Malignant peripheral nerve sheath tumor (MPNST) arising from angiosarcoma: a rare case report

  • Maria Lazaridou
  • Eleni Bourlidou
  • Margarita Vafeiadou
  • Yiannis Papadiochos
  • Nikolaos Barbetakis
  • Anastasia Kiziridou
  • Doxa Mangoudi
Case Report



An extremely rare case of divergent differentiation of a cutaneous angiosarcoma to a malignant peripheral nerve sheath tumor (MPNST) is presented.


A 62-year-old male patient presented with an angiosarcoma of the left buccal region, which was excised with wide surgical margins. Four months after the operation, the tumor recurred locally as a MPNST. The recurrent tumor was treated with surgical excision followed by postoperative radiotherapy. Four years later, the patient presented with solitary lung metastasis. The patient underwent upper right lobectomy.


The patient is alive and disease-free 2 years after the lobectomy and 6 years after initial presentation.


Divergent differentiation of an angiosarcoma to a neurosarcoma supports the theory that cells of the neural crest capable of differentiating both to neuroectoderm and to mesenchyme give origin to these tumors.


Malignant peripheral nerve sheath tumor Head and neck sarcoma Angiosarcoma Divergent differentiation 


Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

Ethical approval

This article does not contain any studies with human participants or animals performed by any of the authors.

Informed consent

This article does not contain any studies with human participants or animals performed by any of the authors.


  1. 1.
    Oztürk O, Tutkun A (2012) A case report of a malignant peripheral nerve sheath tumor of the oral cavity in neurofibromatosis type 1. Case Rep Otolaryngol 2012:936735PubMedPubMedCentralGoogle Scholar
  2. 2.
    Mullins B, Hackman T (2015) Angiosarcoma of the head and neck. Int Arch Otorhinolaryngol 19(3):191–195CrossRefGoogle Scholar
  3. 3.
    Brown RW, Tornos C, Evans HL (1992) Angiosarcoma arising from malignant schwannoma in a patient with neurofibromatosis. Cancer 70(5):1141–1144CrossRefGoogle Scholar
  4. 4.
    Vora R, Anjaneyan G, Gupta R (2014) Cutaneous angiosarcoma of head and neck. Indian J Dermatol 59(6):632. CrossRefPubMedPubMedCentralGoogle Scholar
  5. 5.
    Mendenhall WM, Mendenhall CM, Werning JW, Reith JD, Mendenhall NP (2006) Cutaneous angiosarcoma. Am J Clin Oncol 29(5):524–528CrossRefGoogle Scholar
  6. 6.
    Patel SH, Hayden RE, Hinni ML, Wong WW, Foote RL, Milani S, Wu Q, Ko SJ, Halyard MY (2015) Angiosarcoma of the scalp and face: the Mayo Clinic experience. JAMA Otolaryngol Head Neck Surg 141(4):335–340CrossRefGoogle Scholar
  7. 7.
    Mark RJ, Tran LM, Sercarz J, Fu YS, Calcaterra TC, Juillard GF (1993) Angiosarcoma of the head and neck. The UCLA experience 1955 through 1990. Arch Otolaryngol Head Neck Surg 119(9):973–978CrossRefGoogle Scholar
  8. 8.
    Hwang K, Kim MY, Lee SH (2015) Recommendations for therapeutic decisions of angiosarcoma of the scalp and face. J Craniofac Surg 26(3):e253–e256CrossRefGoogle Scholar
  9. 9.
    Stucky CC, Johnson KN, Gray RJ, Pockaj BA, Ocal IT, Rose PS, Wasif N (2012) Malignant peripheral nerve sheath tumors (MPNST): the Mayo Clinic experience. Ann Surg Oncol 19(3):878–885. CrossRefPubMedGoogle Scholar
  10. 10.
    Rekhi B, Ingle A, Kumar R, DeSouza MA, Dikshit R, Jambhekar NA (2010) Malignant peripheral nerve sheath tumors: clinicopathological profile of 63 cases diagnosed at a tertiary cancer referral center in Mumbai, India. Indian J Pathol Microbiol 53(4):611–618CrossRefGoogle Scholar
  11. 11.
    Mahajan V, Rao S, Gupta P, Munjal M, Agrawal S (2015) Angiosarcoma developing in a vagal schwannoma: a rare case report. Head Neck Pathol 9(3):405–411CrossRefGoogle Scholar
  12. 12.
    Ducatman BS, Scheithauer BW (1984) Malignant peripheral nerve sheath tumors with divergent differentiation. Cancer 54(6):1049–1057CrossRefGoogle Scholar
  13. 13.
    Horstadius SO (1969) The neural crest: its properties and derivatives in the light of experimental research. Hafner, New YorkGoogle Scholar

Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  • Maria Lazaridou
    • 1
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  • Eleni Bourlidou
    • 2
  • Margarita Vafeiadou
    • 3
  • Yiannis Papadiochos
    • 4
  • Nikolaos Barbetakis
    • 5
  • Anastasia Kiziridou
    • 6
  • Doxa Mangoudi
    • 7
  1. 1.ThessalonikiGreece
  2. 2.AthensGreece
  3. 3.Papanikolaou HospitalThessalonikiGreece
  4. 4.Hospital Aglaia KyriakouAthensGreece
  5. 5.Department of Thoracic Surgery“Theagenio” Cancer HospitalThessalonikiGreece
  6. 6.Department of Pathology“Theagenio” Cancer HospitalThessalonikiGreece
  7. 7.Department of Oral and Maxillofacial Surgery “Theagenio” Cancer Hospital (Former)“Theagenio” Cancer HospitalThessalonikiGreece

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