Infantile Fibromatosis

Infantile Fibromatosis is a rare condition that involves the growth of a single or numerous benign (non-cancerous) tumors. It is often referred to as myofibromatosis, due to the abundance of fibrous and smooth muscle stoma. Infantile fibromatosis can affect the skin, muscles, soft-tissues, bones and rarely visceral organs.



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What is Infantile Fibromatosis?

Infantile fibromatosis is a rare condition that arises from mesenchymal cells and involves the growth of a singular or numerous benign (non-cancerous) tumors. This condition is also referred to as infantile myofibromatosis or congenital fibromatosis. Tumors may be present at or shortly after birth, however a majority of all diagnoses occur before the age of two. They appear as firm, round nodules commonly located in the head, neck, trunk, and upper and lower extremities. While they are considered locally aggressive tumors, infantile fibromatosis does not metastasize, or spread to other body parts.

Who is usually affected?
Who is usually affected?
  • • Children under the age of 5, with a majority of cases diagnosed in children 2 years old or younger.
  • • Boys are more commonly affected than girls with a ration of 2:1-3:1.
Common Sites Involved
Common Sites Involved
  • • Shoulder girdle
  • • Muscles and fascia of the head/neck and thigh
  • • Involvement of the long bones may be associated with tumors of the leg
Causes
Causes
  • • The cause of infantile fibromatosis is unknown.
Signs and Symptoms
Signs and Symptoms
  • • Signs and symptoms include a firm, growing mass.
Biological Behavior
Biological Behavior
  • • Localized: most common, more commonly seen in males, 7-10% recurrence after excision, great prognosis. 
  • • Multicentric without visceral involvement: presents with multiple nodules.
  • • Generalized form: multiple nodules with visceral involvement (lungs and GI tract), poorer prognosis.
Diagnosis
Diagnosis
  • • The work-up for Infantile Fibromatosis often consists of a physical examination, X-ray, MRI, CT, and bone scans. The diagnosis is often confirmed with a biopsy, which samples the tumor for further analysis. Differential diagnoses include hemangiomas, infantile fibrosarcoma, and other soft-tissue tumors.

Risk to your limbs

Infantile fibromatosis is a rare condition in which one or many non-cancerous tumors growth. These masses are considered locally aggressive tumors, however infantile fibromatosis does not metastasize, or spread to other body parts. On rare occasions, the tumor may grow back.

Radiographic imaging is used to help form a diagnosis of infantile fibromatosis. These include X-Ray, MRI, CT and Bone Scans

An example of an MRI is shown.

Infantile Fibromatosis2

Treatment of Infantile Fibromatosis

Treatment of infantile fibromatosis includes surgical excision of the tumor through wide or radical resections, both of which are limb-sparing surgeries. The tumor is removed as carefully and completely as possible without interfering with function or causing disfigurement to the affected area.

Surgery
Surgery

Surgical treatment includes wide or radical resections to remove the complete tumor and additional margins. The removal of additional, surrounding margins ensures that the tumor is completely removed and decreases the chances of the tumor coming back.

I've seen many doctors and I can confidently attest Dr. Wittig is the preeminent orthopaedic specialist. He is genuinely kind and caring, as he demonstrated by completely addressing my concerns and compassionately relating to what I was dealing with. He clearly outlined the plan of attack, and recommended the two additional doctors who would become part of my 'team'. Dr. Wittig was so effective in allaying our fears and bringing us optimism. My surgery was significant, but I was up and walking the next day and back at the gym 5 weeks later. This is further testament to Dr. Wittig's skill. He saved my leg and my life, and I feel so very blessed to say he is my doctor. I have already recommended him to others, and I will continue to do so. I would trust him with my closest family and lifelong friends. BEST DOCTOR EVER.

S.G.

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Hey! I am Dr. Wittig.

Myself and my amazing team are dedicated to saving your life and your limb. Losing a limb because of a tumor can be a terrifying experience. But, it does not have to be the only option. I’ve spent 20+ years as a Board-Certified Orthopedic Surgeon and Orthopedic Oncologist.

I’ve devoted my career to helping children and adults afflicted with bone and soft tissue masses by performing complex limb saving surgeries. Most patients can have their limb saved, which may require innovative techniques.

Patients afflicted with musculoskeletal tumors have complex conditions that are best taken care of at large hospitals. I am the Chairman of Orthopedics and Chief of Orthopedic Oncology at Morristown Medical Center. My philosophy is a multidisciplinary team approach, working together to tailor treatment to individual patients. Education and research are essential to my practice, providing the best setting for extraordinary patient care. Because of this, we have some of the top results in the country.