Fibromatosis

The fibromatosis is a benign connective tissue, often aggressively grows and infiltrates into the environment. After surgical removal, there is a high risk of recurrence. The tumor formation are mostly related to myofibroblasts. Generally can not superficial encapsulated forms with fibroids less than 5 cm divided by deep forms in the chest and abdomen. A degeneration is extremely rare.

Classification

The WHO grouped the fibromatosis with other connective tissue tumors and fibroids as a class of " fibroblastic - myofibroblastic tumors " and divided according to histological malignancy in the group of benign, locally aggressive / intermediary who rarely metastatic / intermediate and malignant tumors ( but only from the fibrosarcomas and Fibromyxoidsarkomen exists ).

Fibromatosis as nodular fasciitis occur also reactive.

Superficial fibromatosis

The various forms frequently occur in combination. A distinction is made depending on the localization, inter alia:

• Palmar - fibromatosis ( Dupuytren's disease ) in the palms with characteristic flexion contracture of the fingers.
• Plantar fibromatosis - (Crohn Ledderhose ) on the soles
• Penile curvature ( Peyronie's disease, also known as Peyronie's disease )
• Gingival fibromatosis ( Jones Syndrome )
• Fibromatosis colli as a rare cause of congenital torticollis with tumor in the distal third of the sternocleidomastoid muscle.
• Desmoid tumor that forms in muscle fascia;

Deep fibromatosis

• Retroperitoneal fibrosis, also Ormond's disease, as fibromatosis between the posterior peritoneum ( retroperitoneal ) and the spine, often due to a narrowing of the ureter ( ureter ) for urinary obstruction ( hydronephrosis ) in the kidney leads. Differences are secondary forms, eg after irradiation, is discussed from a primary fibromatosis, for an autoimmune cause.
• Sclerosing mediastinitis
• Sclerosing mesenteritis

Aggressive fibromatosis

The so-called aggressive fibromatosis grow infiltrative and elude any therapy. The attempt is to remove surgically fatal; because he can not completely succeed and stimulates the growth - which otherwise comes by itself to a standstill.