Pleomorphic adenoma

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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: Pleomorphic adenoma of the salivary glands; BMT; Benign mixed tumors of the salivary gland

Overview

Pleomorphic adenoma (also known as "Benign mixed tumor of the salivary gland") is a benign neoplastic tumor of the salivary glands. Pleomorphic adenoma is the most common type of salivary gland tumor and the most common tumor of the parotid gland.[1]

Historical Perspective

  • Pleomorphic adenoma was first discovered by Beahrs in in 1957.


Classification

  • Pleomorphic adenoma may be classified according to [classification method] into [number] subtypes/groups:
  • [group1]
  • [group2]
  • [group3]
  • Other variants of pleomorphic adenoma include [disease subtype 1], [disease subtype 2], and [disease subtype 3].

Pathophysiology

  • The pathogenesis of pleomorphic adenoma is characterized by [feature1], [feature2], and [feature3].
  • The [gene name] gene/Mutation in [gene name] has been associated with the development of pleomorphic adenoma, involving the [molecular pathway] pathway.
  • On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of pleomorphic adenoma.
  • On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of pleomorphic adenoma.

Causes

  • Common causes of pleomorphic adenoma, include:


Differentiating Pleomorphic Adenoma from Other Diseases

  • Pleomorphic adenoma must be differentiated from other diseases that cause [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as:
  • [Differential dx1]
  • [Differential dx2]
  • [Differential dx3]

Epidemiology and Demographics

  • The prevalence of pleomorphic adenoma is approximately [number or range] per 100,000 individuals worldwide.
  • In [year], the incidence of pleomorphic adenoma was estimated to be [number or range] cases per 100,000 individuals in [location].

Age

  • Pleomorphic adenoma is more commonly observed among patients aged [age range] years old.
  • Pleomorphic adenoma is more commonly observed among [elderly patients/young patients/children].

Gender

  • Pleomorphic adenoma affects men and women equally.
  • [Gender 1] are more commonly affected with pleomorphic adenoma than [gender 2].
  • The [gender 1] to [Gender 2] ratio is approximately [number > 1] to 1.

Race

  • There is no racial predilection for pleomorphic adenoma.

Risk Factors

  • Common risk factors in the development of pleomorphic adenoma, include:

Natural History, Complications and Prognosis

  • The majority of patients with pleomorphic adenoma remain asymptomatic for [duration/years].
  • Early clinical features include [manifestation 1], [manifestation 2], and [manifestation 3].
  • If left untreated, the majority of patients with pleomorphic adenoma may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
  • Common complications of pleomorphic adenoma include [complication 1], [complication 2], and [complication 3].
  • Prognosis is generally [excellent/good/poor], and the [1/5/10­year mortality/survival rate] of patients with pleomorphic adenoma is approximately [#%].

Diagnosis

Diagnostic Criteria

  • The diagnosis of pleomorphic adenoma is made when at least [number] of the following [number] diagnostic criteria are met:
  • [criterion 1]
  • [criterion 2]
  • [criterion 3]
  • [criterion 4]

Symptoms

  • Pleomorphic adenoma is usually asymptomatic.
  • Symptoms of pleomorphic adenoma may include the following:
  • [symptom 1]
  • [symptom 2]
  • [symptom 3]
  • [symptom 4]
  • [symptom 5]
  • [symptom 6]

Physical Examination

  • Patients with pleomorphic adenoma usually appear [general appearance].
  • Physical examination may be remarkable for:
  • [finding 1]
  • [finding 2]
  • [finding 3]
  • [finding 4]


Laboratory Findings

  • There are no specific laboratory findings associated with pleomorphic adenoma.


Imaging Findings

  • MRI is the imaging modality of choice for pleomorphic adenoma.
  • On CT, characteristic findings of pleomorphic adenoma, include:
  • Smooth, and well-marginated tumors
  • The attenuation values of the mass are usually homogeneous and higher than that of the surrounding gland
  • Tumor enhancement is variable and can result in a missed diagnosis if delayed images are not acquired
  • Pleomorphic adenomas are poorly enhancing in the early phase of contrast enhancement, though the amount of enhancement increases over time).
  • On MRI, characteristic findings of pleomorphic adenoma, include:
  • Pleomorphic adenomas are usually well-circumscribed
  • Homogeneous masses with low intensity on T1-weighted images and high intensity on T2-weighted images.
  • They commonly have a rim of decreased signal intensity on T2-weighted images.
  • After gadolinium enhancement, the tumors are homogeneously enhancing unless they are large.

Gallery

Other Diagnostic Studies

Treatment

Medical Therapy

  • There is no treatment for pleomorphic adenoma; the mainstay of therapy is surgery.
  • In some cases, patients with pleomorphic adenoma can receive medications to stimulate saliva production, such as:
  • Pilocarpine
  • Cevimeline

Surgery

  • Surgery is the mainstay of therapy for pleomorphic adenoma.
  • Surgical excision in conjunction with pre-surgical biopsy is the most common approach to the treatment of pleomorphic adenoma.

Prevention

  • There are no primary preventive measures available for pleomorphic adenoma.
  • Once diagnosed and successfully treated, patients with pleomorphic adenoma are followed-up every 6 or 12 months.
  • Follow-up testing includes [test 1], [test 2], and [test 3].


References

  1. Ewa J. Bialek, Wieslaw Jakubowski, Piotr Zajkowski, Kazimierz T. Szopinski, and Antoni Osmolski. US of the Major Salivary Glands: Anatomy and Spatial Relationships, Pathologic Conditions, and Pitfalls. RadioGraphics 2006 26: 745-763.