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| __NOTOC__
| | #REDIRECT [[Breast cancer]] |
| {{SI}}
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| {{CMG}} {{AE}} {{MV}}
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| {{SK}} Cystosarcoma phyllodes; Cystosarcoma phylloides; Phyllodes tumours
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| ==Overview==
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| '''Phyllodes tumor''' (also known as ''cystosarcoma phyllodes''), is a typically large, fast-growing mass that arises from the periductal [[stromal cell]]s of the [[breast]]. Phyllodes tumors account for less than 1% of all breast [[Neoplasia|neoplasms]]. Phyllodes tumor was first discovered by Johannes Muller, a German physician in 1838. Phyllodes tumor may be classified according to histological grade into 3 subtypes: benign, borderline, and malignant. The pathogenesis of phyllodes tumor is characterized by the overgrowth of [[stromal cells]]. Genes involved in the development of phyllodes tumor, include:
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| [[p53]] gene, [[EGFR]] gene, and IGF1R gene. There are no established causes for phyllodes tumor. Phyllodes tumors are rare, and approximately 6% of all phyllodes tumors are malignant. The diagnosis of phyllodes tumor is preferably made with core needle biopsy. The majority of patients with phyllodes tumor remain asymptomatic for years. The most important early clinical feature is a rapid growing palpable mass. [[Mammography]] is the imaging modality of choice for phyllodes tumor, characteristic findings include: large rounded oval or lobulated mass, well circumscribed, and smooth margins. [[Wide local excision]] is the most common approach to the treatment of phyllodes tumor.<ref name="radio"> Phyllodes tumor. Dr Henry Knipe. Radiopedia. http://radiopaedia.org/articles/phyllodes-tumour Accessed on May 19, 2016</ref>
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| ==Historical Perspective==
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| *Phyllodes tumor was first discovered by Johannes Muller, a German physician in 1838.<ref name="wiki"> Phyllodes tumor. Wikipedia. https://en.wikipedia.org/wiki/Phyllodes_tumor </ref>
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| ==Classification==
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| *Phyllodes tumor may be classified according to histological grade into 3 subtypes:<ref name="wiki"> Phyllodes tumor. Wikipedia. https://en.wikipedia.org/wiki/Phyllodes_tumor </ref>
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| :*Benign (most common)
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| :*Borderline
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| :*Malignant
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| ==Pathophysiology==
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| *The pathogenesis of phyllodes tumor is characterized by the overgrowth of stromal cells.<ref name="radio"> Phyllodes tumor. Dr Henry Knipe. Radiopedia. http://radiopaedia.org/articles/phyllodes-tumour Accessed on May 19, 2016</ref>
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| *Phyllodes tumor arises from the periductal [[stromal cell]]s of the [[breast]], which are normally involved in the supportive function of the parenchymal tissue.<ref name="radio"> Phyllodes tumor. Dr Henry Knipe. Radiopedia. http://radiopaedia.org/articles/phyllodes-tumour Accessed on May 19, 2016</ref>
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| *Genes involved in the development of phyllodes tumor, include:<ref name="radio"> Phyllodes tumor. Dr Henry Knipe. Radiopedia. http://radiopaedia.org/articles/phyllodes-tumour Accessed on May 19, 2016</ref>
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| :*[[p53 gene]]
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| :*[[EGFR|EGFR gene]]
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| :*[[Insulin-like growth factor-I|IGF1R gene]]
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| *On gross pathology, characteristic findings of phyllodes tumor, include:
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| :*Cleft/leaf-like structures
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| :*Friable mass
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| :*Well-defined margins
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| *On microscopic histopathological analysis,characteristic findings of phyllodes tumor, include:
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| :*Large slit-like spaces
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| :*Cellular myxoid stroma
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| :*Infiltrative border
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| :*Stromal overgrowth
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| ==Causes==
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| * There are no established causes for phyllodes tumor.<ref name="wiki"> Phyllodes tumor. Wikipedia. https://en.wikipedia.org/wiki/Phyllodes_tumor </ref>
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| ==Differentiating Phyllodes Tumor from Other Diseases==
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| *Phyllodes tumor must be differentiated from other diseases that cause a breast mass, such as:<ref name="wiki"> Phyllodes tumor. Wikipedia. https://en.wikipedia.org/wiki/Phyllodes_tumor </ref>
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| :*[[Fibroadenoma|Juvenile fibroadenoma]] (most common)
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| :*[[Breast abscess]]
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| :*[[Adenocarcinoma]]
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| :*[[Mastitis]]
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| ==Epidemiology and Demographics==
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| *The prevalence of phyllodes tumor is approximately 0.05 per 100,000 individuals worldwide.<ref name="radio"> Phyllodes tumor. Dr Henry Knipe. Radiopedia. http://radiopaedia.org/articles/phyllodes-tumour Accessed on May 19, 2016</ref>
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| *Approximately 6% of all phyllodes tumors are malignant.
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| *Phyllodes tumor accounts for less than 0.3-1% of all breast neoplasms.<ref name="radio"> Phyllodes tumor. Dr Henry Knipe. Radiopedia. http://radiopaedia.org/articles/phyllodes-tumour Accessed on May 19, 2016</ref>
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| ===Age===
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| *Patients of all age groups may develop phyllodes tumor.
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| *However, phyllodes tumor is more commonly reported in adult women between 40 and 60 years.<ref name="wiki"> Phyllodes tumor. Wikipedia. https://en.wikipedia.org/wiki/Phyllodes_tumor </ref>
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| ===Gender===
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| *Females are more commonly affected with phyllodes tumor than males.
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| ===Race===
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| *Latina-whites are more commonly affected with phyllodes tumor.
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| ==Risk Factors==
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| *There are no risk factors associated in the development of phyllodes tumor.<ref name="wiki"> Phyllodes tumor. Wikipedia. https://en.wikipedia.org/wiki/Phyllodes_tumor </ref>
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| *In some cases, patients with Li-Fraumeni syndrome may have an increased risk.
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| == Natural History, Complications and Prognosis==
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| *The majority of patients with phyllodes tumor remain asymptomatic for years.<ref name="radio"> Phyllodes tumor. Dr Henry Knipe. Radiopedia. http://radiopaedia.org/articles/phyllodes-tumour Accessed on May 19, 2016</ref>
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| *The most important early clinical feature is a rapid growing palpable mass.
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| *If left untreated, patients with phyllodes tumor may progress to develop malignant degeneration.
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| *Complications of phyllodes tumor are usually related to surgery (mastectomy).<ref name="wiki"> Phyllodes tumor. Wikipedia. https://en.wikipedia.org/wiki/Phyllodes_tumor </ref>
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| *Common complications, include:<ref name="radio"> Phyllodes tumor. Dr Henry Knipe. Radiopedia. http://radiopaedia.org/articles/phyllodes-tumour Accessed on May 19, 2016</ref>
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| :*[[Infection]]
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| :*[[Seroma|Seroma formation]]
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| :*Local or distant recurrence
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| *Prognosis is generally good, and the 5 year survival rate of patients with phyllodes tumor is approximately 60 to 80%.<ref name="wiki"> Phyllodes tumor. Wikipedia. https://en.wikipedia.org/wiki/Phyllodes_tumor </ref>
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| == Diagnosis ==
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| ===Diagnostic Criteria===
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| *The diagnosis of phyllodes tumor is preferably made with core needle biopsy.<ref name="radio"> Phyllodes tumor. Dr Henry Knipe. Radiopedia. http://radiopaedia.org/articles/phyllodes-tumour Accessed on May 19, 2016</ref>
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| === Symptoms ===
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| *Phyllodes tumor is usually asymptomatic.<ref name="wiki"> Phyllodes tumor. Wikipedia. https://en.wikipedia.org/wiki/Phyllodes_tumor </ref>
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| *There are no remarkable symptoms of phyllodes tumor.
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| *A directed history should be obtained to ascertain:
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| :*Family history of breast malignancy
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| === Physical Examination ===
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| *Patients with phyllodes tumor are usually well-appearing.
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| *Breast physical examination may be remarkable for:
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| *Breast mass, with the following characteristics:<ref name="wiki"> Phyllodes tumor. Wikipedia. https://en.wikipedia.org/wiki/Phyllodes_tumor </ref>
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| :*Firm
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| :*Mobile
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| :*Well-circumscribed
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| :*Non-tender
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| === Laboratory Findings ===
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| *There are no specific laboratory findings associated with phyllodes tumor.
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| ===Imaging Findings===
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| *Mammography is the imaging modality of choice for phyllodes tumor.<ref name="radio"> Phyllodes tumor. Dr Henry Knipe. Radiopedia. http://radiopaedia.org/articles/phyllodes-tumour Accessed on May 19, 2016</ref>
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| *On mammography, findings of phyllodes tumor include:<ref name="wiki"> Phyllodes tumor. Wikipedia. https://en.wikipedia.org/wiki/Phyllodes_tumor </ref>
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| :*Non specific large rounded oval or lobulated mass
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| :*Generally well circumscribed
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| :*Lesions with smooth margins
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| :*A radiolucent halo may be present
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| :*Calcification (typically coarse and plaque like) may be seen in a very small proportion.
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| <gallery>
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| Image: Phyllodes-tumour.jpg|Mammography of phyllodes tumor <br> <SMALL> Adapted from Radiopedia </SMALL>
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| </gallery>
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| *On ultrasound, findings of phyllodes tumor include:<ref name="radio"> Phyllodes tumor. Dr Henry Knipe. Radiopedia. http://radiopaedia.org/articles/phyllodes-tumour Accessed on May 19, 2016</ref>
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| :*Inhomogeneous
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| :*Solid-appearing mass
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| :*Solid mass containing single or multiple, round or cleft like cystic spaces
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| :*Posterior acoustic enhancement strongly suggests the diagnosis of phyllodes tumor
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| :*Vascularisation is usually present in the solid components
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| *On MRI, findings of phyllodes tumor include:
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| :*T1: usually of low signal
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| :*T2: can be variable ranging from homogenous low 8 to high 4-5 signal
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| :*T1 C+ (Gd): the solid components enhance after contrast administration
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| :*Dynamic contrast: the kinetic curve pattern can be gradual slow or have rapid enhancement
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| == Treatment ==
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| === Medical Therapy ===
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| *There is no medical therapy for phyllodes tumor.
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| *Existing medical therapies, such as hormonal therapy and chemotherapy are proven to be ineffective among patients with phyllodes tumor.
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| === Surgery ===
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| *Surgery is the mainstay of therapy for phyllodes tumor.<ref name="radio"> Phyllodes tumor. Dr Henry Knipe. Radiopedia. http://radiopaedia.org/articles/phyllodes-tumour Accessed on May 19, 2016</ref>
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| *Wide local excision is the most common approach to the treatment of phyllodes tumor.
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| *The risk of developing local recurrence or metastases among patients with phyllodes tumor is related to the histologic grade.
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| === Prevention ===
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| *Effective measures for the secondary prevention of phyllodes tumor include periodical self-breast examination, and routine mammography.
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| *Once diagnosed and successfully treated, patients with phyllodes tumor are followed-up periodically.
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| *Follow-up testing, includes: ultrasound examination, breast exam, and mammography.
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| ==References==
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| {{Reflist|2}}
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| [[Category:Oncology]]
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| [[Category:Up-To-Date]]
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| [[Category:Oncology]]
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| [[Category:Medicine]]
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| [[Category:Gynecology]]
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