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