title: Dermatofibrosarcoma Protu-Berans with Lung Metastasis Requiring Pneumonectomy.
abstract: We report the case of a young man diagnosed with dermatofibrosarcoma protuberans lung metastases seven years after primary tumor resection. Notably, no previous local recurrences had been observed. A multimodal approach was used for the management of this patient: surgery, radiotherapy and targeted therapy with Imatinib. The patient is alive with stable disease after thirty months of the metastases diagnoses. Dermatofibrosarcoma protu-berans metastasizes very rarely, and when it does, it is usually either after local recurrence or whenever fibrosarcomatous transformation is found in the histopathological analysis, which confers an increased risk of local recurrence and metastases. This is the second report of a metastatic dermatofibrosarcoma protuberans occurring in a patient with no previous local recurrence or histological fibrosar-comatous features, emphasizing the rarity of the disease presentation and the importance of targeted therapy in improving patient quality of life and survival.
authors.affiliation: Department of Medical Oncology, Hospital Universitario de la Princesa, Madrid, Spain; Department of Pathology, Icahn School of Medicine at Mount Sinai, New York, NY, USA.
authors.forename: Ana
authors.initials: A
authors.lastname: Collazo Lorduy
authors.affiliation: Department of Medical Oncology, Hospital Universitario de la Princesa , Madrid, Spain.
authors.forename: Berta
authors.initials: B
authors.lastname: Obispo
authors.affiliation: Department of Pathology, Hospital Universitario de la Princesa , Madrid, Spain.
authors.forename: Karen
authors.initials: K
authors.lastname: Villar
authors.affiliation: Department of Radiation Oncology, Hospital Universitario de la Princesa , Madrid, Spain.
authors.forename: David
authors.initials: D
authors.lastname: Buchser
authors.affiliation: Department of Medical Oncology, Hospital Universitario de la Princesa , Madrid, Spain.
authors.forename: Jacobo
authors.initials: J
authors.lastname: Rogado
authors.affiliation: Department of Medical Oncology, Hospital Universitario de la Princesa , Madrid, Spain.
authors.forename: Maria Dolores Fenor
authors.initials: MD
authors.lastname: de la Maza
authors.affiliation: Department of Medical Oncology, Hospital Universitario de la Princesa , Madrid, Spain.
authors.forename: Yolanda Lage
authors.initials: YL
authors.lastname: Alfranca
authors.affiliation: Department of Medical Oncology, Hospital Universitario de la Princesa , Madrid, Spain.
authors.forename: Ramon
authors.initials: R
authors.lastname: Colomer
keywords: Dermatofibrosarcoma protuberans; imatinib; lung metastases; pneumonectomy