Genetics of dermatofibrosarcoma protuberans
family of tumors: from ring chromosomes to tyrosine kinase inhibitor treatment.
Differential diagnosis of dermatofibroma Benign lesions Malignant lesions Cyst Basal cell carcinoma Hypertrophic scar Dermatofibrosarcoma protuberans
Neurilemmoma (or schwannoma) Giant cell tumor of the skin Neurofibroma Nodular melanoma Piloleiomyoma Sclerosing sweat duct carcinoma Tophus Erythema elevatum diutinum
was first described by Darier and Ferrand in 1924 and named by Hoffmann in 1925.
is a slow-growing lesion involving the trunk or proximal extremity of young and middle-aged adults.
Giant cell fibroblastoma: a juvenile form of dermatofibrosarcoma protuberans
The solid tumor is dermatofibrosarcoma protuberans
(DFSP), a type of tumor that begins as a hard lump found in the skin of the chest, abdomen or leg.
The diseases addressed in the research studies include prostate cancer, Philadelphia chromosome-positive (Ph+) chronic myeloid leukemia (CML), dermatofibrosarcoma protuberans
(DFSP), Kit-positive gastrointestinal stromal tumors (GISTs), hypereosonophilia (HES), Kaposi's sarcoma and carcinoid tumors, among many others.
D2-40 may represent a helpful adjunct in distinguishing between dermatofibromas (DFs) and dermatofibrosarcoma protuberans
(DFSP), which can occasionally be difficult.
The cells may be disposed in varying manners, such as in a lamellar arrangement, perivascular whorling formation (Figure 2, A and B), or even short bundles with a vague storiform pattern reminiscent of that seen in dermatofibrosarcoma protuberans
(Figure 2, C).
In addition, 6 monophasic synovial sarcomas, 9 leiomyosarcomas, and 5 dermatofibrosarcoma protuberans
were included as controls.
1] The relationship of GCF to dermatofibrosarcoma protuberans
(DFSP) is not known, although cases of GCF with DFSP-like areas and the recurrence of GCF as frank DFSP and vice versa suggests that the 2 lesions may be related.
5,7] CD34 is also expressed on vascular endothelium and vascular tumors, DICs, endoneurium, stromal tumors of the gastrointestinal tract, solitary fibrous tumors, epithelioid sarcomas, dermatofibrosarcoma protuberans
, and some peripheral nerve sheath tumors.