Background Extra-abdominal desmoid tumor fibromatosis (DTF) is a rare, locally aggressive soft tissue tumour. The best treatment modality for this patient cohort is still object of debate. Questions/purpose This paper aimed to (1) to compare the outcomes of DTF after different treatment modalities, (2) to assess prognostic factors for recurrence following surgical excision, and (3) to assess prognostic factors for progression during observation. Methods This was a retrospective multicenter study under the patronage of the European Musculoskeletal Oncology Society (EMSOS). All seven centres involved were tertiary referral centres for soft tissue tumours. Baseline demographic data was collected for all patients as well as data on the diagnosis, tumour characteristics, clinical features, treatment modalities and whether they had any predisposing factors for DTF. Results Three hundred eighty-eight patients (240 female, 140 male) with a mean age of 37.6 (±18.8 SD, range: 3–85) were included in the study. Two hundred fifty-seven patients (66%) underwent surgical excision of ADF, 70 patients (18%) were observed without therapy, the residual patients had different conservative treatments. There were no significant differences in terms of tumour recurrence or progression between the different treatment groups. After surgical excision, younger age, recurrent disease and larger tumour size were risk factors for recurrence, while tumours around the shoulder girdle and painful lesions were at risk of progression in the observational group. Conclusion Local recurrence rate after surgery was similar to progression rates under observation. Hence, observation in DTF seems to be justified, considering surgery in case of dimensional progression in 2 consecutive controls (3 and 6 months) and in painful lesions, with particular attention to lesions around the shoulder girdle.
Patient: Male, 54-year-old Final Diagnosis: Femoral fracture due to gigantic pseudoaneurysm secondary to the rupture of a previously ligated and bypassed popliteal artery aneurysm Symptoms: Swelling and pain Clinical Procedure: — Specialty: Orthopedics and Traumatology • Surgery Objective: Unusual clinical course Background: This case report describes a giant pseudoaneurysm that grew in size during the years following surgical treatment of a popliteal artery aneurysm, eventually causing a femoral fracture. Bone fractures secondary to vascular injuries are rarely described in the literature. Case Report: A 54-year-old man underwent surgical ligation and bypass for left popliteal artery aneurysm. Seven years later, he suffered a left distal femur pathologic fracture surrounded by a giant soft-tissue mass. The patient came to us with a diagnostic hypothesis of angiosarcoma from another hospital at imaging evaluation. After computed tomography angiography (CTA) and angio-magnetic resonance imaging (MRI), we made a diagnosis of femoral pathologic fracture caused by a giant pseudoaneurysm of a treated popliteal artery aneurysm refilled by an aberrant anterior tibial artery (IIA2, Kim classification). We performed excision of the mass and open reduction and internal fixation, with anatomic plate, of the fracture. Fracture healing and good functional outcome were observed at follow-up. Conclusions: A possible complication of surgical treatment of popliteal artery aneurysms is refilling of the excluded aneurysm due to collateral blood flow or, such as in the present case, aberrant vessels. Therefore, the knowledge of anatomical variants of the vessels is important in surgery. Follow-up evaluation after surgery is advisable and a growing mass should be further investigated with an angio-CT scan. In case of a non-pulsating soft-tissue mass causing pathologic bone fracture, a biopsy is mandatory to exclude malignancy.
<p><strong>Aim</strong> <br />Uncommon and rare hip diseases are sources of pain and functional limitation particularly in young patients. Some of these conditions may be nowadays treated by arthroscopy due to the expertise and technical tips that high-volume hip arthroscopies have achieved during the last decades ensuring a wider range of indications for such a procedure. The aim of this study was to evaluate clinical results of arthroscopy in treating uncommon or rare diseases of the hip at a single Institution.<br /><strong>Methods</strong> <br />Thirteen patients affected by several types of diseases were treated by a hip arthroscopy and retrospectively evaluated.<br />All patients were operated by the same surgeon, instrumentation and technique, but postoperative rehabilitative protocol was tailored on each patient and his disease. Each patient underwent a specific imaging, consisting of dedicated x-rays and arthro-MRI. Modified Harris Hip score (mHHS) and Non-Arthritic Hip score (NAHS) were used before and after surgery to clinically assess the outcome.<br /><strong>Results</strong> <br />All patients reported satisfaction, pain relief, and a good functional recovery at the latest follow-up visit. Only one patient<br />affected by chondromatosis reported a recurrence of synovitis and needed a further arthroscopy 25 months after the index operation. No complications were reported at the latest follow-up. The NAHS and mHHS showed good improvements and all patients referred high satisfaction.<br /><strong>Conclusion</strong> <br />Hip arthroscopy performed by expert and high-volume surgeons may ensure good results in patients affected by uncommon and rare hip diseases.</p>
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