Year : 2022 | Volume
: 18 | Issue : 3 | Page : 777--779
Ovarian squamous cell carcinoma
Paulina Reyes Tobar, Roberto Altamirano Assad, Omar Nazzal Nazal, Eugenio R Suárez
Department of Gynecologic Oncology, University of Chile, San Borja Arriarán Hospital, Santiago, Chile
Paulina Reyes Tobar
Santa Rosa 1234, Santiago of Chile
Ovarian squamous cell carcinoma is a rare and aggressive ovarian neoplasm, where most of the cases arise from mature teratoma, which represents up to 20% of all ovarian tumors. Moreover, primary squamous cell carcinoma is considered to be related to human papillomavirus infection. The diagnosis is difficult and metastasis from bladder, genital tract, lung, and digestive tract cancers must be ruled out. There are no guidelines for treatment, however, radical surgery combined with adjuvant chemotherapy and radiation therapy, are the most common approach. We present the case of a young women diagnosed with a de novo ovarian squamous cell carcinoma, as well as the treatment and follow-up that she received.
|How to cite this article:|
Tobar PR, Assad RA, Nazal ON, Suárez ER. Ovarian squamous cell carcinoma.J Can Res Ther 2022;18:777-779
|How to cite this URL:|
Tobar PR, Assad RA, Nazal ON, Suárez ER. Ovarian squamous cell carcinoma. J Can Res Ther [serial online] 2022 [cited 2022 Oct 5 ];18:777-779
Available from: https://www.cancerjournal.net/text.asp?2022/18/3/777/348229
Ovarian squamous cell carcinoma is a rare entity that represents <1% of ovarian primary tumors. No more than 36 cases of primary ovarian squamous cell carcinoma have been described to date. The majority of squamous cell carcinomas are derived from the malignant transformation of mature teratoma, endometriosis or less frequently, Brenner tumors. Moreover, and even less common, is “de novo” or primary squamous cell carcinoma.
It normally affects women between 27 and 90 years of age (average 52.9 years of age) provoking symptoms related to tumor growth or metastasis. The diagnosis is difficult since the majority of cases are detected by biopsy in the cases where surgical removal of a mature teratoma is required. However, some clinical and laboratory markers can be useful to detect the presence of a suspected squamous cell carcinoma.
At present there are no protocols for the management of ovarian squamous cell carcinoma. Indeed, most scientific publications agree that radical surgery and platinum-based chemotherapy as an adjuvant treatment improve the outcome. Despite this, it remains as an aggressive tumor with a 5-year survival rate of 15%–52%.
Here, we present a case of an ovarian squamous cell carcinoma, diagnosed as a finding in an emergency surgery secondary to acute abdomen in a young female patient.
The patient was 28 years old with no prior diseases or substantial surgical background, nulliparous, sexually active, and user of oral contraceptives. PAP smear in January 2019 was negative. In addition, she had not received an human papillomavirus (HPV) vaccination. She had no relevant known family diseases.
An exploratory laparoscopy was performed in a private health center in Santiago de Chile on December 3, 2018, due to an acute abdomen that was suspected secondary to a twisted left teratoma found in the emergency echography carried out at her arrival. In the surgical protocol was described a left ovarian five centimeters cyst, of smooth surface with no other lesions visible at the right ovary, uterus surface, neither on the omentum, peritoneum nor liver surface. A left ovarian cystectomy was performed, and the subsequent differed biopsy (1st biopsy) showed a malignant solid neoplasia, suggesting of probable urothelial carcinoma. After this, a second surgery was performed, the protocol described the normal aspect of both adnexa and no other lesions, however and because the high suspicion of an ovarian origin of the carcinoma, a left salpingo-oophorectomy was performed, the biopsy (2nd) concluded a malignant epithelial neoplasia with partial squamous differentiation.
A positron emission tomography scan (PET-CT) was carried out on January 21, 2019, showing peritoneal carcinomatosis and hypermetabolic left paraaortic lymph nodes. Moreover, a HPV typification was performed which resulted negative.
For insurance reasons, the patient was transferred to another private health center. A review of the sample obtained in the second surgery (2nd biopsy) was requested, resulting positive to p40 and negative to uroplakine, Ck7, CK20, Wt-1 and Ca 125, concluding “ovarian tissue infiltrated by moderate differentiated squamous cell carcinoma.” Serum markers were requested: Quantitative Beta- human chorionic gonadotropin, CA125 and Alpha-fetoprotein, which all resulted negative.
Suspecting a primary ovarian neoplasm, on March 8, the oncology committee after a review of the clinical findings as well as a new physical exam-that did not found suspicions lesions in the genital tract nor at the digital rectal examination-decided on surgical staging for ovarian carcinoma, which was performed on March 18 through supra and infraumbilical laparotomy. Neoplastic implants on the uterine surface, right ovary, peritoneum and omentum, as well as a six centimeters sigmoid implant, and a similar one found in the left paraaortic region were described in the surgical protocol. There were no lesions described on hepatic or gastric surfaces, or on the spleen or retroperitoneum. A total hysterectomy and right salpingo-oophorectomy was performed, along with a partial omentectomy, paraaortic, and inferior mesenteric lymphadenectomy, removal of peritoneum implants and proctosigmoidectomy with colostomy, obtaining zero residual mass.
The patient evolved well clinically. The definitive biopsy of the surgical staging and debulking concluded: Moderate differentiated espinocelular carcinoma implants in all areas with exception of the cervix, and four out of five paraaortic lymph nodes were positive for infiltration. During a new oncology committee meeting on April 12, platinum-based chemotherapy was indicated. The patient completed three cycles (carboplatin–paclitaxel 670 plus paclitaxel 293 mg) with regular tolerance. During this treatment, she presented with left internal iliac thrombosis requiring anticoagulant management. An imaging control was required, which showed a three millimeter lung nodule, along with pathological lymph nodes in the left paraaortic region, mesorrectal and internal iliac territory, as well as solid images in the abdominal wall, peritoneum, spleen and liver parenchyma. Because of all these findings, it was considered to be a platinum-resistant ovarian squamous cell carcinoma and in July a second line chemotherapy treatment with gemcitabine (990 mg) was indicated. For clinical progression after two cycles of gemcitabine, on October 4, the oncology committee prescribed a third line chemotherapy treatment with liposomal doxurribicine (64 mg). She received two cycles that month, however, she presented a new iliac thrombosis that required a cave vein filter. Finally, she died on March 5 of sepsis of unknown origin.
We presented the case of young women, diagnosed with an ovarian squamous cell carcinoma after an emergency surgery, she received debulking surgery up to zero residual mass, and chemotherapy up to third line, however, she finally deceased 15 months after the first surgery.
The international literature agrees that ovarian squamous cell carcinoma is a rare and aggressive tumor. Regarding its origin, it is considered that 80% of cases are derived from mature teratoma, and less frequently from endometriosis foci or Brenner tumors.
Despite mature teratoma represents 20% of ovarian tumors, the malignant transformation occurs in no more than up to 5.5% of cases and up to 75% of these cases are squamous cell carcinomas. This malignancy phenomenon occurs more often in post-menopausal women. The a priori diagnosis of malignancy of a mature teratoma is difficult, however, the literature suggests that cists over 10 centimeters diagnosed in women above 40 years of age, with high squamous cell antigen, have a sensitivity of 77% and specificity of 96% for malignancy prediction.
Regarding squamous cell carcinoma deriving from endometriosis, is has been observed that it mostly arises from ovary and vaginal septum foci. The literature agrees on its aggressiveness with a mortality rate of up to 80% in 6 months.
The primary squamous cell carcinoma is considered to be related to HPV infection, since between 36 and 52% of patients are HPV positive. One of the theories suggests that HPV infection acts as an oncologic stimulus, whereas other theories suggest that there is contiguous metastasis or microinvasive angioinvasion when cervix intraepithelial neoplasias are present.
One of the challenges is the differential diagnosis between a primary squamous cell carcinoma and metastasis. In this case, this was a healthy woman with no prior diagnosis of an ovarian cyst up to the emergency surgery, where only a left ovarian cyst was described, and in the second one, there were no findings. The Immunohistochemistry was compatible with a squamous cell carcinoma. In the positron emission tomography-computed tomography no other implants where observed, but the peritoneal carcinomatosis. Moreover, at the debulking surgery biopsy, the cervix was no infiltrated by neoplasia. The clinical examination performed by the members of the oncology committee did not found any other lesions that could represent an eventual metastasis origin.
The treatment of squamous cell carcinoma in over 75% of the cases includes: Total hysterectomy, bilateral adnexectomy, with or without omentectomy. Some series showed a tendency to improve in the outcomes when lymphadenectomy was performed. In patients with stage IB tumors and beyond, platinum-based chemotherapy combined with paclitaxel or gemcitabine could improve survival. Because squamous tumors are considered to be radiosensitive, pelvic radiotherapy could be added. Despite this, the survival at stages II and beyond is no more than 34% at 5 years, considering that surgical staging and residual mass after surgery are the most relevant predictors.
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Conflicts of interest
There are no conflicts of interest.
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