Retroperitoneal primary adenocarcinoma of Mullerian origin: case report with radiology review

Abstract

Retroperitoneum Mullerian neoplasms are extremely rare malignancies. We report a case of a 35-year-old woman who presented with a 12-year history of lower abdominal cystic lesion, presumed of renal origin and benign, as such was not followed for 10 years. Prior to pregnancy, the patient received additional imaging and the lesion was again redemonstrated and questioned to be of ovarian origin. As such, the patient underwent laparoscopy for planned cystectomy and was found to have normal ovaries. After pregnancy, the lesion had increased in size and surgical excision revealed that the cystic mass was retroperitoneal in origin. The histopathology was reported as microinvasive mucinous adenocarcinoma with Mullerian origin. Positron emission tomographic scanning, colonoscopy, and endoscopy were unrevealing. Tumor markers were followed and follow-up scans demonstrated no recurrence. The preoperative diagnosis of primary retroperitoneal adenocarcinomas of Mullerian origin is difficult, and a definitive diagnosis cannot be made without postsurgical histopathological analysis. However, it is important for radiologists to recognize imaging features of this entity and include it in the differential diagnosis. Here we report a case and review imaging features reported for retroperitoneal primary adenocarcinomas of Mullerian origin.

Keywords: Adenocarcinomas of Mullerian origin; Retroperitoneum Mullerian neoplasms; Women's imaging.

Fig. 1

Demonstrates a large cystic and solid mass (white arrow) located within the left hemiabdomen with the superior portion abutting the lower pole of the left kidney. The solid component is mildly enhancing (yellow arrow). The mass extended into the pelvis and resulted in outward bulging of the left abdominal wall. The lesion resulted in displacement of the left colon medially, as well as, displacement of the aorta and IVC to the right. There was additional compression of the left ureter causing moderate hydronephrosis. The lesion measured 17 × 14.5 × 9.8 cm and was predominantly cystic, however, there was a 5.3 × 3.2 × 5 cm solid enhancing component. Axial pre-contrast (G), postcontrast (H), and 8-minute delay postcontrast (I) further shows the mass (white arrows) flattening the left psoas muscle (red asterisk). There is enhancement of the solid component (yellow arrow) on postcontrast images and persistent enhancement (yellow arrow) on delayed imaging.

Fig. 1

Demonstrates a large cystic and solid mass (white arrow) located within the left hemiabdomen with the superior portion abutting the lower pole of the left kidney. The solid component is mildly enhancing (yellow arrow). The mass extended into the pelvis and resulted in outward bulging of the left abdominal wall. The lesion resulted in displacement of the left colon medially, as well as, displacement of the aorta and IVC to the right. There was additional compression of the left ureter causing moderate hydronephrosis. The lesion measured 17 × 14.5 × 9.8 cm and was predominantly cystic, however, there was a 5.3 × 3.2 × 5 cm solid enhancing component. Axial pre-contrast (G), postcontrast (H), and 8-minute delay postcontrast (I) further shows the mass (white arrows) flattening the left psoas muscle (red asterisk). There is enhancement of the solid component (yellow arrow) on postcontrast images and persistent enhancement (yellow arrow) on delayed imaging.

Fig. 2

Images A (Low Power Photomicrograph at 20× magnification) and B (High Power Photomicrograph at 40× magnification) with hematoxylin and eosin stain. Photomicrograph demonstrates mucinous neoplasm with cytologic atypia, complex papillary growth, and multiple infiltrative foci of invasive carcinoma

Fig. 3

Shows thin-walled cystic structure measuring 18.5 × 15.5 × 12.5 cm, focally covered by pink, red congested fibro-membranous tissue consistent with peritoneum measuring 13.0 × 7.0 cm in surface area. The cyst contains brown, semi-cloudy fluid (red asterisk). The cyst lining demonstrates diffusely scattered areas of pink tan and is granular to nodular appearance, with the largest nodule measuring 6.0 × 5.0 × 2.5 cm (white arrow).