Review for 10.1186/s12876-022-02463-y (2022)

Reviewer’s report
Title: 
Response to Chemotherapy Combined with Anlotinib Plus Anlotinib
Maintenance in Intra-abdominal Desmoplastic Small Round Cell Tumors
(IADSRCT): A Case report and literature review
Version: 
0 
Date: 
18 Mar 2022
Reviewer: 
Tarkan Jäger
Reviewer's report:
This is an interesting and valuable case report. Main reason for this is the fact
that desmoplastic round cell tumors (DSRCT) are very rare and any additional
information can only be beneficial. 
The case report is well written and the strategy using Anlotinib is interesting.
Two years after initial response to the provided therapy, a progression is
reported.
I have some questions and remarks:
1. Was a diagnostic laparoscopy performed and if not, why?
2. Who decided that complete resection is not possible (Was there a
cytoreductive surgeon present in the multidisciplinary team)?
3. You report "Hematochezia disappeared after active management." How was
hematochezia managed?(discontinuation of Anlotinib only? Other interventions
(Endoscopy)?) 
4. This case should be included in table 1: Eur Surg (2021) 53:251-255
https://doi.org/10.1007/s10353-021-00696-x
5. Have you performed fluorescence in-situ hybridization (FISH)?
6. Add PR (partial response) to abbreviations section
Summary:
DSRCT is associated with a very poor prognosis with mortality rates up to 70%
in the first 3 years after diagnosis (Eur Surg (2021) 53:251-255
https://doi.org/10.1007/s10353-021-00696-x). So far therapeutic options for
treatment of DSRCT include chemotherapy, radiotherapy, cytorecductive
surgery, and/or hyperthermic intraperitoneal chemotherapy (HIPEC).
Final diagnosis can only be revealed by tissue biopsy following complex
immunohistochemical and molecular analysis. DSRCT is characterized by a
translocation involving fusion of the Ewing sarcoma (EWS) and Wilms tu mor
(WT1) gene leading to formation of a chimeric protein (Eur Surg (2021) 53:251-
255 https://doi.org/10.1007/s10353-021-00696-x).
The concept of neoadjuvant therapy with Anlotinib is an interesting approach.
The value could be enhanced in a multidisciplinary strategy following the
intention to do the surgery after neoadjuvant therapy. (in your case the patient
refused surgery). Patients should know the survival rates clearly and all efforts
should be undertaken to remove tumor after neoadjuvant therapy whenever
possible. 
A diagnostic laparoscopy could had helped to see the real extension of disease
burden. 
At least from the CT scans presented in your case report a resection could be
possible after tumor response. 
Finally, the status of fluorescence in-situ hybridization (FISH) should be
elucidated as it could provide further information on the type of DSRCT. In our
case the diagnosis of DSRCT was only possible after FISH showing a
translocation in the Ewing sarcoma breakpoint region 1 gene (EWSR1), which
led to the diagnosis of an intraperitoneal desmoplastic small round cell tumor
(DSRCT).
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