Osteosarcoma and Other Primary Tumors of Bone Flashcards by Mario Luna

Osteosarcoma and Other Primary Tumors of Bone

What are the most common types of primary bone tumors?

Osteosarcoma, chondrosarcoma, and Ewing sarcoma are the most common, comprising 35%, 30%, and 16% of cases, respectively.

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Osteosarcoma and Other Primary Tumors of Bone

What is the most common malignant bone tumor in children?

Osteosarcoma

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Osteosarcoma and Other Primary Tumors of Bone

Osteosarcoma peaks in early adolescence and adults older than 65 years of age.

TRUE or FALSE?

True

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Osteosarcoma and Other Primary Tumors of Bone

Where is the most common site of involvement in osteosarcoma?

areas of greatest increase in bone length

metaphysis of the distal femur, proximal tibia, and proximal humerus

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Osteosarcoma and Other Primary Tumors of Bone

What is the characteristic translocation or genetic abnormality defined for osteosarcoma?

NONE

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Osteosarcoma and Other Primary Tumors of Bone

What benign disease has been most commonly associated with the development of osteosarcoma especially in older adults?

Paget disease

Other benign bone lesions have also been associated with risk of osteosarcoma, including chronic osteomyelitis, multiple hereditary exostoses, fibrous dysplasia, osteochondromas, enchondromas, sites of bone infarcts, and sites of metallic implants.

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Osteosarcoma and Other Primary Tumors of Bone

5 genetic conditions associated with the development of osteosarcoma

RB
Li-Fraumeni syndrome
Bloom syndrome
Werner syndrome
Rothmund-Thomson syndrome

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Osteosarcoma and Other Primary Tumors of Bone

Most common presenting symptom.
Osteosarcoma

localized pain

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Osteosarcoma and Other Primary Tumors of Bone

Most common site of metastatic involvement.
Osteosarcoma

Lung

followed by bone

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Osteosarcoma and Other Primary Tumors of Bone

This anatomic variation is seen on plain radiograph of ostesarcoma patients when the periosteum is raised away from the bone by a tumor (osteoid formation)

Codman triangle

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Osteosarcoma and Other Primary Tumors of Bone

Osteosarcoma

Enneking Staging System

Presence of metastases

III

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Osteosarcoma and Other Primary Tumors of Bone

Osteosarcoma

Enneking Staging System

Non metastatic, extracompartmental,
low grade

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Osteosarcoma and Other Primary Tumors of Bone

Osteosarcoma

Enneking Staging System

Non metastatic, extracompartmental,
high grade

IIB

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Osteosarcoma and Other Primary Tumors of Bone

Osteosarcoma

Enneking Staging System

Non metastatic, intracompartmental,
high grade

IIA

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Osteosarcoma and Other Primary Tumors of Bone

Osteosarcoma

Enneking Staging System

Non metastatic, intracompartmental,
low grade

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Osteosarcoma and Other Primary Tumors of Bone

Osteosarcoma

AJCC Staging System

Non metastatic, low grade
8 cm or less

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Osteosarcoma and Other Primary Tumors of Bone

Osteosarcoma

AJCC Staging System

Non metastatic, low grade
>8 cm

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Osteosarcoma and Other Primary Tumors of Bone

Osteosarcoma

AJCC Staging System

Non metastatic, low grade
discontinuous

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Osteosarcoma and Other Primary Tumors of Bone

Osteosarcoma

AJCC Staging System

Non metastatic, high grade
8 cm or less

IIA

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Osteosarcoma and Other Primary Tumors of Bone

Osteosarcoma

AJCC Staging System

Non metastatic, high grade
>8 cm

IIB

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Osteosarcoma and Other Primary Tumors of Bone

Osteosarcoma

AJCC Staging System

Non metastatic, high grade
discontinuous

III

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Osteosarcoma and Other Primary Tumors of Bone

Osteosarcoma

AJCC Staging System

pulmonary metastases

IVA

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Osteosarcoma and Other Primary Tumors of Bone

Osteosarcoma

AJCC Staging System

bone metastases

IVB

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Osteosarcoma and Other Primary Tumors of Bone

Osteosarcoma

What are the two main categories of osteosarcoma?
Which is more common in younger patients?
Which is composed of more high-grade tumors?

Conventional and surface type.

Conventional is more common in adolescents, and is usually of high-grade type.

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Osteosarcoma and Other Primary Tumors of Bone Osteosarcoma What are variants of the conventional type osteosarcoma?

osteoblastic chondroblastic fibroblastic mixed others: (small cell telangiectatic malignant fibrous histiocytoma multifocal)

Osteosarcoma and Other Primary Tumors of Bone Osteosarcoma What are variants of the surface type osteosarcoma?

periosteal parosteal high-grade

Osteosarcoma and Other Primary Tumors of Bone Osteosarcoma Which of the variants of the surface type osteosarcoma is considered low-grade and has a low metastatic potential and can be treated with chemotherapy alone?

Parosteal

Osteosarcoma and Other Primary Tumors of Bone Osteosarcoma What rare type of osteosarcoma arises in soft tissues generally associated with prior radiation exposure?

extraosseous osteosarcoma

Osteosarcoma and Other Primary Tumors of Bone Osteosarcoma What is the typical treatment sequence?

neoadjuvant chemotherapy surgery (limb-sparing if possible) adjuvant chemotherapy

Osteosarcoma and Other Primary Tumors of Bone Osteosarcoma Limb-sparing surgery have equivalent results with amputation as long as margins are adequate. TRUE or FALSE?

True

Osteosarcoma and Other Primary Tumors of Bone Osteosarcoma What are the contraindications to limb-sparing procedure?

nerve or vascular encasement presence of large, biopsy-related hematoma and *pathologic fracture. ***Some data suggest that pathologic fracture does not increase the risk of local recurrence after limb-sparing surgery as previously believed.

Osteosarcoma and Other Primary Tumors of Bone Osteosarcoma Although not statistically significant, there was a favorable trend in DFS after adjuvant chemotherapy compared to observation in patients with high-grade osteosarcoma treated with primary surgery, hence further trials on neoadjuvant chemotherapy were greenlit. TRUE or FALSE?

False. Eilber et al. reported on 59 patients with nonmetastatic osteosarcoma randomized to surgery followed by observation versus adjuvant chemotherapy. Disease-free survival at 2 years was 55% with chemotherapy and 20% with observation (P < .01). Overall survival was also superior at 2 years: 80% versus 48% with and without chemotherapy, respectively (P < .01). Link et al. reported similar results in a group of 36 patients with nonmetastatic, high-grade osteosarcoma randomized to observation versus adjuvant chemotherapy after primary surgery. Disease-free survival at 2 years was 66% with chemotherapy and 17% with observation (P < .001).

Osteosarcoma and Other Primary Tumors of Bone Osteosarcoma What is the role of RT in osteosarcoma?

as adjuvant treatment for incompletely resected tumors / resected with + margins palliation of symptoms sometimes, as adjuvant to poor responders to neoadjuvant chemotherapy.

Osteosarcoma and Other Primary Tumors of Bone Osteosarcoma Margins used for RT?

Typically, a 2-cm margin is used for axial tumors, which can be extended to 4 to 5 cm for extremity tumors.

Osteosarcoma and Other Primary Tumors of Bone Osteosarcoma Typical RT dose prescription for microscopically involved margins?

60 / 2 / 30

Osteosarcoma and Other Primary Tumors of Bone Osteosarcoma Typical RT dose prescription for gross macroscopic residual tumor?

66 / 2 / 33

Osteosarcoma and Other Primary Tumors of Bone Osteosarcoma Typical RT dose prescription for inoperable tumors?

70 / 2 /35

Osteosarcoma and Other Primary Tumors of Bone Osteosarcoma Should whole-lung irradiation be given to patients? If yes, what are the subsets of patient who will benefit the most? If no, give the rationale.

No. Whole-lung irradiation has been shown to be beneficial in several other pediatric tumors with a propensity for lung metastases. This led to the rationale that this treatment may improve outcomes in osteosarcoma, which has a high propensity for metastases and for which the lung is the most common site of spread. Two initial small randomized trials in the prechemotherapy era showed a trend for improved disease-free and overall survival for whole-lung irradiation. This led to the EORTC-20781/SIOP-03 phase III trial, which randomized 240 patients to three arms: chemotherapy, whole-lung irradiation, or both. The whole-lung dose was 20 Gy. The 4-year disease-free survival and overall survival were 43% and 24%, respectively, with no difference between the arms. Therefore, with the recognition of the other advantages of systemic therapy, whole-lung irradiation has fallen out of favor.

Osteosarcoma and Other Primary Tumors of Bone Chondrosarcoma can develop from malignant transformation of benign cartilaginous lesions—osteochondromas and endochondromas. Osteochondroma is a cartilagecapped bony projection arising on the external surface of bones. It is usually located on long bones, particularly around the knee. Multiple osteochondromas are associated with what autosomal dominant syndrome?

hereditary multiple exostoses

Osteosarcoma and Other Primary Tumors of Bone Chondrosarcoma can develop from malignant transformation of benign cartilaginous lesions—osteochondromas and endochondromas. Enchondroma is a benign cartilaginous tumor developing in the marrow cavity of bone. Multiple enchondromas, or enchondromatosis, are usually associated with what congenital disorders?

Ollier disease | Maffucci syndrome

Osteosarcoma and Other Primary Tumors of Bone The rate of metastatic disease for chondrosarcoma is very dependent on _________.

tumor grade

Osteosarcoma and Other Primary Tumors of Bone Where is the main site of metastases of chondrosarcoma?

Lung

Osteosarcoma and Other Primary Tumors of Bone What are the 2 pathological categories of chondrosarcoma?

conventional and nonconventional

Osteosarcoma and Other Primary Tumors of Bone What are the 3 types of conventional chondrosarcoma?

central peripheral periosteal

Osteosarcoma and Other Primary Tumors of Bone Where are the most common locations of chondrosarcoma?

proximal femur, pelvis, and proximal humerus

Osteosarcoma and Other Primary Tumors of Bone What type of chondrosarcoma arises from a pre-existing osteochondroma?

peripheral

Osteosarcoma and Other Primary Tumors of Bone What type of chondrosarcoma arises from the surface of long bones and is rare?

periosteal

Osteosarcoma and Other Primary Tumors of Bone What type of chondrosarcoma tends to affect younger patients and have a good prognosis?

periosteal

Osteosarcoma and Other Primary Tumors of Bone Give some variants of nonconventional chondrosarcoma

clear cell dedifferentiated myxoid mesenchymal

Osteosarcoma and Other Primary Tumors of Bone What is the role of radiation therapy in chondrosarcoma?

adjuvant for incompletely resected high-grade or locally recurrent tumors or unresectable tumors

Osteosarcoma and Other Primary Tumors of Bone What is the role of chemotherapy in chondrosarcoma?

no defined role. especially for the conventional type.

Osteosarcoma and Other Primary Tumors of Bone What is the recommended total dose for preoperative RT in chondrosarcoma?

50 Gy

Osteosarcoma and Other Primary Tumors of Bone What is the recommended total adjuvant RT dose in the setting of positive margins in post-operative chondrosarcoma?

60 to 66 Gy

Osteosarcoma and Other Primary Tumors of Bone What is the recommended total definitive RT dose in the setting of unresectable chondrosarcoma?

70 Gy

Osteosarcoma and Other Primary Tumors of Bone Chordoma is a rare, malignant neoplasm arising from the ________?

remnant of the primitive notochord.

Osteosarcoma and Other Primary Tumors of Bone What areas do most of chordomas arise?

1/2 in sacrococcygeal areas | 1/3 from base of skull, typically the clivus.

Osteosarcoma and Other Primary Tumors of Bone Base-of-skull chordomas can present with cranial nerve deficits, particularly cranial nerve palsies. What nerves are usually involved?

3 & 6

Osteosarcoma and Other Primary Tumors of Bone Chordomas, in general, have a high metastatic potential. TRUE or FALSE?

False

Osteosarcoma and Other Primary Tumors of Bone What are the pathologic classifications of chordomas?

conventional (classic) chondroid dedifferentiated

Osteosarcoma and Other Primary Tumors of Bone Which subtype of chordomas have a better prognosis?

chondroid.

Osteosarcoma and Other Primary Tumors of Bone Which subtype is more aggressive and more likely to metastasize?

dedifferentiated.

Osteosarcoma and Other Primary Tumors of Bone What is the most common pattern of failure?

local recurrence

Osteosarcoma and Other Primary Tumors of Bone Minimum dose requirement in RT for chordomas?

66 Gy

Osteosarcoma and Other Primary Tumors of Bone It is a malignant neoplasm of mesenchymal origin characterized by predominance of fibroblasts without tumor osteoid or cartilage production. It has a predilection for long bones and has a high metastatic potential. It is treated with complete surgical resection and often with adjuvant or neoadjuvant chemotherapy. Radiation therapy can be used for incompletely resected or unresectable tumors.

Fibrosarcoma

Osteosarcoma and Other Primary Tumors of Bone It is characterized by a mixture of spindle-shaped fibroblastic cells in a storiform pattern and admixed with mononuclear cells with histiocytic morphology and anaplastic giant cells without tumor osteoid or cartilage production. The mainstay of treatment is complete surgical resection. Like osteosarcoma, it has a high rate of metastases.

Malignant fibrous histiocytoma

Osteosarcoma and Other Primary Tumors of Bone (from in-service bank) 13. For RT of Osteosarcoma, the ff are true except a. RT is the primary treatment of Osteosarcoma b. RT plays important role in the tumors of axial skeleton c. RT plays important role in the treatment of facial bones d. RT is the frequently use in the management of primitive neuroectodermal tumors of bones. e. none of of above.