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(1):249. DOI: 10.1186/1752-1947-5-249
Source: PubMed


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.

Download full-text


Available from: Issam Lalya
  • [Show abstract] [Hide abstract]
    ABSTRACT: Introduction Minor breast concerns in childhood and adolescence are common and lead to increased anxiety among young patients and their families, particularly due to high connotations with breast cancer. However, most breast services aim at managing adults and triaging patients with breast cancer, whereas adolescent medicine specialists or paediatricians are usually not appropriately trained to identify and treat breast pathology. Methods We reviewed hospital records of all patients attending a pediatric and adolescent gynaecology or breast clinic of a tertiary referral hospital, with a breast related symptom between January 2009 and December 2011. We collected information regarding age at presentation, age at menarche, diagnosis, management and outcome. Results We identified 81 patients of which 11 presented with an abnormal nipple or areolar secretion, 33 had a palpable lump, 20 had mastitis and 16 had unequal breast development. One patient presented with virginal breast hypertrophy. 3 out of 11 the patients with an abnormal secretion had a cyst identified on ultrasound. Out of the palpable lumps 12 were fibroadenomas, 3 were phylloeides tumours and 14 were cystic in nature. The phylloeides tumours and half of the fibroadenomas were removed. The remaining fibroadenomas remain under regular ultrasound follow up. All cases of mastitis were treated conservatively and resolved with broad spectrum antibiotic treatment. Conclusion In our series, no malignancies were identified. Although 8 patients required surgical treatment, the majority of cases were treated conservatively.
    No preview · Article · Jun 2014 · Journal of Pediatric and Adolescent Gynecology
  • Source
    [Show abstract] [Hide abstract]
    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.
    Full-text · Article · Sep 2014 · Oncology letters
  • Source

    Full-text · Article · Dec 2014 · The Korean Journal of Pathology
Show more