International Journal of Surgery Case Reports 114 (2024) 109158

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International Journal of Surgery Case Reports

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

Case report

A case report of chest wall desmoplastic small round cell tumor in children
Junlei Shi a, 1, Xiaofeng Chang b, 1, Deguang Meng a, Shichao Chen a, Huanmin Wang b, *
a
Baoding Hospital of Beijing Children's Hospital, China
b
Department of Surgical Oncology, Beijing Children's Hospital, National Center for Children's Health, Capital Medical University, China

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

Keywords: Introduction: Desmoplastic small round cell tumor (DSRCT) is a rare sarcoma predominantly afflicting young
Comprehensive management males.
Chest wall Case presentation: In this current report, a two-year-old boy was admitted to the hospital for the evaluation of a
Children
left chest wall mass. Imaging revealed the tumor's presence in the left chest, compressing lung tissue. Subse­
Desmoplastic small round cell tumor
quently, histological analysis confirmed the DSRCT diagnosis following a biopsy. The patient underwent a
Case report
comprehensive management strategy centered around surgery, successfully completing the entire treatment
course without experiencing relapse during subsequent follow-up assessments.
Discussion: When chest wall tumors are inoperable upon initial diagnosis, a biopsy is essential to clarify the
pathology and assist in the diagnostic process. If a patient is diagnosed with DRSCT and conventional chemo­
therapy fails with surgical resection still not feasible, timely adjustment of the chemotherapy regimen coupled
with targeted drug administration can reduce the tumor, enable complete resection, and improve the overall
prognosis.
Conclusion: DSRCT is a rare malignancy associated with a generally poor prognosis. The administration of a
combined treatment approach involving oral targeted medication (anlotinib), chemotherapy, radiotherapy, and
aggressive surgical resection holds the potential to enhance the prognosis for pediatric patients with this
condition.

1. Introduction involving targeted combination chemotherapy was administered, fol­

Desmoplastic small round cell tumor (DSRCT) is a rare and aggres­
sive mesenchymal malignancy initially described in 1989 [1]. It pre­
dominantly afflicts individuals aged 5 to 30, with a heightened
occurrence among adolescents and young adults [2]. Despite the utili­
zation of diverse treatment approaches, DSRCT remains associated with
a bleak prognosis, marked by a 3-year survival rate of under 30 % and a
5-year survival rate as low as 18 % [3].
DSRCT occurring in the chest wall is exceptionally rare compared to
more typical locations. Diagnosis is frequently missed when the chest
wall mass is inconspicuous. Moreover, the tumor's unique location in­
creases the likelihood of infiltration into nearby critical structures such
as the lungs, nerves, and blood vessels, heightening the surgical risk.
Consequently, a combination of more efficacious treatments is typically
required to attain superior therapeutic outcomes. In our case, following
diagnosis via puncture biopsy, a course of preoperative treatment
lowed by complete tumor resection and subsequent postoperative care.
This comprehensive approach resulted in a notably improved outcome
for the patient, serving as a valuable reference for the treatment of
similar pediatric cases.
Our work has been reported in line with the SCARE Guidelines 2023
criteria [4].
2. Case presentation
A two-year-old male patient was admitted to our hospital with
complaints of a left-sided chest wall mass. There were no associated
symptoms of fever, respiratory distress, or wheezing. On physical ex­
amination, a fixed, painless mass was detected on the left chest wall,
accompanied by reduced respiration on the left side. No superficially
enlarged lymph nodes, abdominal abnormalities, or bone pain were
observed. Ultrasonography (USG) detected a solid tumor measuring 9.1

* Corresponding author at: Department of Surgical Oncology, Beijing Children's Hospital, National Center for Children's Health, Capital Medical University, 56
Nanlishi Road, Beijing 100045, People's Republic of China.
E-mail address: zlwk2012@163.com (H. Wang).
1
Those authors have contributed equally to this work and share the first authorship.

https://doi.org/10.1016/j.ijscr.2023.109158
Received 23 October 2023; Received in revised form 6 December 2023; Accepted 8 December 2023
Available online 13 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/).
J. Shi et al. International Journal of Surgery Case Reports 114 (2024) 109158

cm in maximum diameter within the left chest wall, with no signs of

hydrothorax. Subsequently, an enhanced contrast computed tomogra­
phy (CT) scan illustrated a 6.6 × 8.8 × 7.1 cm solid tumor situated in the
posterior aspect of the left chest wall, accompanied by deformation of
adjacent ribs (8–9) (Fig. 1A). Importantly, no distant metastases were
detected. Due to the inability to achieve complete tumor removal upon
evaluation, a tumor puncture biopsy was performed, confirming the
diagnosis of Desmoplastic Small Round Cell Tumor (DSRCT) via histo­
logical examination (Fig. 2). Additionally, fluorescence in situ hybridi­
zation (FISH) revealed the fusion of the EWSR1 gene with the WT1 gene
(Fig. 3). Subsequently, the patient underwent chemotherapy using the
VAIA regimen (ifosfamide, vincristine, dactinomycin, doxorubicin) for
two cycles. However, a follow-up CT scan indicated that the tumor size
remained nearly unchanged at 6.0 × 8.0 × 7.1 cm (Fig. 1B). Subse­
quently, following thorough risk disclosure and parental consent, the
treatment approach was adjusted to incorporate anlotinib, an oral tar­
geted drug, alongside an alternating chemotherapy regimen comprising
ICE (ifosfamide, cisplatin, and etoposide) and VDC (vincristine, adria­
mycin, and cyclophosphamide). Subsequent CT scans revealed a
Fig. 2. Microscopically, the tumor cells present as small, round, and predom­
maximum tumor diameter of 7.1 cm (Fig. 1C), and it was deemed
inantly blue cells. They exhibit a relatively uniform morphology, a high
amenable to resection following assessment. Since the tumor remained nucleoplasmic ratio, and scant cytoplasmic staining. Nuclei appear fine, with
substantial post-chemotherapy and the impracticality of minimally inconspicuous nucleoli, although some pathological nuclear abnormalities are
invasive approaches, the patient underwent an open lumpectomy. A observable. Moreover, glassy degeneration of the fibrous interstitium surrounds
section of the ribs was excised in tandem with the tumor to ensure en- the tumor. (200×, narrow arrow). (For interpretation of the references to colour
bloc tumor resection. Microwave ablation was employed to treat the in this figure legend, the reader is referred to the web version of this article.)
tumor bed using a microwave scalpel (SurgiNeer M120, Beijing Heng­
FuSiTe Co., Ltd.). Postoperative histological analysis indicated approx­ Throughout the treatment course, the patient exhibited increased tri­
imately 20 % tumor necrosis with clear margins. Subsequently, the glyceride levels as the only notable side effect. To date, approximately
patient received eight cycles of combined targeted drug therapy and three years have passed since the completion of the treatment proced­
chemotherapy, followed by a 45 Gy radiotherapy dose after two cycles. ure, with no observed instances of relapse (Fig. 1D).

Fig. 1. A the chest CT scan when diagnosis (transverse section，narrow arrow).

B the chest CT scan after the treatment of VAIV for twice (transverse section，narrow arrow).
C the chest CT scan after the treatment with a combination of anlotinib and chemotherapy for twice (transverse section, narrow arrow)
D the chest CT scan after the whole treatment procedure.

2
J. Shi et al.
Fig. 3. the fusion of EWSR1 gene with the WT1gene (narrow arrow).
3. Discussion
Desmoplastic Small Round Cell Tumor (DSRCT) is an uncommon and
highly aggressive malignant sarcoma. It most frequently arises in the
pelvic region, although it can also manifest in rarer sites such as the
omentum, retroperitoneum, and mesentery [5]. Manifestation in the
chest wall is exceptionally rare. When DSCRT does occur in the chest
wall, it often presents with nonspecific symptoms like coughing, dys­
pnea, chest pain, and a feeling of tightness [6]. Notably, our patient
deviated from the typical literature descriptions, as they were signifi­
cantly younger and remained asymptomatic without discomfort.
The clinical diagnosis of DSRCT can be challenging due to the
absence of specific clinical signs and tumor markers. The patient pre­
sented with a localized mass that couldn't be surgically removed
initially, leading to a diagnostic approach involving a needle biopsy.
Subsequently, histological examination confirmed the DSRCT diagnosis.
DSRCT management typically involves a multimodal approach
comprising chemotherapy, radiation therapy, and aggressive surgical
resection. In cases where the tumor is deemed unresectable upon diag­
nosis, neoadjuvant chemotherapy is recommended to reduce its size.
According to the literature [7], the VAIA regimen is associated with the
most favorable prognosis. Consequently, the patient initiated VAIA
treatment twice. Unfortunately, subsequent evaluations did not yield the
desired outcome, and the tumor remained unresectable. Certain litera­
ture suggests that anlotinib, a small-molecule tyrosine kinase inhibitor
targeting multiple pathways, has been employed to manage soft tissue
sarcomas in adults [8]. However, its application in pediatric soft tissue
sarcomas is lacking in experience. Consequently, the patient received a
cautious regimen at the minimal dose of 8 mg daily for 14 consecutive
days every three weeks. Given the uncertainty regarding potential side
effects, the chemotherapy regimen was subsequently adjusted to include
alternating cycles of ICE and VDC. After two cycles of this modified
treatment, the patient exhibited elevated triglyceride levels in their
biochemical results, with no other adverse reactions attributed to the
3
International Journal of Surgery Case Reports 114 (2024) 109158
targeted drugs. Subsequent CT scans indicated tumor shrinkage,
enabling the patient to undergo successful tumor resection, including
the removal of affected ribs. Following the operation, the patient un­
derwent eight additional cycles of combined treatment involving tar­
geted drugs and chemotherapy.
Despite the improvement in survival rates achieved through com­
bined therapies for DSRCT patients, long-term prognoses remain
discouraging. In our particular case, the initial treatment regimen
resulted in limited tumor reduction. However, a substantial reduction in
the tumor's size occurred after adjusting the treatment approach, ulti­
mately culminating in complete excision. Following the completion of
post-operative treatments, no instances of recurrence were observed
during the subsequent period. This favorable outcome may potentially
be attributed to the administration of the targeted drug. Nonetheless, the
follow-up period remains relatively short, necessitating additional time
for the assessment of long-term prognosis. Furthermore, considering the
patient's young age at the time of localized radiotherapy, it is imperative
to extend the monitoring of radiotherapy-related side effects.
In this specific case, the preoperative administration of a targeted
agent alongside chemotherapy contributed to a successful complete
tumor resection, resulting in enhanced outcomes. These discoveries
provide valuable therapeutic guidance for the treatment of pediatric
patients afflicted with this ailment. It is important to highlight that
anlotinib is primarily employed in adults with soft tissue sarcomas, and
there is a paucity of data concerning its usage in children. The lack of
literature concerning its application in pediatric tumors underscores the
necessity for additional research into potential adverse reactions and
organ effects within the pediatric demographic.
4. Conclusion
DSRCT is a rare malignancy associated with a generally poor prog­
nosis. The administration of a combined treatment approach involving
oral targeted medication (anlotinib), chemotherapy, radiotherapy, and
J. Shi et al.
aggressive surgical resection holds the potential to enhance the prog­
nosis for pediatric patients with this condition.
Ethical approval
Ethical approval for this study (Ethical Committee [2021]-E-213-R)
was provided by the Ethical Committee of the Beijing Children's Hos­
pital on 25, November 2021.
Funding
There are no funding sources.
CRediT authorship contribution statement
Junlei Shi, Xiaofeng Chang = Study concept, original draft writing.
Junlei Shi, Xiaofeng Chang, Deguang Meng, Shichao Chen =
photography collection, Case summary writing
Huanmin Wang = study concept
All authors read the manuscript and approved the final manuscript.
Guarantor
The guarantor is Huanmin Wang,
Registration of research studies
This report is not applicable to this topic.
Consent
Written informed consent was obtained from the patient's parents/
legal guardian for publication and any accompanying images. A copy of
International Journal of Surgery Case Reports 114 (2024) 109158
the written consent is available for review by the Editor-in-Chief of this
journal on request.
Statement
The authors declare that they have no known competing financial
interests or personal relationships that could have appeared to influence
the work reported in this paper. Parental consent has been obtained for
child information and case data.
Declaration of competing interest
The authors declare that they have no competing interests.
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