Fibrogenic and Histogenic tumours Flashcards
Non ossifying fibroma Benign fibrous histocytoma desmoplastic fibroma Malignant fibrous histocytoma Fibrosarcoma
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
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
What are the symtpoms of non ossifying fibroma?
- Usually Asymptomatic
- Pathological Fx
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
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
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
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
What is the histology of histocytoma/ benign fibrous histiocytoma
- Spindle cells
- foamy macrophages
- storiform pattern- helciopter in wheat field
What is the tx of histocytoma/ benign fibrous histiocytoma?
Operative
- Currettage and bone grafting
- reoccurance is rare
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%
What are the symptoms of desmoplastic fibroma?
- A painful lesion
What are the xrays signs of desmoplastic fibroma?
- Purely lytic lesion
- may involve cortex
- may have soft tissue mass
What is the tx of desmoplastic fibroma?
Operative
- Wide surgical resection vs intralesional currettage
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
What are the symptoms of malignant fibrous histiocytoma?
- Painless mass
- path fx
- swelling
- limp
- fever
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
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
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
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
What are the symtpoms of Fibrous sarcoma?
- Pain and swelling
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
What is seen on histology of fibrosarcoma?
- Atypical spindle cells
- Herringbone pattern
- similar to soft tissue sarcoma
What is the tx for fibrosarcoma?
Operative
-
Wide surgical resection & multiagent chemotherapy
- all malignant cases
- chemo if high grade
