Congenital Atrophic Dermatofibrosarcoma Protuberans: A Case Report and Review of the Literature

Round 1

Reviewer 1 Report

Interesting case of congenital atrophic DFSP

I have some comments in order to improve the manuscript, especially in the discussion paragraph

1 the references are vot complete and you missed an important article published un 2007 in Archives of Derm regarding congenital DFSP. In this article there was a series of 8 congenital DFSP, all were diagnosed was confirmed by Coll1A1-PDFFB trnaslocation by FISH. All were atrophic plaques , 4 of them were ewcessively difficult to diagnose clinically AND histologically and were often misdiagnosed at first.  Maire G et al 2007

You have to review and make chanfes in your table, taking into account this paper

One important differential diagnosis is tufted hemangioma you not mentioned

2 In the second paragraph the classification into atrophic plaque, keloid, ...has no interest and can be removed

3 Giant cell fibroblastoma is NOT a differental diagnosis. It as a histological variety of DFSP, called the "juvenile" form of DGSP with the same translocation

4 Importantly the treatment of choic, nowadays, especially in children, is excision with Slow Mohs thechnique, in order to be less mutilating and safer. It is a pity you didn't offered this technique to this young cjild! See and add these 2 articles: A Sleiwah et al. pad Dermatol 2022 and Chicaud M et al Ped Dermatol 2021

 

 

 

Author Response

We thank the reviewer for taking the time to review our manuscript and making these very valuable suggestions and comments. Please find the below responses.

Point 1) the references are vot complete and you missed an important article published un 2007 in Archives of Derm regarding congenital DFSP. In this article there was a series of 8 congenital DFSP, all were diagnosed was confirmed by Coll1A1-PDFFB trnaslocation by FISH. All were atrophic plaques , 4 of them were ewcessively difficult to diagnose clinically AND histologically and were often misdiagnosed at first.  Maire G et al 2007

You have to review and make chanfes in your table, taking into account this paper

Response 1: Thank you for drawing our attention to this important paper. Six cases were included from this reference and the necessary changes were made throughout the manuscript to reflect this addition. Case 1, 6 and 9 were excluded because their image and or description at presentation was not atrophic. 

Point 2) One important differential diagnosis is tufted hemangioma you not mentioned

Response 2) Thank you for bringing this important differential diagnosis to our attention. A paragraph was added to explain similarities and differences between DFSP and tufted angiomas.

Point 3) In the second paragraph the classification into atrophic plaque, keloid, ...has no interest and can be removed

Response 3) Thank you for the comment. This classification was removed.

Point 4) Giant cell fibroblastoma is NOT a differental diagnosis. It as a histological variety of DFSP, called the "juvenile" form of DGSP with the same translocation

Response 4) Thank you for this comment. The GCF paragraph was replaced by a paragraph explaining two histologic variants of DFSP, giant cell fibroblastoma and Bednar tumor.

Point 5) Importantly the treatment of choic, nowadays, especially in children, is excision with Slow Mohs thechnique, in order to be less mutilating and safer. It is a pity you didn't offered this technique to this young cjild! See and add these 2 articles: A Sleiwah et al. pad Dermatol 2022 and Chicaud M et al Ped Dermatol 2021

Response 5) Thank you for the raising this important point and drawing our attention to these two articles. Micrographic techniques were discussed however due to patient, tumor, procedure and logistic factors, these options were not pursued. The discussion at a multidisciplinary tumor board, involving plastic surgery, Mohs surgery, pediatric dermatology, and dermatopathology, arrived at the following reasons to pursue wide local excision:  1) The patient’s young age, would require doing these interventions under general anesthesia, 2) Mohs may incur a long waiting time for tissue processing of a large excision which would have resulted in a prolonged exposure to anesthesia in this pediatric patient, 3) Delayed wound closure, in the case of staged excision/”slow Mohs”, would have been difficult for the young child to tolerate 4) Much of the literature reporting outcomes of these techniques in DFSPs are based on data from older patients. In such patients, DFSP has often been present multiple decades, and have a significant risk of subclinical spread. In our case, this risk is deemed to be small, 5) Due to the low likelihood of subclinical spread, removing the tumor with the required margins would give the plastic surgeon a high likelihood of clearance with a linear closure; 6) The absence of tissue rearrangement would mean that, even if there was a positive margin, simply submitting a clearly oriented excision to pathology would allow for later identification and removal of residual tumor.  

A paragraph was added to the text to explain the surgical options and the reasoning behind the decision to pursue wide local excision. Additionally, table 2, comparing description, advantage, and disadvantages of these options, was also included.

Author Response File: Author Response.docx

Reviewer 2 Report

Dear authors,

I found reading your article very interesting. It was explained clearly and linearly.

I have only a few minor caveats to point out:

1) I would rephrase in the abstract the sentence "And is a typically disease of adulthood."

2) Anamnestic data of the girl should be reported

3) It should be cited a scientific work that describes the Arche FusionPlex Sarcoma Kit

4) For completeness, MRI should be written for extent  for the first time, even though it is a widely used term

5) Integra is a registered trademark, It should be changed (describing the thickness dermal substitute... etc etc)

6) As this is a literature review, the terms used for the search should be mentioned, in what combination and on which database. Finally, with what criteria have been discarded or kept? A "prism" type diagram would be much appreciated

7) When introducing giant cell fibroblastoma, the acronym GCF should be introduced immediately after it.

8) Bigger clinical images would be more appreciated

 

Author Response

We thank the reviewer for taking the time to review our manuscript and making these very valuable suggestions and comments. Please see below our responses.

I found reading your article very interesting. It was explained clearly and linearly.

I have only a few minor caveats to point out:

• I would rephrase in the abstract the sentence "And is a typically disease of adulthood."

Response 1: Thank you for the comment. The sentence was rephrased to “ It typically presents in adults”

• Anamnestic data of the girl should be reported.

Response 2: Thank you for your comment. The patient stated that sometimes the area is a little painful and intermittently looks a little puffy with exercise. 

• It should be cited a scientific work that describes the Arche FusionPlex Sarcoma Kit

Response 3: Thank you for this suggestion. A scientific work by Lam et al., 2018 was added in reference 7

• For completeness, MRI should be written for extent  for the first time, even though it is a widely used term

Response 4: Thank you for the comment. The magnetic resonance imaging (MRI) was added.

• Integra is a registered trademark, It should be changed (describing the thickness dermal substitute... etc etc)

Response 5: Thank you for the comment. Changes were made. Integra was replaced by split-thickness bilayer matrix meshed bovine graft

• As this is a literature review, the terms used for the search should be mentioned, in what combination and on which database. Finally, with what criteria have been discarded or kept? A "prism" type diagram would be much appreciated

Response 6: Thank you for the comment. A paragraph was added to explain the search terms and inclusion, exclusion criteria. A prism diagram was also added as figure 5.

• When introducing giant cell fibroblastoma, the acronym GCF should be introduced immediately after it.

Response 7: Thank you for the comment. The acronym GCF was  introduced immediately after giant cell fibroblastoma in the case description.

• Bigger clinical images would be more appreciated

Response 8: Thank you for the suggestion. Clinical images were enlarged.

Author Response File: Author Response.docx

Round 2

Reviewer 1 Report

Much improved

Paragraph about tufted angioma can be shortened Just mention it!

Author Response

We thank the reviewer for taking the time to review our manuscript.

 

Comment 1: Paragraph about tufted angioma can be shortened Just mention it!

Response 1: Thank you for the comment. The paragraph was shortened to only mention tufted angioma and the differentiating points from DFSP.