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| __NOTOC__
| | #REDIRECT [[Breast cancer]] |
| {{SI}}
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| {{CMG}} {{AE}} {{MV}}
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| {{SK}} Cystosarcoma phyllodes;
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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]].
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| ==Historical Perspective==
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| *Phyllodes tumor was first discovered by Johannes Muller, a German physician in 1838.
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| ==Classification==
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| *Phyllodes tumor may be classified according to histological grade into 3 subtypes:
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| :*Benign
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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.
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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.
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| *Genes involved in the development of phyllodes tumor, include:
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| :*p53 gene
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| :*EGFR gene
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| :*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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| *The image below demonstrates gross pathology of phyllodes tumor.
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| <gallery>
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| Image: |
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| </gallery>
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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.
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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:
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| :*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.
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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
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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.
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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.
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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.
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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).
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| *Common complications, include:
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| :*Infection
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| :*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%
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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.
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| === Symptoms ===
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| *Phyllodes tumor is usually asymptomatic.
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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:
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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.
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| *On mammography, findings of phyllodes tumor include:
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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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| *On ultrasound, findings of phyllodes tumor include:
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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.
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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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