TY - JOUR
T1 - Primary Ewing's sarcoma of the sinonasal tract in adults
T2 - A challenging disease
AU - Lombardi, Davide
AU - Mattavelli, Davide
AU - Redaelli De Zinis, Luca O.
AU - Accorona, Remo
AU - Morassi, Maria L.
AU - Facchetti, Fabio
AU - Ferrari, Vittorio
AU - Farina, Davide
AU - Bertulli, Rossella
AU - Nicolai, Piero
PY - 2017/3/1
Y1 - 2017/3/1
N2 - Background: Sinonasal localization of Ewing's sarcoma in adults is an exceedingly rare event. Methods: The clinical records of 5 patients with primary sinonasal Ewing's sarcoma treated from 1992 to 2012 were retrospectively analyzed. All pathologic slides were reviewed by 2 experienced pathologists. All patients underwent multimodality treatments. Results: Median age was 36 years (range, 25–52 years). At referral, 2 patients had the original diagnosis changed by review of the histologic slides. Tumors were classified as T4aN0M0 (4 patients) and T2N0M0 (1 patient). Median follow-up was 110 months (range, 70–139 months). Only 1 patient, who started treatment elsewhere based on an incorrect histologic diagnosis, experienced multiple recurrences and eventually died of widespread metastasis. Conclusion: Correct pathologic diagnosis can have a crucial impact on treatment planning and outcome. Multimodality therapy is the key for long-term successful results. Because of the rarity of the tumor, referral to highly experienced care centers is strongly recommended.
AB - Background: Sinonasal localization of Ewing's sarcoma in adults is an exceedingly rare event. Methods: The clinical records of 5 patients with primary sinonasal Ewing's sarcoma treated from 1992 to 2012 were retrospectively analyzed. All pathologic slides were reviewed by 2 experienced pathologists. All patients underwent multimodality treatments. Results: Median age was 36 years (range, 25–52 years). At referral, 2 patients had the original diagnosis changed by review of the histologic slides. Tumors were classified as T4aN0M0 (4 patients) and T2N0M0 (1 patient). Median follow-up was 110 months (range, 70–139 months). Only 1 patient, who started treatment elsewhere based on an incorrect histologic diagnosis, experienced multiple recurrences and eventually died of widespread metastasis. Conclusion: Correct pathologic diagnosis can have a crucial impact on treatment planning and outcome. Multimodality therapy is the key for long-term successful results. Because of the rarity of the tumor, referral to highly experienced care centers is strongly recommended.
KW - chemotherapy
KW - Ewing's sarcoma
KW - multimodality treatment
KW - sinonasal cancer
KW - transnasal endoscopic surgery
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U2 - 10.1002/hed.24649
DO - 10.1002/hed.24649
M3 - Article
AN - SCOPUS:85012950262
VL - 39
SP - E45-E50
JO - Head and Neck Surgery
JF - Head and Neck Surgery
SN - 1043-3074
IS - 3
ER -