Fibrogenic and Histogenic tumours Flashcards Preview

Pathology FRCS > Fibrogenic and Histogenic tumours > Flashcards

Flashcards in Fibrogenic and Histogenic tumours Deck (23):
1

What is a non ossifying fibroma?

  • A benign fibrogenic lesion
  • **most common benign bone tumour in childhood**
  • common 5-15 years
  • location
    • metaphysis of long bones
    • 80% lower extremity
    • knee ( distal femur/prox tibia) distal tibia

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2

Name any associated conditions of  non ossifying fibroma?

  • Jaffe- Campanaccci syndrome
    • congential syndrome of multiple  non ossifying fibroma and
    • cafe au lait pigmentation
    • mental retardation
    • heart, gonads and eyes involved
  • Neurofibromatosis
  • familial multifocal NOF
  • ABC

3

What are the symtpoms of  non ossifying fibroma?

  • Usually Asymptomatic
  • Pathological Fx

4

What is seen on radiographs of  non ossifying fibroma?

  • Metaphsyeal BUBBLY Lytic lesion surrounded by sclerotic rim
  • cortex maybe expanded and thin
  • as bone grows migrates to diaphysis, enlarge 1-7cm

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5

What is seen on histology of  non ossifying fibroma?

  • Fibroblastic spindle cells in whirled or storiform pattern- helicopter in wheat field
  • numerous lipophages and giant cells
  • hemosiderin pigmentation

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6

Describe the tx of  non ossifying fibroma?

Non operative

  • Observation
    • first line of tx
    • most resolve spontaneously 
  • ​Casting
    • path fx

Operative

  • Curettage and bone graft
    • symptomatic and large lesion

7

Describe a histocytoma/ benign fibrous histiocytoma?

  • A benign histiocytic lesion of bone
  • Extremely rare
  • presents with pain and swelling
  • xrays - lytic lesion with sclerotic border

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8

What is the histology of histocytoma/ benign fibrous histiocytoma

  • Spindle cells
  • foamy macrophages
  • storiform pattern- helciopter in wheat field

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9

What is the tx of histocytoma/ benign fibrous histiocytoma?

Operative

  • Currettage and bone grafting
  • reoccurance is rare

10

What is a desmoplastic fibroma?

  • A rare low grade malignant fibrogenic tumour of bone
  • usually occur in metaphysis of long bones
  • reocurrance rate is 40%

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11

What are the symptoms of desmoplastic fibroma?

  • A painful lesion

12

What are the xrays signs of desmoplastic fibroma?

  • Purely lytic lesion
  • may involve cortex
  • may have soft tissue mass

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13

What is the tx of desmoplastic fibroma?

Operative

  • Wide surgical resection vs intralesional currettage

14

What is malignant fibrous histiocytoma?

  • A rare malignant histocytic lesion of bone
  • 25% arise secondary lesion from bone infarct, paget's disease
  • similar presentation to fibrosarcoma
  • **most common soft tissue sarcoma in adults 55-80**
  • male>female 2:1
  • often Metaphyseal of long bones
  • genetics unknown
  • 50-60% survival at 5 years

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15

What are the symptoms of malignant fibrous histiocytoma?

  • Painless mass
  • path fx
  • swelling
  • limp
  • fever

16

What is seen on xrays of malignant fibrous histiocytoma

  • Lytic and destructive lesion often in metaphysis
  • variable periosteal reaction
  • MRI- T1 low signal, T2 high signal
  • Bone scan - hot

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17

What is seen on histology of malignant fibrous histiocytoma?

  • Plemorphic spindle cells and histiocytic cells in storiform pattern
  • malignant multinucleated giant cells with grooved/indented nuclei

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18

What is the tx of malignant fibrous histiocytoma?

Operative

  • similar to osteosarcoma
    • Neoadjuvant chemotherapy, wide resection, post op chemo +/- radiation
    • neo given 8-12 wks followe by maintenance for 6-12 months after surgery
    • surgery - limb salvage
    • radiation if lesion >5cm

19

What is fibrosarcoma of bone?

  • A malignant fibrogenic tumour of bone
  • usually affects older pts >50 years
  • majority High grade
  • Prognosis is poor
    • high grade 30% survival at 5 years
    • Low grade 80% survival at 10 years

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20

What are the symtpoms of Fibrous sarcoma?

  • Pain and swelling

21

What are the radiographic characteristics of fibrosarcoma?

  • Purely lytic lesions w bone destruction in a permeative pattern
  • ill defined features
  • looks like osteosarcoma
  • Bone scan V hot

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22

What is seen on histology of fibrosarcoma?

  • Atypical spindle cells
  • Herringbone pattern
  • similar to soft tissue sarcoma

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23

What is the tx for fibrosarcoma?

Operative

  • Wide surgical resection & multiagent chemotherapy
    • all malignant cases
    • chemo if high grade