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  Table of Contents    
CASE REPORT  
Year : 2016  |  Volume : 59  |  Issue : 2  |  Page : 229-231
Primary retroperitoneal mucinous cystadenocarcinoma in a male patient


Department of Histopathogy, Max Super Speciality Hospital, New Delhi, India

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Date of Web Publication9-May-2016
 

   Abstract 

Primary retroperitoneal mucinous neoplasms are extremely rare tumors. Less than sixty cases have been reported in international literature until date, with striking female predominance. We report a case of primary mucinous cystadenocarcinoma of the retroperitoneum in a 51-year-old male presenting with nonspecific abdominal complaints. This case is being highlighted for its rare occurrence in a male patient.

Keywords: Cystadenocarcinoma, male, mucinous, retroperitoneum

How to cite this article:
Khurana A, Mukherjee U, Singaravel S. Primary retroperitoneal mucinous cystadenocarcinoma in a male patient. Indian J Pathol Microbiol 2016;59:229-31

How to cite this URL:
Khurana A, Mukherjee U, Singaravel S. Primary retroperitoneal mucinous cystadenocarcinoma in a male patient. Indian J Pathol Microbiol [serial online] 2016 [cited 2019 Dec 15];59:229-31. Available from: http://www.ijpmonline.org/text.asp?2016/59/2/229/182022



   Introduction Top


Primary retroperitoneal mucinous cystadenocarcinoma (PRMC) is a rare neoplasm, reported in literature as single case reports. With only a few case reports described in males, it has been reported exclusively in females.[1] The exact pathogenesis, behavior, and mode of treatment remain controversial. PRMC remains unsuspected preoperatively due to the nonspecific symptoms and inability of radiology to determine the exact site of origin. We report a case of PRMC in a male patient, with a brief review of the existing literature.


   Case Report Top


A 57-year-old male with no significant history presented to the surgical outpatient department with nonspecific complaints of loss of appetite and weight loss for 2 months duration. On physical abdominal examination, a firm to hard, nontender, fixed intra-abdominal mass was palpated with left renal angle fullness.

The routine blood examination along with upper and lower gastrointestinal endoscopy revealed no significant findings. The patient underwent a contrast-enhanced computed tomography of the abdomen which revealed a large peripherally enhancing predominantly cystic mass in the retroperitoneum, in close relation to the middle and upper pole of the left kidney, encasing it and displacing the adjacent viscera and vasculature [Figure 1]. The mass showed areas of peripheral postcontrast enhancement (likely solid areas) along with few nonenhancing areas. The mass was separate from the intestinal loops and the pancreas and appeared to have a renal origin though a primary retroperitoneal origin was also considered.
Figure 1: Contrast enhanced computed tomography film showing a large cystic retroperitoneal mass in relation to the left kidney

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A guided tru-cut biopsy was done which showed a hypocellular fibrocollagenous stroma, partly lined by pseudostratified bland columnar epithelium. No mitotic figures were noted. The differentials considered were cystic teratoma, cystic mucinous neoplasm, and a retroperitoneal bronchogenic cyst. Serum tumor marker work up was done; serum carcinoembryonic antigen levels were found to be markedly raised (1802.00 ng/ml) with normal levels being <3.0 ng/ml. The serum alpha-fetoprotein, beta human chorionic gonadotrophin, and free prostate specific antigen were within normal ranges. A mass excision was recommended. Intraoperatively, a large encapsulated tumor was seen, involving the upper pole of the left kidney. The capsule was adherent to the diaphragm, tail of the pancreas, and hilum of spleen without their gross invasion. The patient underwent a left radical nephrectomy along with excision of the mass which was sent for histopathological examination. Prosector noted an encapsulated cystic tumor mass measuring 21 × 18 × 7 cm with areas of capsular breech. The cut section revealed a multilocular multicystic mass with a variegated appearance; consisting of varying sizes of cystic spaces filled with inspissated mucoid and gelatinous material (denoting the solid component), and blood clots giving a sponge-like an appearance [Figure 2]a. The tumor was encasing the left kidney, predominantly its upper and middle poles; the left kidney measured 10 × 5.5 × 4.5 cm. The ureter and the left suprarenal gland were identifiable and grossly normal in appearance.
Figure 2: (a) The gross photograph reveals cut section of the multicystic mucoid tumor mass along with lower pole of the kidney (black arrow). (b) Microphotograph showing neoplasm consisting of multiple variably sized cysts filled with mucin and lined by atypical mucinous epithelium with cuboidal to columnar appearance (H and E, ×100). (c) Microphotograph showing renal parenchyma invaded by neoplastic mucinous epithelium and pools of extracellular mucin (H and E, ×200). The unremarkable glomerulus is highlighted by the black arrow

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The microscopic examination showed a multicystic neoplasm comprising of variably-sized cystic spaces lined by atypical mucinous epithelium with cuboidal to columnar appearance and interspersed goblet cells [Figure 2]b. The epithelium showed mild to moderate nuclear atypia, scattered mitoses along with nuclear stratification. At places, the epithelia showed back to back arrangement, broken glands and small nests floating in pools of extracellular mucin. The sections showed intermixed areas of stromal fibrosis, inflammatory response, and congestion. The tumor was microscopically involving the adjacent adherent renal parenchyma which was otherwise histologically unremarkable [Figure 2]c. No evidence of mucinous metaplasia of the renal pelvis and renal cortex was seen.

Immunohistochemistry was performed, and the lining epithelium was positive for CK7 and cdx-2 with focal positivity for CK20 [Figure 3]; Ki67 index was 15%. A final histopathological diagnosis of primary mucinous cystadenocarcinoma of the retroperitoneum was made.
Figure 3: Immunohistochemistry (IHC) showing CK7 membranous expression (DAB, ×400). Inset showing focal CK20 positivity (a) and diffuse nuclear cdx-2 expression (b)

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The patient had an uneventful postoperative period. Our patient was followed-up of 15 months without evidence of disease recurrence or metastases after the operation.


   Discussion Top


The retroperitoneal tumors of epithelial origin are rare, this being attributed to the lack of definite lining epithelium in the region. The mucinous tumors of retroperitoneum have been reported in literature, exhibiting a spectrum of benign, borderline, and malignant histology.[2],[3] The first case of carcinoma with Mullerian-type epithelium was reported by Roth and Ehrlich in 1977.[4] The pathogenesis and the site of origin of this uncommon entity are ambiguous, with various theories being postulated. In view of the fact that majority of the initially detected cases were females, it was hypothesized that possible origin of this tumor could be ectopic or supernumerary ovarian tissue or a possible transformation occurring in a teratoma.[4],[5] With the passage of time, the entity was also diagnosed in male individuals; hence, putting forward the other popular theory of coelomic metaplasia occurring in the retroperitoneum or a possible source being undescended testis.[6] The latter was ruled out in the indexed case as both the testes were descended.

To the best of our knowledge, this is the ninth case of primary retroperitoneal mucinous cystadenocarcinoma (PRMC) to be reported worldwide in males. We consider our case to be primarily originating from retroperitoneum. The immunohistochemical coexpression of CK7 and CK20 along with nuclear cdx-2 denotes a gastrointestinal phenotype, hence postulating a possibility of having originated from a sequestrated enteric-type epithelium in the retroperitoneum. The tumor in the indexed case was separate from the intestines, pancreas, but invaded the left kidney. CK7 and CK20 were positive in our tumor which are usually absent in primary renal carcinomas.

Preoperatively, PRMC is rarely suspected owing to its nonspecific symptoms. The study of tumor markers and radiology is not much useful in reaching the diagnosis or determining the site of origin. The mainstay of diagnosis and, in fact, initial treatment of this rare tumor remains exploratory laparotomy and enucleation/excision of the retroperitoneal mass, as performed in our case. Adjuvant therapy subsequent to surgery is a controversial issue, as no definite therapeutic guidelines have been laid. Role of chemotherapy is debatable but is considered in cases of tumor capsule rupture or invasion of adjoining organs. The differential diagnoses in PRMC include metastatic mucinous tumors from various sites such as ovaries, intestines, and pancreas which are more common and have to be excluded.[6]

The prognosis of these tumors remains uncertain because of their rarity and short follow-up of the patients. However, the follow-up period of the available case reports in literature were uneventful and did not show evidence of recurrence with documented disease-free survival ranging from 6 to 79 months.[1],[6],[7] Local recurrence was observed in the case of pelvic retroperitoneal mucinous cystadenocarcinoma.[8] This case is being presented to emphasize on the rarity of primary mucinous tumors of the retroperitoneum. It should be kept in differential diagnosis of mucinous neoplasm arising in that region.

Acknowledgment

Dr. Anuj Thakral, Department of Radiology, Max Hospital, Saket.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
   References Top

1.
Feng J, Liu H, Chen D. Primary retroperitoneal mucinous cystadenocarcinoma in a male patient: A rare case report. Hippokratia 2013;17:271-3.  Back to cited text no. 1
    
2.
Navin P, Meshkat B, McHugh S, Beegan C, Leen E, Prins H, et al. Primary retroperitoneal mucinous cystadenoma – A case study and review of the literature. Int J Surg Case Rep 2012;3:486-8.  Back to cited text no. 2
    
3.
Bifulco G, Mandato VD, Giampaolino P, Nappi C, De Cecio R, Insabato L, et al. Huge primary retroperitoneal mucinous cystadenoma of borderline malignancy mimicking an ovarian mass: Case report and review. Anticancer Res 2008;28:2309-15.  Back to cited text no. 3
    
4.
Roth LM, Ehrlich CE. Mucinous cystadenocarcinoma of the retroperitoneum. Obstet Gynecol 1977;49:486-8.  Back to cited text no. 4
    
5.
Song DE, Kim MJ, Shin SK, Yu E, Cho KJ. Primary mucinous cystic neoplasm of the retroperitoneum: A report of three cases. Korean J Pathol 2003;37:2003.  Back to cited text no. 5
    
6.
Thamboo TP, Sim R, Tan SY, Yap WM. Primary retroperitoneal mucinous cystadenocarcinoma in a male patient. J Clin Pathol 2006;59:655-7.  Back to cited text no. 6
    
7.
Shiau JP, Wu CT, Chin CC, Chuang CK. Long-term survival after hand-assisted laparoscopic approach of primary retroperitoneal mucinous cystadenocarcinoma in male: Case report and review of literature. Eur Surg 2013;45:106-109.  Back to cited text no. 7
    
8.
Williams AK, Boyle SL, Chan SM, Martinez CH, Lu C, Chin JL. Primary mucinous cystadenocarcinoma of the pelvic retroperitoneum in a male. Can Urol Assoc J 2010;4:E169-71.  Back to cited text no. 8
    

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Correspondence Address:
Anuj Khurana
98 SFS Flats, Phase - 4 Ashok Vihar, New Delhi - 110 052
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.182022

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    Figures

  [Figure 1], [Figure 2], [Figure 3]

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