Desmoid tumor

The desmoid tumor is a tumor from the group of fibromatoses that forms on the sheaths of muscles ( muscle fascia ). Due to its tendency to infiltrate into the surrounding tissue, the generally benign desmoid tumor is clinically counted among the aggressive lesions of the soft tissue.

The general incidence is 1 in 4 million. It is a rare disease, only 0.1% of all tumors are desmoid tumors. Women after pregnancy are most commonly affected (due to a desmoid tumor in the abdomen). In children and adolescents, the extremities, head and neck area are more likely to be affected; men are more often affected than women.

A desmoid tumor can regress spontaneously or progress slowly. This can lead to painful inflammation, impairment of the function of neighboring organs and restrictions in the mobility of limbs.

pathology

etiology

The causes are largely unexplained, except for Gardner syndrome , a combination of familial adenomatous polyposis of the intestine, desmoid tumor and osteomas , there is no hereditary disposition . Estrogen probably plays a role , since the tumor often occurs as a result of pregnancy, often resolves spontaneously during menopause and sometimes responds to anti-hormonal therapy. In addition, the tumor is observed more often as a result of an injury in scar tissue.

morphology

The gray-white tumor has a coarse macroscopic consistency and is usually more than 5 cm in size.

Histologically similar looking fibroblasts with only minor nuclear atypia occur in the tumor . The cells are aligned in the same direction and are located in a wave-like or vortex-shaped collagen network .

therapy

Desmoid tumor of the iliac bone with soft tissue infiltration

According to the current status of the interdisciplinary discussion, once the diagnosis has been confirmed, one should first wait, as many tumors recede spontaneously or stop growing. One possible therapy is surgery. The prerequisite is that the tumor can be completely removed without affecting the function of limbs or organs. In the discussion, however, is the safety margin to be achieved. Recurrences are relatively common. Radiation therapy can be performed after an incomplete resection or in inoperable cases . But here, too, it remains unclear under which conditions this is to be indexed exactly.

In many cases, especially in relapses or inoperable cases, drug therapy is used. This is usually antibody therapy or chemotherapy . In some cases, anti-hormonal treatment or treatment with anti-inflammatory agents is also effective. However, the mode of action has not yet been researched. Treatment with interferon has also been successfully tested, but is often poorly tolerated.

The desmoid has the property of growing into the surrounding tissue via fibers. These extremely thin tumor areas are then very difficult or even impossible to detect in the usual quick sections, which is why they may be wrongly stated as tumor-free.

Medical therapy

In drug therapy, promising results were achieved with the combination of high-dose antiestrogen (e.g. tamoxifen ) in conjunction with Sulindac .

Since sulindac is not approved in Germany, it is often first substituted with indomethacin . The results here are, however, much worse than with therapy with Sulindac. This is because both drugs are used to treat rheumatism , but are not identical in their mode of action.

Sulindac is transformed in the liver into

1. Sulindac sulfide, a COX-2 inhibitor , similar to indomethacin and
2. Sulindac sulfone (exisulind), which apparently has an additional dampening effect on the growth of the degenerated cells (more detailed information on this is lacking so far), which is missing with indomethacin.

In addition to these substances, which should rather be seen as the first step in drug therapy, tyrosine kinase inhibitors such as imatinib , sunitinib , sorafenib or pazopanib have been introduced into therapy in recent years . In a phase III study (ClinicalTrials.gov number: NCT02066181) the effect of sorafenib was tested in patients with progressive, symptomatic or relapsed desmoid tumors. There was a significantly longer progression-free survival time and a sustained response. Sorafenib has not been approved for the treatment of desmoid tumors in either Germany or the USA (as of February 26, 2019).

In severe cases, classic chemotherapy with anthracycline- based protocols, such as for soft tissue sarcomas, is also possible.

literature

• JR Döhler , H. Hamelmann, U. Lasson: Aggressive fibromatoses. In: The surgeon . 55, 1984, pp. 174-178.
• Uta Höhle: The recurrences of aggressive fibromatoses - prognostic clinical and immunohistochemical parameters for their development and consequences for therapy . Dissertation. EMU, 2008.
• Matthias Keller: The extra-abdominal fibromatosis: evaluation of operative and radiotherapy measures with special consideration of the relapse case . Dissertation. Technical University of Munich, 2014.
• Charisse Litchman: Desmoid Tumors . Springer Netherland, Berlin 2011, ISBN 978-94-007-1684-1 .
• B. Kasper, C. Baumgarten, S. Bonvalot, R. Haas, F. Haller, P. Hohenberger, G. Moreau, WT van der Graaf, A. Gronchi; Desmoid Working Group: Management of sporadic desmoid-type fibromatosis: a European consensus approach based on patients 'and professionals' expertise - a sarcoma patients EuroNet and European Organization for Research and Treatment of Cancer / Soft Tissue and Bone Sarcoma Group initiative. In: Eur J Cancer. 51 (2), Jan 2015, pp. 127-136.

Web links

• Project website of the Universities of Heidelberg and Berlin on the Desmoid
• German Interdisciplinary Sarcoma Group
• Video of the focus on tumor orthopedics at the Orthopedic Clinic of the LMU Munich

Individual evidence

1. ↑ Macroscopic image of the desmoid on Patho Pic
2. ↑ Review of the European Consensus on Therapy
3. ↑ MT Ballo, GK Zagars, A. Pollack: Radiation therapy in the management of desmoid tumors. In: International Journal of Radiation Oncology - Biology - Physics . Volume 42, Number 5, December 1998, pp. 1007-1014, ISSN  0360-3016 . PMID 9869223 .
4. ^ Rolf Sauer : Radiation Therapy and Oncology . Elsevier, Urban & FischerVerlag, 2009, ISBN 978-3-437-47501-6 , pp. 477– ( google.com [accessed October 7, 2011]).
5. ↑ Sulindac can have life-threatening side effects such as skin damage and blindness. See New York Times. March 5, 2013
6. ↑ Mrinal M. Gounder, Michelle R. Mahoney, Brian A. Van Tine, Vinod Ravi, Steven Attia: Sorafenib for Advanced and Refractory Desmoid Tumors . In: New England Journal of Medicine . tape 379 , no. 25 , December 20, 2018, ISSN  0028-4793 , p. 2417–2428 , doi : 10.1056 / NEJMoa1805052 ( nejm.org [accessed February 26, 2019]).

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