A Scary Onset of a Rare and Aggressive Type of Primary Breast Sarcoma: A Case Report
Identifieur interne : 008C27 ( Ncbi/Merge ); précédent : 008C26; suivant : 008C28A Scary Onset of a Rare and Aggressive Type of Primary Breast Sarcoma: A Case Report
Auteurs : Inês Ramalho ; Sara Campos ; Teresa Rebelo ; Margarida Figueiredo DiasSource :
- Case Reports in Oncology [ 1662-6575 ] ; 2016.
Abstract
Primary breast sarcoma, arising from connective tissue within the breast, is extremely rare, accounting for less than 1% of all primary breast malignancies and no more than 5% of all sarcomas. The rarity of this pathology limits most studies to case reports and small retrospective studies, which has led to a lack of consensus on the clinical management. We report a clinical case of a 52-year-old woman, perimenopausal, previously healthy, with regular breast surveillance, who presented with a large (>20 cm) and rapidly expanding hypervascularized tumor of the left breast developed over 10 days, with a very thin preulcerative skin over the last 4 days. There was no systemic dissemination. The patient was submitted to total mastectomy and excision of axillary adenopathy. The tumor was diagnosed histologically as malignant phyllodes tumor associated with areas of high-grade sarcoma. Due to rapid growth and aggressive histological characteristics, adjuvant chemotherapy and radiotherapy were performed. There is a lot of evidence that tumors larger than 5 cm are associated with a poor prognosis. Despite the poor prognosis associated with this aggressive entity, the patient had no recurrence during 5 years of follow-up. We review the relevant literature about primary breast sarcomas.
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DOI: 10.1159/000452946
PubMed: 28101028
PubMed Central: 5216215
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<author><name sortKey="Rebelo, Teresa" sort="Rebelo, Teresa" uniqKey="Rebelo T" first="Teresa" last="Rebelo">Teresa Rebelo</name>
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<author><name sortKey="Figueiredo Dias, Margarida" sort="Figueiredo Dias, Margarida" uniqKey="Figueiredo Dias M" first="Margarida" last="Figueiredo Dias">Margarida Figueiredo Dias</name>
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<front><div type="abstract" xml:lang="en"><p>Primary breast sarcoma, arising from connective tissue within the breast, is extremely rare, accounting for less than 1% of all primary breast malignancies and no more than 5% of all sarcomas. The rarity of this pathology limits most studies to case reports and small retrospective studies, which has led to a lack of consensus on the clinical management. We report a clinical case of a 52-year-old woman, perimenopausal, previously healthy, with regular breast surveillance, who presented with a large (>20 cm) and rapidly expanding hypervascularized tumor of the left breast developed over 10 days, with a very thin preulcerative skin over the last 4 days. There was no systemic dissemination. The patient was submitted to total mastectomy and excision of axillary adenopathy. The tumor was diagnosed histologically as malignant phyllodes tumor associated with areas of high-grade sarcoma. Due to rapid growth and aggressive histological characteristics, adjuvant chemotherapy and radiotherapy were performed. There is a lot of evidence that tumors larger than 5 cm are associated with a poor prognosis. Despite the poor prognosis associated with this aggressive entity, the patient had no recurrence during 5 years of follow-up. We review the relevant literature about primary breast sarcomas.</p>
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<front><journal-meta><journal-id journal-id-type="nlm-ta">Case Rep Oncol</journal-id>
<journal-id journal-id-type="iso-abbrev">Case Rep Oncol</journal-id>
<journal-id journal-id-type="publisher-id">CRO</journal-id>
<journal-title-group><journal-title>Case Reports in Oncology</journal-title>
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<issn pub-type="epub">1662-6575</issn>
<publisher><publisher-name>S. Karger AG</publisher-name>
<publisher-loc>Allschwilerstrasse 10, P.O. Box · Postfach · Case postale, CH–4009, Basel, Switzerland · Schweiz · Suisse, Phone: +41 61 306 11 11, Fax: +41 61 306 12 34, karger@karger.ch</publisher-loc>
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<article-id pub-id-type="publisher-id">cro-0009-0796</article-id>
<article-categories><subj-group subj-group-type="heading"><subject>Case Report</subject>
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<title-group><article-title>A Scary Onset of a Rare and Aggressive Type of Primary Breast Sarcoma: A Case Report</article-title>
</title-group>
<contrib-group><contrib contrib-type="author"><name><surname>Ramalho</surname>
<given-names>Inês</given-names>
</name>
<xref ref-type="corresp" rid="cor1">*</xref>
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<contrib contrib-type="author"><name><surname>Campos</surname>
<given-names>Sara</given-names>
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<contrib contrib-type="author"><name><surname>Rebelo</surname>
<given-names>Teresa</given-names>
</name>
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<contrib contrib-type="author"><name><surname>Figueiredo Dias</surname>
<given-names>Margarida</given-names>
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<aff>Gynecology A Department, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal</aff>
<author-notes><corresp id="cor1">*Inês Ramalho, Gynecology A Department, Centro Hospitalar e Universitário de Coimbra, Praceta Prof. Mota Pinto, PT-3000-075 Coimbra (Portugal), E-Mail <email>ines.silvaramalho@gmail.com</email>
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<pub-date pub-type="collection"><season>Sep-Dec</season>
<year>2016</year>
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<pub-date pub-type="epub"><day>25</day>
<month>11</month>
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<pmc-comment> PMC Release delay is 0 months and 0 days and was based on the . </pmc-comment>
<volume>9</volume>
<issue>3</issue>
<fpage>796</fpage>
<lpage>801</lpage>
<history><date date-type="received"><day>31</day>
<month>10</month>
<year>2016</year>
</date>
<date date-type="accepted"><day>31</day>
<month>10</month>
<year>2016</year>
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<permissions><copyright-statement>Copyright © 2016 the Author(s)</copyright-statement>
<copyright-year>2016</copyright-year>
<license license-type="open-access" xlink:href="http://creativecommons.org/licenses/by-nc/4.0/"><license-p>This article is licensed under the Creative Commons Attribution-NonCommercial-4.0 International License (CC BY-NC) (http://www.karger.com/Services/OpenAccessLicense). Usage and distribution for commercial purposes requires written permission.</license-p>
</license>
</permissions>
<abstract><p>Primary breast sarcoma, arising from connective tissue within the breast, is extremely rare, accounting for less than 1% of all primary breast malignancies and no more than 5% of all sarcomas. The rarity of this pathology limits most studies to case reports and small retrospective studies, which has led to a lack of consensus on the clinical management. We report a clinical case of a 52-year-old woman, perimenopausal, previously healthy, with regular breast surveillance, who presented with a large (>20 cm) and rapidly expanding hypervascularized tumor of the left breast developed over 10 days, with a very thin preulcerative skin over the last 4 days. There was no systemic dissemination. The patient was submitted to total mastectomy and excision of axillary adenopathy. The tumor was diagnosed histologically as malignant phyllodes tumor associated with areas of high-grade sarcoma. Due to rapid growth and aggressive histological characteristics, adjuvant chemotherapy and radiotherapy were performed. There is a lot of evidence that tumors larger than 5 cm are associated with a poor prognosis. Despite the poor prognosis associated with this aggressive entity, the patient had no recurrence during 5 years of follow-up. We review the relevant literature about primary breast sarcomas.</p>
</abstract>
<kwd-group><title>Key Words</title>
<kwd>Primary breast sarcoma</kwd>
<kwd>Malignant phyllodes tumor</kwd>
<kwd>Breast cancer</kwd>
</kwd-group>
<counts><fig-count count="3"></fig-count>
<ref-count count="14"></ref-count>
<page-count count="6"></page-count>
</counts>
</article-meta>
</front>
<floats-group><fig id="f1" orientation="portrait" position="float"><label>Fig. 1</label>
<caption><p>On admission, the patient presented an extremely voluminous tumor in the left breast (>20 cm), with bosselated contours, firm, and hypervascularized with soft cystic and hemorrhagic areas.</p>
</caption>
<graphic xlink:href="cro-0009-0796-g01"></graphic>
</fig>
<fig id="f2" orientation="portrait" position="float"><label>Fig. 2</label>
<caption><p>A few hours after admission, the breast started to ulcerate and bleed. Ulceration increased dramatically in 3 days.</p>
</caption>
<graphic xlink:href="cro-0009-0796-g02"></graphic>
</fig>
<fig id="f3" orientation="portrait" position="float"><label>Fig. 3</label>
<caption><p>Breast scar 5 years after diagnosis.</p>
</caption>
<graphic xlink:href="cro-0009-0796-g03"></graphic>
</fig>
</floats-group>
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<affiliations><list></list>
<tree><noCountry><name sortKey="Campos, Sara" sort="Campos, Sara" uniqKey="Campos S" first="Sara" last="Campos">Sara Campos</name>
<name sortKey="Figueiredo Dias, Margarida" sort="Figueiredo Dias, Margarida" uniqKey="Figueiredo Dias M" first="Margarida" last="Figueiredo Dias">Margarida Figueiredo Dias</name>
<name sortKey="Ramalho, Ines" sort="Ramalho, Ines" uniqKey="Ramalho I" first="Inês" last="Ramalho">Inês Ramalho</name>
<name sortKey="Rebelo, Teresa" sort="Rebelo, Teresa" uniqKey="Rebelo T" first="Teresa" last="Rebelo">Teresa Rebelo</name>
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