Hand Tumours

Most common hand tumours 95% hand tumours comprised of: – Ganglion – Giant Cell Tumour of tendon sheath – Epidermoid Inclusion Cysts – Haemangioma – Lipoma Other tumours arise from bone & cartilage, muscle, nerve, skin & adnexae, subcutaneous tissue, tendon and synovium, vessel

Mankin’s Hand Tumour Axioms Primary bone tumours are usually benign The most common bone tumours are enchondroma and osteocartilaginous exostosis, except in distal phalanx which is epidermal inclusion cyst Deep soft tissue tumours (other than ganglion, lipoma, GCT) are often malignant Epithelioid sarcoma, synovial sarcoma and clear cell sarcoma are common malignancies in hand/forearm. They are highly malignant and may metastasize to lymph nodes Synovial chrondromatosis, synovial sarcoma and liposarcoma may show calcification on radiology Metastatic carcinoma, except lung, lymphoma and myeloma are rarely seen in the hand

History Age Duration Pain Neurological effects Nature of onset Variation in size Previous similar swellings Similar swellings elsewhere Symptoms related to other sites

Examination – S3, C2, M (NI) Size Site Shape Colour Consistency Mobility Nodes Imaging

Investigations Plain Xray – Calcification – Anatomical location – Changes in bony architecture – Response of host bone – Internal contents Xeroradiography Bone scans, CT, MRI, Angiogram CXR FBC, EUC, U/A – Also ESR, BSL, CMP, LFT, TFT, PTH

Enneking’s staging Criteria for G2 – Pain & rapid growth – Marked destruction on Xray – Extensive uptake on bone scan – Cortical disruption on CT – Satellite lesions on MRI – Biopsy = frequent mitoses, cellular atypia, poor differentiation and necrosis Compartments (T grade) – Intraosseous – Paraosseous – Intra articular – Intra muscular Some locations are not compartmentalised, so T2 by definition – Vascular plane – Mid hand – Antecubital fossa – Axilla Surgical GradeAnatomical location Metastases G0 BenignT1 Intra- compartmental M0 No mets G1 Low GradeT2 Extra- compartmental M1 Metastases G2 High Grade T1T2 G1IAIB G2IIAIIB

Ganglion Most common mass in the hand (50-70%) Probably arise from myxoid degeneration of collagen Lined with flattened mesothelium, not synovium, not secretory cells Can be found in association with any synovial lined cavity, but unlikely to be true synovial hernia 4 common sites – Dorsal wrist ganglia are the most common type of ganglion (60-70%), associated with scapholunate ligament – Volar wrist ganglion, may be associated with multiple ligaments – Proximal digital crease, associated with A1/A2 pulley – Distal interphalangeal joint 30-40% spontaneously resolve within the first year

Giant cell tumour of flexor sheath Second most common swelling in hand Arises in presence of synovial tissue (flexor sheath, IPJs) Usually asymptomatic Characteristic yellow brown colour from haemosiderin High recurrence rate due to perforations in macroscopic capsule Recurrence rates associated with multiple lesions, thumb lesions, presence of bony erosion, nm23 gene

Inclusion cyst Minor skin wound drives basal skin cells beneath skin surface Forms smooth, spherical tumour attached to skin but mobile over deep structures Almost exclusively palmar surface, most common around fingertips and amputation stumps Smooth lytic lesion, no calcification, mildly expansile, marginal sclerosis, cortex intact, no periosteal reaction

Glomus tumour Benign growth of cells of normal glomus apparatus, an a-v anastomosis in dermis used to control skin circulation Essentially a hamartoma Triad of symptoms – paroxysmal pain, pin point tenderness (Love’s test) and temperature sensitivity to cold Usually solitary, most commonly subungal Presents due to symptoms rather than mass Can precipitate pain with ethyl chloride spray If found can be seen as a small purplish patch MRI can detect 5mm lesions, U/S 2mm

Enchondroma 90% bone tumours of hand 35% enchondromas occur in hand Benign hyaline cartilaginous growth within medullary cavity Peaks in 3 rd and 4 th decades, M=F May degenerate into chondrosarcoma (<1%) heralded by unremitting pain, rapid growth and cortical disruption Painless swelling, often incidental finding or pathological fracture Central, usually in tubular bones, lytic with calcifications, expansile, margins well defined, cortex intact but often very thin Multiple enchondromatosis is found in Ollier’s disease and Maffuci syndrome – Rate of malignant degeneration is much higher, 30% in Ollier’s and up to 100% in Maffuci

Chondrosarcoma Most common malignant primary bone tumour in hand (60%), but still rare hand swelling 25% arise secondary to degenerative change of multiple enchondromatosis Peak incidence in 7 th – 8 th decades Pain Lies in subchondral bone, epiphyseal equivalent of tubular bones Central, scattered lysis with punctate calcifications, no expansile, no marginal sclerosis, late cortical disruption Associated soft tissue shadow with radiating spicules (flattened at ends unlike osteosarcoma), Codman’s triangle Requires wide margins, no role for radiotherapy or chemotherapy Histopath can be difficult 5 year survival 70%, 10 year survival 60-70%

Osteosarcoma Heterogenous group of malignant neoplasms Primary osteosarcoma peaks in 10-25yo, secondary (to Paget’s, radiation etc) peaks in 5 th – 6 th decades Aching, constant pain, worse at night Metaphyseal, destructive lytic lesion associated with widely variable amount of new bone formation Cortical disruption, spiculation, Codman’s triangle Skip lesions and metastases Bone scan + MRI to define lesion and skip lesions Staging Chest CT, and staging biopsy advisable Poor prognosis, dependent on duration, location (proximal worse), size, histological grade and presence of metastases – 10 year survival up to 70%

Rhabdomyosarcoma Embryonal form peaks in infancy, adult form peaks in young adults Actively growing soft tissue masses Routine Xrays show spread to bone, but better assessed by bone scan MRI and angiogram are important investigations Radical extracompartmental excision – Later generations of chemotherapy and radiotherapy may allow some limb sparing 5 year survival 50% embryonal, 30% adult

Giant cell tumour of bone Occurs in skeletally mature, peak incidence in 3 rd – 4 th decades, F 57% 2% GCT bone occur in hand, most commonly distal phalanx Intermittent pain and local swelling Epiphyseal equivalent, eccentric Central radiolucency, fine trabeculation, expansile, no marginal sclerosis, cortical erosion, soft tissue extension in later or more aggressive tumours Multiple GCT common, need bone scan to locate May develop secondary aneurysmal bone cysts 80% recurrence rate in hands (1/3 cured after 1 treatment, 1/3 after 2, 1/3 need > 2 treatments)