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

  • There is no racial predilection for phyllodes tumor.

Risk Factors

  • Common risk factors in the development of phyllodes tumor, include:

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

  • [Imaging study 1] is the imaging modality of choice for phyllodes tumor.
  • On [imaging study 1], findings of phyllodes tumor include:

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.

References