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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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Fibromatosis
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Congenital fibromatosis affecting the scalp in an infant.

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Fibromatoses can be divided in three syndromes:

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  1. 1. Hereditary desmoid disease

  2. 2. Congenital generalized fibromatosis

  3. 3. Jaffe Campanacci syndrome

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At a Glance

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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.

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Incidence and Genetic Inheritance

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Incidence unknown. Autosomal dominant inheritance.

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Clinical Aspects

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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.

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Anesthetic Considerations

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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).

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References

Dormans JP, Spiegel D, Meyer J, et al: Fibromatoses in childhood: The desmoid/fibromatosis complex. Med Pediatr Oncol 37:126, 2001.  [PubMed: 11496351]

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At a Glance

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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.

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Synonym

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Juvenile Myofibromatosis.

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Incidence and Genetic Inheritance

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

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Clinical Aspects

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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.

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Anesthetic Considerations

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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.

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References

Netscher DT, Eladoumikdachi F, Popek EJ: Infantile myofibromatosis: Case report of a solitary hand lesion with emphasis on differential diagnosis and management. Ann Plast Surg 46:62, 2001.  ...

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