June 2007

Tumor Unknown Conference

 Case 1

C.G. - 21 year old male with a history

of curretage and bone grafting of a
distal ulnar lesion eighteen months
ago
 Aneurysmal Bone Cyst (ABC)
Osteolytic, hyperplastic, hyperemic-hemorrhagic
lesion of unknown origin
Epidemiology
80% before 20 years of age, rare after 30 years old
Most common sites: long bones LE > UE
30% secondary lesions due to pre-existing tumor
Radiographs
Eccentric, expansile, lytic lesion of metaphysis
Cortical attenuation or destruction
Rim of reactive bone
Fluid-Fluid levels on MRI are characteristic
 ABC
Histology
Cavernous spaces filled with blood, lacking
endothelial lining
Fibroblastic cells, multinucleated giant cells
and thin strands of bone
 ABC
Treatment
Excision, curettage, and bone grafting
Resection in case of an expendable bone.
Other modalities include adjuvant cryo,
chemical cautery, injection of steroids
Radiation not recommended
Prognosis
Local recurrence rate approx 10%, increased if
open physis
 Case 2

T.K. - 18 year old male with

asymptomatic left ankle swelling for
several months
 Aneurysmal Bone Cyst (ABC)
Osteolytic, hyperplastic, hyperemic-hemorrhagic
lesion of unknown origin
Epidemiology
80% before 20 years of age, rare after 30 years old
Most common sites: long bones LE > UE
30% secondary lesions due to pre-existing tumor
Radiographs
Eccentric, expansile, lytic lesion of metaphysis
Cortical attenuation or destruction
Rim of reactive bone
Fluid-Fluid levels on MRI are characteristic
 ABC
Histology
Cavernous spaces filled with blood, lacking
endothelial lining
Fibroblastic cells, multinucleated giant cells
and thin strands of bone
 ABC
Treatment
Excision, curettage, and bone grafting
Resection in case of an expendable bone
Other modalities include adjuvant cryo,
chemical cautery, injection of steroids
Radiation not recommended
Prognosis
Local recurrence rate approx 10%, increased if
open physis
 Case 3

S.P. - 12 year old male with left

hip pain after a minor fall
 Unicameral Bone Cyst
Benign fluid filled cystic lesion of bone
of unknown cause
Epidemiology
5-15 years of age
Male > Female
Proximal humerus most common, followed
by proximal femur
 Unicameral Bone Cyst
Symptoms
Asymptomatic unless fracture occurs
Radiographs
Central lytic lesion of metaphysis
Attenuation and slight expansion of cortex
Fluid filled on CT/MRI
 Unicameral Bone Cyst
Histology
Thin fibrous membrane lining cyst
Few macrophages, giant cells, leukocytes
and slivers of bone and osteoid
 Unicameral Bone Cyst
Treatment
Cysts become inactive at skeletal maturity
and typically resolve without surgical
intervention
Multiple treatments including curettage and
bone grafting, steroid injections have been
tried with variable efficacy
Fractures tend to lead to resolution of cyst
Displaced fractures are the only absolute
surgical indication
 Case 4

A.M. - 87 year old female with an

asymptomatic posterior thigh soft tissue
mass present for many years, the patient
reports that it has increased in size over the
past three months
 Lipoma
Benign tumor of mature fat
Classification
Superficial: (more common)
contained within subcutis
prevalence for back, neck, proximal limbs
Deep: (less common)
within muscle or intermuscular spaces
Age
Most frequent between 40-60 years old
Presentation
Slow growing asymptomatic mass
 Lipoma
Imaging
Resembles normal fat on all CT and MRI images
Histology
Mature fat cells (lipocytes)
Rarely small areas of hemmorrage, necrosis,
calcification
Absence of lipoblasts and pleomorphic, hyperchromatic
nuclei differentiate lipoma from liposarcoma
Treatment
Marginal Excision
Local recurrence is rare
 Liposarcoma
Primary malignant tumor arising from fat
15% of all soft tissue tumors
Usually presents at 40-60 years of age
Two main types
Myxoid: usually low grade Stage I lesion
Cytogenic marker: reciprocal translocation on chromosome
12
Pleomorphic: usually high grade Stage II lesion
 Liposarcoma
Can be very large at presentation due to
anatomic location and deep-seated, slow-growing
nature
Also typically not painful or tender
MRI
Similar to other soft tissue sarcomas,
with multilobular configuration
T1) Low intensity
T2) Bright signal
 Liposarcoma
Myxoid Histology
Sheets of lipocytes or lipoblasts interspersed in myxo-
matous amorphous matrix with low cell to matrix ratio
Fine branching capillaries with arborization pattern,
resemble a road map
Pleomorphic Histology
More cellular
Lacks plexiform capillary network
May have large, bizarre lipoblasts with abundant
eosinophilic cytoplasm
 Liposarcoma
Treatment
Preoperative adjuvant radiation therapy
Wide surgical excision
Treatment
Wide local excision
Radiotherapy may be used to control local recurrence
and lessen the risk of metastasis
Prognosis
Five year survival: well differentiated 85-100%,
myxoid 75-95%, round cell and pleomorphic
20-50%, dedifferentiated 30%
Local recurrence 10% with adequate margins
 Case 5

T.S. - 44 year old male with six month

history of an anterior shoulder mass
 Pleomorphic Sarcoma
Malignant soft tissue tumor of
mesechymal origin
Epidemiology
7000 new cases of soft tissue sarcomas
diagnosed yearly
Lesions classified according to direction of
differentiation
Can be caused by radiation exposure
 Pleomorphic Sarcoma
Symptoms
Enlarging mass, most are large, deep and
firm at diagnosis
Can be painless or painful
Radiographs
MRI is the best imaging modality
 Pleomorphic Sarcoma
Histology
Dedifferentiated mesenchymal cells
Grade of tumors determined on histology
Treatment
Radiation therapy
Wide surgical excision, limb salvage if possible
Prognosis depends on
Tumor Grade
Size (> 5 cm)
Location superficial or deep to fascia
 Case 6

D.E. - 15 year old male with left knee pain

after falling from a skateboard three weeks
ago
 Chondroblastoma
Benign tumor composed of chondroblasts
Epidemiology
Male: female is 3:1
10-20 years of age
Localization
Epiphysis, apophysis, or short bone
Extension to metaphysis and even through
physeal plate.
Proximal humerus>distal femur>proximal
tibia>proximal femur
 Chondroblastoma
Clinical Presentation
Pain, usually referred to a joint
Moderate to long duration
Imaging
XR: Osteolytic with smooth boarders,
eccentric
CT/MRI: Well defined boarders, fuzzy mottled
intratumoral radio densities
 Chondroblastoma
Gross
Rubbery soft with sharp limits toward the
surrounding bone
Pink to gray to tan in color
Whitish foci or chalky granules (calcifications)
Histo
Highly cellular: Chondroblasts and Giant cells
Pathognomonic: calcium in fine granules
deposited on a reticular network producing a
chicken-wire pattern
 Chondroblastoma
DDX
Giant Cell Tumor, Clear Cell Chondrosarcoma,
Chondroma, Chronic Brodies abscess
Treatment
Stage 1, 2, and some stage 3: intralesional
excision after frozen confirmation
Stage 3: wide intra or extraarticular segmental
resection
Prognosis
Local recurrence < 10% after curettage in
stage 3 tumors, rare after wide resection
 Case 7

T.L. - 49 year old male with left elbow

pain and swelling after a fall
 Osteomyelitis
Bone Infection
Acute hematogenous, subacute focal disease,
and chronic types
Epidemiology
Most common in infants and children
Declining incidence due to better dx/tx
Staph aureus most common in all age groups
 Osteomyelitis
Symptoms
Pain, decreased ROM, swelling, erythema,
fever, malaise, irritability
Radiographs
Delayed changes on imaging (2-4 weeks)
Lytic lesion with mottled appearance
usually in metaphysis
Periosteal elevation/reaction
 Osteomyelitis
Histology
Mixed cell population
Sequestrum: dead cortical bone
Involucrum: new cortical bone
Treatment
Biopsy/culture
Acute
IV antibiotics surgical debridement
Chronic
IV antibiotics with surgical debridement
 Case 8

K.M. - 13 year old female with a painful

soft tissue mass of the left anterior knee
 Epithelioid Granulomatous
Inflammation
Chronic inflammation
A compact collection of cells of the
mononuclear phagocyte system
Types
Low Turnover: Foreign body
High Turnover: Epithelioid, hypersensitivity
Treatment
Marginal excision
 Case 9

N.S. - 19 year old male with a chest wall mass

discovered on an x-ray for suspected
bronchitis
 Calcifying Fibrous Tumor of Pleura
Very rare benign fibrous tumor of pleura
Composed of hyalinised collagen with psam-
momatous calcification and inflammatory
infiltrate
Rare cases reported in the literature are in
patients less than 30 years old
Imaging shows circumscribed lesion with
increased density centrally which represents
areas of calcification
Treatment is typically excisional biopsy to rule
out sarcoma
 Case 10

A.Z. - 70 year old female with left distal

thigh pain and swelling for several
weeks
 B-Cell Lymphoma
Neoplasm of B-Lymphocytes
Most common Non-Hodgkin lymphoma
Fourth through seventh decades
Primary lymph node disease
Extranodal sites
More than 50% of patients will have
extranodal involvement at diagnosis
Primary bone lymphomas are rare
(5% of extranodal disease)
Most common site is the thigh
 Large B Cell
Lymphoma of Soft Tissue
Symptoms
Often mild and prolonged before diagnosis
Radiographs
Lytic lesion with moth eaten appearance
Histology
Dense sheets of round cells
CD-20 positive
 Large B Cell
Lymphoma of Soft Tissue
Treatment
Combined chemotherapy and radiation to
bony sites
Chemo: Cytoxan, adriamycin, vincristine,
and prednisone
If remission is obtained by 6 cycles of
chemo, cure rates approximate 60-70%