Retroperitoneal leiomyosarcoma fatal spontaneous rupture: literature review and presentation of extremely rare case

   Retroperitoneal sarcoma (RPS) is a heterogeneous group of malignant tumors with an incidence of approximately 1 case per 100,000 population per year, which includes leiomyosarcoma. Leiomyosarcoma is a malignant neoplasm with smooth muscles differentiation. It is the second most common sarcoma affecting the retroperitoneal space. In this article, we describe a case of fatal hematoma of the retroperitoneal space, caused by spontaneous rupture of leiomyosarcoma. A 65-year-old woman presented with severe pain in her left side and hypovolemic shock. After an additional examination of hemodynamic stabilization, she was operated on an emergency basis. The tumor was removed and the bleeding was stopped. The patient was transferred to the intensive care unit (ICU) after surgery, despite the ongoing intensive therapy, the condition remained extremely serious, against which the deterioration result from cardiac arrest and death is occurred. In the world literature, spontaneous rupture of retroperitoneal leiomyosarcoma is described in isolated cases. Moreover, the main publications are aimed at managing patients with tumors of the retroperitoneal space only in a planned manner. Thus, this case could be of clinical interest among emergency medical practitioners as well as in the scientific community. Literature search for a review of the problem was carried out in the following scientific databases and search engines: PubMed, Web of Science, Scopus, Google Scholar, eLIBRARY.

1. L. M. Almond, F. Tirotta, H. Tattersall, J. Hodson, T. Cascella, M. Barisella, A. Marchianò, G. Greco, A. Desai, S. J. Ford, A. Gronchi, M. Fiore, C. Morosi, Diagnostic accuracy of percutaneous biopsy in retroperitoneal sarcoma, British Journal of Surgery, Volume 106, Issue 4, March 2019, Pages 395 – 403.

2. Marko J., Wolfman D. J. Retroperitoneal Leiomyosarcoma from the Radiologic Pathology Archives. Radiographics. 2018 Sep-Oct; 38 (5): 1403-1420. doi: 10.1148/rg.2018180006. PMID: 30207936; PMCID: PMC6166742.

3. van Houdt W. J., Raut C. P., Bonvalot S., Swallow C. J., Haas R., Gronchi A. New research strategies in retroperitoneal sarcoma. The case of TARPSWG, STRASS and RESAR: making progress through collaboration. Curr Opin Oncol. 2019 Jul; 31 (4): 310-316. doi: 10.1097/CCO.0000000000000535. PMID: 30893150.

4. van Houdt W. J., Zaidi S., Messiou C., Thway K., Strauss D. C., Jones R. L. Treatment of retroperitoneal sarcoma: current standards and new developments. Curr Opin Oncol. 2017 Jul; 29 (4): 260-267. doi: 10.1097/CCO.0000000000000377. PMID: 28509807.

5. Grasso M., Blanco S., Fortuna F., Crippa S., Di Bella C. Spontaneous rupture of renal leiomyosarcoma in a 45-year-old woman. Archivos Espanoles de Urologia. 2004 Oct; 57 (8): 870-872. PMID: 15560282.

6. Cantwell C. P., Stack J. Abdominal aortic invasion by leiomyosarcoma. Abdom Imaging 31, 120 – 122 (2006). doi: 10.1007/s00261-005-0163-5.

7. Tamaki M., Terai A., Terachi T., et al. [A case of incidental renal leiomyosarcoma] Hinyokikakiyo. Acta Urologica Japonica. 1994 May; 40 (5): 415-418. PMID: 8023763.

8. Aksoy Y., Gürsan N., Ozbey I., Biçgi O., Keleş M. Spontaneous rupture of a renal angiosarcoma. Urol Int. 2002; 68 (1): 60-2. doi: 10.1159/000048419. PMID: 11803270.

9. Girszyn N., Mabro M., Kahn J. E., Roullet-Audy J. C., Sautet A., Bletry O. Synovialosarcome rétropéritonéal révélé par un choc hémorragique [Primary retroperitoneal synovial sarcoma revealed by hemorrhagic shock]. Presse Med. 2006 Jul-Aug;35 (7-8):1185-7. French. doi: 10.1016/s0755-4982(06)74778-9. PMID: 16840897.

10. Samà L., Tzanis D., Bouhadiba T., Bonvalot S. Emergency Retroperitoneal Sarcoma Surgery for Preoperative Rupture and Hemoperitoneum: A Case Report. Cureus. 2021 Mar 17; 13 (3): e13936. doi: 10.7759/cureus.13936. PMID: 33868865; PMCID: PMC8050804.

11. Al Sheikh M., Simson N., Obi-Njoku O., Qteishat A. Acute haemorrhage from a retroperitoneal liposarcoma: a rare presentation. BMJ Case Rep. 2018 Feb 8; 2018: bcr 2017222737. doi: 10.1136/bcr-2017-222737. PMID: 29437802; PMC43.

12. Samà L., Tzanis D., Bouhadiba T., et al. (March 17, 2021) Emergency Retroperitoneal Sarcoma Surgery for Preoperative Rupture and Hemoperitoneum: A Case Report. Cureus 13 (3): e13936. doi:10.7759/cureus.13936.