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Nimodia D, Dudhe S, Parihar PH, Patil R, Kotla R, Choudhary A. From subtle to striking: A rare case of dermatofibroma protuberans and its clinical journey. Radiol Case Rep 2025; 20:2557-2561. [PMID: 40129841 PMCID: PMC11930717 DOI: 10.1016/j.radcr.2025.02.045] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/27/2024] [Revised: 02/06/2025] [Accepted: 02/10/2025] [Indexed: 03/26/2025] Open
Abstract
Dermatofibrosarcoma protuberans (DFSP) is a rare type of soft tissue sarcoma, which is slow-growing. It arises from the dermal, subcutaneous layer and is locally aggressive. It commonly affects adults between 20 and 50 years old, with a slight male predominance. This case report emphasizes the case of a 35-year-old male patient who presented with a large, firm, nodular mass on his left upper arm that had been progressively increasing in size over 3 years. On inspection, the lesion measured approximately 13 cm in diameter, was large and lobulated with overlying red-brown discolouration. The patient reported movement discomfort, and mild itching was noted. Diagnostic evaluation was advised, including X-ray, Ultrasound, Computed Tomography and Magnetic resonance imaging. A provisional diagnosis of soft tissue sarcoma was made. An excisional biopsy was performed, and histopathological analysis demonstrated a storiform pattern of spindle-shaped cells infiltrating the dermis and subcutis, consistent with dermatofibrosarcoma protuberans. The patient underwent wide local excision with clear margins to prevent recurrence, and no metastasis was detected. Dermatofibrosarcoma protuberans is characterized by a high rate of local recurrence but a low risk of metastasis. Early diagnosis and complete surgical excision are treatment options for good prognosis. This case underscores the importance of diagnosing and treating Dermatofibrosarcoma protuberans without delays.
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Affiliation(s)
- Devyansh Nimodia
- Department of Radiodiagnosis, Datta Meghe Institute of Medical Sciences, Wardha, Maharashtra, India 442001
| | - Sakshi Dudhe
- Department of Radiodiagnosis, Datta Meghe Institute of Medical Sciences, Wardha, Maharashtra, India 442001
| | | | - Ravishankar Patil
- Department of Radiodiagnosis, Datta Meghe Institute of Medical Sciences, Wardha, Maharashtra, India 442001
| | - Rishitha Kotla
- Department of Psychiatry, Datta Meghe Institute of Medical Sciences, Wardha, Maharashtra, India 442001
| | - Abhishek Choudhary
- Department of Orthopedics, Datta Meghe Institute of Medical Sciences, Wardha, Maharashtra, India 442001
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2
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Alenezy K, Dashti Y, Dashti M, Alenezi M. Multiple Eruptive Dermatofibromas: A Case Series and Review of Diagnostic Challenges and Systemic Associations. Cureus 2025; 17:e77929. [PMID: 39991326 PMCID: PMC11847600 DOI: 10.7759/cureus.77929] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 01/24/2025] [Indexed: 02/25/2025] Open
Abstract
Multiple eruptive dermatofibromas (MEDF) is a rare dermatological condition characterized by the sudden appearance of multiple benign skin lesions, often associated with systemic or autoimmune conditions. This case series describes three female patients with MEDF, highlighting their distinct clinical features and systemic associations. Diagnoses were confirmed through consistent histopathological findings, demonstrating the importance of integrating clinical, dermoscopic, and histopathological evaluations in diagnosing MEDF. The differential diagnosis included other dermatological conditions with overlapping features, which were excluded based on clinical and pathological findings. This report emphasizes the potential role of immune dysregulation and genetic predisposition in MEDF pathogenesis. It underscores the need for further research to understand its mechanisms better, improve diagnostic accuracy, and establish standardized management approaches.
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Affiliation(s)
| | - Yousef Dashti
- Dermatology, Mubarak Al-Kabeer Hospital, Jabriya, KWT
| | - Maryam Dashti
- Dermatology, Mubarak Al-Kabeer Hospital, Jabriya, KWT
| | - Manar Alenezi
- Dermatology, Mubarak Al-Kabeer Hospital, Jabriya, KWT
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3
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Irshad A, Akram MA, Ijaz S, Rehman A, Khalid A, Iftikhar M. Management of Forehead Dermatofibrosarcoma Protuberans With Wide Local Excision and Paramedian Forehead Flap Reconstruction: A Case Report. Cureus 2024; 16:e76322. [PMID: 39850162 PMCID: PMC11756705 DOI: 10.7759/cureus.76322] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 12/24/2024] [Indexed: 01/25/2025] Open
Abstract
Dermatofibrosarcoma protuberans (DFSP) is a rare, low to intermediate-grade soft tissue sarcoma that presents significant diagnostic and therapeutic challenges. We report the case of a 40-year-old male patient who presented with a slow-growing, asymptomatic lesion on his forehead that had developed over two years. Clinical examination revealed a firm, non-tender multinodular mass measuring 5 x 3 cm in the supraorbital region. The diagnosis was confirmed through histopathological examination and immunohistochemical staining, which demonstrated the characteristic storiform pattern and CD34 positivity. The patient underwent wide local excision with 3 cm margins, followed by reconstruction using a paramedian forehead flap. The procedure involved upper and lower eyelid release, lateral canthotomy, and canthoplasty to facilitate proper reconstruction. Postoperative recovery was satisfactory, though a subsequent procedure was required for further excision and secondary healing of a residual lesion. This case highlights the importance of early recognition, appropriate imaging, and the complex surgical approach often required for complete excision and reconstruction of DFSP in anatomically challenging areas. Long-term follow-up remains essential due to the high recurrence risk associated with this neoplasm.
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Affiliation(s)
| | | | - Sajid Ijaz
- Plastic and Reconstructive Surgery, Mayo Hospital, Lahore, PAK
| | | | - Amna Khalid
- Internal Medicine, Northampton General Hospital NHS Trust, Northampton, GBR
| | - Maryam Iftikhar
- Pathology, Margalla Institute of Health Sciences, Rawalpindi, PAK
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4
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Viedma-Martínez M, Millán-Cayetano JF, Grassi-García C, Linares-Barrios M. [Translated article] Dermatofibrosarcoma Protuberans: Hospital Casuistry and Comparative Analysis in the Management of This Entity by the Dermatology Unit Compared to Other Units. ACTAS DERMO-SIFILIOGRAFICAS 2024; 115:T1049-T1051. [PMID: 39454854 DOI: 10.1016/j.ad.2024.10.055] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/22/2023] [Revised: 05/11/2023] [Accepted: 05/14/2023] [Indexed: 10/28/2024] Open
Affiliation(s)
- M Viedma-Martínez
- Departamento de Dermatología, Hospital Universitario Puerta del Mar, Cádiz, Spain.
| | - J F Millán-Cayetano
- Departamento de Dermatología, Hospital Universitario Puerta del Mar, Cádiz, Spain
| | - C Grassi-García
- Departamento de Cirugía Plástica, Hospital Universitario Puerta del Mar, Cádiz, Spain
| | - M Linares-Barrios
- Departamento de Dermatología, Hospital Universitario Puerta del Mar, Cádiz, Spain
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5
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Viedma-Martínez M, Millán-Cayetano JF, Grassi-García C, Linares-Barrios M. Dermatofibrosarcoma Protuberans: Hospital Casuistry and Comparative Analysis in the Management of This Entity by the Dermatology Unit Compared to Other Units. ACTAS DERMO-SIFILIOGRAFICAS 2024; 115:1049-1051. [PMID: 38552782 DOI: 10.1016/j.ad.2023.05.040] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/22/2023] [Revised: 05/11/2023] [Accepted: 05/14/2023] [Indexed: 05/26/2024] Open
Affiliation(s)
- M Viedma-Martínez
- Departamento de Dermatología, Hospital Universitario Puerta del Mar, Cádiz, España.
| | - J F Millán-Cayetano
- Departamento de Dermatología, Hospital Universitario Puerta del Mar, Cádiz, España
| | - C Grassi-García
- Departamento de Cirugía Plástica, Hospital Universitario Puerta del Mar, Cádiz, España
| | - M Linares-Barrios
- Departamento de Dermatología, Hospital Universitario Puerta del Mar, Cádiz, España
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6
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Yogesh TL, Jha AK, Jayaraman S, Jayaraman V. Low-grade dermatofibrosarcoma protuberance - A rare case report. J Oral Maxillofac Pathol 2024; 28:315-320. [PMID: 39157824 PMCID: PMC11329102 DOI: 10.4103/jomfp.jomfp_135_23] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/16/2023] [Revised: 09/15/2023] [Accepted: 10/09/2023] [Indexed: 08/20/2024] Open
Abstract
Dermatofibrosarcoma protuberans (DFSP) is a slow-growing, soft-tissue tumour of early or mid-adult life, affecting both the genders equally. The most common sites are soft tissue of the trunk (50 to 60%), followed by proximal extremities (20 to 30%) and the head and neck region (10 to 15%). Its metastatic potential is low though the local recurrence rate is high. Here, we report a case of a female patient with a large soft tissue growth located at the right cheek, chin and neck region. Local excision was done under the impression of a benign tumour such as lipoma or sebaceous cyst. Histological evaluation showed bland spindle cells arranged in a storiform pattern questioning the provisional diagnosis of the lesion. Further evaluation with the immunohistochemistry (IHC) panel confirmed the diagnosis of DFSP. Since it is a rare tumour of the head and neck region with non-alarming initial presentation and the potential for erroneous diagnosis as another lesion, we present a case of DFSP.
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Affiliation(s)
- T. L. Yogesh
- Department of Oral Pathology and Microbiology, Rajiv Gandhi College of Dental Sciences and Research Centre, Hebbal, Bengaluru, Karnataka, India
| | - Anjani Kumar Jha
- Department of Oral and Maxillofacial Surgery, Mithila Minority Dental College and Hospital, Ekmighat, Darbhanga, Bihar, India
| | - Sindhumati Jayaraman
- Department of Oral Pathology and Microbiology, Rajiv Gandhi College of Dental Sciences and Research Centre, Hebbal, Bengaluru, Karnataka, India
| | - Vidhya Jayaraman
- Department of Oral Pathology and Microbiology, Rajiv Gandhi College of Dental Sciences and Research Centre, Hebbal, Bengaluru, Karnataka, India
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Zeaiter N, Aoun CB, Sfeir J, Ghandour M, Hreibe W. Dermatofibrosarcoma Protuberans Arising From a Chronic Wound in the Left Shoulder: A Case Report. Cureus 2024; 16:e55638. [PMID: 38586739 PMCID: PMC10995738 DOI: 10.7759/cureus.55638] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 03/06/2024] [Indexed: 04/09/2024] Open
Abstract
Dermatofibrosarcoma protuberans (DFSP) is a rare, slow-growing soft tissue sarcoma, typically presenting as a cutaneous lesion. However, its occurrence in chronic wounds is infrequently documented, posing diagnostic and therapeutic challenges. This report details the case of a 59-year-old female with no significant medical history, presenting with a chronic, non-healing wound on the left shoulder, persisting for three years. Initially a small nodule, it progressed into an ulcerating lesion. Physical examination revealed a contracted scar with restricted shoulder mobility. After obtaining informed consent, a surgical excision of the lesion was performed by an electrocautery. Histopathology confirmed DFSP, characterized by spindle fibrous cells, with skin ulceration and deep dermal infiltration. A split-thickness skin graft achieved successful closure. This case underscores the importance of considering DFSP in chronic, non-healing wounds. Timely intervention and appropriate surgical management are crucial for favorable outcomes.
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Affiliation(s)
- Nancy Zeaiter
- Plastic and Reconstructive Surgery, Lebanese University, Beirut, LBN
- Plastic and Reconstructive Surgery, Al-Zahraa Hospital University Medical Center, Beirut, LBN
| | - Charbel B Aoun
- Plastic and Reconstructive Surgery, Lebanese University, Beirut, LBN
- Plastic and Reconstructive Surgery, Al-Zahraa Hospital University Medical Center, Beirut, LBN
| | - Joseph Sfeir
- Plastic and Reconstructive Surgery, Lebanese University, Beirut, LBN
- Plastic and Reconstructive Surgery, Al-Zahraa Hospital University Medical Center, Beirut, LBN
| | | | - Walid Hreibe
- Plastic and Reconstructive Surgery, Lebanese University, Beirut, LBN
- Plastic and Reconstructive Surgery, Al-Zahraa Hospital University Medical Center, Beirut, LBN
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8
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Ge LL, Wang ZC, Wei CJ, Huang JX, Liu J, Gu YH, Wang W, Li QF. Unraveling intratumoral complexity in metastatic dermatofibrosarcoma protuberans through single-cell RNA sequencing analysis. Cancer Immunol Immunother 2023; 72:4415-4429. [PMID: 37938367 PMCID: PMC10992304 DOI: 10.1007/s00262-023-03577-2] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/01/2023] [Accepted: 10/31/2023] [Indexed: 11/09/2023]
Abstract
Dermatofibrosarcoma protuberans (DFSP) stands as a rare and locally aggressive soft tissue tumor, characterized by intricated molecular alterations. The imperative to unravel the complexities of intratumor heterogeneity underscores effective clinical management. Herein, we harnessed single-cell RNA sequencing (scRNA-seq) to conduct a comprehensive analysis encompassing samples from primary sites, satellite foci, and lymph node metastases. Rigorous preprocessing of raw scRNA-seq data ensued, and employing t-distributed stochastic neighbor embedding (tSNE) analysis, we unveiled seven major cell populations and fifteen distinct subpopulations. Malignant cell subpopulations were delineated using infercnv for copy number variation calculations. Functional and metabolic variations of diverse malignant cell populations across samples were deciphered utilizing GSVA and the scMetabolism R packages. Additionally, the exploration of differentiation trajectories within diverse fibroblast subpopulations was orchestrated through pseudotime trajectory analyses employing CytoTRACE and Monocle2, and further bolstered by GO analyses to elucidate the functional disparities across distinct differentiation states. In parallel, we segmented the cellular components of the immune microenvironment and verified the presence of SPP1+ macrophage, which constituted the major constituent in lymph node metastases. Remarkably, the CellChat facilitated a comprehensive intercellular communication analysis. This study culminates in an all-encompassing single-cell transcriptome atlas, propounding novel insights into the multifaceted nature of intratumor heterogeneity and fundamental molecular mechanisms propelling metastatic DFSP.
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Affiliation(s)
- Ling-Ling Ge
- Department of Plastic and Reconstructive Surgery, Shanghai Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, 639 Zhizaoju Road, Shanghai, 200011, People's Republic of China
| | - Zhi-Chao Wang
- Department of Plastic and Reconstructive Surgery, Shanghai Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, 639 Zhizaoju Road, Shanghai, 200011, People's Republic of China
| | - Cheng-Jiang Wei
- Department of Plastic and Reconstructive Surgery, Shanghai Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, 639 Zhizaoju Road, Shanghai, 200011, People's Republic of China
| | - Jing-Xuan Huang
- Department of Plastic and Reconstructive Surgery, Shanghai Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, 639 Zhizaoju Road, Shanghai, 200011, People's Republic of China
| | - Jun Liu
- Department of Plastic and Reconstructive Surgery, Shanghai Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, 639 Zhizaoju Road, Shanghai, 200011, People's Republic of China
| | - Yi-Hui Gu
- Department of Plastic and Reconstructive Surgery, Shanghai Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, 639 Zhizaoju Road, Shanghai, 200011, People's Republic of China
| | - Wei Wang
- Department of Plastic and Reconstructive Surgery, Shanghai Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, 639 Zhizaoju Road, Shanghai, 200011, People's Republic of China
| | - Qing-Feng Li
- Department of Plastic and Reconstructive Surgery, Shanghai Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, 639 Zhizaoju Road, Shanghai, 200011, People's Republic of China.
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9
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Alhabeeb AY, Idrees AO, Alhowaish TS, Alhamadh MS, Masudi E, Alanazi A, Aljuhani W. Outcome of Surgical Management of Dermatofibrosarcoma Protuberance: A Single-Institution Multidisciplinary Approach. Orthop Res Rev 2023; 15:237-243. [PMID: 38028653 PMCID: PMC10675642 DOI: 10.2147/orr.s437175] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/25/2023] [Accepted: 11/09/2023] [Indexed: 12/01/2023] Open
Abstract
Introduction Dermatofibrosarcoma protuberans (DFSP) is a rare, slow-growing, and locally aggressive soft tissue tumor with a high recurrence rate and metastatic potential, even with the proper treatment. Methods This was a retrospective (case series) study that took place at King Abdulaziz Medical City, Riyadh, Kingdom of Saudi Arabia, to determine the outcomes of and appropriate margin excision for DFSP. All patients who were diagnosed with DFSP from 2016 to 2021 were included. The following variables were assessed: demographics, tumor characteristics, management options, and most importantly, whether patients were managed with an oncology-oriented approach or a non-oncology-oriented approach. Results There were a total of seventeen patients with DFSP, four of whom had fibrosarcomatous differentiation (FS-DFSP). The majority (N = 13, 76.5%) of the patients were females. The lower extremities and back were the most common locations for DFSP, accounting for 47.1% and 23.5%, respectively. Only two (11.76%) patients had metastatic disease, one of whom had FS-DFSP. The minimum resection margin was 3 cm, and the maximum was 5 cm. Thirteen (76.47%) patients were managed with an oncology-oriented approach (Group I), 23% of whom had post-excision positive margins. However, all patients who were managed with a non-oncology-oriented approach (Group II) had positive margins post-excision. More than three-quarters (76.9%) of group I underwent wide resection. Split-thickness skin grafting and primary closure were the most commonly used reconstruction methods in groups I and II, respectively. The mean planned margins in groups I and II were 3.9 cm and 1.7 cm, respectively. Conclusion The findings of this study suggest that a planned wide-margin excision with a minimum safe margin of 3-5 cm should be implemented to reduce the recurrence, metastasis, and need for further surgeries in patients with DFSP.
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Affiliation(s)
- Abdulrahman Yousef Alhabeeb
- College of Medicine, King Saud bin Abdulaziz University for Health Sciences, Ministry of the National Guard-Health Affairs, Riyadh, Kingdom of Saudi Arabia
- King Abdullah International Medical Research Center, Ministry of the National Guard-Health Affairs, Riyadh, Kingdom of Saudi Arabia
| | - Ahmed O Idrees
- King Abdullah International Medical Research Center, Ministry of the National Guard-Health Affairs, Riyadh, Kingdom of Saudi Arabia
- Department of Orthopedic Surgery, King Abdulaziz Medical City, Ministry of National Guard - Health Affairs, Riyadh, Saudi Arabia
| | - Thamer S Alhowaish
- King Abdullah International Medical Research Center, Ministry of the National Guard-Health Affairs, Riyadh, Kingdom of Saudi Arabia
- Division of Neurology, King Abdulaziz Medical City, Ministry of the National Guard Health Affairs, Riyadh, Kingdom of Saudi Arabia
| | - Moustafa S Alhamadh
- College of Medicine, King Saud bin Abdulaziz University for Health Sciences, Ministry of the National Guard-Health Affairs, Riyadh, Kingdom of Saudi Arabia
- King Abdullah International Medical Research Center, Ministry of the National Guard-Health Affairs, Riyadh, Kingdom of Saudi Arabia
| | - Emad Masudi
- College of Medicine, King Saud bin Abdulaziz University for Health Sciences, Ministry of the National Guard-Health Affairs, Riyadh, Kingdom of Saudi Arabia
- King Abdullah International Medical Research Center, Ministry of the National Guard-Health Affairs, Riyadh, Kingdom of Saudi Arabia
| | - Abdullah Alanazi
- College of Medicine, King Saud bin Abdulaziz University for Health Sciences, Ministry of the National Guard-Health Affairs, Riyadh, Kingdom of Saudi Arabia
- King Abdullah International Medical Research Center, Ministry of the National Guard-Health Affairs, Riyadh, Kingdom of Saudi Arabia
| | - Wazzan Aljuhani
- King Abdullah International Medical Research Center, Ministry of the National Guard-Health Affairs, Riyadh, Kingdom of Saudi Arabia
- Department of Orthopedic Surgery, King Abdulaziz Medical City, Ministry of National Guard - Health Affairs, Riyadh, Saudi Arabia
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Stoneham S, Hunter A, Raahimi M, Livesey A, Mitchell CD, Keohane S. Cutaneous sarcoma: a review and practical approach to management. Clin Exp Dermatol 2023; 48:866-872. [PMID: 37127912 DOI: 10.1093/ced/llad166] [Citation(s) in RCA: 4] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/19/2022] [Accepted: 04/18/2023] [Indexed: 05/03/2023]
Abstract
Sarcomas arising in the skin are rare but potentially fatal. These tumours originate from mesenchymal cells and can be divided between those that arise in soft tissue and those arising from bone. General guidelines exist for the management of soft-tissue sarcomas; however, there are no specific guidelines for cutaneous sarcomas. Current literature was reviewed for management of seven cutaneous sarcomas including atypical fibroxanthoma, pleomorphic dermal sarcoma, dermal and subcutaneous leiomyosarcoma, dermatofibroma sarcoma protuberans, Kaposi sarcoma, cutaneous angiosarcoma and malignant peripheral nerve sheath tumour. All suspected sarcomas should be discussed in a sarcoma multidisciplinary team meeting. This article is not a clinical guideline but should serve as a practical summary of how these tumours present, how they are recognized histologically, and how best to manage and follow-up patients. The aim is to support clinicians and facilitate the best and most evidence-based standard of care available.
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Affiliation(s)
- Sophie Stoneham
- Department of Dermatology, St Mary's Hospital, Portsmouth Hospitals University NHS Trust, Portsmouth, UK
| | - Alys Hunter
- Department of Dermatology, St Mary's Hospital, Portsmouth Hospitals University NHS Trust, Portsmouth, UK
| | - Mina Raahimi
- Department of Dermatology, St Mary's Hospital, Portsmouth Hospitals University NHS Trust, Portsmouth, UK
| | - Amy Livesey
- Department of Dermatology, St Mary's Hospital, Portsmouth Hospitals University NHS Trust, Portsmouth, UK
| | - Charles D Mitchell
- Department of Dermatology, St Mary's Hospital, Portsmouth Hospitals University NHS Trust, Portsmouth, UK
| | - Stephen Keohane
- Department of Dermatology, St Mary's Hospital, Portsmouth Hospitals University NHS Trust, Portsmouth, UK
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11
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Go CC, Lahaie Luna GM, Briceño CA. Epidemiological trends and survival outcomes for dermatofibrosarcoma protuberans of the head and neck region. Int J Dermatol 2023; 62:664-671. [PMID: 36318642 DOI: 10.1111/ijd.16459] [Citation(s) in RCA: 2] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/09/2022] [Revised: 08/16/2022] [Accepted: 10/06/2022] [Indexed: 04/19/2023]
Abstract
BACKGROUND Given the rarity of dermatofibrosarcoma protuberans (DFSP) of the head and neck, the incidence and prognosis specific to this region are poorly defined. The purpose of this study was to determine epidemiology, clinicopathological characteristics, and prognostic factors of patients with DFSP of the head and neck region, using the Surveillance, Epidemiology, and End Results (SEER) database. METHODS A cohort analysis was performed for primary head and neck DFSP reported to the SEER database between 2000 and 2018. Overall survival was determined using Kaplan-Meier analysis while Cox regression modeling was used to examine predictive factors. RESULTS A total of 681 cases were reported to the SEER database between 2000 and 2018. Incidence rates decreased over time. Overall survival was 94%, and disease-specific survival was 99% at 5 years. Cases of head and neck DFSP were found to occur more frequently in males. There was no difference in incidence rates between White patients and Black patients. Age ≥ 60 years old, tumor size, and living location were the most significant predictors of overall survival. CONCLUSIONS This analysis of DFSP of the head and neck demonstrates a downward trend in incidence, higher age-standardized incidence in males, and similar race-based incidences, which differs from data reported on DFSP of other anatomic locations.
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Affiliation(s)
- Cammille C Go
- Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA
| | - Gabriela M Lahaie Luna
- Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA
- Scheie Eye Institute, Philadelphia, PA, USA
- Department of Ophthalmology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA
| | - César A Briceño
- Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA
- Scheie Eye Institute, Philadelphia, PA, USA
- Department of Ophthalmology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA
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12
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Panwar V, Liu Y, Gwin K, Chen H. COL1A1-PDGFB Fusion Associated Fibrosarcoma of the Uterine Corpus: A Case Report and Literature Review. Int J Gynecol Pathol 2023; 42:143-146. [PMID: 36729934 DOI: 10.1097/pgp.0000000000000850] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/03/2023]
Abstract
Uterine collagen type 1 alpha 1 (COL1A1) and platelet-derived growth factor beta chain (PDGFB) fusion associated fibrosarcoma is a recently described entity characterized by a specific translocation t(17;22) (q22;q13) leading to the formation of COL1A1-PDGFB fusion transcripts that are typically associated with dermatofibrosarcoma protuberans. So far, only 4 cases of COL1A1-PDGFB fusion associated fibrosarcoma involving the female reproductive system have been reported in the literature. All cases showed strong diffuse expression of CD34. COL1A1-PDGFB fusion associated fibrosarcomas are aggressive tumors with a propensity for chemotherapy resistance and a poor prognosis. We are reporting the fifth case of a uterine COL1A1-PDGFB fusion associated fibrosarcoma. A 58-yr-old female presented with a large uterine mass with extension into bilateral pelvic sidewalls, mesentery of rectosigmoid colon and the vagina. A hysterectomy, bilateral salpingo-oophorectomy, and tumor debulking procedure was performed. Microscopic evaluation revealed a mitotically active cellular spindle cell neoplasm with focal osteoclast like giant cells, myxoid changes and necrosis. CD34 was diffusely and strongly positive throughout the tumor. Next-generation sequencing showed presence of the COL1A1-PDGFB fusion. The patient was treated with multiple chemotherapy regimens, however, progressed under therapy with worsening symptoms and development of extensive pelvic disease. She died of disease 13 mo after the initial diagnosis. In summary, uterine COL1A1-PDGFB fusion associated fibrosarcomas are rare tumors with aggressive clinical behavior that need to be considered in the differential diagnosis of CD34-positive uterine spindle cell neoplasms. Novel treatment options may include imatinib, a tyrosine kinase inhibitor used for treatment of advanced and unresectable dermatofibrosarcoma protuberans that was given in 1 uterine sarcoma case and showed promising initial response.
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Affiliation(s)
- Vandana Panwar
- Department of Pathology, University of Texas Southwestern Medical Center, Dallas, Texas
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13
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Martin ECS, Vyas KS, Batbold S, Erwin PJ, Brewer JD. Dermatofibrosarcoma Protuberans Recurrence After Wide Local Excision Versus Mohs Micrographic Surgery: A Systematic Review and Meta-Analysis. Dermatol Surg 2022; 48:479-485. [PMID: 35353755 DOI: 10.1097/dss.0000000000003411] [Citation(s) in RCA: 13] [Impact Index Per Article: 4.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
Abstract
BACKGROUND Local recurrence (LR) rates of dermatofibrosarcoma protuberans (DFSP) treated with different surgical modalities are unknown. OBJECTIVE To evaluate the differences in LR rates of DFSP treated with wide local excision (WLE) versus Mohs micrographic surgery (MMS). MATERIALS AND METHODS Pertinent studies of DFSP treated with either WLE or MMS were identified through a search of multiple databases, including Ovid MEDLINE (1946-2018), Embase (1988-2018), Web of Science (1975-2018), and Scopus (1970-2018). Comparative 2-arm and noncomparative single-arm studies were assessed through meta-analyses. RESULTS Of the 517 studies identified, 88 met inclusion criteria (12 comparative studies; 76 single-arm studies). In the 12 comparative studies, 352 patients with DFSP underwent MMS and 777 patients with DFSP underwent WLE. The LR rate was 1.7% after MMS and 3.7% after WLE (odds ratio, 1.549; 95% CI, 0.710-3.381; p = .27). In the 76 noncomparative studies, 980 patients underwent MMS (LR rate, 1.5%; 95% CI, 0.9%-2.1%; p < .001), and 2,215 patients underwent WLE (LR rate, 9.4%; 95% CI, 7.5%-11.3%; p < .001). CONCLUSION The LR rate of DFSP in patients treated with MMS is lower than in patients treated with WLE. Because of high rates of postoperative DFSP LR, MMS should be strongly considered when available.
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Affiliation(s)
- Emma C St Martin
- Mayo Clinic Alix School of Medicine, Minnesota campus, Rochester, Minnesota
| | - Krishna S Vyas
- Mayo Clinic School of Graduate Medical Education, Mayo Clinic College of Medicine and Science, Rochester, Minnesota
| | - Sarah Batbold
- Mayo Clinic Alix School of Medicine, Minnesota campus, Rochester, Minnesota
| | | | - Jerry D Brewer
- Department of Dermatology, Division of Dermatologic Surgery, Mayo Clinic, Rochester, Minnesota
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Hermans S, Clarysse K, Las DE, de Mey J, Mommaerts MY, De Brucker Y. Massive Facial Presentation of Dermatofibrosarcoma Protuberans. Case Rep Radiol 2022; 2022:2953579. [PMID: 35535304 PMCID: PMC9078855 DOI: 10.1155/2022/2953579] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/02/2021] [Accepted: 04/08/2022] [Indexed: 11/23/2022] Open
Abstract
Dermatofibrosarcoma protuberans is a low-grade cutaneous sarcoma typically located on the trunk or proximal extremities. Less common locations include the head, face, and neck area. This tumour is slow growing with variable clinical appearance. It is known for its locally invasive nature and low metastatic propensity. Because imaging findings are rather nonspecific, biopsy is needed for definite diagnosis. This case describes an unusually large example of dermatofibrosarcoma protuberans in the less common preauricular region.
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Affiliation(s)
- Sebastiaan Hermans
- Department of Radiology and Medical Imaging, University Hospital Brussels, Laarbeeklaan 101, B-1090 Brussels, Belgium
| | - Karlijn Clarysse
- Vrije Universiteit Brussel (VUB), Universitair Ziekenhuis Brussel (UZ Brussel), Department of Dermatology, SKIN research group, Laarbeeklaan 101, 1090 Brussels, Belgium
| | - David Emmanuel Las
- European Face Centre, University Hospital Brussels, Laarbeeklaan 101, B-1090 Brussels, Belgium
| | - Johan de Mey
- Department of Radiology and Medical Imaging, University Hospital Brussels, Laarbeeklaan 101, B-1090 Brussels, Belgium
| | - Maurice Yves Mommaerts
- European Face Centre, University Hospital Brussels, Laarbeeklaan 101, B-1090 Brussels, Belgium
| | - Yannick De Brucker
- Department of Radiology and Medical Imaging, University Hospital Brussels, Laarbeeklaan 101, B-1090 Brussels, Belgium
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15
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Brahmachari S, Pandey A, Singh MP, Agarwal V. An Integrated Surgical Management for Giant Dermatofibrosarcoma Protuberans of Anterior Abdominal Wall. Cureus 2021; 13:e17038. [PMID: 34405080 PMCID: PMC8354299 DOI: 10.7759/cureus.17038] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 08/09/2021] [Indexed: 11/23/2022] Open
Abstract
Giant dermatofibrosarcoma protuberans (DFSP) is a very rare dermal sarcoma whose diagnosis and management are important because of the high local recurrence but low metastatic potential. Complete surgical excision of giant DFSP in a single stage is difficult but has a high cure rate. A 47-year-old man presented with a gradually increasing large (18 x 15 x 7 cm) DFSP in the epigastrium. A 3 cm circumferential wide local excision (WLE) with microscopic tumor-free margin confirmed by frozen section was performed. Immediate single staged tension-free primary closure of resultant defect was done on the principle of abdominal wall reconstruction (AWR) in ventral hernia repair. This technique of anterior component separation and bridge meshplasty is functional, avoids multiple surgeries, is cost-effective, and can be done in a resource-limited setting in developing countries. A multidisciplinary and integrated surgical approach to treat giant DFSP over epigastrium, by three-dimensional WLE and immediate AWR with anterior component separation technique (CST) and bridging meshplasty, can be of immense help in managing such rare cases in developing countries.
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Affiliation(s)
- Swagata Brahmachari
- Department of General Surgery, All India Institute of Medical Sciences, Bhopal, IND
| | - Anubha Pandey
- Department of Pathology, Atal Bihari Vajpayee Government Medical College, Vidisha, IND
| | | | - Vandana Agarwal
- Department of Pathology, LN Medical College and Research Centre, Bhopal, IND
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16
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Xiong JX, Cai T, Hu L, Chen XL, Huang K, Chen AJ, Wang P. Risk factors related to postoperative recurrence of dermatofibrosarcoma protuberans: A retrospective study and literature review. World J Clin Cases 2021; 9:5442-5452. [PMID: 34307598 PMCID: PMC8281415 DOI: 10.12998/wjcc.v9.i20.5442] [Citation(s) in RCA: 8] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/19/2021] [Revised: 04/20/2021] [Accepted: 05/19/2021] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Dermatofibrosarcoma protuberans (DFSP) is a rare low-grade malignant soft tissue tumor characterized by rosette-like infiltrative growth. Postoperative recurrence of this tumor is very common.
AIM To evaluate the risk factors related to recurrence after wide local excision (WLE) of DFSP and to guide clinical diagnosis and treatment.
METHODS The medical records of 44 DFSP patients confirmed by pathology at our hospital from 2012 to 2019 were retrospectively reviewed. The relationship between clinical features, tumor characteristics, treatment, and recurrence risk were analyzed, and the possible risk factors for postoperative tumor recurrence were evaluated.
RESULTS There were 44 patients in total, including 21 males and 23 females. The median progression free survival was 36 mo (range, 1-240 mo). Twenty patients were treated for the first time, while 24 had previous treatment experience. Forty-two cases were followed for 25.76 ± 22.0 mo, among whom four (9.52%) experienced recurrence after WLE (rate was 9.52%). The recurrence rate in the recurrent group was higher than that in the patients with primary tumor (19.05% vs 0%, P = 0.028). Eighteen cases had a history of misdiagnosis (rate was 40.91%). The recurrence rate among patients with previous experience of misdiagnosis was significantly higher than in patients without (68% vs 36.84%, P = 0.04). The tumor diameter in patients with a history of treatment was larger than in patients treated for the first time (4.75 ± 0.70 cm vs 2.25 ± 0.36 cm, P = 0.004).
CONCLUSION To sum up, the clinical manifestations of DFSP are not specific and are easily misdiagnosed, thus commonly causing the recurrence of DFSP. After incomplete resection, the tumor may rapidly grow. Previous recurrence history may be a risk factor for postoperative recurrence, and tumor location may have an indirect effect on postoperative recurrence; however, we found no significant correlation between sex, age, course of the disease, or tumor size and postoperative recurrence.
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Affiliation(s)
- Jian-Xia Xiong
- Department of Dermatology, The First Affiliated Hospital of Chongqing Medical University, Chongqing 400010, China
| | - Tao Cai
- Department of Dermatology, The First Affiliated Hospital of Chongqing Medical University, Chongqing 400010, China
| | - Li Hu
- Department of Dermatology, The First Affiliated Hospital of Chongqing Medical University, Chongqing 400010, China
| | - Xiao-Li Chen
- Department of Dermatology, The First Affiliated Hospital of Chongqing Medical University, Chongqing 400010, China
| | - Kun Huang
- Department of Dermatology, The First Affiliated Hospital of Chongqing Medical University, Chongqing 400010, China
| | - Ai-Jun Chen
- Department of Dermatology, The First Affiliated Hospital of Chongqing Medical University, Chongqing 400010, China
| | - Ping Wang
- Department of Dermatology, The First Affiliated Hospital of Chongqing Medical University, Chongqing 400010, China
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17
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Loukil I, Zouari A, Abid B. [Dermatofibrosarcoma protuberans (Darier-Ferrand) of the abdomen: a case report]. Pan Afr Med J 2021; 38:365. [PMID: 34367444 PMCID: PMC8308937 DOI: 10.11604/pamj.2021.38.365.26770] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/02/2020] [Accepted: 03/21/2021] [Indexed: 11/11/2022] Open
Abstract
Dermatofibrosarcoma is a rare skin tumor. Morphological characteristics can be misleading and unknown to most of physicians. Diagnostic delay may affect patient´s management and prognosis. We here report the case of a young patient with lesions protruding from the abdominal wall mistaken for benign cysts. X-ray examination revealed subcutaneous adipose tissue mass. This was suspected of being a fibrosarcoma. The mass was resected with a macroscopic safety margin. The anatomo-pathological study confirmed the diagnosis of dermatofibrosarcoma. Clinical and radiological follow-up examinations didn´t show any recurrence. This study highlights the importance of suspecting this rare tumor requiring specialist treatment.
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Affiliation(s)
- Issam Loukil
- Service de Chirurgie Générale, Tataouine, Tunisie
| | | | - Bassem Abid
- Service de Chirurgie Générale, Sfax, Tunisie
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18
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Li Y, Liang J, Xu X, Jiang X, Wang C, Chen S, Xiang B, Ji Y. Clinicopathological features of fibrosarcomatous dermatofibrosarcoma protuberans and the construction of a back-propagation neural network recognition model. Orphanet J Rare Dis 2021; 16:48. [PMID: 33499900 PMCID: PMC7836157 DOI: 10.1186/s13023-021-01698-4] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/07/2020] [Accepted: 01/19/2021] [Indexed: 02/08/2023] Open
Abstract
BACKGROUND Fibrosarcomatous dermatofibrosarcoma protuberans (FS-DFSP) is a form of tumor progression of dermatofibrosarcoma protuberans (DFSP) with an increased risk of metastasis and recurrence. Few studies have compared the clinicopathological features of FS-DFSP and conventional DFSP (C-DFSP). OBJECTIVES To better understand the epidemiological and clinicopathological characteristics of FS-DFSP. METHODS We conducted a cohort study of 221 patients diagnosed with DFSP and built a recognition model with a back-propagation (BP) neural network for FS-DFSP. RESULTS Twenty-six patients with FS-DFSP and 195 patients with C-DFSP were included. There were no differences between FS-DFSP and C-DFSP regarding age at presentation, age at diagnosis, sex, size at diagnosis, size at presentation, and tumor growth. The negative ratio of CD34 in FS-DFSP (11.5%) was significantly lower than that in C-DFSP (5.1%) (P = 0.005). The average Ki-67 index of FS-DFSP (18.1%) cases was significantly higher than that of C-DFSP (8.1%) cases (P < 0.001). The classification accuracy of the BP neural network model training samples was 100%. The correct rates of classification and misdiagnosis were 84.1% and 15.9%. CONCLUSIONS The clinical manifestations of FS-DFSP and C-DFSP are similar but have large differences in immunohistochemistry. The classification accuracy and feasibility of the BP neural network model are high in FS-DFSP.
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Affiliation(s)
- Yanan Li
- Division of Oncology, Department of Pediatric Surgery, West China Hospital of Sichuan University, #37 Guo-Xue-Xiang, Chengdu, 610041 China
| | - Jiaqi Liang
- Shaanxi Zhongtian Rocket Technology Co, Ltd, Xi’an, 710025 China
| | - Xuewen Xu
- Department of Burns and Plastic Surgery, West China Hospital of Sichuan University, Chengdu, 610041 China
| | - Xian Jiang
- Department of Dermatology, West China Hospital of Sichuan University, Chengdu, 610041 China
| | - Chuan Wang
- Division of Oncology, Department of Pediatric Surgery, West China Hospital of Sichuan University, #37 Guo-Xue-Xiang, Chengdu, 610041 China
| | - Siyuan Chen
- Pediatric Intensive Care Unit, Department of Critical Care Medicine, West China Hospital of Sichuan University, Chengdu, 610041 China
| | - Bo Xiang
- Division of Oncology, Department of Pediatric Surgery, West China Hospital of Sichuan University, #37 Guo-Xue-Xiang, Chengdu, 610041 China
| | - Yi Ji
- Division of Oncology, Department of Pediatric Surgery, West China Hospital of Sichuan University, #37 Guo-Xue-Xiang, Chengdu, 610041 China
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19
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Ogun GO, Ezenkwa US, Ayandipo OO. Dermatofibrosarcoma protuberance in a black African cohort-a clinicopathologic study. Ecancermedicalscience 2020; 14:1086. [PMID: 33014128 PMCID: PMC7498272 DOI: 10.3332/ecancer.2020.1086] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/19/2020] [Indexed: 11/17/2022] Open
Abstract
Background Dermatofibrosarcoma protuberance (DFSP) is the commonest, yet rare, dermal sarcoma globally. There are few reports in the literature of this neoplasm in Nigerians and indeed in sub-Saharan Africa. This study documents our institutional practice observation and compares it with those from other regions of the world. Methods and materials This study was a retrospective review of all cases of histologically diagnosed DFSP at the University College Hospital, Ibadan, Nigeria, spanning a period of 27 years (January 1989–December 2016). Data on patient age, gender, tumour location, size, tumour recurrence and metastasis status were obtained from clinical and surgical pathology archival files and records. Results Sixty-nine cases of DFSP were recorded over the period reviewed with a male–female ratio of 1.6:1. The mean age of the study population was 39.6 years. The youngest patient was 5-year old, while the oldest was 86 years and the modal age group was the 4th decade. The trunk was the commonest anatomic tumour location. Recurrences were seen in seven cases with recurrence interval ranging from 6 to 240 months. The correlation between tumour size and age was non-significant (r = −0.183; p = 0.182). There was fibrosarcoma-like transformation in three cases (4.3%) studied. Conclusion Dermatofibrosarcoma protuberance is rare in our population and occurs more commonly in males and on the trunk. Recurrence can occur beyond the recommended follow-up period of 10 years.
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Affiliation(s)
- Gabriel Olabiyi Ogun
- Department of Pathology, University of Ibadan/University College Hospital, Ibadan, 200001, Nigeria.,https://orcid.org/0000-0001-8535-3240
| | - Uchenna Simon Ezenkwa
- Department of Pathology, University of Ibadan/University College Hospital, Ibadan, 200001, Nigeria
| | - Omobolaji Oladayo Ayandipo
- Department of Surgery (Division of Oncology), University of Ibadan/University College Hospital, Ibadan, 200001, Nigeria
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20
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Hao X, Billings SD, Wu F, Stultz TW, Procop GW, Mirkin G, Vidimos AT. Dermatofibrosarcoma Protuberans: Update on the Diagnosis and Treatment. J Clin Med 2020; 9:E1752. [PMID: 32516921 PMCID: PMC7355835 DOI: 10.3390/jcm9061752] [Citation(s) in RCA: 86] [Impact Index Per Article: 17.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/12/2020] [Revised: 05/31/2020] [Accepted: 06/04/2020] [Indexed: 12/23/2022] Open
Abstract
Dermatofibrosarcoma protuberans (DFSP) is a slow growing, low- to intermediate-grade dermal soft-tissue tumor. It has a high local recurrence rate but low metastatic potential. It is characterized by a uniform spindle cell arrangement, classically with a storiform pattern and CD34 immunoreactivity. The histomorphology and immunophenotype overlap with a broad range of other neoplasms. The standard treatment is complete surgical excision. The surgical procedures include wide local excision (WLE) with tumor free margins, Mohs micrographic surgery (MMS) and amputation. Unresectable DFSPs are treated with radiation therapy and/or targeted therapy. DFSP has characteristic t(17; 22) (q22; q13), resulting in a COL1A1- PDGFB fusion transcripts in more than 90% of DFSPs. Molecular detection of the gene rearrangement or fusion transcripts is helpful for the diagnosis of patients with atypical morphology and for screening candidates for targeted therapy with tyrosine kinase inhibitors. The aims of the present review are to update the clinical presentation, tumorigenesis and histopathology of DFSP and its variants for diagnosis and differential diagnosis from other benign and malignant tumors, to compare the advantages and drawbacks of WLE and MMS, to propose the baseline for selecting surgical procedure based on tumor's location, size, stage and relationship with surrounding soft tissue and bone structures, and to provide a biologic rationale for the systemic therapy. We further propose a modified clinical staging system of DFSP and a surveillance program for the patients after surgical excision.
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Affiliation(s)
- Xingpei Hao
- Foot and Ankle Specialists of the Mid-Atlantic, Rockville, MD 20850, USA
| | - Steven D. Billings
- Department of Pathology, Cleveland Clinic, Cleveland, OH 44195, USA; (S.D.B.); (G.W.P.)
| | - Fangbai Wu
- Department of Radiology, Cleveland Clinic, Cleveland, OH 44195, USA;
| | - Todd W. Stultz
- Imaging Institute, Section of Neuroradiology, Cleveland Clinic, Cleveland, OH 44195, USA;
| | - Gary W. Procop
- Department of Pathology, Cleveland Clinic, Cleveland, OH 44195, USA; (S.D.B.); (G.W.P.)
| | - Gene Mirkin
- Foot and Ankle Specialists of the Mid-Atlantic, Rockville, MD 20850, USA
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Nedu ME, Matei IR, Georgescu AV. Giant keystone type III perforator flaps for dermatofibrosarcoma protuberans defect reconstruction. Injury 2019; 50 Suppl 5:S21-S24. [PMID: 31679835 DOI: 10.1016/j.injury.2019.10.041] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/02/2023]
Abstract
INTRODUCTION Dermatofibrosarcoma protuberans (DFSP) is a relatively rare malignant tumor, with important local aggressiveness. CASE REPORT We present a case of a 59-years-old man with a history of Psoriasis and viral hepatitis C presented in our department with a giant lesion on the posterior trunk, of 24/36 cm, with pus and local bleeding, causing important anemia. The tumor was excised, creating an elliptical defect of around 34 × 42 cm down to fascial level, which was covered by two lateral flaps - modified Keystone type III. RESULTS The flaps were completely viable and integrated with small dehiscence, which were assisted to heal secondarily. CONCLUSION Our paper presents a reliable alternative to skin grafting for a very large defect on the posterior trunk, the modified type III keystone flap, to our knowledge, for the first time presented for such a wide defect and on adult.
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Affiliation(s)
- Maria Eliza Nedu
- Department of Plastic Surgery, University of Medicine and Pharmacy "IuliuHatieganu", Cluj-Napoca, Romania; Department of Plastic Surgery Spitalul Clinic de Recuperare, Rehabilitation Clinical Hospital Cluj-Napoca, Str. Viilor 46-50, Cluj Napoca 400347, Romania
| | - Ileana Rodica Matei
- Department of Plastic Surgery, University of Medicine and Pharmacy "IuliuHatieganu", Cluj-Napoca, Romania; Department of Plastic Surgery Spitalul Clinic de Recuperare, Rehabilitation Clinical Hospital Cluj-Napoca, Str. Viilor 46-50, Cluj Napoca 400347, Romania.
| | - Alexandru Valentin Georgescu
- Department of Plastic Surgery, University of Medicine and Pharmacy "IuliuHatieganu", Cluj-Napoca, Romania; Department of Plastic Surgery Spitalul Clinic de Recuperare, Rehabilitation Clinical Hospital Cluj-Napoca, Str. Viilor 46-50, Cluj Napoca 400347, Romania
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22
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Malan M, Xuejingzi W, Quan SJ. The efficacy of Mohs micrographic surgery over the traditional wide local excision surgery in the cure of dermatofibrosarcoma protuberans. Pan Afr Med J 2019; 33:297. [PMID: 31692830 PMCID: PMC6815477 DOI: 10.11604/pamj.2019.33.297.17692] [Citation(s) in RCA: 21] [Impact Index Per Article: 3.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/15/2018] [Accepted: 07/09/2019] [Indexed: 12/11/2022] Open
Abstract
Usually most patients with dermatofibrosarcoma protuberans (DFSP) may present rather late when the tumor is in protuberant phase due to its rarity and indolent onset. It has a high propensity for local recurrence and destructive nature. Management of DFSP requires a biopsychosocial and Multidisplinary approach regardless of the clinical or immunohistochemical variant. Surgery is the Gold standard management of localized disease. DFSP rarely exhibits any lymphatic or hematogenous dissemination. It is because of its high recurrence rate associated with Wide Local Excision (WLE), the introduction of Mohs micrographic surgery (MMS) has really helped in reducing the rates of recurrence of DFSP. Thus, the aim of this meta-analysis and systemic review is to advocate for MMS over WLE for DFSP and other cutaneous malignancies using DFSP as a prototype. The objective of this study were to conduct a meta-analysis on comparative surgical methods used in the cure of DFSP with regards to WLE verses MMS, to evaluate the cure rates with relation to recurrence rates, offer a recommendation on the various treatment modalities based on the location of lesion, and use of adjuvant therapy in different clinical-medical setups. A comprehensive retrospective analysis search in EMBASE, Google Scholar and Medline (PubMed) for studies published from 2008 to 2018 containing the surgical management of DFSP with WLE verses MMS were reviewed. Five studies of moderate-quality evidence (level B) with a pooled patient load of 684 was analyzed and found for recurrence of DFSP after WLE and MMS to be 9.10% and 2.72% respectively after an average follow-up time for both groups of 5.32 years with a female predominance of 1.58. The trunk is the commonest site for the DFSP lesion which was at 52.80% then the upper and lower extremities zones and the head and neck zones at 31.75% and 15.45% respectively. The pooled adjusted odds ratio (OR) analysis indicated that there was a direct relationship with regards the reduced recurrence rate of DFSP in the MMS group compared to the WLE group (OR:0.31;95%; CI :0.17-0.56). Furthermore, there was significant association between the reduced recurrence rate with the MMS in DFSP patients with a statistical P-value of 0.0001 at 95% CI. The expected increased recurrence rate by zones was in WLE head and neck zone at 38.19% then trunk and extremities zone at 13.34%. In the MMS group it was at of 23.4% as compared to 16.0% in the head and neck zone. Mohs Micrographic Surgery (MMS) is more efficacious in the cure rate and recurrence reduction of DFSP and should be advocated for as first line therapy especially in high recurrence prone zones.
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Affiliation(s)
- Malumani Malan
- Department of Dermatology and Venereology at Zhongnan Hospital of Wuhan University, Wuhan City, Hubei Province, Peoples Republic of China
- Department of Dermatology and Venereology at Zhongnan Hospital of Wuhan University, Wuhan City, Hubei Province, Peoples Republic of China
| | - Wu Xuejingzi
- Department of Dermatology and Venereology at Zhongnan Hospital of Wuhan University, Wuhan City, Hubei Province, Peoples Republic of China
| | - Song Ji Quan
- Head of Department of Dermatology and Venereology at Zhongnan Hospital of Wuhan University, Wuhan City, Hubei Province, People's Republic of China
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Kupilas FJ, Kleinheinz J. [Alternative therapy option for plastic-aesthetic treatment of defects after resection in the upper third of the facial region in the sense of subtotal scalping instead of local flap plastics]. Hautarzt 2018; 70:123-126. [PMID: 30467587 DOI: 10.1007/s00105-018-4308-1] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/30/2022]
Abstract
In the presented case, the resulting defect size after resection of a dermatofibrosarcoma protuberans exceeded the treatment capability with local flaps in the region of the exposed facial skin. Through the use of conventional wound healing in combination with a meshed split-thickness skin graft, plastic aesthetic soft tissue treatment with an aesthetically satisfactory result was possible.
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Affiliation(s)
- F J Kupilas
- Klinik für Mund‑, Kiefer- und Gesichtschirurgie, Universitätsklinikum Münster, Waldeyerstr. 30, 48149, Münster, Deutschland.
| | - J Kleinheinz
- Klinik für Mund‑, Kiefer- und Gesichtschirurgie, Universitätsklinikum Münster, Waldeyerstr. 30, 48149, Münster, Deutschland
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24
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Park S, Cho S, Kim M, Park JU, Jeong EC, Choi E, Park JH, Lee C, Chang MS. Dermatofibrosarcoma protuberans: A retrospective study of clinicopathologic features and related Akt/mTOR, STAT3, ERK, cyclin D1, and PD-L1 expression. J Am Acad Dermatol 2018; 79:843-852. [DOI: 10.1016/j.jaad.2018.05.016] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/26/2018] [Revised: 05/03/2018] [Accepted: 05/08/2018] [Indexed: 11/26/2022]
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Sung TH, Tam AC, Khoo JL. Dermatofibrosarcoma Protuberans: A comprehensive review on the spectrum of clinico-radiological presentations. J Med Imaging Radiat Oncol 2016; 61:9-17. [PMID: 27753236 DOI: 10.1111/1754-9485.12549] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/08/2016] [Accepted: 09/11/2016] [Indexed: 11/27/2022]
Abstract
Dermatofibrosarcoma Protuberans (DFSP) is a rare malignant soft-tissue neoplasm which is often misdiagnosed due to its indolent clinical course and non-specific radiological appearances. An observation case series was conducted with retrospective review of clinical and radiological data of DFSP patients presenting to a major tertiary hospital in Hong Kong for radiological assessment between November 2006 and February 2016. Seven patients with confirmed histological diagnosis of DFSP were included. Tumour sizes at presentation ranged from 1 to 5 cm, most commonly (n = 6) occurred over chest wall and abdominal wall. History of previous local trauma or surgery was identified in the majority of cases (n = 4). There was poor correlation between pre-imaging clinical diagnoses and pathological diagnoses. Local recurrence and tumour de-differentiation with sarcomatous changes occurred in the minority of cases (n = 2). A common radiological 'claw' sign at the lesion/skin interface formed by elongated appendages of the tumour superficially was appreciated in most cases (n = 6). A history of previous local trauma or surgery serves as a possible etiological factor for the development of DFSP. High clinical suspicion for the entity is essential in its detection and differentiation from simple wound complications and local recurrence of other benign lesions. The radiological 'claw' sign at the lesion/skin interface might serve as a tell-tale sign for cutaneous tumour involvement. A comprehensive analysis of imaging findings in conjunction with individual clinical presentations is the key to accurate diagnoses and proper management.
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Affiliation(s)
- Tony Ht Sung
- Department of Radiology, Pamela Youde Nethersole Eastern Hospital, Chai Wan, Hong Kong
| | - Annie Cw Tam
- Department of Radiology, Pamela Youde Nethersole Eastern Hospital, Chai Wan, Hong Kong
| | - Jennifer Ls Khoo
- Department of Radiology, Pamela Youde Nethersole Eastern Hospital, Chai Wan, Hong Kong
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