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AP RCPA VIVA > Bone and Soft Tissue > Flashcards

Bone and Soft Tissue Flashcards

1
Q

Giant Cell Rich Lesions of Bone

A

GCT of Bone (G34W mutation)

Primary ABC (USP rearranged)

Non-Ossifying Fibroma

Central and peripheral giant cell granuloma of jaw

Brown tumour of hyperparathyroidism

Chondroblastoma (K36M mutation)

Giant cell rich osteosarcoma

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2
Q

Risk Stratification in Solitary Fibrous Tumour

A

Age of patient (< or > 55 yo)

Tumour size (5, 10, 15cm cut offs)

Mitotic count (per 10 HPF)

Presence of necrosis (< or >10%)

Score for each parameter which are then added to give a total score (low, intermediate or high risk of metastasis)

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3
Q

Staging and Grading of Soft Tissue Sarcomas

A

Unlike other organ systems staging of soft tissue sarcomas is largely determined by grade.

No agree upon grading system but AJCC 8th ed uses FNCLCC system (other main system is NCI).

Not all soft tissue sarcomas are staged or graded:

e. g. DFSP and WDLPS are of intermediate malignancy and low grade by definition.
e. g. Ewing sarcoma, Angiosarcoma, Malignant rhabdoid tumour, DSRCT are all high grade by definition
e. g. ESMC, ASPS, ES, LGFMS and clear cell sarcoma not graded as grade does not predict outcome

FNCLCC system is based on:

Tumour differentiation score (depends on how closesly rumour resembles normal tissue of origin, table in CAP)

Mitotic activity (10 HPF or per 1 mm2)

Necrosis (< or > 50% tumour cell necrosis)

Each parameter is assigned a score then the scores are summed to give an overall grade (Grade 1 = low grade, Grade 2 & 3 = high grade)

AJCC Staging System is based on:

Site (H&N, trunk, extremites, retroperitoneum)

Size of tumour

Extent of involvement

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4
Q

Synoptic Reporting of Soft Tissue Sarcomas

A

Clinical:

Pre resection therapy and what type

Specimen:

Procedure, tumour focality, tumour site, tumour size

Histologic type (WHO)

Histologic Grade (FNCLCC)

Mitotic Rate

Necrosis

Treatment Effect

Lymphovascular Invasion

Margins

Regional lymph nodes (if included)

Distant Metastases (if applicable)

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5
Q

EWSR1 Rearranged Tumours

A

Ewing Sarcoma (FLI1, ERG)

Extraskeletal myxoid chondrosarcoma (NR4A3)

Clear cell sarcoma (ATF1, CREB1)

Clear cell carcinoma of salivary gland (ATF1)

Angiomatoid fibrous histiocytoma (ATF1, CREB1)

Desmoplastic small round cell tumour (WT1)

Soft tissue myoepithelial tumour (POU5F1, PBX1)

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6
Q

FUS Rearranged Tumours

A

Angiomatoid fibrous histiocytoma (ATF1)

LGFMS (CREBL2/3)

Sclerosing epithelioid fibrosarcoma (CREB3L2)

Myxoid liposarcoma (DDIT3)

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7
Q

Gene Rearrangements in Fatty Tumours

A

ALT / WDLPS / DDLPS (MDM2 amplification)

Myxoid liposarcoma (DDIT3 - EWSR1 or FUS)

Spindle cell lipoma (RB deletion)

Lipoblastoma (PLAG1)

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8
Q

Sarcomas that Metastasis to Lymph Nodes

A

SCARE

Synovial sarcoma
Clear cell sarcoma
Angiosarcoma
Rhabdomyosarcoma
Epithelioid sarcoma

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9
Q

MDM2 Amplified Tumours

A

ALT / WDLPS / DDLPS
Low grade central osteosarcoma
Parosteal osteosarcoma
Intimal sarcoma

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10
Q

USP6 Rearranged Tumours

A

Primary ABC
Giant cell lesion of the small bones
Nodular fasciitis
Myositis ossificans
Fibroosseous pseudotumour of digits
Cellular fibroma of tendon sheath

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11
Q

Molecular Changes in Bone Tumour

A

Osteochondroma: EXT1, EXT2 loss of function mutations
Enchondroma: IDH1, IDH2 mutations
Osteoid osteoma & OB: FOS, FOSB rearrangements

NOF: KRAS, FGFR1, NF1 alterations
Desmoplastic fibroma: CTNNB1, APC mutations

GCT of bone: H3 G34M mutation, RANKL overexp.
Chondroblastoma: H3 K36M mutation
ABC: USP6 rearrangement

LCH: BRAF V600E
Fibrous dysplasia: GNAS activating mutations

LG central & Parosteal OS: MDM2 amp, CDK4 (chr 12)

LG central CS: IDH1 / 2
LG peripheral CS: EXT1 / EXT2

Ewing Sarcoma: EWSR1 rearrangements
PMT: FGF gene rearrangements
Mesenchymal CS: HEY1-NCOA2 fusion

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12
Q

Syndromes Associated with Chondroid Tumours

A

Olliers (multiple enchondromas)

Mafucci (enchondromas & haemangiomas)

Multiple hereditary exostoses

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13
Q

Risk Factors for Osteosarcoma in Adults

A

Paget disease of the bones

Radiotherapy to the bones

Herediatry conditions: Hereditary retinoblastoma, Li-Fraumeni syndrome

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14
Q

Epiphyseal Bone Tumours

A

Chondroblastoma

Giant cell tumour of bone

Clear cell chondrosarcoma

Can also consider: ABC, UBC, osteomyelitis

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15
Q

Osteolytic Lesions in <30 y/o

A

Infection / osteomyelitis

Chondroblastoma

Chondromyxoid fibroma

Enchondroma

ABC

Fibrous dysplasia

Non-ossifying fibroma

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16
Q

Osteolytic Lesion in >30 y/o

A

Infection / osteomyelitis

Metastasis

Myeloma / lymphoma

Chondrosarcoma

GCT of bone

AP RCPA VIVA (14 decks)

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