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Malignant Proliferating Pilar Tumor

Last updated March 29, 2021

Reviewed by: Lester Fahrner, MD

Approved by: Krish Tangella MD, MBA, FCAP

Malignant Proliferating Pilar Tumor (PPT) is a rare hair follicle tumor of skin that is generally observed in women during middle and older age.


What are the other Names for this Condition? (Also known as/Synonyms)

  • Malignant Pilar Tumor of Scalp
  • Malignant Proliferating Trichilemmal Tumor (MPTT)
  • Proliferating Pilar Tumor (PPT)

What is Malignant Proliferating Pilar Tumor? (Definition/Background Information)

  • Malignant Proliferating Pilar Tumor (PPT) is a rare hair follicle tumor of skin that is generally observed in women during middle and older age. It is also known as a Malignant Proliferating Trichilemmal Tumor/Cyst
  • This cyst is usually painless and solitary, and can be present anywhere in the body; however, the most common location is the scalp. In some cases, it can ulcerate at the top of the lesion
  • The cause and risk factors for Malignant Proliferating Pilar Tumor are unknown, but if multiple cysts are present on the body, then a familial inheritance may be observed
  • This cancerous tumor type may infiltrate local tissues, but only rarely metastasizes. There may be recurrence after removal of the Malignant PPT
  • In majority of cases, a complete surgical excision is the preferred mode of treatment for this malignant tumor. In rare cases of metastasis, a combination of chemotherapy, radiation therapy, and surgical procedures may be used
  • The outcome for an individual with Malignant Proliferating Pilar Tumor depends upon various factors, but is usually good

Who gets Malignant Proliferating Pilar Tumor? (Age and Sex Distribution)

  • Malignant Proliferating Pilar Tumor is a rare and malignant cyst that is more common in middle-aged and elderly adults
  • Both males and females may be affected, though there is a preference for females
  • Individuals of all racial and ethnic background may be affected. Worldwide, no geographical localization has been reported

What are the Risk Factors for Malignant Proliferating Pilar Tumor? (Predisposing Factors)

  • Currently, no risk factors have been identified for Malignant Proliferating Pilar Tumor
  • Nevertheless, a positive family history may influence the development of multiple cysts on the body

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Malignant Proliferating Pilar Tumor? (Etiology)

  • The cause of development of Malignant Proliferating Pilar Tumor is unknown. Research is currently being undertaken to identify the relevant causal factors
  • If numerous cysts appear all over the body, then it may be due to a family history of the condition

What are the Signs and Symptoms of Malignant Proliferating Pilar Tumor?

The signs and symptoms of Malignant Proliferating Pilar Tumors may include:

  • It usually occurs as a single cyst on the skin. It can range in size from 2-10 cm
  • The skin over the nodules may have a reddish discoloration
  • These cysts are usually painless and non-itchy. They are mostly well-demarcated and firm to touch
  • Though Malignant PPT can occur at any location in the body, it is commonly present on the scalp

Malignant Proliferating Pilar Tumor typically occurs as a single cyst that grows slowly. However, multiple cysts may also be seen, when a family history of the condition is present.

How is Malignant Proliferating Pilar Tumor Diagnosed?

A diagnosis of Malignant Proliferating Pilar Tumor may involve the following:

  • A thorough medical history and physical examination
  • Dermoscopy: It is a diagnostic tool where a dermatologist examines the skin using a special magnified lens
  • Wood’s lamp examination: In this procedure, the healthcare provider examines the skin using ultraviolet light. It is performed to examine the change in skin pigmentation
  • Skin biopsy: A skin biopsy is performed and sent to a laboratory for a pathological examination. The pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Malignant Proliferating Pilar Tumor?

Malignant Proliferating Pilar Tumor complications may include:

  • Sometimes, the cysts can proliferate and multiply. These cysts have the potential to infiltrate into the local tissues and destroy surrounding structures
  • The presence of multiple cysts may also result in cosmetic issues
  • Sometimes, they can rupture, become infected and release pus. When these heal, they can leave a scar behind
  • Metastasis of the tumor is rare, but it can occur
  • Sometimes, recurrence of the tumor on its surgical removal is also possible

How is Malignant Proliferating Pilar Tumor Treated?

The treatment of Malignant Proliferating Pilar Tumor may involve the following:

  • In most cases, a wide surgical excision and removal of the entire tumor is adequate treatment. This may be followed by radiation therapy and/or chemotherapy
  • Mohs micrographic surgery uses real-time examination of frozen section pathology by the surgeon to check margins for residual tumor. Mohs has a higher rate of attaining a tumor-free defect before suturing up the scalp
  • If the tumor has metastasized (in rare cases), then a combination of chemotherapy, radiation therapy, and invasive procedures may be used to treat the tumor
  • Follow-up care with regular screening and check-ups are important and encouraged

How can Malignant Proliferating Pilar Tumor be Prevented?

  • Currently, there are no known methods available to prevent the occurrence of Malignant Proliferating Pilar Tumors
  • Regular medical screening at periodic intervals with blood tests, scans, and physical examinations, are mandatory, due to its metastasizing potential and possibility of recurrence. Often several years of active vigilance is necessary

What is the Prognosis of Malignant Proliferating Pilar Tumor? (Outcomes/Resolutions)

  • The prognosis of Malignant Proliferating Pilar Tumor depends upon the stage of the tumor, the location of the tumor, and the age and health of the individual. But, the prognosis is generally good with surgery
  • Early diagnosis and adequate treatment of the tumor is important to ensure better outcomes

Additional and Relevant Useful Information for Malignant Proliferating Pilar Tumor:

  • There is no evidence to prove that oily foods and chocolate-based products have an influence on Proliferating Pilar Tumor
  • Cleaning the skin too hard with strong chemicals or soaps may aggravate the skin condition. Care must be taken avoid strong soaps and chemicals that could potentially worsen the condition
  • The presence of dirt on the body is not a causative factor for the condition. However, it helps to be clean and hygienic, which may help the condition from getting worse

What are some Useful Resources for Additional Information?


References and Information Sources used for the Article:


Helpful Peer-Reviewed Medical Articles:


Reviewed and Approved by a member of the DoveMed Editorial Board
First uploaded: Nov. 26, 2015
Last updated: March 29, 2021