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Menopause Review/Przegląd Menopauzalny
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vol. 13
Case report

Orbital metastasis of endocervical stromal sarcoma: a rare tumor and an uncommon metastasis

Mehmet Metin Seker
Ali Ugur Uslu
Hatice Ozer
Ayse Seker
Turgut Kacan
Nalan Babacan
Handan Aker
Sahande Elagoz

Prz Menopauzalny 2014; 13(6):356-358
Online publish date: 2014/12/30
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Sarcomas constitute less than 1% of all uterine and cervical malignancies. Endocervical stromal sarcoma (ECSS) is a very rare uterine sarcoma [1]. To date there are only 17 patients in the literature. The most common presentation is pelvic mass and vaginal bleeding. The mainstay of the treatment is surgery. Although adjuvant radiation therapy and chemotherapy have been applied to some patients, the survival benefit of radiation therapy and chemotherapy in the adjuvant setting is unclear. So there is no consensus on the adjuvant treatment [2, 3]. Relapses are usually in the pelvic and abdominal regions. To a lesser extent, lung, liver and bone metastases may be seen [4]. The choice of treatment in local recurrences is surgery or radiation therapy, but systemic chemotherapy is preferred in the presence of distant metastases. To date, orbital metastasis due to ECSS has not been presented.

Case report

A 46-year-old female patient was admitted one year ago to the hospital with vaginal bleeding. During gynecologic examination, an endocervical polyp was detected and excised. After pathologic examination, the diagnosis was mesenchymal stromal polyps. After 4 months, she was admitted to the hospital with vaginal bleeding again. Thus, a total abdominal hysterectomy-bilateral salpingo oophorectomy (TAH-BSO) was performed. The diagnosis was mesodermal stromal polyp, chronic cervicitis and leiomyoma after pathologic examination (Fig. 1A). Six months after TAH-BSO, she was admitted to hospital with cough and hemoptysis. On chest computed tomography, a 61 mm × 54 mm lobulated contoured mass lesion was obliterating the lower lobe bronchus and surrounding the descending aorta; a biopsy was performed. The diagnosis was low-grade malign mesenchymal tumor with pathologic examination. One week after biopsy, loss of vision in the left eye developed. Orbital magnetic resonance imaging was performed, and a 30 mm × 20 mm × 30 mm lesion was detected involving the orbital apex and posterior cavernous sinus; it was infiltrating the neural foramen and extending to the sphenoid sinus and ethmoid cells in the anteromedial (Fig. 2).
After detecting a malignant mesenchymal tumor in the patient’s lung biopsy, the patient’s endometrial and cervical tissue specimens were reexamined; the tumor comprised slightly to moderately pleomorphic stellate and spindle cells within an edematous or myxoid background (Fig. 1B). Alternating hypercellular and hypocellular regions were noted. In some areas, there was high mitotic activity. Proliferating hyalinized-walled blood vessels, myxoid background and hypercellularity were more pronounced, especially in the lung tumor tissue. Thus, we diagnosed ECSS. We performed a mammography and thorax, abdomen and cranial imaging to rule out other malignancies that may cause lung and orbital metastasis.
Because of the rapid progression, we preferred systemic chemotherapy as an initial treatment (ifosfamide 2500 mg/m2, d1-3; mesna 2500 mg/m2, d1-3; doxorubicin 60 mg/m2, d1; q3 weeks). After three cycles of chemotherapy, our patient received orbital palliative radiotherapy. We had a partial response. Both the thoracic and orbital tumors regressed, and visual activity improved, even if it was blurred.


The vast majority of cervical malignancies are squamous cell carcinomas. Endocervical stromal sarcoma accounts for less than 0.5% of cervical malignancies. The most common presentations are vaginal bleeding, pelvic mass, urinary urgency and constipation. Enlargement of the uterus or cervix can be detected with pelvic examination [5, 6]. However, a differential diagnosis distinguishing between a benign lesion and sarcoma is not possible through history and physical examination. The definitive diagnosis is made by pathological examination. Without a specific pathological finding, it is not easy to diagnose ECSS. In our patient, the diagnosis of ECSS was made by reassessment of pathological specimens after the development of lung metastases. There was no specific sign except for poor focal staining with CD10 in the stroma in the lung and the endocervical tumor. Because of the insignificance of malignancy criteria in the endocervical tumor, the lung metastasis and reassessment of histomorphological signs led to a diagnosis of ECSS.
The prognosis correlates with stage, grade, surgical margins and number of mitoses in cervical and uterine sarcomas. Vascular and lymphatic invasion are negative prognostic factors [7]. The choice of treatment in nonmetastatic patient is surgery. Unfortunately, the relapse rate is very high with surgery alone [8]. Due to a small number of cases, there is no survival information or consensus about adjuvant treatment of ECSS. In the study of Reed et al., local recurrence was less frequent in patients who received adjuvant radiotherapy [9]. But there was no disease-free (DFS) or overall survival (OS) difference. Khosla et al. treated a stage IB2 ECSS patient with surgery followed by radiotherapy and chemotherapy; a stage IIIB patient was treated with primary radiotherapy and chemotherapy [10]. Both patients were disease free at follow-up. The choice of treatment in recurrent and metastatic disease is chemotherapy. The most commonly used agents are ifosfamide, doxorubicin, cisplatin, carboplatin and paclitaxel. We also treated our patient with ifosfamide and doxorubicin and achieved partial remission.
To the best of our knowledge, to date there are 17 ECSS cases in the literature. Our patient is the 18th case and the first ECSS patient to develop orbital metastasis. Based on our experience in this case, we may say that although surgery is the mainstay treatment for nonmetastatic ECSS patients, ifosfamide and doxorubicin are active chemotherapeutic agents for ECSS. Radiotherapy may also be effective in symptomatic treatment of these patients.


Authors report no conflict of interest.


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