Pure Primary Squamous Cell Carcinoma of the Ovary: A Report of Two Cases and Review of the Literature

Abstract

Pure primary squamous cell carcinoma (SCCa) of the ovary not associated with pre-existing ovarian lesions including dermoid cysts, Brenner tumors, or endometriosis is extremely rare. The last 2 types of SCCa and pure primary SCCa have been classified by the World Health Organization criteria as surface epithelial-stromal tumors. On account of the rarity of pure primary SCCa of the ovary, the clinical features and effective treatments have not been established. This study describes 2 such cases that we recently encountered in a review of the literature. The literature shows that patients with advanced-stage tumors have very poor survival outcomes, despite a combination of surgery, radiation therapy, and chemotherapy.

Squamous Lesions of the Ovary

“…Young et al 10 have identified epidermoid cysts, some in concert with small Walthard rests, but in the absence of any other teratomatous features. Other case reports and small series [11][12][13][14][15][16][17][18][19] have identified de novo squamous cell carcinoma of the ovary, some associated with endometriosis. By all accounts, these entities are exceedingly rare, and questions remain about their histogenesis.…”

“…[11][12][13][14][15][16] In their case series, Pins et al 9 noted that squamous cell carcinoma (arising in association with a dermoid, with endometriosis or de novo endometriosis) usually presents at 52 years; it would seem, therefore, that nonmetastatic squamous cell carcinoma of the ovary is a disease of perimenopausal women, again in contradistinction to a number of other aggressive ovarian neoplasms. A review of reported cases, [6][7][8][9][10][11][12][13][14][15][16][17][18][19] furthermore, suggests that most cases of primary squamous cell carcinoma of the ovary present at advanced stages.…”

“…[6][7][8][9][10][11][12][13][14][15][16][17][18][19] Neither primary squamous cell carcinoma nor squamous cell carcinoma associated with dermoid cysts appears to be associated with significant ascites, in contradistinction to a number of other aggressive gynecologic neoplasms. [6][7][8][9][10][11][12][13][14][15][16][17][18][19] Other symptoms may relate to the invasion of the tumor into neighboring structures (eg, the urinary system and other gynecologic organs) and to distant sites (eg, liver). [11][12][13][14][15][16] In their case series, Pins et al 9 noted that squamous cell carcinoma (arising in association with a dermoid, with endometriosis or de novo endometriosis) usually presents at 52 years; it would seem, therefore, that nonmetastatic squamous cell carcinoma of the ovary is a disease of perimenopausal women, again in contradistinction to a number of other aggressive ovarian neoplasms.…”

“…[6][7][8][9][10][11][12][13][14][15][16][17][18][19] Cyst contents may be sebaceous, and areas of necrosis may also be visible. [6][7][8][9][10][11][12][13][14][15][16][17][18][19] Some reports 11 note adhesion of such lesions to surrounding structures. Also, primary squamous cell carcinoma arising in endometriosis may show endometriotic cysts on gross examination.…”

“…Areas of necrosis are often visible, and there are often adhesions to surrounding pelvic structures. [6][7][8][9][10][11][12][13][14][15][16][17][18][19] Frozen sections are often requested at the time of surgery for intraoperative and postoperative planning. In cases of clinically suspicious malignant tumors, a diagnosis of squamous cell carcinoma can be made if all the required histologic features are present.…”