Phyllodes tumor

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Maria Fernanda Villarreal, M.D. [2]

Synonyms and keywords: Cystosarcoma phyllodes;

Overview

Phyllodes tumor (also known as cystosarcoma phyllodes), is a typically large, fast-growing mass that arises from the periductal stromal cells of the breast. Phyllodes tumors account for less than 1% of all breast neoplasms.

Historical Perspective

• Phyllodes tumor was first discovered by Johannes Muller, a German physician in 1838.

Classification

• Phyllodes tumor may be classified according to histological grade into 3 subtypes:

• Benign
• Borderline
• Malignant

Pathophysiology

• The pathogenesis of phyllodes tumor is characterized by the overgrowth of stromal cells.
• Phyllodes tumor arises from the periductal stromal cells of the breast, which are normally involved in the supportive function of the parenchymal tissue.
• Genes involved in the development of phyllodes tumor, include:

• p53 gene
• EGFR gene
• IGF1R gene

• On gross pathology, characteristic findings of phyllodes tumor, include:

• Cleft/leaf-like structures
• Friable mass
• Well-defined margins

• The image below demonstrates gross pathology of phyllodes tumor.

• On microscopic histopathological analysis,characteristic findings of phyllodes tumor, include:

• Large slit-like spaces
• Cellular myxoid stroma
• Infiltrative border
• Stromal overgrowth

Causes

• There are no established causes for phyllodes tumor.

Differentiating Phyllodes Tumor from Other Diseases

• Phyllodes tumor must be differentiated from other diseases that cause a breast mass, such as:

• Juvenile fibroadenoma
• Breast abscess
• Adenocarcinoma
• Mastitis

Epidemiology and Demographics

• The prevalence of phyllodes tumor is approximately 0.05 per 100,000 individuals worldwide.
• Approximately 6% of all phyllodes tumors are malignant.
• Phyllodes tumor accounts for less than 0.3-1% of all breast neoplasms

Age

• Patients of all age groups may develop phyllodes tumor.
• However, phyllodes tumor is more commonly reported in adult women between 40 and 60 years.

Gender

• Females are more commonly affected with phyllodes tumor than males.

Race

• Latina-whites are more commonly affected with phyllodes tumor.

Risk Factors

• There are no risk factors associated in the development of phyllodes tumor.
• In some cases, patients with Li-Fraumeni syndrome may have an increased risk.

Natural History, Complications and Prognosis

• The majority of patients with phyllodes tumor remain asymptomatic for years.
• The most important early clinical feature is a rapid growing palpable mass.
• If left untreated, the minority of patients with phyllodes tumor may progress to develop metastases.
• Complications of phyllodes tumor are usually related to surgery (mastectomy).
• Common complications, include:

• Infection
• Seroma formation
• Local or distant recurrence

• Prognosis is generally poor, and the 5 year survival rate of patients with phyllodes tumor is approximately

Diagnosis

Diagnostic Criteria

• The diagnosis of phyllodes tumor is made with biopsy.

Symptoms

• Phyllodes tumor is usually asymptomatic.
• There are no remarkable symptoms of phyllodes tumor.

Physical Examination

• Patients with phyllodes tumor usually appear [general appearance].
• Breast physical examination may be remarkable for:
• Breast mass, with the following characteristics:

• Firm
• Mobile
• Well-circumscribed
• Non-tender

Laboratory Findings

• There are no specific laboratory findings associated with phyllodes tumor.

Imaging Findings

• Mammography is the imaging modality of choice for phyllodes tumor.
• On mammography, findings of phyllodes tumor include:

• Non specific large rounded oval or lobulated mass
• Generally well circumscribed
• Lesions with smooth margins
• A radiolucent halo may be present
• Calcification (typically coarse and plaque like) may be seen in a very small proportion

• On ultrasound, findings of phyllodes tumor include:

• Inhomogeneous
• Solid-appearing mass
• Solid mass containing single or multiple, round or cleft like cystic spaces
• Posterior acoustic enhancement strongly suggests the diagnosis of phyllodes tumor
• Vascularisation is usually present in the solid components

Treatment

Medical Therapy

• There is no medical therapy for phyllodes tumor.

Surgery

• Surgery is the mainstay of therapy for phyllodes tumor.
• Wide local excision is the most common approach to the treatment of phyllodes tumor.

Prevention

• Effective measures for the primary prevention of phyllodes tumor include periodical self-breast examination, and routine mammography.
• Once diagnosed and successfully treated, patients with phyllodes tumor are followed-up periodically.
• Follow-up testing, includes: ultrasound examination.