@article{3121467, title = "Prognostic factors for recurrence and survival in uncommon variants of vulvar cancer", author = "Zapardiel, I. and Gracia, M. and Díez, J. and Buda, A. and Noya, M.C. and Iaco, P.D. and Vieira-Baptista, P. and Iacoponi, S. and Arones, M. and Arque, M. and Barazi, D. and Bartusevicius, A. and Bernal, M.T. and Blecharz, P. and Buda, A. and Ceccaroni, M. and Chen, F. and Coronado, P. and Covo-Pinto, L. and Cristobal, I. and Cruz, J.L. and De Iaco, P. and De la Torre, J. and Diaz, M. and Diaz-De la Noval, B. and Diez, J. and DiFiore, H. and Dogan, A. and Dursun, P. and Etxabe, I. and Fargas, F. and Feijoo, L. and Fernandez, A. and Fernandez, L. and Fernandez, M. and Festi, A. and Fotopoulou, C. and Franco, S. and Fruscio, R. and Garcia, E. and Garcia-Casals, C. and Gardella, B. and Garrido, R.A. and Gil-Ibañez, B. and Gil-Moreno, A. and Gines, A. and Gomez, A.I. and Gomez, I. and Gonçalves, E. and Gonzalez, L. and Grane, N. and Grigoriadis, C. and Grimm, C. and Gutierrez, L. and Haidopoulos, D. and Herraiz, N. and Iacoponi, S. and Irslinger, E. and Iyibozkurt, A. and Jach, R. and Joigneau, L. and Karlsson, H. and Kondi-Pafiti, A. and Kotsopoulos, I.C. and Macuks, R. and Mardas, M. and Marino, M. and Martinez, A. and Martinez, C. and Martinez-Serrano, M.J. and Martos, M.A. and Menjon, S. and Mitsopoulos, V. and Mora, P. and Morales, S. and Moreno, A. and Novo, A. and Noya, M.C. and Oehler, M. and Papatheodorou, D.C. and Perez, I. and Piek, J. and Polterauer, S. and Prado, A. and Reula, M.C. and Robles, M. and Romeo, M. and Rosado, C. and Rubio, P. and Ruiz, C. and Rzepka, J. and Sanz, R. and Sehouli, J. and Soler, C. and Sukhin, V. and Sznurkowski, J.J. and Tsolakidis, D. and Vieira-Baptista, P. and Yildirim, Y. and Zalewsk, K. and Zapardiel, I. and Zuñiga, M.A. and VULCAN Study collaborative group", journal = "Archives of Gynecology and Obstetrics", year = "2020", publisher = "Springer Science and Business Media Deutschland GmbH", issn = "0932-0067, 1432-0711", doi = "10.1007/s00404-020-05813-x", keywords = "antineoplastic agent, adjuvant chemotherapy; adult; aged; female; human; lymph node; lymph node dissection; middle aged; pathology; prognosis; retrospective study; risk factor; treatment outcome; tumor recurrence; very elderly; vulva tumor; vulvectomy, Adult; Aged; Aged, 80 and over; Antineoplastic Agents; Chemotherapy, Adjuvant; Female; Humans; Lymph Node Excision; Lymph Nodes; Middle Aged; Neoplasm Recurrence, Local; Prognosis; Retrospective Studies; Risk Factors; Treatment Outcome; Vulvar Neoplasms; Vulvectomy", abstract = "Purpose: To analyze the prognostic factors of recurrence and overall survival in rare histotypes of vulvar cancer. Methods: An international multicenter retrospective study including patients diagnosed with vulvar cancer was performed. One hundred centers participated in the study and 2453 vulvar cancer cases were enrolled from January 2001 until December 2005. After exclusion of squamous vulvar cancer, Paget´s disease and vulvar melanoma 112 tumors were analyzed for the present study. Results: The mean age at diagnosis was 64.9 ± 17.2 years. 99 (88.4%) patients had a single lesion, in 25 (22.3%) cases the vulvar tumor involved the midline, and only 13 (11.5%) patients had clinically positive inguinal lymph nodes. The mean size of the lesion was 33.8 ± 33.9 mm. Regarding the surgical treatment, 2 (1.8%) patients underwent skinning vulvectomy, 63 (56.3%) local excision, 41 (36.6%) vulvectomy, 3 (2.7%) exenteration and 3 (2.7%) did not receive any surgical treatment. The mean free surgical margin was 8.2 ± 9 mm and 7 (6.2%) patients presented positive inguinal nodes. Radiotherapy was administered in 22 (19.6%) patients and it was performed postoperatively in all cases; 14 (12.5%) patients received adjuvant chemotherapy. The mean overall follow-up time was 44.1 ± 35.7 months. The risk factors associated with overall survival were chemotherapy and radiotherapy, tumor size and stromal invasion (p < 0.05). The only independent factor significantly associated with global recurrence and absence of metastasis was radiotherapy (p = 0.02 and p = 0.002, respectively). Conclusion: Postoperative radiotherapy seems to be the only independent factor associated with recurrence and overall survival in uncommon types of vulvar cancer. © 2020, Springer-Verlag GmbH Germany, part of Springer Nature." }