An Isolated Infected Retroperitoneal Enteric Duplication Cyst Mimicking Renal Carcinoma: A Rare Presentation

Dear Editor,

Enteric duplication cysts (EDCs) are rare congenital anomalies, which occur anywhere between mouth and rectum.¹ In 1961, Melish and Koop defined “enteric duplications” as spherical or tubular structures that possess a mucosal lining characteristic of one or more portions of the alimentary tract supported by muscular and serosal layers.² In rare cases, duplication cysts are isolated from the digestive tract and have a unique blood supply, known as Isolated Enteric duplication cysts (IEDCs).³ The incidence rate of EDCs is 1 in every 4,000 to 5,000 live births.⁴

Herein, we report this rare case in 13-year-old female, who presented with left flank pain associated with fever and vomiting since a month. Her hematological, liver function test, renal function test, and urinalysis were within normal limits. She was put on a course of antibiotics, but her symptoms persisted. Ultrasound [Figure 1a], contrast MRI abdomen [Figure 1b] and CT urography [Figure 1c] revealed a partly exophytic complex left renal cystic lesion at its upper pole showing solid enhancing areas and coarse calcification, with possible differentials being mitotic etiology or infected cyst. In view of the above mentioned imaging findings, histopathological examination was advised to rule out malignancy. Laparoscopic left nephrectomy was done. Grossly specimen, measured 8 × 4 × 3 cm, showed an ulcerated cystic gray white lesion at upper pole of kidney [Figure 1d]. Microscopically, it revealed mucosal lining with underlying smooth muscle associated with marked inflammation [Figure 1e and f]. Features were consistent with inflamed retroperitoneal isolated EDC with organizing abscess and unremarkable renal parenchyma. In this case, preoperatively, it mimicked renal cell carcinoma, which is an unusual presentation of this rare anomaly; hence, a definitive diagnosis was made by constellation of clinical, imaging, and most crucial histopathological examination to come to a final conclusion.

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Figure 1:

(a) Ultrasonography showing thick-walled lobulated cystic lesion at upper pole of left kidney with internal echoes and echogenic foci, suggestive of complex cystic lesion; (b) MRI abdomen: Thin-walled cystic lesion at the upper pole of left kidney with heterogeneously enhancing solid component; (c) CT urogram: Complex partly exophytic left renal cystic lesion at the upper pole with solid enhancing areas and coarse calcification. (d) Gross photograph of nephrectomy specimen showing an ulcerated cystic lesion at upper pole of the left kidney, (e) Microphotograph showing colonic mucosa with underlying smooth muscle component along with inflammation (hematoxylin and eosin stain: ×100), (f) Microphotograph showing colonic mucosa with underlying smooth muscle and inflammation (hematoxylin and eosin stain: ×400).

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