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\begin{document}

\title{Rare presentation of retroperitoneal leiomyosarcoma mimicking a myoma in
a 46-year-old woman: a case report}



\author[1]{Setareh Akhavan}%
\author[1]{Shahrzad   Sheikhhasani}%
\author[2]{Mohades Peydayesh}%
\author[1]{Shima Alizadeh}%
\author[1]{Fatemeh  Zamani}%
\author[1]{narges zamani}%
\affil[1]{Tehran University of Medical Sciences}%
\affil[2]{Bam University of Medical Sciences}%


\vspace{-1em}



  
  \date{May 25, 2024}


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\selectlanguage{english}
\begin{abstract}
Retroperitoneal sarcoma is relatively uncommon. We share our experience
in encountering retroperitoneal sarcoma with vascular and urethral
adhesion in a 46-year-old woman. Given the rarity of these tumors,
evaluation and management should ideally be performed in a center
equipped with multidisciplinary expertise in treating sarcomas.%
\end{abstract}%



\sloppy


\textbf{Introduction}

LMS, a subset of soft tissue sarcomas, refers to malignant smooth muscle
neoplasms accounting for about 5-10\% of all sarcomas. Natural history
depends on the anatomic location where they arise. About 50\% arise in
the retroperitoneum/abdomen, including the visceral, uterine, and
retroperitoneal, with the uterus being the most common location. Given
these tumors' enormous size and frequent location, abdominal pain,
nausea, vomiting, anorexia, weight loss, fatigue, and malaise are their
most common symptoms. {[}1{]} Retroperitoneal sarcoma (RPS) is
relatively uncommon, accounting only for 10-15\% of all soft tissue
sarcomas. {[}2{]} In a population-based series from the Surveillance,
Epidemiology, and End Results (SEER) database, the average annual
prevalence of RPS was approximately 2.7 cases per one million
population. {[}3{]} The LMS of the retroperitoneum arise from the
inferior vena cava, its tributaries, or any small vessel. When
diagnosed, they often emerge as a mass, usually in an enormous size.
Most knowledge in treating inferior vena cava (IVC) LMS comes from case
reports and case series. Surgical resection offers the best chance of
long-term survival and for the likelihood of cure. Remarkably, the
five-year disease-free survival rate of patients undergoing resection
was between 30and 60\%; however, the survival in those who did not
undergo surgery was often below one year. {[}4-8{]} We aimed to share
our experience in encountering RPL with vascular and ureteral adhesion
in the operation room about a patient scheduled for surgery as a case of
leiomyoma.

\textbf{Case presentation}

A 46-year-old Gravida 3 para 3~woman with no medical, allergy, and drug
use history and with a history of abdominal pain, hypermenorrhea, and
pelvic pressure was referred to the gynecology and oncology ward of a
university Hospital a few months ago. In the vaginal exam, there was
bulging in the right side, which completely deviated the cervix to the
left and upward position. On abdominal sonography, the uterus size was
63*42 mm with an endometrial thickness of 9 mm. A heterogeneous
114*99-mm mass in a posterior cul-de-sac, probably with the uterine
origin, was detected. Magnetic Resonance Imaging (MRI) revealed a
well-defined heterogeneous soft-tissue mass from degeneration and
necrosis in the pelvic cavity posterior to the uterus was observed and
separated from the uterus and ovaries. In post-contrast images,
heterogeneous enhancement at the tumor was noticed (Figures 1\&2). This
mass lesion posed pressure on the right ureter, causing
hydroureteronephrosis, and was in close contact with the right external
iliac vein (Figure3). She had a surgical history of cesarean section
with\emph{pfannenstiel incision} for three times, and her last child was
ten years old. The attempts for ureteral stent placement before surgery
had failed; hence, she was scheduled for surgery. The surgery was
performed by the fellows of oncogynecology under the direct supervision
of their attending surgeon. She underwent an exploratory laparotomy
under the general anesthesia by a midline incision on the suspicion of
leiomyoma. A huge solid retroperitoneal mass (16*12*11 cm) was detected
in the right broad ligament, which was attached to the pelvic floor and
seemed to be separated from the uterus. Surgical excision aimed to
resect the tumor completely. Because of the dense adhesion of the tumor
to the ureter and vessels, the internal iliac vein and ureteral injury
were torn during dissection. The internal iliac vein was repaired by a
vascular surgeon. The intraoperative frozen section analysis suggested
smooth muscle tumor without determining its being benign or malignant.
Hysterectomy and bilateral salpangectomy was performed, and the ovaries
were preserved.

An internal ureteral DJ stent was inserted, and the primary ureteral was
repaired by the urologist. After consulting with an anesthesiologist, we
transfused three-unit Pack Cell to the patient during the operation due
to blood loss. The oral diet began one day after surgery, and the
patient was discharged 72 hours after admission. Before surgery, Hb was
10.5, and the patient was discharged with Hb=9.8. Ureteral DJ was
removed six weeks later with no complication. Pathologic examination
revealed grade II / III LMS with the mitotic rate of 12 / 10 HPF,
including atypical forms. Necrosis extent was \textless{}50\%, and the
margin could not be assessed. The pathologic stage was pT4. The patient
was a candidate for radiation; hence, external beam radiotherapy was
provided for her for 25 sessions.

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. {[}\emph{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.{[}\emph{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.

\textbf{Authors'} \textbf{contributions} :

\textbf{A.S.} : study concept or design, Investigation performed the
surgery

\textbf{Sh.Sh.} : performed the surgery

\textbf{P.M.} : performed the surgery.

\textbf{A.SH.} : Writing

\textbf{Z.F.} : data collection, Imaging determination

\textbf{Z.N.} : Writing - Review \& Editing; critical revision, study
concept, performed the surgery

\textbf{References:}

{[}1{]} Bonvalot S, Rivoire M, Castaing M, Stoeckle E, Le Cesne A, Blay
JY, Laplanche A. Primary retroperitoneal sarcomas: a multivariate
analysis of surgical factors associated with local control. J Clin
Oncol. 2009 Jan 1;27(1):31-7. doi: 10.1200/JCO.2008.18.0802. Epub 2008
Dec 1. PMID: 19047280.

{[}2{]} Raut CP, Pisters PW. Retroperitoneal sarcomas: Combined-modality
treatment approaches. J Surg Oncol. 2006 Jul 1;94(1):81-7. doi:
10.1002/jso.20543. PMID: 16788949.

{[}3{]}Porter GA, Baxter NN, Pisters PW. Retroperitoneal sarcoma: a
population-based analysis of epidemiology, surgery, and radiotherapy.
Cancer. 2006 Apr 1;106(7):1610-6. doi: 10.1002/cncr.21761. PMID:
16518798.

{[}4{]} Mann GN, Mann LV, Levine EA, Shen P. Primary leiomyosarcoma of
the inferior vena cava: a 2-institution analysis of outcomes. Surgery.
2012 Feb;151(2):261-7. doi: 10.1016/j.surg.2010.10.011. Epub 2010 Dec
22. PMID: 21176932.

{[}5{]} Ito H, Hornick JL, Bertagnolli MM, George S, Morgan JA, Baldini
EH, Wagner AJ, Demetri GD, Raut CP. Leiomyosarcoma of the inferior vena
cava: survival after aggressive management. Ann Surg Oncol. 2007
Dec;14(12):3534-41. doi: 10.1245/s10434-007-9552-z. Epub 2007 Sep 25.
PMID: 17896156.

{[}6{]}Kieffer E, Alaoui M, Piette JC, Cacoub P, Chiche L.
Leiomyosarcoma of the inferior vena cava: experience in 22 cases. Ann
Surg. 2006 Aug;244(2):289-95. doi: 10.1097/01.sla.0000229964.71743.db.
PMID: 16858193; PMCID: PMC1602179.

{[}7{]} Illuminati G, Calio' FG, D'Urso A, Giacobbi D, Papaspyropoulos
V, Ceccanei G. Prosthetic replacement of the infrahepatic inferior vena
cava for leiomyosarcoma. Arch Surg. 2006 Sep;141(9):919-24; discussion
924. doi: 10.1001/archsurg.141.9.919. PMID: 16983035.

{[}8{]}.~Hollenbeck ST, Grobmyer SR, Kent KC, Brennan MF. Surgical
treatment and outcomes of patients with primary inferior vena cava
leiomyosarcoma. J Am Coll Surg. 2003 Oct;197(4):575-9. doi:
10.1016/S1072-7515(03)00433-2. PMID: 14522326..

{[}9{]} Stoeckle E, Coindre JM, Bonvalot S, Kantor G, Terrier P,
Bonichon F, Nguyen Bui B; French Federation of Cancer Centers Sarcoma
Group. Prognostic factors in retroperitoneal sarcoma: a multivariate
analysis of a series of 165 patients of the French Cancer Center
Federation Sarcoma Group. Cancer. 2001 Jul 15;92(2):359-68. doi:
10.1002/1097-0142(20010715)92:2\textless{}359::aid-cncr1331\textgreater{}3.0.co;2-y.
PMID: 11466691.

{[}10{]} Weiss SW, Rao VK. Well-differentiated liposarcoma (atypical
lipoma) of deep soft tissue of the extremities, retroperitoneum, and
miscellaneous sites. A follow-up study of 92 cases with analysis of the
incidence of ''dedifferentiation''. Am J Surg Pathol. 1992
Nov;16(11):1051-8. doi: 10.1097/00000478-199211000-00003. PMID: 1471725.

{[}11{]} Storm FK, Mahvi DM. Diagnosis and management of retroperitoneal
soft-tissue sarcoma. Ann Surg. 1991 Jul;214(1):2-10. doi:
10.1097/00000658-199107000-00002. PMID: 2064467; PMCID: PMC1358407.

{[}12{]} Messiou C, Moskovic E, Vanel D, Morosi C, Benchimol R, Strauss
D, Miah A, Douis H, van Houdt W, Bonvalot S. Primary retroperitoneal
soft tissue sarcoma: Imaging appearances, pitfalls and diagnostic
algorithm. Eur J Surg Oncol. 2017 Jul;43(7):1191-1198. doi:
10.1016/j.ejso.2016.10.032. Epub 2016 Dec 9. PMID: 28057392.

{[}13{]} Liles JS, Tzeng CW, Short JJ, Kulesza P, Heslin MJ.
Retroperitoneal and intra-abdominal sarcoma. Curr Probl Surg. 2009
Jun;46(6):445-503. doi: 10.1067/j.cpsurg.2009.01.004. PMID: 19414097.

{[}14{]} Jaques DP, Coit DG, Hajdu SI, Brennan MF. Management of primary
and recurrent soft-tissue sarcoma of the retroperitoneum. Ann Surg. 1990
Jul;212(1):51-9. doi: 10.1097/00000658-199007000-00008. PMID: 2363604;
PMCID: PMC1358074

{[}15{]} 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 Sep;228(3):355-65. doi:
10.1097/00000658-199809000-00008. PMID: 9742918; PMCID: PMC1191491.

{[}16{]} Hassan I, Park SZ, Donohue JH, Nagorney DM, Kay PA, Nasciemento
AG, Schleck CD, Ilstrup DM. Operative management of primary
retroperitoneal sarcomas: a reappraisal of an institutional experience.
Ann Surg. 2004 Feb;239(2):244-50. doi:
10.1097/01.sla.0000108670.31446.54. PMID: 14745333; PMCID: PMC1356218.

{[}17{]} Catton CN, O'Sullivan B, Kotwall C, Cummings B, Hao Y,
Fornasier V. Outcome and prognosis in retroperitoneal soft tissue
sarcoma. Int J Radiat Oncol Biol Phys. 1994 Jul 30;29(5):1005-10. doi:
10.1016/0360-3016(94)90395-6. PMID: 8083069.

{[}18{]} Lehnert T, Cardona S, Hinz U, Willeke F, Mechtersheimer G,
Treiber M, Herfarth C, Buechler MW, Schwarzbach MH. Primary and locally
recurrent retroperitoneal soft-tissue sarcoma: local control and
survival. Eur J Surg Oncol. 2009 Sep;35(9):986-93. doi:
10.1016/j.ejso.2008.11.003. Epub 2009 Jan 12. PMID: 19138832.

{[}19{]} Anaya DA, Lev DC, Pollock RE. The role of surgical margin
status in retroperitoneal sarcoma. J Surg Oncol. 2008 Dec
15;98(8):607-10. doi: 10.1002/jso.21031. PMID: 19072853.

{[}20{]} Pierie JP, Betensky RA, Choudry U, Willett CG, Souba WW, Ott
MJ. Outcomes in a series of 103 retroperitoneal sarcomas. Eur J Surg
Oncol. 2006 Dec;32(10):1235-41. doi: 10.1016/j.ejso.2006.07.002. Epub
2006 Aug 21. PMID: 16919908.

{[}21{]} Avanc\selectlanguage{ngerman}ès C, Mottet N, Mahatmat A, Chapuis E, Serre I, Culine S.
Prognostic factors for first recurrence in patients with retroperitoneal
sarcoma. Urol Oncol. 2006 Mar-Apr;24(2):94-6. doi:
10.1016/j.urolonc.2005.09.004. PMID: 16520270.

.

{[}22{]} Petrou A, Constantinidou A, Kontos M, Papalampros A, Moris D,
Bakoyiannis C, Neofytou K, Kourounis G, Felekouras E. Comprehensive
Surgical Treatment as the Mainstay of Management in Retroperitoneal
Sarcomas: Retrospective Study from Two Non-sarcoma Specialist Centers.
Anticancer Res. 2017 Apr;37(4):2025-2031. doi:
10.21873/anticanres.11547. PMID: 28373477.

{[}23{]} Raut CP, Pisters PW. Retroperitoneal sarcomas:
Combined-modality treatment approaches. J Surg Oncol. 2006 Jul
1;94(1):81-7. doi: 10.1002/jso.20543. PMID: 16788949.

{[}24{]} van Doorn RC, Gallee MP, Hart AA, Gortzak E, Rutgers EJ, van
Coevorden F, Keus RB, Zoetmulder FA. Resectable retroperitoneal soft
tissue sarcomas. The effect of extent of resection and postoperative
radiation therapy on local tumor control. Cancer. 1994 Feb
1;73(3):637-42. doi:
10.1002/1097-0142(19940201)73:3\textless{}637::aid-cncr2820730322\textgreater{}3.0.co;2-y.
PMID: 8299085.

Figure legand:

Figure 1. Axial T1-weighted fat-suppressed (A), T2-weighted (B), and
gadolinium-enhanced T1-weighted fat-suppressed (C) MR images show a
large relatively well-defined, homogeneous, non--fat-containing solid
intra-abdominal mass that is isointense to skeletal muscle on
T1-weighted images (arrows on A), heterogeneously T2 hyperintense
(arrows on B), and heterogeneously enhanced (arrows on C). Uterus=U on
A-C.

Figure 2. Axial gadolinium-enhanced T1-weighted fat-suppressed (A, B)
manifest normal uterus (arrow on A, B)and normal ovaries(arrow head on
A,B).

Figure3.Coronal and axial gadolinium-enhanced T1-weighted fat-suppressed
(A-C) MR images show right side hydroureteronephrosis(arrow on A and B)
and right external iliac vein(arrow on C).\selectlanguage{english}
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