Histopathology of giant cell tumors of the bone: With special emphasis on fibrohistiocytic and aneurysmal bone cyst like components

Objective

The aim of this study was to define histopathological features of giant cell tumor of bone, especially accompanying fibrohistiocytic or aneurysmal bone cyst like components, in the light of our institutions experience.

Tomographic and histomorphometric evaluation of socket healing after tooth extraction using leukocyte- and platelet-rich fibrin: A randomized,single-blind,controlled clinical trial

The use of platelet concentrate in alveolar ridge preservation has been broadly studied. However, no randomized clinical trials with histomorphometric analysis and low risk of bias are available in the literature. We conducted a prospective, single-blind, parallel, randomized, controlled clinical trial to evaluate the efficacy of leukocyte- and platelet-rich fibrin (L-PRF) in socket preservation after tooth extraction. Additionally, the effect of L-PRF on bone formation was analyzed histologically using bone biopsy specimens obtained during implant placement.A total of 48 subjects who underwent a non-molar tooth extraction were randomly assigned to the L-PRF group (n = 24) or the control group (n = 24). Cone-beam computed tomographies were performed immediately after tooth extraction and at 3 months after tooth extraction, prior to implant surgery. A significant difference in bone resorption was registered 1 mm below the crest: 0.93 ± 0.9 mm for the L-PRF group and 2.27 ± 1.2 mm for the control group (p = 0.0001). Histomorphometric analysis showed a higher percentage of new bone formation in the L-PRF group compared with the control group. The values were 55.96 ± 11.97% and 39.69 ± 11.13%, respectively (p = 0.00001). These findings indicate that the administration of L-PRF should always be considered when socket preservation is planned (Clinicaltrials.gov NCT03408418).     

Rapid in-situ hybridization and immunohistochemistry: a pilot comparative study of two rapid diagnostic techniques for establishing monoclonality in plasma cell dyscrasias

BackgroundLight chain restriction needs to be established on the paraffin embedded tissue in certain types of plasma cell dyscrasias when serum levels of monoclonal immunoglobulins and light chain assays in the urine and serum may be normal. Rapid-in-situ-hybridisation (RISH) is thought to be a superior to immunohistochemistry (IHC) for kappa and lambda staining due to brighter and crisp staining without any background.MethodsFifty cases were included in this pilot study. Serum light chain restriction status of the case was taken as gold standard. The results of standard IHC for kappa and lambda immunoglobulins on the bone marrow biopsy of these cases was compared with RISH performed by the two commercially available kits. The results of the two methods were compared for sensitivity, need to repeat the test and background staining.ResultsThe study found that in IHC first run sensitivity was 58% which improved to 88% after the second run. For RISH the sensitivity was 100%.ConclusionRapid-in-situ-hybridisation (RISH) is a superior technique to IHC for detecting kappa and lambda light chain in plasma cells. The test is as labour intensive and time consuming as the routine IHC but has no background staining with more bright and crisp staining quality.     

Support system of Lisfranc joint complex: An anatomical investigation with an evolutionary perspective

BackgroundThe anatomical arrangement of the Lisfranc joint between the midfoot and forefoot is complex and not just critical for bipedal gait but also for prevention, management, and rehabilitation of injuries in this region.Material and methodsIn forty adult cadaveric lower limbs, the Lisfranc mortise, the ligaments and supports were observed and noted.ResultsThe structural arrangement that accords stability to the joint has osseous, ligamentous, and tendinous components. A bony mortise, which is deep medially, disrupts the linearity of the joint line. An extensive Lisfranc ligament with confluent interosseous and plantar parts was observed. Tibialis posterior, peroneus Longus and Lisfranc ligament exhibit a unique anatomical arrangement that supports the joint inferiorly.ConclusionThe study documents a unique lattice of tendons and ligament offering dynamic support to the joint. Demands of assumption of erect posture and bipedal walking in humans like adduction of the first ray of the foot, maintenance of longitudinal and transverse arches of the foot and ability stiffen midfoot for efficient forefoot take-off are well reflected in the joint structure and supports.     

Genetic syndromes predisposing to paediatric brain tumours: the chicken or the egg?

Cancer in childhood is a disorder of growth and development. Up to 10% of patients diagnosed with cancer during childhood have a known underlying genetic predisposition syndrome. Affected individuals usually have multisystem involvement from the underlying syndrome and certain syndromes are associated with development of characteristic tumours with sites of predilection within the neuraxis. For the healthcare professionals involved with paediatric patients it is important to have basic knowledge of the cancer susceptibility syndromes. A holistic multidisciplinary approach is required for the overall management of the syndrome itself with specific recommendations for imaging surveillance and genetic counselling based on the pattern of inheritance and the relative risk of developing a tumour. Appropriate knowledge of these syndromes will help paediatricians manage and refer patients at risk to specialist neuro-oncology centres. A typical brain tumour diagnosis can also indicate certain underlying genetic disorders and examples of such tumours include optic pathway glioma, choroid plexus carcinoma and subependymal giant cell astrocytoma. A detailed family history can be helpful in identifying at risk patients and families as the typical clinical signs associated with the genetic condition are often not fully apparent in young children. This article focuses on well-known genetic diagnoses associated with or predisposing to childhood brain tumours. In some instances, the brain tumour diagnosis subsequently leads to the diagnosis of an underlying genetic syndrome.     

How to implement guidelines and models of care

In subjects older than 50 years, the presence of clinical risk factors (CRFs) for fractures or a recent fracture is the cornerstone for case finding. In patients who are clinically at high short- and long-term risk of fractures (those with a recent clinical fracture or with multiple CRFs), further assessment with bone mineral density (BMD) measurement using dual-energy absorptiometry (DXA), imaging of the spine, fall risk evaluation and laboratory examination contributes to treatment decisions according to the height and modifiability of fracture risk. Treatment is available with anti-resorptive and anabolic drugs, and from the start of treatment a lifelong strategy is needed to decide about continuous, intermittent, and sequential therapy. Implementation of guidelines requires further initiatives for improving case finding, public awareness about osteoporosis and national policies on reimbursement of assessment and therapy.     

A case of VIPoma

This case report addresses the management of a pregnant woman in the peripartum period with a VIPoma. This rare and highly malignant neuroendocrine tumour secretes vasoactive intestinal peptide (VIP), a substance that may cause potentially life-threatening disruption to physiology.A 36-year-old woman presented for induction of labour with a three-year history of chronic diarrhoea, hypophosphataemia, palpitations and skin flushing. Raised VIP levels indicated presence of a VIPoma, however despite extensive investigation prior to pregnancy by neuroendocrine specialists, the tumour location remained unidentified.The patient delivered a healthy boy with the aid of forceps in theatre following an epidural top-up. Key features of management were a multidisciplinary approach, avoidance of triggers for VIP secretion, strict management of electrolytes and avoidance of severe changes in sympathetic tone during labour with epidural analgesia.     

Current surgical management of metastatic pathological fractures of the femur: A multicentre snapshot audit

AimsWe aimed to find out: the typical workload for metastatic bone disease, the conventional treatment for femoral metastases and whether there is a trend for arthroplasty and endoprosthetic reconstruction.Materials and methodsAll sequential patients undergoing surgery for femoral metastatic lesions (both pathological fracture and impending pathological fracture) of any age patient were included in the multicenter snapshot audit. Data on demographics, institutions and operative procedures were recorded.Results24 UK Institutions were enrolled, including 7 Major Trauma Centres (MTCs). It was a 2 month audit from 1^stMarch 2018. 95 cases were recorded. The mean age was 71 and 65% were female. 66 patients had a fracture at presentation and 23 an impending fracture. Breast carcinoma was the primary tumour at 23%. The mean Mirel’s score is 9. The commonest fixation was with a long cephalomedullary nail (38%). Endoprostheses accounted for 24%. None of the endoprostheses were implanted at MTCs.ConclusionThis audit revealed large numbers of cases of femoral metastases. Although the use of endoprostheses may be increasing in Trauma Units, intramedullary nailing still predominates. Future pathways may benefit from directing resources to allow greater arthroplasty.     

Outcomes,Prognostic Factors and Salvage Treatment for Recurrent Chordoma After Pencil Beam Scanning Proton Therapy at the Paul Scherrer Institute

AimsThe outcome of chordoma patients with local or distant failure after proton therapy is not well established. We assessed the disease-specific (DSS) and overall survival of patients recurring after proton therapy and evaluated the prognostic factors affecting DSS.Materials and methodsA retrospective analysis was carried out of 71 recurring skull base (n = 36) and extracranial (n = 35) chordoma patients who received adjuvant proton therapy at initial presentation (n = 42; 59%) or after post-surgical recurrence (n = 29; 41%). The median proton therapy dose delivered was 74 GyRBE (range 62–76). The mean age was 55 ± 14.2 years and the male/female ratio was about one.ResultsThe median time to first failure after proton therapy was 30.8 months (range 3–152). Most patients (n = 59; 83%) presented with locoregional failure only. There were only 12 (17%) distant failures, either with (n = 5) or without (n = 7) synchronous local failure. Eight patients (11%) received no salvage therapy for their treatment failure after proton therapy. Salvage treatments after proton therapy failure included surgery, systemic therapy and additional radiotherapy in 45 (63%), 20 (28%) and eight (11%) patients, respectively. Fifty-three patients (75%) died, most often from disease progression (47 of 53 patients; 89%). The median DSS and overall survival after failure was 3.9 (95% confidence interval 3.1–5.1) and 3.4 (95% confidence interval 2.5–4.4) years, respectively. On multivariate analysis, extracranial location and late failure (≥31 months after proton therapy) were independent favourable prognostic factors for DSS.ConclusionThe survival of chordoma patients after a treatment failure following proton therapy is poor, particularly for patients who relapse early or recur in the skull base. Although salvage treatment is administered to most patients with uncontrolled disease, they will ultimately die as a result of disease progression in most cases.