Panda BB, Gunasekar S, Agarwal U, Koppalu Lingaraju T, Adhya AK. Recurrent dermatofibrosarcoma protuberans involving the lacrimal sac: A case report. World J Clin Cases 2025; 13(30): 111020 [DOI: 10.12998/wjcc.v13.i30.111020]
Corresponding Author of This Article
Bijnya Birajita Panda, Assistant Professor, Department of Ophthalmology, All India Institute of Medical Sciences, Sijua, Bhubaneshwar 751019, Odisha, India. bigyan_panda@yahoo.co.in
Research Domain of This Article
Ophthalmology
Article-Type of This Article
Case Report
Open-Access Policy of This Article
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Bijnya Birajita Panda, Utkarsh Agarwal, Thilakraj Koppalu Lingaraju, Department of Ophthalmology, All India Institute of Medical Sciences, Bhubaneshwar 751019, Odisha, India
Sudhakar Gunasekar, Department of Surgical Oncology, Jawaharlal Institute of Postgraduate Medical Education & Research, Tamil Nadu 110029, India
Amit Kumar Adhya, Department of Pathology, All India Institute of Medical Sciences, Bhubaneshwar 751019, Odisha, India
Author contributions: Panda BB designed and conducted the study; Panda BB and Koppalu Lingaraju T wrote the manuscript; Agarwal U provided clinical input; Panda BB and Gunasekar S critically analyzed the manuscript and provided crucial input that was essential for preparing the final version of the manuscript; Agarwal U and Koppalu Lingaraju T acquired the clinical data, prepared the first draft of the manuscript, searched the literature, and were responsible for the preparation of figures; Adhya AK provided histopathological data and figure preparation and critically analyzed the manuscript.
Informed consent statement: Informed written consent was obtained from the patient for publication of this report and any accompanying images.
Conflict-of-interest statement: The authors have no conflicts of interest to declare.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Bijnya Birajita Panda, Assistant Professor, Department of Ophthalmology, All India Institute of Medical Sciences, Sijua, Bhubaneshwar 751019, Odisha, India. bigyan_panda@yahoo.co.in
Received: June 24, 2025 Revised: July 18, 2025 Accepted: August 13, 2025 Published online: October 26, 2025 Processing time: 112 Days and 22.6 Hours
Abstract
BACKGROUND
Dermatofibrosarcoma protuberans (DFSP) is a rare, low-grade, locally aggressive cutaneous sarcoma. DFSP in the periocular region is exceedingly rare, leading to diagnostic and surgical challenges due to anatomical constraints in the periocular region. Precise diagnosis is essential to guide appropriate surgical management and prevent recurrence.
CASE SUMMARY
A 32-year-old female presented with a recurrent tumor in the medial canthus, previously diagnosed as a solitary fibrous tumor in an outside institution. After complete radiological and systemic workup, she was scheduled for a wide local excision followed by reconstruction after getting tumor clear margins on frozen section. Histopathology confirmed DFSP, characterized by storiform spindle cell proliferation, diffuse cluster of differentiation 34 positivity, and signal transducer and activator of transcription 6 negativity.
CONCLUSION
This case highlights the challenges in the diagnostic and surgical management of DFSP in periocular tumors. Comprehensive surgical excision with appropriate reconstruction is critical for achieving oncological control while preserving aesthetics and function.
Core Tip: Periocular dermatofibrosarcoma protuberans (DFSP) of the lacrimal sac is extremely rare, often mimicking benign or inflammatory lesions, leading to delayed diagnosis. High clinical suspicion is warranted for slowly enlarging, firm, painless medial canthal masses. Histopathology with cluster of differentiation 34 immunopositivity confirms the diagnosis. Complete surgical excision with histologically clear margins is crucial to prevent recurrence, with consideration for Mohs micrographic surgery in select cases. Long-term follow-up is essential due to the locally aggressive nature of DFSP and potential for late recurrence. Early recognition and meticulous margin control are key to optimal functional and cosmetic outcomes.
