Case presentation

Intestinal Clear Cell Sarcoma - A case presentation

of an extremely rare tumor
Elena Chitoran 1,2, Vlad Rotaru 1,2,*, Mădălina-Nicoleta Mitroiu 3, Sinziana-Octavia Ionescu
1,2,*
, Ariana Neicu 4, Raluca Mihăilă5, Ciprian Cirimbei 1,2, Mihnea Alecu1,2, Daniela-Cristina
Ștefan1 and Laurentiu Simion 1,2

Citation: Chitoran, E.;

Rotaru, V.; Mitroiu, M.-N..;
Ionescu, S.-O.; Neicu, A.;
Mihăilă, R.; Cirimbei, C.;
Alecu, M.; Ștefan, D.-C.;
Simion, L. Intestinal Clear
Cell Sarcoma - A case
presentation of an extremely
rare tumor. Journal name,
volume, issue, pages, Doi.

1
“Carol Davila” University of Medicine and Pharmacy, 050474 Bucharest, Romania;
2
General Surgery and Surgical Oncology Department I, Bucharest Institute of Oncology “Prof.
Dr. Al.
Trestioreanu”, 022328 Bucharest, Romania
3
Department of Obstetrics and Gynecology, “Filantropia” Clinical Hospital, 011132 Bucharest,
Romania
4
Pathology Department, Bucharest Institute of Oncology “Prof. Dr. Al. Trestioreanu”, 022328
Bucharest, Romania
5
Medical Oncology Department, Bucharest Institute of Oncology “Prof. Dr. Al. Trestioreanu”,
022328 Bucharest, Romania
* Correspondence: rotaru.vlad@gmail.com (V.R); sinziana.ionescu@umfcd.ro (S.-O.I.)

Abstract
Background: Clear cell sarcoma (CCS) is an extremely rare form of sarcoma
representing less than 1% of all soft-tissue sarcomas. It has morphological,
structural, and immunohistochemical similarities with malignant melanoma, affecting
young adults, equally affecting both sexes, usually located in the tendinous sheaths
and aponeuroses of the limbs. Case presentation: We present an extremely rare
case of intestinal CSS in a 44-year-old female. The patient presented with symptoms
of intestinal obstruction, underwent a segmental enterectomy with isolation of the
vascular bundle supplying the affected loop at its origin, including lymphatic
clearance. Over the two years of follow-up, no signs of recurrence were detected.
After 30 and at 56 months respectively, from the primary treatment, the patient
developed unique liver metastasis treated by hepatic resections. The patient remains
under observation. Discussion: Gastro-intestinal localization of CCS is exceptional;
to date less than 100 cases have been reported in literature. The gene fusion of
activating transcription factor 1 (ATF1) and the Ewing sarcoma breakpoint region 1
(EWSR1) is pathognomonic for clear cell sarcoma, representing the key to the
diagnosis. A variant fusion protein EWSR1-CREB1 can occur in gastrointestinal CCS.
CCS is an extremely aggressive tumor, >30% having distant or lymphatic metastasis
at the time of diagnostic, and a high recurrence rate of over 80% in the first year
after treatment and high tendency for metastatic dissemination. Given the rarity of
this tumor, there is not a standardized treatment. Radical surgery is essential in the
treatment of CCS both for the primary tumor and for the recurrences or metastasis.
Conclusions: In the case of CCS, early diagnosis plays a crucial role in terms of the
prognosis of the disease. Treatment is not standardized by guidelines and seldom
consist of only surgical excision with oncological safe margins and meticulous follow-
up. Chemo-radiotherapy and have very little effect and are rarely indicated and
targeted therapies have an unclear role and are still under investigation.
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Keywords: clear cell sarcoma, rare sarcomas; soft-tissue sarcoma; intestinal clear
cell sarcoma, soft-tissue melanoma, EWSR1-ATF1 fusion protein; EWSR1-CREB1;
soft-tissue melanoma.

1. Introduction
Clear cell sarcoma (CCS) also called "soft-tissue melanoma" is an
extremely rare malignant tumor and was first described by F. Enzinger in
1965, which poses diagnostic challenges due to similarities in structure,
morphology and immunohistochemistry very much resembling that of
malignant melanoma (MM) [1,2] . Among the common phenotypic
characteristics shared with malignant melanoma are the presence of
melanin, as well as the expression of melanoma-associated markers such as
HMB-45, microphthalmia transcription factor (MiTF), S100 protein and
Melan-A [1] . From histological and immunohistochemical point of view CCS
and MM are almost identical, and most often cannot be differentiated such.
The two forms can be differentiated by fluorescence in situ hybridization
(FISH) and real-time Polymerase Chain Reaction (RT-PCR). There main key
of the differential diagnosis, is a reciprocal chromosomal translocation
t(12;22) (q13;q12) leading to the occurrence of a fusion between two genes,
namely the activating transcription factor 1 (ATF1) gene and the Ewing
sarcoma breakpoint region 1 (EWSR1) gene and resulting in a fusion protein
EWSR1-ATF1. The presence of this gene fusion is a relevant indicator, as it
occurs in the vast majority of patients. A variant fusion protein EWSR1-
CREB1 can occur in gastrointestinal CCS resulting from the chromosomal
translocation t(12;22)(q34;q12). Also, MM frequently present BRAF
mutations, which are absent in CCS.
CCS originates from the neural crest cells (as proven by the presence of
melanosomes in the cytoplasm of tumoral cells), represents less than 1% of
all soft-tissue sarcomas, affecting young adults and equally affecting both
sexes, usually located in the tendinous sheaths and aponeuroses of the
limbs. Besides MM, other differential diagnoses are made with Kaposi's
sarcoma and malignant peripheral nerve sheath tumor (MPNST). Kaposi's
sarcoma typically occurs in immunocompromised patients, while MPNST is
often found in patients with neurofibromatosis type 1 [3] .
The most common clinical presentation is a painful (pain present in 33-
55% of cases [4] ), rapid growing, tumoral mass located around the ankles.
More than 90% of all CCS are located in the extremities and the neck. There
are also rare reports of CCS located inside the thoracic and abdominal
cavity. Only 6-7% of CCS cases originate from the gastro-intestinal tract,
making it an extremely rare form. To the best of our ability, we could locate
less than 100 cases in international literature, most being solitary tumors
and presenting as intestinal occlusion or anemic syndrome. The differential
diagnostic of gastro-intestinal CCS include MM, malignant gastrointestinal
neuroectodermal tumors or GNET (which also present EWSR1-ATF1 or
EWSR1-CREB1 fusion and S100 positivity, but lack melanocytic markers and
frequently have gigantic osteoclast-like cells), clear-cell carcinomas (but
these are cytokeratin positive), gastrointestinal neuroendocrine tumors
(differentiated by serologic test and scintigraphy), intestinal
adenocarcinomas and gastrointestinal stromal tumors (GIST).
CCS is an extremely aggressive tumor, >30% of cases having distant or
lymphatic metastasis at the time of initial diagnostic and a recurrence rate
of over 80% in the first year after primary treatment. More than 60% of all
cases will develop metastasis within 12 months from diagnostic. Several risk
factors have been described for clear cell sarcoma, in the absence of a clear
cause, including chemotherapy, radiotherapy, and genetic predisposition
[4,5] .
Given the rarity of this tumor, there is not a standardized treatment.
Radical surgery is essential in the treatment of CCS both for the primary
tumor and for the recurrences or metastasis.

2. Case presentation
 3 of 7

The 44-year-old female, patient, known for hereditary predisposition to

digestive neoplasms (the mother had colon neoplasm and the father
gallbladder neoplasm), was diagnosed with mild iron-deficiency anemia in
August 2018. In response, an abdominal CT scan and an entero-MRI were
performed, revealing a slightly thickened jejunal parietal area without signs
of upstream stasis. Both superior and inferior endoscopies performed were
normal. In the subsequent period, the patient experienced mild diffuse
recurrent abdominal pain that did not significantly impact daily routine or
sleep quality.
In May 2020 the patient became abruptly symptomatic presenting with
predominantly nocturnal bilious vomiting, followed by colicky pain. These
symptoms recurred every 3-4 days, accompanied by repetitive episodes of
belching, hiccups, a sensation of fullness, diffuse abdominal gurgling, more
pronounced in the upper abdominal region. The patient also reported a
progressive weight loss of approximately 12 kg in the last month. Over a
month, the patient underwent multiple gastroenterology consultations. The
symptoms of intestinal obstruction prompted the performance of an entero-
MRI on June 4, 2020, which revealed a high intestinal obstruction due to a
tight stenosis at the level of a jejunal loop. The presence of an invagination is
maintained throughout the examination at the level of the described stenotic
lesion. Small satellite lymph node images measuring 7/5mm were also
observed. Biochemical investigations revealed hypopotassemia and a slight
coagulation deficiency. All tumoral markers were in normal limits (CA19-9,
CEA, CA125, CA15-3, CA72-4, AFP, NSE and SCC).
In these conditions, with the diagnosis of intestinal obstruction, surgical
intervention was performed. Upon entering the peritoneal cavity, the
following is observed: dilated small intestinal loops with a diameter of
approximately 5 cm, thickened jejunal walls, and liquid content up to the
level of a jejunal stenosis located about 80 cm from the duodeno-jejunal
angle and approximately 120 cm from the ileocecal valve. Additionally,
numerous lymph nodes with a maximum diameter of 1.5-2 cm are noted
along the course of the vascular bundle associated with the affected loop,
without definitive macroscopic cancer characteristics (Figure 1).

A. B.

Figure 1. A. Intraoperative image of the circumferentially thickened

jejunal loop, leading to significant dilation upstream of the jejunal loops; B.
Dilated small intestinal loops with a diameter of approximately 5 cm.
Under these circumstances, a segmental enterectomy was performed
with isolation of the vascular bundle supplying the affected loop at its origin
and lymphatic clearance. The resection is carried out within oncological safe
limits, with a 10 cm margin on each side of the stenosis of unspecified
etiology. Restoration of digestive continuity is achieved through a
mechanically assisted side-to-side entero-entero anastomosis, followed by
closure of the mesenteric gap.
The patient is discharged with a favorable postoperative course, with
the complete resumption of intestinal transit on the 5th postoperative day, in
a satisfactory general condition, afebrile, and with balanced hemodynamic
and respiratory status.
 4 of 7

The extracted specimen was analyzed. Thus, macroscopically, a

fragment of the small intestine with a dilated proximal portion and an area
of vegetative tumor aspect measuring 1.5/1.5/1.2 cm in depth was observed,
covered with focally ulcerated, congested mucosa. On section, the tumor had
a white color, firm consistency, predominantly submucosal location with
ulceration of the serosa. It covered approximately 30% of the lumen.
Additionally, the resected triangular mesenteric portion, measuring 8/8 cm,
had numerous small-sized lymph nodes.
Microscopy revealed the resection with clear proximal and distal
margins. The intestinal wall showed tumor proliferation covered with
mucosa, which it infiltrates and ulcerates focally, extending into the
submucosa, muscular layer, subserosa, with infiltration of the serosa. Tumor
cells were epithelioid and spindle-shaped, with alveolar, solid, or storiform
architecture, relatively monomorphic, with medium-sized, pleomorphic,
vesicular, and nucleolated nuclei in epithelioid areas. The cytoplasm was
pale, weakly eosinophilic, with a mitotic index of 7 mitoses/10 HPF. No
necrosis was evidentiated. Tumor emboli were detected in capillary-caliber
vessels of the submucosa. The submitted specimen contained 23 intact
lymph nodes (chronic nonspecific lymphadenitis with follicular hyperplasia)
and 1 lymph node with a small area of hypocellular fibrosis.
Immunohistochemistry: vimentine positive, CD99, CD15 and CD56
positive, S100 and SOX10 positive, Synaptophisin positive, negative for
HMB45, MART-1, Cytokeratine, Desmin, Actin, DOG-1, CD34, CDX-2,
CKIT/CD117; Ki67 35%.
The definitive diagnosis and further therapeutic approach were
determined concomitantly with the histopathological diagnosis. The
histopathological results and immunohistochemical profile support the
diagnosis of gastrointestinal malignant neuroectodermal tumor/clear cell
sarcoma of the gastrointestinal tract (CCS).
The case has been taken over by an oncologist. Given that this diagnosis
is a rare one, with few cases described in the literature and limited
information about therapeutic approaches for adjuvant therapy, a PET-CT
has been recommended to determine the actual extent of the disease and
revealed no additional tumoral sites. Additionally, a repeat of
immunohistochemistry and analyses of tissue by Next generation sequencing
(NGS) or Foundation One have been recommended and performed.
Furthermore, the patient has been advised to seek consultation in other
international institutes specializing in sarcomas/rare diseases for case
analysis and therapeutic guidance.
Genetic testing: Microsatellite stable; Tumor mutational Burden 4
Muts/Mb; BRAF mutations absent; NGS based assay result showed
alteration of the MYC gene, alteration of the EWSR1 gene - EWSR1-CREB1
fusion was present, alteration of STAG2 Gene. Further, more genetic
variants of unknown significance (VUS) were detected in this patient's
tumor. These variants may not have been adequately characterized in
the scientific literature at the time this report was issued, and/or the
genomic context of these alterations makes their significance unclear
and yet they decided to include them in this report in the event that
they become clinically meaningful in the future. BCOR-V679I; CDK12-
P1257del, CREBBP-S128C; ERBB3 rearrangement, IRS2-K1170R; KDM5C-
R1435C; MLL2-L4077F; SMO-Q745R
The patient received secondary medical opinions in multiple
international medical facilities specializing in sarcomas/rare diseases from
Turkey, USA, Austria, Greece and Spain. No clinic provided an indication for
adjuvant treatment; the only recommendation was regular follow-up.
The CT scans and MRIs during the first two years of follow-up did not
detect any signs of tumor recurrence or metastasis. However, at 30 months
after primary treatment, the patient developed a unique liver metastasis
which was treated by atypical hepatic resection. Similar, at 56 months she
developed a second unique liver metastasis which was also surgically
removed.
The patient remains scheduled for periodic follow-up.
 5 of 7

3. Discussion
Given the highly heterogeneous clinical presentation, clinicians,
especially those without experience, can easily be misled, struggling to
differentiate clear cell sarcoma from malignant melanoma correctly,
especially for the intraabdominal localization. This combined with the fact
that immunohistochemistry cannot distinguish between the two types of
tumors, makes clear cell sarcoma often misdiagnosed as malignant
melanoma. Due to CCS rarity, considering FISH and RT-PCR testing is
essential for an accurate diagnosis [3] . Correct and rapid diagnostic is
necessary for insuring the optimal therapeutic response and a better
oncological outcome, and physicians should always consider genetic testing
for CCS when faced with a MM diagnostic.
In literature we could find few studies focusing on the prognostic factors
and the survival after CCS [6–8] . The largest of those studies [8] , including
489 patients, determined that 38% of patients had distant organ metastases
at diagnostic (most common site being the lung). At diagnostic only a third
of patients were stage I. The same study calculated a median overall survival
of 57,2 months, with 5- and 10-year survival rates of 50 and 38%,
respectively. Patients with localized disease have a better 5- and 10-years
survival rates that those with regional dissemination (82.4% respectively
68.8% vs. 44% respectively 32,5%) and none of the patients with distant
dissemination survive at 5 years [7] . Besides regional and distant
dissemination there are other prognostic factors associated with the
reduction of specific disease-free survival (DFS) and overall survival (OS) for
patients with CCS. A diminished DFS was associated with tumors larger
than 5cm (median DFS, 7.5 vs. 25.5 months, p = 0.0043), positive surgical
margins (median DFS, 3.5 vs. 13 months, p = 0.0233) and Neutrophile-
Lymphocyte ratio greater than 2.73 (median DFS, 7.5 vs. 25.5, p = 0.0009).
Similar reduced OS rates were associated with tumor size larger than 5cm
(median OS, 23.5 vs. 63 months, p = 0.0075), positive surgical margins
(median OS, 21.5 vs. 63 months, p = 0.0101), Neutrophile-Lymphocyte ratio
greater than 2.73 (median OS, 26 vs. 85 months, p = 0.0126), Lymphocyte-
Monocyte ratio smaller than 4,2 (median OS, 26 vs. 85 months, p = 0.0445)
and a Thrombocyte-Lymphocyte greater than 103.89 (median OS, 26 vs.
85 months, p = 0.0147) [7] .
Given the rarity of this tumor, there is not a standardized treatment for
CCS. Both ESMO and NCCN guidelines do not discuss CCS separately and
only offer general principles for treatment for all soft-tissue sarcomas.
Radical surgery is the “gold-standard” in the treatment of CCS both for the
primary tumor and for the recurrences or metastasis. For a favorable
prognosis, early diagnosis followed by surgical treatment with a radical
approach is necessary [9] . There are no studies which evaluate the benefits
of chemo- and radiotherapy in CCS patients neither in neoadjuvant and
adjuvant settings. Adjuvant chemoradiotherapy may improve DFS but
without affecting OS. Systemic therapy is used for unresectable or
metastatic cases and is anthracycline-based. Radiation therapy is of little use
in gastrointestinal CCS.
As for targeted therapies, there currently are none approved for human
use and no specific therapeutic agents directly targeting EWSR1-CREB1 and
EWSR1-ATF1 fusion proteins. However, there are a few small studies
investigating IGF1R inhibitors for tumors exhibiting EWSR1 fusion proteins
[10,11] . EWSR1-ATF1 fusions proteins have been reported to upregulate
expression MET [12] and MiTF [13] , a transcription factor that has been
shown to drive MET expression [14,15] . This raises the possibility of using
the antibody AMG102 (which suppresses MET signaling) for treating CCS.
Using sunitinib for patients with CCS harboring EWSR1-ATF1 fusion has
been showed to elicit a therapeutic response but it is not clear if other
genetic alterations were present [16,17] . Combination treatment with
crizotinib and pazopanib can determine a durable partial response in a
patient with a metastatic GNET tumors harboring ESWR1-CREB1 fusion by
an unknown mechanism [18] . All these represent potential directions for
future research, together with potential therapies targeting STAG2 and MYC
 6 of 7

gene alterations. As can be observed in our patient NGS's response, several

other genetic alterations were identified but thus far their significance is
still unknown but can possibly represent ways of targeting SCC: BCOR -
V679I; CDK12 - P1257del, CREBBP-S128C; ERBB3 rearrangement, IRS2 -
K1170R; KDM5C - R1435C; MLL2 - L4077F; SMO-Q745R.

4. Conclusions
Intra-abdominal CCS is an extremely rare tumor, most often
misdiagnosed as a MM due to the clinical, histological and
immunohistochemical similarities between the two forms. MM and CCS can
only be distinguished through FISH and RT-PCR which demonstrate the
presence of the fusion protein EWSR1-CREB1 or EWSR1-ATF1
pathognomonic for CCS. When faced with an intra-abdominal MM, the
physician should have in mind the possibility of CCS and consider ordering
genetic tests.
CCS is an extremely aggressive tumors with high recurrence and
metastatic dissemination rates. Early and accurate diagnosis, is the primary
deterrent of the best oncologic outcome. Treatment options are extremely
limited, and radical surgery remains the therapeutic “gold-standard” for
both primary tumor and recurrent/metastatic disease. Our case
demonstrates that a correct surgical technique, with excision with clear
margins, can yield good results, as evidenced by a 2-year follow-up without
tumor recurrence or distant metastasis despite the lack of adjuvant therapy.
Surgery can also be used successfully to control unique resectable
metastasis.
Author Contributions: Conceptualization, C.E. and R.V.; methodology, C.E. and
R.V.; investigation, I.S.-O.; resources, N.A.; writing—original draft preparation, M.M.-
N.; writing—review and editing, C.E. and R.V.; supervision, C.C.; project
administration, S.L. and S.D.-C.; All authors have read and agreed to the published
version of the manuscript.
Funding: This research received no external funding
Institutional Review Board Statement: Not applicable.
Informed Consent Statement: Written informed consent has been obtained from
the patient to use medical records for educational and scientific purposes.
Data Availability Statement: Not applicable.
Conflicts of Interest: The authors declare no conflict of interest.

References

[1] Haouimi A. Clear cell sarcoma of soft tissue - neck. RadiopaediaOrg 2023.
https://doi.org/10.53347/RID-174362.
[2] Elousrouti LT, Hammas N, Elmernissi F zahra, Elfatemi H, Chbani L. Clear-Cell Sarcoma With an Unusual
Presentation Mimicking Metastatic Melanoma. Cureus 2022;14. https://doi.org/10.7759/CUREUS.32010.
[3] Cassalia F, Cavallin F, Danese A, Del Fiore P, Di Prata C, Rastrelli M, et al. Soft Tissue Sarcoma Mimicking
Melanoma: A Systematic Review. Cancers (Basel) 2023;15:3584. https://doi.org/10.3390/CANCERS15143584/S1.
[4] Kazakos CJ, Galanis VG, Giatromanolaki A, Verettas DAJ, Sivridis E. Clear cell sarcoma of the scapula. A case
report and review of the literature. World J Surg Oncol 2006;4:48. https://doi.org/10.1186/1477-7819-4-48.
[5] Suehara Y, Yazawa Y, Hitachi K, Terakado A. Clear cell sarcoma arising from the chest wall: a case report.
Journal of Orthopaedic Science 2004;9:171–4. https://doi.org/10.1007/S00776-003-0751-6.
[6] Clark MA, Johnson MB, Thway K, Fisher C, Thomas JM, Hayes AJ. Clear cell sarcoma (melanoma of soft parts):
The Royal Marsden Hospital experience. European Journal of Surgical Oncology 2008;34:800–4.
https://doi.org/10.1016/j.ejso.2007.10.006.
[7] Li AB, Jiang BJ, Wang HH, Yang YS, Zhang XB, Lan GH, et al. Prognostic Factors for Survival in Patients with
Clear Cell Sarcoma: An Analysis of the Surveillance, Epidemiology, and End Results (SEER) Database. Med Sci
Monit 2019;25:6939. https://doi.org/10.12659/MSM.916705.
[8] Gonzaga MI, Grant L, Curtin C, Gootee J, Silberstein P, Voth E. The epidemiology and survivorship of clear cell
sarcoma: a National Cancer Database (NCDB) review. J Cancer Res Clin Oncol 2018;144:1711–6.
https://doi.org/10.1007/s00432-018-2693-6.
 7 of 7

[9] Rodríguez-Martín M, Sáez-Rodríguez M, Esquivel B, González R, Cabrera A, Herrera A. CLEAR CELL SARCOMA:
A CASE MIMICKING PRIMARY CUTANEOUS MALIGNANT MELANOMA. Indian J Dermatol 2009;54:168.
https://doi.org/10.4103/0019-5154.53193.
[10] Erkizan H V, Kong Y, Merchant M, Schlottmann S, Barber-Rotenberg JS, Yuan L, et al. A small molecule blocking
oncogenic protein EWS-FLI1 interaction with RNA helicase A inhibits growth of Ewing’s sarcoma. Nat Med
2009;15:750–6. https://doi.org/10.1038/nm.1983.
[11] Li Y-J, Zhao X, Vecchiarelli-Federico LM, Li Y, Datti A, Cheng Y, et al. Drug-mediated inhibition of Fli-1 for the
treatment of leukemia. Blood Cancer J 2012;2:e54. https://doi.org/10.1038/bcj.2011.52.
[12] Davis IJ, McFadden AW, Zhang Y, Coxon A, Burgess TL, Wagner AJ, et al. Identification of the receptor tyrosine
kinase c-Met and its ligand, hepatocyte growth factor, as therapeutic targets in clear cell sarcoma. Cancer Res
2010;70:639–45. https://doi.org/10.1158/0008-5472.CAN-09-1121.
[13] Davis IJ, Kim JJ, Ozsolak F, Widlund HR, Rozenblatt-Rosen O, Granter SR, et al. Oncogenic MITF dysregulation in
clear cell sarcoma: defining the MiT family of human cancers. Cancer Cell 2006;9:473–84.
https://doi.org/10.1016/j.ccr.2006.04.021.
[14] McGill GG, Haq R, Nishimura EK, Fisher DE. c-Met expression is regulated by Mitf in the melanocyte lineage. J
Biol Chem 2006;281:10365–73. https://doi.org/10.1074/jbc.M513094200.
[15] Beuret L, Flori E, Denoyelle C, Bille K, Busca R, Picardo M, et al. Up-regulation of MET expression by alpha-
melanocyte-stimulating hormone and MITF allows hepatocyte growth factor to protect melanocytes and
melanoma cells from apoptosis. J Biol Chem 2007;282:14140–7. https://doi.org/10.1074/jbc.M611563200.
[16] Stacchiotti S, Grosso F, Negri T, Palassini E, Morosi C, Pilotti S, et al. Tumor response to sunitinib malate
observed in clear-cell sarcoma. Ann Oncol 2010;21:1130–1. https://doi.org/10.1093/annonc/mdp611.
[17] Tazzari M, Palassini E, Vergani B, Villa A, Rini F, Negri T, et al. Melan-A/MART-1 immunity in a EWS-ATF1
translocated clear cell sarcoma patient treated with sunitinib: a case report. BMC Cancer 2015;15:58.
https://doi.org/10.1186/s12885-015-1044-0.
[18] Subbiah V, Holmes O, Gowen K, Spritz D, Amini B, Wang W-L, et al. Activity of c-Met/ALK Inhibitor Crizotinib and
Multi-Kinase VEGF Inhibitor Pazopanib in Metastatic Gastrointestinal Neuroectodermal Tumor Harboring
EWSR1-CREB1 Fusion. Oncology 2016;91:348–53. https://doi.org/10.1159/000449204.