Dedifferentiated chondrosarcoma of the ring finger: a case report of an exceptional surgical treatment and review of literature.

The DDCS is a rare, highly malignant tumor characterized by two distinct histopathologic components. The diagnosis is insidious, and the prognosis is poor. The therapy is primarily surgical. It is possible to associate chemotherapy if the nonchondrogenic component is a responder. Unfortunately, very few cases of DDCS of the hand are described in the literature. Therefore, few scientific comparisons are possible regarding diagnosis and therapy. For this reason, we decided to present a sporadic case of DDCS of the fourth finger of the left hand, presented with a seven months history of pain and swelling, which appeared after the treatment of recurrent enchondroma and was treated with amputation and complex reconstruction. To our knowledge, no similar cases have been previously reported in the literature.

Hybrid Simulation and Planning Platform for Cryosurgery with Microsoft HoloLens.

Cryosurgery is a technique of growing popularity involving tissue ablation under controlled freezing. Technological advancement of devices along with surgical technique improvements have turned cryosurgery from an experimental to an established option for treating several diseases. However, cryosurgery is still limited by inaccurate planning based primarily on 2D visualization of the patient's preoperative images. Several works have been aimed at modelling cryoablation through heat transfer simulations; however, most software applications do not meet some key requirements for clinical routine use, such as high computational speed and user-friendliness. This work aims to develop an intuitive platform for anatomical understanding and pre-operative planning by integrating the information content of radiological images and cryoprobe specifications either in a 3D virtual environment (desktop application) or in a hybrid simulator, which exploits the potential of the 3D printing and augmented reality functionalities of Microsoft HoloLens. The proposed platform was preliminarily validated for the retrospective planning/simulation of two surgical cases. Results suggest that the platform is easy and quick to learn and could be used in clinical practice to improve anatomical understanding, to make surgical planning easier than the traditional method, and to strengthen the memorization of surgical planning.

Proximal Humerus Reconstruction after Tumor Resection: An Overview of Surgical Management.

Proximal humerus is one of the anatomical sites that are most frequently involved by bone and soft tissue malignant tumors. Alone or in association with adjuvant treatments, surgery represents the main therapeutic option to treat and eradicate these diseases. Once the first-line option, in the last decades, amputation lost its role as treatment of choice for the large majority of cases in favor of the modern limb sparing surgery that promises to preserve anatomy and-as much as possible-upper limb functionality. Currently, the main approaches used to replace proximal humerus after a wide resection in oncologic surgery can be summarized in biological reconstructions (allografts and autografts), prosthetic reconstructions (anatomic endoprostheses, total reverse shoulder prostheses), and graft-prosthetic composite reconstructions. The purpose of this overview is to present nowadays surgical options for proximal humerus reconstruction in oncological patients, with their respective advantages and disadvantages.

Image-guided Cryotherapy for Musculoskeletal Tumors.

BACKGROUND: This article represents a review of the use of image-guided cryotherapy in the treatment of musculoskeletal tumor lesions. Cryotherapy is able to induce a lethal effect on cancer cells through direct and indirect mechanisms. In this manuscript, we combined our experience with that of other authors who have published on this topic in order to provide indications on when to use cryotherapy in musculoskeletal oncology. DISCUSSION: Image-Guided percutaneous cryotherapy is a therapeutic method now widely accepted in the treatment of patients with musculoskeletal tumors. It can be used both for palliative treatments of metastatic bone lesions and for the curative treatment of benign bone tumors, such as osteoid osteoma or osteoblastoma. In the treatment of bone metastases, cryotherapy plays a major role in alleviating or resolving disease-related pain, but it has also been demonstrated that it can have a role in local disease control. In recent years, the use of cryotherapy has also expanded for the treatment of both benign and malignant soft tissue tumors. CONCLUSION: Percutaneous cryotherapy can be considered a safe and effective technique in the treatment of benign and malignant musculoskeletal tumors. Cryotherapy can be considered the first option in benign tumor lesions, such as osteoid osteoma, and a valid alternative to radiofrequency ablation. In the treatment of painful bone metastases, it must be considered secondarily to other standard treatments (radiotherapy, bisphosphonate therapy, and chemotherapy) when they are no longer effective in controlling the disease or when they cannot be repeated (for example, radiotherapy).

Bone metastases.

Bone is the most frequent site for metastasis for many cancers, notably for tumours originating in the breast and the prostate. Tumour cells can escape from the primary tumour site and colonize the bone microenvironment. Within the bone, these disseminated tumour cells, as well as those arising in the context of multiple myeloma, may assume a state of dormancy, remaining quiescent for years before resuming proliferation and causing overt metastasis, which causes bone destruction via activation of osteoclast-mediated osteolysis. This structural damage can lead to considerable morbidity, including pain, fractures and impaired quality of life. Although treatment of bone metastases and myeloma bone disease is rarely curative, disease control is often possible for many years through the use of systemic anticancer treatments on a background of multidisciplinary supportive care. This care should include bone-targeted agents to inhibit tumour-associated osteolysis and prevent skeletal morbidity as well as use of appropriate local treatments such as radiation therapy, orthopaedic surgery and specialist palliative care to minimize the impact of metastatic bone disease on physical functioning. In this Primer, we provide an overview of the clinical features, the pathophysiology and the specific treatment approaches to prevent and treat bone metastases from solid tumours as well as myeloma bone disease.

One-Step Reconstruction with Custom-made 3D-printed Scapular Prosthesis After Partial or Total Scapulectomy.

En bloc scapulectomy with covering muscles was historically considered the only procedure available for surgical treatment of bone and soft tissue tumors of the scapula. When possible, reconstruction with scapular allograft is the gold standard, and gives satisfactory functional, cosmetic, and oncological outcomes. While good results have recently been reported with 3D-printed prostheses for reconstruction of bone loss, there is little information available in the medical literature regarding scapula reconstruction with a 3D-printed prosthesis. Between 2016 and 2018, we performed four scapular resections (two total and two involving the superior 1/3) followed by reconstruction with a 3D-printed prosthesis made of a porous titanium alloy (Ti-6Al-4V, diameter between 100 and 400 mm), using computer-aided design (CAD) and patient-specific implants (PSI) with previously acquired CT-MR fusion images. At 2 years follow-up, the patients with partial scapulectomy had an MSTS score of 76%, no local or systemic recurrence, good clinical results and no pain. At 1 year 6 months follow-up, the patients with total scapulectomy had an MSTS score of 46%, no local or systemic recurrence, fair clinical results and no pain. Thus, custom-made 3D-printed prostheses appear to be valuable in orthopedic surgery. However, a larger cohort and longer-term analysis are needed to evaluate the scapular 3D-printed prosthesis as a reliable reconstruction technique.

Hereditary Multiple Exostoses: Current Insights.

Hereditary multiple exostoses (HME), also called hereditary multiple osteochondromas, is a rare genetic disorder characterized by multiple osteochondromas that grow near the growth plates of bones such as the ribs, pelvis, vertebrae and especially long bones. The disease presents with various clinical manifestations including chronic pain syndromes, restricted range of motion, limb deformity, short stature, scoliosis and neurovascular alteration. Malignant transformation of exostosis is rarely seen. The disease has no medical treatment and surgery is only recommended in symptomatic exostoses or in cases where a malignant transformation is suspected. HME is mainly caused by mutations and functional loss of the EXT1 and EXT2 genes which encode glycosyltransferases, an enzyme family involved in heparan sulfate (HS) synthesis. However, the peculiar molecular mechanism that leads to the structural changes of the cartilage and to osteochondroma formation is still being studied. Basic science studies have recently shown new insights about altering the molecular and cellular mechanism caused by HS deficiency. Pediatricians, geneticists and orthopedic surgeons play an important role in the study and treatment of this severe pathology. Despite the recent significant advances, we still need novel insights to better specify the role of HS in signal transduction. The purpose of this review was to analyze the most relevant aspects of HME from the literature review, give readers an important tool to understand its clinical features and metabolic-pathogenetic mechanism, and to identify an effective treatment method. We focused on the aspects of the disease related to clinical management and surgical treatment in order to give up-to-date information that could be useful for following best clinical practice.

Surgical Treatment of Sacral Chordoma: En Bloc Resection with Negative Margins is a Determinant of the Long-Term Outcome.

STUDY DESIGN: Retrospective case series. OBJECTIVE: To report the outcome of a series of patients with sacral chordoma who were surgically treated at a single center. SUMMARY: Chordomas are low-grade malignant tumors that arise from remnants of the notochord. They are most often found in the sacrum, spine and skull-base. These tumors have a slow clinical evolution and may eventually metastasize, even after adequate treatment. Rarely, they can dedifferentiate into high-grade sarcomas. Traditionally, chordomas were considered to be resistant to chemotherapy and standard radiation therapy. However, recently, adrotherapy has been shown to be effective for local and systemic control of the disease. In this study, clinical outcomes and local and systemic recurrence were reviewed to identify prognostic factors for local and systemic control. METHODS: Thirty-three patients with sacral chordoma (19 males, 14 females; median age 61 y, range 43-80) who were surgically treated at our institution between 1994 and 2015 were reviewed. In 24 patients, resection was performed above S2. No patients received pre-operative radiotherapy (RT). Three cases received RT (carbon ion therapy) as treatment for local recurrence. Wide (R0) surgical margins were achieved in 17 patients, marginal (R1) margins in 14 patients and intralesional (R2) margins in 2 patients. RESULTS: At a median follow-up of 53 months (range 0-198), 19 patients were continuously disease-free, 6 were disease-free after local recurrence (5) or metastases (1), 3 were alive with disease (2 local recurrence and 1 metastasis), 4 were dead of disease (1 patient died intraoperatively) and 1 was dead of another cause. Local recurrence was observed in 9 cases (27%); all 9 were treated surgically and 3 received carbon ion therapy after surgical intralesional excision. Overall survival at 10 years was 86.6%. Local recurrence-free survival at 10 years was 51%. A statistical analysis confirmed the importance of negative surgical margins (R0) to achieve local control of the disease (p = 0.0007). High resections (above S2) were associated with lower survival and higher risk of local recurrence. CONCLUSION: Surgical en bloc resection is the primary treatment for sacral chordoma. Carbon ion therapy is used when it is difficult to obtain wide surgical margins. Due to morbidity and the disabling sequelae of surgery, adrotherapy may be considered an alternative to high (above S2-S3) sacral chordoma resections.

En bloc resection of primary tumors of the thoracic spine: indications, planning, morbidity.

OBJECTIVES: We aimed to describe surgical planning, technique, and complications of en bloc resection in the thoracic spine in patients who opted for surgery with en bloc resection and a tumor-free margin. METHODS: Oncologic and functional results were recorded for 134 patients (53% male, age 44 ± 18 years) who had undergone en bloc resection for primary tumors (90 cases) and bone metastases (44 cases). Patients were followed until death or the latest follow-up examination (0-211 months, median 47 months). Surgeries were performed from 1990 to 2007 by the same team. An ongoing critical analysis of local control rates, surgical complications, and expected versus actual loss of function enabled the authors to refine the surgical technique and propose seven different types of resection. RESULTS AND DISCUSSION: En bloc resection is a demanding procedure and requires careful planning after a careful decision-making process. The patient has to correctly understand the purpose of the surgery, based on oncological staging, in order to accept or decide against the procedure after weighing the possible morbidity and functional loss against the expected final result. TECHNIQUES OF EN BLOC RESECTION: Seven different strategies to perform en bloc resection in the thoracic spine, based on four combinations of surgical approaches (anterior, posterior, anterior followed by posterior, and posterior followed by simultaneous anterior and posterior) are identified and proposed. This planning is based on Weinstein-Boriani-Biagini (WBB) surgical staging. The surgical plan was designed to achieve the required oncologic margin with the minimum achievable morbidity.

Aneurysmal bone cyst-like areas as a sign of metastatic disease in the spinal column.

Aneurysmal bone cysts (ABCs) are vascular lesions with well-defined radiological signs. A fairly common MRI finding in both ABC and other primary bone tumors with high vascularity is the presence of cysts with fluid-fluid levels. Metastatic lesions with fluid-fluid leveling have not previously been defined in the literature. We report three cases of fluid-fluid leveling of the axial skeleton in metastatic lesions. Two patients had single-level and one multi-level neoplastic disease with distinctive cysts showing fluid-fluid levels on MRI evaluation. Diagnosis of gastric carcinoma in two patients and breast carcinoma in the third was achieved with trocar biopsies. At the time of this report all patients had completed initial oncologic treatments and were disease free.

Complications in minimally invasive percutaneous fixation of thoracic and lumbar spine fractures and tumors.

PURPOSE: We propose to evaluate the complication rate in minimally invasive stabilization (MIS) for spine fractures and tumors, as a common alternative to open fusion and conservative treatment. METHODS: From 2000 to 2010, 187 patients were treated by minimally invasive percutaneous fixation in 133 traumatic fractures and 54 primitive and/or secondary spine tumors. Complications were classified, according to the period of onset as intraoperative and postoperative, and according to the severity, as major and minor. RESULTS: A total of 15 complications (8 %) were recorded: 5 intraoperative (3 %), 6 early postoperative (3 %) and 4 late postoperative (2 %); 6 were minor complications (3 %) and 9 were major complications (5 %). CONCLUSIONS: Minimally invasive stabilization of selected spine pathologies appears to be a safe technique with low complication rate and high patient satisfaction. MIS reduces hospitalization and allows a fast functional recovery improving the quality of life.

Minimally Invasive Posterior Stabilization Improved Ambulation and Pain Scores in Patients with Plasmacytomas and/or Metastases of the Spine.

Background. The incidence of spine metastasis is expected to increase as the population ages, and so is the number of palliative spinal procedures. Minimally invasive procedures are attractive options in that they offer the theoretical advantage of less morbidity. Purpose. The purpose of our study was to evaluate whether minimally invasive posterior spinal instrumentation provided significant pain relief and improved function. Study Design. We compared pre- and postoperative pain scores as well as ambulatory status in a population of patients suffering from oncologic conditions in the spine. Patient Sample. A consecutive series of patients with spine tumors treated minimally invasively with stabilization were reviewed. Outcome Measures. Visual analog pain scale as well as pre- and postoperative ambulatory status were used as outcome measures. Methods. Twenty-four patients who underwent minimally invasive posterior spinal instrumentation for metastasis were retrospectively reviewed. Results. Seven (29%) patients were unable to ambulate secondary to pain and instability prior to surgery. All patients were ambulating within 2 to 3 days after having surgery (P = 0.01). The mean visual analog scale value for the preoperative patients was 2.8, and the mean postoperative value was 1.0 (P = 0.001). Conclusion. Minimally invasive posterior spinal instrumentation significantly improved pain and ambulatory status in this series.

Surgical treatment of grade I central chondrosarcoma.

UNLABELLED: The grade of chondrosarcoma relates to the likelihood of local recurrence and metastases. Many Grade I chondrosarcomas behave benignly if aggressively, and the question arises regarding whether wide resection is essential to control the disease. We therefore asked whether intralesional surgery also could be extended to Grade I chondrosarcomas without an increase in recurrence. We retrospectively reviewed 31 patients with Grade I chondrosarcomas of the limbs. The minimum followup was 66 months (mean, 157 months; range, 66-296 months). None of the 16 patients treated by resection had recurrences during the followup and two of the 15 patients with intralesional excision had recurrences, both of which resolved with resection of the site involved by the recurrence without progression of the disease. The Musculoskeletal Tumor Society scores averaged 72% in patients treated with wide resection compared with 89% in the 15 patients treated by intralesional surgery. The two recurrences occurred in patients whose radiographs showed thinning of the cortex combined with bone enlargement and marked endosteal scalloping; histologic examination in these two patients also showed a correlation between radiographic aggressiveness and the presence of myxoid areas and hypercellularity. LEVEL OF EVIDENCE: Level II, prognostic study. See Guidelines for Authors for a complete description of levels of evidence.

Giant cell tumor of the extremity: A review of 349 cases from a single institution.

Giant cell tumor is still one of the most controversial and discussed bone tumors. Surgical treatment options include intralesional excision or segmental resection. Curettage has a higher recurrence rate but does preserve adjacent joint function. After curettage, the use of adjuvant therapies is still controversial. Three hundred forty-nine patients with giant cell tumors of the extremity, treated in a single institution, were analyzed in a retrospective study. Two hundred patients underwent curettage of the lesion and in 64 of these cases, three local adjuvants, such as phenol, alcohol and cement, were employed. The hypothesis is that an "aggressive curettage" with phenol, alcohol and cement provides better local control and functional results. The correlation between tumor in the proximal femur and higher recurrence rate has not been recorded before. The results of the present study suggest that an "aggressive curettage" reduces the recurrence rate in a disease whose aggressiveness is not easy to predict. Special attention must be given to giant cell tumors not only in the distal radius, but also in the proximal femur, where the treatment is more difficult and associated with a higher rate of local recurrence.

Allograft-prosthetic composite in the proximal tibia after bone tumor resection.

UNLABELLED: We consider an allograft-prosthesis composite in the proximal tibia one of the better reconstructive options in this site because it combines the mechanical stability of a prosthesis with the biologic reconstruction of the extensor mechanism. We retrospectively reviewed 62 patients who had proximal tibia reconstructions with allograft-prosthesis composites to ascertain the complications and functional outcomes. By combining an allograft with a prosthesis, placing cement in the graft, and press-fitting the prosthesis in the tibial diaphysis, we obtained satisfactory Musculoskeletal Tumor Society scores in 90.4% of patients, with a 5-year survival rate (73.4%) comparable to that of reconstruction with a modular prosthesis. However, we observed high infection rates (24.2%) and rotation of the medial gastrocnemius seemed not to reduce this complication. For this reason, we do not recommend using this reconstructive technique in patients who will receive postoperative chemotherapy or in patients in whom a previous reconstructive method failed. We believe the ideal candidate is the young patient with a benign aggressive or malignant low-grade tumor who has not undergone previous surgery. LEVEL OF EVIDENCE: Level IV, therapeutic study. See the Guidelines for Authors for a complete description of levels of evidence.

Management of pelvic giant cell tumours involving the acetabular bone.

The purpose of this study was to establish the optimal surgical treatment in patients with a Giant Cell Tumour (GCT) involving the acetabular bone. The surgical outcome in 10 patients with GCT involving the acetabular bone was reviewed. Patients were divided into two groups: group 1 in which 5 patients were primarily treated by curettage, and group 2, in which 5 patients were treated by resection and pelvic reconstruction. In group 1, local recurrence occurred in two cases. The functional outcome was excellent or good in 4, and poor in one case. There were no recurrences in group 2, in which the functional outcome was excellent or good in 4 and poor in one patient. The optimal surgical treatment modality should be based upon the tumour extension. Tumours located primarily in the ischiopubic region and not extending proximally beyond the supra-acetabular line can be adequately treated by extended curettage while those with further proximal extension are better treated by en-bloc resection. However, the possible complications of the different methods of pelvic reconstruction should always be considered.

Clear cell chondrosarcoma of bone: long time follow-up of 18 cases.

INTRODUCTION: Clear cell chondrosarcoma is a variant of conventional chondrosarcoma behaving as low grade malignant bone tumor. We reviewed our cases of clear cell chondrosarcoma with the aim to assess the best diagnostic and curative approach to this rare tumor. MATERIALS AND METHODS: We report 18 cases of clear cell chondrosarcoma treated at the authors' institution. Pertinent clinical information, pre and postoperative serum alkaline phosphatase (SAP) level were obtained from the patients charts. Available imaging studies including plain roentgenograms, computed tomography (CT) images (in 6 patients) and magnetic resonance imaging (MRI) images (in 2 patients). RESULTS: Eight cases were firstly treated in other institutions, seven with one or more curettage, while one, presented with pathologic fracture, was treated with repeated osteosynthesis. Of the seven patients treated with curettage, five were referred with local recurrence, the mean time between surgical treatment and local recurrence was 52.2 months (from 12 to 132). The other two patients were curetted out 4 and 1 month before, respectively, and were then referred to us to decide about further treatment after incidental diagnosis. Of these cases, three (37.5%) recurred again after our treatment and two of them presented with late bone metastases. Eight patients were originally treated with wide (six) or marginal (two) surgical margins by us. Among them only one proximal humerus (wide margins) had local recurrence 26 months from the first operation. Two patients underwent curettage in our institution, and one of them had a local recurrence. Pre-operative SAP level were obtained in 17 patients. Among them, 11 patients had high pre-operative SAP level (64.7%) and 6 cases were normal. Post-operative SAP levels were obtained in nine patients. After removal of the tumors; SAP levels decreased to normality in seven of the nine patients with high pre-operative SAP. In the other two patients, although the SAP levels were normal, post-operative SAP levels were decreased. CONCLUSION: Serum alkaline phosphatase can be used as a good tumor marker in diagnosis and follow-up. Due to the occurrence of local recurrences and distant metastasis after curettage, we suggest surgical treatment with "en bloc" resection from the first presentation.