- Email: [email protected]
Late recurrence of juvenile granulosa cell tumor of the ovary
American Journal of Obstetrics and Gynecology (2004) 191, 366e7
www.elsevier.com/locate/ajog
Late recurrence of juvenile granulosa cell tumor of the ovary Stephen D. Frausto, MS,a John P. Geisler, MD,a Mavis S. Fletcher, MD,b Anil K. Sood, MDc,* Division of Gynecologic Oncology, Department of Obstetrics and Gynecology,a and Department of Pathology,b University of Iowa, Iowa City, Iowa, Department of Gynecologic Oncology, University of Texas M.D. Anderson Cancer Center,c Houston, Tex Received for publication May 29, 2003; accepted September 4, 2003
–––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––– KEY WORDS Juvenile granulosa cell tumor
Juvenile granulosa cell tumor (JGCT) of the ovary, if diagnosed at an early stage, has a favorable prognosis. Recurrences are uncommon but typically occur within the first year. The patient presented here was treated with a left oophorectomy after initial presentation. Tumor recurrence in the left adnexa, diagnosed 48 months later, was treated with cytoreductive surgery followed by chemotherapy; she remains disease free 19 months after this recurrence. Late recurrences of JGCT can occur and continued close surveillance is recommended. Ó 2004 Elsevier Inc. All rights reserved.
–––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––
More than 80% of granulosa cell tumors are encountered in women older than 40 years of age.1 Juvenile granulosa cell tumor (JGCT) of the ovary makes up less than 5% of the ovarian tumors in childhood and adolescence.2 These tumors have distinctive histologic features and consist of nests of granulosa cells interspersed with follicles. The majority of JGCTs are diagnosed as stage I and are associated with a favorable outcome.2 If they do recur, it is most often within the first year after the initial diagnosis.1,2 These recurrences are usually rapid, leading to death within 13 to 16 months.1 In contrast, adult granulosa cell tumors tend to have a protracted course and recur 4 to 6 years after initial diagnosis. * Reprint requests: Anil K. Sood, MD, University of Texas M.D. Anderson Cancer Center, Department of Gynecologic Oncology, 1515 Holcombe Blvd, Unit 440, Houston, TX 77030. E-mail: [email protected] 0002-9378/$ - see front matter Ó 2004 Elsevier Inc. All rights reserved. doi:10.1016/j.ajog.2003.09.019
Case report A 21-year-old woman, gravida 1, para 0, was seen at her local hospital in labor with an intrauterine pregnancy at 36 weeks’ gestation. The fetus was in breech presentation; therefore, a primary low transverse cesarean section was performed. A 30-cm left adnexal mass was incidentally found at this time. A left oophorectomy was performed with no gross disease found outside the ovary, but a moderate amount of ascites was noticed, which was negative for malignant cells. Final pathologic examination of the adnexal mass revealed JGCT (Figure). Histologically, there were lobular and trabecular microscopic morphologic features with scattered Call-Exner bodies. There was marked cellular pleiomorphism with grade 3 atypia, extensive necrosis, and >10 mitotic figures per highpower field.
367
Frausto et al
ceived three cycles of bleomycin, etoposide, and cisplatin and remains disease free 19 months after the diagnosis of recurrent disease.
Comment
Figure A, Follicle surrounded by sheets of densely packed granulosa cells. B, Granulosa cells with rounded, hyperchromatic nuclei.
The patient received no further treatment but was advised to receive close surveillance. She had a subsequent pregnancy 24 months after the original diagnosis and underwent a cesarean delivery without any problems. The remaining ovary was noted to be grossly normal. About 48 months after the original diagnosis, the patient began complaining of abdominal pressure and urinary urgency. The CA 125 level was 69 U/mL and the inhibin B level was 1.9 pg/mL. Ultrasonography and magnetic resonance imaging revealed an 8-cm mass superior to the uterine fundus with no free fluid or lymphadenopathy. The patient declined surgical intervention at this time but returned to the clinic 1 month later with increased pain in her left side, bloating, and weight gain. She was referred to the University of Iowa Hospitals and Clinics for further management. Transvaginal ultrasonography revealed that the mass had increased to 12 9 cm. She underwent an exploratory laparotomy, total abdominal hysterectomy, bilateral salpingo-oophorectomy, omental biopsy, anterior sigmoid resection and closure, and bilateral pelvic and periaortic lymph node sampling. A 13 8 cm left-sided mass was found to be densely adherent to the sigmoid colon and uterus and extended into the retroperitoneal space, where it was adherent to the ureter. Her 5-day postoperative stay was uncomplicated. The final pathologic examination revealed recurrent JGCT in the left adnexa. There was no tumor in the right ovary, and all remaining pathology samples were negative for metastasis. The patient subsequently re-
In contrast to adult granulosa cell tumors, which are known for late recurrences, JGCTs rarely recur.1,2 Overall, patients with JGCT have a good outcome, but the clinical course can be more aggressive than adult granulosa cell tumors.2 Young et al1 followed up 23 patients with JGCT for 5 to 10 years and 10 additional patients for 10 to 21 years without a recurrence. JGCTs are usually confined to one ovary and, because of the young age of patients with JGCT, most women undergo conservative surgery with preservation of the uterus and contralateral ovary.1 The JGCT is usually not difficult to identify. They are typically solid or cystic with some being multiloculated.1 Hemorrhagic areas are frequently seen, along with increased mitotic activity and nuclear atypia.1 These tumors have distinctive histologic features and are characterized by high mitotic activity and atypical, immature nuclei. Most of the previous studies have reported that it is very uncommon for JGCT to recur after 3 years.1 Therefore, some authors have recommended that close surveillance is only necessary for up to 3 years after definitive therapy. However, as illustrated by the case presented here, late recurrences nearly 4 years after the original diagnosis can be associated with JGCT. Thus, close surveillance may be warranted for a longer duration.
References 1. Young R, Dickerson G, Scully R. Juvenile granulosa cell tumor of the ovary: a clinicopathologic analysis of 125 cases. Am J Surg Pathol 1984;8:575-96. 2. Calaminus G, Wessalowski R, Harms D, Gobel U. Juvenile granulosa cell tumors of the ovary in children and adolescents: results from 33 patients registered in a prospective cooperative study. Gynecol Oncol 1997;65:447-52.
www.elsevier.com/locate/ajog
Late recurrence of juvenile granulosa cell tumor of the ovary Stephen D. Frausto, MS,a John P. Geisler, MD,a Mavis S. Fletcher, MD,b Anil K. Sood, MDc,* Division of Gynecologic Oncology, Department of Obstetrics and Gynecology,a and Department of Pathology,b University of Iowa, Iowa City, Iowa, Department of Gynecologic Oncology, University of Texas M.D. Anderson Cancer Center,c Houston, Tex Received for publication May 29, 2003; accepted September 4, 2003
–––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––– KEY WORDS Juvenile granulosa cell tumor
Juvenile granulosa cell tumor (JGCT) of the ovary, if diagnosed at an early stage, has a favorable prognosis. Recurrences are uncommon but typically occur within the first year. The patient presented here was treated with a left oophorectomy after initial presentation. Tumor recurrence in the left adnexa, diagnosed 48 months later, was treated with cytoreductive surgery followed by chemotherapy; she remains disease free 19 months after this recurrence. Late recurrences of JGCT can occur and continued close surveillance is recommended. Ó 2004 Elsevier Inc. All rights reserved.
–––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––––
More than 80% of granulosa cell tumors are encountered in women older than 40 years of age.1 Juvenile granulosa cell tumor (JGCT) of the ovary makes up less than 5% of the ovarian tumors in childhood and adolescence.2 These tumors have distinctive histologic features and consist of nests of granulosa cells interspersed with follicles. The majority of JGCTs are diagnosed as stage I and are associated with a favorable outcome.2 If they do recur, it is most often within the first year after the initial diagnosis.1,2 These recurrences are usually rapid, leading to death within 13 to 16 months.1 In contrast, adult granulosa cell tumors tend to have a protracted course and recur 4 to 6 years after initial diagnosis. * Reprint requests: Anil K. Sood, MD, University of Texas M.D. Anderson Cancer Center, Department of Gynecologic Oncology, 1515 Holcombe Blvd, Unit 440, Houston, TX 77030. E-mail: [email protected] 0002-9378/$ - see front matter Ó 2004 Elsevier Inc. All rights reserved. doi:10.1016/j.ajog.2003.09.019
Case report A 21-year-old woman, gravida 1, para 0, was seen at her local hospital in labor with an intrauterine pregnancy at 36 weeks’ gestation. The fetus was in breech presentation; therefore, a primary low transverse cesarean section was performed. A 30-cm left adnexal mass was incidentally found at this time. A left oophorectomy was performed with no gross disease found outside the ovary, but a moderate amount of ascites was noticed, which was negative for malignant cells. Final pathologic examination of the adnexal mass revealed JGCT (Figure). Histologically, there were lobular and trabecular microscopic morphologic features with scattered Call-Exner bodies. There was marked cellular pleiomorphism with grade 3 atypia, extensive necrosis, and >10 mitotic figures per highpower field.
367
Frausto et al
ceived three cycles of bleomycin, etoposide, and cisplatin and remains disease free 19 months after the diagnosis of recurrent disease.
Comment
Figure A, Follicle surrounded by sheets of densely packed granulosa cells. B, Granulosa cells with rounded, hyperchromatic nuclei.
The patient received no further treatment but was advised to receive close surveillance. She had a subsequent pregnancy 24 months after the original diagnosis and underwent a cesarean delivery without any problems. The remaining ovary was noted to be grossly normal. About 48 months after the original diagnosis, the patient began complaining of abdominal pressure and urinary urgency. The CA 125 level was 69 U/mL and the inhibin B level was 1.9 pg/mL. Ultrasonography and magnetic resonance imaging revealed an 8-cm mass superior to the uterine fundus with no free fluid or lymphadenopathy. The patient declined surgical intervention at this time but returned to the clinic 1 month later with increased pain in her left side, bloating, and weight gain. She was referred to the University of Iowa Hospitals and Clinics for further management. Transvaginal ultrasonography revealed that the mass had increased to 12 9 cm. She underwent an exploratory laparotomy, total abdominal hysterectomy, bilateral salpingo-oophorectomy, omental biopsy, anterior sigmoid resection and closure, and bilateral pelvic and periaortic lymph node sampling. A 13 8 cm left-sided mass was found to be densely adherent to the sigmoid colon and uterus and extended into the retroperitoneal space, where it was adherent to the ureter. Her 5-day postoperative stay was uncomplicated. The final pathologic examination revealed recurrent JGCT in the left adnexa. There was no tumor in the right ovary, and all remaining pathology samples were negative for metastasis. The patient subsequently re-
In contrast to adult granulosa cell tumors, which are known for late recurrences, JGCTs rarely recur.1,2 Overall, patients with JGCT have a good outcome, but the clinical course can be more aggressive than adult granulosa cell tumors.2 Young et al1 followed up 23 patients with JGCT for 5 to 10 years and 10 additional patients for 10 to 21 years without a recurrence. JGCTs are usually confined to one ovary and, because of the young age of patients with JGCT, most women undergo conservative surgery with preservation of the uterus and contralateral ovary.1 The JGCT is usually not difficult to identify. They are typically solid or cystic with some being multiloculated.1 Hemorrhagic areas are frequently seen, along with increased mitotic activity and nuclear atypia.1 These tumors have distinctive histologic features and are characterized by high mitotic activity and atypical, immature nuclei. Most of the previous studies have reported that it is very uncommon for JGCT to recur after 3 years.1 Therefore, some authors have recommended that close surveillance is only necessary for up to 3 years after definitive therapy. However, as illustrated by the case presented here, late recurrences nearly 4 years after the original diagnosis can be associated with JGCT. Thus, close surveillance may be warranted for a longer duration.
References 1. Young R, Dickerson G, Scully R. Juvenile granulosa cell tumor of the ovary: a clinicopathologic analysis of 125 cases. Am J Surg Pathol 1984;8:575-96. 2. Calaminus G, Wessalowski R, Harms D, Gobel U. Juvenile granulosa cell tumors of the ovary in children and adolescents: results from 33 patients registered in a prospective cooperative study. Gynecol Oncol 1997;65:447-52.