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 Grade Anatomical location

Metastases

G0 Benign T1 Intra-compartmental

M0 No mets

G1 Low Grade T2 Extra-compartmental

M1 Metastases

G2 High Grade

T1 T2

G1 IA IB

G2 IIA IIB

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 3rd and 4th 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 7th – 8th 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 5th – 6th 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 3rd – 4th 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)