Squamous cell carcinoma arising in mature cystic teratoma of the ovary: A case series and review of the literature

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Abstract

Objectives.

Malignant transformation of mature cystic teratomas is rare, with squamous cell carcinoma being the most common type. The prognosis is generally poor when disease has spread beyond the ovary. We conducted this study to review our experience with this disease and describe our current treatment modality.

Methods.

During a 22-year period (1983–2005), we identified 17 women treated for squamous cell carcinoma arising in a mature cystic teratoma of the ovary. All pathologic diagnoses were confirmed at our institution. A retrospective chart review and comprehensive review of the literature were conducted.

Results.

The median age was 55 (mean, 54.8; range, 37–75). Eight cases were stage I, 5 were stage II, and 4 were stage III. Mean tumor size was 14.2 cm. All patients underwent surgery, with positive lymph nodes noted in 0 of 10 cases that included lymph node dissection. Ten patients received adjuvant treatment—6 with chemotherapy and 4 with chemoradiation. Six patients had recurrent disease in the pelvis after adjuvant treatment. Four patients died of disease. The overall 1-year survival rate was 60%. The 4 patients with stages IA–IIB disease treated with adjuvant platinum-based chemotherapy and radiation survived at 12–56 months' follow-up.

Conclusions.

Squamous carcinomas arising in mature cystic teratomas are commonly large ovarian tumors that occur in perimenopausal women and often present as an incidental pathologic finding. While the prognosis seems highly dependent on surgical stage, there is a lack of consensus in the literature regarding adjuvant treatment. Platinum-based chemotherapy with pelvic radiation may be a reasonable adjuvant therapy for early-stage disease.

Introduction

Mature cystic teratoma (MCT) is the most common germ cell tumor of the ovary and accounts for 10–20% of all ovarian tumors. Malignant transformation occurs in 1–2% of MCTs, and the majority of these (80–90%) are squamous cell carcinomas (SCC). Compared to benign MCT, malignant transformation occurs in an older patient population, with a mean age range of 45–60 [1]. The clinical presentation is similar to other ovarian tumors and includes abdominal pain and distension secondary to a pelvic mass, but it may also present with bowel or bladder symptoms in cases of locally advanced disease. In early-stage disease, the diagnosis is frequently made unexpectedly in the operating room or on final pathology, which presents a dilemma regarding the need for surgical staging and adjuvant therapy. The prognosis is generally poor when disease has spread beyond the ovary. Because of the relative rarity of these tumors, the literature is limited to descriptive case series, and there is no consensus regarding optimal management. We conducted this study to review our experience with this disease and describe our current treatment modality.

Section snippets

Materials and methods

During a 22-year period (1983–2005), we identified 17 women treated for squamous cell carcinoma arising in an MCT of the ovary. All pathologic diagnoses were confirmed at our institution. A retrospective chart review was conducted, with information obtained from operative reports, medical oncology and radiation oncology records, and pathology reports. The date of last known follow-up was used to calculate overall survival. A comprehensive literature review was performed. Case series describing

Results

The median age was 55 (mean, 54.8; range, 37–75). Eight cases were stage I, 5 were stage II, and 4 were stage III. Mean tumor size was 14.2 cm (range, 4–28 cm) (Fig. 1, Fig. 2). All patients underwent surgery, and 10 (59%) of 17 patients underwent lymph node sampling, with positive nodes noted in none at initial surgery. Six patients (35%) were treated with adjuvant chemotherapy and four (23%) with chemoradiation. Six of these ten patients had recurrent disease in the pelvis after adjuvant

Discussion

Malignant transformation of MCT, occurring with an average frequency of 1–2%, has been reported in isolated case reports and small case series. The tumor may arise from any of three germ cell layers present in the teratoma and has been observed adjacent to both normal and metaplastic cells [4], [5]. Squamous cell carcinoma is the most common transformation, although adenocarcinoma, adenosquamous carcinoma, sarcoma, carcinoid, and melanoma have also been reported, albeit rarely. Due to the

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