Clear cell adenocarcinoma of the peritoneum: a case report and literature review
© Shigeta et al.; licensee BioMed Central Ltd. 2014
Received: 5 August 2014
Accepted: 27 August 2014
Published: 2 October 2014
Clear cell adenocarcinoma (CCC) is generally thought to originate from ovarian, endometrial, or renal tissue. A CCC of the peritoneum (CCAP) is an extremely rare medical condition and is associated with a poor prognosis. To date, only 10 cases of CCAP have been reported, of which half resulted in death or recurrence within 6 months after initial treatment because CCAP is commonly resistant to multiple drugs. In this report, we present a case of CCAP of the pouch of Douglas coexisting with an endometriosis and we offer a review of the related literature.
Clear cell adenocarcinoma of the peritoneum is an extremely rare medical condition with poor prognosis. Because of its reality, no standard treatment has been established. Here, we report a case with primary clear cell adenocarcinoma in the pouch of Douglas treated by debulking surgery followed by chemotherapy.
A 59-year-old postmenopausal woman, gravida 2, para 2, was referred to our hospital for the treatment of a pelvic mass. Her initial complaints were appetite loss and 12 kg weight loss over 3 months. Her past medical history was unremarkable, except for an appendectomy, and her gynecological medical history included administration of a gonadotropin releasing hormone agonist for the treatment of adenomyosis manifesting as severe dysmenorrhea.
Six courses of combination chemotherapy with paclitaxel and carboplatin (TC) as adjuvant therapy was planned. At 3 months after the primary surgery, the patient presented with strangulated ileus, which was corrected by a second surgery. No recurrent disease was observed in the abdomen at the surgery. Then, the patient completed the planned adjuvant therapy and has remained well with no evidence of disease 10 months after the primary surgery.
Primary peritoneal carcinoma was first described by Swerdlow in 1959 . The Gynecologic Oncology group defines the diagnostic criteria for primary peritoneal carcinoma, where both ovaries are of normal size, with an extraovarian involvement greater than the surface ovarian involvement . Our case met this criterion and was histologically diagnosed as CCC.
Summary of clear cell adenocarcinoma of the peritoneum
Tumor size and location
Past history or concurrent disease
Evans et al. 
Both Ov EM, AM, EH
DS followed by RT (pelvic; 4500R,upper abdomen;3000 R)
Lee et al. 
Concurrent EMA G1 of Ut (stageIb)
Tziortzioti et al. 
Concurrent CCC in EP
DS+TAH+BSO +OM followed by CHT (6 cycles, regimen NA)
DOD at 6 mo
Peritoneal and omental
Ichimura et al. 
Both Ov EM
DS+TAH+BSO followed by CHT (3 cycles of CPA,CDDP, CBDCA)
ROD at 32 mo
Hama et al. 
DS+BSO followed by CHT (regimen NA)
DOD at 5 mo
Ascites and small peritoneal solid lesions
Terada et al. 
3 cm, 2 cm
Past EMA G3 of Ut (TAH+BSO)
NED at 6 mo
Gastric peritoneal, splenic hilus
Takano et al. 
DS followed by CHT (1 cycle of CPT-11,CDDP, 1 cycle of TC)
DOD at 5 mo
Upper abdomen between liver and diaphragm and omentum
Takano et al. 
DS+TAH+BSO+OM+PLD+PAD followed by CHT (6 cycles of CPT-11, CDDP)
NED at 20 mo
Infracolic omentum and peritoneum of right abdominal wall
Matsuo et al. 
DS followed by CHT (6 cycles of TC), Secondary devulking surgery
ROD at 18 mo
Abdominal scar of EM surgery
Muezzinoglu et al. 
DS+TAH+BSO following by CHT (regimen NA)
NED at 12 mo
Johnson et al. 
3xMMs, TAH+BSO for LM and menorrhagia
CHT (6 cycles of TC) followed by EBR followed by the interstitial HDR BT boost
ROD at 4 mo
proximal vagina and vaginal cuff
DS+TAH+BSO+PLD followed by CHT (6 cycles of TC)
NED at 5 mo
Pelvic (pouch of douglas)
We offered TC combination chemotherapy to our patient. One report had found that a TC combination therapy was effective for the treatment of CCAP. Outcomes were poor in five cases. Of these, three patients died within 6 months, and disease recurrence had occurred in two cases at 4 months and 32 months, respectively, after the initial therapy ,–. Three patients died within 6 months and each had a residual tumor of >2 cm ,,. Early recurrence occurred in the inoperable patients. The characteristic features of the cases with particularly poor prognosis were large residual tumors after primary surgery and inoperability. On the other hand, there were three cases with relatively good prognosis ,,. The primary features of these cases were having had an optimal or complete debulking surgery followed by chemotherapy.
CCAP can closely resemble ovarian clear cell adenocarcinoma (OCC) and it is difficult to distinguish CCAP from OCC by preoperative imaging alone, especially if the tumor is located in the pelvis close to the ovary or uterus, even though the radiological features of CCAP have been reported in some cases. One report cited a multicystic mass with a multiseptate appearance and a heterogeneous solid part arising from the peritoneum, suggesting the occurrence of a CCAP .
In our case, we did not observe such features and we were unable to determine whether the pelvic tumor originated from the peritoneum. We recommend further imaging studies to establish a definitive diagnosis of CCAP. An endometriosis coexisted with the CCAP in our case. CCC is seen most frequently in the ovary and is often associated with pelvic endometriosis , as an association with endometriosis has been well established . CCC occasionally develops from extraovarian endometriosis ,. There are several reports of CCAP cases with coexisting endometrial lesions. In some reports, endometriosis was a required precursor of CCC in peritoneal locations. However, others found that the tumor arose de novo from the peritoneum . The pathogenesis of carcinoma coexisting with endometriosis has not been fully elucidated. In past reports, two possible theories for the association of carcinoma and endometriosis were mentioned. These theories included a genetic defect in already existing endometriosis or a defect in the immune response of patients with endometriosis that leads to the progression of endometriosis to subsequent malignant transformation . Hence, the pathogenesis of CCAP associated with endometriosis remains controversial.
The pathogenesis of peritoneal serous carcinoma is similar to that of ovarian cancer and may be dependent on its origin from ovarian tissue remnants in the peritoneum remaining from embryonic development or from the mesoderm that gives rise to both the ovarian epithelium and peritoneum . Therefore, female pelvic and abdominal mesothelium may give rise to primary peritoneal carcinoma resembling ovarian cancer . In addition to these reports, non-serous carcinoma of the peritoneum has also been reported . Therefore, CCAP may arise de novo from the peritoneum.
Here, we report a case of primary clear cell adenocarcinoma arising from the pouch of Douglas. Although 10 similar cases have been reported, there is as yet no established treatment regimen for this disease. Combination chemotherapy with debulking surgery has been suggested for the treatment of residual tumors <2 cm with postoperative chemotherapy, such as TC or CPT-P. To establish an effective treatment regimen for CCAP, a review of a larger number of cases is still needed.
Written informed consent was obtained from the patient for publication of this Case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
KY designed and conceived of the study and drafted the manuscript. NS participated in drafting the manuscript and performed literature review. SM and YU contributed critical reading of the manuscript and editing. EM supervised and participated in histopathological evaluation. All authors read and approved the final manuscript.
The authors thank Dr Buzard GS for his editing of the manuscript. We also thank Asami Yagi and Kanako Sakiyama for their help with this manuscript.
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