A 25-year-old woman presented with a 4 months history of progressive pain and tumefaction in the right parietal region. Deformity of the scalp was evident, and a biopsy was taken, reporting a high- grade dermatofibrosarcoma. She underwent surgical management, achieving a gross total resection. Dermatofibrosarcoma protuberans is a rare tumor arising from the dermis. It tends to have an indolent course and local recurrence after excision.
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| http://dx.doi.org/10.25259/SNI_458_2019 | DOI Listing |
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Giant scalp dermatofibrosarcoma protuberans at mid-occipital scalp: A rare case report.
Surg Neurol Int
November 2024
Department of Neurosurgery, Padjadjaran University/Dr. Hasan Sadikin General Hospital, Bandung, West Java, Indonesia.
Background: Dermatofibrosarcoma protuberans (DFSP) is a rare type of skin cancer that arises in the deeper layers of the skin, most commonly on the trunk and limbs. The presentation of DFSP on the scalp is worth documentation due to its extremely rare occurrence in the literature.
Case Description: We describe a case of a 41-year-old female presented with a lump on the back of the head with gradual enlargement.
Accuracy of Preoperative Imaging in Assessing the Positivity of the Deep Periosteal Margin in Dermatofibrosarcoma Protuberans of the Scalp.
J Craniofac Surg
September 2024
Department of Surgery, Division of Plastic and Reconstructive Surgery, Mayo Clinic.
Article Synopsis
- Dermatofibrosarcoma protuberans (DFSP) is a locally aggressive skin cancer with a high recurrence rate, particularly challenging when it occurs on the scalp due to its potential to invade deeper structures like bone and brain.
- A study of 33 patients treated for DFSP at a tertiary center analyzed demographics, imaging results, and surgical outcomes to evaluate the effectiveness of preoperative imaging (CT and MRI) in guiding treatment.
- The findings indicated that while imaging has low sensitivity and negative predictive value for identifying periosteal invasion, it shows high specificity and positive predictive value, making it valuable for surgical planning and reconstruction.
Asymptomatic CD34 plaque on the scalp of a child.
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Sagis Diagnostics, Houston, Texas, USA.
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- Fibroblastic connective tissue nevus (FCTN) is a rare, non-cancerous skin lesion derived from fibroblasts, which was identified in a 2-year-old boy with an unchanging red plaque on his scalp.
- A biopsy revealed abnormal growth patterns of fibroblastic cells, and although it tested positive for CD34 (which can indicate other conditions), further genetic testing ruled out a more aggressive tumor known as dermatofibrosarcoma protuberans.
- The case emphasizes the need for dermatologists and pathologists to accurately differentiate between various mesenchymal tumors, as they can have vastly different implications for treatment and management.
ALK-rearranged, CD34-positive spindle cell neoplasms resembling dermatofibrosarcoma protuberans: a study of seven cases.
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Department of Anatomic Pathology, Cleveland Clinic, Cleveland, OH, USA.
Aims: The majority of dermatofibrosarcoma protuberans (DFSP) harbour PDGFB or PDGFD rearrangements. We encountered ALK expression/rearrangement in a PDGFB/D-negative CD34-positive spindle cell neoplasm with features similar to DFSP, prompting evaluation of ALK-rearrangements in DFSP and plaque-like CD34-positive dermal fibroma (P-LDF).
Methods And Results: We searched the archives of academic institutions for cases previously coded as DFSP and P-LDF.
Dermatofibrosarcoma Protuberans in "Slow Mohs-tion": Multidisciplinary Approach of A Pediatric Dermatofibrosarcoma Protuberans of the Scalp with Slow Mohs Micrographic Surgery and A Double Rotational-Advancement Scalp Flap.
J Craniofac Surg
July 2024
Department of Otolaryngology - Head and Neck Surgery, Naval Medical Center Portsmouth, Portsmouth, VA.
Dermatofibrosarcoma protuberans (DFSP) is an uncommon soft-tissue sarcoma with primary incidence of 4.1 per million person-years and accounts for 0.1% of all malignancies.
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