Bone Tumor Characteristics Quiz
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Questions and Answers

Which of the following is a characteristic symptom of osteoid osteoma?

  • Painless growth on the skull.
  • Asymptomatic cartilaginous growth in the hands.
  • Pain that is worse at night and relieved by aspirin. (correct)
  • Multiple symmetric osteochondromas.
  • Osteomas commonly involve the diaphysis of long bones.

    False (B)

    What is the typical location of an enchondroma?

    medullary cavity of bone

    An osteochondroma originates from the ______ growth plate.

    <p>epiphyseal</p> Signup and view all the answers

    Match each bone tumor with its primary location:

    <p>Osteoma = Skull and facial bones Osteoid osteoma = Diaphysis of long bones Osteochondroma = Ends of long bones Enchondroma = Medullary cavity of bone</p> Signup and view all the answers

    Which condition is characterized by multiple enchondromas and may present with pain?

    <p>Ollier disease (B)</p> Signup and view all the answers

    Osteoblastomas are typically smaller than 2 cm in size.

    <p>False (B)</p> Signup and view all the answers

    Which of the following benign bone tumors has the highest potential for malignant transformation?

    <p>Osteochondroma (B)</p> Signup and view all the answers

    Maffucci syndrome is characterized by multiple what?

    <p>Enchondromas and soft tissue hemangiomas (A)</p> Signup and view all the answers

    Osteosarcomas are more prevalent in females than males.

    <p>False (B)</p> Signup and view all the answers

    What is the most common primary malignant tumor of bone?

    <p>Osteosarcoma</p> Signup and view all the answers

    Osteosarcomas are characterized by the formation of bone matrix or ______ by malignant tumor cells.

    <p>osteoid</p> Signup and view all the answers

    Match the following genetic abnormalities with their associated risks in osteosarcoma:

    <p>RB gene mutation = Increased risk of osteosarcoma Li-Fraumeni syndrome = Greater incidence of osteosarcoma INK4a abnormalities = Associated with osteosarcoma</p> Signup and view all the answers

    Which of the bone disorders is associated with secondary osteosarcoma?

    <p>Paget disease of bone (D)</p> Signup and view all the answers

    Primary osteosarcomas develop in association with pre-existing bone disorders.

    <p>False (B)</p> Signup and view all the answers

    Where do osteosarcomas typically arise in long bones?

    <p>Metaphyseal region (A)</p> Signup and view all the answers

    A patient presents with a bulky mass, pain, and X-rays showing an expanding lytic lesion with a thin rim of bone around the knee area. Gross examination reveals a red-brown mass with cystic degeneration involving the epiphyses. Which of the following is the most likely diagnosis?

    <p>Giant Cell Tumor (B)</p> Signup and view all the answers

    Ewing sarcoma is more common in females than males.

    <p>False (B)</p> Signup and view all the answers

    What is the characteristic translocation associated with Ewing sarcoma, and what fusion protein does it produce?

    <p>t(11;22), EWS-FLI1</p> Signup and view all the answers

    Ewing sarcoma often affects the ______ of long bones, while giant cell tumors in adults involve both the epiphyses and the metaphyses.

    <p>diaphysis</p> Signup and view all the answers

    Match the histological features with the corresponding bone tumor:

    <p>Giant Cell Tumor = Multiple osteoclast-like giant cells within a background of mononuclear stromal cells. Ewing Sarcoma = Sheets of undifferentiated small round blue cells resembling lymphocytes, with characteristic Homer-Wright pseudorosettes.</p> Signup and view all the answers

    What is the typical treatment approach for aneurysmal bone cysts?

    <p>Chemotherapy, surgery, and/or radiation (A)</p> Signup and view all the answers

    Aneurysmal bone cysts predominantly affect individuals in their sixth and seventh decades of life.

    <p>False (B)</p> Signup and view all the answers

    What are the primary routes through which metastatic tumors spread to bone?

    <p>Direct extension; lymphatic or hematogenous dissemination; intraspinal seeding (via the Batson plexus of veins)</p> Signup and view all the answers

    In adults, more than 75% of bone metastases originate from cancers of the prostate, breast, __________, and __________.

    <p>kidney; lung</p> Signup and view all the answers

    Match the following childhood cancers with their likelihood to metastasize to bone:

    <p>Neuroblastoma = Common Wilms tumor = Common Rhabdomyosarcoma = Common</p> Signup and view all the answers

    Which chromosomal abnormality is most frequently observed in cases of benign schwannoma?

    <p>Monosomy 22 (A)</p> Signup and view all the answers

    Which histological subtype is NOT a recognized variant of rhabdomyosarcoma?

    <p>Fibrous (D)</p> Signup and view all the answers

    Rhabdomyomas are a common type of malignant soft-tissue tumor found in adults.

    <p>False (B)</p> Signup and view all the answers

    Leiomyomas are more common in men than in women.

    <p>False (B)</p> Signup and view all the answers

    What is the most common type of soft tissue tumor in adulthood?

    <p>lipoma</p> Signup and view all the answers

    Uterine leiomyoma is associated with the translocation ____.

    <p>t(12;14)(q15;q24)</p> Signup and view all the answers

    What cellular feature is characteristic of rhabdomyoblasts, regardless of the type of rhabdomyosarcoma?

    <p>eccentric eosinophilic granular cytoplasm rich in thick and thin filaments</p> Signup and view all the answers

    Malignant spindle cells with cigar-shaped nuclei arranged in interweaving fascicles are characteristic of ________.

    <p>leiomyosarcomas</p> Signup and view all the answers

    Which of the following factors is NOT typically associated with the development of soft tissue tumors?

    <p>Balanced Diet (D)</p> Signup and view all the answers

    Match the following tumor types with their primary tissue of origin:

    <p>Leiomyoma = Smooth Muscle Hemangioma = Vascular Tissue Lymphangioma = Lymphatic System Rhabdomyosarcoma = Skeletal Muscle</p> Signup and view all the answers

    Match each tumor type with its primary tissue of origin:

    <p>Liposarcoma = Adipose Tissue Fibrosarcoma = Fibrous Tissue Rhabdomyosarcoma = Skeletal Muscle Leiomyosarcoma = Smooth Muscle</p> Signup and view all the answers

    What is the typical size range for leiomyomas?

    <p>1 to 2 cm (C)</p> Signup and view all the answers

    Which subtype of lipoma is known for being painful?

    <p>Angiolipoma (B)</p> Signup and view all the answers

    Rhabdomyosarcomas are most commonly found in the extremities, specifically in relation to skeletal muscle.

    <p>False (B)</p> Signup and view all the answers

    Leiomyosarcomas are more prevalent in men than in women.

    <p>False (B)</p> Signup and view all the answers

    In what ratio do Hemangiomas have a predilection for females?

    <p>3 : 1 (A)</p> Signup and view all the answers

    Flashcards

    Giant Cell Tumor

    A benign tumor often in the knee area, featuring giant cells and causing pain and swelling.

    Soap Bubble Appearance

    A radiological sign seen in giant cell tumors, indicating lytic lesions with surrounding bone changes.

    Ewing Sarcoma

    A malignant tumor in young individuals, often affecting long bones and causing painful swelling.

    Onion Skinning

    Characteristic x-ray finding in Ewing Sarcoma showing concentric layers of periosteal bone formation.

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    Homer-Wright Pseudorosettes

    Microscopic features of Ewing Sarcoma, indicating small round blue cells resembling lymphocytes.

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    Mafucci Syndrome

    A condition characterized by multiple enchondromas and soft tissue hemangiomas, with a risk of malignancy.

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    Osteosarcoma

    A highly malignant bone tumor that forms bone matrix by malignant cells; most common in ages 10-25.

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    Bimodal age distribution

    Osteosarcoma commonly occurs in two age groups: 10-25 years and after 40 years.

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    Primary Osteosarcoma

    Type of osteosarcoma where underlying bone is unremarkable and often has genetic abnormalities.

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    Secondary Osteosarcoma

    Osteosarcoma developing in older patients due to pre-existing bone disorders or treatments.

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    Paget disease

    A disorder causing abnormal bone remodeling often linked to secondary osteosarcoma.

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    Genetic mutations associated with Osteosarcoma

    Mutations in tumor suppressor genes like RB and p53 are linked to increased osteosarcoma risk.

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    Metaphysis

    The region of long bones where osteosarcoma typically arises, especially near the knee.

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    Aneurysmal Bone Cyst

    A benign bone tumor with multiloculated blood-filled cysts, typically found in the first two decades of life.

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    Common Locations for ABC

    Aneurysmal Bone Cysts are commonly found in the metaphyses of long bones and vertebral bodies.

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    Symptoms of ABC

    The most common symptoms of an Aneurysmal Bone Cyst are pain and swelling in the affected area.

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    Metastatic Bone Tumors

    Tumors that spread to bone, often from cancers of prostate, breast, kidney, and lung in adults.

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    Soft Tissue Tumors

    A diverse group of tumors arising from mesenchymal cells, classified by their origin like fibrosarcoma from fibroblasts.

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    Osteoma

    A benign skull and facial bone tumor with slow growth.

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    Osteoid Osteoma

    A benign, painful bone growth, often in long bones.

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    Osteoblastoma

    Larger benign tumor (>2 cm), often affects vertebrae.

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    Osteochondroma

    Benign growth capped with cartilage from the growth plate.

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    Osteochondromatosis

    Condition with multiple bening osteochondromas, often symmetrical.

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    Enchondroma

    Benign cartilaginous tumor in the medullary cavity, often asymptomatic.

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    Ollier Disease

    Non-hereditary syndrome with multiple enchondromas in hands/feet.

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    Chondrosarcoma

    Malignant cartilage-forming tumor, can arise from benign tumors.

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    Rhabdomyosarcoma Variants

    Subclassified into embryonal, alveolar, and pleomorphic types.

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    Rhabdomyoblast

    Diagnostic cell of rhabdomyosarcoma with unique cytoplasm structure.

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    Tadpole Cells

    Elongate rhabdomyoblasts that may show cross-striations.

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    Leiomyoma

    Benign smooth muscle tumor, often in the uterus; most common in women.

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    Symptoms of Leiomyomas

    Can cause infertility and other symptoms based on their size and location.

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    Leiomyosarcoma

    Malignant smooth muscle tumor, more common in women, accounts for 10-20% of soft-tissue sarcomas.

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    Hemangioma

    Common vascular tumors in infants, resulting from normal vascular tissue overgrowth.

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    Lymphangioma

    Malformation of the lymphatic system's vessels; affects fluid return in tissues.

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    Benign Schwannoma

    A benign tumor arising from Schwann cells in peripheral nerves.

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    Lipoblastoma

    A benign tumor of immature fat cells, typically occurring in children.

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    Liposarcoma

    A malignant tumor of adipose tissue, aggressive and can metastasize.

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    Rhabdomyosarcoma

    The most common soft-tissue sarcoma in children, arising from skeletal muscle.

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    Neuroma

    A tumor that develops from nerve tissue, can be benign or malignant.

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    Dermatofibrosarcoma Protuberans

    A rare skin cancer that arises from the fibrous tissue of the dermis.

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    Fibrosarcoma

    A malignant tumor of fibrous connective tissue, often aggressive.

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    Study Notes

    Bone, Cartilage, Soft Tissue, Vascular Tumors

    • Benign and malignant lesions are discussed
    • Classification of bone and cartilage tumors is presented, differentiating between benign and malignant types.
    • Osteoma is a benign tumor affecting skull and facial bones, growing extremely slowly. It can extend into the orbit or sinuses (Gardner's syndrome) and is most common in the 40-50 year age group.
    • Osteoid osteoma is a benign, painful growth usually in the diaphysis of long bones (often tibia or femur) with peak incidence in 5-25 year olds, mostly males. Its symptoms include worse pain at night, relieved by aspirin. On x-rays, a central radiolucency is seen surrounded by a sclerotic rim.
    • Osteoblastoma is similar to osteoid osteoma but larger ( > 2cm) and frequently involves vertebrae.
    • Osteochondroma (exostosis) is a benign bone growth originating from the epiphyseal growth plate. It commonly affects adolescent males as a firm, solitary growth at the ends of long bones. It can be asymptomatic but may cause pain and deformity. It is rarely malignant.
    • Osteochondromatosis (Multiple hereditary exostosis) is characterized by multiple, often symmetrical, osteochondromas.
    • Enchondroma is a benign cartilaginous growth within the medullary cavity of bone, most commonly found in the hands and feet. It is usually a solitary, asymptomatic lesion.
    • Ollier disease is a non-hereditary syndrome characterized by multiple enchondromas, with symptoms including pain, and potential for malignant transformation to chondrosarcoma.
    • Maffucci syndrome is a syndrome with multiple enchondromas and soft tissue hemangiomas. This carries an increased risk of malignant transformation (ovarian cancer and brain gliomas).
    • Osteosarcoma (osteogenic sarcoma) is a highly malignant bone tumor characterized by the formation of bone matrix. Males are more commonly affected than females (2:1 ratio) and the most common age group is (10-25 years old). Osteosarcoma also has a high incidence in elderly after 40 years. Patients with familial retinoblastoma have a higher risk for developing osteosarcoma. Symptoms include localized pain and swelling.
    • Pathogenesis of primary osteosarcomas involves acquired genetic abnormalities like ploidy changes and chromosomal aberrations. Key mutations to look for are RB (retinoblastoma gene), INK4a (p16 and p14 genes), and p53.
    • Secondary osteosarcomas develop in association with pre-existing bone disorders, including Paget's disease, radiation exposure, chemotherapy (especially alkylating agents), and pre-existing benign bone lesions (fibrous dysplasia and osteomyelitis).
    • Morphology of osteosarcoma typically shows an intramedullary tumor originating from the metaphysis, often close to the knee joint (50%). Tumours can be multicentric, synchronous (at once) or metachronous (at different times). Histologically, they can show different degrees of differentiation and diverse histological features (osteoblastic, chondroblastic, fibroblastic, telangiectatic, small cell, and giant cell).
    • Chondrosarcoma is a malignant tumor of cartilage. Males are more commonly affected in the age group of 30-60 years. It can arise from pre-existing enchondromas, exostoses, or Paget's disease. Symptoms include a progressively enlarging mass with pain and swelling, typically affecting the pelvic bones, spine, and shoulder girdle. Microscopically, it's composed of atypical chondrocytes and chondroblasts, often displaying multiple nuclei in a lacunar structure. The most common histological type is conventional (hyaline and/or myxoid).
    • Giant cell tumor (osteoclastoma) is an uncommon malignant tumor. Females are more commonly affected than males (with ages usually between 20-50 years). It typically involves the epiphyses of long bones, often around the knee (distal femur and proximal tibia). Microscopically, the tumor is characterized by multinucleated giant cells interspersed with stromal cells. Symptoms usually involve a pain in a bulky mass.
    • Ewing's sarcoma is a malignant neoplasm of undifferentiated cells within the marrow cavity, most common in teenagers (5-20 years old). It commonly affects long bones (femur, tibia, humerus, fibula) and also pelvic bones, ribs, and vertebrae. It typically presents with pain, swelling, and tenderness. A classic X-ray finding is concentric onion skin layering of new periosteal bone.
    • Aneurysmal bone cyst is a benign tumor characterized by multiloculated blood-filled cystic spaces, typically arising during the first two decades of life. It commonly affects the metaphyses of long bones and posterior elements of vertebral bodies, and presents with pain and swelling.

    Metastatic Tumors to Bone

    • Metastases to bone, especially in adults, frequently originate from cancers of the prostate, breast, kidney, and lung. In children, metastases to bone commonly arise from neuroblastoma, Wilms tumor, and rhabdomyosarcoma.

    Soft Tissue Tumors

    • Soft tissue tumors are a heterogeneous group of neoplasms, often characterized by histogenetic features. Data suggests sarcomas arise from primitive, multipotential mesenchymal cells. Factors involved in etiology include cytogenetic abnormalities (e.g. Benign schwannoma, Monosomy 22; Lipoblastoma; Rearrangement of 8q; certain subtypes of uterine leiomyoma; etc), environmental carcinogens, infection, trauma, and chronic lymphoedema.

    Tumors of Adipose Tissue

    • Lipomas (benign) are the most common adult soft tissue tumor. They are subclassified based on morphology (conventional, fibrolipoma, angiolipoma, spindle cell lipoma, myelolipoma, pleomorphic).

    Tumors of Skeletal Muscle

    • Rhabdomyosarcomas are malignant and the most common soft-tissue sarcoma in children and adolescents.
    • Its morphology is variably classified into embryonal, alveolar, and pleomorphic variants. Key cells in the diagnosis of these types are rhabdomyoblasts with distinctive morphology.

    Tumors of Smooth Muscle

    • Leiomyomas are benign tumors of smooth muscle, often found in the uterus.
    • Leiomyosarcomas are malignant smooth muscle tumors, arising frequently in soft tissues of the extremities and the retroperitoneum.

    Vascular Tumors

    • Hemangiomas are the most common benign soft tissue tumor in children.
    • Lymphangiomas are malformations of the lymphatic system. Two types include capillary and cavernous, which can invade surrounding tissues.
    • Glomus tumors are rare benign tumors arising from glomus bodies.
    • Vascular malformations arise at birth; these vascular anomalies do not regress like hemangiomas; they grow at a rate commensurate with the rest of the patient's body. They may be capillary, venous, arterial, or combinations.
    • Bacillary angiomatosis is a form of angiomatosis associated with bacteria of the Bartonella genus.
    • Kaposi sarcoma is a tumor caused by human herpesvirus 8. Microscopically it often displays small branching blood vessels, spindle cells, and intracellular hyaline globs extravasated RBCS.
    • Angiosarcoma is a malignant tumor of endothelial cells lining vessel walls.

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