Peripheral primitive neuroectodermal tumor/primary cutaneous Ewing’s sarcoma (PPNET/ES) of the upper eyelid in an adult patient

Peripheral primitive neuroectodermal tumor/primary cutaneous Ewing’s sarcoma (PPNET/ES) of the... Peripheral primitive neuroectodermal tumor (PPNET) and Ewing’s sarcoma (ES) are two variants of a highly malignant bone tumor usually affecting children and young adults with a tendency for early metastasis. Cutaneous localization of PPNET/ES is very uncommon in adult age and is normally confused with other tumors. We report the first recorded case of PPNET of the eyelid in an adult patient. The patient underwent one biopsy, two surgical wide excisions, and two series of 4 cycles of chemotherapy without radiotherapy. At 8 years of follow-up from the first wide excision, the patient is tumor free. Cutaneous PPNET/ES is rare and not considered in the usual differential diagnosis of skin lesions, especially those involving the eyelid. Once diagnosed, a multidisciplinary approach to treatment offers the highest survival rates. Level of Evidence: Level V, diagnostic study. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png European Journal of Plastic Surgery Springer Journals

Peripheral primitive neuroectodermal tumor/primary cutaneous Ewing’s sarcoma (PPNET/ES) of the upper eyelid in an adult patient

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Publisher
Springer Berlin Heidelberg
Copyright
Copyright © 2014 by Springer-Verlag Berlin Heidelberg
Subject
Medicine & Public Health; Plastic Surgery
ISSN
0930-343X
eISSN
1435-0130
D.O.I.
10.1007/s00238-014-0979-8
Publisher site
See Article on Publisher Site

Abstract

Peripheral primitive neuroectodermal tumor (PPNET) and Ewing’s sarcoma (ES) are two variants of a highly malignant bone tumor usually affecting children and young adults with a tendency for early metastasis. Cutaneous localization of PPNET/ES is very uncommon in adult age and is normally confused with other tumors. We report the first recorded case of PPNET of the eyelid in an adult patient. The patient underwent one biopsy, two surgical wide excisions, and two series of 4 cycles of chemotherapy without radiotherapy. At 8 years of follow-up from the first wide excision, the patient is tumor free. Cutaneous PPNET/ES is rare and not considered in the usual differential diagnosis of skin lesions, especially those involving the eyelid. Once diagnosed, a multidisciplinary approach to treatment offers the highest survival rates. Level of Evidence: Level V, diagnostic study.

Journal

European Journal of Plastic SurgerySpringer Journals

Published: Oct 1, 2014

References

  • Superficial primitive neuroectodermal tumor/Ewing sarcoma (PN/ES): same tumor as deep PN/ES or new entity?
    Ehrig, T; Billings, SD; Fanburg-Smith, JC
  • Pelvic primitive neuroectodermal tumor associated with a cluster of small round cell tumors: case report and review of current literature
    Gostout, BS; Lindor, NM; DiMarco, CS; Peethambaram, PP; Clayton, AC

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