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 Table of Contents  
CASE REPORT
Year : 2020  |  Volume : 6  |  Issue : 1  |  Page : 84-86

Primary peritoneal tumor: A rare malignancy with atypical presentation


1 Assistant Professor, IGIMS, India
2 Senior Resident, IGIMS, India
3 Professor & Head, Dept. of Gynecological Oncology, IGIMS, India
4 Professor & Head, Dept. of Hematology, IGIMS, India

Date of Submission14-Jan-2020
Date of Acceptance17-Jan-2020
Date of Web Publication16-Nov-2020

Correspondence Address:
Sangeeta Pankaj
Professor & Head, Gynecological Oncology, SCI, IGIMS, Patna
India
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Source of Support: None, Conflict of Interest: None


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  Abstract 


In females abdominal masses are mostly of uterine or ovarian origin. Rarely primary serous peritoneal tumors may present in similar fashion and should be considered as one of the differential diagnosis.
A 62 year-old female patient presented to us with an abdominal mass. Her CA-125 was significantly elevated and the radiological picture was suggestive of solid adnexal mass with lymphadenopathy but no ascites. Optimal debulking surgery was performed including Total hysterectomy, bilateral oophorectomy, total omentectomy, lymphadenectomy and appendectomy. Histopathological examination revealed the excised specimen possessing characteristics of metastatic adenocarcinoma of mullerian origin however uterus and adnexa were unremarkable. After excluding other primary causes of metastatic adenocarcinoma, diagnosis of a primary peritoneal carcinoma was finally established.
The objective of sharing this case is to emphasize over keeping PSPC in mind while establishing diagnosis of abdominal tumor without ascites for better management and patient outcome.

Keywords: Peritoneum, Primary serous peritoneal carcinoma, Ovary, Omentum


How to cite this article:
Rani J, Abhilashi K, Kumari P, Kumari S, Pankaj S, Choudhary V. Primary peritoneal tumor: A rare malignancy with atypical presentation. J Indira Gandhi Inst Med Sci 2020;6:84-6

How to cite this URL:
Rani J, Abhilashi K, Kumari P, Kumari S, Pankaj S, Choudhary V. Primary peritoneal tumor: A rare malignancy with atypical presentation. J Indira Gandhi Inst Med Sci [serial online] 2020 [cited 2020 Nov 24];6:84-6. Available from: http://www.jigims.co.in/text.asp?2020/6/1/84/300748




  Introduction: Top


Primary serous peritoneal carcinoma (PSPC) is rare epithelial tumor; mostly of serous type with incidence of 6.78/1,000,000 individuals.[1] It originates from peritoneal lining and share similar clinical and histological features of serous ovarian carcinoma. Establishing preoperative diagnosis of primary peritoneal carcinoma is difficult as its diagnosis is based on exclusion and made after intraoperative assessment as well histological confirmation of minimal or no involvement of ovary. Traditionally, diagnosis of primary serous peritoneal carcinoma is based on criteria laid down by Gynecologic oncology group i.e. (1) bilateral ovaries are of normal size or enlarged due to benign pathology; (2) extra-ovarian involvement is more than ovarian involvement; (3) absence of invasive ovarian carcinoma or cortical invasion with tumors measuring less than 5 × 5 mm2; and (4) histologically tumor is of a serous type.[2]

Primary serous peritoneal tumor is commonly associated with features of carcinomatosis, ascites or omental caking, however an abdominal lump without ascites is its rare presentation.

Here we present such case of abdominal tumor in a postmenopausal female where diagnosis of PSPC was made postoperatively after histopathological examination confirmed the pelvic mass as adenocarcinoma of mullerian origin with unremarkable uterus and adnexae and excluding other primary causes of adenocarcinoma.


  Case Report: Top


The patient was a 62-year-old female, referred to gynecologic oncology OPD, IGIMS, with complaint of lower abdominal swelling associated with anorexia. Abdominal examination, revealed a solid non-tender mass of 12*8 cm in the left iliac fossa and had no ascites. Speculum examination was normal and on P/V, uterus was retroverted, normal size, right fornix free and through anterior and left fornix same mass was palpable with restricted mobility. CECT scan of abdomen and pelvis was suggestive of 9*6 cm heterogeneously enhanced mass involving left ovary with another solid tissue lesion of 4*3cm in the right hypochondrium. Although liver portal vein, heaptic and splenic vein had normal enhancement and common bile duct and intrahepatic bile duct were not dilated. Gallbladder was not visualized as she had history of cholecystectomy. Tumor markers were elevated with serum value of CA125 =3778 U/ml (normal range 0-35 U/ml), CA19-9=19.2 U/ml (normal range 0-37 U/ml) and CEA=4.6 (<3ng/ml). Impression of Ca ovary was made and following required preoperative work-up, patient underwent optimal debulking surgery. Intraoperatively, uterus, bilateral fallopian tube and ovaries were normal looking and there was no ascites. On exploration, gut, liver, spleen and under surface of diaphragm were smooth and normal looking macroscopically. 10*8 cm and 4*3 cm metastatic deposits were found on left lateral pelvic wall. There was omental cake of 4*3 cm and paraaortic and left external lymph nodes were enlarged. Total abdominal hysterectomy, bilateral salpingo-ooporectomy, total omentectomy and retroperitoneal lymphadenectomy were done along with resection of metastatic deposits. Her postoperative stay at hospital was uneventful. On histopathological examination of specimen, no evidence of ovarian or uterine involvement was found and the mass was reported as adenocarcinoma of mullerian origin [Figure 1]. Specimen was examined for immunohistochemistry and was found positive for cytokeratin7, WT1, PAX8 whereas negative for CDX2 and TTF1, specific for adenocarcinoma of intestinal origin and of the lung respectively [Figure 2]. This again favored the diagnosis of primary serous peritoneal carcinoma excluding other causes of adenocarcinoma. Presently she is 2 months postoperative, alive & healthy. She was advised for follow up further for chemotherapy but she declined.
Figure 1: H&E section of the tumor shows lymph node structure infiltrated by a tumor arranged in sheets and islands. Tumor cells exhibit moderate pleomorphism having vesicular nuclei, prominent nucleoli and moderate to abundant cytoplasm with areas of necrosis seen.

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Figure 2: IHC analysis showing positivity for CK7, WT1, PAX8 &P53 (from left to right)

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  Discussion: Top


PSPC mostly occurs in postmenopausal females and can be confused with serous ovarian carcinoma due to common clinical presentation like anorexia, abdominal pain, distension and ascites. Peritoneum and ovary share common embryonal origin as both are derived from coelomic epithelium and has mullerian potential leading to difficult histological differentiation between these two primary peritoneal tumor and ovarian carcinoma.[3]

Although PSPC and ovarian carcinoma shares common genetic linkage as well clinical presentation, PSPC has significantly lower incidence than that of epithelial ovarian carcinoma (6.78 cases/ 1,000,000 versus 120.5 cases per/1,000,000 individuals, respectively).[1]

In postmenopausal females, pelvic tumors are mostly ovarian, of benign or malignant nature. Occasionally they may present with uncommon conditions such as fibroid, leiomyosarcoma or non gynaecological tumors. Literature search till date reveals only 3 such cases of primary serous peritoneal tumor without ascites and with no ovarian or other malignancies, later diagnosed as PSPC.[4],[5],[6] Index case too had an abdominal mass without ascites and diagnosis was established following histopathological examination declaring it as metastatic adenocarcinoma of mullerian origin with unremarkable uterus and ovaries. There are also case reports of Sister Mary Joseph’s nodules as atypical presentation of primary peritoneal serous tumor.7

Conventional imaging methods are not accurate to detect PSPC however comparing with MRI and CT, PET/CT may offer more diagnostic details.[8]

Serum level of CA-125 can be of prognostic value and helpful in evaluation of treatment response and recurrence of disease.

Roh et al reported elevated preoperative CA-125 values in 21 out of 22 PSPC patients in range of 35-7647 U/mL with a median value of 706 U/ml.9 In index case, CA 125 was significantly elevated (3778 U/ml).

On immunohistochemistry examination, PSPCs are typically positive for cytokeratin-7, CA-125, estrogen receptor and Wilms’ tumor-1 (WT-1) and negative for TTF1.10 Index case also had positive staining for cytokeratin-7, WT-1 and PAX-8 and negative for TTF1.

The optimal management of PSPC is cytoreduction and platinum based chemotherapy similar to Ca ovary, however intraperitoneal chemotherapy has recently demonstrated a survival benefit in patients with PSPC comparing to those managed with surgery alone or surgery in combination with systemic chemotherapy.[11]

Primary serous peritoneal carcinoma is considered to have poor prognosis than carcinoma of ovary with the overall survival time of around 30 months.[11] Index case is at present 2 months postoperative, healthy and asymptomatic at but she refused for further follow up and chemotherapy.


  Conclusion: Top


Primary serous peritoneal carcinoma presenting with abdominal mass without ascites is a rare entity and due to elevated CA125 can be misdiagnosed as ovarian tumor metastases. High index of suspicion for this clinical entity in such cases can be helpful in order to formulate a thorough preoperative examination and management accordingly to improve the prognosis.

Conflict of Interest : Nil



 
  References Top

1.
Goodman MT, Shvetsov YB. Incidence of ovarian, peritoneal and fallopian tube carcinomas in the United States, 1995-2004. Cancer Epidemiol Biomarkers Prev. 2009;18:132-139.  Back to cited text no. 1
    
2.
Bloss JD, Liao S, Buller RE et al. Extraovarian peritoneal serous papillary carcinoma: a case-control retrospective comparison to papillary adenocarcinoma of the ovary. Gynecol Oncol. 1993;50:347-351  Back to cited text no. 2
    
3.
Mok SC, Schorge JO, Welch WR, Hendricksen MR, Kempson RL. Peritoneal tumours. Tavassoli FA, Devilee P, editors. Pathology and genetics of tumours of the breast and female genital organs. Lyon: IARC; 2003;197-202.  Back to cited text no. 3
    
4.
Efthimiadis C, Ioannidis A, Kofina K, Grigoriou M. Primary solitary peritoneal tumor of the abdominal wall-report of a rare case and review of the literature. J Surg Case Rep. 2017(6):rjx094  Back to cited text no. 4
    
5.
Matsuura T, Sugihara K, Kohmura Y, Sugimura H, Kanayama M. Rare form of extraovarioan peritoneal serous papillary carcinoma with solitary tumor in the abdominal wall: a case report and literature review. J Obstet Gynaecol Res 2009;35:1142-7  Back to cited text no. 5
    
6.
Wong WL, Tay EH. Adenocarcinoma of the abdominal wall. Singapore Med J 2008;49:359-60.  Back to cited text no. 6
    
7.
Robyn L. Schickler, Reem Abdallah, E. Clair McClung, Mian M.K. Shahzad. Primary peritoneal carcinoma presenting as a Sister Mary Joseph’s nodule: A case report and review of the literature. Gynecol Oncol Rep. 2016;17: 20-22.  Back to cited text no. 7
    
8.
Klumpp BD, Schwenzer N, Aschoff P, Miller S, Kramer U, Claussen CD, Bruecher B, Koenigsrainer A, Pfannenberg C. Preoperative assessment of peritoneal carcinomatosis: intraindividual comparison of 18F-FDG PET/CT and MRI. Abdom Imaging. 2013;38(1):64-71.  Back to cited text no. 8
    
9.
S.Y. Roh, S.H. Hong, Y.H. Ko, T.H. Kim, M.A. Lee, B.Y. Shim, et al. Clinical characteristics of primary peritoneal carcinoma Cancer Res Treat. 2007;39:65-68  Back to cited text no. 9
    
10.
von Riedenauer WB, Janjua SA, Kwon DS, Zhang Z, Velanovich V. Immunohistochemical identification of primary peritoneal serous cystadenocarcinoma mimicking advanced colorectal carcinoma: a case report. J Med Case Reports. 2007;1:150  Back to cited text no. 10
    
11.
Bakrin N, Gilly FN, Baratti D, Bereder JM, Quenet F, Lorimier G, Mohamed F, Elias D, Glehen O. Association Française de Chirurgie: Primary peritoneal serous carcinoma treated by cytoreductive surgery combined with hyperthermic intraperitoneal chemotherapy. A multi-institutional study of 36 patients. Eur J Surg Oncol. 2013;39:742-747.  Back to cited text no. 11
    


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