Tumors of the hand Flashcards
Cartilage Tumor: benign
Enchondroma
Definition:
most common bone tumor of the hand (90%), comprised hyaline cartilage islands seperated from the growth plate, slowly growing after the growth plate closed
no sex predilection
most commonly diagnosis in the 2nd or 3rd decade
most common:
proximal phalanx - metacarpus - middle phalanx with most common from ulnar to radial
carpus ralely involved
Presentation:
no growth, clinical symptoms by pathologic fracture, when growth maybe transformation into chondrosarcoma, less than 1% per year of observation
Imaging:
lucent lesion intramedullary, maybe matrix calcification - chondroid calcification, endosteal erosion with thinning the surrounding cortex
(cave: cortical remodelling and endosteal erosion can be the possibility of an low-grade chondrosarcoma)
Differential Diagnosis:
giant cell tumor
giant cell reparative granuloma
aneurysmal bone cyste (ABC)
intraosseous ganglion cyste
chondrosarcoma (cotical destruction and soft tissue mass)
Associated Conditions (Ollier/Maffucci):
M. ollier (mutiple enchondromas of one body half)
often eccentric cartilage lesions of the phalangeal bones, mostly first decade, 30% malignant transformation, hand 80-90% involved,
Maffucci Syndrome: enchondromatosis with deef cavernic haemangioma non osseus, 80-90% of the hand (100% maligne transformationj - higher than ollier disease)
Treatment:
curettage, bone filling oder PMMA, more stability, when pathologic fracture there is a better outcome, when first treated the fracture und delay curettage of the bone
maybe Phenol for the cavity to avoid recurrence
Complications/Recurrence:
Recurrence 5 - 14%, oberservation 2 - 5 years
transformation to chondrosarcoma is very rare
Cartilage Tumor: benign
Osteochondroma
very rare, 3-7% of the tumors of this area, only 4% of all osteochondromas, arise from a defect in the perichondral ring, allowing cartilage from the physis to protrude through, than calcify with cortical and trabecular bone and an associates cartilage cap with cortical and medullary continuity with the host bone, most presentation during the first 2 decades, predilection for the proximal phalanx
Presentation:
painless mass with symptoms resulting from affecting mechanical irritation to the surrounding soft tissue, growth away from the joint, when physis is open, ceasing at skeletal maturity
when found - evaluation for multiple hereditary exostosis
Imaging:
continuity between the cortex and medullary canal and the lesion
MRT ist necessary, bursa above the lesion or thick cartilage cap, maybe malignant transformation with mass of the soft tissue, irregularity of the cortical margin and heterogenous mineralization
growing cartilage cap (normal only a few milimeters) maybe sign for malignant transformation into a chondrosarcoma
Associated conditions:
multiple hereditary osteochondromas, autosomal dominant disorder, mutation in EXT of genes 1 and 2, first decade of life, limb-length discrepancies and deformities, 79% involving the hand, malignant transformation in 5-25%
Treatment:
if necessary waiting for skeletal maturity, malignant transformation maybe needs wide excision of the mass, amputation
Cartilage Tumor: benign
Chondroblastoma
benign cartilage tumors, epiphysis of long bones, associated with the growth plate, derive from this structure, mostly young male patients, 90% of chondroblastomas between 5-25years, in hand rare
mononuclear and giant cells, cartilage matrix may calcify in a “lice-lake” or “chicken-wire” pattern
Presentation:
mostly pain!!!! longstanding pain, swelling 10%, joint effusion, ROM limitation, when lesion at the joint line
Imaging:
eccentric lesions, epiphysis of the long bones, may cross the physis and involve the metaphysis, thin rim of sclerosis, may expand the overlying host bone cortex
exuberant surrounding bone marrow
soft-tissue swelling
joint effusion
Hand: not epiphyseal region - tendency to involve the diaphysis or metaphysis of the involved bone
Differential:
epiphyseal predilection: GCT, intraosseus Ganglion cyst, infection (Brodie abscess)
Carpus: ganglion cyst, osteoid osteoma, osteoblastoma, infection (Brodie abscess)
Treatment:
curettage, maybe secondary development of ABC, filling with autologous bone from the iliacal crest, Phenol application possible, maybe PMMA for the defect to see recurrence early
Complications:
when secondary ABC 100% Chondroblastoma recurrence
38% recurrence of all chondroblastoma
Cartilage Tumor: benign
Chondromas
periosteal Chondroma
rare benign lesions, from periosteoum or the connective tissue
second or third decade
lobulated fibrocarilaginous matrix with hyaline cartilage
25% involve bones of the hand, mostly proximal and middle phalanx in the diaphysis or metaphysis
Presentation:
painless enlarging mass, mechanical symptoms near to a joint, restricted ROM, snapping of tendons
Imaging:
associated with the surface of the bone with chronig extrinsic scalloping of the underlying bone, rim of sclerosis between the lesion and the medullary cavity, thin cortical shell of bone along the peripheral margin of the soft-tissue component of the mass, intralesional calcification in 50%
Differential Diagnosis
intracortical chondroma
surface ABC
osteoblastoma
periosteal chondrosarcoma
periosteal osteosarcoma
Treatment:
lokal excision with resection of the underlying cortex
Complications:
15% recurrence
Chondromas
soft-tissue Chondroma
uncommon benign tumor of the hand and feet, mostly 96%, 80% fingers - palmar aspect, inimately associated with tendons and arise from the surrounding synovial tissue
age from 30 to 60, not attached to the periosteum, histologically hyaline cartilage with chondrozyte maturation and associated with intrasubstance calcification, arranged in lobules and classically well-encapsulated
Cartilage Tumor: benign
Bizarre Parosteal Osteochondromatous Proliferation (BPOP)
“Nora’s Lesion”
on phalangeals, metacarpals and metatarsals, no sex predilection, third and fourth decade of life
most related to an injury (30%), trauma causes subperiosteal hemorrhage, than periostitis, etiology not clearly defined, histologically cartilage bone with spindle cells, cartilage cap overlying the lesion, irregular bone trabeculae, no communcation with the underlying host bone
Presentation:
painless slow-growing mass, nearly a joint maybe restricted range of motion
Imaging:
surface lesion, present a nodular, densely mineralized masses on the bone surface, (likely osteochondroma), no medullary continuity, arise directly from the periosteum, no disruption of the unterlying cortical bone
heavily mineralized masses, comes from the surface, no continuity to the cortex or the medullary bone
Differential diagnosis:
osteochondroma, subungual exostosis, paraosteal osteosarcoma, heterotopic ossification, periostitis, myositis ossificans
Treatment:
Excision, aggressive resection because of 54% recurrence with marginal resection, includes the fibrous cap and the underlying periosteum
Complication/Recurrence:
50% recurrence, no metastatic spread
Cartilage Tumor: benign
Synovial Chondromatosis
proliferate synovial process comprised of multiple cartalaginous nodules, larger joints including hip, knee, elbow, shoulder and ankle
intra- and extraarticular, metaplasia of the synovial tissure surrounding a joint or tendon, male predominance 3th to 5th decade, over the age of 50 involvement of the hand of both sexes
nodules histologically hyline cartilage with atypical appearing chondrocytes, calcification
Presentation:
pain and swelling, decrease range of motion, any joint about the wrist, progress over an extended time, delay in diagnosis is common, patient with extraarticular chondromatosis have no pain in compare to intraarticular chondromatosis, maybe similar to a trigger finger with chondromatosis in the flexor tendons
Imaging:
detection in x-ray depends on calcification, 75% calcification, MRI in T2 low signal with high signal of joint fluid surround the tumor
Differential diagnosis:
joint included: pigmented villonodular synovitis, crystal deposition desease, rheumatoid arthritis with rice bodies, chronic proliferative synovitis
soft-tissue: soft-tissue chondroma, tumoral calcinosis, rarely soft-tissure chondrosarcoma, maybe degenerative joint disease
Associated conditions:
Wagner-stickler-Syndrome - multifocal synovial chondromatosis
Treatment:
stage 1 (early disease): active synovial desease without intraarticular loose bodies
stage 2 (transitional stage: both active synovial disease, as well as intra-articular loose bodies
stage 3 (late disease): intra-articular loose bodies without active synovial disease
Excision, maybe with arthrodesis when degenerative disease, wide synovialectomy
Complications/Recurrence:
from rare to 60%, malignant transformation very rare to a synovial chondrosarcoma, if left untreated can causes advanced or recalctrant synovial chondromatosis may occur with secondary degenerative arthritis
Cartilage Tumors
malignant
Chondrosarcoma
clinical presentation:
mostly pain and swelling, can go over years, 25% pathological fracture, regional or distant lymph node involvement is rare,
clinical features of secondary chondrosarcoma
M. ollier
Maffucci Syndrom
solitary osteochondroma (average 50-55y)
multiple osteochondromas (25-30y)
- increasing mass
- development of pain or tenderness after skeletal maturity
Diagnostic Imaging:
Low-grade chondrosarcoma
endosteal erosion, cortical destruction, indistinct margins, greater than 5cm, occasionally soft-tissue extension, mostly metaphysis, not over the joint but possible (extremely rare)
High-grade chondrosarcoma
larger than 5cm , pathological fracture, mature and immature periosteal reaction, cortical thickening
threshold of 2cm cartilage cap, increased cap thickness maybe conversion to a low grade chondrosarcoma,
Histopathology
grade I:
low degree of cellularity, sparse multinucleated cells with minimal cytologic atypia, no mitotic figures
grade II:
degeneration of cartiaginous matrix, increased cellularity, moderate atypia, few mitotic figures
grade III:
predominantly myxoid matrix, high degree of cellularity, nuclear pleomorphism, numerous mitotic figures
Treatment:
nonsurgical treatment - rarlely - maybe splinting in poor prognosis, - cave: resistant against radiation and chemotherapeutics
operative Treatment:
biopsy necessary for staging purposes
low-grade: intraleasional with cryotherapy, maybe wide resection
high-grade: wide en bloc resection
chondrosarcomas are treated with limb-sparing surgery
Oncologic outcome
depends on the grade of the tumor and the resection strategy - 11 - 50%, survival is less than 25% at 5y
Follow up:
low grade: ct scan 6-12 month for 2y
high grade: ct scan 3-6 month for 5y - then annualy up to 10y
primary Chondrosarcomas are rare - 1,5-3,2%, 85% are primary chondrosarcomas, 15% are malignant transformations, mostly the proximal humerus
chondrosarcomas are the most common malignant bone tumor of the hand, incidence less than 1%, mostly the proximal phalanx, small finger, 40-60y, slow growing, rist of metastasis is low, mostly the lung, other viscera or cutaneous involvement has been reported
Bone tumors
benign
major occur in the phalanges 70%
metacarpales 25%
carpal bones 5%
- enchondroma
- epidermoid inclusion cyst
- osteoid osteoma
- osteochondroma
- giant cell tumor (GCT)
- aneurysmal bone cyst (ABC)
- bizarre parosteal osteochondromatous proliferation (BPOP - Nora’s lesion)
- osteoblastoma
bone tumors
benign
enchondroma
most common tumor of the hand, 60% found in the hand, 50% in proximal phalanges - 20-40y, soft tissue involvement can be a sign of a low-grade chondrosarcoma
Histology:
enchondroma:
hypocellular matrix with bland-appearing hyaling cartilage and endochondral ossification surrounding cartilage with lamellar bone
chondrosarcoma:
hypercellular cartilage with binucleate cells and giant cells with clumps of chromatin
bone tumor
benign
osteoid osteoma
second most common osseous hand tumor, 10% of all hand tumors in the hand, 20-30y, classic presentation is a localized sharp pain worse at night, improves with NSAR, result from nerve fibers within the blood vessels associated with the the nidus
metacarpales, proximal phalanges and carpal bones, mostly in the scaphoid
radiographic nidus with a slerotic border, when the nidus is greater than 1,5cm the diagnosis is osteoblastoma
ct or mri with a hyperintense signal on T2 sequence
Treatment:
excision or radio-frequency ablation, for surgical marking administration of tetracycline is helpful to use a Woods UV lamp to localize the tumor with its nidus
bone tumor
benign
epidermoid bone cyst
third most common bone tumor of the hand, caused by trauma - forces keratinizing epthelium into the subcutanenous tissue or bone, over time the cells grow und produce an epithelial cell-lined cyst filled with keratin, mostly at the distal phalanx, more men, 30-40y,
recurrence is rare, curettage and bone filling, DD osteomyelitis or malignant process
bone tumor
benign
giant cell tumor (GCT)
lytic tumor, local aggressive and destructive, 20% of all benign bone tumors, rarely seen in the small bones of the hand (1-5%), distal radius is the third most common location overall, women > men, 30-50y,
metaphyseal and epiphyseal regions, GCT cann metastases into the lung, hand GCT have a higher incidence of local recurrence and pulmonary metastases after interlesional treatment,
bright on T2 mri, histology disorganized trabecular bone with multiple multinucleatesd giant cells, recurrence 50%, radiation is contraindicates with GCT because of radiation induced sarcoma
bone tumor
benign
aneurysmal bone cyst (ABC)
5% of all benign bone tumors, hand involvement is very rare with 3-5%, metacarpal > phalangeal, filled with multiple blood-filled cavaties, locally destructive, occurs with patient of 20y,
associated with other bone tumors
GCT, chondroblastoma, fibrous dysplasia, chondromyxoid fibroma, nonossifiying fibroma
primary (gen defect) and secondary
pain and swelling, pathologic fractures, imaging shows a metaphyseal, expansile, eccentric and lytic lesion with bony septae
bone tumor
benign
osteoblastoma
benign but aggressive tumor characterized by osteoid and woven bone production, nidus larger than 1,5cm, mostly posterior aspect of the spine, 5% of all benign tumors of the hand, involvement of the carpus scaphoid, capitate and hamate has been reported, 2x men than women, 10-30y
DD
osteoid osteom, ABC, osteosarcoma, osteomyelitis
bone tumor
benign
Osteochondroma
osseus growth with a hyline cap - orginated from the physis or tendon insertion site, most common benign bone tumor of the human body, only 4% at the hand
painless, slow-growing mass in the metaphysis of long bones, hand distal aspects of the metacarpal and proximal phalanx, massive growing results in joint limitations or tendon snapping, contact to the medullary canal
recurrence extremely rare, no risk of malignant transformation
bone tumor
benign
benign parosteal osteochondromatous proliferation (BPOP)
Nora’s lesion
hand and feet, hand typical proximal and middle phalanx, as well as the metacarpals
localized swelling and discomfort in the volar aspect of the hand, do not appear in the distal phalanx
DD:
osteochondroma, parosteal osteosarcoma, peripheral chondrosarcoma, healing stress fracture callus, turret exostosis, ossified hematoma, periostitis and myositis ossificans
calcified pedunculated lesion that ist located directly off the corex but is seperated from the intermedullary canal or medullary cavitiy
chondrocytes are so called “bizarre” because of bony trabeculae with irregular endochondral ossification
wide excision, 50% local recurrence
bone tumor
malignant
painful mass, swelling, deformity or pathologic fracture, malignant primary bone tumors of the hand and upper extremity are rare
mostly:
- chondrosarcoma
- osteosarcoma
- Ewing’s sarcoma
- metastatic disease
bone tumor
malignant
osteosarcoma
mostly children and young adults - 10y - 30y, bimodal distribution with another peak at the age of 60y, typically associated with radiation or Paget’s disease (ostitis deformans - higher activity of osteoclasts)
osteosarcoma starts in the medullary cavity and often breaches the cortex and expands into the soft tissues, aggressive periosteal reaction with “sunburst” or “Codman triangle”, tumor produces a matrix of osteoid or chondroid
imaging: T2 hyperintense signal with fat suppression
Subtypes:
classical (80%)
chondroblastic
fibroblastic
Juxtacortical (surface) (parosteal, low grade - well differentiated, periosteal, more aggressive - higher grade, high grade
low-grade intramedullary
small cell (rare)
telangiectatic (25% with pathologic fracture, often with ABC)
secondary (radiation induced, Paget’s associated)
pathogenesis:
malignant primary bone tumor that arises from primitive mesenchymal cells
10% humerus
0,18% hand
metastatic into the lung and the bone
riskfactors:
inactivation of the retinoblastoma gene
Paget’s disease
Li-Fraumeni Syndrome
Rothmund-Thomson syndrome
mostly sporadically, 70% with chromosomal abnormality, radiation in older ages
Treatment:
neoadjuvant (2 month) - surgical - adjuvant
Adriamycin, methotrexate, cisplatin, ifosfamide
bone tumor
malignant
Ewing’s sarcoma
most common in males, peak incidence between 10y and 20y of age, often bone pain, maybe present with systemic symptoms such as fever, anemia, leukocytosis and an increase of lactate dehydrogenase and/or erythrocyte sedimentation rate
Imaging:
poorly marginated, aggressive appearing lytic lesion with permeative appearance and laminated onion skin periosteal reaction, sclerosis of codman triangle or sunburst patterns of the periosteum
Pathogenesis:
rare malignant tumor occurs from the bone or soft-tissue
small-blue-round-cell tumor characteristic from the hematoxylin-eosin staining
10% humerus
3% forearm, wrist and hand
Treatment:
neoadjuvant (2 month) - surgical - adjuvant
vincristine, doxorubicin, cyclophosphamid, (ifosfamine and etoposide)
bone tumor
malignant
metastatic disease
acrometastases exceedingly rare, present with pain or pathologic fracture, 40y or older, 33% of hand metastases are from a previously undiagnosed primary cancer, distal phalanx is the most common location, poor prognosis, life expectancy is less than 6 month
mostly:
thyroid, breast and prostata
treatment:
mostly non-surgical, when risk for pathologic fracture stabilization is needed
Mirel’s scoring System
Score 1 - 2 - 3
site, pain, lesion, size
over 8 points!!! stabilization is needed
Nerve tumors
benign
are uncommon, only 2-5% of all tumors,
Differential diagnosis:
- schwannoma
- neurofibroma
- lipofribromatous hamartoma
- granular cell tumor
- perineurioma
- neurothekeoma
- intraneural ganglion/cyst
- traumatic neuroma
- lipoma
- malignant peripheral nerve sheath tumor
etioloy largely unknown, abnormalities of chromosomes 17 und 22 associated with multiple neurofibromas
difficult diagnosis, (invasion into surrounding tissue, rapid increase in size, constitutional symptoms, large size >5cm, persence of night pain - malignancy)
histologic analysis is the reference standard
mostly painless mass, paresthesias and/or motor weakness, (rapid growth into the nerve fascicles)
mostly the forearm and the volar hand, less than 2cm, in the Hand positive Tinel sign
nerve tumor
benign
Schwannoma
most common benign periphal nerve sheath tumor, solitary and involves large major nerves
asymptomatic and painless, maybe parasthesias, 30-50y, eccentric position to the nerve, tinel sign positive,
MRI bright on T2, hypointense capsule, “tail” on mri,
Pathogenesis:
Tumor arises from the Schwann cells, histologic two areas, Hypercellular (Antoni A) areas with palisading nuclei and Verocay bodies, Hypocellular (Antoni B) areas are less cellular, mostly extracellular matrix
Treatment:
mostly encapsulet, making complete exzision possible, if there is adherence to the soft-tissue around, sign for malignancy, carefully seperation from all nerve fascicles from the tumor, if nerve fascicles enters the tumor maybe a sign for malignancy
nerve tumor
benign
neurofibromas
benign peripheral nerve sheath tumors arising from the schwann cells, fibroblasts and perineural cells, in association with von Recklinghausen disease, 85% cases are solitary in nature
solitary neurofibromas have a low risk of malignant transformation, (von Recklinghausen 4% malignant transformation)
3 main types:
dermal neurofibromas:
affect cutaneus nerves and penetrate into the subcutaneous tissue - “plaque-like swellings”
plexiform neurofibromas:
found along larger nerves and form tortuous masses
localized neurofibromas:
most common with 85% of upper extremity cases
Clinic:
similar to schwannoma, typical not painful, remain small less than 2cm, pain and greater size is a sign for malignancy, associated with the fascicles and not encapsulated, often the fascicles are in the tumor,
Imaging:
“Target sign” in MRI in T2 (lipomas have a hypertense signal on T1)
Pathogenesis:
interlacing bundles or spindle cells with nerve fibers throughout the lesion
Treatment:
surgical excision is difficult because of the contact to the nerv fascicles, 18-44% worsening pain, 15-50% increased weakness
nerve tumor
benign
lipofibromatous harmatoma
benign fibroadipose tumor slowly growing in the volar forearm, mostly like a cts because the median nerve is often involved
one third have a macrodactyly, typical slow growing small tumor, but maybe can reach sizable proportions
Imaging:
MRI - enlarged nerve with “pathognomonic coaxial cables” correspond to 2 / 3mm diameter bundles of axons encased in epineural fibrous tissue, nerve conduction and electromyography is pathologic
pathogenesis:
distinguish from Schwannoma and Neurofibroma from the mass of fat in the tumor, histologic individual nerv bundles with fibroadipose tissue and expansion of the epineurium
treatment:
controversial, Epineurotomy with intraneuronal debulking has been attempted, permanent sensory or motor loss may occur, may occur in the plexus brachialis - than only debulking due pain or disfigurement
nerve tumor
benign
granular cell tumor
from Schwann cell origin, smaller than 3cm, may or may not associated with a major nerve 20% in the upper extremity,
histologic:
large, granular-appearing eosinophilic cells, granular cell tumor stain is positiv for PAS and S-100
difficult to differentiate from schwannoma or neurofibroma without histology, malignant versions exists, treatment with wide excision,
nerve tumor
benign
perineurioma
solitary tumors composed of perineurial cells,
2 main categories:
soft tissue and intraneural
monosomy of Chromosome 22
soft tissue perineurioma are slow growing, painless nodules that occur in young adults, no clear association to a nerve, dermis or subcutaneous fat
intraneural perineuriomas arise within the nerve - commonly with loss of sensory or motor, mostly in the pediatric population, 70% less than 30y, mostly the median nerve is involved
histologic:
perineural cells arranged in whorls and interweaving fascicles - positive for EMA and negative for S-100 perineural cells forming “onion bulbs”
treatment:
controversial, no malignant transformation, maybe tumor resection with nerve autograft,
nerve tumor
benign
neurothekeoma
arise from the Schwann cells or perineural cells, small dermal lesions of the face or upper extremity usually in young adults, less than 20y and more likely to female
histologic:
demonstrates nests and cords of cells with a mucinous matrix, located around small nerves
treatment:
no malignant transformation, oberservation!!!, when neurologic symptoms or diagnosis is unclear, biopsy is performes, mostly encapsulated so they can remove without harming the nerve
nerve tumor
benign
intraneural ganglion / intraneural cyst
uncommon, benign peripheral nerve tumors, peripheral nerve mucoid degeneration, cystic nerve tumor, and neural mucoid pseudocyst
firm mass with motor and or sensory findings due to nerve compression, ulnar nerve within the Guyon’s canal is the most commonly affected nerve in the upper extremity
MRI: hyperintense T2
Pathogenesis:
mucoid containing nonepithelialized cells consistent with a typical ganglion cyst, arises from a stalk from a tendon sheath or joint capsule
treatment:
excision, 30% of patients have persistent symptoms and neurologic dysfunction after excision
Nerve tumor
malignant
MPNST - malignant peripheral nerve sheath tumors
MPNST - malignant peripheral nerve sheath tumor
arise from peripheral nerves or pre-existing benign nerve sheath tumor
development idiopathic, 10% prior radiation, metastize via hematogeneous spread but also local extension and lymphatic spread
origin neuroectodermal and derived from one of the cellular components of the nerve sheath
in contrast to benign tumors this tumor has contact to the axon!!!
very rare - 0,001% incedence in the general population, 20-30% in the upper extremity, 50% of the MPNSTs are from patients with von Recklinghausen disease (neurofibroamatosis Type I) - autosomal dominant condition, loss of the function of the NF1 tumor suppressor gene, which encodes neurofibromin on chromosome 17q11.2
symptoms: 2 or more
- relative first degree
- cafe-au-lait spots
- neurofibromas
- axillary and inguinal freckling
- optic glioma
- Lisch nodules (hyperpigmented iris hamartomas)
- distinctive osseous lesion (sphenoid dysplasia or congenital pseudarthrosis of the tibia)
malignant transformation 10-15%
clinical presentation and diagnosis
pain, enlarging mass and associated neurologic symptoms, diagnosis delayed because of delayed symptoms, malignant transformation - rapid growth over a short time after a long time of slow growth
50y, (neurofibromatosis 30-40y),
Imaging:
“target sign” , MRI can not really differentiate between Neurofibromatosis and MPNSTs, better positronen emissions tomography, CT and x-ray to rule out metastases of the lung and the bone, maybe magnet resonance angiography for surgical planning
MRI: T1 equivalent between tumor and muscle, T2 surrounding edema
amputation and nerve sacrifice is often required
adjuvant radiation maybe helpful for outcome
recurrence is guarded, recurrence 40%, metastasis 67% mostly in lung and bone,
poor prognostic:
proximal tumor
large tumor greater than 5cm
neurofibromatosis
subtotal resection
5y survival rate 40 to 60%, 10y survival rate 23 to 45%
soft tissue tumor
benign
ganglions
most common wrist mass - develop gradually as a swelling on the dorsum of the wrist
20-50y, maybe asymptomatic, maybe pain, cosmetic concern or malignancy concern,
stalk without epithel, not a true herniation, maybe proliferation of benign synovial cells
mostly dorsal scapholunate ligament (SLL), volar - space of Poirier (between the radioscaphocapitate and long radiolunate ligaments), secondary maybe from the scaphotrapeziotrapezoid joint. adults not common from tendon sheaths, children more tendon sheats,
DD: mucous cysts, volar retinacular cysts
soft tissue tumor
benign
dorsal wrist ganglion
treatment:
oberservation, NSAIDs, splinting, aspiration or closed rupture with recurrence rates from 64 - 83%, ASK resection is better because the SLL can be seen and sometimes there are other intraarticular pathologies (42-100%), postoperative splinting for 7-10d, normal function after 4 weeks
recurrence 4-10%, stalk excision recurrence about 1%, incomplete resection recurrence 40%, recurrence open and arthroscopic excision is comparable (0-11%)
soft tissue tumor
benign
volar wrist ganglion
radial side of the distal radius, rarely with the TFCC on the ulnar side of the wrist, rarely are found with the median or ulnar nerve due compression in the carpal tunnel or the loge of Guyon
mostly between the FCR and the first extensor sheath, the rest origin from the scaphotrapezoidal joint, ulnocarpal joint, metacarpotrapezoidal joint or the FCR sheath
treatment:
carefully, radial artery, dorsal sensory branch of the radial nerve, lateral antebrachial cutaneous nerve, maybe exploration through the floor of the FCR sheath, Space of Poirier - between the radioscaphoidcapitate and the long radiolunate ligament
recurrence:
as for the dorsal wrist ganglion - 6%
soft tissue tumor
benign
volar retinacular cyst
firm nodule overlying the palmar crease ranging from 3 to 7mm, cysts origin from the flexor tendon sheath, A1 and A2 pulley, more common in the 3rd decade,
maybe ultrasound and mri, but mostly clinical diagnosis
Treatment:
observation possible - 42% - 65% resolution, mostly in children, aspiration possible with success from 58-100%, recurrence after aspiration 42-45%
excision with a little portion of the flexor tendon sheath, recurrence with open excision is 0
soft tissue tumor
benign
mucous cyst
dorsal distal interphalangeal joint in the index and long finger in the middle-aged and elderly - associated with degenerativ joint disease, originates from the dorsal aspect of the joint and the cyst overlying the terminal tendon, maybe nail deformity because of affection to the eponychium or the germinal matrix
DD:
- Heberden nodes
- gout
- giant cell tumor (GCT) of the tendon sheath
Treatment:
pain, nail deformity, impending rupture is a relative indication for aspiration or surgical excision, when worse degenerative disease maybe arthrodesis must be performed
Aspiration better with corticosteroids, 40% recurrence
maybe skin flap for coverage, remove all osteophytes at the dorsal aspect of the joint, nail deformities will resolve after removing the cyst
soft-tissue tumor
benign
Giant cell tumor of the Tendon sheath
pigmented villonodular synovitis, fibrous xanthom, nodular synovitis
second most common hand tumor, middle-aged or older patients, typically at the interphalangeal joints of the 3 radial digits, mostly volar, symptoms with interaction with digital nerves or joint motion, firm nodular mass without translumination (in comparison to cysts), ultrasound shows a typically homogeneously hypoechoic mass
MRI: low signal on T1 and T2
Pathogenesis:
stromal cells, giant cells and lipid laden foam cells, they have no risk of malignant transformation, but maybe local aggressiv
two types
with and without a pseudocapsule, both types may be multilobulated
Treatment:
conservative only with minimal symptoms - observation
marginal excision is the recommended treatment,
over expression of RANKL in stromal cells, no chemotherapy
recurrence from 4-44%, mostly of primary incomplete excision, if there is a joint erosion - maybe arthrodesis, routine follow up
soft-tissue tumor
benign
epidermal inclusion cysts
third most frequent benign hand tumor, slow-growing, usually painless, tactile surface of the digits
Pathogenesis:
develop from displaced keratinizing epithelia cells from the surface to deeper soft tissue, mostly male laborer, encapsulated, than produce sebum and other skin elements
histologic epithelial lined cavity filled with keratin, no malignancy transformation, ruptur of the cyst may lead to infection
Marginal excision, when adherence to the - then curettage
soft tissue tumor
benign
fibromas of the tendon sheath
3% of the hand tumors, 40y, predilection for men,
fingers - hand - wrist and elewhere in the body, mostly right hand and thumb
painless, slow-growing, depending on location they can cause neuropathies
Pathogenesis:
emanate from the tendon sheaths, reported also from intra-articular structures of the wrist as well as other joints
histologic - hypercellular areas of spindle-shaped fibroblasts surrounded by collagen und other stroma, no risk of malignancy, encapsulated but maybe multilobulated
Treatment:
surgical excision - rule out aggressive conditions, recurrence of 24%
soft-tissue tumors
benign
lipoma
mature adipocytes, subcutaneous but may also be intermuscular, intramuscular, intratendinous, parosteal or visceral
painless, symptoms through the growing, MRI isointense to fat in T1, low-signal in T2
Treatment:
oberservation with small lipomas, surgery if there are symptomas through the tumor mass, very rare transformation into malignant liposarcomas
malignant transformation: rapid growth, pain, systemic symptoms, or size greater than 5cm
recurrence is rare, surgical intervention maybek on two sides
soft tissue tumors
benign
glomus tumor
arise from the glomus body, a thermoregulatory arteriovenous shunt, middle-aged women,
mostly volar fingertips and subungual, also in the palm and other regions of the body, neurofibromatosis is predisposed
Trias:
- severe paroxysmal pain
- pinpoint tenderness
- cold hypersensivity
love test: pain with pressur and relief when not pressed or pressed elsewhere, when tourniquet is released patient has sudden pain,
red or blue tint of the lesion, nail deformity, digital pulp nodule, 1/3 affect the bone
MRI: low-signal on T1 - high signal on T2
Pathogenesis:
neuromyoarterial elements of a normal glomus body and has been described a a type of vascular hamartoma
Treatment:
non-operative:
sclerotherapy, argon, carbon dioxide laser and injection with hypertonic saline, surgical excision is the best treatment, overall 3mm
complications:
nail deformity and recurrence, recurrence rate from 0-20%,
soft-tissue tumors
benign
nodular fasciitis
rare benign reactive myofibroblastic tumor with predilection for the subcutaneous tissues
solitary lesion of the forearm from young adults, rarely in the hand itself
lesions are solid, nodular, rubbery, firm
rapidly growing mass present for a few month, important to diffentiate to a soft tissue sarcoma
histologic: well-circumscribed but not encapsulated
4 characteristics
- spindle-shaped fibroblasts
- clefts separating fibroblasts
- extravasated erythrocytes
- interstitial mucoid material
Treatment:
maybe oberservation because of self limiting
surgical:
excision, recurrence rare
soft-tissue
benign
lipofibromatous hamartomas
LFH - lipofibromatous hamartomas
rare benign localized expansion of epineuial adipose and fibrous tissue affecting periphal nerves
LFH mostly congenital, typically young adults, motor or sensory changing, mostly one third have symptoms in the first year of life, upper extremity and the median nerve are commonly involved,
isolated macrodactyly should prompt suspicion for LFH
Pathogenesis:
sausage shaped expansions surrounding and invading the peripheral nerv, similar to neurofibromatosis, but can easily be distinguished by neurocutaneous manifestions, family history and microscopic appearance
Histology:
mature fibroadipose tissue seperating individual nerve fibers and expanding the space between the perineurium and epineurium
MRI:
coaxial cable-like appearance of nerve fascicles expanded by intraneural fat
Treatment:
excision with neural deficits, carpal tunnel release is the most common procedure for LFH and median neuropathy, case to case decision
soft-tissue tumors
benign
lipofibromatosis
rare pediatric neoplasm, presentation show a 1-2cm solitary firm poorly demarcated nontender subcutaneous nodule of the distal extemities, most common in the hand (40%), mostly childs are 3y and male
Histologic
local aggressive and destructive, no malignancy, predominantly mature fat cells and fascicles of fibroblastic cells, occasionally myxoid with poorly defined margins, involves the whole surrounding tissue
treatment:
surgical excision with negative margins, recurrence is high 72% - better 31%
soft-tissue tumors
benign
calcifying aponeuotic fibroma
rare benign tumor occuring mainly in the hands of infants and young children, predelection for males, tumor is aboutn 1-5cm with granular calcifications
Pathogenesis
unclear, spindled fibroblasts with chondroid foci and calcification, infiltrates soft tissue, bone is rare
treatment
surgical excision with recurrence of 50%
soft-tissue tumors
benign
lipoblastoma and lipoblastomatosis
rare rapidly growing proliferations occurring exclusively in young children
first year of life!!! with up to 88% in the 3 first years of life
lipoblastoma - solitary subcutaneous well-circumscribed lesion
lipoblastomatosis - deep, diffuse infiltrative tumor
Pathogenesis:
lobules of fat in various stages of maturation, seperated by fibrous septa, prominent blood vessels and sometimes myxoid foci
treatment:
surgical excision, recurrence 9 - 14%
soft-tissue
benign
angiolipoma
rare, soft-tissue tumor subcutaneous extremities or trunk, differ from lipomas, they are smaller, less than 2cm, appear at an early age (median 21y), maybe painful, multiple nodules with predilection for males, familar inheritance
Pathogenesis:
adipose foci with prominent capillaries with fibrin thrombi and spindle cells, well encapsulated
Treatment:
excision with low rate of recurrence, infiltrating angiolipomas have no capsule and can be aggressive - than wide excision - 33% recurrence
palmar fasciitis and polyathritis syndrome
paraneoplastic syndrome associated especially with an ovarian cancer
A gynecologic examination is recommended in any woman presenting with the sudden onset of
- unexplained hand pain
- palmar inflammatoryfasciitis
- palmar fibromatosis
- digital contractures
soft-tissue tumor
malignant
soft-tissue sarcoma
12% upper extremity,
Pathogenesis:
often unknown
association with:
neurofibromatosis, Li-fraumeni syndrome, familial adenomatous polyposis, hereditary retinoblastomas,
radiotherapy can induced sarcomas
different types:
undifferential pleomorphic sarcoma
spindle cell sarcoma
high-grade osteosarcomas
Lymphangiosarcomas caused by chonical lymphedema, so described be Stewart and Treves, caused by previous surgery, chronic filarial infection or radiotherapy
toxins:
dioxin, arsenic, vinyl chloride
clinical:
painless mass, no loss of function even with large malginant tumors, sometimes atypically - epitheloid sarcoma can present as an ulcer, synovial sarcoma can present as nodules
large size >5cm, deep intramuscular location, attachment to surrounding structures, overlying skin changes with prominent superficial vessels, growth about weeks or months, sometimes lymphnodes are involved (epitrochlear, axillar or cervical) only 5% of soft-tissue sarcomas, at the hand sarcomas are good detected because they are more visible, mostly less than 5cm
Evaluation:
MRI is the best imaging modality, core needle biopsy is better than fine needle biopsy, nearly 88% accuracy
well planned operation!!!
Treatment:
excision with a negative margin from 1cm, adjuvante Chemotherapy
surgical planning:
- anatomic location
- inflammatory zone surrounding the tumor
- Location of critical neurovascular structures
- possible areas of close/positive margins should marked for radiotherapy
- tissue deficits which require reconstruction
single ray amputation - 66% grip strength
double ray amputation - 24% grip strength
SLNB - sentinel lymph node biopsy
not well established like melanoma or breast cancer, can be good by
- rhabdomyosarcoma
- angiosarcoma
- clear cell sarcoma
- epitheloid sarcoma
adjuvant treatment:
radiotherapy - reduce lokal recurrence - high-rate sarcoma with close resection margin or large tumors >5 cm, development of a radiation sarcoma
chemotherapy, especially with treatment of rhabdomyosarcoma, effect is limited
agents: doxorubicin, in combination with ifosfamide
surgical:
thumb, goal is a sensate terminal thumb, than lengthening, amputation more proximal than pollicization or toe-to-thumb transfer, smaller tumors at the tip can be excised an defect covered with full thickness skin graft
postoperative care:
every 3 month in the first year, every 4 month in the 2. and 3. year and every 6 month in the 4. and 5. year and than yearly after that,
recurrence 10% by wide resection,
specific tumors:
epitheloid sarcoma, 2 types - classical type which occurs in the distal extremities and a proximal type occurring in the perineum and the groin,
is a common tumor of the forearm, wrist and hand, slow-growing, painless, firm nodule on the palmar surface of the hand, finger or forearm
DD: infection, Dupuytren, palmar fibroma
very aggressive, wide resection, radiation or brachytherapy, maybe resection of the sentinel lymphnodes
synovial sarcoma:
5-10% of all soft-tissue sarcomas, commonly at the carpus, arising close to joints and bursa, slow-growing, painless, solid mass, 15y-40y, 30% calcification, wide resection with negative margins, adjuvant radiotherapy for increasing the local recurrence, more responsive to chemotherapy than other sarcomas, especially with large tumors, less than 5cm 10y-survival is 88%, greater than 10cm only 8%
Liposarcoma:
most common sarcomas, only 10-24% in the upper extremity, 25% in the forearm, hand and wrist is very rare,
atypical lipomatous tumors and well-differentiated liposarcomas have a high local recurrence rate but do not metastasize
dedifferentiated liposarcomas contain a “nonlipomatous” component
myxoid liposarcomas second most common type, metastasize to extrapulmonary sites like the spine or soft-tissue, sensitive to radiotherapy and chemotherapy
pleomorphic liposarcomas are the least common and most aggressive subtype, metastasize into different sites
Undifferentiated sarcoma/ Malignant fibrous histiocytoma - no line of differentation, high-grade and effect older patients, normal treatment principles
Rhabdomyosarcoma - 80% embryonal - 20% alveolar - two other subtypes (pleomorphic and spindle cell rhabdomyosarcoma)
50% on the extremities are alvelar Rhabdomyosarcoma, resemble embryonic skeletal muscle with rhabdomyoblasts in different stages of differentation - oval nuclei and eosinophilic cytoplasm
treatment: systemic chemotherapy with vincristine, dactinomycin and cyclophosphamide together with surgery and/or radiotherapy, resection of the sentinel lymphnode is required also there is no clinic
vascular tumors
benign
most common on the upper extremity
mostly asymptomatic
the fourth most common type of mass seen in the hand
- ganglion
- giant cell tumor of the tendon sheath
- epidermal inclusion cysts
- vascular tumor
vascular tumors
benign
hemangioma of infancy
most common tumor of infancy with reported 7-10% incidence, mostly in caucasian individuals, females, premature infants, chorionic villus sampling
only 30% are present at the birth, typically evident in the first 4 weeks, proliferate phase after initiation with rapid growth, than a slow involution phase
50% of the lesions involute by 5y of age and 90% by 9y
Pathogenesis:
arise secondary to both angiogenic and hormonal factors, after involution there is an area of depigmentation, telangiectasia or inelastic skin
treatment:
non operative - corticosteroide, propranolol is an effective medication, accelerates the involution by increasing apoptosis or timolol maleate
maybe: interferon, vincristine and bleomycin, lasertherapy
operative:
lesions disfiguring children over 3y, after that, compression bandage to protect from swelling
complications:
bleeding ulceration, pain, infection, congestive heart failure in large lesions
vascular tumors
benign
pyogenic granuloma (lobular capillary hemangioma)
common lesion occur in both children and adults, frequently at the hand and maybe at the nail bed
usually echogenic mass with small hypoechoic foci, other tumors are hypoechoic
friable (brüchig) glistening, reddish papules that bleed easily with minimal trauma, grow rapidly, maybe a collarette of scale (narrow rim of loosened keratin overhanging the periphery of a circumscribed skin lesion), patient use to bandage it - “band-aid sign”
Pathogenesis:
recent injuries, medications, peripheral nerve injuries, systemic inflammatory diseases,
histologic: polypoid exophytic ulcerated mass containing numerous of capillaries and venules, granulation tissue, neutrophils, and fibrocytes in an edeamtous stroma
treatment:
non-operative: silver nitrate, cauterization, pulsed-dye laser, carbon dioxide laser, cryotherapy, scleotherapy with sodium tetradecyl, local corticosteroids
operativ: elliptical excision with negative margins because of recurrence
DD: amelanotic melanoma or squamous cell carcinoma
vascular tumors
benign
capillary malformation
most common congenital vascular malformation, typically on the extremities with port-wine stains or nevus flammeus, appearing as pink to red patches,
port-wine stains:
- Klippel-Trenauy syndrome
- proteus syndrome
- cobb syndrome
Pathogenesis:
congenital with more than 25% origin, histologically normal number of ectatic capillaries
Treatment:
observation when they are small, especially when they are great at size or deepen their colour - pulse dye laser or pulsed light - photodynamic therapy, alexandrite or neodymium:yttrium-aluminium-garnet (Nd:YAG) laser can be used for hypertrophic or nodular lesions with deeper vessels
operative: quite rare!!!
vascular tumors
benign
telangiectasias
small dilated vessels course on the surface of the skin, fine linear vessels or as red macules
- chronic sun damage in olderly
- trauma
- radiation dermatitis
- chronic graft-versus-host disease
- collagen vascular disease
UNT - unilateral nevoid telangiectasia - congenital - more common to males, autosomal dominant (assiciated with ipsilateral melorheostosis)
CMTC - cutis marmorata telangiectasia - present at birth as a blue reticulated vascular network
associated with:
- atrophy or hypertrophy of the limb
- syndactyly
- brachydactyly
- acral cyanosis
- simian lines on the palms
- weakness of the extensor pollicis longus
- numerous additional dysmorphic failures
Syndromes:
- Cornelia de Lange syndrome
- down syndrome
- Trisomy 18
more generalized telangiectasias can be associated with an internal disease:
- hereditary hemorrhagic telangiectasia
- Osler-Weber-Rendu or ataxia-telangiectasia
- Louis-Bar syndrome
Pathogenesis:
not well understood
treatment:
special vacular specific laser such as a 532 nm potassium titanyl phosphate laser or 595-nm pulsed-dye laser, observation is the important treatment and this for 3y to find out if there is a syndrome
vascular tumors
benign
angiokeratoma
red to purplish-blac, slightly keratotic papules
special subtypes
solitary papular angiokeratoma
associated with trauma, dark red to blue-black colour
angiokeratoma circumscriptum
hyperkeratotic plaques contains warty red-blue papules and nodules, mostly on the legs
angiokeratoma of Mibelli
females on the dorsal side of the digits, inherited in autosomal-dominant fashion, superficial trauma may cause the lesions to bleed easily or ulcerate
treatment:
cryosurgery or laser ablation
argon laser
copper vapor laser
frequency-doubled Nd:YAG
1064-nm Nd:YAG
pulsed-dye laser best for multiple angiokeratomas
Nd:YAG best for angiokeratoma of Mibelli
maybe electrodessication and curettage
electrocautery
differentiate between an angiokeratoma or a malignant melanoma
operative:
indistinguishable from malignant melanoma, resection as a melanoma
complications
recurrence is rare, maybe scar sensitivitiy
vascular tumors
benign
venous malformation
low flow lesions common in females and males, 75% diagnosed at birth, 25% in childhood or adolescence
typically blue to purple compressible nodules with overlying hyperhidrosis
pain, increases with activity or cold weather, during exercise there may be recurrent intralesional thrombosis and dilatation with will bring out the pain
found in isolation or be part of a syndrome:
blue rubber bleb nevus syndrome
congenital disorder that has multiple venous malformations in the skin and gastrointestinal tract, cause pain by night - anemia and melena (bleeding in the gastrointestinal tract)
Klippel-Trenaunay syndrome
port-wine stains, venous malformations, bony/soft-tissue hypertrophy, may affect one or more extremities, mostly the lower extremities
Pathogenesis:
vascular lesion develop through hormones, growing during puberty and the second and third trimenon of pregnancy
treatment:
without symptoms - observation
mildly symptoms - elevation, compression garments, low-dose aspirin, sclerotherapy or laser therapy - common agents: ethanol, polidocanol foam, sodium tetradecyl sulfate, Ethibloc (combination of amino acids, ethanol and contrast material)
rubber bleb nevus syndrome - etidronate has been shown to reduce pain and prevent further the calcification of the lesion, rapamycin has shown to rapidly reduce the size of the lesion
operative:
carefully!!! maybe staged for great lesions, goog planning, from distal to proximal and from dorsal to volar, after surgery compressing bandage,
complications:
sclerotherapy - necrosis and extravasation with nerve injury, hyperpigmentation or infection
surgical 20% - most common - persistent swellingg and edema, recurrence of lesions is associated with incomplete excision and with diffuse lesions
vascular tumors
benign
arteriovenous malformations
AVMs - arteriovenous malformations -
40% diagnosed at birth
35% after 10y of age
common in females and more commonly on the right upper extremity
direct arteriovenous shunts without intervening capillaries, associated with with erythema, hyperhydrosis, painful with exertion (Anstrengung), do not compress when elevate the extremity
AVMs are associated with:
Parkes-Weber-Syndrome (AVMs and features of Klippel-Trenauny Syndrome) - overlying pigmentary changes, pseudo karposi sarcoma, pain, ulceration, hemorrhage, ischemic digits and high-output congestive heart failure
angiography is the gold standard
Pathogenesis:
congenital in natur, influenced by hormones, during puberty and pregnancy more than 80% note increasing pain and enlargement of the lesions
treatment:
compression garments, arterial embolization with painful lesions (risk of distal ischemia!!!), thermoablation or embolo-/sclerotherapy
operative:
vascular surgery outlined by Upton and colleagues
complications:
embolization / sclerotherapy - skin necrosis and/or distal ischemia
surgical - 30% - bleeding and wound issues (seroma and wound dehiscence or gangrene)
vascular tumors
malignant
- angiosarcoma
- hemangioendothelioma
- hemangiosarcoma
- glomangiosarcoma
- malignant hemangiopericytoma (HPC)
- lymphangiosarcom
- Kaposi sarcom
less than 1% of tumors of the upper extremity,
Lymphangiosarcoma - Stewart-Treves-Syndrome
HIV - Kaposi-Sarcoma
rapid increase of size, ulceration, fungation or hemorrhage - may indicate malignanat potential, often extensive soft-tissue infiltration, hematogenous spread to the lung is the most metastaziation of angiosarcomas, others are regional lymph nodes, liver and bone, lymph node metastasis are more common than in soft-tissue sarcomas, sentinel lymph node is under investigation, tumor markes have little prognostic value
vascular tumors
malignant
angiosarcoma
2% of all soft-tissue sarcomas, common among males, mostly occur in the sixth decade, survival quite poor, 5y survivial after resection about 60%
Radiation is a high risk factor, other chemicals such as:
vinyl chloride, arsenic, anabolic steroids or syndromes:
neurofibromatosis
Maffucci-Syndrome
Klippel-Trenaunay-Syndrome
typical multifocal and arise from de novo, cutaneous related to chronic lymphedema or viscera in origin, skin involved painless, ecchymotic-appearing patch,
metastase to the lung most common (liver, bone, lymph nodes)
histology:
abnormal, pleomorphic, malignant endothel cells, absence of melanocytoic markes distinguish from melanoma
treatment:
wide resection, negativ margins, adjuvant radiotherapy to minimize the risk of a recurrence
vascular tumors
malignant
lymphangiosarcoma
Stewart and Treves Syndrome,
patients with lymphangiosarcoma postmastectomy, most cases after breast cancer, etiology is a chronical lymphedema
fifth and sixth decade of life, 5-25y after mastectomy, 10y after mastectomy and radiation
development over:
- chronic lymphedema
- angiomatoisis
- lymphangiosarcoma
90% at the upper extremity, tumor often seen as discoloration with ecchymosis or trophic skin with wrinkle (faltig) lines, maybe hyperkeratosis, telangiectasias, subcutaneous palpable mass or poorly healing eschar with recurrent bleeding, tumor spreads hematogenously to the lung
histologic:
endothelial cells lining channels, hyperchromatic and pleomorphic
treatment:
wide resection with negative margins, mostly forequater amputation is the only treatment to rule the tumor, use of adjuvant RT or chemotherapy are not proofed, survival is poor, survival median 2,5y, untreated 5-8Month,
vascular tumors
malignant
Kaposi Sarcoma
4 clinical variants:
- classic form
- endemic in Africa
- immunosuppressive-associated (transplant recipients)
- HIV associated
spindle-shaped cells, vascular channels and contains lymphoplasmatic infiltrates, HIV or endemic caused by human herpesvirus 8,
regression with sirolismus - rapamycin inhibitor, antiherpetic medication is not effective
treatment:
extensive cutaneous disease, visceral disease, unresponsive disease generally treated with chemotherapy - antracycline and taxanes
doxorubicin
bleomycin
vincristine
radiation to treat KS that is to extensive to treat locally
vascular tumors
malignant
Hemangiopericytoma
Hemangiopericytoma - HPC
rare tumor - occurs from pericyte proliferation - 1% of all vascular tumors, develop in subcutaneous tissue and skeletal muscle, tumor of adults in the fifth and sixth decade
most commonly in the lower extremity, pelvis, retroperitoneum and head/neck - only 10% in the upper extremity
painless mass or site of swelling, histologic - numerous of vascular spaces seperated by packed oval to spindle-shaped cells, thick walled vessels so calles “staghorn” in character, special stain for reticulin can help differentiate HPC from hemangioendothelioma (reticulin surrounds a group of cells) - reticulin surrounds individual cells in HPC, poor outcome with worse prognosis
treatment:
radical resection, metastatic disease most commonly occurs in the lung and less commonly in bone, lymph nodes or liver.
adjuvant chemotherapy:
actinomycin-D, adriamycin, cyclophosphamid, methotrexate and vincristine (temozolomide and bevacizumab) - progression free survival median 8,6Month
vascular tumors
malignant
Hemangioendothelioma
epitheloid Hemangioendothelioma (EHE)
aggressive subtype of the malignant vascular tumor
subtypes:
- epitheloid
- Kaposiform
- hobnail or Dabska-retiform
- polymorphous hemangioendotheliom
Kaposiform mostly found in pediatric patients
Hobnail arises from the dermis and subcutaneous tissue - metastasis into regional lymphnodes
available literature regarding to the EHE - epitheloid hemangioendothelioma - most common and aggressive subtype - arises from the large veins of the lower extremity - iliac and femoral veins - tumor of adults in the second or third decade
clinic:
pain and local swelling, maybe present as deef vein thrombosis, slow-growing tumors - mostly unifocal - when multifocal than bone and liver, high mitotic rate (more than 2 mitoses per high-power field) correlates with a poor survival prognosis
treatment:
surgical resection - recurrence 10-20% without metastasis - 17-60% with metastasis, overall survival is less than 20% in 5y
osseus EHE - RT is an effective tool in treatment of EHE
vascular tumors
malignant
Glomangiosarcoma
malignant glomus tumor, cutaneous neoplasm
malignant glomus tumors which arise from the glomus bodies - arteriovenous anastomoses in the retinacular dermis - involved in thermoregulation
adults from 20 - 89y, lower extremity more often involved than the face or upper extremity, distinguish between benign and malignant is the mitotic activity of pleomorphic cells - do not metastasize but maybe local agressive
classification:
- locally infiltrative glomus tumors
- glomangiosarcoma arising from a pre-existing glomustumor
- glomangiosarcoma arising de novo (GADN) - high mitotic rate
treatment:
wide excision is the best treatment
skin tumors
benign
Epidermoid cyst (Epidermal inclusion cyst)
occur from trauma which drives skin deep to the epidermal surface, serving as a nidus, produce keratin which can accumulate
mostly males and the palm side of the hand
associated with Gardner’s Syndrome - autosomal dominant genetic disorder with various tumors including colon cancer - some of these patiens tend to have numerous of these cysts
surgical treatment:
excision
complications:
infection, recurrence, thickened scar, recurrence rate is about 11%
skin tumors
benign
sebaceous cyst (Steatocystoma)
only on the dorsum of the hand (there are no sebaceous glands in the palm), arise from the obstructed apocrine glands and contain sebum, communicating central pore that is blocked
solitary or multiple, multiple is a rare autosomal dominant or sporadic mutation with lesions occuring most commonly on the upper extremity, chest, axilla and groin
mobile masses without pain
treatment:
marginal excision, (multiplex: aspiration, radiofrequency with excision, carbon dioxide laser
skin tumors
benign
cutaneous warts
human papillomavirus, skin to skin action, typical from human papillomavirus type 1-4
3 categories:
- verrucous warts (verruca vulgaris)
- flat warts (verruca plana)
- periungual/subungual warts
flat warts account about 5% of all hand warts, 50% resolve spontaneously within 2y
treatment:
topical keratinolytics - salicylic acid applied over 6-12 weeks with a recurrence rate from 73%, cryotherapy is not used in hand warts - too much complications, removing with duct tape for 6 days and debridement with pumice stone - 85% resolution
potential to transform into squamous cell carcinoma, particulary in the periungeal region, risk for immunocompromised patients - biopsy!!!
treatment:
excision widely in the normal skin and tissue
skin tumors
benign
pyogenic granuloma
vascular lesion, arises from the skin and mucosa, common in the hand, especially in the fingers - solitary, friable, glistening red papula or nodule that easilay bleeds or goes on to ulcerate
distinguish from pyderma gangrenosum, a distinct disease with skin ulceration and granulation tissue, especially with autoimmune and inflammatory disease - will be treated with corticosteroids and dapsone - difficult in diagnosis
treatment:
silver nitrate cauterization achieves complete resolution in 85% of patients with 1-3 treatments, when excision 1mm of normal tissue, periungual und subungual rule out amelanotic melanoma and squamous cell carcinoma, maybe excision with full thick skin transplantation
normally no recurrence
seborrheic keratoses
benign skin lesions, particulary the elderly, appear “stuck-on” the skin, sharp borders and elevated and sometimes verrucous, diameter between 5 - 10mm,
treatment:
do not need to be removed, removed by dermatologist by curettage, cryotherapy or ablative laser, Vitamin D promote lesion regression
skin tumors
benign
cherry angioma (senile angioma)
vascular lesion, whose frequency increases with age, typically on the upper trunk and the extremities, starts as petechiae and then becomes a smooth, round, red papule that can vary in size from a millimeter to a larger nodule
treatment:
no treatment - maybe cryotherapy, laser ablation or surgical excision
skin tumors
benign
keratoacanthoma
rapidly growing tumor that typically occurs on sun-exposed skin areas of light-skinned persons, especially in the elderly
3 stages:
- proliferative stage - firm smooth papule rapidly enlarges for 2-4 weeks
- mature stage - bud shaped lesion or hemisphere-shaped nodule with a central keratinous core
- involutional stage - after several month, tumor undergoes necrosis leaving a puckered hypopigmented scar
distinguish from squmous cell carcinoma - rarely reported to be metastasize, local recurrence in 4%,
treatment:
malignant tumor principles should be applied, removed with a margin of 4-6mm, in the nail bed maybe amputation
skin tumors
benign
dermatofibroma
cutaneous fibrous histiocytoma
common benign tumor caused by little trauma or insect bites - shoulder, lower extemities, buttock and are rare at the hand
represent proliferation of fibrocytes and histiocytes which involves the dermis and sometimes the subcutaneous tissue, normally asymptomatic, some can be tender or pruritic, colour can be vary from skin-coloured to reddish brown, when pinched the lesion dimple inward - “dimple sign”
treatment:
normally no treatment neccessary, if it is unclear if there is malignant part - excision is performed
skin tumors
benign
Lentigines
benign hyperpigmented macules with increased basal epidermal melanocyes and epidermal hyperpigmentation
sun-exposed areas, after the age of 50, larger and dark brown colour
treatment:
no treatment
skin tumors
benign
actinic keratoses
common lesion, proliferations of transformed neoplastic keratinocytes
lesions are typically hyperkeratotic, appearing rough and scaly (schuppig) with a diffuse erythematous base, sandpaper like texture
can transform into squamous cell carcinoma, about 5-8% carry the risk when they are over 10-25y, signs of malignant transformation - ulceration, induration, enlarging size or bleeding
treatment:
designed to be destructive to the lesion, including cryotherapy, topical 5-flourouracil, topical imiquimod, photodynamic therapy (topical administration of photosensitizer accumultes in the cells), then light is applied which activates the photosensitizer and produce free radicals that damage the cells, better than cryotherapy
treatment:
excision - with large margins from 4-6mm,
skin tumors
benign
melanocytic nevi
benign neoplasms of melanocytes, involvement of nevus cells, have the risk to transform into melanoma, melanocytic nevi - clusters or “nests” of melanocytes
2 categories:
congenital - present at birth or appear in the first week of life, greater size with higher risk of malignant transformation, giant congenital melanocytic nevi are associated with neuromelanosis and melanoma of the brain, many treatments - excision is the best
acquired melanocytic nevi - after 6 month of age - associated with sun exposure - round or oval shaped, less than 1cm, well demarcated borders
3 groups:
junctional (cells in the dermal-epidermal junction), compound (in both areas), intradermal (only in the dermis)
at hand and feet so called - acral nevi, when there is a sign for malignant transformation than biopsy
Dysplastic or atypical nevi also known as Clark’s nevus - precursor to malignant melanoma - typical display:
- asymmetry
- irregular borders
- variable colour
- larger size >6mm
excision should be performed with a margin minimum of 1-2mm,
skin tumors
malignant
common cancer in humans overall with an estimated lifetime incidence of 1 in 5 adults, 10-15% of all skin cancers occur at the hand, mostly the dorsum of the hand
cutaneous malignancies are most frequently nonmelanoma skin cancers - mostly basal cell cancer (BCC) or squamous cell cancer (SCC)
rare: Melanoma (MM), dermatofibrosarcoma protuberans (DFSP), Merkel cell carcinoma (MCC), very rare arising from the sebaceous glands (eccrine porocarcinoma, malignant acrosproma and papillary adenocarcinoma)
metastasis to the hand:
33% - bronchogenic tumors
25% - GIT
10% - renal cell carcinoma
5% - breast carcinoma
distal phalanx the most involved bone, thumb involved overall,
skin tumors
malignant
Basal cell carcinoma (BCC)
most common cutaneous malignancy in humans, compromising 80% of skin cancers, less seen in the hand than squamous cell carcinoma (SCC).
cell of origin is the basal cell of the epithelium, mostly sun-exposed areas and more seen with fair skin individuals, ultraviolet radiation exposure is the main risk for development (others: male, ionizing radiation, arsenic exposure, photosensitizing drug use)
associated with genetic disorders - such as:
- xeroderma pigmentosum
- Gorlin’s syndrome (basal cell nevus syndrome)
associated with immunosuppressed or post-transplant patients
mostly on the dorsum of the hand between the metacarpal heads and wrist crease, slow growing, metastasis occur in tumors >3cm
several subtypes:
high risk - superficial
high risk - micronodular
high risk - morpheaform, locally destructive, recurrence
high risk - infiltrative, locally destructive, recurrence
low risk - nodular (most common)
pink, dome-shaped papule with central ulceration, red or brownish discoloration, skin atrophy, telangiectasias
treatment:
primarily surgical - primary excision or Moh’s micrographic surgery - low risk cure rate - 95% at 5y
should be excised with a margin of 4mm, Re-excision with any positive margin, adjuvant radiation with permanent positive margins
surgical margins for BCC and SCC
size smaller than 2cm than 4mm margin
size greater than 2cm wider local excision or Moh’s resection, SCC 6mm if size greater than 2cm, or Moh’s resection, or frozen section margins
skin tumors
malignant
Squamous Cell Carcinoma (SCC)
second most common cutaneous malginancy, but the most common cutaneous malignancy and malignant tumor of the hand overall
origin from keratinocytes, different histologic types including well, moderately or poorly differentiated
association with ultraviolet radiation, organic chemicals, arsenic, human papillomavirus, chronic healing wound, chronic inflammatory disorders, immunsuppression, post-transplantation and precursor lesions such as actinic keratoses (6-10% malignant transformation) and SCC in situ (Bowen’s disease- 3%)
dorsal hand or forearm, present as erythematous plaque or nodule with scaling, ulcerate or easy bleeding, or large mass with fungation or necrosis, subungual SCC presents with nail destruction, subungual SCC mimics a chronic paranychia or a subungual wart
treatment:
lesion <1cm treated with cryotherapy with liquid nitrogen, or electrodessication and curettage - SCC <2cm excision with a 4mm margin, >2cm excision with a 6mm margin, deep margin with subcutaneous fat
best treatment:
- assessing margins via frozen section
- Moh’s resection
- prior wound closure when margins all negative
high-risk of metastasis with transplant patients - 65 times higher than control group, risk of axillary lymph node metastasis is low - 2-5%
5y primary cure rate is 92%, 5y recurrence rate is 8%, positive lymph nodes 5y cure rate is 30-40%, with distant metastasis the 10y survival is less than 10%
tumor >2cm with 2-3 additional high-risk recurrence and metastases - sentinel lymph node biopsy (SLNB)
skin tumor
malignant
Malignant Melanoma (MM)
4% of all malignant primary skin cancers, but 78% of all skin cancer related deaths, mortality is higher with african americans and with hispanic americans, sun exposure to light-skin individuals is a very high risk (caucasians in Australia or New Zealand)
MM of the hand and forearm is rare - only 0,9-3% occur in the hand, majority is the dorsal forearm and wrist, Melanoma of the nail matrix is also uncommon with 0,7-3,5% of all cases
incidence various by ethnicity - european - subungual MM only 1-2%, asian about 10-23%, african about 25%
origin from the melanocytes - a melanin producing cell
melanonychia striata - brown nail bed - involvement of the dorsal nail fold is called “Hutchinson’s sign”
Evaluation of a MM:
A - asymmetry
B - border irregularity
C - color variation
D - diameter >5mm
E - evoluation of a pigmented lesion
Melanoma classification:
superficial spreading - 70%
nodular - 15%
lentigo maligna - 13%
acral lentiginous - 2-3% (mostly asians and africans - palm and sole, subungual or mucous membranes)
Melanoma may invade and spread perineurally, hematogenously or lymphatically - satellite lesions as “intransit metastasis” is a poor prognosis
Staging:
3 factors are used
T - thickness of tumor
N - lymph node involvement
M - distant metastases
Biopsy:
resection with the subcutaneous layer
treatment:
excision is based upon the thickness of the tumor,
melanoma in situ only requires negative margins - 5-9mm margins for MM in situ
T1 - MM <1mm - margins 1cm
T2 - MM 1,01-2mm - margins 1-2cm
T3 - MM 2,01-4mm - margins 2-3cm
T4 - MM >4mm - margins > 2cm
deep margins should include the deep fascia, paratendon, muscle fascia, SLNB when T2 stadium is reached - all tumors >1mm
subungual MM:
thumb mostly involved, treatment normally is an amputation, recurrence is 12,2% in comparison to amputation (only 2%)
lymph node management
all MM >1mm, SLNB, if positive than - lymphadenectomy
skin tumors
malignant
Merkel Cell Carcinoma (MCC)
rare aggressive carcinoma arising from the neuroendocrine Merkel cells - localized in the epidermal basal layer - slowly adapting receptors - responsible for light touch discrimination
males are common, incidence increases with age, hand and upper extremity involved in 20-40%, aggressive cancer with high recurrence and metastatic spread via lymphatic system
painless, firm, nontender rapidly growing nodule with a bluish and reddish colour, mean age of 76y, 40% have SCC, BCC or precursor lesions (actinic keratoses or Bowen’s disease), 30% are immunsuppressed by organ transplantation or malignant hemotologic disease, 30% of patients have nodal disease at time of diagnosis, poor prognosis - median survival after diagnosis is 13 month
treatment:
surgical excision with a margin of 3cm, including the deep fascia, local recurrence 33% when margin is 2-3cm and about 10% when margin is 3cm,
skin tumors
malignant
dermatofibrosarcoma protuberans
rare tumor, low grade sarcoma of fibroblastic origin, difficult to distinguish from a benign dermatofibroma
irregular dimensions and tentacle-like extensions into the adjacent surrounding tissue, local recurrence from 10 - 60%, distant metastases 4-5%, local metastasis only 1%
treatment:
deep punch excision!!!, superficial portion only benign lesion, Moh’s resection or margins from 2-4cm, no flap for reconstruction - only full thickness skin graft, maybe radiation adjuvant and tyrosine kinase inhibitor - “Imatinib mesylate”
