Unexpected diagnosis of multiple sclerosing pneumocytomas in a patient with chondrosarcoma of the hand.

Sclerosing pneumocytomas are rare, benign pulmonary neoplasms that predominantly affect Asian female patients in the age category of 40-70 years, mostly non-smokers. We report on a 72-year-old Caucasian woman with chondrosarcoma of the hand who developed multiple bilateral progressive lung nodules suspicious of lung metastases. Staged lung resections were performed, and pathological diagnosis was confirmed by immunohistochemical analysis of the resected specimens. Next-generation sequencing (NGS) was used to detect gene mutations. Immunohistochemistry demonstrated sclerosing pneumocytomas, and NGS showed an IDH1 mutation. Eventually, the patient developed lung metastases for which rethoracotomy was performed. The differentiation of sclerosing pneumocytoma from lung cancer is a diagnostic challenge, and sclerosing pneumocytoma should be considered in the differential diagnosis of pulmonary nodules. Gene mutation analysis does not always show classical and common mutations, which should be kept in mind when interpreting its results.

Clinical Outcome of Surgically Treated Leiomyosarcoma of the Extremities: A Retrospective Overview.

AIM: This study was interested in extremity leiomyosarcoma with focus on clinical outcome after surgery with or without adjuvant therapy. PATIENTS AND METHODS: A retrospective case series of all patients with leiomyosarcoma, surgically treated between 2000 and 2015 and a minimum follow-up of 2 years, was drawn from institutional databases in Belgium and the Netherlands. Postoperative complications were reported with the Radiation Therapy Oncology Group (RTOG) and the Henderson classification. RESULTS: Seventy-five patients were operated on, of whom 47 underwent (neo)adjuvant therapy. Infection was observed in 11 patients, seven associated with (neo)adjuvant radiotherapy. Dermatological complaints were observed in 26 patients, 10 associated with (neo)adjuvant radiotherapy. Overall survival was 60%. Local recurrence occurred in 11 (15%) patients. CONCLUSION: This study describes favourable clinical outcome following (neo)adjuvant radiotherapy. In the future, larger databases on leiomyosarcoma should enhance the power of these findings and define the benefits of adjuvant therapy in leiomyosarcoma.

Femoral anteversion measurement : evaluation of inter- and intraobserver reliability.

The aim of this study was to evaluate the inter- and intraobserver reliability of a CT-based femoral anteversion measurement. 17 CT scans showing an abnormal anteversion on one side were presented to 6. Three measurements of all scans were obtained : two bilateral measurements and a third measurement with a flipped CT scan. Interobserver correlation results using the spearman test for left, right and anteversion difference had a mean of respectively : 0.918, 0.760 and 0.757. Intraobserver correlation had a maximum of respectively : 0,99, 0,89 and 0,94. Correlation coefficients were consistently higher for the second measurement. The lower correlation boarder of 0,8 was often exceeded. Intraobserver correlation was higher than interobserver correlation. As we evaluated a high variance in interobserver reliability, we recommend an accurate and objective measurement of the anteversion angle. A personal measurement and comparison to the radiological protocol is necessary.

Massive femur defect after Ewing's sarcoma resection reconstructed with a free vascularised fibular graft in a four-year-old girl.

Background: Ewing's sarcoma is the second most common primary malignant bone tumour in children. Depending on localisation and extent, massive bone defects remain when curative surgery is performed. Whereas in the past, limb amputation was often unavoidable to obtain curative resection, nowadays different limb saving reconstructive options are available.Case report: We report the successful reconstruction of a massive femur defect with a free vascularised fibular graft (FVFG) after Ewing's sarcoma resection in a four-year-old girl. Both the age of the patient and the extensive graft hypertrophy makes this case exceptional.Conclusion: Femur reconstruction with a FVFG is a rarely performed and complex procedure in young children. It is an excellent reconstructive technique for large long bone defects, which can avoid limb amputation without compromising oncologic outcome.

Long term clinical results of hallux varus correction by a reversed abductor hallucis transfer.

BACKGROUND: Iatrogenic hallux varus is a rare complication after hallux valgus surgery. Operative treatment comprises a wide variety of techniques, of which the reversed transfer of the abductor hallucis tendon is the most recent described technique. METHODS: This paper will present the long-term clinical results of the reversed transfer of the abductor hallucis longus. Therefore, we performed a prospective clinical observational study on 16 female patients. Our hypothesis is that the tendon transfer will persist in a good alignment and patient satisfaction on long term. There is a 100% follow-up rate with a range from 10 to 101 months. Patients were subjected to a clinical examination, three questionnaires and their general satisfaction. RESULTS: Out of 16 patients, at time of follow-up, we found a positive correlation between the subjective outcome score and alignment (r=0.59), and between the general satisfaction and alignment (r=0.77). Based on the general satisfaction we achieved a success satisfaction rate of 69% (11 patients). The other 31% (5 patients) patient group was only satisfied with major reservations or not satisfied at all. The two most invalidating complications were a coronal or sagittal malalignment or the combination of both. CONCLUSIONS: Our results suggest that the reverse abductor hallucis tendon transfer is a good technique to treat a supple iatrogenic hallux varus with an observed success satisfaction rate of 69% at a mean follow-up time of 48 (range 10-101) months. However, patients should be informed that on the long-term loss of correction is possible. LEVEL OF EVIDENCE: Prospective clinical observational study: Level IIb.

Desmoid tumors display a strong immune infiltration at the tumor margins and no PD-L1-driven immune suppression.

Desmoid tumors (DTs) are local aggressive neoplasms, whose therapeutic approach has remained so far unsolved and in many instances controversial. Nowadays, immunotherapy appears to play a leading role in the treatment of various tumor types. Characterization of the tumor immune microenvironment (TME) and immune checkpoints can possibly help identify new immunotherapeutic targets for DTs. We performed immunohistochemistry (IHC) on 33 formalin-fixed paraffin-embedded (FFPE) tissue sections from DT samples to characterize the TME and the immune checkpoint expression profile. We stained for CD3, CD4, CD8, CD20, FoxP3, CD45RO, CD56, CD68, NKp46, granzyme B, CD27, CD70, PD1 and PD-L1. We investigated the expression of the markers in the tumoral stroma, as well as at the periphery of the tumor. We found that most of the tumors showed organization of lymphocytes into lymphoid aggregates at the periphery of the tumor, strongly resembling tertiary lymphoid organs (TLOs). The tumor expressed a significant number of memory T cells, both at the periphery and in the tumoral stroma. In the lymphoid aggregates, we also recognized a significant proportion of regulatory T cells. The immune checkpoint ligand PD-L1 was negative on the tumor cells in almost all samples. On the other hand, PD1 was partially expressed in lymphocytes at the periphery of the tumor. To conclude, we are the first to show that DTs display a strong immune infiltration at the tumor margins, with formation of lymphoid aggregates. Moreover, we demonstrated that there is no PD-L1-driven immune suppression present in the tumor cells.

An intradural, subpial lipoma.

Introduction - patients: Intradural lipomas are rare congenital tumors. A case of intradural lipoma in the absence of any congenital spinal anomalies is reported. Patient presented with spinal cord compression syndrome. Methods - results - conclusions: Treatment of this disorder is still controversial.

Tibial Component In Total Knee Replacement :The Effect Of Overhang And Sizing On Outcome.

The aim of this study was to find a relationship between tibial overhang, malpositioning and oversizing, and the functional outcome. 188 patients were included in this retrospective study. All patients completed an Oxford knee score questionnaire at mean followup of 5 years. Anteroposterior radiographs of the total knee replacements were reviewed for medial and lateral overhang and oversizing. Lateral overhang was seen in 32.9% of patients with a mean Oxford knee score of 24.7. However lateral overhang proved no significant correlation with the functional outcome. Oversizing was seen in 31.9% of patients and, with a mean of 25.6, oversizing did have a correlation with Oxford Knee score. This study confirms that neither medial or lateral overhang of the tibial component influences functional outcome, independent of the severity of overhang. Oversizing the tibial component however, does show worse functional outcome scores at a 5 year review.

Dual Window Approach With Two-Side Screw Fixation for Isolated Talonavicular Arthrodesis.

Isolated talonavicular arthrodesis is a commonly performed surgical intervention. Nonunion is a dreaded complication. The aim of the present study was to analyze the clinical and radiologic outcome of talonavicular arthrodesis using a dual approach with 2-side screw fixation. From February 2012 to September 2014, isolated talonavicular arthrodesis was performed on 17 joints of 16 patients (mean age 59.12 years). All procedures were performed by a single surgeon. The incidence of union, visual analog scale scores, and complications were analyzed. Radiographic union was achieved in all 17 cases (100%) at a mean period of 13.12 weeks. The visual analog scale scores had improved significantly (p < .001) at a mean follow-up of 48.53 weeks. One patient (6%) had minimal wound problems, and one (6%) showed prolonged swelling postoperatively. We have concluded that the dual window approach with 2-side screw fixation for isolated talonavicular arthrodesis results in excellent clinical and radiographic results and high fusion rates.

Soft tissue sarcoma of the extremities: pending questions on surgery and radiotherapy.

Soft tissue sarcomas are uncommon tumours of mesenchymal origin, most commonly arising in the extremities. Treatment includes surgical resection in combination with radiotherapy. Resection margins are of paramount importance in surgical treatment of soft tissue sarcomas but unambiguous guidelines for ideal margins of resection are still missing as is an uniform guideline on the use of radiotherapy.The present paper reviews the literature on soft tissue sarcomas of the extremities regarding the required resection margins, the impact of new radiotherapy techniques and the timing of radiotherapy, more particularly if it should be administered before or after surgical resection.This review was started by searching guidelines in different databases (National Guideline Clearinghouse, EBMPracticeNet, TRIP database, NCCN guidelines,…). After refinement of the query, more specific articles were found using MEDLINE, PubMed, Web of Science and Google Scholar. Used keywords include "soft tissue sarcoma"; "extremities OR limbs"; "radiotherapy", "surgery", "margins", "local recurrence" and "overall survival". Finally, the articles were selected based on the accessibility of the full text, use of the English language and relevance based on title and abstract.Literature demonstrates positive resection margins to be an important adverse prognostic factor for local recurrence of soft tissue sarcomas of the extremities. Still, no consensus is reached on the definition of what a good margin might be. The evolution of new radiation techniques, especially Intensity Modulated Radiotherapy, resulted in a s healthy surrounding tissues. However, the timing of radiotherapy treatment remains controversial as both preoperative and postoperative radiotherapy are characterised by several advantages and disadvantages.

Surgical treatment of the adult acquired flexible flatfoot.

In this review article, the authors give an overview of the currently available soft tissue and bony procedures in the treatment of the adult acquired flexible flatfoot. Instead of starting from the classification for posterior tibial tendon dysfunction, described by Johnson and Storm, the authors address the flatfoot from a more anatomical point of view. Based on this, they will try to define a treatment algorithm.

Remodeling of the Proximal Tibia Subsequent to Bone Graft Harvest: Postoperative CT Study.

BACKGROUND: Proximal tibia bone autograft (PTBG) is regularly used in reconstructive foot, ankle, and maxillofacial surgery. Although many surgeons prefer PTBG rather than the conventional iliac crest, little is known about the remodeling capacity of the proximal tibia after harvesting cancellous bone. METHODS: Via bilateral postoperative computed tomography of 17 harvesting sites, comparing the defect side with the healthy side, we measured the repair capacity of the proximal tibia in response to bone defect created by the harvest at medium-term follow-up (mean 29 months; range, 7-55 months). RESULTS: 16 of 17 (94%) cortical defects showed complete consolidation. Cancellous remodeling was graded 0 to 3, with 2 defects showing complete remodeling (grade 3), 4 defects partial remodeling (more than 50%), 8 defects partial remodeling (less than 50%) and 3 defects no remodeling at all (grade 0). CONCLUSION: The proximal tibia has the potential to form new cancellous bone after cancellous bone graft harvesting. More data are required to identify possible variables influencing this remodeling capacity. When performing knee surgery, knee surgeons should take into account the relatively small defect size and the consolidation of the cortical window after proximal tibia bone harvesting. LEVEL OF EVIDENCE: Level IV, case series.

Sarcomatous degeneration in fibrous dysplasia of the rib cage.

Malignant degeneration in fibrous dysplasia is a rare occurrence. Most cases are reported in polyostotic fibrous dysplasia with predisposition of the femur, tibia, maxilla, and mandible. The most commonly observed malignant tumors are osteosarcoma, fibrosarcoma, and chondrosarcoma. We describe a case of a low-grade osteosarcoma occurring in polyostotic fibrous dysplasia of the rib cage in a 50-year-old man.

Cytokine production by infrapatellar fat pad can be stimulated by interleukin 1ß and inhibited by peroxisome proliferator activated receptor α agonist.

BACKGROUND: Infrapatellar fat pad (IPFP) might be involved in osteoarthritis (OA) by production of cytokines. It was hypothesised that production of cytokines is sensitive to environmental conditions. OBJECTIVES: To evaluate cytokine production by IPFP in response to interleukin (IL)1ß and investigate the ability to modulate this response with an agonist for peroxisome proliferator activated receptor α (PPARα), which is also activated by lipid-lowering drugs such as fibrates. METHODS: Cytokine secretion of IPFP was analysed in the medium of explant cultures of 29 osteoarthritic patients. IPFP (five donors) and synovium (six donors) were cultured with IL-1ß and PPARα agonist Wy14643. Gene expression of IL-1ß, monocyte chemoattractant protein (MCP1), (IL-6, tumour necrosis factor (TNF)α, leptin, vascular endothelial growth factor (VEGF), IL-10, prostaglandin-endoperoxide synthase (PTGS)2 and release of TNFα, MCP1 and prostaglandin E(2) were compared with unstimulated IPFP and synovium explants. RESULTS: IPFP released large amounts of inflammatory cytokines, adipokines and growth factors. IL-1ß increased gene expression of PTGS2, TNFα, IL-1ß, IL-6 and VEGF and increased TNFα release in IPFP. MCP1, leptin, IL-10 gene expression and MCP1, leptin and PGE(2) release did not increase significantly. Synovium responded to IL-1ß similarly to IPFP, except for VEGF gene expression. Wy14643 decreased gene expression of PTGS2, IL-1ß, TNFα, MCP1, VEGF and leptin in IPFP explants and IL-1ß, TNFα, IL-6, IL-10 and VEGF in synovium that responded to IL-1ß. CONCLUSION: IPFP is an active tissue within the joint. IPFP cytokine production is increased by IL-1ß and decreased by a PPARα agonist. The effects were similar to effects seen in synovium. Fibrates may represent a potential disease-modifying drug for OA by modulating inflammatory properties of IPFP and synovium.

Experience with a frontal core biopsy device in soft tissue and bone lesions.

OBJECTIVE: To assess the efficacy and cost of a new frontloading biopsy system, Spirotome® (system 1), in musculoskeletal lesions, and to compare the results with those obtained with commonly used biopsy devices. METHODS: System 1 was used in all soft tissue lesions (STL) and osteolytic bone lesions (OBL) of patients who presented at our department for CT-guided biopsy between January 2009 and June 2010. Accuracy and cost were compared to those of Bonopty® (system 2) and Tru-cut (system 3) procedures. RESULTS: The efficacy of system 1 was 85% in STL and 89% in OBL. The procedure was well tolerated and caused no complications. System 3 had an efficacy of 84% in STL and OBL combined. The efficacy of system 2 in OBL was 85%. The cost of single-use system 1 and system 2 was comparable, the cost of system 3 and multiuse system 1 compared to single-use system 1 was 25 and 7%, respectively. CONCLUSIONS: The efficacy of system 1 in biopsy of STL and OBL was better than that of system 3. In OBL, the efficacy of system 1 was better than that of system 2. In STL at hazardous locations and small OBL with a thin cortical shell, system 1 offers the advantage of variable length and controlled loading. In these cases, single-use system 1 was cost-effective when compared to surgical biopsy. The cost per procedure of multiuse system 1 was lower than of system 3.

Relationship of the Scarf valgus-inducing osteotomy of the calcaneus to the medial neurovascular structures.

BACKGROUND: The Scarf valgus inducing osteotomy of the calcaneus is an operative technique to correct varus deformity of the hindfoot. It is versatile with significant corrective power; however, the neurovascular structures are in close proximity on the medial side and thus may be harmed during the osteotomy. Moreover, because this type of osteotomy can cause a great lateral translation, traction of the medial neurovascular structures is possible. We performed an anatomic study to evaluate the medial soft tissues after a lateralizing Scarf-type calcaneal osteotomy. MATERIALS AND METHODS: The osteotomies were carried out on ten fresh-frozen cadaver specimens. We performed the osteotomy and induced valgus. Then we performed a medial dissection to identify the important medial structures: the medial and lateral plantar nerve (MPN , LPN) and the posterior tibial artery (PTA). We noted their relation to the osteotomy and their integrity. RESULTS: In several cases, one or more of the structures were sectioned. In five cases, all the structures crossed the osteotomy, four of which even a transection of one or both of the plantar nerves occurred. Although the PTA crossed the osteotomy in eight specimens, there was no transection of this structure. CONCLUSION: Scarf osteotomy of the calcaneus is a highly corrective osteotomy. However, caution must be exercised when performing as the medial neurovascular structures cross the osteotomy lines and transection can occur. CLINICAL RELEVANCE: When performing the osteotomy one should keep in mind that vigorous sawing and large displacement can cause damage to the medial neurovascular structures.

Recurrent chordoma of the L2 vertebra: a case report.

Chordoma is a rare slow-growing, locally invasive primary malignant bone tumour arising from notochord remnants. It is characterised by a high local recurrence rate. Most chordomas (60%) are found in the sacrococcygeal region: only 15% originate in the mobile spine. CT-scan and MRI can help evaluate the tumour extension. En bloc resection of the tumour mass is the standard treatment. The main prognostic factor is tumour-negative surgical margins. Due to the location of spinal chordomas, adjacent to vital neural and vascular structures, this can be difficult to achieve. Therefore radiotherapy has been used when adequate excision is not possible and in case of local recurrence. We report the case of a 72-year-old female with a recurrent chordoma of the L2 vertebra, previously treated with resection and radiotherapy. The recurrent chordoma was resected using a right-sided thoraco-phreno-laparotomy as the tumour could not be resected using the left anterior approach or posterior approach due to extensive fibrosis following surgery and radiotherapy.

Post-traumatic incongruent hip in a 12-year-old boy.

Traumatic hip dislocation is uncommon in the paediatric population. Post reduction radiographs often show an incongruent hip because of tissue interposition. We report the case of a 12-year-old boy who was transferred to our hospital with an incongruent hip after a skiing accident, without a history of hip dislocation.