Bone Tumors

Dr. I Made Naris Pujawan, M.Biomed, Sp.PA

• Bone tumors are diverse in their gross and morphologic features, and
vary in their natural history from innocuous to the rapidly fatal
• Most bone tumors are classified according to the normal cell or tissue
type they recapitulate
• Osteosarcoma is the most common primary cancer of bone, followed
by chondrosarcoma and Ewing sarcoma

• BONE-FORMING TUMORS
• CARTILAGE-FORMING TUMORS
• FIBROUS AND FIBRO-OSSEOUS TUMORS
Osteosarcoma
• Osteosarcoma is a malignant mesenchymal tumor in which the
cancerous cells produce bone matrix
• Osteosarcoma occurs in all age groups but has a bimodal age
distribution; 75% occur in persons younger than 20 years of age, The
smaller second peak occurs in the elderly, who frequently suffer from
conditions known to predispose to osteosarcoma—Paget disease,
bone infarcts, and prior irradiation
• Men are more commonly affected than women (1.6 : 1)
• The tumors usually arise in the metaphyseal region of the long bones
of the extremities, and almost 50% occur about the knee
Osteosarcoma
• Grossly, osteosarcomas are big bulky tumors that are gritty, gray-white,
and often contain areas of hemorrhage and cystic degeneration
• The tumor cells vary in size and shape and frequently have large
hyperchromatic nuclei.
• Bizarre tumor giant cells are common, as are mitoses, The formation of
bone by the tumor cells is characteristic
• When malignant cartilage is abundant, the tumor is called
chondroblastic osteosarcoma
• Vascular invasion is usually conspicuous, and up to 50% to 60% of an
individual tumor may be necrotic
Chondrosarcoma
• Chondrosarcoma is subclassified according to site as central (intramedullary) and
peripheral (juxtacortical and surface)
• Histologically, they include conventional (hyaline and/or myxoid), clear cell,
dedifferentiated, and mesenchymal variants
• Conventional central tumors constitute about 90% of chondrosarcomas
• Chondrosarcoma of the skeleton is about half as frequent as osteosarcoma and
is the second most common malignant matrix-producing tumor of bone
• Individuals with chondrosarcoma are usually in their 40s or older
• The tumor affects men twice as frequently as women
• About 15% of conventional chondrosarcomas (usually peripheral tumors) arise
from a preexisting enchondroma or osteochondroma
Chondrosarcoma
• Conventional chondrosarcoma is composed of malignant hyaline and
myxoid cartilage
• The adjacent cortex is thickened or eroded, and the tumor grows with
broad pushing fronts into the surrounding soft tissue
• The malignant cartilage infiltrates the marrow space and surrounds
pre-existing bony trabeculae
• The tumors vary in degree of cellularity, cytologic atypia, and mitotic
activity
SOFT-TISSUE TUMORS
AND
TUMOR-LIKE LESIONS
• Traditionally, soft-tissue tumors are defined as mesenchymal proliferations that
occur in the extraskeletal, nonepithelial tissues of the body, excluding the
viscera, coverings of the brain, and lymphoreticular system
• They are classified according to the tissue they recapitulate (muscle, fat,
fibrous tissue, vessels, and nerves)
• Some soft-tissue tumors have no normal tissue counterpart but have constant
clinicopathologic features warranting their designation as distinct entities
• The true frequency of soft-tissue tumors is difficult to estimate, because most
benign lesions are not removed
• A conservative estimate is that benign tumors outnumber their malignant
counterparts (sarcomas) by a ratio of at least 100 : 1
• In contrast to carcinomas, sarcomas usually metastasize via hematogenous
routes, making the lung and skeleton common sites of dissemination
Fatty Tumors
• Benign tumors of fat, known as lipomas, are the most common soft-
tissue tumor of adulthood
• They are subclassified according to particular morphologic features as
conventional lipoma, fibrolipoma, angiolipoma, spindle cell lipoma,
myelolipoma, and pleomorphic lipoma
• The conventional lipoma, the most common subtype, is a well-
encapsulated mass of mature adipocytes that varies considerably in
size
• Infrequently, lipomas are large, intramuscular, and poorly
circumscribed
• Cured by simple excision
LIPOSARCOMA
• Liposarcomas are one of the most common sarcomas of adulthood and appear
in the 40s to 60s
• They usually arise in the deep soft tissues of the proximal extremities and
retroperitoneum, and are notorious for developing into large tumors
• Liposarcomas are histologically divided into well-differentiated, myxoid/round
cell, and pleomorphic variants
• The cells in well-differentiated liposarcomas are readily recognized as lipocytes
• lipoblasts; they mimic fetal fat cells and contain round clear cytoplasmic
vacuoles of lipid that scallop the nucleus
• The myxoid/round cell variant of liposarcoma has a t(12;16)(q13;p11)
chromosomal abnormality in most cases
Fibrous Tumors and tumors like lesion
• Nodular Fasciitis
• It most often occurs in adults on the volar aspect of the forearm, followed in
order of frequency by the chest and back
• Affected individuals typically present with a several-week history of a
solitary, rapidly growing, and sometimes painful mass
• Preceding trauma is reported in only 10% to 15% of cases
• Nodular fasciitis arises in the deep dermis, subcutis, or muscle
• The lesion is richly cellular and contains plump, immature-appearing
fibroblasts and myofibroblasts arranged randomly or in short intersecting
fascicles
FIBROSARCOMA
• Fibrosarcomas occur anywhere in the body, but are most common in the
deep soft tissues of the extremities
• Typically these neoplasms are unencapsulated, infiltrative, soft, fish-flesh
masses often having areas of hemorrhage and necrosis
• Better differentiated lesions may appear deceptivelyencapsulated
• Histologic examination discloses all degrees of differentiation, from slowly
growing tumors that closely resemble cellular fibromatosis and sometimes
having spindled cells growing in a herringbone fashion to highly cellular
neoplasms dominated by architectural disarray, pleomorphism, frequent
mitoses, and areas of necrosis
• Fibrosarcomas are aggressive tumors, recurring in more than 50% of cases
and metastasizing in more than 25%
Fibrohistiocytic Tumors
• Fibrohistiocytic tumors contain cellular elements that resemble both
fibroblasts and histiocytes (macrophages)
• The phenotype of the neoplastic cells most closely resembles
fibroblasts, and the term fibrohistiocytic should be viewed as
descriptive in nature and not one that connotes the cell of origin
• BENIGN FIBROUS HISTIOCYTOMA (DERMATOFIBROMA)
• MALIGNANT FIBROUS HISTIOCYTOMA
Tumors of Skeletal Muscle
• Rhabdomyosarcoma, the most common soft-tissue sarcoma of childhood and
adolescence, usually appears before age 20
• It may arise in any anatomic location, but most occur in the head and neck or
genitourinary tract, where there is little if any skeletal muscle as a normal constituent
• Rhabdomyosarcoma is histologically subclassified into embryonal, alveolar, and
pleomorphic variants
• The rhabdomyoblast—the diagnostic cell in all types—contains eccentric eosinophilic
granular cytoplasm rich in thick and thin filaments
• Rhabdomyoblasts may be round or elongate; the latter are known as tadpole or strap
cells, and may contain cross-striations visible by light microscopy
• Ultrastructurally, rhabdomyoblasts contain sarcomeres, and immunohistochemically
they stain with antibodies to the myogenic markers desmin, MYOD1, and myogenin.
Tumors of Smooth Muscle
• Leiomyomas, the benign smooth muscle tumors, often arise in the uterus
• Leiomyomas may also arise from the arrector pili muscles found in the skin,
nipples, scrotum, and labia and less frequently develop in the deep soft
tissues and the wall of the gut
• Leiomyomas are usually not larger than 1 to 2 cm in greatest dimension and
are composed of fascicles of spindle cells that tend to intersect each other at
right angles
• The tumor cells have blunt-ended, elongated nuclei and show minimal atypia
and few mitotic figures
• Solitary lesions are easily cured; however, multiple tumors may be so
numerous that complete surgical removal is impractical
LEIOMYOSARCOMA
• Leiomyosarcomas account for 10% to 20% of soft-tissue sarcomas
• They occur in adults and afflict women more frequently than men
• Most develop in the skin and deep soft tissues of the extremities and retroperitoneum
• Leiomyosarcomas present as painless firm masses. Retroperitoneal tumors may be
large and bulky and cause abdominal symptoms
• Histologically they consist of malignant spindle cells with cigar-shaped nuclei arranged
in interweaving fascicles
• Immunohistochemically, they stain with antibodies to smooth muscle actin and desmin
• Treatment depends on the size, location, and grade. Superficial or cutaneous
leiomyosarcomas are usually small and have a good prognosis, whereas those of the
retroperitoneum are large, cannot be entirely excised, and cause death by both local
extension and metastatic spread
Synovial Sarcoma
• Synovial sarcoma is so named because it was once believed to recapitulate synovium, but the cell of
origin is still unclear
• In addition, although the term synovial sarcoma implies an origin from the joint linings, less than
10% are intra-articular
• Most occur in the 20s to 40s
• The majority develop in the deep soft tissue and about 60% to 70% involve the lower extremity,
especially around the knee and thigh
• Patients usually present with a deep-seated mass that has been noted for several years
• Uncommonly, these tumors occur in the head and neck or in viscera
• Synovial sarcomas are morphologically biphasic or monophasic.
• The histologic hallmark of biphasic synovial sarcoma is dual lines of differentiation (i.e., epithelial-like and
mesenchymal-like).
• The epithelial cells are cuboidal to columnar and form glands or grow in solid cords or aggregates
• The spindle cells are arranged in densely cellular fascicles that surround the epithelial cell
• Synovial sarcomas are treated aggressively with limb-sparing therapy and frequently chemotherapy
 MATUR
SUKSMA