The incidence and relevance of non-fatty components in trunk and extremity lipomatous soft tissue masses

Objectives:To determine the incidence and diagnostic relevance of non-fatty ‘solid appearing’ components within lipomatous tumours of the trunk and extremity.Methods and materials:Retrospective review of patients referred to a specialist musculoskeletal oncology service over a 12-month period with a lipomatous trunk or extremity soft tissue tumour. The presence and morphology (solitary/multifocal; homogeneous/heterogeneous; well-defined/poorly defined) of non-fatty components was recorded based on MRI and compared with the final histological diagnosis.Results:213 patients with 217 lipomatous tumours were included, 119 (55.9%) males and 94 (44.1%) females with mean age of 54.6 years (range 7–93 years). Seventy-seven (35.5%) lesions arose superficial to the fascia and 139 (64.1%) deep, while a single case involved both compartments. Mean maximal tumour dimension was 94.9 mm (range 12–288 mm). Non-fatty ‘solid appearing’ components were identified in 28 (12.9%) cases, of which eight were solitary and 20 were multifocal, six had homogeneous SI and 22 had heterogeneous SI, and eight had well-defined margins, while 20 had poorly defined margins. Histological diagnosis was available in 20 of the tumours containing non-fatty components, 16 of which were benign, two intermediate grade and two malignant (a dedifferentiated liposarcoma and a myxoid liposarcoma). The commonest diagnosis was spindle cell lipoma, which accounted for 10 of 20 (50%) cases with confirmed histology.Conclusions:Non-fatty components are identified in ~13% of trunk and extremity lipomatous tumours. The majority of such lesions are benign lipoma variants, most commonly spindle cell lipoma.Advances in knowledge:Solid non-fatty components are identified in approximately 13% of lipomatous tumours referred to a specialist sarcoma service. Despite the concern that these may represent dedifferentiated liposarcomas, high-grade tumours were seen in only two cases, the commonest diagnosis being a spindle cell lipoma.     

A rare case of primary ovarian carcinoid

We present a rare case in an elderly Caucasian lady of primary ovarian carcinoid tumor, causing symptoms of the carcinoid syndrome and severe carcinoid heart disease. Carcinoid tumors are the commonest neuroendocrine tumors, the majority of which are found in the gastrointestinal tract and lungs. Ovarian carcinoids represent only 0.1% of all ovarian neoplasms and 1% of all carcinoid tumors. Carcinoid syndrome can present with flushing, diffuse watery diarrhea, wheezing and breathlessness and in advanced cases lead to carcinoid heart disease. This involves damage to the right heart structures and pulmonary hypertension, which can cause significant breathlessness and peripheral edema, and is associated with a worse prognosis. Diagnosis involves multi-modality radiological imaging and biochemical analysis of neuroendocrine tumor markers, and the first line treatment should always be resection of the tumor where possible. Prognosis is generally favorable, except in some cases with metastasis.     

Structure, chemical composition and mechanical properties of coronal cementum in human deciduous molars

ObjectivesIt was hypothesized that the coronal cementum containing collagen forms a weak junction with enamel unlike the well integrated DEJ and CDJ.MethodsThe hypothesis was investigated in two parts: (1) evaluate the structure, chemical composition and mechanical properties of coronal cementum and its junction with enamel using scanning electron microscopy, micro-X-ray computed tomography, and atomic force microscopy. The chemical composition and mechanical properties were determined by evaluating the spatial variations of inorganic (PO₄^3? ν₁ mode at 960 cm^?1) and organic (C–H deformation at 1452 cm^?1; C–H stretch at 2940 cm^?1) contents using Raman microspectroscopy and elastic modulus and hardness values using nanoindentation. (2) Estimate the strength and evaluate the microstructure of coronal cementum interface with enamel using SEM and MicroXCT?.Results and conclusionsCoronal cementum is heterogeneous because it is a combination of laminar acellular afibrillar cementum and acellular extrinsic fiber cementum with relatively higher organic content. It integrates micromechanically via a scallop-like weak interface with enamel unlike the biomechanically efficient DEJ and CDJ and is continuous with primary root cementum. A single tooth could exhibit all three types of cementum enamel junctions; an overlap, butt and a gap depending on the sectioning plane. The elastic modulus of coronal cementum (11.0 ± 5.8 GPa) is significantly lower (p < 0.05; Student's t-test with 95% confidence interval) than primary cementum (15.8 ± 5.3 GPa).     

INVESTIGATION OF BREAST LUMPS: AN EVALUATION

70 patients with 72 palpable breast lumps were assessed by Physical Examination (PE), Mammography (MG), Ultrasound Scan (USS) and Fine Needle Aspiration Cytology (FNAC). The individual and collective value of these investigations have been assessed and a cost effective algorithm suggested.KEY WORDS: FNAC, Mammography, Palpable breast lump, Physical examination     

Gene Delivery in Neuro-Oncology

Purpose of Review

Glioblastoma multiforme (GBM) is the most common primary malignant brain tumor in adults with a dismal prognosis despite aggressive multimodal management thus novel treatments are urgently needed. Gene therapy is a versatile treatment strategy being investigated in multiple cancers including GBM. In gene therapy, a variety of vectors or “carriers” are used to deliver genes designed for different anti-tumoral effects. Gene delivery vehicles and approaches to treatment will be addressed in this review.

Recent Findings

The most commonly studied vectors are viral based, however, driven by advances in biomedical engineering, mesenchymal and neural stem cells, as well as multiple different types of nanoparticles have been developed to improve tumor tropism and also increase gene transfer into tumor cells. Different genes have been studied including suicide genes, which convert non-toxic prodrug into cytotoxic drug; immunomodulatory genes, which stimulate the immune system; and tumor suppressor genes which repair the defect that allow cells to divide unchecked.

Summary

Gene therapy may be a promising treatment strategy in neuro-oncology as it is versatile and flexible due to the ability to tailor vectors and genes for specific therapeutic activity. Pre-clinical studies and clinical trials have demonstrated feasibility and safety of gene therapy; however, further studies are required to determine efficacy.

     

Dermatofibrosarcoma Protuberans: A comprehensive review on the spectrum of clinico‐radiological presentations

Dermatofibrosarcoma Protuberans (DFSP) is a rare malignant soft‐tissue neoplasm which is often misdiagnosed due to its indolent clinical course and non‐specific radiological appearances. An observation case series was conducted with retrospective review of clinical and radiological data of DFSP patients presenting to a major tertiary hospital in Hong Kong for radiological assessment between November 2006 and February 2016. Seven patients with confirmed histological diagnosis of DFSP were included. Tumour sizes at presentation ranged from 1 to 5 cm, most commonly (n = 6) occurred over chest wall and abdominal wall. History of previous local trauma or surgery was identified in the majority of cases (n = 4). There was poor correlation between pre‐imaging clinical diagnoses and pathological diagnoses. Local recurrence and tumour de‐differentiation with sarcomatous changes occurred in the minority of cases (n = 2). A common radiological ‘claw’ sign at the lesion/skin interface formed by elongated appendages of the tumour superficially was appreciated in most cases (n = 6). A history of previous local trauma or surgery serves as a possible etiological factor for the development of DFSP. High clinical suspicion for the entity is essential in its detection and differentiation from simple wound complications and local recurrence of other benign lesions. The radiological ‘claw’ sign at the lesion/skin interface might serve as a tell‐tale sign for cutaneous tumour involvement. A comprehensive analysis of imaging findings in conjunction with individual clinical presentations is the key to accurate diagnoses and proper management.     

A model for transport of membrane-associated phototransduction polypeptides in rod and cone photoreceptor inner segments

We discuss putative mechanisms of membrane protein transport in photoreceptors based on Pde6d and Gucy2e/Gucy2f knockout mice. Knockout of the Pde6d gene encoding PrBP/delta, a prenyl binding protein present in the retina at relatively high levels, was shown to impair transport of G-protein coupled receptor kinase 1 (GRK1) and cone phosphodiesterase alpha' subunit (PDE6alpha') to the rod and cone outer segments. Other prenylated proteins are minimally affected, suggesting some specificity of interaction. Knockout of the Gucy2e gene encoding guanylate cyclase 1 (GC1) disrupted transport of G-protein coupled receptor kinase 1 (GRK1), cone PDE6alpha', cone transducin alpha and gamma subunits (cTalpha and cTgamma) to the cone outer segments, while a GC1/GC2 double knockout prevented transport of rod PDE6, but not transducin, GRK1, or rhodopsin, to the rod outer segments. These knockout phenotypes suggest that PrBP/delta functions in extracting prenylated proteins from the endoplasmic reticulum (ER) where they dock after prenylation, and that GC-bearing membranes may co-transport peripheral membrane proteins in vesicles. We conclude that distinct pathways have evolved in rods and cones for transport of integral and peripherally membrane-associated proteins.     

Who pays for health care in Asia?

We estimate the distributional incidence of health care financing in 13 Asian territories that account for 55% of the Asian population. In all territories, higher-income households contribute more to the financing of health care. The better-off contribute more as a proportion of ability to pay in most low- and lower-middle-income territories. Health care financing is slightly regressive in three high-income economies with universal social insurance. Direct taxation is the most progressive source of finance and is most so in poorer economies. In universal systems, social insurance is proportional to regressive. In high-income economies, the out-of-pocket (OOP) payments are proportional or regressive while in low-income economies the better-off spend relatively more OOP. But in most low-/middle-income countries, the better-off not only pay more, they also get more health care.