Obstet Gynecol Sci.  2014 Jan;57(1):77-81.

Unusual presentation of retroperitoneal leiomyosarcoma mimicking an adnexal tumor with highly elevated serum CA-19-9

• Cho DH
• Lee JH
• Oh BC

Abstract

When retroperitoneal leiomyosarcoma develops in pelvic cavity, it often presents similar symptoms and radiological findings to adnexal tumor, therefore obscures diagnostic approaches until an exploratory laparotomy is performed. We report an unusual presentation of retroperitoneal leiomyosarcoma mimicking an adnexal tumor with extremely elevated serum CA-19-9. Though the most of the prominent mass was removed during surgery, there was massive bleeding due to tearing of internal iliac vein while dissecting the ureter close to vessels. This case focuses on the significance of considering retroperitoneal tumor even if the mass is located in ovarian fossa and has highly elevated serum level of CA-19-9. And in attempt of tumor removal, the excision needs to be clean-cut without damaging nerves or vessels around the mass and avoid causing any prospective complications.

Keyword

Adnexal tumor; CA-19-9; Leiomyosarcoma; Retroperitoneal

MeSH Terms

Hemorrhage
Iliac Vein
Laparotomy
Leiomyosarcoma*
Ureter

Figure

• 

  Fig. 1 (A) A large, lobulated, and heterogenously enhancing mass occupies the right retroperitoneal space (arrow). (B) Intraoperative findings: a 9.4 × 6.3 × 5.1 cm sized smooth round mass (arrow) surrounded by light grayish membrane is attached to right retroperitoneal wall close to the right adnexa (arrowhead).

• 

  Fig. 2 Microscopic features of retroperitoneal leiomyosarcoma. (A) Low magnification of tumor showing intersecting fascicles (H&E, ×100). (B) Tumor cells show strong reactivity for smooth muscle actin (smooth muscle actin, ×400).

Reference

1. Kransdorf MJ. Malignant soft-tissue tumors in a large referral population: distribution of diagnoses by age, sex, and location. AJR Am J Roentgenol. 1995; 164:129–134.

2. Felix EL, Wood DK, Das Gupta TK. Tumors of the retroperitoneum. Curr Probl Cancer. 1981; 6:1–47.

3. Bretan PN Jr, Williams RD, Hricak H. Preoperative assessment of retroperitoneal pathology by magnetic resonance imaging: primary leiomyosarcoma of inferior vena cava. Urology. 1986; 28:251–255.

4. Hill MA, Mera R, Levine EA. Leiomyosarcoma: a 45-year review at Charity Hospital, New Orleans. Am Surg. 1998; 64:53–60.

5. Dalal KM, Kattan MW, Antonescu CR, Brennan MF, Singer S. Subtype specific prognostic nomogram for patients with primary liposarcoma of the retroperitoneum, extremity, or trunk. Ann Surg. 2006; 244:381–391.

6. Lewis JJ, Leung D, Woodruff JM, Brennan MF. Retroperitoneal soft-tissue sarcoma: analysis of 500 patients treated and followed at a single institution. Ann Surg. 1998; 228:355–365.

7. Van Rooij WJ, Martens F, Verbeeten B Jr, Dijkstra J. CT and MR imaging of leiomyosarcoma of the inferior vena cava. J Comput Assist Tomogr. 1988; 12:415–419.

8. Marcouizos G, Ignatiadou E, Papanikolaou GE, Ziogas D, Fatouros M. Highly elevated serum levels of CA-19-9 in choledocholithiasis: a case report. Cases J. 2009; 2:6662.

9. Guz BV, Wood DP Jr, Montie JE, Pontes JE. Retroperitoneal neural sheath tumors: Cleveland Clinic experience. J Urol. 1989; 142:1434–1437.

10. Hueman MT, Herman JM, Ahuja N. Management of retroperitoneal sarcomas. Surg Clin North Am. 2008; 88:583–597.

11. Gronchi A, Casali PG, Fiore M, Mariani L, Lo Vullo S, Bertulli R, et al. Retroperitoneal soft tissue sarcomas: patterns of recurrence in 167 patients treated at a single institution. Cancer. 2004; 100:2448–2455.

12. Zhang G, Chen KK, Manivel C, Fraley EE. Sarcomas of the retroperitoneum and genitourinary tract. J Urol. 1989; 141:1107–1110.

13. Van Doorn RC, Gallee MP, Hart AA, Gortzak E, Rutgers EJ, van Coevorden F, et al. Resectable retroperitoneal soft tissue sarcomas: the effect of extent of resection and postoperative radiation therapy on local tumor control. Cancer. 1994; 73:637–642.

14. Todd CS, Michael H, Sutton G. Retroperitoneal leiomyosarcoma: eight cases and a literature review. Gynecol Oncol. 1995; 59:333–337.

15. Sutton G, Blessing JA, Malfetano JH. Ifosfamide and doxorubicin in the treatment of advanced leiomyosarcomas of the uterus: a Gynecologic Oncology Group study. Gynecol Oncol. 1996; 62:226–229.