|Ahead of print
Primary retroperitoneal serous adenocarcinoma: A case report of rare malignancy with literature review
Thin Thin Win1, Saint Nway Aye1, Nurul Shuhada Abdul Hamad2, Sharifah Emilia Tuan Sharif2
1 Pathology Division, School of Medicine, International Medical University, 126, Jalan Jalil Perkasa 19, Bukit Jalil, Kuala Lumpur, Malaysia
2 Department of Pathology, Hospital Universiti Sains Malaysia, 16150, Kubang Kerian, Kelantan, Malaysia
|Date of Submission||10-Jun-2019|
|Date of Decision||17-Jun-2019|
|Date of Acceptance||17-May-2020|
|Date of Web Publication||02-Nov-2020|
Thin Thin Win,
Pathology Division, School of Medicine, International Medical University, 126, Jalan Jalil Perkasa 19, Bukit Jalil, Kuala Lumpur
Source of Support: None, Conflict of Interest: None
The primary retroperitoneal serous adenocarcinoma (PRSAC) is a rare malignant tumor of the retroperitoneum. It shares the same pathological and biological behavior with ovarian serous carcinoma. Most of the cases develop as peritoneal adenocarcinoma and rarely occur in the retroperitoneum. It is reported as serous surface papillary carcinoma of the peritoneum and extraovarian peritoneal serous papillary carcinoma. We present a case of PRSAC in a 60-year-old woman. Only 11 cases of PRSAC have been reported from 1983 to 2019. Histopathological features with immunohistochemical expressions are important to diagnose PRSAC. The outcome and survival mainly depend on the possibility of surgical resection. Molecular genetics of PRSAC should also be studied in relation with its ovarian counterpart.
Keywords: Metaplasia, peritoneum, retroperitoneal neoplasms, serous adenocarcinoma
|How to cite this URL:|
Win TT, Aye SN, Abdul Hamad NS, Tuan Sharif SE. Primary retroperitoneal serous adenocarcinoma: A case report of rare malignancy with literature review. Indian J Cancer [Epub ahead of print] [cited 2021 May 5]. Available from: https://www.indianjcancer.com/preprintarticle.asp?id=299722
| » Introduction|| |
Primary retroperitoneal serous adenocarcinoma (PRSAC) is a rare malignancy and is known to arise from the heterotopic ovarian tissue or invaginations of the peritoneal mesothelial layer with concurrent or subsequent müllerian metaplasia. It shares the same pathological and biological behaviors as ovarian serous carcinoma. Most of the cases develop as peritoneal adenocarcinoma and rarely occur in the retroperitoneum. It is reported under various names such as surface serous papillary carcinoma of the peritoneum and extraovarian peritoneal serous papillary carcinoma. Therefore, it is considered as a subtype or the variant of primary peritoneal serous carcinoma.
We present a case of PRSAC in a 60-year-old woman whose ovaries were surgically removed 20 years ago. In our literature search, only 12 cases of PRSAC were published till 2019, which are reviewed here with our case.
| » Case Summary|| |
A 60-year-old woman who was a known case of urolithiasis presented with an abdominal swelling, left flank pain, urinary frequency, constipation, and weight loss for 2 months. She had had a history of total abdominal hysterectomy and bilateral salpingo-oophorectomy in her 40s for severe endometriosis. Histopathological diagnoses at that time were benign endometrial polyp and bilateral ovarian endometriosis without any evidence of malignancy.
On examination at the current admission, there was an abdominal mass extended from the suprapubic to the umbilical region. Serum cancer antigen 125 (CA-125) was 253 units/mL and serum carcinoembryonic antigen (CEA) was 5.1 ng/mL. Abdominal ultrasound scan showed a cystic intra-abdominal mass extending from epigastrium to the pelvic region with septations and solid component, hydronephrosis with hydroureter in the left kidney, and multiple calculi in both kidneys. Computerized tomography (CT) scan confirmed a huge complex solid cystic mass in the left pelvic cavity extending into the abdominal cavity and attached to the retroperitoneal structures. [Figure 1].
|Figure 1: CT abdomen and pelvis showing complex solid cystic mass in the left pelvic cavity attached to the retroperitoneal structures|
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During the exploratory laparotomy, the uterus and adnexa were absent with dense pelvic adhesions and a large retroperitoneal solid cystic mass. The mass was multiloculated with small solid component. Complete excision was impossible as the posterior part of the mass adhered to the underlying structures. After the stenting of the left kidney, left ureter, and bladder, the mass was drained yielding blood-stained fluid, and it was partially excised. Liver and the peritoneal lining were free of tumor deposits.
Histopathological sections of the cystic part showed a cyst wall lined by a single layer of flattened-to- columnar epithelium. The solid part of the mass was composed of complex papillary structures and the glands were lined by marked pleomorphic tumor cells [Figure 2]a with brisk abnormal mitoses. The underlying stroma was desmoplastic with inflammation. Endometrial tissue was not seen in all sections including cyst walls. The tumor cells were immunohistochemically positive for CK7 [Figure 2]b, EMA, vimentin [Figure 2]c, p53, E-cadherin, and WT-1 [Figure 2]d with focal CD10 positivity within the stroma. They were negative for CK20, CEA, TTF-1, CK5/6, estrogen receptor (ER), and progesterone receptor (PR). Histopathological diagnosis of PRSAC was confirmed with the suggestion of the tumor arising from the underlying retroperitoneal müllerian cyst.
After the surgery, six cycles of chemotherapy with combined cisplatin and paclitaxel were given. While receiving chemotherapy, condition of the patient deteriorated with developing pleural effusion, ascites, multiple metastasis in the lungs, liver, pelvic lymph nodes, and brain; and the patient died 6 months after the surgery.
| » Discussion|| |
The primary retroperitoneal epithelial neoplasms are extremely rare due to the absence of epithelial cells in the area. Pathologically, they resemble ovarian epithelial tumors; and are serous or mucinous and benign, borderline or malignant. Primary retroperitoneal mucinous adenocarcinomas (PRMACs) are more common than PRSAC as 78 cases have been reported in the literature, whereas only 11 cases of PRSAC have been reported, before our case. A summary of the reported cases of PRSAC is shown in [Table 1]. The neoplasms of the müllerian origin can develop infrequently in the retroperitoneum such as mucinous cystadenoma, serous/mucinous adenocarcinoma, clear cell carcinoma, and endometrial stromal sarcoma.,
|Table 1: Summary of reported cases of primary retroperitoneal serous adenocarcinoma|
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Ulbright et al. first reported a case of PRSAC in a 11-year-old girl in 1983. The most widely accepted theory in the pathogenesis of PRSAC is metaplasia of the celomic epithelium.,, PRSACs are thought to be analogous to ovarian neoplasm from invagination of the surface epithelium. The subsequent metaplasia and neoplasia of invaginated peritoneal mesothelial lining could account for the retroperitoneal müllerian tumors. Therefore, it is believed to arise from the secondary müllerian system; and metaplasia of the mesothelium may sporadically allow the development of primary serous carcinoma in the retroperitoneum, such as in the peritoneum and ovaries.
High-grade serous ovarian, tubal, and peritoneal carcinomas of müllerian origin have an association with TP53 and BRCA1/2 mutations. As the PRSAC shares the same pathological and biological behavior as ovarian serous adenocarcinoma, molecular genetics should be studied. However, due to the rarity of PRSAC, molecular genetics have not been reported in the literature. As the biological behavior of PRSAC is very close to ovarian carcinoma, most of the cases are seen in postmenopausal women. However, a case of PRSAC was reported in a 71-year-old man, and a case of PRMAC was reported in a 63-year-old man. PRSAC in a women of reproductive age,,, and in a 11-year-old girl were also reported. In this case, the absence of both tubes and ovaries during laparotomy excluded the possibility of primary ovarian carcinoma. Although both ovaries had been surgically removed, malignant transformation from the retroperitoneal endometriosis should be excluded. However, no endometrial tissue was detected in all resected sections. Cyst wall lined by a single layer of benign flattened cuboidal to columnar epithelium supported that the tumor originated from the underlying benign müllerian cyst of the retroperitoneum. The Gynaecologic Oncology Group has developed the criteria to define primary peritoneal/retroperitoneal serous carcinoma: (i) both ovaries are normal in size or enlarged by a benign process; (ii) the involvement in extraovarian sites is greater than the involvement on the surface of either ovary; (iii) microscopically, the ovaries are not involved with the tumor or exhibit only serosal or cortical invasions with dimensions smaller than 5 × 5 mm; (iv) the histopathological and cytological characteristics of the tumor are predominantly of the serous type., According to these criteria and prior surgical removal of both ovaries, this case met the criteria to diagnose PRSAC.
Most of the reported PRSAC cases showed elevated tumor markers, such as CEA, CA-125, and CA19-9, which are often elevated in ovarian serous adenocarcinomas. In the ovary, elevated serum CA-125 is more common seen in the serous adenocarcinoma, whereas elevated serum CEA is more common in the mucinous adenocarcinoma. Our case showed increased CA-125 (253 unit/mL) preoperatively. Serial detection of serum tumor markers is necessary for disease monitoring and prognosis.
As PRSAC has a similar biological behavior as ovarian serous carcinoma, it can express the same epithelial and immunohistochemical markers of ovarian counterpart such as CK7, EMA, CEA, p53, WT-1, PAX-8, ER, CA-125, and E-cadherin. As the PRSAC is a high-grade malignancy, most tumor cells express p53. In our case, tumor cells expressed CK7, EMA, vimentin, p53, E-cadherin, WT-1, and were negative for CK20, CEA, TTF-1, CK5 / 6, ER/PR. Focal CD10 positivity suggested that the tumor was of müllerian origin. In some cases, vimentin was negative. Although PAX-8 could not be performed in our institution, WT-1 expression and the finding of the benign serous cyst supported the retroperitoneal müllerian origin.
There is no standard therapy for PRSAC. At present, complete resection with a safe margin is the only curative option to achieve the most favorable outcome. However, the long-term survival and the prognosis cannot be discussed because of its rarity. In some literatures, complete resection was impossible as the tumor was attached to the adjacent structures.,,
Regarding the outcome of PRSAC in the literature, 3 cases were recorded alive with the disease for 15, 23, and 32 months.,, Five cases were alive with no evidence of the disease for 10, 24, 6, 5, and 12 months.,,,, Two cases, including our case, died 24 and 10 months after the surgery. The remaining two cases did not report the outcome., In our case and another case, complete removal was impossible due to the adhesion of the tumor to the adjacent structures. Therefore, the outcome of the disease is favorable in cases with complete surgical removal.
In conclusion, PRSAC is a rare entity of the retroperitoneal adenocarcinoma. In women, serous adenocarcinoma of ovarian origin should be excluded. The diagnosis of PRSAC should be made after correlation of clinical, radiological, and pathological information. The outcome and survival mainly depend upon the possibility of surgical resection of the tumor. The molecular genetic association should also be studied in relation with its ovarian counterpart.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]