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Molecules 2019 Apr 17;24(8). Epub 2019 Apr 17.

Medical Academy named after S.I. Georgievsky, V.I. Vernadsky Crimean Federal University, Lenin Boulevard 5/7, 295051 Simferopol, Crimea.

Skin cancer has always been and remains the leader among all tumors in terms of occurrence. One of the main factors responsible for skin cancer, natural and artificial UV radiation, causes the mutations that transform healthy cells into cancer cells. These mutations inactivate apoptosis, an event required to avoid the malignant transformation of healthy cells. Read More

Iran J Otorhinolaryngol 2019 Mar;31(103):97-102

Research Committee, Dental School of Shahid Beheshti University of Medical Sciences, Tehran, Iran.

Introduction: Sarcomas are rare malignancies with aggressive biological behavior. They are categorized into soft and hard tissue types. The main objective of this study was to analyze the prevalence of head and neck sarcomas (HNS) among the Iranian population. Read More

Zhongguo Xiu Fu Chong Jian Wai Ke Za Zhi 2019 Apr;33(4):471-474

Department of Plastic Surgery, Changhai Hospital, Naval Medical University, Shanghai, 200433,

Objective: To explore the effectiveness of keystone flap in repairing skin and soft tissue defects around joint.

Methods: Between March 2013 and December 2017, 10 patients of skin and soft tissue defects around the joint were repaired with keystone flaps. There were 6 males and 4 females. Read More

J Fr Ophtalmol 2019 Apr 10. Epub 2019 Apr 10.

Department of ophthalmology, Feiz hospital, Isfahan university of medical sciences, Isfahan, Iran; Isfahan eye research center (IERC), Feiz hospital, Isfahan university of medical sciences, Isfahan, Iran; Isfahan medical students research center (IMSRC), Isfahan university of medical sciences, Isfahan, Iran. Electronic address:

Objective: To provide a systematic review on ophthalmologic presentations of dermatofibrosarcoma protuberans (DFSP) in conjunction with a case report of primary orbital involvement by this tumor.

Methods: A thorough electronic search on PubMed, Medline, Scopus, EMBASE and web of science databases was performed. All available data from reported cases along with our described case were extracted and analyzed. Read More

Int J Dermatol 2019 Apr 10. Epub 2019 Apr 10.

Bone and Soft Tissue Pathology Department, University of Pittsburgh Medical Center Shadyside Hospital, Pittsburgh, PA, USA.

Dermatofibrosarcoma protuberans with fibrosarcomatous transformation (DFSP-FS) is a higher grade tumor arising from dermatofibrosarcoma protuberans (DFSP). Recent literature highlights its impact on recurrence rates, metastatic rates, and survival. In this article, we aim to describe our experience with 13 cases of DFSP-FS in terms of pathologic findings, molecular alterations, clinical outcomes, management, and also perform a short recent literature review. Read More

Anticancer Res 2019 Apr;39(4):2105-2111

Laboratory of Pathology, Foot and Ankle Specialists of the Mid-Atlantic, LLC., Rockville, MD, U.S.A.

Background: Dermatofibrosarcoma protuberans (DFSP) is an uncommon, low-grade, dermal soft-tissue neoplasm with high recurrence, but low metastatic potential. It mainly occurs on the trunk, proximal extremity, head and neck, but rarely on the toes. Herein we report a case of DFSP on the right hallux. Read More

J Cutan Pathol 2019 Apr 4. Epub 2019 Apr 4.

Department of Pathology and Laboratory Medicine, Dartmouth Hitchcock Medical Center and Geisel School of Medicine at Dartmouth, One Medical Center Drive, Lebanon, New Hampshire.

Dermatofibrosarcoma protuberans (DFSP) is a low-grade sarcoma with fibroblastic differentiation characterized, in the great majority of cases, by fusions between the COL1A1 and PDGFB genes. In some instances, transformation to a genetically more complex sarcoma, most commonly fibrosarcoma, is observed. However, the molecular pathogenesis of this transformation is not well understood. Read More

Diagn Cytopathol 2019 Mar 27. Epub 2019 Mar 27.

Department of Pathology, The University of Chicago, Chicago, Illinois.

Dermatofibrosarcoma protuberans (DFSP) is a low-grade spindle cell tumor of the skin commonly arising on the trunk and extremities which tends to be slow growing yet locally aggressive. DFSPs are associated with a good prognosis when surgical excision with negative margins is achieved. Although local recurrences occur up to 50% of incompletely resected cases, distant metastases are very rare. Read More

Pan Afr Med J 2018 12;31:25. Epub 2018 Sep 12.

Department of Oral and Maxillofacial Surgery, College of Medicine University of Lagos, Nigeria.

Introduction: Dermatofibrosarcoma protuberance (DFSP) is in general a rare low grade malignant sarcoma and possesses a tendency for local recurrence. It has a site predilection for the trunk. Occurrence in the facial area is extremely rare. Read More

Cureus 2019 Jan 8;11(1):e3857. Epub 2019 Jan 8.

Radiation Oncology, National Radiotherapy Oncology and Nuclear Medicine Centre, Korle-Bu Teaching Hospital, Accra, GHA.

Local tumor control and symptom relief have been the major advantage of radiotherapy in clinical practice. In the past years, the systemic anti-tumor effect of radiotherapy, also known as the abscopal effect, has been reported with limited studies. With the advent of immunotherapy, the frequency of the abscopal effect has increased in patients who receive sequential treatment with radiotherapy and immunotherapy or patients who receive radiotherapy after acquiring resistance to immunotherapy. Read More

Br J Dermatol 2019 Mar 18. Epub 2019 Mar 18.

Biology of Infection Unit, Laboratory of Pathogen Discovery, Inserm U1117, Paris.

Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous neoplasm, which rarely metastasizes, although local relapses are common. No environmental factors have been clearly identified. The pathomechanisms of DFSP involves the activation of the platelet-derived growth factor receptor (PDGF-R) by a fusion transcript resulting from genomic instability, mainly a chromosomal translocation t(17;22), thus leading to a proliferation of CD34+, vimentin+, FXIIIa-, fibroblastic cells. Read More

Mod Pathol 2019 Mar 16. Epub 2019 Mar 16.

Department of Pathology, Belfast Health and Social Care Trust, Belfast, UK.

Mesenchymal neoplasms of the uterus (corpus and cervix) encompass a heterogeneous group of tumors with differing morphologies, immunophenotypes and molecular alterations. With the advent of modern molecular techniques, such as next generation sequencing, newly defined genetic abnormalities are being reported in this group of neoplasms. Herein we report the clinicopathological and molecular features of a series of 13 spindle cell sarcomas of the uterus and vagina (10 cervix, 2 uterine corpus, 1 vagina) with morphology resembling fibrosarcoma. Read More

Curr Treat Options Oncol 2019 Mar 14;20(4):29. Epub 2019 Mar 14.

Department of Anatomic Pathology, Graduate School of Medicine Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka, 812-8582, Japan.

Opinion Statement: Cutaneous sarcoma is a group of malignant mesenchymal tumors primarily involving the dermis, and it is characterized by extreme clinicopathological heterogeneity. Although its occurrence rate is rare, dermatofibrosarcoma protuberans (DFSP) is one of the most common types of dermal sarcoma. DFSP grows slowly and tends to relapse locally after inadequate resection. Read More

Skin Res Technol 2019 Mar 12. Epub 2019 Mar 12.

Department of Dermatology, University Hospital of Saint-Etienne, Saint-Etienne, France.

Pediatr Dermatol 2019 Mar 10. Epub 2019 Mar 10.

Cooper Medical School of Rowan University, Camden, NJ.

Ataxia telangiectasia (AT) is a rare autosomal recessive neurodegenerative disorder caused by a mutation in the ATM gene. An impaired immune response due to the gene mutation leads to an increased risk of infection and malignancy. We present a rare case of dermatofibrosarcoma protuberans arising in a patient with AT. Read More

Med J Malaysia 2019 Feb;74(1):82-84

Sarawak General Hospital, Department of Plastic and Reconstructive Surgery, Sarawak, Malaysia.

Dermatofibrosarcoma protuberans (DFSP) is a slowgrowing, locally invasive tumour of the dermis. It commonly presents in the trunks and proximal extremities but is seen to a lesser extent in the head and neck regions. We present a case report of a recurrent DFSP in a 48-year-old Iban woman at the occipital region. Read More

Clin Exp Dermatol 2019 Feb 28. Epub 2019 Feb 28.

Department of Dermatology, Nagoya University Graduate School of Medicine, Nagoya, Japan.

Int J Surg Pathol 2019 Feb 27:1066896919832658. Epub 2019 Feb 27.

1 Henry Ford Health System, Detroit, MI, USA.

Dermatofibrosarcoma protuberans (DFSP) is categorized as a fibrohistiocytic tumor of intermediate malignant potential. It has significant risk for local recurrence and, less commonly, local or distant metastasis. Initially, these tumors typically arise as a firm plaque on the skin that slowly progresses to a nodular and protuberant dermal lesion. Read More

Pediatr Dermatol 2019 Feb 27. Epub 2019 Feb 27.

Department of Plastic and Reconstructive Surgery, Teikyo University Chiba Medical Center, Ichihara-shi, Chiba, Japan.

Both medallion-like dermal dendrocyte hamartoma and fibroblastic connective tissue nevus are rare benign dermal lesions composed of CD34-positive spindle cells. Although regarded as different diseases, it is sometimes difficult to distinguish between them due to their clinical and pathological similarities. We present a case of medallion-like dermal dendrocyte hamartoma that could also be diagnosed as fibroblastic connective tissue nevus and propose the possibility of overlap in these diseases. Read More

Autops Case Rep 2018 Oct-Dec;8(4):e2018039. Epub 2018 Sep 26.

Mercer University School of Medicine. Columbus, GA, United States of America.

Dermatofibrosarcoma protuberans (DFSP) is an uncommon soft tissue neoplasm of low metastatic potential notable for its progressive growth and high rate of local recurrence after surgical excision. Fibrosarcomatous transformation of DFSP (FS-DFSP) is a rare variant characterized by higher rates of local recurrence and metastasis. Trauma has been hypothesized as a potential risk factor for the development of DFSP, although clear evidence has been lacking. Read More

J Cutan Pathol 2019 Feb 9. Epub 2019 Feb 9.

Dermatology, Department of Experimental, Diagnostic and Specialty Medicine, University of Bologna, Bologna, Italy.

Plaque-like myofibroblastic tumor is a rare and benign pediatric soft tissue tumor. It presents as a slowly growing plaque reaching several centimeters in diameter, made up of multiple nodules. The clinical and histological features of this benign entity are similar to other fibrohistiocytic or myofibroblastic tumors occurring in childhood, so the diagnosis can be difficult. Read More

Gynecol Oncol Rep 2019 May 25;28:9-11. Epub 2019 Jan 25.

Ohio State University James Cancer Hospital, Division of Gynecologic Oncology, United States.

•Biopsies of a large mass are prone to sampling errors and may lead to an incorrect diagnosis.•MRI imaging of vulvar tumors can aid in surgical planning.•Large sarcomas of the vulva require a multi-disciplinary approach. Read More

Ophthalmic Plast Reconstr Surg 2019 Mar/Apr;35(2):e36-e39

Departments of Ophthalmology and Visual Sciences.

Dermatofibroma sarcoma protuberans (DFSP) is a rare, locally aggressive soft tissue sarcoma with a tendency for recurrence after excision. Although reports of unilateral orbital and bilateral eyelid disease exist, there have been no prior reports of DFSP with bilateral orbital involvement and no previously described cases of DFSP associated with transient optic neuropathy. The authors present a case report of a 34-year-old woman with a giant scalp DFSP involving the bilateral orbits. Read More

Indian J Dermatol Venereol Leprol 2019 Mar-Apr;85(2):204-208

Department of Dermatology, Seth GS Medical College and KEM Hospital, Mumbai, Maharashtra, India.

J Dermatol Sci 2019 Feb 16;93(2):139-141. Epub 2019 Jan 16.

Department of Dermatology, Gunma University Graduate School of Medicine, 3-39-22 Showa, Maebashi, Gunma, 371-8511, Japan.

Int J Surg Case Rep 2019 22;55:58-61. Epub 2019 Jan 22.

Head and Neck Oncology Center, Kazakh Institute of Oncology and Radiology, 91 Abay Ave, Almaty, Kazakhstan.

Introduction: Dermatofibrosarcoma protuberans (DFSP) is a cutaneous malignancy that arises from the dermis and invades deeper tissue. The cellular origin of DFSP is not clear. Evidence supports the cellular origin being fibroblastic, histiocytic, or neuroectodermal. Read More

Radiat Oncol 2019 Jan 29;14(1):20. Epub 2019 Jan 29.

Department of Radiation Oncology, Fujian Medical University Cancer Hospital, Fujian Cancer Hospital, Fuzhou, 350014, China.

Objective: This study aimed to evaluate the role of postoperative radiotherapy (RT) in dermatofibrosarcoma protuberans (DFSP) and identify the prognostic factors influencing the disease-free survival (DFS).

Methods: A total of 184 patients with DFSP were analyzed from 2000 to 2016. The regression model was used to examine the prognostic factors for DFS. Read More

Appl Immunohistochem Mol Morphol 2019 Jan 19. Epub 2019 Jan 19.

Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, Indianapolis, IN.

STAT6 stain has proved to be a good surrogate marker for the genetic alteration (NAB2-STAT6 gene fusion) in solitary fibrous tumor (SFT). This study aims to validate the use of STAT6 rabbit monoclonal antibody in differentiating SFT from its histologic mimics. Forty-five cases of SFT and 110 cases from 9 other spindle cell tumors were collected for STAT6 immunostaining. Read More

Cancer 2019 Mar 15;125(5):670-672. Epub 2019 Jan 15.

Sarcoma Site Group, Princess Margaret Cancer Centre and Department of Surgery, University of Toronto, Toronto, Ontario, Canada.

Cancer 2019 Mar 15;125(5):735-741. Epub 2019 Jan 15.

Sarcoma Unit, Department of Surgery, Royal Marsden Hospital, London, United Kingdom.

Background: Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue sarcoma for which clinical examination up to 10 years is recommended. The objective of this study was to identify prognostic factors for recurrences and metastases that can be used to evaluate the validity of follow-up schedules after treatment for DFSP.

Methods: Patients with DFSP who received treatment between 1991 and 2016 at 3 tertiary centers were included. Read More

Dermatol Surg 2019 Jan 11. Epub 2019 Jan 11.

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

J Cutan Pathol 2019 Apr 10;46(4):297-300. Epub 2019 Feb 10.

Department of Dermatology, Philadelphia College of Osteopathic Medicine, Roswell, Georgia.

Medallion-like dermal dendrocyte hamartoma is a rare congenital lesion that is more commonly seen in females. It often presents at birth on the neck or upper trunk as a well-circumscribed, atrophic patch with wrinkling of the overlying skin. Clinically, the differential diagnosis includes atrophoderma, anetoderma, and congenital atrophic dermatofibrosarcoma protuberans. Read More

J Pak Med Assoc 2019 Jan;69(1):113-115

Aga Khan University Hospital, Karachi.

In 1924, Darier and Ferrand described Dermatofibrosarcoma Protuberans as a progressive and recurring dermatofibroma. It is a locally aggressive sarcoma originating from dermal and subdermal tissue of the skin. It usually begins as a small plaque that grows over a period and later manifests as multiple small subcutaneous nodules. Read More

F1000Res 2018 20;7. Epub 2018 Dec 20.

Pathology and Laboratory Medicine, Children's Hospital Los Angeles, Los Angeles, CA, USA.

Fibroblastic and myofibroblastic tumors comprise a morphologically diverse and biologically variable group of neoplasms that affect a wide age range. Specific entities tend to occur most frequently in infants and young children. Recent years have witnessed a proliferation of information concerning the unique biology of these tumors. Read More

JAMA Dermatol 2019 Jan 2. Epub 2019 Jan 2.

Dermatology Service, Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, New York.

Importance: Dermatofibrosarcoma protuberans (DFSP) has the potential for local destruction and recurrence, although it carries a low risk of metastasis. Complete surgical resection with negative margins is considered the gold standard for treatment; however, there are cases that are unresectable owing to tumor extension or size or owing to risk of cosmetic and/or functional impairment. Imatinib treatment has been used for locally advanced or metastatic DFSP. Read More

Rev Esp Patol 2019 Jan - Mar;52(1):62-68. Epub 2018 Apr 5.

UGC de Cirugía General y Aparato Digestivo, Complejo Hospitalario Torrecárdenas, Almería, España.

Dermatofibrosarcoma protuberans is a low-grade sarcoma typically originating in the dermis but with local invasion of subcutaneous cell and muscle tissue. We report a case of perianal dermatofibrosarcoma protuberans in a 41-year-old male complaining of anal pain and constipation. To date, only two cases of perianal dermatofibrosarcoma protuberans have been reported. Read More

Pan Afr Med J 2018 16;30:213. Epub 2018 Jul 16.

General Practitioner Office Henri IV 45, Boulevard Henri IV, Ambert France.

Medicine (Baltimore) 2018 Dec;97(51):e13851

Department of Orthopedic Surgery, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea.

Rationale: Large soft tissue defects on the lower back represent a treatment challenge. Among a variety of reconstructive procedures, the latissimus dorsi (LD) muscle or musculocutaneous flap is one of most frequently used pedicled flaps. However, the pedicled LD flap carries a bulky pivot point and a short arc of rotation. Read More

Rev Chil Pediatr 2018 Oct;89(5):655-659

Servicio de Anatomía Patológica, Clínica Santa María, Santiago, Chile.

Introduction: Fibrous hamartoma of infancy (FHI) is a benign, soft tissue tumor that usually oc curs in children and has a characteristic histological morphology.

Objective: To describe a case of congenital FHI with atypical histological and clinical characteristics.

Clinical Case: Full-term male newborn, with no perinatal morbid history was referred to dermatology due to a congenital erythe matous plaque in the umbilical region. Read More

JAAD Case Rep 2019 Jan 6;5(1):60-62. Epub 2018 Dec 6.

Department of Dermatology, University of California, Irvine, California.

Int J Pediatr Otorhinolaryngol 2019 Jan 2;116:92-96. Epub 2018 Oct 2.

University of Central Florida College of Medicine, Orlando, FL, USA; Division of Otolaryngology-Head Neck Surgery/Audiology, Nemours Children's Hospital, Orlando, FL, USA. Electronic address:

Objective: To examine pediatric head and neck fibrosarcoma cases and review the demographics, management, and survival for these patients.

Methods: Pediatric patients in the Surveillance, Epidemiology, and End Results (SEER) database were included from 1973 to 2014 based on a diagnosis of a head and neck fibrosarcoma using ICD-O-3 head and neck primary sites and histology codes. Patients were included from birth-18 years of age. Read More

Actas Dermosifiliogr 2018 Dec 4;109(10):868-877. Epub 2018 Jul 4.

Servicio de Dermatología, Instituto Valenciano de Oncología, Valencia, España.

Sarcomas comprise a broad group of tumors, many of whose biological behavior and aggressiveness differ from one type to another. The therapeutic approach is generally multidisciplinary and often complex. Developments in surgical and oncological dermatology during the last few decades have positioned dermatologists as specialists in the diagnosis and treatment of skin cancer. Read More

J Dtsch Dermatol Ges 2018 Dec;16(12):1434-1442

Department of Dermatology, Eberhardt Karls University, Tübingen Germany.

Background: Cutaneous sarcomas are rare and characterized by pathogenetic heterogeneity. Knowledge about local infiltration patterns and recurrence rates may be useful in improving patient care and outcomes. The objective of the present study was to compare these two characteristics in sarcomas that had been treated using the identical surgical procedure. Read More

Hematol Oncol Clin North Am 2019 Feb;33(1):87-101

Department of Dermatology, Brigham and Women's Hospital, 221 Longwood Avenue, Boston, MA 02115, USA; Cutaneous Oncology Program, Dana Farber Cancer Institute, Boston, MA 02215, USA. Electronic address:

Cutaneous sarcomas are rare malignancies that may present with a variety of clinical manifestations. This article focuses on 4 of the most common cutaneous sarcomas (Kaposi sarcoma, cutaneous angiosarcoma, dermatofibrosarcoma protuberans, and cutaneous leiomyosarcoma) and reviews clinical, diagnostic, and therapeutic aspects of these rare skin malignancies. Read More

Head Neck Pathol 2018 Nov 22. Epub 2018 Nov 22.

Tissue Pathology and Diagnostic Oncology, Royal Prince Alfred Hospital, Sydney, Australia.

Head and neck pathology present a unique set of challenges including the morphological diversity of the neoplasms and presentation of metastases of unknown primary origin. The detection of human papillomavirus and Epstein-Barr virus associated with squamous cell carcinoma and newer entities like HPV-related carcinoma with adenoid cystic like features have critical prognostic and management implications. In salivary gland neoplasms, differential diagnoses can be broad and include non-neoplastic conditions as well as benign and malignant neoplasms. Read More

Hautarzt 2019 Feb;70(2):123-126

Klinik für Mund‑, Kiefer- und Gesichtschirurgie, Universitätsklinikum Münster, Waldeyerstr. 30, 48149, Münster, Deutschland.

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. Read More

Urology 2018 Nov 10. Epub 2018 Nov 10.

Department of Urology, University of Kentucky, Lexington, KY; Department of Surgery, Division of Urology, University of Colorado, Aurora, CO. Electronic address:

Dermtofibrosarcoma protuberans is a rare cutaneous malignancy known to be locally aggressive. It is uncommonly seen in the pediatric population and can be difficult to distinguish from other benign skin lesions. We present a case of dermatofibrosarcoma protuberans of the penis in a 6-month-old child managed with surgical resection. Read More

In Vitro Cell Dev Biol Anim 2019 Jan 8;55(1):62-73. Epub 2018 Nov 8.

Department of Innovative Seeds Evaluation, National Cancer Center Research Institute, 5-1-1 Tsukiji, Chuo-ku, Tokyo, 104-0045, Japan.

Dermatofibrosarcoma protuberans (DFSP) is a common type of dermal sarcoma, characterized by the presence of the unique collagen type I alpha 1 chain (COL1A1)-PDGFB translocation, which causes constitutive activation of the platelet-derived growth factor β (PDGFB) signaling pathway. Patients with DFSP exhibit frequent local recurrence, and novel therapeutic approaches are required to achieve better clinical outcomes. Patient-derived cancer cell lines are essential in the preclinical research. Read More