Calcifying Fibrous Tumor

Calcifying Fibrous Tumor

Article
Diseases & Conditions
Cancer & Benign Tumors
+1
Contributed byKrish Tangella MD, MBAAug 09, 2018

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

  • Calcifying Fibrous Pseudotumor (CFP)
  • CFT (Calcifying Fibrous Tumor)
  • Childhood Fibrous Tumor with Psammoma Bodies

What is Calcifying Fibrous Tumor? (Definition/Background Information)

  • Calcifying Fibrous Tumor (CFT) is a rare and benign tumor seen with chronic inflammation
  • The tumors are associated with a variety of factors including trauma, Castleman disease, and inflammatory myofibroblastic tumors. Although, currently, no conclusive proof has been established between the tumor and these conditions
  • The tumor is seen in both children and adults with frequent sites being the extremities, neck, and back. Calcifying Fibrous Tumors are generally painless tumors
  • Some tumors may grow to large sizes and compress the surrounding tissue structures and organs. Otherwise, Calcifying Fibrous Tumors may be only detected incidentally
  • A tissue biopsy helps in a definitive diagnosis of Calcifying Fibrous Tumor, which is treated through a surgical excision for removal of tumor
  • This may result in a cure, although recurrence of the condition is observed. Nevertheless, the prognosis of Calcifying Fibrous Tumors on appropriate treatment is good

Who gets Calcifying Fibrous Tumor? (Age and Sex Distribution)

  • Calcifying Fibrous Tumor may occur in individuals of all races, ethnic groups, and both gender
  • It is seen in a wide age range of individuals from young children to adults
    • Somatic tissue tumors are seen in children (average age 3 years); such tumors are seen in the extremities and other places
    • Visceral tumors are observed in adults (average age 43 years); the organs are affected by visceral tumors

What are the Risk Factors for Calcifying Fibrous Tumor? (Predisposing Factors)

Currently, no definite risk factors for Calcifying Fibrous Tumor are generally known. However, the following conditions have been linked to CFT:

  • Sometimes, it is seen after trauma
  • The tumors are known to be present along with Castleman disease; a rare condition characterized by the abnormal proliferation of lymphocytes (white blood cell type)
  • Rarely, some tumors are known to be inherited (presenting a familial background)
  • Some reports say that Calcifying Fibrous Tumors may be linked to inflammatory myofibroblastic tumor or even to IgG4-related sclerosing disease (a complex and chronic inflammatory condition). But such associations have not been completely proven

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 Calcifying Fibrous Tumor? (Etiology)

The cause of Calcifying Fibrous Tumor formation is unknown.

  • Some studies have indicated that the tumor is associated with various conditions including trauma, family history, disorders, and other tumors
  • However, the exact associations between CFT and the aforementioned conditions have not been fully understood

What are the Signs and Symptoms of Calcifying Fibrous Tumor?

The signs and symptoms of Calcifying Fibrous Tumor include:

  • The presence of a soft tissue tumor at any location in the body, under the skin or deep in the body tissues, including in the arms and legs, neck, back, male genitals, abdominal wall. The tumors at these locations are especially noted in children
  • Also, CFT can occur inside the body and intracavity areas affecting the organs such as the digestive tract, intestines, chest wall cavity, peritoneum, and pleura in lungs. The tumors at these locations are noted in adults
  • The tumors are mostly painless; sometimes multiple tumors are observed
  • Generally, the tumors are firm and well-circumscribed
  • Their size may range from 5 mm to as much as 25 cm (while deep inside the body). Large tumors may cause compression and mass-like effect

Normally, Calcifying Fibrous Tumors remain silent and are discovered when assessing the body for other health conditions.

How is Calcifying Fibrous Tumor Diagnosed?

Calcifying Fibrous Tumor diagnosis may involve the following tests and exams:

  • Physical examination and evaluation of complete medical history
  • Radiological studies, such as X-ray of the affected region, MRI and CT scan of the affected region. Such studies may show areas of calcification (hence, the term ‘calcifying’ is associated with the tumor)
  • Tissue biopsy of the tumor: A tissue biopsy is performed and sent to a laboratory for a pathological examination, who 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 Calcifying Fibrous Tumor?

The possible complications from Calcifying Fibrous Tumor include:

  • Some tumors recur after surgical excision; rarely, multiple recurrences are observed
  • Large-sized tumors may severely impair the function of the adjacent organs

How is Calcifying Fibrous Tumor Treated?

  • Treatment of Calcifying Fibrous Tumor is a complete surgical excision, which results in a cure. However, the recurrence risk of the condition remains
  • Hence, follow-up care with regular screening and check-ups are important and encouraged

How can Calcifying Fibrous Tumor be Prevented?

Currently, the formation of Calcifying Fibrous Tumor is not preventable, as the cause and risk factors have not been definitively established.

What is the Prognosis of Calcifying Fibrous Tumor? (Outcomes/Resolutions)

  • The prognosis of Calcifying Fibrous Tumor is generally excellent with appropriate treatment, since it is a benign tumor
  • However, some tumors are observed to recur following their surgical removal

Additional and Relevant Useful Information for Calcifying Fibrous Tumor:

Please visit our Cancer & Benign Tumor Health Center for more physician-approved health information:

http://www.dovemed.com/diseases-conditions/cancer/

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Krish Tangella MD, MBA picture
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Krish Tangella MD, MBA

Pathology, Medical Editorial Board, DoveMed Team

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