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Periductal stromal sarcoma in a child: a case report.

Masbah O, Lalya I, Mellas N, Bekkouch I, Allaoui M, Hassouni K, Kebdani T, Regragui A, Benjaafar N, Elgueddari BK - J Med Case Rep (2011)

Bottom Line: To the best of our knowledge, this tumor has never been described in a child.At 50 months of close follow-up, no recurrence was observed.Therefore, close follow-up is required.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Radiotherapy, National Institute of Oncology, Allal fassi Street, Rabat 10100, Morocco. issamlalya@yahoo.fr.

ABSTRACT

Introduction: 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.

Case presentation: 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.

Conclusion: 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.

No MeSH data available.


Related in: MedlinePlus

Stromal periductal proliferation arranged within fat tissue (hematoxylin and eosin stain; original magnification, × 50).
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Figure 1: Stromal periductal proliferation arranged within fat tissue (hematoxylin and eosin stain; original magnification, × 50).

Mentions: We report the clinical case of a 14-year-old Arabic boy with no history of disease who was presented to our hospital one year ago with a nodule of the right breast that was gradually increasing in size. Upon clinical examination, we found, in the upper outer quadrant of the right breast, a small mass measuring approximately 2cm in size, round in shape, with no signs of inflammation and not associated with axillary lymph nodes. He underwent a lumpectomy. Grossly, the tumor was well circumscribed, nodular, and well delineated and measured 1.5 cm × 1 cm. Microscopic examination revealed no leafy architecture, but we found a biphasic proliferation composed of epithelial and mesenchymal components. The epithelial component corresponded to ducts with borders, sometimes double and sometimes with mild to moderate hyperplasia without atypia. These ductal structures were often dilated and surrounded by a spindle-cell mesenchymal tumor which showed moderate cellular density and mitotic activity of about two to three mitoses/10 high-power fields (Figure 1, Figure 2 and Figure 3). These mesenchymal cells showed moderately hyperchromatic nuclei of variable sizes, sometimes with distinct nucleoli. The closest distance between the tumor and resection margins ranged from 2mm to 13mm. Immunohistochemistry showed the tumor cells to be CD34-positive (Figure 4) and S-100-, ER- and PR-negative. The lesion was histologically compatible with low-grade PSS. The resection margins were negative (range, 2mm to 13mm). No adjuvant treatment was given. In a follow-up period of 50 months, the patient did not show any symptoms or signs of local or distant recurrence.


Periductal stromal sarcoma in a child: a case report.

Masbah O, Lalya I, Mellas N, Bekkouch I, Allaoui M, Hassouni K, Kebdani T, Regragui A, Benjaafar N, Elgueddari BK - J Med Case Rep (2011)

Stromal periductal proliferation arranged within fat tissue (hematoxylin and eosin stain; original magnification, × 50).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3141703&req=5

Figure 1: Stromal periductal proliferation arranged within fat tissue (hematoxylin and eosin stain; original magnification, × 50).
Mentions: We report the clinical case of a 14-year-old Arabic boy with no history of disease who was presented to our hospital one year ago with a nodule of the right breast that was gradually increasing in size. Upon clinical examination, we found, in the upper outer quadrant of the right breast, a small mass measuring approximately 2cm in size, round in shape, with no signs of inflammation and not associated with axillary lymph nodes. He underwent a lumpectomy. Grossly, the tumor was well circumscribed, nodular, and well delineated and measured 1.5 cm × 1 cm. Microscopic examination revealed no leafy architecture, but we found a biphasic proliferation composed of epithelial and mesenchymal components. The epithelial component corresponded to ducts with borders, sometimes double and sometimes with mild to moderate hyperplasia without atypia. These ductal structures were often dilated and surrounded by a spindle-cell mesenchymal tumor which showed moderate cellular density and mitotic activity of about two to three mitoses/10 high-power fields (Figure 1, Figure 2 and Figure 3). These mesenchymal cells showed moderately hyperchromatic nuclei of variable sizes, sometimes with distinct nucleoli. The closest distance between the tumor and resection margins ranged from 2mm to 13mm. Immunohistochemistry showed the tumor cells to be CD34-positive (Figure 4) and S-100-, ER- and PR-negative. The lesion was histologically compatible with low-grade PSS. The resection margins were negative (range, 2mm to 13mm). No adjuvant treatment was given. In a follow-up period of 50 months, the patient did not show any symptoms or signs of local or distant recurrence.

Bottom Line: To the best of our knowledge, this tumor has never been described in a child.At 50 months of close follow-up, no recurrence was observed.Therefore, close follow-up is required.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Radiotherapy, National Institute of Oncology, Allal fassi Street, Rabat 10100, Morocco. issamlalya@yahoo.fr.

ABSTRACT

Introduction: 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.

Case presentation: 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.

Conclusion: 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.

No MeSH data available.


Related in: MedlinePlus