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Clear Cell Carcinoma of the Ovary

A Remarkable Case

Giblin, Joan, M.S.N., R.N.C.S., A.O.C.N.; Fanucchi, Michael P., M.D., and; McGuire, William P., M.D.

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American Journal of Clinical Oncology: February 2001 - Volume 24 - Issue 1 - p 99-100
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Clear cell carcinoma of the ovary, especially in an adolescent patient, is a rare disorder. This report describes one such patient currently being maintained on single-agent, third-line, intravenous chemotherapy, but who otherwise continues with her routine daily activities, including a normal social life and a busy schedule of school-related activities.


The patient initially sought treatment in April 1996, at the age of 12, with enlarged bilateral lymph nodes in the inguinal area. A pelvic examination was unremarkable. Computed tomography imaging revealed multiple areas of retroperitoneal adenopathy with a left inguinal adenopathy measuring 5 cm × 6 cm (Fig. 1). Plasma concentrations of α-fetoprotein and β-human chorionic gonadotropin were normal, but her CA-125 was 294 U/dl (normal: <35). An excisional biopsy of a left inguinal lymph node returned a pathologic diagnosis of metastatic clear cell carcinoma. The diagnosis was subsequently verified by an independent pathologist.

FIG. 1.
FIG. 1.:
Initial presentation (April 1996): Multiple areas of retroperitoneal adenopathy with a left inguinal adenopathy measuring 5 cm × 6 cm.

The patient was given four courses of bleomycin/etoposide/cisplatin chemotherapy (BEP). After four cycles, restaging revealed evidence of progressive disease, and second-line therapy was initiated with doxorubicin/paclitaxel (AT). There was further radiologically demonstrated progression after two cycles of AT (Fig. 2).

FIG. 2.
FIG. 2.:
Tumor progression after bleomycin/etoposide/cisplatin chemotherapy and doxorubicin/paclitaxel (August 1996), before chemotherapy with topotecan.

Topotecan, an agent that had recently been shown to be active in epithelial carcinoma of the ovary, 1–4 was selected for further therapy. A regimen of single-agent topotecan was started at a dose of 1.5 mg/m2/d intravenously for 5 days, originally every 3 weeks, subsequently every 4 weeks. There was no further tumor progression. Continuous cytokine support with filgrastim and epoetin alpha was also started; no platelet transfusions have been required.

To date, the patient has received 43 cycles of topotecan during 3 1/2 years. Her CA-125 has remained elevated but stable, and her most recent restaging reveals stability in her retroperitoneal masses and her inguinal adenopathy (Fig. 3); a second biopsy of her right inguinal node after 18 months of therapy again revealed clear cell carcinoma. There have been no nonhematologic toxic effects of the topotecan, and full therapeutic doses are still being administered. While receiving therapy, the patient has continued to attend school and has maintained a near-normal activity schedule. With puberty she has had normal and regular menses. She has gained weight, partially attributable to lower extremity edema, but largely because of normal growth and development.

FIG. 3.
FIG. 3.:
Arrest of tumor progression: stable disease (July 1999) after 42 cycles of topotecan.


The patient, now 16 years old, is 5';10″; tall and weighs 225 pounds. She is a sophomore in high school and aside from cyclic visits to the chemotherapy clinic, she leads a normal life. She travels, is an honor student, is on the school flag team, has a boyfriend, and counsels other young patients with cancer.

The patient is reassessed every 4 weeks. Clinical evaluation and imaging studies show little change in the disease. Her CA-125 is still elevated but without any upward trend. Although there are no data to support maintenance therapy with topotecan in stable clear cell ovarian carcinoma, this patient is opposed to discontinuation of therapy; she feels that topotecan is maintaining disease stability with minimal adverse effects.

Several recent reports have contributed to a growing body of evidence supporting the long-term use of topotecan in ovarian malignancy of epithelial histology. For example, the Gynecologic Oncology Group performed a phase II trial of topotecan in patients with recurrent platinum-sensitive epithelial ovarian cancer, reporting 2 complete and 13 partial remissions (overall response rate: 31.9%) in 47 evaluable patients. Stable disease was observed in 23 (48.9%) patients. 5 However, clear cell carcinoma of the ovary is a rare tumor without a defined optimal chemotherapeutic regimen. This report is the first describing the use of topotecan in this disease.


1. Creemers GJ, Bolis G, Gore M, et al. Topotecan, an active drug in the second-line treatment of epithelial ovarian cancer: results of a large European phase II study. J Clin Oncol 1996; 14: 3056–61.
2. Kudelka AP, Tresukosol D, Edwards CL, et al. Phase II study of intravenous topotecan as a 5-day infusion for refractory epithelial ovarian carcinoma. J Clin Oncol 1996; 14: 1552–7.
3. Gordon A, Bookman M, Malmstrom H, et al. Efficacy of topotecan in advanced epithelial ovarian cancer after failure of platinum and paclitaxel [Abstract 763]. Proc Am Soc Clin Oncol 1996; 15: 282.
4. Hochster H, Speyer J, Wadler S, et al. Phase II study of topotecan 21-day infusion in platinum-treated ovarian cancer: a highly active regimen [Abstract 775]. Proc Am Soc Clin Oncol 1996; 15: 285.
5. McGuire WP, Blessing JA, Bookman MA, et al. Topotecan (Hycamtin) has substantial antitumor activity as first-line salvage therapy in platinum-sensitive epithelial ovarian cancer: a Gynecologic Oncology Group study. J Clin Oncol 2000; 18: 1062–7.

Ovarian carcinoma; Clear cell histology; Topotecan

© 2001 Lippincott Williams & Wilkins, Inc.