====== Dermatofibrosarcoma protuberans ====== Dermatofibrosarcoma protuberans (DFSP) is a rare low-grade [[sarcoma]] of the [[fibroblast]] originating from the dermal layer of the [[skin]], characterized by a locally aggressive growth and high rate of local [[recurrence]]. [[Mutation]]s in [[PDGFB]] are associated with [[meningioma]]. Reciprocal [[translocation]]s between [[chromosome 22]] and [[chromosome 17]], at sites where this [[gene]] and that for [[COL1A1]] are located, are associated with a particular type of skin tumor called [[dermatofibrosarcoma protuberans]] resulting from unregulated expression of [[growth factor]]. Two [[splice]] variants have been identified for this gene. ===== Outcome ===== A successful treatment and management depends on achieving local control and preventing cosmetic and functional deficit; all efforts should be made for complete excision. Postoperative follow-up recommended for highly suspicious cases and annual checkups should be performed up to 5 years after definitive therapy ((Faried A, Hadisaputra W, Arifin MZ. Recurrence case of rare scalp dermatofibrosarcoma protuberans: Two case reports of a wide radical excision, craniectomy bone involvement followed by cranioplasty and reconstruction. Surg Neurol Int. 2017 May 26;8:82. doi: 10.4103/sni.sni_26_17. eCollection 2017. PubMed PMID: 28607816; PubMed Central PMCID: PMC5461560.)) ===== Case reports ===== Two patients underwent a wide radical excision of recurrent scalp DFSP which was reconstructed with translational skin flap and split-thickness skin graft. We described above cases several years ago with a local excision of the tumor; recently, they developed local recurrence of DFSP with calvarial involvement. We then performed a wide radical excision, with craniectomy of the cranial defect followed by cranioplasty using titanium mesh, continuing with reconstruction. A successful treatment and management depends on achieving local control and preventing cosmetic and functional deficit; all efforts should be made for complete excision. Postoperative follow-up recommended for highly suspicious cases and annual checkups should be performed up to 5 years after definitive therapy ((Faried A, Hadisaputra W, Arifin MZ. Recurrence case of rare scalp dermatofibrosarcoma protuberans: Two case reports of a wide radical excision, craniectomy bone involvement followed by cranioplasty and reconstruction. Surg Neurol Int. 2017 May 26;8:82. doi: 10.4103/sni.sni_26_17. eCollection 2017. PubMed PMID: 28607816; PubMed Central PMCID: PMC5461560.)) ===== Unclassified ===== 2: Carloni R, Herlin C, Chaput B, De Runz A, Watier E, Bertheuil N. Scalp Tissue Expansion Above a Custom-Made Hydroxyapatite Cranial Implant to Correct Sequelar Alopecia on a Transposition Flap. World Neurosurg. 2016 Nov;95:616.e1-616.e5. doi: 10.1016/j.wneu.2016.08.116. Epub 2016 Sep 5. PubMed PMID: 27609453. 3: Bashir S, Tariq M, Aslam HM, Hashmi AS, Malik B, Amin A, Mumtaz S. Orbital dermatofibrosarcoma protuberans with intracranial extension preceded by recurrent leiomyoma of the orbit: a case report. J Med Case Rep. 2015 Apr 29;9:96. doi: 10.1186/s13256-015-0561-4. PubMed PMID: 25924890; PubMed Central PMCID: PMC4423146. 4: Liansheng L, Xialiang L, Yaodong Z, Yajun X, Meiqing L. Report of two cases of recurrent scalp dermatofibrosarcoma protuberans and literature review. Indian J Dermatol. 2014 Nov;59(6):602-5. doi: 10.4103/0019-5154.143536. PubMed PMID: 25484394; PubMed Central PMCID: PMC4248501. 5: Hourani R, Taslakian B, Shabb NS, Nassar L, Hourani MH, Moukarbel R, Sabri A, Rizk T. Fibroblastic and myofibroblastic tumors of the head and neck: comprehensive imaging-based review with pathologic correlation. Eur J Radiol. 2015 Feb;84(2):250-60. doi: 10.1016/j.ejrad.2014.10.017. Epub 2014 Nov 1. Review. PubMed PMID: 25467227. 6: Akhaddar A, Lakouichmi M. Darier Ferrand dermatofibrosarcoma of the scalp with frontal intracranial extension. Pan Afr Med J. 2014 Jul 30;18:264. doi: 10.11604/pamj.2014.18.264.4023. eCollection 2014. PubMed PMID: 25426211; PubMed Central PMCID: PMC4242117. 7: Arifin MZ, Yudoyono F, Dahlan RH, Hernowo BS, Sutiono AB, Faried A. A rare giant scalp dermatofibrosarcoma protuberans. Surg Neurol Int. 2014 Apr 1;5:45. doi: 10.4103/2152-7806.129909. eCollection 2014. PubMed PMID: 24818052; PubMed Central PMCID: PMC4014828. 8: Kim JD, Lee HW. Hibernoma: Intense Uptake on F18-FDG PET/CT. Nucl Med Mol Imaging. 2012 Sep;46(3):218-22. doi: 10.1007/s13139-012-0150-z. Epub 2012 Jun 27. PubMed PMID: 24900064; PubMed Central PMCID: PMC4043036. 9: Hidaka T, Fujimura T, Hashimoto A, Aiba S. Successful treatment of pigmented dermatofibrosarcoma protuberance on the nasal root with cyberknife radiosurgery. Acta Derm Venereol. 2012 Nov;92(6):658-9. doi: 10.2340/00015555-1357. PubMed PMID: 22504888. 10: Mattox AK, Mehta AI, Grossi PM, Cummings TJ, Adamson DC. Response of malignant scalp dermatofibrosarcoma to presurgical targeted growth factor inhibition. J Neurosurg. 2010 May;112(5):965-77. doi: 10.3171/2009.7.JNS09522. PubMed PMID: 19681684. 11: Abe T, Kamida T, Goda M, Inoue R, Fujiki M, Kobayashi H, Hatano Y, Shibuya H, Fujiwara S, Terashi H, Mori T. Intracranial infiltration by recurrent scalp dermatofibrosarcoma protuberans. J Clin Neurosci. 2009 Oct;16(10):1358-60. doi: 10.1016/j.jocn.2008.07.088. Epub 2009 Jun 27. PubMed PMID: 19560926. 12: Marakovic J, Vilendecic M, Marinovic T, Lambasa S, Grahovac G. Intracranial recurrence and distant metastasis of scalp dermatofibrosarcoma protuberans. J Neurooncol. 2008 Jul;88(3):305-8. doi: 10.1007/s11060-008-9567-8. Epub 2008 Mar 15. PubMed PMID: 18345515. 13: Kim SD, Park JY, Choi WS, Kim SH, Lim DJ, Chung HS. Intracranial recurrence of the scalp dermatofibrosarcoma. Clin Neurol Neurosurg. 2007 Feb;109(2):172-5. Epub 2006 Jun 6. PubMed PMID: 16757109. 14: Kashima A, Yamashita A, Moriguchi S, Marutsuka K, Tsumori S, Yoshizato K, Ishihara A, Setoyama M, Asada Y. Detection of COL1A1-PDGFB fusion transcripts and platelet-derived growth factor alpha and beta receptors in giant cell fibroblastoma of the postsacrococcygeal region. Br J Dermatol. 2006 May;154(5):983-7. PubMed PMID: 16634907. 15: Nestor SL, Perry A, Kurtkaya O, Abell-Aleff P, Rosemblat AM, Burger PC, Scheithauer BW. Melanocytic colonization of a meningothelial meningioma: histopathological and ultrastructural findings with immunohistochemical and genetic correlation: case report. Neurosurgery. 2003 Jul;53(1):211-4; discussion 214-5. PubMed PMID: 12823892. 16: Sinha VD, Dharker SR, Kalra GS. Dermatofibrosarcoma protuberans of scalp : a case report. Neurol India. 2001 Mar;49(1):81-3. PubMed PMID: 11303249. 17: Das L, Grover SB, Chand K, Dawson L. Intracranial extension of a dermatofibrosarcoma protuberans of the scalp: a case report with brief review of literature. Surg Neurol. 2000 Dec;54(6):452-4. Review. PubMed PMID: 11240177.