LMNA‐NTRK1 rearranged mesenchymal tumor (lipofibromatosis‐like neural tumor) mimicking pigmented dermatofibrosarcoma protuberans

2020 ◽  
Author(s):  
Gauri Panse ◽  
Emily Reisenbichler ◽  
Matija Snuderl ◽  
Wei‐Lien Wang ◽  
William Laskin ◽  
...  
Keyword(s):  
Dermatofibrosarcoma Protuberans ◽  
Mesenchymal Tumor ◽  
Neural Tumor

scholarly journals Update on Superficial Spindle Cell Mesenchymal Tumors in Children

2021 ◽  
Vol 8 (3) ◽  
pp. 285-300
Author(s):  
Philippe Drabent ◽  
Sylvie Fraitag
Keyword(s):  
Spindle Cell ◽  
Dermatofibrosarcoma Protuberans ◽  
Diagnostic Value ◽  
Mesenchymal Tumors ◽  
Infantile Fibrosarcoma ◽  
Genetic Features ◽  
Fibrous Hamartoma Of Infancy ◽  
Neural Tumor ◽  
Prognostic Implications ◽  
Plexiform Fibrohistiocytic Tumor

The diagnosis of cutaneous and subcutaneous spindle cell neoplasms in children is often challenging and has potential therapeutic and prognostic implications. Although correctly diagnosing dermatofibrosarcoma protuberans and infantile fibrosarcoma is paramount, pathologists should not ignore a number of diagnostic pitfalls linked to mostly rare tumors with completely different clinical outcomes. In the last decade, a spectrum of novel entities has been described; information from molecular biology has helped to shape this new landscape for spindle cell tumors. Here, we review the most noteworthy neoplasms in this spectrum, with a focus on their histological similarities: fibroblastic connective tissue nevus, medallion-like dermal dendrocyte hamartoma, or plaque-like CD34-positive dermal fibroma, which share features with fibrous hamartoma of infancy; lipofibromatosis and lipofibromatosis-like neural tumor; and plexiform myofibroblastoma, a recently described neoplasm that should be distinguished from plexiform fibrohistiocytic tumor. These tumors also have genetic similarities, particularly gene rearrangements involving NTRK3 or NTRK1. These genetic features are not only essential for the differential diagnosis of infantile fibrosarcoma but are also of diagnostic value for lipofibromatosis-like neural tumors. The more recently described RET, RAF1, and BRAF gene fusions are also discussed.

scholarly journals Dermoscopy of Dermatofibrosarcoma Protuberans: What Do We Know?

2019 ◽  
pp. 139-145 ◽  
Author(s):  
Gabriela F. Escobar ◽  
Caroline  K.  Ribeiro ◽  
Leandro L. Leite ◽  
Carolina R. Barone ◽  
André Cartell
Keyword(s):  
Literature Search ◽  
Single Case ◽  
Delayed Diagnosis ◽  
Dermatofibrosarcoma Protuberans ◽  
Clinical Suspicion ◽  
Mesenchymal Tumor ◽  
Benign Lesions ◽  
Common Features ◽  
Slow Progression

Background: Dermatofibrosarcoma protuberans (DFSP) is an uncommon mesenchymal tumor of intermediate malignancy. Its rarity and slow progression often imply a delayed diagnosis. There are few previous reports of dermoscopic features of DFSP and most are single case descriptions. Objectives: To report 2 cases of DFSP and their dermoscopic features, as well as conduct a review of all previous cases published addressing the use of dermoscopy in this tumor. Methods: We conducted a literature search for all dermoscopic cases of DFSP. In addition, we presented 2 additional cases and compared them to the earlier findings. Results: We summarized the main dermoscopic findings of DFSP based on analysis from 32 patients. The most common features of this tumor are the presence of vessels (81%), followed by a pigmented network (78%) and a pinkish background (66%). Conclusions: DFSP can mimic benign lesions and the diagnosis may be challenging. Dermoscopy is an important tool that may enhance clinical suspicion toward the diagnosis of DFSP.

scholarly journals Dermatofibrosarcoma protuberans: surgical management of a challenging mesenchymal tumor

2019 ◽  
Vol 17 (1) ◽  
Author(s):  
Felix Wiesmueller ◽  
Abbas Agaimy ◽  
Aristotelis Perrakis ◽  
Andreas Arkudas ◽  
Raymund E. Horch ◽  
...  
Keyword(s):  
Surgical Management ◽  
Dermatofibrosarcoma Protuberans ◽  
Mesenchymal Tumor

Dermatofibrosarcoma protuberans der Mamma – ein Fallbericht

2018 ◽  
Author(s):  
II Iordache ◽  
G Schmidt ◽  
A Leingartner ◽  
R Sima ◽  
D Benndorf ◽  
...  
Keyword(s):  
Dermatofibrosarcoma Protuberans

Atypical mesenchymal tumor of the uterus, represented by cystadenoma (A case from practice Literature review)

2018 ◽  
pp. 16-18
Author(s):  
V.S. Sukhin ◽  
◽  
O.V. Slobodyanyuk ◽  
L.D. Skrypnyk ◽  
◽  
...  
Keyword(s):  
Literature Review ◽  
Mesenchymal Tumor ◽  
Practice Literature

Dermatofibrosarcoma Protuberans Growing Around Plantar Aponeurosis: Excision by Mohs Micrographic Surgery

2000 ◽  
Vol 26 (10) ◽  
pp. 941-945 ◽  
Author(s):  
Gregory J. Kricorian ◽  
Carl F. Schanbacher ◽  
Paul A. Kelly ◽  
Richard G. Bennett
Keyword(s):  
Dermatofibrosarcoma Protuberans ◽  
Mohs Micrographic Surgery ◽  
Micrographic Surgery ◽  
Plantar Aponeurosis

scholarly journals A Case of Dermatofibrosarcoma Protuberans of the Chest Wall with Metastases to the Lung and the Small Intestine after Rocal Recurrence

2015 ◽  
Vol 76 (8) ◽  
pp. 1861-1869
Author(s):  
Gen YUNOME ◽  
Shin TESHIMA ◽  
Ayako ENDOU ◽  
Toshihiro SAITOU ◽  
Kazunori TAKEDA ◽  
...  
Keyword(s):  
Small Intestine ◽  
Chest Wall ◽  
Dermatofibrosarcoma Protuberans
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