Giant retroperitoneal leiomyosarcoma and multiorgan block removal - Beyond the Abstract

Retroperitoneal sarcomas include tumors that are located in the retroperitoneal space and do not originate in any of the organs that sit there. Due to its location the treatment of these tumors enter in the field of urology and general surgery, being treated in 60% of cases by urologists and 40% by general surgeons (1).

Because of their low prevalence there are still many points of discussion in terms of diagnosis and especially in its treatment. They are not demonstrated the existence of genetic or environmental factors in the development of sarcomas, but certain genetic syndromes are associated with an increased risk of development of this tumors. Among them, the Von Recklinghausen disease, hereditary retinoblastoma and Li-Fraumeni syndrome. In relation to the diagnosis, computed tomography is the most sensitive test. Classic angiography has been replaced by CT angiography with three-dimensional reconstruction and MRI in order to diagnose their relationship with the great vessels. The realisation of preoperative biopsy is not required for patients who go directly to the operating room. It is reasonable the filling of the preoperative study with a biopsy when neoadjuvant therapies are proposed, or the tumor is of uncertain diagnosis, as in the lymphoproliferative process or metastatic testicular neoplasia (2). The biopsy is usually performed by radiologist with a retroperitoneal core needle biopsy. However sometimes open excisional biopsy is needed and is only in this indication where minimally invasive surgery may have some field in this surgery.

Neoadjuvant chemotherapy has so far demostrated benefit in the treatment of retroperitoneal sarcoma, while radiotherapy has been used but without a level I evidence demonstrating improvements in survival. However it appears to reduce the risk of local recurrence so their use has increased from 3% of patients in 2005 to 10% in 2011 (3). It has been shown that after its use there is no significant difference in terms of morbidity, mortality or hospitalization time. Regarding surgery, circumscribed and pseudoencapsulated presence of this tumor is misleading, providing easy cleavage plane adjacent to the tumor boundary, but this pseudocapsule is always infiltrated (4). Thus, the aim of surgery is to remove the tumor with an impeller block of healthy tissue around including adjacent viscera for this whenever possible. Up to 50% of cases required resection of adjacent organs: kidneys and adrenals in 46%; colon in 24%; pancreas in 15% and 10% in the spleen (5). In-block resection has decreased abdominal recurrence three times. The tumor extension should be carefully evaluated before surgery. The expansion through the diaphragm, inguinal canal or foramen obturator, sciatic or spinal, conditions the surgical approach (6). The tumors that extend through the diaphragm require a thoracoabdominal approach generally. Involvement of the inguinal canal, forces a laparotomy that usually affects the groin and upper thigh. When they grow along the sciatic foramen a laparotomy with a subsequent combined approach through the gluteal area is needed.

Nowadays the two absolute contraindication to resection are the involvements of the mesenteric vessels and diffuse peritoneal dissemination. The rate of recurrence after complete resection is up to 45%, appreciating a loss of cell differentiation in 25% cellularity at first relapse and 44% in the second one (7). Debulking surgery for primary disease may play some role in highly selected patients with very slow tumor growth or for the relief of symptoms. Shibata et al, found in 55 patients with unresectable liposarcomas an improvement of survival of 4 months with biopsy alone to 26 months with cytoreductive surgery (8). Surgical resection of local recurrence is usually more difficult than resection for the primary disease and its recurrence rate is much higher. It is difficult to decide when operating in relapsed. Patients with recurrence, and apparently resectable disease, usually are operated. An observational strategy can be followed in cases with aggressive tumor biology in high-risk patients and in those with tumors of low histological grade and biology of slow growth (9).

Leiomyosarcomas have a lower rate of local recurrence and a high rate of hematic dissemination, being the lung and liver the most frequent places of metastasis.

Adjuvant chemotherapy has been used empirically using regimens that include a large number of drugs: adtriamicina, cyclophosphamide, doxorubicin, ifosfamide, pazopanib, trabectedin, etc, until now they have not demostrated benefit outside the research results. Currently some working groups are investigating whether intraoperative hyperthermic chemotherapy could reduce locoregional recurrence (10).

Although in many cases the kidney appears affected by sarcoma, infiltration is less frequently observed. While the high vessel resection is not a common practice, only in 10% of cases, they are infiltrates up to 67% of cases (10). Therefore, the urologist should always try to save the kidney whereever is possible and for that many authors have described techniques such as autologous renal transplant, renal and ureteral resection and ureteral derivations. All publications agree on the need for treatment of these patients with an experienced multidisciplinary units. They must counts with the present oncologists, radiation oncologists, pathologists, urologists and general and vascular surgeons minimum.

1. Virseda Rodríguez JA, Donate Moreno MJ, Pastor Navarro H, Carrión López P, Martínez Ruiz J et al. Tumores Retroperitoneales Primarios. Revisión de nuestros casos de los diez últimos años. Arch Esp Urol 2010; 63: 13-22
2. Tan MC, Yoon SS. Surgical Management of Retroperitoneal and Pelvic Sarcomas. J Surg Oncol 2015 Apr;111(5):553-61. doi: 10.1002/jso.23840. Epub 2014 Dec 5.
3. Nussbaum DP, Speicher PJ, Gulack BC, Ganapathi AM, Keenan JE, et al. The effect of neoadjuvant radiation therapy on perioperative outcomes among patients undergoing resection of retroperitoneal sarcomas. Surg Oncol. 2014 Sep;23(3):155-60. doi: 10.1016/j.suronc.2014.07.001. Epub 2014 Jul 23
4. Garcia del Muro X, Martín J, Maurel J, Cubedo R, Bagué S et al. Guía de Práctica Clínica en los Sarcomas de Partes Blandas. Med Clin 2011;136: 408.e1-408.e18
5. Absalón Medina-Villaseñor E, Martínez Macias R, Díaz Rodríguez L, Barra Martínez R, Garza navarro JM et al. Sarcomas Retroperitoneales. Cirujano General 2006: 28: 77-82
6. Bonvalot S, Raut CP, Pollock RE, Rutkowski P, Strauss DC, et al. Technical considerations in surgery for retroperitoneal sarcomas: position paper from E-Surge, a master class in sarcoma surgery, and EORTC-STBSG. Ann Surg Oncol. 2012 Sep;19(9):2981-91. doi: 10.1245/s10434-012-2342-2. Epub 2012 Apr 3
7. Caizzone A, Saladino E, Fleres F, Paviglianiti C, Laropoli F, et al. Giant Retroperitoneal Liposarcoma: Case Report and Review of the Literature. Int J Surg Case Rep. 2015;9:23-6. doi: 10.1016/j.ijscr.2015.02.019. Epub 2015 Feb 17
8. Shibata D, Lewis JJ, Leung DH, Brennan MF. Is there a role for incomplete resection in the management of retroperitoneal liposarcomas? J Am Coll Surg. 2001 Oct;193(4):373-9.
9. Angele MK, Albertsmeier M, Prix NJ, Hohenberger P, Abdel-Rahman S, et al. Effectiveness of regional hyperthermia with chemotherapy for high-risk retroperitoneal and abdominal soft-tissue sarcoma after complete surgical resection: a subgroup analysis of a randomized phase-III multicenter study. Ann Surg.. 2014 Nov;260(5):749-54; discussion 754-6. doi: 10.1097/SLA.0000000000000978
10. Toulmonde M, Bonvalot S, Méeus P, Stoeckle E, Riou O, et al. French Sarcoma Group. Retroperitoneal sarcomas: patterns of care at diagnosis, prognostic factors and focus on main histological subtypes: a multicenter analysis of the French Sarcoma Group. Ann Oncol. 2014 Mar;25(3):735-42. doi: 10.1093/annonc/mdt577.

Written by:
Mateo Vallejo F, Dominguez Reinado MR, Medina Achirica C, Diaz Oteros M, Esteban Ramos JL, Melero Brenes S.
Hospital of Jerez de la Frontera, N-IVa Road s/n, Jerez, Cadiz, Spain.

Abstract: Giant retroperitoneal leiomyosarcoma and multiorgan block removal.