Lipoblastomatosis

Lipoblastomatosis

Article
Kids' Zone
Diseases & Conditions
+1
Contributed byMaulik P. Purohit MD MPHOct 29, 2018

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

  • Benign Lipoblastomatosis 

What is Lipoblastomatosis? (Definition/Background Information)

  • Lipoblastomatosis is a rare, benign tumor of fatty tissue that is observed predominantly in infants and young children
  • These tumors are usually diffused, multiple in numbers and are not well-circumscribed. They may not cause any signs and symptoms
  •  The frequent sites of Lipoblastomatosis include the arms and legs
  • There are no identified risk factors for Lipoblastomatosis and the cause is unknown. However, the involvement of chromosomal abnormalities has been detected in them
  • If the tumors are asymptomatic, then no treatment may be necessary. But, if required, a complete surgical excision of the Lipoblastomatosis may be performed
  • The prognosis is generally excellent, since these tumors are benign. However, tumor recurrence after surgery is high at 15%

Who gets Lipoblastomatosis? (Age and Sex Distribution)

  • Lipoblastomatosis is typically found in infants and very young children
  • Both males and females are affected, though the incidence of the condition is higher in males
  • There is no racial or ethnic preference observed

What are the Risk Factors for Lipoblastomatosis? (Predisposing Factors)

  • The risk factors for Lipoblastomatosis are currently unknown, or have not been established

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 Lipoblastomatosis? (Etiology)

  • The exact cause and mechanism of Lipoblastomatosis formation is unknown
  • However, specific genetic mutations linked to this condition have been detected on chromosome 8

What are the Signs and Symptoms of Lipoblastomatosis?

The signs and symptoms of Lipoblastomatosis are:

  • A number of tumors may be observed at several locations of the body
  • Most common location of the tumor is the arms and legs
  • Other locations include the mediastinum (between the lungs), retroperitoneum (abdominal cavity), chest, and back
  • The nodules are not well-circumscribed, are diffuse, and may be painless
  • Some tumors may not present any signs or symptoms

How is Lipoblastomatosis Diagnosed?

Lipoblastomatosis is diagnosed using the following tools:

  • Complete physical examination with comprehensive evaluation of medical history
  • Radiographic studies of the affected region
  • Tissue biopsy: A tissue 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
  • A differential diagnosis may be used to eliminate other tumor types

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 Lipoblastomatosis?

There are no significant complications due to Lipoblastomatosis.

  • However, tumors in the chest or abdomen may cause compression of the adjoining organs giving rise to associated signs and symptoms
  • 15% of the tumors are known to recur after surgery

How is Lipoblastomatosis Treated?

Treatment measures for Lipoblastomatosis include the following:

  • If there are no symptoms, then conservative treatment may be helpful. However, periodic observation is maintained and if there is any pain, it can be controlled through pain medication
  • Surgical excision with removal of the entire tumor is the standard treatment method used, which can ensure a complete cure
  • Post-operative care is important: One must maintain minimum activity levels, until the surgical wound heals
  • Post-operative follow-up care with regular screening and check-ups are important

How can Lipoblastomatosis be Prevented?

Current medical research has not established a way of preventing Lipoblastomatosis, which are observed in young children.

What is the Prognosis of Lipoblastomatosis? (Outcomes/Resolutions)

  • Lipoblastomatosis is a rare, benign, and generally asymptomatic tumor. They may not require any treatment
  • The prognosis is generally excellent when the tumors are small (and fewer in numbers) and can be completely excised and removed

Additional and Relevant Useful Information for Lipoblastomatosis:

The following DoveMed website link is a useful resource for additional information:

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

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On the Article

Maulik P. Purohit MD MPH picture
Approved by

Maulik P. Purohit MD MPH

Assistant Medical Director, Medical Editorial Board, DoveMed Team

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