Bone & Soft

Tissue Tumors
Tumor Like Lesions

Vernygorodskyi Sergii, MD, PhD, DMedSc

All Saints University School of Medicine
 OBJECTIVES

 Bone tumors and Tumor Like Lesions: Osteoma, Osteoid Osteoma,

Osteoblastoma, Osteosarcoma, Osteochondroma, Chordoma,
Chondroblastoma, Chondrosarcoma, Fibrous dysplasia,
Fibrosarcoma, Ewings Sarcoma, Metastatic disease.
 Soft Tissue Tumors and Tumor like lesions: Lipoma, Liposarcoma,
Fibromas, Fibrosarcoma, Myositis Ossificans, Fibromatosis, Desmoid
Tumors, Benign Fibrous Histiocytomas, Malignant fibrous
Histiocytomas, Rhabdomyosarcoma, Leiomyoma, Leiomyosarcomas,
Synovial Sarcoma.
 Tumors and Tumor Like Lesions of Bone-Ganglion & Synovial cyst,
Tenosynovial giant-cell tumor, Aneurysmal Bone Cyst.
 CLASSIFICATION OF MESENCHYMAL TUMOURS
Origin of the tumours Benign tumours Malignant tumours
Bone tissue Osteoma Osteosarcoma
Chondroma Chondrosarcoma

Connective (fibrous) tissue Fibroma, desmoid Fibrosarcoma

Fat tissue Lipoma Liposarcoma
Muscle tissue: Myoma Sarcoma
smooth muscle Leiomyoma Leiomyosarcoma
striated muscle Rhabdomyoma Rhabdomysarcoma
Blood vessels Hemangioma Angiosarcoma
Lymphatic vessels Lymphangioma Lymphangisarcoma
Synovial tissue Benign synovioma Synovial (malignant
synovioma) sarcoma
Mesothelial tissue Benign mesothelioma Malignant mesothelioma
 Bone tumors Classification
 Primary and Secondary (metastatic). Primary bone tumors:

Histologic type Benign Malignant

Hematopoietic (40%) Myeloma, lymphoma
Osteochondroma, chondroma, Chondroscarcoma,
Chondrogenic (22%) chondroblastoma, dedifferentiated and
chondromyxoid fibroma mesenchymal chondrosarcoma
Osteoid osteoma,
Osteogenic (19%) Osteosarcoma
osteoblastoma
Unknown (10%) Giant cell tumor Ewing sarcoma
Histiocytic Fibrous histiocytoma Malignant fibrous histiocytoma
Desmoplastic fibroma,
Fibrogenic Fibroma
fibrosarcoma
Notochordal Chordoma
Hemangioendothelioma,
Vascular Hemangioma hemangiopericytoma,
angiosarcoma
 Bone tumors
 LOCATION AGE RADIOGRAPHY MORPHOLOGY
Analytic approach to evaluation of the bone neoplasm
  Subchondral cysts

Chondromyxoid fibroma
Anatomic locations of common primary bone tumors
 Radiographic features Benign vs. Malignant Lesions
Non-aggressive Lesions: Well demarcated,
Narrow zone of transition, Absent or geographic
osteolysis, Cortex may be displaced, remodeled
and thin, but not broken, Solid, smooth periosteal
reaction +/- surrounding sclerosis, Static or slow
rate of change
Aggressive Lesions: Poorly demarcated,
Wide zone of transition, Poorly marginated
osteolysis, Cortex interrupted. Interrupted
irregular , periosteal reaction, No surrounding
sclerosis, Rapid rate of change
 Diagnostic approach to radiographs of focal
osseous lesions
1.Identify the segment of bone involved (epiphysis, metaphysis, or diaphysis).
2.Locate the area of primary involvement: medulla, cortex, periosteum, or soft tissue.
3.Identify whether one or several bones, and whether appendicular or axial skeleton.
4.Characterize the type and margin of each individual lesion. Note any matrix within
the lesion. Identify the reaction of the native bone to the lesion.
Multiplicity of lesions in an older adult usually indicates metastatic disease, multiple
myeloma or lymphoma.

In childhood, multiple lesions are more likely

to be benign and the differential includes
polyostotic fibrous dysplasia, Langerhans cell
histiocytosis multiple enchondromatosis
(Ollier’s disease) and multiple exostoses.
 Bone Benign Tumors
Osteoma - benign neoplasm that frequently involves
the skull and surface of facial bones. > 30 yers
"Hyperostosis frontalis interna" describes an osteoma
that extends into the orbit or sinuses.
Associated with Gardner syndrome (colonic polyposis,
fibromatoses, osteomas, and epidermal cysts of skin).

Osteoma: dense cortical-type bone, resembles normal Osteoma: radiodense lesions,

similar to normal cortex . CT.
 Bone Benign Tumors
 Osteoid osteoma
 benign tumor arising from osteoblasts painful growth of the diaphysis of a
long bone, often the tibia or femur
 Small < 2 cm lesion of the cortex. Classically, it can almost always be
“shelled” out from the surrounding bone.
 Males> females; age 5-25 years
 Pain (excess production of prostaglandin E2) that is worse at night and
relieved by aspirin
 X-rays: central radiolucency surrounded by a sclerotic rim
 Grossly, the nidus is red, spherical, and gritty.
 Central nidus of osteoid surrounded by dense sclerotic rim of reactive
cortical bone
 Osteoid osteoma

X-ray: a sharply delineated, small,

cortical lesion

The center of the lesion : fibrovascular

tissue (the).
The periphery: nidus a sharply
delineated bone-forming lesion.
The trabeculae are rimmed by benign-
appearing osteoblasts
Osteoblastoma
Benign, > than 2 cm and involves the vertebrae more frequently; the pain is
dull, achy, and unresponsive to salicylates, and the tumor usually does not
induce a marked bony reaction. 80% < 30 years.
Histologically similar to osteoid osteoma
X-ray: radiolucent lesion
surrounded by narrow rim of
reactive bone.
Osteochondroma
MC benign tumor (an exostosis of the bone). 
Males < 25 years old.
Reduced expression of EXT1 and EXT2
results in defective endochondral ossification 
Mature bone protrusion with cartilaginous  In patients with breast- or prostate cancer a
(chondroid) cap.
Rarely transforms to chondrosarcoma.
Metaphyseal location of tumor
Bone island
Well-differentiated mature bone tissue within
the marrow, also referred to as enostosis. 
20-40 years.
Usually it is seen as a coincidental finding. 
bone island can be mistaken for an
osteoblastic metastasis. 
A bone island normally does not show
increased uptake on a bone scan.
 Osteochondroma
Outer aspect of an osteochondroma arising on the upper fibula demonstrating the
continuity of the cortex of the lesion and the underlying bone (mushroom shaped )
cartilage cap
 Giant cell tumor
 20–40 years old.
 Epiphyseal end of long bones.
 “Osteoclastoma.”
 Locally aggressive benign
tumor often around knee.
 “Soap bubble” appearance on
x-ray.
 Multinucleated giant cells
 Giant osteoclast-like cells are
believed to form via the
RANK/RANKL signaling
pathway
 Differential diagnosis:
 ABC may have the same
radiographic features but is found in
a younger age group.
 Chondroblastoma is also located in
the epiphysis, but is seen
exclusively in the epiphysis without
extention to the metaphysis and is
seen in a younger age group.
 Metastases, especially in older
patients.
 Case Presentation
A 35 year man presented with gradually increasing swelling in wrist from 2
years ago which became painful in last 6 months.
Radiograph wrist AP& lateral views
showing an expansile lesion giving
characteristic "soap bubble appearance"
involving the subarticular region of radius
with cortical breech on medial aspect.
 Osteosarcoma (osteogenic sarcoma)
 2nd most common 1° malignant bone tumor (after multiple myeloma).
 Bimodal distribution: 10–20 years old (1°), > 65 (2°).
 Predisposing factors: Paget disease of bone, bone infarcts, radiation, familial
retinoblastoma
 Li-Fraumeni syndrome (germline p53 mutation, Sarcoma, breast, leukaemia
and adrenal gland (SBLA) syndrome).
 Metaphysis of long bones, often around knee. Path fractures with pain
 Codman triangle (from elevation of periosteum) or sunburst pattern on x-ray.
 Alkaline Phosphatase ↑ (dif. D-s with metastatic carcinomas).
 Sporadic cases: loss of both alleles of RB1, familial: inheritance of one bad
copy, and in those cases retinoblastomas are likely to appear first.
 Aggressive. Treat with surgical en bloc resection (with limb salvage) and
chemotherapy.
 Osteosarcoma

Distal femoral osteosarcoma with prominent bone formation

extending into the soft tissues.
The periosteum, which has been lifted, has laid down a proximal
triangular shell of reactive bone known as a Codman Major sites of origin of
triangle (arrow). osteosarcomas.
"Sunburst” pattern (seen mainly in periosteal osteoSa)
 Osteosarcoma

Coarse, lacelike pattern of neoplastic bone

produced by anaplastic malignant tumor cells.
Note the mitotic figures.
Osteosarcoma of the upper end of the tibia. The tan-white tumor fills most of the
medullary cavity of the metaphysis and proximal diaphysis. It has infiltrated through the
cortex, lifted the periosteum, and formed soft-tissue masses on both sides of the bone.
 Osteosarcoma

IHC staining with antibody to vimentin reveals positive red-brown reaction product within the cytoplasm of
these neoplastic cells. P-ve vimentin staining is a characteristic of many types of sarcomas. Note the
spindle shape of many of these cells, another feature of neoplasms of mesenchymal derivation.
 OSTEOGENIC
 OSTEOMA - skull and facial bones, > 30 yr, Gardner syndrome, resemble
normal bone
 OSTEOID OSTEOMA – DIAPHYSIS of Long Bones (tibia, femur) in cortex,
males>females, Pain (aspirin), < 30 yr, < 2 cm, nidus of osteoid, produced
by osteoblasts surrounded by dense sclerotic rim of reactive cortical bone,
radioluscent with central mineralazation
 OSTEOBLASTOMA vertebrae, > 2cm, m/f 2-3:1, < 30 yr
 OSTEOCHONDROMA, METAPHYSIS, Males < 25 yr, protrusion with
cartilaginous (chondroid) cap.
 GIANT CELL TUMOR, EPIPHYSIS of long bones., > 20yr, Soap bubble
MultGiantC
 OSTEOSARCOMA: METAPHYSIS of long bones (around knee), 10-20 yr
and >65, Codman triangle (from elevation of periosteum) or sunburst
anaplastic cells with osteoid production
Epithelial vs Mesenchymal
IHC
 Chondromas (Chondrogenic tumors)
 Enchondroma - tumor grows within the bone and expands it. Ecchondroma - grows
outward from the bone and this is rare.
 Situated in the metaphysis or diaphysis (in medulla); multiple chondromas can occur
as a skeletal dysplasia. Within the medullary cavity, where they are known as
enchondromas, or on the surface of bone - subperiosteal or juxtacortical chondromas
 Multiple enchondromas sometimes tend toward unilaterality,  affected extremity is
shortened (asymmetric dwarfism)  - Ollier disease.
 Multiple enchondromas can also rarely be associated with hemangiomas of the soft
tissues and phleboliths (calcifications) - Maffucci syndrome.

Pain and periostitis is sign of malignancy, i.e.

chondrosarcoma

Enchondroma with a nodule of hyaline cartilage

encased by a thin layer of reactive bone.
X-ray: “O”-Rings
 “O”-Rings

Enchondroma: X-ray: “O”-Rings

 Chondroblastoma
 A rare benign tumor - less than 1% of primary bone
tumors.
 in young patients in their teens and has a male-to-
female ratio of 2 : 1.
 Most arise about the knee; less common sites
such as the pelvis and ribs are affected in older
patients.
 Chondroblastoma has a striking predilection for
epiphyses
 Painful, and because of their location near a joint
they also cause effusions and restrict joint mobility
 Chondroblastoma
X-ray: Not oval as in enchondral,
but irregular shaped like popcorn.

Chondroblastoma with scant mineralized

matrix surrounding chondroblasts in a
chicken wire–like fashion.
Malignant bone tumor that produces cartilage
Large osteolytic lesion with scalloping of the inner
cortex and rings-and-arcs or popcorn calcifications in
an elderly person with a painful swelling.
Secondary chondrosarcoma (MC, from
enchondroma or osteochondroma). Patients with
multiple enchondromas like in Ollier's disease and
Mafucci's syndrome are at risk.
Features: Elderly patient, Location in long bones
Pelvis. Size > 5 cm, Uptake on bone scan
Endosteal scalloping on MRI
Cortical involvement
Early enhancement on dynamic contrast enhanced
series

On the left a chondrosarcoma in the proximal tibia diaphysis.

The tumor is recognized by subtle calcifications in the proximal
part.
The distal border is not well defined.
Notice endosteal scalloping at the medial side which is a
hallmark of chondrosarcoma.
MR better defines the extension of the lesion.
MRI also demonstrates the endosteal scalloping.
Chondrosarcoma: a translucent firm, glistening bluish gray lesion, with smooth, shiny cut surfaces

Anaplastic chondrocytes within a chondrosarcoma.

 Comparison of benign and malignant cartilaginous tumors
Enchondroma Chondrosarcoma
Pain No Yes
Location Appendicular Axial
Circumscribed without cortical Endosteal scalloping,
Radiologic findings
involvement cortical disruption
Low (but can be higher in
Cellularity Usually high
appendicular sites)
Bone
No Yes
engulfment/permeation
Myxoid change No Yes

Osteochondroma - Metaphysis
Chondroblastoma - Epiphysis
Enchondroma - Diaphysis,
small bones
CHORDOMA

Rare low-grade malignant tumor

usually in older patients.
Typical presentation: expansile,
destructive bone lesion that may be
associated with a soft-tissue mass.
Spine clivus.

On MRI T2-weighted. Lytic lesions of the C2

and C3 vertebrae with cortical destruction
posteriorly.
The differential diagnosis based on the CT-
findings includes primarily metastases and
myeloma.
Abnormalities with soft tissue extension and
compression of the myelum.
 Ewing sarcoma/Primitive neuroectodermal tumor
 Boys < 15 years old.
 Associated with t(11;22) translocation causing fusion protein
EWS-FLI 1. 11 + 22 = 33 (Patrick Ewing’s Jersey number).
 Centrally in medulla of diaphysis of long bones, pelvis,
scapula, ribs. From neuroectoderm
 Anaplastic small blue cell malignant tumor, rosette formation.
 Ill-defined osteolytic lesion with a moth-eaten type of bone
destruction, irregular cortical destruction and aggressive
periostitis in the lower extremity of a child.
 MR imaging reveals the soft tissue extension.
 “Onion skin” periosteal reaction in bone.
 Extremely aggressive with early metastases, but responsive to
chemotherapy.
Differential diagnosis:
Osteosarcoma: particularly when reactive
sclerosis is present.
Primary lymphoma: permeative pattern of
destruction and often a large soft tissue mass.
The mean age of presentation in these
patients is usually higher than in Ewing
sarcoma.
In some cases osteomyelitis or eosinophilic
granuloma may mimic Ewing sarcoma.

A large lytic tumor arising from the right iliac bone.

On the plain film it is very hard to appreciate the
lesion because of the permeative destruction
pattern.
Scintigraphy shows extensive uptake within the iliac
bone.
MR reveals the intra- and extraosseous tumor
extension
Ewing sarcoma (ES). Low-power view (A) shows a small round blue cell neoplasm
composed of uniform cells. High-power view (B) demonstrates fine powdery nuclear
chromatin pattern and focal rosette formation. (C) Immunostaining with CD 99
(MIC2) shows a positive membranous pattern consistent with ES.
 Multiple Myeloma
 clonal proliferation of neoplastic PLASMA CELLS in the bone marrow associated
with MULTIFOCAL LYTIC lesions throughout the skeleton system. Produces large
amounts of IgG (55% of cases) or IgA (25% of cases) 
 MC primary tumor of the bone/ bone marrow in patients > 50 years of age
Symptoms: bone pain, sequelae associated with hypercalcemia, with renal failure
Serum protein electrophoresis: monoclonal immunoglobulin spike (M protein)
Urinalysis: Ig light chains (Bence Jones protein)
Peripheral blood smear: RBC rouleaux formation, Igs coat RBC and neutralize the
ionic charge than normally repels RBCs

Bone marrow aspiration: proliferation of large monoclonal

plasma cells , "fried-egg" appearance
Serology: hypercalcemia result of bone degradation due
to cytokine production that activates RANKL receptor on
osteoclasts, prolonged bleeding time
Imaging: punched-out lytic bone lesions on radiograph
A skull x-ray : "punched-out" holes
 Tumors of Bone

Common Age Morphology

Tumor Type Locations yr
Bone-Forming, BN
Osteoma Facial bones, 40-50 Exophytic growths attached to bone
skull surface; histologically resemble normal
bone
Osteoid osteoma Metaphysis of 10-20 Cortical tumors, characterized by pain;
femur and tibia histologically interlacing trabeculae of
woven bone
Osteoblastoma Vertebral 10-20 Arise in vertebral transverse and spinous
column processes; histologically similar to osteoid
osteoma
MALIGNANT
Primary Metaphysis of 10-20 Grow outward, lifting periosteum, and
osteosarcoma distal femur, inward to the medullary cavity;
proximal tibia, microscopically malignant cells form
and humerus osteoid; cartilage may also be present
Secondary Femur, >40 Complications of polyostotic Paget
osteosarcoma humerus, pelvis disease; histologically similar to primary
osteosarcoma
Cartilaginous, BN yr
Osteochondroma Metaphysis of 10- Bony excrescences with a cartilaginous cap;
long tubular 30 may be solitary or multiple and hereditary
bones
Chondroma Small bones of 30- Well-circumscribed single tumors resembling
hands and feet 50 normal cartilage; arise with medullary cavity
of bone; uncommonly multiple and hereditary

MALIGNANT
Chondrosarcoma Bones of 40- Arise within medullary cavity and erode
shoulder, 60 cortex; microscopically well differentiated
pelvis, proximal cartilage-like or anaplastic
femur, and ribs

Miscellaneous
Giant-cell tumor Epiphysis of 20- Lytic lesions that erode cortex;
(usually benign) long bone 40 microscopically, contain osteoclast-like giant
cells and round to spindle-shaped
mononuclear cells; majority are benign
Ewing tumor Diaphysis and 10- Arise in medullary cavity; microscopically:
(malignant) metaphysis 20 sheets of small round cells that contain
glycogen; aggressive neoplasm
 Aneurysmal Bone Cyst (ABC)
ABC a painful expansile or 'aneurysmal' well-defined
osteolytic benign multicystic lesion of bone, which is
composed of blood-filled spaces, separated by connective
tissue septae containing giant cells and reactive bone in a
patient < 30 years.

Central or eccentric origin in the metaphysis or diaphysis

of a long bone.
In the spine located in the body or in both body and arch.
X-ray expanded bone and a “shell” around
On MR with fluid-fluid levels due to blood sedimentation.
ABC most frequently presents around the knee.
Other: proximal humerus or spine, but many other bones
are possible.

Expansile well-defined osteolytic bone lesion

in the fibula.
The T2-weighted MR-image shows the fluid
content and on the T1-weighted image there is
a subtle fluid-fluid level
 ABC

ABC. A low-power photomicrograph showing a hypocellular lesion with

septations composed of fibroblasts and fibrocollagenous matrix.
Sprinkling of giant cells and osteoid is visible
 Non-ossifying fibroma (NOF)
eccentric well-defined lytic lesion, usually found as a
coincidental finding. usually regress spontaneously and may
then become sclerotic. Metaphysis.
a peak incidence at 10-15 years old, associated with 
neurofibromatosis type 1 (NF1), FD
Storiform pattern created by
benign spindle cells with
scattered osteoclast-type giant NOF at the upper end of the tibia
cells characteristic of a fibrous near the knee joint. About 43% of
cortical defect and non- NOFs occur in the tibia.
ossifying fibroma

On the left : a typical osteolytic NOF with a

sharp sclerotic border. The image on the right
is of a different patient who has an old NOF
that shows complete fill in.
Chondromyxoid Fibroma (CMF)
is a rare lesion, resembles NOF.
Preferential sites: proximal tibia and foot. 
There is an eccentric osteolytic lesion in
the metaphysis of the proximal tibia. 

On the inner side there is a sclerotic margin. 

On the outer side there is a regular cortical
destruction with peripheral bone layer.
The MR also shows a sclerotic margin with
low signal intensity.

CMF with prominent stellate and spindle

cells surrounded by myxoid matrix.
Occasional osteoclast-type giant cells are
also present.
Fibrous Dysplasia
Fibro - osseous tissue that replaces the marrow space
Age: < 30 yrs (Arises in adolescents and becomes quiescent when bone growth is
completed). Does not require treatment (except pathological fractures and cosmetic
problems).
 Monostotic form, MC, asymptomatic solitary lesions, spontaneous fractures (lesion
with central lucency and a thin sclerotic rim). Intramedullary and well circumscribed.
Polyostotic form (multiple, associated with severe deformity )
McCune –Albright syndrome: 1 .Polyostotic fibrous dysplasia (PFD), 2. Café-au-lait
 skin pigmentation, short stature in young girls. 3. Autonomous endocrine
hyperfunction (eg, gonadotropin-independent precocious puberty). Mutation of
the GNAS1  (20q13.3., G protein).
Location. Monostotic form: Ribs, femur, (diaphysis ) tibia, jaws, calvarium
Polyostotic form: Axial and paraxial skeleton with extensive cranio-facial involvement
 Well-circumscribed, Well-defined margins, Intramedullary. Tan-white and gritty

 X- Ray Findings: “Ground-glass” lesion within the medullary cavity

Polyostotic form can progress to osteoSa or MFH (malignant fibrous histiocytoma) .
Fibrous dysplasia composed of curvilinear
trabeculae of woven bone that lack
conspicuous osteoblastic rimming and arise in
a background of fibroblastic proliferation .
Polyostotic fibrous dysplasia.
Notice multifocal areas of high uptake on bone
scintigraphy: acetabulum, femur diaphysis, tibia
and ankle.
The radiographs show ground glass
abnormalities with or without calcifications.
 Metastatic tumors
 MC malignant tumor of bone. 
 Pathways of spread include:
 (1) direct extension
 (2) lymphatic or hematogenous dissemination
 (3) intraspinal seeding.
 MC - axial skeleton, Alkaline Phosphatase ↑ in an older adult
 Purely lytic, purely blastic (sclerotic), or both (mixed)
 In adults more than 75% of skeletal metastases originate from cancers of
the prostate (blastic), breast, kidney, and lung (mixed), thyroid (lytic)
 In children, neuroblastoma, Wilms' tumor, osteosarcoma, Ewing sarcoma,
and rhabdomyosarcoma are the common sources of bony metastases
 CLASSIFICATION OF MESENCHYMAL TUMOURS
Origin of the tumours Benign tumours Malignant tumours
Bone tissue Osteoma Osteosarcoma
Chondroma Chondrosarcoma

Connective (fibrous) tissue Fibroma, desmoid Fibromyosarcoma

Fat tissue Lipoma Liposarcoma
Muscle tissue: Myoma Sarcoma
smooth muscle Leiomyoma Leiomyosarcoma
striated muscle Rhabdomyoma Rhabdomysarcoma
Blood vesels Hemangioma Angiosarcoma
Lymphatic vessels Lymphangioma Lymphangisarcoma
Synovial tissue Benign synovioma Synovial (malignant
synovioma) sarcoma
Mesothelial tissue Benign mesothelioma Malignant mesothelioma
 Soft tissue tumors
Connective (fibrous) tissue tumors
Main benign connective (fibrous) tissue tumors are:
1. Fibroma - a node of differentiated connective tissue with different direction of the
bands:
 a) dense-fibrous structures prevail over the cellular elements;
b) soft (loose connective tissue with great amount of stroma cells — fibroblasts and
fibrocytes).
Localization: skin, breast, on the skin it may have a limb, cranial base, spinal canal,
orbit.
2. Desmoid fibroma is a kind of dense fibroma and characterized by infiltrating
growth and relapses.
3. Dermatofibroma (histiocytoma) - small fibrous node with yellow-brown color. More
often it is located in the skin of the legs (crus). It consists of capillaries and
connective tissue with fibrous structures and fibroblasts, fibrocytes, histiocytes,
macrophages. There are giant polynuclear cells with lipids (Touton giant cell) and
hemosiderin between cells.
 Desmoid (Deep-Seated Fibromatosis)
 Extra-abdominal desmoid (tendon-like, Greek)
 Men and women
 Shoulder, chest wall, back and thigh
 Intercostal and femoral muscles
 Abdominal desmoid
 Pregnant women
 Muscles and aponeuroses of the anterior
abdominal wall
 Intra-abdominal desmoid
 Patients with Gardner syndrome
 Mesentery and pelvic wall
 Desmoid: Morphology
 Grossly
 1 – 15 cm in d
 Poorly demarcated
 Grey-white and firm

 Histology
 Broad fascicles of plump
fibroblasts, which infiltrate
surrounding tissue
 Scanty mitotic figures
 Entrapped muscles produce
appearance of giant cells
 Fibrous Tumors and Tumor-Like Lesions
Reactive Pseudosarcomatous Proliferations
 Nodular fasciitis, (infiltrative or reactive pseudosarcomatous fasciitis) a solid
reactive fibroblastic proliferation seen in the upper extremities and trunk of
young adults, sometimes occurring after trauma. Preceding trauma is reported in
only 10% to 15% of cases. hairdressers
 Volar aspect of the forearm, followed in order of frequency by the chest and back.
 Morphology.  In the deep dermis, subcutis, or muscle.
 Grossly: A solitary, rapidly growing, and sometimes painful mass. Several
centimeters in greatest dimension, is nodular in configuration, and has poorly defined
margins.
 Plump, immature-appearing fibroblasts and myofibroblasts arranged randomly or in
short intersecting fascicles .
 The cells vary in size and shape (spindle to stellate) and have conspicuous nucleoli;
mitotic figures are abundant . Stroma is myxoid and contains lymphocytes and
extravasated red blood cells. Nodular fasciitis rarely recurs after excision
Nodular fasciitis with plump, randomly oriented spindle cells surrounded by
myxoid stroma. Note the mitotic activity and extravasated red blood cells
 Myositis Ossificans Peripherally mineralized myositis
ossificans (arrows) involving the
heterotopic ossification, is an extra-osseous non- posterior thigh.
neoplastic growth of metaplastic bone 
In athletic adolescents and young adults
follows an episode of trauma in more than 50% of
cases.
in the musculature of the proximal extremities.
Clinically: Early phase the involved area is swollen
and painful
during the subsequent several weeks it becomes
more circumscribed and firm.
Eventually, it evolves into a painless, hard, well-
demarcated mass.

Morphology: is 3 to 6 cm, well-demarcated. Plump, elongated fibroblast and myofibroblast-like

cells simulating nodular fasciitis. Cells are surrounded by an intermediate zone that contains
osteoblasts, which deposit ill-defined trabeculae of woven bone. The peripheral zone: well-
formed, mineralized trabeculae that closely resemble cancellous bone. Intertrabecular spaces
become filled with bone marrow. The mature lesion is completely ossified.
 FIBROMATOSES
 Superficial Fibromatosis (Palmar, Plantar, and Penile Fibromatoses)
 nodular or poorly defined broad fascicles of fibroblasts and myofibroblasts
surrounded by abundant dense collagen.
 In the palmar variant (Dupuytren contracture): irregular or nodular thickening of the
palmar fascia either unilaterally or bilaterally (50%). 75% are men. A firm, hard,
cordlike area. Skin puckering and dimpling. A slowly progressive flexion contracture
develops 4th and 5th fingers of the hand.
 Similar changes: plantar fibromatosis except that flexion contractures are
uncommon and bilateral involvement is infrequent.
 In penile fibromatosis (Peyronie disease) a palpable induration or on the
dorsolateral aspect of the penis. Curvature of the shaft, constriction of the urethra,
or both.
 All forms of superficial fibromatosis affect males more frequently than females.
 In about 20% to 25% of cases, the palmar and plantar fibromatoses stabilize and do
not progress, in some instances resolving spontaneously. Some recur after
excision, particularly the plantar variant.
 FIBROMATOSES
Superficial Fibromatosis (Palmar, Plantar, and Penile Fibromatoses)
Deep-seated (Desmoid)
Ledderhose's
disease 

Irregular or nodular thickening of the palmar fascia Peyronie disease : a palpable induration or mass
either unilaterally or bilaterally (50%). 4th and 5th appears, on the dorsolateral aspect of the penis
fingers. Palmar fibromaroses. Dense collagen
 Malignant connective (fibrous) tissue tumors
 anywhere in the body, but are MC in the deep soft tissues of the
extremities. Atypical cells, including loss of the structure
 Macroscopically: fish flesh. As a rule sarcoma metastases are
disseminated hematogenically.
 Fibrosarcoma looks like node or indistinct formation.
 a) Differentiated fibrosarcoma, termed cellulofibrous, when fibrous
component prevails cellular component;
 b) Poorly differentiated fibrosarcoma, termed cellular sarcoma. It produces
metastases more frequently:
 c) Round-cell tumours of unknown origin, termed unclassified tumor.
 Protruding dermatofibroma (malignant histiocytoma) - numerous
polymorphic fibroblast cells with metastases. It grows slowly, its growth is
infiltrating, the relapses of metastases are very rare. Malignant fibrous
histiocytoma (MFH): Undifferentiated Pleomorphic Sarcoma
Fibrosarcoma

Fibrosarcoma composed of malignant spindle cells arranged

in a herringbone pattern.
 Tumors of fat tissue

(A)White fat. Note single lipid vesicles and peripheral nuclei.

(B)Brown fat. Note the multivacuolated nature of the cells and the centrally placed nuclei. BV
indicates a small blood vessel. Between the shoulder blades, surrounding the kidneys, the
neck and supraclavicular area, and along the spinal cord.
 Tumors of fat tissue Benign
 Lipoma: a node, sometimes in a capsule, yellow, made of lobules of
different size. It may develop in every site where there is fat tissue. This
mass is benign and will not recur
 Intramuscular infiltrating lipoma is a tumor without distinct borders, it
infiltrates to intermuscular connective tissue.

Hibernoma: a rare tumour of brown fat.

MC: remains of embryonic brown fat
(surrounding adrenal glands, along of
spinal column).

Large polygonal/oval cells: Nucleus -

central location, small. Cytoplasm –
multivacuolated (fat), oval, eosinophilic,
granular.
 Lipoma - well circumscribed
mass of yellowish fat

Here is the lipoma at high magnification.

This is a good example of how a benign
neoplasm mimics the tissue of origin
 Comparison of lipoma and atypical lipomatous tumor
(ALT)/well-differentiated liposarcoma
Atypical Lipomatous Tumor/Well-
Lipoma
Differentiated Liposarcoma
Age 40–60 years of age Middle-aged to elderly
Superficial (subcutaneous), Deep (intramuscular or retroperitoneal
Site
occasionally intramuscular including groin)
Mature adipose
Mature adipose tissue
Fibrous septae
Morphology No atypical hyperchromatic
stromal cells Atypical hyperchromatic stromal cells
(can be focal)
Supernumerary ring and giant marker
Translocation involving
Cytogenetics chromosomes with amplified
12q13–15
sequences of 12q14-15 region
 Tumors of fat tissue Malignant
 Liposarcoma a rare, large tumor, which is built of
lipocytes of different degree of maturity and
lipoblasts.
 There are several types of liposarcoma:
 a) mainly highly differentiated;
 b) mainly myxoid (embryonic);
 c) mainly round-cell;
 d) mainly polymorphocellular.
 It grows slowly, the metastases develop late.
 Malignant hybernoma is a very rare tumor with
cellular polymorphism and a lot of giant cells.
 Liposarcoma
Atypical lipomatous tumor/well-
differentiated liposarcoma. Lipoblasts.
Atypical hyperchromatic stromal cells
with smudged nuclei are prominent in
this specimen that also contains
numerous fibrous bands.

t(12;16)(q13;p11) with amplification of

MDM2 gene.
Myxoid liposarcoma with abundant ground substance in which are scattered
adult-appearing fat cells and more primitive cells, some containing small lipid
vacuoles (lipoblasts)
 Tumors of muscles (smooth benign)
 Leiomyoma - smooth muscle with chaotic location of the muscular
tissue bands, the stroma with vessels and nerves. Mutation in the
fumarate hydratase gene located on chromosome 1q42.3.
 If stroma prevails this tumor is termed fibromyoma. For diff.
diagnose between they we use a special stain – picrofuchsin (van
Gieson method).
 The MC benign stromal tumor in the uterus is a leiomyoma.
 Macroscopically: as rule in the form of the node, different sizes,
solid consistence, whitish color with distinct borders.
 Secondary changes: necrosis, hemorrhages, cysts, hyalinosis,
petrifaction are characterized for leiomyoma.
 Leiomyomas of
varying size, but all
benign and well-
circumscribed firm
white masses.

According to the uterine wall there are 3 types of fibromyoma:

Submucosal (frequently bleeding).
Intramuscular.
Subserous (may be torsion of the node with necrosis and peritonitis).
Tumors of muscles (smooth)
Leiomyoma Leiomyosarcoma
 Tumors of muscles (striated benign)
 Rhabdomyoma consists of striated muscles.
 It resembles embryonic muscular fibres and myoblasts.
 It appears against a background of tissue shifts and is
accompanied by other development defects (large masses of
striated muscles).
 Cardiac rhabdomyoma is associated with tuberous sclerosis
(TS) mutations of the TS1 (9q34) or TS2  (16p13) genes.
TS: multiple hamartomatous lesions in the skin, CNS, and
visceral organs, kidney angiolipoma. 
 Granular-cell tumor (Abrikosov's tumor): this is small tumor in a
capsule.
 It is located in the tongue, esophagus, skin. The cells are
round, large with granular cytoplasm (no lipids).
 Tumors of muscles (malignant)
 Leiomyosarcoma with cellular and tissue atypism, a large
number of mitoses (high mitotic index) are characteristic.
 Rhabdomyosarcoma: extreme polymorphism, loss of tissue
characteristic (it is necessary to use special immune
antibodies – myogenin, desmin to verify the tumor). MC in
children.
 Malignant granular-cell tumor (malignant myoblastoma)
resembles malignant rhabdomyoma but the cytoplasm is
granular.
 Classification of rhabdomyosarcoma
 Rhabdomyosarcoma
 Embryonal rhabdomyosarcoma
 Botryoid rhabdomyosarcoma (vagina, < 5 ys)
 Spindle cell/Sclerosing rhabdomyosarcoma
 Anaplastic rhabdomyosarcoma
 Alveolar rhabdomyosarcoma
 Pleomorphic rhabdomyosarcoma
Characteristics of embryonal and alveolar rhabdomyosarcoma
Embryonal RMS Alveolar RMS
Age Young children (0–15 years) Adolescents
Site Head/neck, GU tract Extremities
Morphology Small round blue cell Small round blue cells
Desmin, myogenin and myoD1 Desmin, myogenin and myoD1
IHC
positive, Vimentin positive, Vimentin
t(1;13) and t(2;13), PAX3 to
Cytogenetics No recurrent abnormalities
the FOXO1a
Prognosis Better prognosis Worse prognosis
 Rhabdomyosarcoma
Alveolar rhabdomyosarcoma (left): Nests of loosely cohesive small round blue cells
appear to cluster to septae at the edge of nests giving an "alveolar" appearance.
Pleomorphic
rhabdomyosarcoma
(right): Malignant cells
are large, polygonal in
shape and tend to have
marked eosinophilic
cytoplasm. Atypical
nuclei are prominent,
vesicular, and may have
prominent nucleoli.

Spindle-shaped but markedly pleomorphic cells that

have pink cytoplasm with a hint of striations
mimicking skeletal muscle cells
Rhabdomyosarcoma, microscopic
Note the appearance of a characteristic “strap cell,” which has recognizable
cross-striations (arrow) mimicking a skeletal muscle fiber.
 Synovioma
 A malignant mesenchymal neoplasm characterized by the chromosomal
translocation t(X;18)(p11;q11).
 Any age, but mainly affects young adults, more commonly males.
 Although any site can be affected (tendon), the vast majority of the cases arise in
the deep soft tissues of extremities and around a joint, especially around the knee.
 Microscopically, classified as monophasic (with a spindle or epithelial cell
component) or biphasic (with both spindle and epithelial cell components).
Synovial sarcomas can recur or metastasize to the lungs, bones, and lymph
nodes.

Synovial sarcoma revealing the classic

biphasic spindle cell and glandular-like
histologic appearance.
 GANGLION AND SYNOVIAL CYST
 A small (1–1.5 cm) cyst that is almost always located
near a joint capsule or tendon sheath.
 Is around the joints of the wrist, a firm, fluctuant, pea-
sized translucent nodule with mucoid fluid.
 as a result of cystic or myxoid degeneration of
connective tissue; the cyst wall lacks a true cell lining.
May be multilocular. Wall: fibrous tissue.
 The fluid that fills the cyst is similar to synovial fluid;
however, there is no communication with the joint
space.
 Synovial cyst - herniation of synovium through a joint
capsule or massive enlargement of a bursa.
 A well-recognized example is in the popliteal space in
the setting of RA (Baker cyst).
 The synovial lining may be hyperplastic and contain
inflammatory cells and fibrin.
 TENOSYNOVIAL GIANT-CELL
TUMOR
Villonodularhyperplasia with synovial cells mixed with
MNGCs, foam cells, siderophages and inflammatory cells
80 % in the hand joints, 50% - recurrence
Grossly, are red-brown to mottled orange-yellow.

In diffuse tumors: red-brown folds, finger-like projections, and nodules. Well circumscribed
and resemble a small walnut. In the diffuse variant : spread along the surface and infiltrate
the subsynovial issue.
In nodular : the cells grow in a solid aggregate, attached to the synovium by a pedicle.
Only 2% to 16% of the cells in the mass, are neoplastic
polyhedral, moderately sized, and resemble synoviocytes.
Both variants are heavily infiltrated by macrophages,
which may contain hemosiderin and lipid-filled vacuoles,
or coalesce into multinucleated giant cells (MNGS).
Pain with “popping and catching” knee and hands
 M-CSF is useful in treating (promoter of the collagen
COL6A3 gene).
 Reference Text

Kumar, V., Abbas, A.K., Aster, J.C. Robbins

Basic Pathology 9th Ed. Saunders 2013.
ISBN: 978-1-4377-1781-5.