Dr.

/Ahmed EL-Adl 6B Lecture4, Pathology

Bone tumors
A) Primary bone tumors:
1- Bone-forming tumors (osteogenic)
2- Cartilage-forming tumors (chondrogenic)
3- Fibrous and Fibro-osseous tumors
4- Miscellaneous bone tumors
B) Secondary bone tumors (metastatic tumors).

Fibrous and Fibro-osseous tumors

1- Fibrous cortical defect and non-ossifying fibroma:
FCD are very common, occurring in 30% to 50% of all children older than 2 years.
Most are small (<0.5 cm) and undergoes spontaneous differentiation into normal cortical bone
within a few years; Only larger lesions (5 cm) can develops into NOF which usually presents as
pathological fracture.
X-ray of both FCD and NOF:
Both appear as sharply demarcated radiolucency surrounded by a thin zone of sclerosis.
Most arise in the metaphysis of the distal femur or proximal tibia 50% of cases are bilateral or
multiple.
ME:
Cellular lesions formed of intersecting fascicles of bland spindle shaped cells with areas of
storiform pattern + scattered histiocytes including multinucleated giant cells.
Fibrous dysplasia
Benign tumor which is a localized developmental arrest, all components of normal bone are
present but they fail to differentiate towards normal structure.
It has three patterns:
1- Monostotic (involving single bone – 70% of cases):
a. Appears at early adolescence and stop growth at epiphyseal closure
b. It occurs in ribs, femur, tibia, jaw-bones – causing swelling and disfigurement.
2- Polyostotic (involving multiple bones):
a. appears at earlier age of onset
b. It occurs more in hip and shoulder girdles – causing deformity and fractures.

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Dr./Ahmed EL-Adl 6B Lecture4, Pathology

3- McCune-Albright syndrome (Polyostotic form associated with skin 'pigmentation ‘café-au-

lait pigmentation’ + endocrinal abnormality especially precocious puberty).

ME: Curved trabeculae of woven bone (mimicking Chinese characters), without osteoblastic
rimming; and surrounded by a moderately cellular fibroblastic proliferation.

Miscellaneous bone tumors

1- Primitive neuroectodermal neuroendocrinal tumor (PNET)/Ewing’s sarcoma.
2- Giant cell tumor of the bone.
3- Plasmacytoma (multiple myeloma).

1- Primitive neuroectodermal neuroendocrinal tumor(PNET)/Ewing’s sarcoma:

• Aggressive bone tumor.
• The second most common pediatric bone sarcoma (after osteosarcoma) (6-10%).
• Most patients between 10-15 years and 80% of patients are less than 20 years
• It harbors specific translocation causing the fusion of EWS gene with a member of the ETS
family of transcription factors FL1 [t(11,22)} or ERG [t(21,22)].
• It usually arises in the medullary cavity of long tubular bones (especially the femur) and the
pelvic flat bones; and invades the cortex and periosteum to produce a soft tissue mass
X-ray:
Destructive lytic tumor with infiltrative margins and extending into the surrounding soft tissue.
It shows a characteristic periosteal reaction known as onion skin appearance.
ME:
• Malignant round cell tumor (sheets of uniform small round cells with hyperchromatic nuclei
and scanty glycogen-rich cytoplasm within little intervening stroma.
• The presence of Homer-Wright rossettes (tumor cells circled around a central fibrillary
space) indicates neural differentiation (ie: PNET > Ewing).

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Dr./Ahmed EL-Adl 6B Lecture4, Pathology

2- Giant cell tumor of the bone

• These tumors are characterized by large number of multinucleated osteoclast-type giant cells
(osteoclastoma)
• Low grade locally aggressive tumor arising most between 20-40 years
• Most in the epiphysis of long bones (around the knee); and rarely can metastasize to lungs.
• X-ray: Large expansible lytic lesion without peripheral bone sclerosis or periosteal reaction.

3- Plasmacytoma (multiple myeloma):

• Proliferation of neoplastic plasma cells in the bone marrow which leads to lytic lesion/lesions
in the bone
• One lytic lesion = Plasmacytoma (solitary myeloma)
• Multiple lytic lesions = Multiple myeloma
Metastatic tumors (secondaries)
• More common than primary.
• Reach bone by blood.
• Primary sites are breast, prostate, lung, thyroid and kidney.
• Most bone metastases are osteolytic (produce bone destruction) except cancer prostate
that may be osteosclerotic.