International Journal of Surgery Case Reports 114 (2024) 109118

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International Journal of Surgery Case Reports

journal homepage: www.elsevier.com/locate/ijscr

Case report

Solitary fibrous tumor in the retroperitoneum: A case report

Lenggo Septiady Putra a, *, Rachmat Budi Santoso b, Edward Usfie Harahap b, Dian Cahyanti c,
Ikhlas Arief Bramono b, Agus Rizal A.H. Hamid a
a
Department of Urology, Faculty of Medicine, Universitas Indonesia – Cipto Mangunkusumo National Referral Hospital, Jakarta, Indonesia
b
Department of Urology, National Cancer Centre - Dharmais Cancer Hospital, Jakarta, Indonesia
c
Department of Pathological Anatomy, National Cancer Centre - Dharmais Cancer Hospital, Jakarta, Indonesia

A R T I C L E I N F O A B S T R A C T

Keywords: Introduction and importance: Solitary fibrous tumor (SFT) is an uncommon mesenchymal tumor that can manifest
Case report in a variety of locations, including the retroperitoneum. The most effective standard diagnostic approach and
Malignancy treatment is yet to be determined due to unpredictable behavior of SFT, including retroperitoneal SFT.
Solitary fibrous tumor
Case presentation: A 43-year-old female with a retroperitoneal SFT presented with a palpable mass and symp­
Retroperitoneal tumor
tomatology. Surgical exploration disclosed a tumor encompassing the left renal artery and vein, necessitating left
nephrectomy and retroperitoneal mass removal. Initial histological examination suggested rhabdomyosarcoma,
but subsequent immunohistochemistry confirmed the diagnosis of retroperitoneal SFT. No adjuvant therapy was
administered, and there was no detectable mass on follow-up imaging. The patient remained symptom-free.
Clinical discussion: Retroperitoneal SFTs are difficult to diagnose due to their non-specific morphology, thus
immunohistochemistry plays a crucial role in confirming its diagnosis. Surgical excision with negative resection
margins continues to be the standard treatment. Recurrence rates are low in comparison to other retroperitoneal
sarcomas, hence routine chemotherapy or radiation therapy is not advised.
Conclusion: This case demonstrates the significance of contemplating SFT as the differential diagnosis of retro­
peritoneal tumors and the role of immunohistochemistry in confirming the diagnosis. The optimal management
strategies for retroperitoneal SFTs should be determined by additional research.

1. Introduction 2. Case presentation

Solitary fibrous tumor (SFT) is an uncommon form of spindle-cell
mesenchymal tumor that comprises a wide range of benign and malig­
nant neoplasms. Mostly, they are located inside the pleural cavity,
however, around 30 % of cases develop outside of the pleura, including
the mediastinum, pericardium, peritoneum, retroperitoneum, and liver
[1]. Identical to other retroperitoneal sarcomas, surgical resection with
wide margins is the cornerstone of the treatment as the tumor tends to
grow slowly, resulting in a good prognosis. Despite its good prognosis
after resection, this type of tumor may be challenging to identify due to
variations and rarity of the histological pattern. Currently, the common
approach for histopathological diagnosis is based on exclusion criteria
and identification of a “pattern-less pattern” of the spindle cells. We
describe a rare case report of this type of tumor located in the retro­
peritoneum, along with its diagnostic approach and treatment modality.
This study has been reported in line with the SCARE criteria [2].
A 43-year-old woman was admitted because of a palpable mass in her
left flank, with accompanying symptoms of dysuria and worsening pain
in the abdomen. A huge tumor occupying the left side of the abdomen
was found on CT-Scan examination, therefore she was referred to our
hospital for further treatment. Six years ago, she had experienced left
abdominal pain. The pain was dull, and intermittent, with a visual
analog scale (VAS) of 1–2, which was eventually left untreated. She also
denied any weight loss. During admission, a physical examination
showed remarkable bulging on the left flank. A painless, mobile hard
tumor was found on palpation. Blood examinations showed mild anemia
and hypoalbuminemia. By the pattern of the disease, the patient was
initially diagnosed with liposarcoma (Fig. 1).
Surgical exploration with excision of the retroperitoneal mass was
decided as treatment modality in this case. Intraoperative findings
showed a mass surrounding both the left renal artery and vein with

* Corresponding author.
E-mail address: lenggo.putra@gmail.com (L.S. Putra).

https://doi.org/10.1016/j.ijscr.2023.109118
Received 30 July 2023; Received in revised form 28 November 2023; Accepted 3 December 2023
Available online 11 December 2023
2210-2612/© 2023 The Authors. Published by Elsevier Ltd on behalf of IJS Publishing Group Ltd. This is an open access article under the CC BY-NC-ND license
(http://creativecommons.org/licenses/by-nc-nd/4.0/).
L.S. Putra et al.
persistent adherence. Preservation of renal vascular failed, therefore
subsequent left nephrectomy was performed along with resection of the
retroperitoneal tumor (Fig. 2). There were no other invasions or re­
lations to any adjacent organs, including the rectum, sigmoid colon, or
uterus. The patient had uneventful postoperative care and was dis­
charged as planned.
Histologically, the neoplasm appeared sarcomatoid, composed of a
relatively solid and diffuse cell pattern with ovoid and stellate nuclei and
spindle cells. The neoplasm had moderate nuclear anaplasia with min­
imal necrotic areas and mitotic figs. (0–2 mitoses per High Power Fields)
(Fig. 3). This neoplasm was initially judged as a rhabdomyosarcoma.
However, subsequent immunochemistry examination demonstrated
positive staining for vimentin, desmin, and focal SMA, while myogenin,
MyoD1, MDM2, and S-100 were negative. The Ki-67 index was
expressed in 5–10 % of tumor cells with minimal proliferation showing
the indolent characteristics of this neoplasm (Fig. 4). Based on these
findings, the diagnosis was changed into a retroperitoneal solitary
fibrous tumor.
No adjuvant treatment was given following the procedures, and the
patient remained symptom-free. A post-operative CT-scan was per­
formed 6 months later, with no detectable mass on the left renal fossa or
any adjacent organs (Fig. 5).
3. Discussion
Solitary fibrous tumors (SFTs) are spindle-cell neoplasms, with rare
occurrence and varied presentation. Klemperer et al. described SFTs as
pleural tumors, which were later corrected as extra-pleural sites were
also identified in about 30 % of cases [1,3,4]. Extra-thoracic SFTs are
benign tumors that rarely metastasize, however, recent studies showed
SFTs may mimic malignant characteristics with local and distant
recurrence post-surgery [5,6].
Retroperitoneal SFT is part of extra-pleural SFTs that are not exten­
sively studied due to the low number of cases reported. It is often mis­
judged as another type of neoplasm or incidentally detected during
diagnostic workup for another disease. This tumor grows slowly without
exhibiting any specific symptoms, resulting in delayed treatment and the
need for extensive resection afterward. Presenting symptoms, such as
abdominal pain, hip pain, or urinary symptoms, occur when the pressure
effect is applied to adjacent organs. Hypoglycemia could also occur due
to Insulin-like Growth Factor-2 (IGF-2) production within the tumor.
Aside from non-specific symptoms, the diagnosis is rather challenging
Fig. 1. Initial CT-Scan examination of the tumor.
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International Journal of Surgery Case Reports 114 (2024) 109118
due to unspecific morphology and pattern-less microscope findings. SFT
is characterized by a greyish, solitary, and well-circumscribed mass with
common histological findings which include a storiform pattern of
spindle cells, accompanied by hypercellular and hypocellular area with
collagenous and hyalinised stroma. In rare cases, it could exhibit ma­
lignancy behavior, showing elevated cellularity and mitotic figures
(more than 4/10 mitoses per High Power Fields), nuclear pleio­
morphism, necrosis, and infiltrative border [7–9].
Several differential diagnoses for SFT are usually neoplasm with
spindle-cell morphology, including leiomyoma, inflammatory myofi­
broblastic tumor, angiomyolipoma, and gastrointestinal stromal tumor.
Radiological imaging, such as a CT-scan, is not helpful to differentiate
this tumor from other solid retroperitoneal sarcomas. Confirmation of
the diagnosis can be done through immunochemistry (IHC) examina­
tion, as SFT tested positive for Bcl-2, vimentin, CD34, and CD99 stain­
ing. Negative results for S-100, cytokeratin, EMA, SMA, CD117, CD31,
and desmin is the usual findings of SFT, which further confirm the
diagnosis [10]. STAT6 test could be performed if necessary as it is the
most sensitive and specific IHC staining for SFT [11]. Immunochemistry
examination of the resected tumor in our case demonstrated positive
staining for vimentin, desmin, and focal SMA, while myogenin, MyoD1,
MDM2, and S-100 were negative.
As mentioned earlier, the majority of SFTs, including retroperitoneal
SFT, are benign tumors. This tumor is often labelled as an “intermediate
malignant, rarely metastazing” neoplasm. However, due to the unpre­
dictable nature of the neoplasms, local or distance recurrence and
metastasis could also happen in approximately 5–10 % of benign SFT
and 20–30 % of malignant SFT [5]. Aside from tumor cell appearance
(size, cellularity, mitotic activity, hemorrhage, necrosis, location), Ki-67
labelling index above 20 % should be another hint of malignant capacity
[5]. In our case, the tumor had a low Ki-67 labelling index of 5–10 %
with minimal moderate nuclear anaplasia with minimal necrotic areas
and mitotic figs. (0–2 mitoses per High Power Fields), thus predicting a
lower chance of malignant capacity.
Similar to sarcoma-like tumors, surgery is still the preferred modality
with satisfying results. Resection margins influence the recurrence rate
therefore effective surgical strategy must be prepared to achieve clear
surgical margins [12]. In most cases, retroperitoneal SFT were a solid,
well-defined mass that could be removed immediately without inter­
fering with the kidney, however, removal of adjacent organs such as the
kidney may become necessary when extensive invasion was found. In
our case invasion of both main renal artery and vein is present thus
L.S. Putra et al. International Journal of Surgery Case Reports 114 (2024) 109118

Fig. 2. Retroperitoneal tumor and kidney.

Fig. 3. Histopathologic examination. (A: Tumor showed variable cellularity, with numerous dilated, staghorn-like vessels; B: Tumor cells arranged haphazardly
(patternless) or in short fascicles; C: Ovoid to fusiform spindle cells, with indistinct borders arranged in short fascicles; D: Tumor cells among hyalinized/collagenized
stroma with overall low mitotic rate.)

necessitating a radical nephrectomy to be performed along with radical
excision of the tumor as renal vessels could not be preserved. Different
conditions were reported in other studies depending on the tumor in­
vasion in each case. Kuneida, et al. reported a right retroperitoneal SFT
which compresses the colon and the kidney. This tumor did not involve
the kidney structure, except for a small area of fibrous kidney capsule in
the lower part of the kidney. Therefore, the tumor could be completely
removed without making any incision to the kidney cortex [13]. While
another study from Kopel, et al. reported a radical nephrectomy with
resection of the retroperitoneal mass and peraortic and paracaval lymph
node dissection in a case of extra-pleura SFT which arises from pararenal
fascia of the anterior capsule. Radical nephrectomy, in particular, was
chosen because the mass tightly adhered to the kidney within Gerota's
fascia, therefore this procedure was needed to remove the mass with
minimal blood loss and prevent any vascular complications (e.g. AV
fistula or pseudoaneurysm). A partial nephrectomy was not considered
due to a higher risk of bleeding and urine leakage [14].
The most effective treatment approach of retroperitoneal SFT is not
defined yet, as less than 100 cases have been reported. The current
accepted standard therapy is surgery, with a negative resection margin
as it has been correlated with lower recurrence. In addition, the recur­
rence rates were low as opposed to other retroperitoneal sarcomas (17

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L.S. Putra et al. International Journal of Surgery Case Reports 114 (2024) 109118

Fig. 4. Immunohistochemistry examination. (A: Positive staining on vimentin; B: Positive staining on desmin; C: Positive staining on focal SMA; D: Ki67 index
staining showed low proliferation rate).

Fig. 5. Follow-up CT-Scan examination of the tumor.

vs. 52–61 %) [15,16]. Therefore, routine use of chemotherapy, radio­
therapy, or other agents is not usually recommended, until proven
otherwise.
4. Conclusion
Due to the unpredictable behavior of SFT, including retroperitoneal
SFT, the determination of the most effective strategy is nearly impos­
sible. Retroperitoneal solitary fibrous tumor (SFT) presents diagnostic
challenges due to its non-specific morphology, therefore immunohisto­
chemistry plays a crucial role in confirming the diagnosis of
retroperitoneal SFT, distinguishing it from other neoplasms. Analysis of
current published reports shows that surgical excision is still a feasible
standard of treatment without any impactful morbidity and mortality. In
cases where retroperitoneal SFTs invade adjacent structures, radical
nephrectomy becomes necessary in some cases. Post-resection, the
recurrence rates of retroperitoneal SFTs are lower compared to other
sarcomas, and routine chemotherapy or radiation therapy is not
recommended.

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L.S. Putra et al. International Journal of Surgery Case Reports 114 (2024) 109118

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