Desmoid Tumours of the extremity and trunk. A retrospective study of 44 patients

Abstract

BackgroundDesmoid-type fibromatosis (DF) is a aggressive (myo)fibroblastic neoplasm with an infiltrative growth and a tendency to local recurrence. Resection of the tumour and/or radiation were proposed as principal treatment. The aim of this retrospective study was to analyze the local control rates focusing on the effect of surgical margins and radiotherapy.MethodsFrom 1981 to 2014, 44 patients had been treated. Fifty four therapies had been applied, in 50 cases surgery +/− radiation therapy, NSAIDs or chemotherapy. In 4 cases a conservative approach was chosen. Thirty seven patients had primary, 17 recurrent disease. Endpoint was either local recurrence (LR), progression of residual disease or rare non-metastatic secondary lesions at the same extremity.ResultsThe mean age was 39,4 years. In 17 cases a R0, in 27 a R1 and in 6 cases a R2 resection was achieved. Four patients were treated conservatively. All together in 21 cases radiotherapy, in 5 NSAIDs, in 3 imatinib and in 2 cases each tamoxifen or chemotherapy had been applied. The median follow-up was 119 months. 5-year recurrence free survival after resection was 78%. 10 (20.4%) patients developed LR between 5 and 42 months after therapy. Recurrent disease was a negative factor on LR. Margins, radiotherapy, sex, or size of the tumour had no significant impact on LR. Patients younger than 40 years had a significant higher risk of LR.ConclusionsSurgical margins are less important than keeping function. Radiotherapy might be an option in unresectable lesions, the role of adjuvant radiotherapy is controversially discussed.

Clinical features and management of superficial fibromatoses

“…palmar fibromatosis (Dupuytren's contracture), plantar fibromatosis (Ledderhose disease), penile fibromatosis (induratio penis plastica), knuckle pads and pachydermodactyly . Superficial fibromatoses are more frequent and less aggressive than deep fibromatoses .…”

“…Adult‐onset deep or desmoid‐type fibromatosis (DTF) may be further subclassified into extra‐abdominal, abdominal and mesenteric types . Desmoid‐type fibromatosis may arise sporadically or may be related to disorders caused by germline mutations, such as Gardner's syndrome and familial adenomatous polyposis .…”

Klinische Merkmale und Management oberflächlicher Fibromatosen

“…Die oberflächlichen Dermatosen des Erwachsenenalters manifestieren sich in kleinen Läsionen an Händen, Füßen oder am Penis und umfassen die Palmarfibromatose (Dupuytren‐Kontraktur), die Plantarfibromatose (Morbus Ledderhose), die Penisfibromatose (Induratio penis plastica), die Fingerknöchelpolster und die Pachydermodaktylie . Die oberflächlichen Fibromatosen sind häufiger und weniger aggressiv als die tiefen Fibromatosen .…”

“…Die im Erwachsenenalter auftretenden tiefen Fibromatosen vom Desmoid‐Typ (DTF) können weiter in extraabdominale, abdominale und mesenteriale Formen unterteilt werden . Die Fibromatose vom Desmoid‐Typ kann sporadisch auftreten oder mit Erkrankungen assoziiert sein, welche wie das Gardner‐Syndrom und die familiäre adenomatöse Polyposis durch Keimbahnmutationen verursacht werden .…”

Possible involvement of cyclooxygenase-2 in the recurrence of desmoid fibromatosis: case series and mini-review of the literature

“…Between puberty and 40 y old, tumors are most common in women, and predominantly arise in the abdominal wall [6,7]. Later in adulthood, the distribution between abdominal and extra-peritoneal tumors becomes more even, and the sex ratio of affected patients tends towards parity [1,[5][6][7].…”