Chien S.-C.BOR-CHING SHEUWEN-CHUN CHANGMU-ZON WUHuang S.-C.2020-07-032020-07-0320050001-6349https://scholars.lib.ntu.edu.tw/handle/123456789/508316Ovarian squamous cell carcinoma (SCC) is extremely rare. Very few such cases occur purely from within the ovary itself, while secondary ovarian SCC often comes from the genital organs (mostly the cervix) (1). To date, there has been no effective treatment protocol for dealing with primary ovarian SCC. Here we present a case of pure primary ovarian SCC with FIGO (International Federation of Gynecology and Obstetrics) stage IV, grade III. We believe that this is the first case with a poor outcome in our hospital after optimal debulking surgery and adjuvant chemotherapy. Literature related to this disease is reviewed. A 63-year-old female, gravida 5, para 5, presented with a 2-"week history of progressive low abdominal pain and severe right flank soreness. She had not undergone any pap smears previously and only tubal ligation 30 years ago. She was transferred to our hospital due to a pelvic mass with suspicion of malignancy at a local clinic. Physical examination revealed a low abdominal mass immediately below the umbilicus and enlarged lymph nodes in the left supraclavicular area. Normal appearances of the vulva, vagina and cervix were noted upon pelvic examination. A sonographic examination revealed a huge hypoechoic cystic mass with a solid'component. The serum levels of tumor markers were examined, including cancer antigen 125 (CA 125) (67 µ/mL), carcino-embryonic antigen (CEA) (2.29 ng/mL), tumor "marker of squamous-cell carcinoma (SCC) (6.7 ng/mL) and alpha-fetoprotein (AFP) (< 20 ng/mL). Abdominal and pelvic computed tomography revealed a right ovarian cystic tumor associated with papillary growth, while para-aortic and inguinal lymphadenopathy were also demonstrated. Following colon preparation, exploratory laparotomy was performed. At laparotomy, a small amount of yellowish ascites was noted. The ovarian tumor appeared to be tightly adhered to the cecum, measuring about 15 × 10 × 8 cm in size. The mass ruptured during separation from the cecum, and some white to yellowish, thick and dirty-looking contents leaked out. Multiple whitish and firm abdominal implants were found distributed over the peritoneum, omentum, small intestine and bladder base, mostly of a size less than 1 × 1 cm. Optimal debulking surgery with total abdominal hysterectomy, bilateral salpingo-oophorectomy, tumor resection, bilateral pelvic lymph-node dissection and omentectomy were performed. Grossly, the right ovary, weighing 350 g, revealed a "unilocular tumor containing a solid component. Micro"scopically, it showed a high-grade SCC with keratin pearls, and both spindle-shaped and bizarre giant cells. The cyst wall exhibited a picture of epithelial pseudo-stratification, with a transition from squamous metaplasia to squamous dysplasia and carcinoma in situ (Fig. 1). There was no "evidence of endometriosis or teratomatous components. No pathological lesions were found in the cervix and endocervix and washing cytology proved negative. In addition, per"cutaneous needle aspiration of the lymph node from the left neck revealed metastatic poorly differentiated SCC. (a) The tumor cells display a transition from single columnar epithelium, which is the original epithelial lining of the unilocular cyst (hematoxylin–eosin stain; original magnification × 100). (b) The cystic wall exhibits epithelial pseudostratification, squamous metaplasia, dysplasia of squamous epithelium and evidence of a squamous cell carcinoma in situ (hematoxylin–eosin stain; original magnification × 100). Computed tomography of the patient's chest, head and neck and a clinical nasal and oral pharyngeal examination were arranged postoperatively to exclude any possibility of the tumor deriving from other origins. They disclosed only multiple heterogeneous lymphadenopathy at the mediastinum and the neck. No visible lung and nasopharyngeal lesions were found. In addition, this patient exhibited no symptoms from gastrointestinal or urinary tract organs. Under the impression of a pure primary ovarian SCC with FIGO stage IV, grade III, the patient underwent adjuvant chemotherapy with paclitaxel (212 mg) and cisplatin (117 mg) at 3-week intervals for six courses. The serum level of tumor marker SCC returned to normal range immediately following the first course of chemotherapy. After completion of her chemotherapy regimens, lymph nodes in the left of the neck appeared to have dramatically decreased in size. However, at the time of follow-up, the patient presented with rapid progression of the disease 7 months subsequent to surgery and she then died. From the literature, it would appear that ovarian SCC is a rare entity. Malignant SCC comprises a variety of ovarian tumors, including nonmetastatic and metastatic diseases. Primary ovarian SCC is mostly associated with mature cystic teratoma, Brenner's tumor, mucinous cystadenoma and endometriosis (2). Statistically, almost all cases of ovarian SCC appear to have developed from a mature cystic teratoma. The incidence of malignant transformation of a mature cystic teratoma is about 2% (3). Among the tumors metastatic to the ovary, only 2.5% are of the squamous cell type and the most common metastatic SCC originates from the cervix by direct extension (1). Pure primary ovarian SCC appears to be extremely rare, and only a few cases of advance-staged pure primary SCC have been illustrated in previous reports (Table I) (2, 4–7). Patients suffering from primary ovarian SCC in its early stages may remain disease free for a period of time following complete tumor reduction (7), although patients presenting with the more advanced stages of the disease may face a poorer outcome even after postoperative chemotherapy and/or radiotherapy. The stage of the tumor may correlate most satisfactorily with overall patient survival for the pure form of this neoplasm. Because of the tumor's rare nature, there appears to be no effective supplemental chemotherapy or radiotherapy regimens available. Paclitaxel has been useful to treat advanced ovarian epithelial carcinoma but no clinical data appear to be available for the treatment of pure primary ovarian SCC. A good response to postoperative chemotherapy using "cisplatin and paclitaxel for primary ovarian SCC associated with endometriosis has been reported (8). In our case, a dramatic shrinkage of the metastatic lymph nodes, a decrease in serum levels of tumor marker SCC and no evidence of tumor recurrence were detected initially after optimal debulking surgery and adjuvant chemotherapy with cisplatin and paclitaxel. Serum SCC antigen levels have been useful in the prognosis and management of cervical SCC (9), but there is no evidence about the utility of the marker in pure primary ovarian SCC so far. Our patient still'finally died of the rapidly progressive disease in the seventh month after the operation. The chemotherapy "regimens or their dosages may be ineffective for this malignant cell type of the ovary and more clinical investigations are needed.[SDGs]SDG2[SDGs]SDG3cisplatin; paclitaxel; abdominal hysterectomy; abdominal pain; adult; cancer adjuvant therapy; cancer diagnosis; case report; computer assisted tomography; female; human; human tissue; laparotomy; lymph node dissection; mortality; ovary carcinoma; postoperative care; priority journal; review; salpingooophorectomy; squamous cell carcinoma; treatment outcome; Aged; Carcinoma, Squamous Cell; Fatal Outcome; Female; Humans; Ovarian Neoplasmsreview10.1111/j.0001-6349.2005.0383d.x159548862-s2.0-21444454747