Indian Journal of Pathology and Microbiology
Home About us Instructions Submission Subscribe Advertise Contact e-Alerts Ahead Of Print Login 
Users Online: 12431
Print this page  Email this page Bookmark this page Small font sizeDefault font sizeIncrease font size

  Table of Contents    
Year : 2020  |  Volume : 63  |  Issue : 2  |  Page : 286-288
Primary low-grade mucinous adenocarcinoma spleen resulting in Pseudomyxoma peritonei: A case report

1 Department of Pathology, Pathology Resident, St Stephens Hospital, Tis Hazari, New Delhi, India
2 H. O. D. Laboratory and Diagnostic Services, St Stephens Hospital, Tis Hazari, New Delhi, India

Click here for correspondence address and email

Date of Web Publication18-Apr-2020


A 61-year-old man presented with complaints of abdominal pain, loss of weight, and splenomegaly since past 6 months. On computed tomography, the spleen was totally replaced by multiloculated cysts and splenectomy was performed. Pathological examination revealed a low-grade mucinous adenocarcinoma. Postoperative PET scan was negative, and in the absence of a primary tumor elsewhere, we considered this tumor to be primary in the spleen, and it was presumed that the tumor arose from invaginated capsular mesothelium of the spleen.

Keywords: Mucinous adenocarcinoma, pseudomyxoma peritonei, spleen

How to cite this article:
Gupta B, Jindal R, Joseph M, Kaur K, Chopra S, Lakhiani L. Primary low-grade mucinous adenocarcinoma spleen resulting in Pseudomyxoma peritonei: A case report. Indian J Pathol Microbiol 2020;63:286-8

How to cite this URL:
Gupta B, Jindal R, Joseph M, Kaur K, Chopra S, Lakhiani L. Primary low-grade mucinous adenocarcinoma spleen resulting in Pseudomyxoma peritonei: A case report. Indian J Pathol Microbiol [serial online] 2020 [cited 2020 Aug 10];63:286-8. Available from: http://www.ijpmonline.org/text.asp?2020/63/2/286/282705

   Introduction Top

Pseudomyxoma peritonei (PMP) is a descriptive term, characterizing mucin filled cysts accumulation in peritoneal cavity due to spread of mucinous neoplasm. Mucin secreting adenocarcinomas are found most commonly in ovary, pancreas, and appendix. PMP most commonly develops secondary to spread of a mucinous tumor of the appendix. Metastatic spread of mucinous adenocarcinoma of appendix,[1],[2] ovary,[3] and pancreas[4],[5] to spleen have been reported, but primary mucinous adenocarcinoma of spleen is extremely uncommon tumor. We report an extremely rare case of primary mucinous adenocarcinoma of spleen leading to PMP.

   Case Report Top

A 61-year-old gentleman presented with complaints of swelling in the left upper abdomen for 6 months, which was insidious in onset and gradually increase in size. It was associated with loss of appetite and weight loss. There was no history of constipation, obstipation, hematemesis, melena, or jaundice. He had a history of laparoscopic appendectomy 10 years ago and right side inguinal hernioplasty 5 years ago, but no histological diagnosis was available. On clinical examination per abdomen a large around 13 × 10 cm firm, non-tender mass was palpable in left hypochondriac region with irregular surface, moving with respiration and noncompressible. Rest of the abdomen was unremarkable. A CT scan [Figure 1]a of whole abdomen was done which showed spleen enlarged in size measuring 192 mm and replaced by multiloculated cystic lesions with patchy calcification along its wall. A provisional diagnosis of hydatid cyst was made and patient was offered splenectomy. Per operative findings were enlarged spleen with lower pole rupture and multiple cysts adherent to it and whole of the peritoneal cavity, mesentery, bowel wall, liver surface, anterior abdominal wall, and diaphragm giving it an appearance of Pseudomyxoma peritonei. Spleen was densely adherent to its surrounding structures. Pancreas and kidney were normal.
Figure 1: (a) CT image of the enlarged spleen with multiple cysts. (b) Grossly enlarged spleen. (c) Cysts attached to the splenic hilar area. (d) On cutting open, splenic tissue replaced by multiple cysts filled with gelatinous material. (e) Splenic parenchyma replaced by multiple cysts with mucin pools (H and E 10×). (f) Cyst wall lined by neoplastic cells with mild atypia and intracellular mucin (H and E 40×)

Click here to view

On gross examination, spleen measured 18 × 16 × 11 cm and weighed 1.65 kg. The external surface was congested with multiple cysts adherent toward the hilar area ranging from 0.2 to 0.5 cm in greatest dimensions [Figure 1]b and [Figure 1]c. Cut surface spleen showed multiple cysts completely replacing the splenic parenchyma ranging in size from 0.5 to 7.5 cm in greatest dimensions filled with mucoid gelatinous material [Figure 1]d. On microscopy, it revealed multiple cysts lined by neoplastic cells with mild atypia and intracellular mucin production in both splenic hilar and parenchymal areas. Adenomatous pattern was present. Cysts have fibrous nests giving a diagnosis of low-grade mucinous adenocarcinoma [Figure 1]e and [Figure 1]f. Further, to rule out site of primary tumor elsewhere, a PET scan was performed which was negative and did not show any other primary site of the tumor.

   Discussion Top

Cystic lesions in the spleen can be congenital (Epidermoid cyst) or acquired cysts caused by trauma, parasite infection, pancreatitis, or neoplasm. Mucinous epithelial cysts of the spleen are extremely rare; only few cases have been reported.[6] Splenic metastasis of mucinous appendix adenocarcinoma was reported by Pimental in 2018,[2] where it presented as splenomegaly with nodular lesions and thickened appendix. Histopathological examination stated that appendix was the primary site. In the present case, the patient had undergone appendectomy 10 years ago although the histological diagnosis of appendix could not be confirmed, but the possibility of delayed metastasis is almost negligible.

The present tumor is not a primary splenic tumor, as its pathology is questionable because how can an epithelial tumor arise in spleen without any epithelial component. It can have two possibilities, either it can arise from invaginated mesothelium of splenic capsule, but patient does not have any significant history of abdominal trauma or it can be because of heterotopic pancreatic tissue or enteric tissue.[4],[5],[7] But, no heterotopic pancreatic or intestinal tissue was found in the histopathological examination in the specimen.

In 1992 Morinaga, et al.[8] presented a case which was very similar to the present case. An enlarged spleen was found by CT scan to be multicystic and history of appendectomy 36 years back. On histological examination revealed as low-grade mucinous adenocarcinoma. The tumor markers CEA and CA 19.9 were elevated per operatively returned to normal postoperatively. It was considered primary in the absence of tumor elsewhere. In the present case, PET scan was negative, which concludes that it was primary tumor of splenic origin.

   Conclusion Top

Primary low-grade mucinous adenocarcinoma of spleen is a very rare tumor and it holds importance as it can present with Pseudomyxoma peritonei. All cases of PMP should be investigated for splenic involvement or conversely, any mucinous tumor elsewhere in the abdomen should be ruled out before considering a diagnosis of primary splenic mucinous tumor with help of imaging and tumor markers and histopathological examination. Splenomegaly is considered as a part of treatment.

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.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

   References Top

Pimental JA, Mayca JL. Splenic metastasis of mucinous appendix adenocarcinoma: Case report. Gastroentrol Hepatol Open Access 2017;7:00255.  Back to cited text no. 1
Holder PD, Fehir KM, Schwartz MR, Smigocki G, Madewell JE. Primary mucinous cystadenocarcinoma of the appendix with pseudomyxoma peritonei manifested as a splenic mass. South Med J 1989;82:1029-31.  Back to cited text no. 2
Ronnett BM, Kurman RJ, Zahn CM, Shmookler BM, Jablonski KA, Kass ME, et al. Pseudomyxoma peritonei in woman: A clinicopathological analysis of 30 cases with emphasis on site of origin, prognosis, and relationship to ovarian mucinous tumours of low malignant potential. Hum Pathol 1995;26:509-24.  Back to cited text no. 3
Zanetti G, Riccioni L, Gallo C, Salfi N, Martinelli GN. Splenic mucinous cystadenocarcinoma arising in heterotopic pancreatic tissue. Tumori 1998;84:606-10.  Back to cited text no. 4
Nisar PJ, Zaitoun AM, Lobo DN, Rowlands BJ. Heterotopic pancreas in the spleen: Malignant degeneration to mucinous cystadenocarcinoma. Eur J Gastroenterol Hepatol 2002;14:793-6.  Back to cited text no. 5
Du Plessis DG, Louw JA, Warnz PA. Mucinous epithelial cysts of the spleen associated with pseudomyxoma peritonei. Histopathology 1999;35:551-7.  Back to cited text no. 6
Miracco C, De Martino A, Lio R, Botta G, Volterrani L, Luzi P. Splenic cyst lined with mucus-secreting epithelium. Evidence of an intestinal origin. Arch Anat Cytol Pathol 1986;34:304-6.  Back to cited text no. 7
Morinaga S, Ohyama R, Koizumi J. Low-grade mucinous cystadenocarcinoma in the spleen. Am J Surg Pathol 1992;16:903-8.  Back to cited text no. 8

Correspondence Address:
Richa Jindal
Department of Pathology, Senior Consultant, St Stephens Hospital, Tis Hazari, New Delhi - 110 054
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/IJPM.IJPM_624_19

Rights and Permissions


  [Figure 1]


    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Email Alert *
    Add to My List *
* Registration required (free)  

   Case Report
    Article Figures

 Article Access Statistics
    PDF Downloaded22    
    Comments [Add]    

Recommend this journal