Desmoid tumours are a rare fibroblastic proliferation of monoclonal origin, arising in deep soft-tissues. Histologically, they are characterized by locally aggressive behaviour and an inability to metastasize, and clinically by a heterogeneous and unpredictable course. Desmoid tumours can occur in any anatomical site, but commonly arise in the limbs. Despite their benign nature, they can be extremely disabling and sometimes life-threatening, causing severe pain and functional limitations. Their surgical management is complex and challenging, due to uncertainties surrounding the biological and clinical behaviour, rarity, and limited available literature. Resection has been the first-line approach for patients with a desmoid tumour but, during the last few decades, a shift towards a more conservative approach has occurred, with an initial ‘wait and see’ policy. Many medical and regional forms of treatment are also available for the management of this condition, and others have recently emerged with promising results. However, many areas of controversy remain, and further studies and global collaboration are needed to obtain prospective and randomized data, in order to develop an appropriate shared stepwise approach. Cite this article: Bone Joint J 2023;105-B(7):729–734

Desmoid tumors are rare benign but aggressive lesions. They are characterized by bland-appearing fibroblasts, indistinct margins, and the ability of local invasion and recurrence. Though they are not cancer they may metastasize and can cause significant morbidity. Treatment is primarily surgical, although radiation or systemic therapy can be beneficial if surgery is not feasible. We retrospectively reviewed our patients since 1980 in respect to treatment modalities and outcome. Between 1980 and 2008 26 patients (16 m, 10 f) with desmoid tumours had been surgically treated. The mean age with diagnosis was 37 years (7 – 69 years). The mean age at surgery was 46 years (10 – 81 years). 17 of the patients had only one resection. In 6 patients two resections, in 2 three resections and in one patient four resections had to be performed. Only 7 patients achieved a R0-situation. In 9 patients adjuvant radiotherapy was used. Two patients had several courses of Vinblastine or MTX based chemotherapy, three patients had Sulindac or Tamoxifen, several other patients combinations of different NSARs. All patients were still alive, one patient developed metastatic disease from the initial lesion in the groin to the foot. After an average of 17 months (7 – 42 months) 11 patients showed recurrent disease. 13 patients are without recurrence after an average of more than 9 years. In 4 patient stable disease is seen without progression in 42 to 156 months (Ø 95 months). In conclusion desmoid tumours did show an inpredictable course of disease. Due to many alterations in treatment in the 28 years since the first patient of this study and the rareness of this disease no clear predicitve factor could be established. Remarkably no patient did need an amputation. A more detailed analysis regarding the dose effect of irradiated patients is intented

Purpose: Desmoid tumours are uncommon in the axillary region. We report the clinical course in seven patients operated for desmoid tumours of the axillary region. Material and methods: Our series included two women and five men. Mean age was 52 years (39–60). One tumour was in the trapesium, two were in the retroplexic, two in the axillary fossa, one in the deltoid and one in the upper part of the arm. Biopsy and magnetic resonance imaging were obtained in all patients. Surgical margins were in healthy tissue in five cases; resection was limited to intratumoural tissue in one. The axillary nerve had to be sacrificed in two patients, the spinal nerve in one and the medial nerve in one. Chemotherapy was given to one patient prior to surgery. Results: Mean follow-up was 51 months (23.2–162.1). Five patients experienced at least one relapse requiring one or two complementary procedures. Among the relapse cases, one patient required resection of the median nerve with bypass of the humeral artery. No amputations were necessary and the brachial plexus was not sacrificed. Four patients were given one to five adjuvant chemotherapy courses. Two were given radiotherapy. Shoulder motion was preserved in two patients, moderately reduced in five. Elbow and finger mobility was compromised due to the medial epicondylar site of the tumour in one patient whose median nerve had to be sacrificed. Discussion: Even though the surgical margins were in healthy tissue, the risk of relapse was high in our patients as in other series reported in the literature. The course does however stabilise after several episodes of recurrence, an observation reported in the literature and confirmed in our patients. In case of brachial plexus involvement, several authors advocate preservation of upper limb function despite incomplete tumour resection, proposing postoperative radiotherapy. Conclusion: Desmoid tumours of the axillary region seriously compromise upper limb function. Surgical resection should be as complete as possible but without sacrificing upper limb function

Purpose of the study: Extra-abdominal desmoids tumours are benign tumours generally managed by more or less wide surgical resection. This surgery can be mutilating and carries a risk of recurrence to the order of 50% according to several authors. Material and methods: We followed a series of 26 patients from 1989 to 2009 with non-operated extra-abdominal desmoids tumours. Our objective was to study the clinical, radiographic and pathological course of these tumours and identify prognostic factors. For 11 patients, no medical or surgical treatment was delivered beyond the diagnostic biopsy; for 15, the tumour recurred after surgery and no other adjuvant treatment was given. Results: Twenty-four tumours stabilised at 13.2 months on average for primary tumours and 20.9 for recurrent tumours. Thus en general, the tumour progression was less than 3 years. In all cases, once the tumour stopped progressing, it did not progress again. One primary tumour regressed spontaneously and one recurrence continued to progress at last follow-up of 23 months. Surgery was not required for any of the patients, excepting biopsy procedures to confirm diagnosis. MRI was the exploration of choice for follow-up. Discussion: This series, which is the largest devoted to followed extra-abdominal desmoid fibromas, confirms recent data in the literature. A wait-and-see attitude should always be discussed with this type of tumour

The stiffness of the shoulder can result of many illness. Nevertheless, we observed a severe stiffness of the scapulo thoracic space only in fibromatosis. To precise the real diagnostic value of this symptom, we examined patients with different diseases of shoulder (tumoral and non tumoral). The passive mobility of the shoulder of 11 patients with peri-scapular fibromatosis was compared to the mobility of those in 50 patients with non tumoral diseases of shoulder (arthritis and rotator cuff pathology), 50 peri-scapular soft tissues tumours, and 100 patients with primitive or secondary malignancies of humerus or scapula. Results: in 10 of 11 patients with peri-scapular fibromatosis, the passive mobility of the scapulo thoracic spacewas severely impaired (less than 20°). In non tumoral pathology of shoulder, the passive mobility of the shoulder is frequently impaired but the stiffness hangs only on scapulo humeral articulation. In metastases, sarcoma and soft tissue tumour (except fibromatosis) the passive mobility of the scapulo-humeral joint is usually preserved and the mobility of the scapulothoracic space is always normal even in very huge tumours. After treatment of fibromatosis, 9/11 patients are in complete remission and the mobility of their scapulo thoracic space restored. 2 patients are in stable disease and one suffers of a residual stiffness of the scapulothoracic space. We conclude that the frozen scapulo thoracic is a specific symptom of peri scapulo thoracic fibromatosis. The restoration of the mobility of the scapulo thoracic after cure of the desmoid tumour confirms its specific role and represents a good marker of the tumoral evolution

Desmoid tumour is an histological benign tumour. Nevertheless, peri-scapular relapses can decrease the function and intra thoracic progression threaten life. To prevent these complications, damaging treatment (radiotherapy, amputation) are sometimes proposed. To precise the optimal indications of treatment, we reviewed our cases. Patients: from 1984 to 2008, we treated 11 patients with peri-scapula fibromatosis (mean age 42 (13–58)). Only 4 patients were seen at first hand, 7 for relapses (3 of them after radiotherapy). Treatment was adapted to each patient, in function of age, history of illness, and risks of spontaneous evolution. En bloc extratumoral resection was performed each time, when it didn’t expose to heavy functional risk (8). The other patients were treated by contaminated resection, but never invaliding. 4 patients received pre or/and post-operative chemotherapy. 1 received Interferon alpha, and 7 tamoxifen. Results: with a median follow-up 15 years 3 months, 7 patients suffered of recurrence. No patient died from disease (thoracic complications) or therapeutic complication9 patients are in complete remission and 2 in stable disease. Following radiotherapy, local relapses (7 cases) and repeated surgery, functional sequellaes are numerous: 2 circumflex nerve palsies, 3 articular stiffness. Major functional sequellaes came from radiotherapy (limb discrepancy, lung and thorax deformity, skin and muscle atrophy. Conclusion: in this non predictable illness, therapeutic indications should individually balance risks of spontaneous evolution and of complications of treatment. Besides surgery, needed in fast all cases, but often insufficient, it must be considered the value of interferon, tamoxifen and/or chemotherapy. The most important concept is the necessity to treatment avoiding late sequellaes and particularly radiotherapy or mutilating surgery

Introduction: Soft-tissue sarcomas (STS) in children and young adults are rare. This is a heterogeneous group of tumors, which is traditionally divided to rhabdomyo-sarcomas and non-rhabdomyosarcoma soft-tissue sarcomas (NRSTS). These tumors are further classified to high- and low-grade tumors. Material and Methods: Between 1988 and 1999, the authors treated 50 patients (25 males, 25 females) under the age of 20 who were diagnosed with a soft-tissue sarcoma. Histopathological Diagnoses: rhabdomyosarcoma – 11, synovial sarcoma – 6, other high-grade STS (extraskeletal Ewing’s sarcoma, epitheloid sarcoma, neurofibrosarcoma, hemangiopericytoma, fibrosarcoma, and unclassified sarcoma) – 17. Seven patients were diagnosed with low-grade STS and 9 patients with an aggressive desmoid tumor. Anatomic Location: Lower extremities – 30, upper extremities – 9, shoulder girdle – 2, trunk – 4, pelvic girdle – 5. Preoperative Treatment: Thirty patients received neo-adjuvant chemotherapy, four patients underwent isolated limb perfusion with TNF and melphalan, and one patient received preoperative radiation therapy. Surgery: Forty-seven underwent limb-sparing resections and 3 underwent primary amputation. Wide margins were achieved in 37 patients and marginal margins in 10. Intralesional resection was performed in 3 patients. Postoperative Treatment: Thirty-seven patients received adjuvant chemotherapy and 34 received radiation therapy. Oncological Status: At the most recent follow-up, 24 patients of the 37 patients with high-grade STS have no evidence of disease, three are alive with disease, and seven are dead. Fourteen of the 16 patients with low-grade tumors have no evidence of disease and 2 are alive with disease. There were 4 secondary amputations due to local tumor recurrence. Conclusions: Management of soft-tissue sarcomas in children and young adults requires the judgmental use of pre- and postoperative treatment modalities. Local tumor control can be achieved in the majority of the patients. A longer follow-up is required to determine the overall survival of these patients