Article

Periductal stromal sarcoma in a child: a case report.

Department of Radiotherapy, National Institute of Oncology, Allal fassi Street, Rabat 10100, Morocco. .
Journal of Medical Case Reports 06/2011; 5:249. DOI: 10.1186/1752-1947-5-249
Source: PubMed

ABSTRACT Periductal stromal sarcoma is an extremely rare malignant fibroepithelial tumor of the breast which is characterized by its biphasic histology with benign ductal elements and a sarcomatous stroma made of spindle cells and lacking phyllodes architecture. Its therapeutic management is based on wide surgery with free margins. Adjuvant therapies are not needed. Periductal stromal sarcoma may evolve into a phyllodes tumor with time, as well as a specific soft-tissue sarcoma. To the best of our knowledge, this tumor has never been described in a child.
A 14-year-old Arabic boy was presented to our hospital one year ago with a nodule of the right breast that was gradually increasing in size without signs of inflammation. The histological examination after lumpectomy revealed a periductal stromal sarcoma with free surgical margins. No adjuvant treatment was given. At 50 months of close follow-up, no recurrence was observed.
Periductal stromal sarcoma in a child is a very rare disease which has the same indolent behavior as it does in adults. Therefore, close follow-up is required.

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    ABSTRACT: Periductal stromal sarcoma (PSS), spindle and epithelioid types, is a rare subtype of malignant fibroepithelial tumor. The morphological characteristics of this neoplasm are different from phyllodes tumor and stromal sarcoma. PSS exhibits biphasic histology with benign ductal elements and a sarcomatous stroma composed of spindle cells and lacking phyllodes tumor architecture. The therapeutic management of PSS is based on wide surgery with free margins, and adjuvant therapies are not required. To the best of our knowledge, the recurrence of PSS in ≤5 months has not been reported in the literature to date. This report describes a 43-year-old woman who presented to our hospital with a recurrence of nodules in the left breast. The patient had undergone lumpectomy at a different hospital 5 months previously, and a diagnosis of phyllodes tumor was pathologically confirmed. On presentation at our hospital, the patient underwent a second lumpectomy. Histological examination revealed PSS and the patient underwent a simple mastectomy of the left breast with no adjuvant treatment (such as chemotherapy or radiotherapy). After 9 months of close follow-up examinations, no recurrence was observed. PSS is an extremely rare disease with low-grade sarcomatous behavior, which may evolve into a phyllodes tumor or an entity of breast cancer. Therefore, frequent follow-up examinations are required.
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