Fibromatoses

Group of disorders characterized by expanding benign fibrous tissue that are more aggressive than benign isolated fibromas and less aggressive than fibrosarcoma.

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Fibromatoses can be divided in three syndromes:

1. 1. Hereditary desmoid disease

2. 2. Congenital generalized fibromatosis

3. 3. Jaffe Campanacci syndrome

At a Glance

Autosomal dominant disorder characterized by infiltrative fibromatosis of the mesentery without colonic polyps, osteomas and sebaceous cysts. It is known to predispose to desmoid tumor but its association with colon cancer is unknown.

Incidence and Genetic Inheritance

Incidence unknown. Autosomal dominant inheritance.

Clinical Aspects

These are fibroproliferative disorders in which histologically benign tumors of musculoaponeurotic tissue arise and are often locally invasive. They occur more commonly in patients with familial adenomatous polyposis. The fibromatosis may be intraabdominal (mesenteric) or extraabdominal, such as paraspinal, limb, buttock, flank, head, and neck.

Anesthetic Considerations

Patients receive multidisciplinary treatment, so they may present for surgery after having received chemotherapy or radiotherapy. Patients with tumors of the head and neck may require careful airway management planning according to the site of the tumor(s).

References

At a Glance

Characterized by multiple fibroblastic tumors involving skin, striated muscles, bones, and viscera. Tumors are present at birth or develop during the first weeks of life.

Synonym

Juvenile Myofibromatosis.

Incidence and Genetic Inheritance

Incidence is unknown. Autosomal recessive transmission is suggested, but there are also cases of autosomal dominance.

Clinical Aspects

Solitary or multicentric fibroblastic tumors of skin, striated muscle, bone, and viscera, which are congenital or develop in the first weeks of postnatal life. The multicentric type may be visceral or nonvisceral. The former has a higher mortality and may involve lungs, myocardium, gastrointestinal tract, and rarely the central nervous system. Nonvisceral tumors tend to regress spontaneously.

Anesthetic Considerations

Patients may present for surgery for removal of locally aggressive tumors. Very rarely patients with central nervous system involvement present with extrinsic cord compression or with an intracranial space-occupying lesion. One third of solitary lesions affect head and neck structures.

References

At a Glance

Syndrome of unknown etiology with variable expression characterized mainly by nonossifying fibromata, extraskeletal congenital anomalies such as café au lait spots, mental retardation, hypogonadism or cryptorchidism, and ocular and cardiovascular malformations. Other clinical features include chylothorax, stenosis of aortic isthmus, mitral insufficiency, chylopericardium, mental retardation, and precocious puberty.

Synonym

Multiple Nonossifying Fibromatosis.

Incidence and Genetic Inheritance

Incidence is unknown. Genetics are not understood. Males and females appear to be equally affected. There usually is no family history of the syndrome or of neurofibromatosis, which has similar characteristics.

Clinical Aspects

Usually presents between the ages of 10 and 15 years, typically with a pathologic fracture. Smooth-bordered café-au-lait spots, axillary freckling, and multiple nonossifying fibromas are present without soft tissue neurofibromas.

Anesthetic Considerations

Be careful with handling and movement in view of the predisposition to pathologic fractures.

References