Primary tracheobronchial lipoma is an extremely rare entity, the diagnosis of which is often missed initially. Cases are generally diagnosed late after initial treatment for asthma and bronchitis. We report a case of a 42-year-old man with a left main bronchus lipoma that caused near-total obstruction. The lipoma was treated by bronchoscopic resection with an electrosurgical snare and cryoablation.

MOHs micrographic surgery is a technique of microscopic margin control in the surgical management of skin cancers particularly at cosmetically sensitive sites. This review article is aimed at sharing our initial experience of performing MOHs surgery for skin cancers in Malaysia since 2015.

INTRODUCTIONGranular cell tumours (GrCTs) are uncommon soft tissue tumours that are usually benign (approximately 0.5%-2.0% have been reported as malignant). They are very rarely found at the extremities. Differentiating a malignant GrCT from a benign one is important as the former is aggressive and has a poor prognosis, whereas the latter, after surgical resection, has excellent outcomes. A malignant lesion can be suspected on clinical presentation and confirmed via histopathological examination using the Fanburg-Smith criteria.

METHODSThis was a retrospective review of all cases of GrCT of the extremities that presented to the Orthopaedic Oncology Unit of University Malaya Medical Centre, Malaysia, from September 2006 to March 2013.

RESULTSThere were a total of five cases, all of which involved female patients aged 13-40 (mean age 24) years. Three cases involved the upper limbs and two involved the lower limbs. Using the Fanburg-Smith criteria, three cases were classified as benign, one as atypical and one as malignant. Wide local excision was performed in all five cases and the outcomes were excellent except for the patient with a malignant tumour. That patient presented with lung metastasis about three months after surgery.

CONCLUSIONMalignant and benign GrCTs can be differentiated on clinical presentation and by using the Fanburg-Smith criteria. We believe that wide local excision is the best treatment for both benign and malignant tumours. The role of adjuvant chemotherapy and radiotherapy in malignant GrCTs should be studied. All patients with GrCTs should receive follow-up to check for recurrence and metastasis.

Adolescent ; Adult ; Female ; Granular Cell Tumor ; diagnosis ; surgery ; Humans ; Magnetic Resonance Imaging ; Malaysia ; Neoplasm Recurrence, Local ; Prognosis ; Retrospective Studies ; Soft Tissue Neoplasms ; diagnosis ; surgery ; Treatment Outcome ; Young Adult

Intra-articular haemangioma is a rare and uncommon condition that sometimes presents in infants. The lesion can be a diagnostic challenge, with misdiagnosis often leading to delayed diagnosis and treatment. It is essential to establish and treat the condition early, as intra-articular haemangioma can lead to destruction of the joint and secondary arthrosis. Herein, we report the case of a five-year-old boy who presented with intra-articular haemangioma and discuss the management of his condition.
Arthralgia ; physiopathology ; Biopsy, Needle ; Child, Preschool ; Follow-Up Studies ; Hemangioma ; diagnosis ; pathology ; surgery ; Humans ; Immunohistochemistry ; Joint Capsule ; pathology ; surgery ; Knee Joint ; pathology ; surgery ; Magnetic Resonance Imaging ; methods ; Male ; Orthopedic Procedures ; methods ; Rare Diseases ; Risk Assessment ; Synovectomy ; Synovial Membrane ; pathology ; Treatment Outcome

A variety of ligands differ in their capacity to bind the receptor, elicit gene expression, and modulate physiological responses. Such receptors include Toll-like receptors (TLRs), which recognize various patterns of pathogens and lead to primary innate immune activation against invaders, and G-protein coupled receptors (GPCRs), whose interaction with their cognate ligands activates heterotrimeric G proteins and regulates specific downstream effectors, including immuno-stimulating molecules. Once TLRs are activated, they lead to the expression of hundreds of genes together and bridge the arm of innate and adaptive immune responses. We characterized the gene expression profile of Toll-like receptor 4 (TLR4) in RAW 264.7 cells when it bound with its ligand, 2-keto-3-deoxyoctonate (KDO), the active part of lipopolysaccharide. In addition, to determine the network communications among the TLR, Janus kinase (JAK)/signal transducer and activator of transcription (STAT), and GPCR, we tested RAW 264.7 cells with KDO, interferon-beta, or cAMP analog 8-Br. The ligands were also administered as a pair of double and triple combinations.
Arm ; Gene Expression ; GTP-Binding Proteins ; Heterotrimeric GTP-Binding Proteins ; Immunity, Innate ; Interferon-beta ; Ligands ; Macrophages ; Phosphotransferases ; Sugar Acids ; Toll-Like Receptor 4 ; Toll-Like Receptors ; Transcriptome ; Transducers

Melanoma is a lethal skin cancer that occurs predominantly among Caucasians. In Malaysia, the incidence of melanoma is low. This is a retrospective study of clinical and histopathological features of patients with cutaneous melanoma who were seen at the University Malaya Medical Centre from 1998 to 2008. Thirty-two patients with cutaneous melanoma were recorded during that period. Of these, 24 had sought treatment at the onset of disease at our centre. Chinese patients constituted the largest group (19 cases). The median age of these 24 patients at the time of presentation was 62 years. 16 patients had melanoma involving the lower limb with 12 affecting the sole of the foot. None had melanoma arising from the face. Histopathology showed nodular melanoma in 22 cases (91.6%), with superfi cial spreading and acral lentiginous melanoma diagnosed in 1 case each. The majority of patients (62.5%) were found to be in Stage III of the disease at the time of diagnosis.

Vagal nerve schwannoma is extremely rare. The majority of cases present with a slow growing neck swelling without neurological deficit. Magnetic resonance imaging is the gold standard investigation to establish a pre-operative diagnosis. We report a case of a 32-year-old man with an extensive right vagal nerve schwannoma involving the right jugular foramen and parapharyngeal space. The tumour was resected via a transcervical approach. Complete excision of the tumour is the key to prevent recurrence.

Porokeratosis is a specific disorder of epidermal keratinization, characterised histologically by the presence of cornoid lamella1. Linear is a distinct, mosaic variant of this autosomal dominant condition. There is a well recognized association between porokeratosis and malignancy, especially the linear variant which has the highest malignant potential2, 3.

Objective: In this study, gelatinase producing bacteria were probed from sediment samples of Porto Novo Coastal sites, India. Screening and identification of potential strain were done followed by optimization of physico-chemical parameters; bulk production and gelatinase extraction were carried out. Methods: For probing of gelatinase potential producer primary and secondary screening was carried out for qualitative and quantitative estimation. Optimization of physico-chemical parameters for improved production of gelatinase enzyme and large scale of gelatinase was produced. Gelatinase precipitation was standardized using different saturation rates of ammonium sulphate from 10 to 100% at 4℃.Results: There were 8 morphologically different gelatinase producing bacteria were initially delved through primary screening tests. Bacillus spp produced maximum gelatinase activity (2.1U/mL) in secondary screening test. Optimizing its abiotic and biotic factors, maximum enzyme activity was achieved at 48h incubation period (2.2U/mL), 2.5 pH (2.5U/mL), 35℃ temperature (2.55U/mL), 0.8% lactose (2.6U/mL), 1.4% gelatin (2.9U/mL) as the ideal carbon source and nitrogen source, 1% salinity (2.9U/mL) and 3ml of inoculum containing 5.6×106/ mL (3.3U/mL). From the optimized factors, bulk production was carried out and saturation rate of 40% ammonium sulphate, precipitated out maximum enzyme with lowered dry weight indicates its enzyme purity and recovered enzyme showed 4.1U/mg of activity. Conclusion: The study revealed that the isolated strain Bacillus spp has its potentiality for industrial scale production and the results will stand as a base line data for the application of gelatinase in future.

Extra-adrenal paragangliomata are uncommon entities. They can be classified into four basic groups according to their anatomical sites, i.e. branchiomeric, intravagal, aorticosympathetic and visceral autonomic. Similar tumours may arise in sites away from the usual distribution of the sympathetic and parasympathetic ganglia, e.g. orbit, nose, small intestine and even in the pancreas. We report three instructive cases of extra-adrenal paraganglioma which were found in unusual sites such as urinary bladder, thyroid gland and on the wall of the inferior vena cava.
Paraganglioma, Extra-Adrenal ; Lower case ee ; g <3> ; agreement ; inferiority