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A case of Endometrioid endometrial adenocarcinoma with synchronous low-grade Appendiceal mucinous neoplasm and Pseudomyxoma peritonei

 Department of Gynaec Endoscopy, Womens Hospital, Mumbai, Maharashtra, India

Date of Submission15-Aug-2020
Date of Decision12-Oct-2020
Date of Acceptance12-Oct-2020
Date of Web Publication03-Feb-2021

Correspondence Address:
Sharda Arvind,
Women's Hospital, 674, 16th Cross Road, Behind Khar Gymkhana, Khar West, Mumbai - 400 052, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jmas.JMAS_177_20


Pseudomyxoma peritonei (PMP) is a rare condition usually associated with ruptured low-grade mucinous neoplasm of the appendix. Rarely, PMP can originate from mucinous adenocarcinoma of the ovary. However, the coexistence of adenocarcinoma of the endometrium and appendiceal mucinous neoplasm has not been reported. We present the case of a post-menopausal woman with endometrioid endometrial adenocarcinoma with unexpected low-grade appendiceal mucinous neoplasm and PMP.

Keywords: Appendiceal mucinous neoplasm, pseudomyxoma peritonei, synchronous tumours

How to cite this URL:
Raje S, Arvind S, Rao G. A case of Endometrioid endometrial adenocarcinoma with synchronous low-grade Appendiceal mucinous neoplasm and Pseudomyxoma peritonei. J Min Access Surg [Epub ahead of print] [cited 2021 Mar 1]. Available from:

  Introduction Top

Pseudomyxoma peritonei (PMP) is the intraperitoneal accumulation of mucus due to ruptured mucinous neoplasm of the appendix characterised by redistribution phenomenon. Rarely, PMP may develop from mucinous neoplasms of other organs such as ovary, colon, urachus and pancreas.[1] PMP is classified according to the histology of peritoneal disease as mucin without epithelial cells, low-grade mucinous carcinoma peritonei, high-grade mucinous carcinoma peritonei and high-grade mucinous carcinoma peritonei with signet ring cells.[1]

The incidence of primary appendicular tumour is <2% with 0.3% prevalence of mucinous neoplasms in surgically removed specimens.[2] The incidence is higher in females with a median age of 60 years. The most common symptoms are pain in the right iliac region and palpable abdominal mass.[3] In 25% of cases, patients are asymptomatic and a diagnosis of appendiceal mucinous neoplasm is incidental during gynaecological screening.[2] The appendiceal mucinous neoplasm has a spectrum ranging from adenoma to adenocarcinoma. Low-grade appendiceal mucinous neoplasms (LAMNs) are mucinous neoplasms with low-grade cytological atypia with any of the following features: loss of muscularis mucosae, fibrosis of submucosa, presence of acellular mucin inside the wall and pushing invasion without causing infiltration of the wall, appendiceal rupture and presence of extra-appendiceal mucin or cells.[2],[3]

The most important factor affecting the treatment and survival of patients with appendiceal mucinous tumours is development of PMP.

Appendiceal mucinous tumours have been reported to coexist with mucinous ovarian epithelial tumours. However, the coexistence of adenocarcinoma of endometrium and appendiceal mucinous neoplasm has not been reported.

We present the case of a post-menopausal woman with endometrioid endometrial adenocarcinoma with unexpected LAMN and PMP.

  Case Report Top

An 82-year-old, P4 L4, woman presented with post-menopausal bleeding. Magnetic resonance imaging showed a bulky uterus with a thickened endometrium with focal areas of breach in the junctional zone and intramyometrial extension, suggestive of carcinoma of the endometrium. Both ovaries were atrophic. Mild ascites was seen with no pelvic lymphadenopathy [Figure 1].
Figure 1: Magnetic resonance imaging image

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Hysteroscopy showed vascular growth arising from the right anterolateral wall of the uterus. Endometrial biopsy revealed well-differentiated adenocarcinoma.

Intraoperatively, a mucinous jelly-like material was seen in the pelvis [Figure 2]. The uterus was normal in size. Bilateral Fallopian tubes and ovaries were atrophic. Friable growth arising from the appendix with mucinous deposits was seen [Figure 3]. Frozen section of the appendicular mass contained acellular mucinous material.
Figure 2: Pseudomyxoma peritonei

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Figure 3: Perforated appendicular mass

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We performed total laparoscopic hysterectomy with bilateral salpingo-oophorectomy and bilateral pelvic lymph node dissection and appendicectomy. Multiple peritoneal and omental biopsies were taken.

Histopathology revealed moderately differentiated endometrioid adenocarcinoma of the endometrium, FIGO Stage IB (The International Federation of Gynecology and Obstetrics) with a synchronously occurring low-grade mucinous neoplasm of the appendix showing a perforation and PMP [Figure 4].
Figure 4: H and E images: (a) Endometrioid adenocarcinoma. (b) Pseudomyxoma peritonei. (c) Ruptured appendicular neoplasm. (d) Low.grade mucinous neoplasm of the appendix

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She was referred to an oncosurgeon for further management where she was advised cytoreductive surgery with hyperthermic intraperitoneal chemotherapy.


The authors would like to acknowledge Dr Maria Alina DeSouza (Consultant Pathologist, S.L.Raheja Hospital, Mumbai), for providing the H & E images.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that her name and initial will not be published, and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Carr NJ, Cecil TD, Mohamed F, Sobin LH, Sugarbaker PH, González-Moreno S, et al. A consensus for classification and pathologic reporting of pseudomyxoma peritonei and associated appendiceal neoplasia: The results of the peritoneal surface oncology group international (PSOGI) modified Delphi process. Am J Surg Pathol 2016;40:14-26.  Back to cited text no. 1
Vavinskaya V, Baumgartner JM, Ko A, Saenz CC, Valasek MA. Low-grade appendiceal mucinous neoplasm involving the endometrium and presenting with mucinous vaginal discharge. Case Rep Obstet Gynecol 2016;2016.  Back to cited text no. 2
Gündogar Ö, Kimiloglu E, Komut N, Cin M, Bektas S, Gönüllü D, et al. Evaluation of appendiceal mucinous neoplasms with a new classification system and literature review. Turk J Gastroenterol 2018;29:533-42.  Back to cited text no. 3


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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2004 Journal of Minimal Access Surgery
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Online since 15th August '04