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Aktuelle Dermatologie 2022; 48(10): 445-448
DOI: 10.1055/a-1876-4491
Kasuistik

Hybridtumor eines Riesenzellfibroblastoms und eines Dermatofibrosarcoma protuberans

Hybridtumor of a Giant Cell Fibroblastoma and Dermatofibrosarcoma protuberans
Marcel Boede
1   Klinik für Dematologie und Venerologie, Vivantes Klinikum Neukölln, Berlin, Deutschland
,
Bernd Tillig
2   Klinik für Kinderchirurgie, Neugeborenenchirurgie und Kinderurologie, Vivantes Klinikum Neukölln, Berlin, Deutschland
,
Ulrike Zimmermann
3   Praxis für Pathologie, Vivantes MVZ Kreuzberg, Berlin, Deutschland
,
Silja Guski
4   Vivantes Pathologie, Vivantes Klinikum Neukölln, Berlin, Deutschland
,
Uwe Hillen
1   Klinik für Dematologie und Venerologie, Vivantes Klinikum Neukölln, Berlin, Deutschland
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Zusammenfassung

Wir berichten über ein zum Zeitpunkt der Erstmanifestation 10 Monate altes Kind mit einem Hybridtumor aus Riesenzellfibroblastom (RZFB) und Dermatofibrosarkoma protuberans (DFSP). Das RZFB ist ein seltener Tumor, der mit dem DFSP die Translokation (t17;22) (q22;13) und immunphänotypische Eigenschaften teilt und daher von seinen Erstbeschreibern als kindliche Form des DFSP eingeordnet wurde. Das RZFB hat mit einer Kombination aus Spindelzellproliferaten, häufig hypozellulären Arealen mit kollagenfaserreichem oder myxoidem Stroma, sinusoid-artigen pseudovaskulären Aufweitungen zwischen den Kollagenfasern, die von multinukleären Riesenzellen umsäumt werden, jedoch ein ihm eigenes histologisches Bild. Das RZFB, das sich klinisch als langsam wachsende, i.d.R. schmerzlose Schwellung manifestiert, tritt präferentiell im Kindesalter auf. Das mittlere Erkrankungsalter liegt bei 6 Jahren. Die Therapie der Wahl ist die Exzision mit einem ausreichenden Sicherheitsabstand. Unsere junge Patientin zeigt ein Jahr nach Exzision mit 2 cm Sicherheitsabstand einen rezidivfreien Verlauf.

Abstract

We report on a 10 months old infant with a hybrid lesion of giant cell fibroblastoma (GCF) and dermatofibrosarcoma protuberans (DFSP). GCF is a rare tumor, which shares with DFSP the (t17;22) (q22;13) translocation and immunophenotypic characteristics; therefore GCF is considered as juvenile form of DFSP. GCF is characterized by fascicles of slender spindle cells, often hypocellular areas with collagenous or myxoid stroma and pseudovascular spaces lined by multinuclear giant cells. Clinically, GCF usually presents as a slow growing painless tumor, predominantly evolving in childhood with a mean age of 6 years. Excision with adequate safety margins is the treatment of choice. Our patient was one year after tumor resection with 2 cm margin in complete remission.

Schlüsselwörter

Riesenzellfibroblastom - Dermatofibrosarkoma protuberans - Hybridtumor - pädiatrische Tumore

Keywords

Giant cell fibroblastoma - dermatofibrosarcoma protuberans - hybrid tumor - childhood tumor


Publication History

Article published online:
24 October 2022

© 2022. Thieme. All rights reserved.

Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany

 

  • Literatur

  • 1 Shmookler B, Enzinger F. Giant cell fibroblastoma: A peculiar childhood tumor. Lab Invest 1982; 46: 76-76
    PubMedSearch in Google Scholar
  • 2 Mentzel TDW, Pedeutour F. WHO Classification of Tumours Editorial Board. Giant cell fibroblastoma. In: Soft tissue and bone tumours. Lyon: International Agency for Research on Cancer; 2020. 5th edition. 98-99
    Search in Google Scholar
  • 3 Ugurel S, Kortmann RD, Mohr P. et al. S1-Leitlinie Dermatofibrosarcoma protuberans (DFSP) – Update 2018. J Dsch Dermatol Ges 2019; 17: 663-668
    CrossrefPubMedSearch in Google Scholar
  • 4 Saiag P, Grob JJ, Lebbe C. et al. Diagnosis and treatment of dermatofibrosarcoma protuberans. European consensus-based interdisciplinary guideline. Eur J Cancer 2015; 51: 2604-2608
    CrossrefPubMedSearch in Google Scholar
  • 5 Shmookler BM, Enzinger FM, Weiss SW. Giant cell fibroblastoma. A juvenile form of dermatofibrosarcoma protuberans. Cancer 1989; 64: 2154-2161
    CrossrefPubMedSearch in Google Scholar
  • 6 Aiba S, Tabata N, Ishii H. et al. Dermatofibrosarcoma protuberans is a unique fibrohistiocytic tumour expressing CD34. Br J Dermatol 1992; 127: 79-84
    CrossrefPubMedSearch in Google Scholar
  • 7 Terrier-Lacombe MJ, Guillou L, Maire G. et al. Dermatofibrosarcoma protuberans, giant cell fibroblastoma, and hybrid lesions in children: clinicopathologic comparative analysis of 28 cases with molecular data - a study from the French Federation of Cancer Centers Sarcoma Group. Am J Surg Path 2003; 27: 27-39
    CrossrefPubMedSearch in Google Scholar
  • 8 Brennan B, Zanetti I, De Salvo GL. et al. Dermatofibrosarcoma protuberans in children and adolescents: The European Paediatric Soft Tissue Sarcoma Study Group prospective trial (EpSSG NRSTS 2005). Pediatr Blood & Cancer 2020; 67: e28351
    CrossrefPubMedSearch in Google Scholar
  • 9 Posso-De Los Rios CJ, Lara-Corrales I, Ho N. Dermatofibrosarcoma protuberans in pediatric patients: a report of 17 cases. J Cutan Med Surg 2014; 18: 180-185
    CrossrefPubMedSearch in Google Scholar
  • 10 Rubio GA, Alvarado A, Gerth DJ. et al. Incidence and Outcomes of Dermatofibrosarcoma Protuberans in the US Pediatric Population. J Craniofac Surg 2017; 28: 182-184
    CrossrefPubMedSearch in Google Scholar
  • 11 Serra-Guillen C, Llombart B, Nagore E. et al. Mohs micrographic surgery in dermatofibrosarcoma protuberans allows tumour clearance with smaller margins and greater preservation of healthy tissue compared with conventional surgery: a study of 74 primary cases. Br J Dermatol 2015; 172: 1303-1307
    CrossrefPubMedSearch in Google Scholar
  • 12 Lowe GC, Onajin O, Baum CL. et al. A Comparison of Mohs Micrographic Surgery and Wide Local Exzision for Treatment of Dermatofibrosarcoma Protuberans With Long-Term Follow-up: The Mayo Clinic Experience. Dermatol Surg 2017; 43: 98-106
    CrossrefPubMedSearch in Google Scholar
  • 13 Veronese F, Boggio P, Tiberio R. et al. Wide local excision vs. Mohs Tubingen technique in the treatment of dermatofibrosarcoma protuberans: a two-centre retrospective study and literature review. J Eur Acad Dermatol Venereol 2017; 31: 2069-2076
    CrossrefPubMedSearch in Google Scholar
  • 14 Sleiwah A, Wright TC, Chapman T. et al. Dermatofibrosarcoma Protuberans in Children. Curr Treat Options Oncol 2022; 23: 843-854
    CrossrefPubMedSearch in Google Scholar
  • 15 Zargham H, Khachemoune A. Systematic review of Mohs micrographic surgery in children: Identifying challenges and practical considerations for successful application. J Am Acad Dermatol 2021; 85: 152-161
    CrossrefPubMedSearch in Google Scholar
  • 16 Sleiwah A, Psomadakis C, Craythorne E. et al. Dermatofibrosarcoma protuberans (DFSP) in children: A combined multidisciplinary approach. Pediatr Dermatol 2021; 38: 233-236
    CrossrefPubMedSearch in Google Scholar
  • 17 Rutkowski P, Klimczak A, Lugowska I. et al. Long-term results of treatment of advanced dermatofibrosarcoma protuberans (DFSP) with imatinib mesylate – The impact of fibrosarcomatous transformation. Eur J Surg Oncol 2017; 43: 1134-1141
    CrossrefPubMedSearch in Google Scholar
  • 18 Rodriguez Bandera AI, Moreno Bonilla G, Feito Rodriguez M. et al. Jellyfish-like sonographic pattern can help recognition of dermatofibrosarcoma protuberans. Report of 3 new cases and review of the literature. Aust J Dermatol 2019; 60: e148-e150
    CrossrefPubMedSearch in Google Scholar
  • 19 Diago A, Llombart B, Serra-Guillen C. et al. Usefulness of ultrasound in dermatofibrosarcoma protuberans and correlation with histopathological findings: A series of 30 cases. Skin Res Technol 2021; 27: 701-708
    CrossrefPubMedSearch in Google Scholar
  • 20 Zou MH, Huang Q, Yang T. et al. Role of ultrasound in the diagnosis of primary and recurrent dermatofibrosarcoma protuberans. BMC Cancer 2021; 21: 909
    CrossrefPubMedSearch in Google Scholar