In 1925, Sampson described the first case of malignant transformation of ovarian endometriosis, and defined three criteria for the diagnosis of malignant transformation of endometriosis: (1) Demonstration of both neoplastic tissue and endometriosis within the tumor, (2) histological appearance resembling endometrial stroma surrounding characteristic glands, and (3) no other primary tumor site.
However, a mass in the scar tissue with symptoms of cyclic pain synchronously with menses and a positive history of cesarean section may be nearly pathognomonic. Patient in this study met Sampson’s criteria.
Dissemination of endometrial tissue during a cesarean section or uterine surgery is biologically plausible because of the opportunity to inoculate the abdominal wall with endometrial cells from a hysterotomy.[7-9]
As shown in Table 1, the histological characteristics of malignant transformation in endometriosis of the abdominal wall are primarily represented by clear cell carcinoma. Thirty-five cases of Abdominal Wall Clear Cell Carcinoma have been recorded from 1986 to 2019(Table 1).In all cases, patients underwent a cesarean section. Abdominal wall endometriosis describes the involvement of ectopic endometrial tissues superficial to the peritoneum of the abdominal wall, including lesions secondary to a surgical incision and spontaneous lesions.[8, 10]The use of a tissue retrieval bag might help to prevent endometriosis implants.
In the case series (Table 1), the average age was 46.8 years(range37-60). The interval from cesarean section to the onset of abdominal wall endometriosis was 16.1 years(range1-37). The median duration of lower abdominal pain lasted for 10 years since cesarean section, and abdominal endometriosis may have already developed during that period. These patients, with surgical birth history (cesarean section) and pelvic endometriosis diagnosed for over 10 years, presented with progressive abdominal pain.
The large number of patients(72%) with the occurrence of symptoms, the advancement of tumors in our patients, metastases to lymph nodes, and one positive margin are probably connected with their short survival period. Patients with positive tumor margins and lymph nodes after tumor resection could be used as independent adverse prognostic factors.
In the present study, univariate analysis suggested that patients with tumors larger than 5 cm had a shorter survival time when compared with patients that had tumors smaller than 5 cm.
On the other hand, some cases were reported to experience relatively longer disease-free and overall survival (longer than 30 months) [11-16]; while recognizing that a more thorough molecular characterization could hopefully help define prognosis of this very rare condition, it has to be acknowledged that cases with better outcome presented with masses ranging between 4 and 9 cm, thus suggesting that a prompt recognition and treatment could make the difference.
In our case , abdominal CT can show a mass lesion involving the abdominal wall, a dilated small bowel loop with hyperenhancing and thickened wall, the involvement of the surrounding tissue. It is extremely difficult to detect early because of its deep location and lack of obvious clinical signs in its early stages. All patients underwent standardized preoperative assessment: routine blood tests and tumor markers CEA and CA19-9 determination, abdominal ultrasound (US), computed assisted tomography (CT) scan, and when needed, Magnetic Resonance Imaging or positron emission tomography (PET-CT).An abdominal CT scan revealed thickening of the abdominal wall and enlargement of the abdominal lymph nodes, but no evidence of distant metastasis in 2019.
Many epidemiologic studies supported a link between endometriosis and invasive epithelial ovarian cancer based on high prevalence and incidence of epithelial ovarian cancer and endometriosis. Unlike epithelial cells, endometriotic stromal cells are mutation free but contain widespread epigenetic defects that alter gene expression and induce a progesterone-resistant and intensely inflammatory environment, driven by estrogen via estrogen receptor-beta. The resulting increased estrogenic action in the stroma drives inflammation and sends paracrine signals to neighboring epithelial cells to enhance proliferation. Clear cell carcinoma of the endometrium is a rare type of endometrial cancer generally associated with an aggressive clinical behavior.
They are generally indolent, present in stage I (tumor confined to the ovary) and are characterized by specific mutations, including KRAS, BRAF, ERBB2, CTNNB1, PTEN PIK3CA, ARID1A, and PPPR1A, which target specific cell signaling pathways. Due to the extremely low incidence of clear cell carcinoma, it is unlikely that randomized clinical trials can be feasible to establish the standard treatment of this disease. Even though an additional abdominal wall tumor resection was performed after 1.5 year from the initial operation, there are still remaining local tumor lesions and they are gradually enlarging in size on a serial follow up CT. She received chemotherapy, including platinum-based regimens as adjuvant therapy, or at the time of disease progression (Table 1).The patient underwent four courses of tri-weekly chemotherapy with carboplatin and paclitaxel.
In our case, the clear cell carcinoma showed a very aggressive behavior with rapid local recurrence from which the patient survived only 24 months after diagnosis. We present a case of clear cell carcinoma directly arising from scar endometriosis after a cesarean section and review all 35 cases reported. The treating physician should keep in mind abdominal wall endometriosis as a possible cause of post cesarean section scar-related masses. Treatment of cancer patients with conventional therapies (chemotherapy, hormonal therapy, radiation and Targeted therapy) respond initially well and experience prolonged tumor-free survival. After targeted therapy and radiotherapy, the tumor appeared to shrink significantly. Better understanding of the molecular mechanisms of tumor angiogenesis, especially in clear cell carcinoma, has led to the development of multiple targeted therapies to inhibit key effectors: vascular endothelial growth factor (VEGF), VEGF receptor.