Sporadic desmoid tumors of the chest: long-term follow-up of 28 multimodally treated patients

Eur J Cardiothorac Surg. 2011 Nov;40(5):1170-6. doi: 10.1016/j.ejcts.2011.02.014. Epub 2011 Mar 23.

Abstract

Introduction: Desmoids of the chest are extremely rare borderline tumors. Radical surgical resection is considered to be the primary treatment. Achieving negative margins is often a challenge. Cases with positive surgical margins are associated with high risk of local recurrence.

Methods: A retrospective multicenter review was undertaken of 28 patients who underwent surgery for sporadically appearing desmoids of the chest between 1988 and 2008. Clinico-pathological data were investigated in detail. Authors have statistically analyzed the relationships between gender, age, tumor size, radicality of the first surgery, impact of the pharmacologic treatment, estrogen receptor positivity, and the development of local recurrences after a median follow-up period of 104 months.

Results: Primary surgery was radical in 14 patients (50%). Mean pathologic diameter was 72.14mm. Wide surgical excision was performed in 27 primary cases, out of which 10 cases (37%) were full- and 17 cases (63%) partial-thickness chest wall resections. Synthetic mesh stabilization was used in nine cases and soft tissue coverage in six patients. Morbidity rate was 25%. Recurrences were found in 63% of the cases, with a mean time of 30.5 months to first recurrences. Our investigation confirmed that microscopically free surgical margin of the first tumor resection significantly affected local tumor control.

Conclusions: Because the radicality of the first surgical resection is of essential importance for long-time local control of chest desmoids, accurate preoperative diagnostics and well-planned aggressive surgical resection of the primary tumor is recommended. Due to the low incidence of desmoids, multicentric randomized investigations would be mandatory to establish evidence-based protocol for desmoid tumors.

Publication types

  • Multicenter Study
  • Review

MeSH terms

  • Adolescent
  • Adult
  • Biomarkers, Tumor / analysis
  • Child
  • Combined Modality Therapy
  • Epidemiologic Methods
  • Female
  • Fibromatosis, Aggressive / pathology
  • Fibromatosis, Aggressive / therapy*
  • Humans
  • Male
  • Middle Aged
  • Neoplasm Recurrence, Local
  • Neoplasm, Residual
  • Plastic Surgery Procedures / methods
  • Postoperative Complications
  • Receptors, Estrogen / analysis
  • Surgical Flaps
  • Thoracic Neoplasms / pathology
  • Thoracic Neoplasms / therapy*
  • Treatment Outcome

Substances

  • Biomarkers, Tumor
  • Receptors, Estrogen