Abstract
Background/Aim: Clear cell carcinoma of the abdominal wall is a sporadic event. To date, about thirty cases have been reported in the literature. This article provides a case report and literature review of an infrequent occurrence with poor prognosis. Case Report: A 45-year-old woman with pelvic pain and an abdominal mass came to our attention. Her medical history was notable for two previous cesarean sections. Physical examination revealed a smooth, multilocular mass measuring about 20 cm, arising from the previous surgical scar. Histology revealed clear-cell carcinoma resulting from the transformation of abdominal wall endometriosis. Given the disease extent, the patient underwent front-line chemotherapy. After several and multiple chemotherapy regimens, there was a disease progression that resulted in the death of the patient in 7 months. The literature review showed that a previous cesarean section was present in 91% of cases. Besides, approximately 26.5% of women died within 12 months of being diagnosed. The mean age of women was 45.88 years, while the average size of the lesion was 11 cm. Conclusion: Clear cell carcinoma is a rare but occurring event. Middle-aged women showing an abdominal wall mass in close relation with a surgical scar from a previous cesarean section must be promptly investigated. Treatment options usually include surgery and chemotherapy with poor results.
- Clear cell carcinoma
- abdominal wall endometriosis
- cesarean scar endometriosis
- endometriosis
- surgery
- chemotherapy
Endometriosis is defined as an inflammatory disease characterized by the presence of a functioning endometrial gland and stroma outside the uterus (1). These lesions usually involve the ovaries and, more rarely, bowel, ureters, lung, and abdominal wall (1). In women with abdominal wall endometriosis (AWE), a prior history of gynecological surgery with the opening of the uterine cavity is usually found. In this regard, scar endometriosis can be explained by iatrogenic transplantation of endometrial tissue to the wound edge during the surgical procedure (2, 3).
The incidence of abdominal surgical scar endometriosis ranges between 0.03% and 1.08% of women undergoing pelvic surgery (4, 5). It is often misdiagnosed and detected preoperatively in 20%-50% of cases (2). Women typically report a cyclic menstrual pain referred to the abdominal wall. The differential diagnosis of a subcutaneous mass associated with a previous surgical incision of the abdominal wall includes abscess, hematoma, hernia, desmoid tumor, sarcoma, and metastatic disease (6). An accurate medical history and physical examination can guide towards diagnosis. Definitive treatment includes wide local excision with free margins. Hormonal therapies relieve symptoms but do not prevent recurrences (6).
Although endometriosis is considered a benign condition, malignant transformation is rare but possible. About 80% of endometriosis-associated malignancies have been found in the ovary, whereas 20% are localized in extra-gonadal sites (7). Clear cell histology represents 4.5% of the extra-pelvic endometriosis-associated malignancies. It represents the most common histotype in the case of abdominal wall localization (8).
Computed tomography scan showing intra-abdominal (A), and parietal (B) disease extension after second-line chemotherapy (red arrow).
Published literature on this topic is weak. Here we report the case of a patient presenting with clear cell carcinoma (CCC) of the abdominal wall at the site of the previous cesarean section scar. Furthermore, we included a review of the literature regarding this unusual occurrence.
Case Report
A 45-year-old woman came to our attention for pelvic pain and an abdominal mass present for three months. She noticed a remarkable dimensional increase of the mass in the last two weeks. Her personal history showed two previous Caesarean sections, 22 and 13 years before. No other relevant medical problems were reported. She complained of intense dysmenorrhea on the first day of menstruation, along with pain in the abdominal wall and deep in the uterine scar of cesarean section. She had no family history of gynecological malignancies and had never taken hormone therapy.
Physical examination revealed a smooth, multilocular mass measuring about 20 cm, arising from the middle-left side of the cesarean scar to the umbilical region. Abdomino-pelvic magnetic resonance imaging (MRI) and computed tomography (CT) scan revealed the presence of an anterior abdominal wall mass showing solid and lacunar areas with a diameter of 18 cm, infiltrating bladder wall and multiple iliac lymph nodes. A metastatic pararectal mass of 8.5 cm, multiple liver nodules, and bilateral pulmonary metastases were also found. Uterus and adnexa appeared disease-free. An ultrasound-guided fine-needle aspiration biopsy and core-needle biopsy were performed. Histopathological examination revealed CCC cells deriving from gynecological cancer, suggesting a malignant transformation of AWE.
Given the disease extent, the patient underwent front-line chemotherapy with carboplatin (AUC=2) and paclitaxel (80 mg/m2) weekly. Two months after the first-line chemotherapy, a new MRI documented partial regression of lung metastases, but also revealed an increase of the anterior abdominal wall mass. Furthermore, pelvic skeletal metastases were revealed. Because of bone lesions, the patient had significant pelvic pain requiring external beam radiation therapy (single dose of 800 cGy) at the level of iliac bone metastasis.
Due to progressive disease, second-line chemotherapy with gemcitabine was administered (1000 mg/m2 days 1-8 weekly). After three cycles of gemcitabine, a thoracic/abdominal CT scan revealed a significant progression of abdominopelvic masses (26 cm maximum diameter) and a slight regression of pulmonary metastasis. Figure 1 shows a CT scan revealing a wide intra-abdominal and parietal extension of the lesion. Third-line chemotherapy with Pegylated liposomal doxorubicin 40 mg/m2 every 28 days was started. Still, after three weeks from the first administration, the patient was hospitalized in the Internal Medicine Clinic due to severe cachexia. The patient received palliative care and died a few days later, exactly 7 months after the cancer diagnosis.
Summary of cases of clear cell carcinomas arising from abdominal wall scar.
Discussion
CCC of the abdominal wall is a rare and aggressive carcinoma that is often diagnosed as a metastatic and unresectable disease (9). The majority of cases occur in surgical scar endometriosis after the cesarean section (9). Despite its rarity, an increased number of reported cases have been noted in the last few years (10). This might be due to the increased cesarean section and conservative uterine surgery rates that have been documented worldwide (10).
The diagnostic criteria for the malignant transformation of AWE are not well defined. In 1925, Sampson first described the malignant transformation of ectopic endometrial tissue and proposed three rules for the diagnosis of this process: i) the endometriosis is closely associated with the neoplasm, ii) histology is compatible with the endometrial origin, and iii) no other primary tumor sites are found (11). However, in Mostoufizadeh's opinion, the simple coexistence of a neoplasm and endometriotic tissue is sufficient to demonstrate the endometriotic origin of the lesion (12). Even if the presence of endometriotic tissue is pathognomonic of the disease, according to the literature, the transition zone is detected in only 36-42% of cases (13).
In our tissue sampling, we did not find endometrial tissue. The absence of endometriosis foci could be explained by a sampling error or a complete replacement of healthy endometrial with massive neoplastic proliferation (10). In this context, an accurate medical history collection becomes crucial. In the present case, a disease linked to ectopic endometrial implants was suspected because the woman's clinical history was characterized by two cesarean sections, highly painful menstrual cycles, and abdominal wall cyclic pain during menses.
In Table I, all reported cases of clear cell carcinoma arising from abdominal wall scar with different treatment methods and follow-up are summarized (6, 10, 14-43). The literature review showed that a previous cesarean section was present in 91% of cases. The mean follow-up was approximately 1 year. Also, approximately 26.5% of women died within 12 months of being diagnosed. The mean age of women was 45.88 years, while the average size of the lesion was 11 cm. Histology revealed the presence of endometriotic tissue in over 60% of cases.
When feasible, surgical treatment is the gold standard and definitive therapeutic approach in most cases (10). Usually, both ovaries are removed in more than 80% of cases, and an endometrial biopsy is performed in about 10% of women (10). Depending on the size of the parietal wall excision, a mesh is often needed (10). According to the literature (Table I), surgical treatment was always performed: an extensive surgery with hysterectomy or salpingo-oophorectomy was conducted in about half of the cases. However, no malignant lesions were observed in uterus and adnexa. This may suggest that CCC arising in the abdominal wall has different clinical evolution in respect to ovarian CCC, and it is likely that there is no link between them. Based on these data, we can assume that the significance of extensive pelvic surgery with hysteroannessiectomy remains unclear when the neoplasia is confined to the abdominal parietal wall. In our case, although surgery was not performed, ultrasound, MRI, and CT evaluation revealed a normal uterus and adnexa. An interesting aspect highlighted by the present case is that neoadjuvant chemotherapy had a higher efficacy on secondary lesions than on the primary lesion. Although there is no literature data to confirm our observation, our finding is consistent with a previous study stating that the best primary lesion treatment includes complete resection of the diseased tissue and adjuvant chemotherapy should also be considered; conversely, neoadjuvant chemotherapy was shown less effective (9). Based on this data, the primary lesion of the abdominal wall should be detected early to be removed surgically in order to obtain a better survival outcome.
Adjuvant chemotherapy and radiotherapy have been reported to improve outcomes, although no consensus is established regarding protocols. Although CCC shows poor chemosensitivity, a combination of carboplatin and paclitaxel has been reported as the preferable regime (39). The prognosis of these patients is quite poor; the median survival time is about 30 months (44).
In our patient, due to the extent of abdominopelvic disease and metastatic localization, surgical treatment was not performed, and only chemotherapy and palliative radiation therapy were administered.
Conclusion
CCC of the abdominal wall is a rare condition with just over thirty cases reported in the literature. Middle-aged women showing an abdominal wall mass in correspondence with a surgical scar from a previous cesarean section must be promptly investigated. Treatment options usually include surgery and chemotherapy with poor results. No published treatment guidelines are available because of its rarity. Given the low chemosensitivity, early surgery should be the treatment of choice for the primary lesion. From a preventive point of view, it must be emphasized that every gynecological surgery should always pay close attention not to leave visible tissue remnants on the abdominal wall.
Footnotes
Authors' Contributions
LG, MS and AC reviewed literature data and prepared the draft of the manuscript; EM, JDG, GDC and FS reviewed literature data; EM, RB planned the therapeutic regimens; AC and RB reviewed the final version of the manuscript. All Authors read and approved the final version of the manuscript.
This article is freely accessible online.
Conflicts of Interest
There are no conflicts of interest.
Informed Consent
Standard written informed consent was obtained from the patient for the use of data, pictures and videos for teaching, research purposes, and publication.
- Received March 28, 2020.
- Revision received April 25, 2020.
- Accepted April 28, 2020.
- Copyright© 2020, International Institute of Anticancer Research (Dr. George J. Delinasios), All rights reserved
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