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Peripheral Neural Sheath Breast Sarcoma: Case Report and Literature Review.

Yency Johana Forero1, Martín Ignacio Zapata Laguado2, Ximena Briceño3

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Case Reports in Oncology
|June 15, 2022
PubMed
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Malignant peripheral nerve sheath tumors are rare breast sarcomas. This case highlights a large tumor in a young woman, treated with mastectomy, chest wall reconstruction, and adjuvant therapy, emphasizing a multidisciplinary approach.

Keywords:
Breast neoplasmsNeurofibrosarcomaSoft tissue sarcoma

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Area of Science:

  • Oncology
  • Surgical Oncology
  • Pathology

Background:

  • Primary breast sarcomas are rare, heterogeneous neoplasms originating from non-epithelial breast tissue.
  • Malignant peripheral nerve sheath tumors (MPNSTs) constitute 5-10% of all soft tissue sarcomas, with a low incidence of 1 in 100,000.
  • The rarity and heterogeneity of MPNSTs limit prospective studies, leading to treatment standards based on case reports and retrospective analyses.

Observation:

  • A 36-year-old woman presented with a rapidly growing left breast mass, measuring 20x20 cm over one year.
  • Histopathological analysis confirmed a breast sarcoma with positive SOX-10 and S-100 staining.
  • The patient underwent radical mastectomy with chest wall resection and reconstruction using a latissimus dorsi flap.

Findings:

  • The patient received adjuvant radiotherapy (RT) and chemotherapy following surgical treatment.
  • Treatment strategies for MPNST of the breast show significant heterogeneity in surgical procedures and adjuvant therapies.
  • The case underscores the importance of a multidisciplinary team in managing this rare malignancy.

Implications:

  • This case contributes to the limited evidence base for managing malignant peripheral nerve sheath tumors of the breast.
  • The findings emphasize the need for individualized treatment plans tailored by a multidisciplinary team.
  • Further research, potentially through collaborative efforts, is needed to establish clearer treatment guidelines for this rare condition.