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

• PFHT (Plexiform Fibrous Histiocytoma Tumor)
• PFT (Plexiform Fibrohistiocytic Tumor)
• Plexiform Fibrous Histiocytoma Tumor (PFHT)

What is Plexiform Fibrohistiocytic Tumor? (Definition/Background Information)

• Plexiform Fibrohistiocytic Tumor (PFT) is a rare, low-to-intermediate grade, soft tissue tumor occurring on the skin, or just below the skin; sometimes, it extends deep into the skeletal muscles and tissues
• These slow-growing tumors are generally painless, and occur with extreme infrequence on the upper limbs. Other tumor sites include the lower limbs, head and trunk region
• Young adults and adolescent children, predominantly females, are affected by PFT, which can be diagnosed through a biopsy
• Surgical excision with complete removal of the entire tumor is the preferred mode of treatment. This may be followed by radiation therapy. The prognosis is usually good on a complete surgical removal of the tumor

Who gets Plexiform Fibrohistiocytic Tumor? (Age and Sex Distribution)

• Plexiform Fibrohistiocytic Tumor lesions are generally present in young individuals (children, teenagers, and young adults), with an average age of presentation at around 14.5 years
• However, it has been observed in a wider age range too; infants to very old people may be affected
• Females are affected much more than males, in a 2.5-6.0 : 1 (female-male) ratio
• There is no ethnic or racial preference noted

What are the Risk Factors for Plexiform Fibrohistiocytic Tumor? (Predisposing Factors)

• No risk factors for Plexiform Fibrohistiocytic Tumor are evident
• Isolated cases of genetic anomalies have been found in PFT; though, having a specific genetic defect, has not been shown to increase one’s risk for the tumor

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 Plexiform Fibrohistiocytic Tumor? (Etiology)

• The exact cause of Plexiform Fibrohistiocytic Tumor formation is unknown
• They are thought to occur spontaneously, or due to other morphological or genetic anomalies
• Currently, the role of genetic aberrations discovered with PFT is considered as purely coincidental, and not exactly causative. Further research is being conducted to establish the cause

What are the Signs and Symptoms of Plexiform Fibrohistiocytic Tumor?

The signs and symptoms of Plexiform Fibrohistiocytic Tumor may include:

• PFTs are slow-growing, small, soft tissue tumors, occurring mostly below the skin surface. But, sometimes they are found deep inside the body tissues too
• Initially, these tumors are painless; they may appear as painful masses, after many years of growth
• Most common sites of these lesions are the upper limbs - the hands and wrists account for 65% of the cases. Other locations include the lower limbs (27% of the cases), the trunk, head, and neck region

How is Plexiform Fibrohistiocytic Tumor Diagnosed?

A diagnosis of Plexiform Fibrohistiocytic Tumor would involve:

• Thorough physical exam with evaluation of complete medical history
• CT scan, MRI scan of the affected region
• Histopathological studies conducted on a biopsy specimen - the specimen is examined under a microscope by a pathologist, to arrive at a definitive diagnosis
• Since the tumor is rare, other tumors should be considered as part of differential diagnosis before arriving 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 Plexiform Fibrohistiocytic Tumor?

The complications of Plexiform Fibrohistiocytic Tumor may include the following:

• Recurrence after surgery is high, if surgery does not completely remove the tumor
• The lungs, lymph nodes, and the entire body, are prone to Plexiform Fibrohistiocytic Tumor metastasis. This may lead to life-threatening situations, though this is an extremely rare occurrence
• Sometimes, the fibrous tumor tissues affect the bone and adipose (fat) tissues too
• Damage of vital nerves, blood vessels and surrounding structures, during surgery

How is Plexiform Fibrohistiocytic Tumor Treated?

The treatment measures for Plexiform Fibrohistiocytic Tumor include the following:

• Surgical excision with complete removal of the entire lesion is normally sufficient treatment
• In order to prevent recurrence, radiation therapy may be administered
• For both surgical intervention and radiation therapy, the age and health condition of the individual has to be taken into consideration
• Post-operative care is important: A minimum activity level is to be ensured, until the surgical wound heals
• Periodic observation through regular follow-ups with a healthcare provider needs to be maintained. If there is any pain, it is controlled through pain medications

How can Plexiform Fibrohistiocytic Tumor be Prevented?

• Current medical research have not established a way of preventing Plexiform Fibrohistiocytic Tumor
• Regular medical screening at periodic intervals with blood tests, radiological scans, and physical examinations, are mandatory for those who have been diagnosed with the tumor
• Due to both its metastasizing potential and high chances of recurrence, often several years of active follow-up vigilance is necessary

What is the Prognosis of Plexiform Fibrohistiocytic Tumor? (Outcomes/Resolutions)

• Prognosis for Plexiform Fibrohistiocytic Tumor is generally excellent, when the tumor lesions are small and found below the skin surface; their recurrence risk can be completely avoided with complete surgical excisions
• The probability of PFT recurrence is high after surgical removal (at 12-38%), and the risk from metastases is moderate (at 10%); thus periodic follow-up with screening is strongly recommended

Additional and Relevant Useful Information for Plexiform Fibrohistiocytic Tumor:

• Plexiform Fibrohistiocytic Tumor is characterized by a typical plexiform arrangement of cell groups - the firm, multinodular growth appears as an interconnected or crisscrossed mesh of poorly-defined mass
• There are three distinct recognized growth patterns for Plexiform Fibrohistiocytic Tumor. They are called:
  • Fibrohistiocytic Plexiform Fibrohistiocytic Tumor
  • Fibroblastic Plexiform Fibrohistiocytic Tumor
  • Mixed-type Plexiform Fibrohistiocytic Tumor