Discussion
RPS are relatively uncommon, accounting only for 10-15% of all soft tissue sarcomas. [2] Most tumors are already large and locally advanced when they are first detected (median size at diagnosis is about 15 cm.). [9] Rarely may patients with rapidly expanding high-grade tumors experience flu-like symptoms and present with fevers and leukocytosis. [10,11] Radiographic imaging is a key component of evaluating a patient with a retroperitoneal mass. The preferred diagnostic examination is a contrast-enhanced computed tomography (CT) scan of the abdomen and pelvis to evaluate the primary site. MRI with gadolinium is reserved for patients with an allergy to iodinated contrast agents or with an equivocal muscle or bone involvement on CT. MRI may also be useful for delineating diseases in the pelvis. For patients with preoperative radiation therapy, MRI is useful to assess the local tumor extent and surrounding edema, which are optimally included in the treatment volume. [12] In this case, we assumed both sonography and MRI modality. Ultrasound reports revealed a large myoma; however, MRI revealed well-defined heterogeneous soft-tissue mass, which seemed to be malignant; however, it could not specify the origin. The retro peritoneum is the space posterior to the peritoneal cavity and anterior to the para spinous musculature. [13] The structures of the retro peritoneum include the kidneys, adrenal glands, and bilateral perirenal fat, the aorta and its major branches (e.g., renal arteries), the inferior vena cava and its major tributaries (e.g., renal veins), and the bilateral iliac vessels (namely common, internal, external arteries/veins), the duodenum, and the pancreas. In our case, there was a mass originated in this space, probably from an iliac vein with adhesions to the ureter. Needless to mention that there are several vital structures in the retroperitoneal space, and in our patient, because of the history of three cesarean sections, besides the existence of this mass, accessing the plane for dissection was abstruse. Determining respectability largely depends on the extension of the tumor and the surrounding structures it involves. A relative contraindication is vascular involvement because reconstructive options vary, given tumor location and tissue involvement. [14] We confronted a retroperitoneal mass instead of uterus leiomyoma, and supposing RPL, the ability to perform a complete surgical resection at the time of initial presentation, is the most important prognostic factor for survivall.[10,11,15-20] Remarkably, the five-year disease-free survival rate of patients undergoing resection ranged from 30 to 60%; however, in those with no surgery, the survival was often below one year. [4-8] Our patient was a married 46-year-old woman, and we decided to have complete resection for her survival. Extensive vascular involvement (aorta, vena cava, and/or iliac vessels), although involvement of the vena cava and iliac veins is a relative, rather than absolute, contraindication for respectability, as these vessels can often be ligated or replaced with interposition grafts. [21] Despite distorted anatomy, we dissected the tumor through adhesions from adjacent structures. Managing bulky intra-abdominal extra-luminal tumors is challenging due to their proximity or diffusion of other structures. The surgical resection of retroperitoneal leiomyosarcoma (RPLM) can be associated with significant morbidity, given that they usually invade main vascular structures such as the inferior vena cava (IVC) and tributaries, the duodenum, and the ureter. [22] Management needs to be provided by a specialized team of surgeons. Complete resection often needs the extended dissection of the vascular structures, kidneys, bladder, and gastrointestinal tract. [23] In our experience, we had Urethral Injury that Primary repaired, and DJ placement was done with the cooperation of a urologist. The main challenge was the right internal iliac vein torn during the dissection of the mass. The vascular surgeon repaired the vein. The blood loss estimation made us and the anesthesiology team decide to have the transfusion of three units of Pack-cell during operation.
The intraoperative frozen section analysis suggested smooth muscle tumor without its benign or malignant being determined. Hysterectomy and bilateral salpangectomy was performed, and the ovaries were preserved. She had a fine post-operation course without acute complications. The patient was discharged 72 hours after surgery, and DJ was removed six weeks later with no complication. The permanent pathology report showed LMS; however, the margin could not be assessed. No pathological finding was reported on the uterus, cervix, and fallopian tubes. Finally, she was a candidate for radiation. Van Doorn et al.[24] reported postoperative high-dose radiation therapy in 13 out of 34 RPS patients with significantly decreased recurrence rate. However, the benefits and the effectiveness of radiation therapy have not been rigorously investigated due to the scarcity of cases. Our patient underwent 25 sessions for external radiotherapy. Any discussion on this case would be incomplete without commenting on the ethical aspects of the consent process. she declared that she would be happy if the other doctors and medical students learned from the case and did not mind her condition being discussed. After six months of treatment, her health status is favorable, and there is no recurrence or metastasis.
The COVID-19 pandemic has increased the complexity of cancer care. In this regard, the main issues are balancing the risk of delaying cancer treatment versus COVID-19 damage, minimizing the number of clinic and hospital visits to reduce exposure, mitigating the adverse effects of social distancing on the care delivery, and allocating limited health care resources appropriately and fairly. Because of the rarity of these tumors and the complexity of treatment, evaluation and management should ideally be carried out in centers equipped with multidisciplinary expertise in treating sarcomas in a multidisciplinary tumor board.
Authors’ contributions :
A.S. : study concept or design, Investigation performed the surgery
Sh.Sh. : performed the surgery
P.M. : performed the surgery.
A.SH. : Writing
Z.F. : data collection, Imaging determination
Z.N. : Writing - Review & Editing; critical revision, study concept, performed the surgery