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Soft Tissues
Published in Joseph Kovi, Hung Dinh Duong, Frozen Section In Surgical Pathology: An Atlas, 2019
DIFFERENTIAL DIAGNOSIS: ClinicalDermatofibrosarcoma protuberans should be distinguished from dermatofibroma. See differential diagnosis, clinical, under dermatofibroma.*MicroscopicThe differential diagnosis of dermatofibrosarcoma protuberans and dermatofibroma is discussed under differential diagnosis, microscopic, of dermatofibroma.**
Aetiology of Head and Neck Cancer
Published in John C Watkinson, Raymond W Clarke, Terry M Jones, Vinidh Paleri, Nicholas White, Tim Woolford, Head & Neck Surgery Plastic Surgery, 2018
Pablo H. Montero, Snehal G. Patel, Ian Ganly
Sarcomas of the head and neck constitute less than 1% of head and neck malignancies.161 They are divided into those arising from soft tissue (STS)161 and those arising from bone (osteosarcoma).162 STS comprise a heterogeneous group with varied histology and behaviour and include chondrosarcoma, dermatofibrosarcoma protuberans, Ewings sarcoma, leiomyosarcoma, liposarcoma, malignant fibrous histiocytoma, malignant peripheral nerve sheath tumour (MPNT), rhabdomyosarcoma and synovial sarcoma.162 Rhabdomyosarcoma is rare in adults but is the most common STS in children with over 30% occurring in the head and neck. Dermatofibrosarcoma protuberans is a rare tumour of the dermis that has a high recurrence rate. Malignant fibrous histiocytoma is the most common STS in middle and late adulthood. Only 4% of liposarcomas occur in the head and neck with the neck being the most common site. Synovial sarcomas occur between the ages of 20 and 50 years with the majority arising in the parapharyngeal space. The most common site of chondrosarcoma in the head and neck is the larynx, followed by the maxilla and skull base. Most occur between the ages of 30 and 60 years. The most common site in the larynx is the posterior lamina of the cricoid cartilage (75%). MPNTs are extremely rare but more common in patients with neurofibromatosis type I (NF1).
Tumours
Published in Ashley W. Blom, David Warwick, Michael R. Whitehouse, Apley and Solomon’s System of Orthopaedics and Trauma, 2017
Jonathan Stevenson, Michael Parry
Dermatofibrosarcoma protuberans is a low-grade malignant tumour, more common in males, typically occurring between the third and fifth decades, which arises in the trunk, groin and proximal extremities. These slow-growing, nodular lesions may cause cutaneous erythematous plaques; rapid enlargement may indicate progression to high-grade sarcoma present in 10%. Wide surgical excision may be curative, although the rate of local recurrence may reach 50% and is associated with incomplete resection margins Up to 10% may develop distant metastases which are usually high-grade.
Malignancy post-hematopoietic stem cell transplant in patients with primary immunodeficiency
Published in Expert Review of Clinical Immunology, 2020
Kesserwan et al. reported an association between Dermatofibrosarcoma protuberans (DFSP) and ADA deficient SCID patients. DFSP is normally a particularly rare malignant skin tumor in childhood with a low risk of metastasis [58]. The characteristic histologic finding is a spindle cell tumor with a storiform pattern which is CD34+ . At the cytogenetic level it is associated with a characteristic chromosomal translocation (t[17;22] [q22;q13]) resulting in the COL1A1-platelet-derived growth factor β (PDGFB) fusion gene. Kesserwan et al. described 8 patients with DFSP lesions. Mostly these presented as multiple small brown atrophic plaques, less than 1 cm in diameter which had been present in some cases since birth. Cases have been reported post-treatment with PEG-ADA and HSCT suggesting that despite immunological correction of the underlying disorder this association remains a risk [58–60]. Toxic metabolites in the skin and an increased propensity for DNA strand breaks in patients with ADA deficiency may be involved as mechanisms for this association. Careful histological and cytogenetic testing of lesions is required to make the diagnosis and treatment requires wide excision with margin control due to the infiltrative nature of the lesions.
Recurrent Giant Cell Fibroblastoma in an Infant: A Diagnostic Challenge
Published in Fetal and Pediatric Pathology, 2022
Priyanka Maity, Uttara Chatterjee, Mou Das, Sabita Patra
Giant cell fibroblastoma (GCF) is a pediatric soft tissue neoplasm, first described by Enzinger in 1982 [1]. It has a wide spectrum of morphological patterns which led to misdiagnosis of sarcoma in 40% of cases [2]. Local recurrence is as high as 47%. [2] GCF is a fibroblastic tumor of intermediate grade. Recently it has been shown that GCF shares commonality with dermatofibrosarcoma protuberans (DFSP) with regard to histopathology, immunohistochemistry and molecular features [3].
Dermatofibrosarcoma protuberans of the scrotum
Published in Baylor University Medical Center Proceedings, 2021
Mahati Paravathaneni, Keerthy Joseph, Rajesh Thirumaran
Dermatofibrosarcoma protuberans (DFSP) is an uncommon and locally aggressive cutaneous soft tissue sarcoma. Even though they rarely metastasize, these tumors tend to recur locally. DFSP manifests most commonly in middle-aged adults, between the ages of 25 and 45. Here, we present the case of a young man who presented with complaints of an enlarging right scrotal mass and was later diagnosed with the rare entity of DFSP.