title: Primitive sarcoma of the breast: new insight on the proper surgical management.
abstract: BACKGROUND
  Primitive sarcoma of the breast is a rare and challenging disease at high risk of recurrence and with poor prognosis. There are controversies in the diagnosis and management of such solid tumor due to its rarity and heterogeneity. This sarcoma is poorly responsive to both chemotherapy and radiotherapy, thus, surgery is the first and most important therapeutic approach. However, given the rarity of this type tumor it has not be possible to standardize unique guidelines for the proper surgical strategy to adopt. Here, we performed a retrospective study of new 10 cases of primitive sarcoma of the breast that underwent either mastectomy or a more conservative quadrantectomy, in the attempt to better standardize correct surgical indications.
  
  
  METHODS
  Ten new cases of primitive sarcoma of the breast were registered between 2002 and 2012 and constituted the study group. They underwent either mastectomy or quadrantectomy and the clinical, prognostic and survival characteristics after surgery were analysed.
  
  
  RESULTS
  Within the group of patients treated with mastectomy, two had metastasis and died because of that. Among the five patients treated with quadrantectomy four are alive and free of disease after 3 to 5 years follow-up, while the patient with sarcoma arising in pregnancy, although is still alive, developed lung metastases four years after surgery.
  
  
  CONCLUSIONS
  The first and most important therapeutic approach to primary sarcomas of the breast is surgical which has the purpose to achieve radical tumor excision to prevent local recurrence and skip metastases. However, given the rarity of the condition and the consequent small number of cases in this, like in similar studies, it is not possible to draw any definitive conclusions and further studies with larger numbers are necessary. However it would appear that performing a larger procedure such as mastectomy rather than performing a more limited one such as a quadrantectomy, has no advantage in terms of overall prognosis.
authors.affiliation: Department of Surgical Sciences, "Sapienza" University of Rome, Viale Regina Elena 324, 00161, Rome, Italy. vittorio.pasta@uniroma1.it.
authors.forename: Vittorio
authors.initials: V
authors.lastname: Pasta
authors.affiliation: Department of Surgical Sciences, "Sapienza" University of Rome, Viale Regina Elena 324, 00161, Rome, Italy. massimo.monti@uniroma1.it.
authors.forename: Massimo
authors.initials: M
authors.lastname: Monti
authors.affiliation: Department of General Microsurgery and Hand Surgery, "Fabia Mater" Hospital, Via Olevano Romano 25, 00171, Rome, Italy. michelacialini@gmail.com.
authors.forename: Michela
authors.initials: M
authors.lastname: Cialini
authors.affiliation: Department of Surgical Sciences, "Sapienza" University of Rome, Viale Regina Elena 324, 00161, Rome, Italy. massimo.vergine@uniroma1.it.
authors.forename: Massimo
authors.initials: M
authors.lastname: Vergine
authors.affiliation: Department of Surgical Sciences, "Sapienza" University of Rome, Viale Regina Elena 324, 00161, Rome, Italy. purciuoli@gmail.com.
authors.forename: Paolo
authors.initials: P
authors.lastname: Urciuoli
authors.affiliation: Department of Gynecology, "Fabia Mater" Hospital, Via Olevano Romano 25, 00171, Rome, Italy. nunziaiacovelli@yahoo.it.
authors.forename: Annunziata
authors.initials: A
authors.lastname: Iacovelli
authors.affiliation: Department of Surgical Oncology, University of L'Aquila, Via Vetoio località Coppito, 67100, L'Aquila, Italy. silvio.rea@univaq.it.
authors.forename: Silvio
authors.initials: S
authors.lastname: Rea
authors.affiliation: Department of Surgical Sciences, "Sapienza" University of Rome, Viale Regina Elena 324, 00161, Rome, Italy. info@valeriodorazi.com.|Department of General Microsurgery and Hand Surgery, "Fabia Mater" Hospital, Via Olevano Romano 25, 00171, Rome, Italy. info@valeriodorazi.com.
authors.forename: Valerio
authors.initials: V
authors.lastname: D'Orazi
mesh_terms: D001943:Breast Neoplasms / Q000401:mortality / Q000473:pathology / Q000601:surgery*; D005260:Female; D006801:Humans; D008297:Male; D008408:Mastectomy; D008875:Middle Aged; D011379:Prognosis; D012509:Sarcoma / Q000473:pathology / Q000601:surgery*; D015996:Survival Rate