Fibromatosis
| Classification according to ICD-10 | |
|---|---|
| M72 | Fibromatoses |
| M72.0 | Fibromatosis of the palmar fascia (Dupuytren's contracture) |
| M72.1 | Knuckle pads |
| M72.2 | Fibromatosis of the plantar fascia (Ledderhose contracture) |
| M72.4 | Pseudosarcomatous fibromatosis |
| M72.6 | Necrotizing fasciitis |
| M72.9 | Fibromatosis, unspecified |
| D48 | New formation of unsafe or unknown behavior at other and unspecified locations |
| D48.3 | Retroperitoneum |
| D48.4 | peritoneum |
| ICD-10 online (WHO version 2019) | |
The fibromatosis is a benign connective tissue, which often grows aggressively and infiltrated into the environment. There is a high risk of recurrence after surgical removal . The tumor formation usually starts from myofibroblasts. In general, superficial, non-encapsulated forms with fibroids under 5 cm can be subdivided from deep forms in the chest and abdomen. Degeneration is extremely rare.
Classification
The WHO groups fibromatoses with other connective tissue tumors and fibromas as a class of " fibroblastic- myofibroblastic tumors" and subdivides according to histological malignancy into the groups of benign, locally aggressive / intermediate, rarely metastasizing / intermediate and malignant tumors (but only from the fibrosarcoma and fibromyxoid sarcoma).
There are also hereditary forms such as infantile systemic hyalinosis and infantile myofibromatosis or Zimmermann-Laband syndrome .
Superficial fibromatosis
The different forms often appear together. A distinction is made depending on the location:
- Desmoid tumor in muscle fascia
- Nodular fasciitis
- Fibromatosis colli as a rare cause of a congenital torticollis with a lump in the distal third of the sternocleidomastoid muscle
- Hereditary gingival fibromatosis , Jones syndrome
- Palmar fibromatosis ( Dupuytren's disease ) in the palms of the hands with characteristic flexion contracture of the fingers.
- Plantar fibromatosis ( Ledderhose's disease ) on the soles of the feet
- Penile curvature ( induratio penis plastica , also called Peyronie's disease )
Deep fibromatosis
- Retroperitoneal fibrosis , also Ormond's disease , as fibromatosis between the posterior peritoneum ( retroperitoneal ) and the spine, which often leads to urinary retention ( hydronephrosis ) in the kidneys due to the narrowing of the ureters ( ureter ) . A distinction is made between secondary forms, e.g. B. after radiation, of primary fibromatosis for which an autoimmune cause is being discussed.
- Sclerosing mediastinitis
- Sclerosing mesenteritis
Aggressive fibromatoses
The so-called aggressive fibromatoses grow infiltratively and are difficult, often delayed, to treat. In the case of surgical removal, similar to a tumor operation, a resection far in the healthy (in sano) should be aimed for. The success must be secured histopathologically. If this is successful, additional radiation therapy offers no advantage. In the case of incomplete removal and incision margins in the diseased tissue, additional radiation therapy seems to be beneficial and can delay the time to relapse. Overall, however, recurrences are very common and the complications associated with surgery are reported in up to a third of all cases. The indication must therefore be made individually and carefully.
See also
Web links
Individual evidence
- ↑ Ursus-Nikolaus Riede, Hans-Eckart Schaefer: General and special pathology. 3. Edition. Thieme-Verlag, Stuttgart 1993, ISBN 3-13-683303-9 , p. 1156.
- ↑ Christopher D. Fletcher, Krishnan K. Unni, Frederik Mertens: Pathology & Genetics. Tumors of Soft Tissue and Bone . IARC Press, Lyon 2002, ISBN 92-832-2413-2 .
- ↑ Matthias Keller: The extra-abdominal fibromatosis - evaluation of operative and radiotherapeutic measures with special consideration of the relapse case . Dissertation . TU Munich, 2013.
- ↑ P Prodinger et al .: Surgical resection and radiation therapy of desmoid tumors of the extremities: results of a supra-regional tumor center In: Int Orthop . 37 (2013), pp. 1987-1993.
- ↑ SH Shin et al .: Surgical outcome of desmoid tumors: adjuvant radiotherapy delayed the recurrence, but did not affect long-term outcomes In: Journal of Surgical Oncology . 108 (2013), pp. 28-33.
