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JAMA Network Open
Article . 2019 . Peer-reviewed
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JAMA Network Open
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Perspectives of Patients With Dermatofibrosarcoma Protuberans on Diagnostic Delays, Surgical Outcomes, and Nonprotuberance

Authors: Marjorie Parker David; Ashley E Funderburg; James P. Selig; Rebecca Brown; Pip M. Caliskan; Lee Cove; Gayle Dicker; +3 Authors

Perspectives of Patients With Dermatofibrosarcoma Protuberans on Diagnostic Delays, Surgical Outcomes, and Nonprotuberance

Abstract

Importance Dermatofibrosarcoma protuberans (DFSP) may have a deceptively benign clinical appearance, including a nonprotuberant presentation. Patients with DFSP often perceive misdiagnoses and delays in receiving a diagnosis. Use of existing, patient-designed Facebook patient support groups (FBSGs) to recruit large numbers of patients with rare diseases may be an effective novel research method. Objectives To collaborate with patients with rare disease through social media and answer questions important to both patients and the medical field, including sources of diagnostic delay, risk of recurrence, and flat presentation of DFSP. Design, Setting, and Participants A multiple-choice survey created by a team of medical practitioners and patients with DFSP was administered to 214 patients with DFSP or family members from international DFSP FBSGs and a nonprofit foundation patient database via Lime Survey from October 30 to November 20, 2015. The survey asked questions designed to determine risk of recurrence and metastasis, surgical outcomes, sources of diagnostic delay, symptoms of recurrence, number of recurrences, scar size, and number of clinicians seen before biopsy. Statistical analysis was performed from January 1, 2016, to April 1, 2019. Main Outcomes and Measures The study goal was to collect at least 200 survey responses. Results Of 214 survey respondents (169 females and 45 males; mean [SD] age, 40.7 [12.1] years; range, <1 to 72 years), 199 were patients with DFSP and 15 were family members. Delays occurred between the patient noticing the DFSP lesion and receiving a diagnosis of DFSP (median, 4 years; range, <1 to 42 years). Most patients (112 [52.3%]) believed that they received a misdiagnosis at some point: by dermatologists (35 of 107 [32.7%]), primary care clinicians (80 of 107 [74.8%]), or another type of physician (27 of 107 [25.2%]). The most frequent prebiopsy clinical suspicion included cyst (101 [47.2%]), lipoma (30 [14.0%]), and scar (17 [7.9%]). Many patients first noticed their DFSP as a flat plaque (87 of 194 [44.8%]). Of these lesions, 73.6% (64 of 87) became protuberant eventually. Surgical treatments included Mohs micrographic surgery (56 of 194 [28.9%]), wide local excision (122 of 194 [62.9%]), and conservative excision (16 of 194 [8.2%]). The reported rate of recurrence was 5.4% (3 of 56) for Mohs micrographic surgery, 7.4% (9 of 122) for wide local excision, and 37.5% (6 of 16) for conservative excision. The higher rate of recurrence for conservative excision was significant (P = .001); there was no significant difference in the rate of recurrence between Mohs micrographic surgery and wide local excision (P = .76). Conclusions and Relevance This study reports what appears to be disease-relevant statistics from the largest survey of patients with DFSP to date. Because of the dissonance between the name of the neoplasm and its clinical presentation, the alternative term dermatofibrosarcoma, often protuberant is proposed. This study suggests that FBSGs are useful tools in medical research, providing rapid access to large numbers of patients with rare diseases and enabling synergistic collaborations between patients and medical researchers.

Key Points Question Can dermatofibrosarcoma protuberans Facebook patient support groups work with researchers to determine risk of recurrence and metastasis, surgical outcomes, symptoms of recurrence, and sources of diagnostic delay? Findings In this multiple-choice survey study, 214 patients with dermatofibrosarcoma protuberans reported a median of 4 years between noticing a lesion and receiving a correct diagnosis (range, <1 to 42 years), and many lesions (87 of 194 [44.8%]) first presented as flat. The mean age at noticing dermatofibrosarcoma protuberans was 29.6 years. Meaning Facebook patient support groups seems to provide rapid access to large numbers of patients with rare diseases and enable synergistic collaborations between savvy patients and medical researchers.

This survey study collaborates with patients with dermatofibrosarcoma protuberans through social media to evaluate sources of diagnostic delay, risk of recurrence, and flat presentation of dermatofibrosarcoma protuberans.

Subjects by Vocabulary

Microsoft Academic Graph classification: medicine.medical_specialty medicine.medical_treatment MEDLINE medicine Mohs surgery Dermatofibrosarcoma protuberans business.industry General surgery Wide local excision Clinical appearance medicine.disease Presentation (obstetrics) Risk assessment business Rare disease

Keywords

Adult, Male, Delayed Diagnosis, Skin Neoplasms, Risk Assessment, Surveys and Questionnaires, Humans, Diagnostic Errors, Protein Kinase Inhibitors, Original Investigation, Radiotherapy, Research, Dermatofibrosarcoma, General Medicine, Middle Aged, Mohs Surgery, Online Only, Interdisciplinary Placement, Self-Help Groups, Treatment Outcome, Oncology, Imatinib Mesylate, Female, Neoplasm Recurrence, Local, Social Media

16 references, page 1 of 2

1 Haller J, David MP, Lee NE, Shalin SC, Gardner JM Impact of pathologist involvement in sarcoma and rare tumor patient support groups on Facebook: a survey of 542 patients and family members. Arch Pathol Lab Med. 2018;142(9):-. doi:10.5858/arpa.2017-0408-OA 29377717 [OpenAIRE] [PubMed] [DOI]

2 Kreicher KL, Kurlander DE, Gittleman HR, Barnholtz-Sloan JS, Bordeaux JS Incidence and survival of primary dermatofibrosarcoma protuberans in the United States. Dermatol Surg. 2016;42(suppl 1):S24-S31. doi:10.1097/DSS.0000000000000300 26730971 [OpenAIRE] [PubMed] [DOI]

3 Valdivielso-Ramos M, Torrelo A, Campos M, Feito M, Gamo R, Rodriguez-Peralto JL Pediatric dermatofibrosarcoma protuberans in Madrid, Spain: multi-institutional outcomes. Pediatr Dermatol. 2014;31(6):676-682. doi:10.1111/pde.12371 25424208 [OpenAIRE] [PubMed] [DOI]

4 Llombart B, Monteagudo C, Sanmartín O, Dermatofibrosarcoma protuberans: a clinicopathological, immunohistochemical, genetic (COL1A1-PDGFB), and therapeutic study of low-grade versus high-grade (fibrosarcomatous) tumors. J Am Acad Dermatol. 2011;65(3):564-575. doi:10.1016/j.jaad.2010.06.020 21570152 [OpenAIRE] [PubMed] [DOI]

5 Zhou X, Sun D, Liu Y, Dermatofibrosarcoma protuberans: our 10-year experience on 80 patients [published online June 9, 2019]. J Dermatolog Treat. 31116621 [OpenAIRE] [PubMed]

6 DuBay D, Cimmino V, Lowe L, Johnson TM, Sondak VK Low recurrence rate after surgery for dermatofibrosarcoma protuberans: a multidisciplinary approach from a single institution. Cancer. 2004;100(5):1008-1016. doi:10.1002/cncr.20051 14983497 [OpenAIRE] [PubMed] [DOI]

7 Martin L, Piette F, Blanc P, ; French Group for Cutaneous Oncology Clinical variants of the preprotuberant stage of dermatofibrosarcoma protuberans. Br J Dermatol. 2005;153(5):932-936. doi:10.1111/j.1365-2133.2005.06823.x 16225602 [OpenAIRE] [PubMed] [DOI]

8 Liang CA, Jambusaria-Pahlajani A, Karia PS, Elenitsas R, Zhang PD, Schmults CD A systematic review of outcome data for dermatofibrosarcoma protuberans with and without fibrosarcomatous change. J Am Acad Dermatol. 2014;71(4):781-786. doi:10.1016/j.jaad.2014.03.018 24755121 [OpenAIRE] [PubMed] [DOI]

9 National Comprehensive Cancer Network. 2018 NCCN DFSP guidelines. https://www.nccn.org/professionals/physician_gls/pdf/dfsp.pdf. Accessed July 8, 2018.

10 Lowe GC, Onajin O, Baum CL, A comparison of Mohs micrographic surgery and wide local excision for treatment of dermatofibrosarcoma protuberans with long-term follow-up: the Mayo Clinic experience. Dermatol Surg. 2017;43(1):98-106. doi:10.1097/DSS.0000000000000910 27749444 [OpenAIRE] [PubMed] [DOI]

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citations
This is an alternative to the "Influence" indicator, which also reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
BIP!Citations provided by BIP!
popularity
This indicator reflects the "current" impact/attention (the "hype") of an article in the research community at large, based on the underlying citation network.
BIP!Popularity provided by BIP!
influence
This indicator reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
BIP!Influence provided by BIP!
impulse
This indicator reflects the initial momentum of an article directly after its publication, based on the underlying citation network.
BIP!Impulse provided by BIP!
26
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