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Pan Afr Med J 2022 22;41:234. Epub 2022 Mar 22.

Service de Radiothérapie, CHU Mohammed VI, Oujda, Maroc.

Dermatofibrosarcoma is a rare cancer, accounting for 0.01% of all cancers. We here report the case of a 44-year-old female patient presenting with the 5 recurrence of locally advanced Darier-Ferrand dermatofibrosarcoma, that progressed on many cycles of neoadjuvant therapy and required emergency radiotherapy with good response. Read More

Surg Oncol Clin N Am 2022 Jul 31;31(3):511-525. Epub 2022 May 31.

Sarcoma Service - Department of Surgery, Fondazione IRCCS Istituto Nazionale dei Tumori, Via Venezian, 1, Milan 20133, Italy. Electronic address:

Skin sarcomas are tumors that are superficial and small in size in comparison with other sarcomas arising in intramuscular or intrabdominal sites. Skin sarcomas are often underrecognized and misdiagnosed. A high level of suspicion is needed, as early recognition and appropriate management including initial surgery is important for oncologic outcomes. Read More

Zhonghua Bing Li Xue Za Zhi 2022 Jun;51(6):530-535

Department of Pathology, Henan Provincial People's Hospital, People's Hospital of Zhengzhou University, People's Hospital of Henan University, Zhengzhou 450003, China.

To investigate the clinicopathological features, immunophenotypic and molecular genetic characteristics and differential diagnosis of fibrous hamartoma of infancy (FHI). Thirty-three cases of surgically removed FHI were collected from the Department of Pathology, Henan Provincial People's Hospital from October 2011 to December 2020, the clinical and pathologic data with follow-up were collected and analyzed. Next-generation sequencing (NGS) and quantitative real time polymerase chain reaction (q-PCR) were used to study the molecular genetics. Read More

J Cancer Res Ther 2022 Apr;18(2):476-481

Department of Radiology, Qilu Hospital of Shandong University (Qingdao), Qingdao, China.

Aims: The study highlights diffusion-weighted imaging (DWI) and dynamic enhancement features of DFSP and characterizes unenhanced and enhanced computed tomography (CT) and magnetic resonance imaging (MRI) scans.

Settings And Design: Image findings and clinical histories of 23 patients with DFSP were reviewed. Nine patients underwent CT before and after intravenous administration of contrast material. Read More

Dis Mon 2022 May 26:101427. Epub 2022 May 26.

University of South Alabama University Hospital Emergency Department, Mobile, AL, United States.

Skin Res Technol 2022 May 31. Epub 2022 May 31.

Department of Radiology, The First Affiliated Hospital of Soochow University, Suzhou, China.

Background: Dermatofibrosarcoma protuberans (DFSP) is a kind of low-grade malignant spindle cell neoplasm, the diagnosis, and treatment, which have markedly attracted clinicians' attention for its repeated recurrence. High-resolution magnetic resonance imaging (HR-MRI) has shown unique capabilities in diagnosis of various cutaneous tumors.

Materials And Methods: Data of 29 patients with clinically suspected DFSPs and undergoing dynamic contrast-enhanced (DCE) HR-MRI preoperatively were prospectively collected. Read More

Medicina (B Aires) 2022 ;82(3):441-444

División Ciencias de la Salud, Universidad del Norte, Barranquilla, Colombia.

Gynecological sarcomas are rare and their location in the vulva and vagina has an incidence of 5% of all malignant neoplasms in the female genital tract. We present the case of a 54-year-old patient with a diagnosis of dermatofibrosarcoma protuberans in the vulva, an infrequent pathology with less than 60 cases reported worldwide in this anatomical location. Clinically, it is locally aggressive, due to the proliferation of spindle cells with pleomorphism and frequent mitotic figures infiltrating the reticular dermis and subcutaneous cellular tissue, giving rise to variable size tumors with high local recurrence rates. Read More

J Clin Lab Anal 2022 May 27:e24500. Epub 2022 May 27.

Department of Radiology, The Second Clinical Medicine Faculty, Inner Mongolia University for Nationalities, Yakeshi, China.

Background: Dermatofibrosarcoma protuberans is extremely rare in children, making a correct diagnosis by clinicians is usually difficult due to its nonspecific manifestations, the recurrence of dermatofibrosarcoma protuberans after resection has always been a perplexing problem for clinicians. Ultrasound plays an irreplaceable role in the assessment of dermatofibrosarcoma protuberans, although there is a limitation in the diagnosis of the tumor.

Case Report: A 10-year-old boy led by his parents sought for surgical treatment because of the growing mass. Read More

Clin Nucl Med 2022 May 19. Epub 2022 May 19.

Breast Surgery, The Affiliated Hospital of Southwest Medical University, Luzhou, Sichuan, People's Republic of China.

Abstract: A 47-year-old woman presented with a 6-month history of palpable masses in the right hip. The patient was subsequently enrolled in our clinical trial of 68Ga-FAPI PET/CT study on tumors. Intense 68Ga-FAPI activities were noted in the right hip lesions, suggestive of malignancies. Read More

J Ayub Med Coll Abbottabad 2022 Apr-Jun;34(2):364-365

Nishtar Medical University and Hospital, Multan.

Dermatofibrosarcoma protuberans is a rare cutaneous malignancy. Usual sites of origin are trunk and extremities. Aetiology of the condition is not well understood but a genetic basis is explained as a reciprocal translocation t (17l;22) (q22; q13). Read More

Cureus 2022 Apr 14;14(4):e24147. Epub 2022 Apr 14.

Department of Dermatology, University of Arkansas for Medical Sciences, Little Rock, USA.

Background: Dermatofibrosarcoma protuberans (DFSP) is an uncommon, locally aggressive malignancy with wide local excision (WLE) or Mohs micrographic surgery (MMS) representing the treatment of choice. This article illustrates the experience of a single academic institution in treating DFSP with MMS and adds two particularly large, difficult closures of the glabella/central forehead and sternum to the body of literature.

Objective: To report the results of 15 patients with DFSP treated with MMS over a five-year period by a single Mohs surgeon at the University of Arkansas for Medical Sciences (UAMS). Read More

Ann Diagn Pathol 2022 Aug 6;59:151967. Epub 2022 May 6.

Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN 59005, United States of America. Electronic address:

Introduction: Loss-of-function mutations in EED and SUZ12, core components of the polycomb repressive complex 2 (PRC2), occur in >90% of sporadic and radiation-associated malignant peripheral nerve sheath tumors (MPNST) and in roughly 70% of NF1-related tumors. PRC2 inactivation results in loss of H3K27me3 expression and aberrant downstream transcription. H3K27me3 expression is lost in 40-90% of spindle cell MPNST but is not specific. Read More

Int J Gynecol Pathol 2022 Apr 12. Epub 2022 Apr 12.

Department of Pathology, 12 of October University Hospital, Madrid, Spain.

COL1A1-PDGFB gene fusion uterine sarcoma is a recently described entity which shows some overlapping features with dermatofibrosarcoma protuberans. To date, only 4 cases have been reported in the literature. Due to its rarity, succinct clinicopathologic characteristics are yet to be established. Read More

Case Rep Radiol 2022 30;2022:2953579. Epub 2022 Apr 30.

Department of Radiology and Medical Imaging, University Hospital Brussels, Laarbeeklaan 101, B-1090 Brussels, Belgium.

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. Read More

J Drugs Dermatol 2022 May;21(5):534-535

Dermatofibrosarcoma protuberans (DFSP) is a rare, fibrohistiocytic tumor with intermediate malignancy.1 While these tumors are slow-growing and only metastasize in 6% of cases,2 they are often locally destructive, with relatively high local recurrence rates after initial excision. Overall annual incidence rates in the US are 0. Read More

Appl Immunohistochem Mol Morphol 2022 May 3. Epub 2022 May 3.

Departments of Dermatology.

Immunohistochemical staining can be of great utility in differentiating various cutaneous spindle cell neoplasms, particularly when the histomorphologic appearance of the lesions is inconclusive. Nuclear staining for ETS-related gene (ERG), a highly sensitive endothelial cell marker, has seldom been studied in the context of cutaneous spindle cell neoplasms. Little is known about its specificity for vascular differentiation. Read More

Dermatol Online J 2021 Dec 15;27(12). Epub 2021 Dec 15.

Center for Clinical Studies, Webster, Texas, USA.

We present an 81-year-old man who presented for evaluation of an indurated plaque with an exophytic, pearly, skin-red-brown colored nodule with central ulceration on his chest that evolved over the course of several months and was initially suspected to be basal cell carcinoma. Biopsy demonstrated histological features of dermal spindle cell proliferation in a storiform fashion with CD34 positivity confirming a diagnosis of dermatofibrosarcoma protuberans (DFSP). Dermatofibrosarcoma protuberans are rare, slowly progressive soft tissue sarcomas. Read More

Clin Case Rep 2022 Apr 26;10(4):e05703. Epub 2022 Apr 26.

Department of Plastic and Reconstructive Surgery Galway University Hospital Galway Ireland.

Dermatofibrosarcoma protuberans is a rare entity. Due to its high propensity for local recurrence, knowledge of the appropriate management, both surgical and medical, is important for optimal patient outcomes. Read More

Autops Case Rep 2022 1;12:e2021368. Epub 2022 Apr 1.

CUHK Medical Centre, Department of Pathology, Hong Kong, China.

Dermatofibrosarcoma protuberans (DFSP) is a relatively rare, locally aggressive, and dermal-based fibroblastic tumor. There are several histological variants, in which the usual emphasis is on fibrosarcomatous DFSP, as it acquires metastatic potential. Myoid differentiation in DFSP is rare, and more often found in fibrosarcomatous DFSP. Read More

Clin Nucl Med 2022 Jul 22;47(7):629-631. Epub 2022 Apr 22.

From the Department of Nuclear Medicine, The Affiliated Hospital of Southwest Medical University; Nuclear Medicine and Molecular Imaging Key Laboratory of Sichuan Province; and Academician (Expert) Workstation of Sichuan Province, Luzhou, Sichuan, China.

Abstract: Dermatofibrosarcoma protuberans is a rare soft tissue sarcoma with a high recurrence rate. Herein, we present 68Ga-FAPI and 18F-FDG PET/CT findings of dermatofibrosarcoma protuberans in a 45-year-old man. Dermatofibrosarcoma protuberans only shows limited FDG uptakes on 18F-FDG PET/CT, but demonstrated intense tracer uptakes on 68Ga-FAPI PET/CT. Read More

Br J Dermatol 2022 Jun 19;186(6):997-1009. Epub 2022 Apr 19.

Department of Dermatology, Xiangya Hospital, Central South University, Changsha, Hunan, China.

Background: Dermatofibrosarcoma protuberans (DFSP) is a rare and marginal cutaneous sarcoma of intermediate-grade malignancy, for which the genomic landscape remains unclear. Understanding the landscape of DFSP will help to further classify the genomic pathway of malignant development in soft tissue.

Objectives: To identify the comprehensive molecular pathogenesis of DFSP. Read More

Comput Intell Neurosci 2022 7;2022:5509129. Epub 2022 Apr 7.

Department of Plastic and Reconstructive Surgery, Shanghai Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, Center for Specialty Strategy Research of Shanghai Jiao Tong University China Hospital Development Institute, Shanghai, China.

Dermatofibrosarcoma protuberans (DFSP) is a type of skin cancer that is extremely rare. Its standard treatment is either surgical wide-local excision (WLE) or Mohs micrographic surgery (MMS). Which method has the lowest recurrence rate is unknown. Read More

Cancers (Basel) 2022 Mar 26;14(7). Epub 2022 Mar 26.

Department of Plastic Surgery and Breast Surgery, Zealand University Hospital (SUH) Roskilde, University of Copenhagen, 4000 Roskilde, Denmark.

Primary dermal sarcomas (PDS) belong to a highly clinically, genetically and pathologically heterogeneous group of rare malignant mesenchymal tumours primarily involving the dermis or the subcutaneous tissue. The tumours are classified according to the mesenchymal tissue from which they originate: dermal connective tissue, smooth muscle or vessels. Clinically, PDS may mimic benign soft tissue lesions such as dermatofibromas, hypertrophic scarring, etc. Read More

Transl Cancer Res 2022 Mar;11(3):488-499

Department of Pathology, Second Hospital of Shanxi Medical University, Taiyuan, China.

Background: The accurate diagnosis of sarcoma can be difficult as there are over 70 different subtypes. While molecular profiling in soft tissue sarcoma (STS) has gradually been incorporated into routine diagnostics, conventional methods such as fluorescence in situ hybridization (FISH), reverse transcriptase-PCR (RT-PCR), and Sanger sequencing have several drawbacks. By allowing simultaneous analysis of multiple targets and increasing sequencing depth to achieve ultra-sensitivity, next-generation sequencing (NGS) can not only detect common genetic abnormalities without prior assumptions but also identify uncommon or even new variants. Read More

Rep Pract Oncol Radiother 2022 22;27(1):182-187. Epub 2022 Mar 22.

Division of Radiation Oncology, Department of Radiology, Philippine General Hospital, Manila, Philippines.

Dermatofibrosarcoma protuberans (DFSP) is a rare spindle cell tumor, comprising less than 0.1% of all malignant neoplasms. The trunk is the most commonly affected area, followed by the extremities and the head and neck. Read More

Curr Treat Options Oncol 2022 Jun 8;23(6):843-854. Epub 2022 Apr 8.

Department of Plastic and Reconstructive Surgery, Southmead Hospital, Southmead Road, Bristol, BS10 5NB, UK.

Opinion Statement: Paediatric dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue malignant tumour which displays aggressive local behaviour and has low metastatic potential. The diagnosis is often delayed as DFSP is usually mistaken for other skin conditions, particularly in the early stages of disease. DFSP tends to follow an indolent course after the initial presentation with what is often described as a "rubbery lump". Read More

JAAD Case Rep 2022 May 10;23:8-11. Epub 2022 Mar 10.

Department of Ophthalmology and Visual Sciences, University of Maryland School of Medicine, Baltimore, Maryland.

Dermatol Surg 2022 Jun 6;48(6):606-612. Epub 2022 Apr 6.

Department of Dermatology, University of Nebraska Medical Center, Omaha, Nebraska.

Background: There is a scarcity of information regarding the clinical characteristics of rare cutaneous malignancies in skin of color that has yet to be comprehensively explored.

Objective: To review and compile the racial differences in epidemiology, clinical presentation, histology, treatments, and outcomes of 3 rare skin cancers: dermatofibrosarcoma protuberans (DFSP), Merkel cell carcinoma (MCC), and sebaceous carcinoma (SC).

Methods: Several searches with keywords denoting specific skin cancer type and race were conducted on PubMed to complete this narrative review. Read More

Dermatol Surg 2022 May 30;48(5):479-485. Epub 2022 Mar 30.

Department of Dermatology, Division of Dermatologic Surgery, Mayo Clinic, Rochester, Minnesota.

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). Read More