Reviewer’s report
Title: 
Malignant epithelioid neoplasm of the ileum with ACTB-GLI1 fusion
mimicking an adnexal mass: a case report.
Version: 
0 
Date: 
28 Jan 2022
Reviewer: 
Francesca Cisotto
Reviewer's report:
Dear Respectable Editor, I am grateful to give me the opportunity to review the
paper by Ambrosio et colleagues.
This manuscript (entitled "malignant epithelioid neoplasm of the ileum with
ACTB-GLI1 fusion mimicking an adnexal mass: a case report") addresses an
interesting topic, considering the rarity of this tumor and the peculiar immune-
profile and behavior of this group of neoplasm.
The background section is well structured and the authors provided a complete
description of the features of a such rare tumor.
I appreciate the case report presentation: all important information is reported,
figures are appropriate and useful to understand the followed work up.
I also really appreciated the rigorous application of IOTA rules in a case of non-
adnexal lesion and the use of description of ultrasound characteristics using
standardized terminology. There are prospective studies documenting that in
experienced hands ultrasound may be an alternative to TC, and sometimes can
characterized lesions even better than CT (I suggest to consider these paper: 
doi:
10.1002/uog.23654).
I also suggest to stress the features of the sonic hedgehog (SHH) signaling
pathway, which plays an important role in promoting oncogenesis, tumor growth
and possible drug resistances.
GLI1 (and several others components of SHH pathway) functions as an effort of
the SHH pathway, inducing upregulation and downregulation of multiple
downstream targets. Therefore, different aforementioned pathway components
can be considered suitable for target-therapy (see this paper: doi:
10.3390/cancers8020022). In my opinion, the importance to identify GLI1
alterations and molecular genetic background has to be stressed, especially for
patients with advanced disease who could benefit from treatment with selective
SHH pathway inhibitors, of which GLI1 function as an effector.
The authors successfully applied IOTA rules to a non-adnexal mass and identify
the possible malignancy nature but criteria were not validated in a such scenario.
It could be interesting to apply ADNEX model to verify consistency and
speculate about its use. 
Moreover, was a revision of CT imaging performed? I
would like to know if, in author opinion, the misleading diagnosis was due to the
rarity of this type of cancer or only to the limit of the radiological method. 
Finally, English language is acceptable. In my opinion the manuscript is
published after minor revision.
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Is the study design appropriate to answer the research question (including the use
of appropriate controls), and are the conclusions supported by the evidence
presented?
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from apparent manipulation?
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