumor of the ovary: review of 10 cases. South Med J 1993;86:187–9. 7. Brown JR, Green JD. Yolk sac carcinoma. South Med J 1976;69:728 –31. 8. Talerman A. In: Kurman RB, editor. Blaustein’s pathology of the female genital tract, 4th ed. New York:Springer-Verlag, 1994:868. 9. Nogales FF, Bergerson C, Carvia RE, Alvaro T, Fulwood HR. Ovarian endometrioid tumors with yolk sac tumor component, an unusual form of ovarian neoplasm. Am J Surg Pathol 1996;20:1056 – 66. 10. Mazur MT, Talbot WH Jr, Talerman A. Endodermal sinus tumor and mucinous cystadenofibroma of the ovary: occurrence in an 82-year-old woman. Cancer 1988;62:2011–5.