Unknown origin tumours Flashcards by Sally-Anne Phillips

Describe metastatic cancer of bone?

The commonest reason for a destructive bone lesion in adults
cancers that spread to bone
- breast
- Lung
- thyroid
- renal
- prostate
Boneris 3rd commonest for metastatic disease ( after lung, liver)
usually >40 yrs
common sites
- axial skeleton- thoracic spine most common site
- proximal limb girdle
- prox femur most common site of fx 2ary to met bone lesion

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What is the pathophysiology of bony mets?

Mechanism of osteolysis
- osteolytic bone lesions - caused by Tumour induced activation of osteoclasts
  - occurs thru RANK, RANKL, osteoprotegrin
- Osteoblastic bone mets are due to tumour secreted endothelin 1

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What is the prognosis of pts with metastatic disease with thyroid, prostate, breast, kidney and lung?

Thyroid 48 months
Prostate 40 months
Breast 24 months
Kidney variable short as 6 months
Lung : 6 months

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Name associated conditions of metastatic disease?

Metastatic hypercalaemia
- medical emergency
  - confusion
  - muscle weakness
  - polyuria/polydipsia
  - nausea/vomiting
  - dehydration
- tx with hydration and loop diuretics

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describe the mechanism of metastasis?

Tumour cell intravasation
- E cadherin cell adhesion molecule on tumour cells modulates release from primary tumour focus into bloodstream
Avoidance of immune surveillance
target tissue localization
induction of angiogenesis - via vascular endothelial growth factor (VEGF) expression
genomic instability
decreased apoptosis
Vascular spread
- Batson’s vertebral plexus
- valveless venous plexus of the spine that provides a route of metastasis from organs to axial structure including vertebral bodies, pelvis, skull and prox limb girdles
- Arterial tree metastasis
  - mechanism which lung/renal cancer spreads to distal extremities

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What are the symptoms of metastatic disease?

Pain
- mechanical due to bone destruction or tumorigenic pain at night
- Pathological Fx 8-30%
- metastatic hypercalcaemia
O/E
- Neurological deficit
  - compression of spinal cord with mets disease to spine

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Describe the workup for a pt with suspected metastatic diseasee?

Plain radiographs
- lung, thyroid & renal = lytic
- 60% breast ca= blastic
- 90% prostatic ca= blastic
- lesions distal to elbow/knee often lung/renal primary- see pic
CT chest /adbo/Pelvis
Technetium bone scan- to detect extent of disease
- myeloma & thyroid ca are cold
- elevate with skeletal survey if cold
Labs
- U&E
- ESR
- LFT’s, CA, Phos, Alk Phosphatase
- serum & urine immunoelectrophoresis
Biopsy
- shouldn’t tx bone lesion without tissue dx

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What is the goal of tx in metastatic disease?

Control pain
Maintainence of patient independence

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What is the tx of metastatic disease?

Non operative

Bisphosphonates
- symptomatic to prevent osteclastic bone destruction
- iv pamidronate most commonly used

Operative

Stabilisation of complete fx, postop radiation
- most complete fx are treated if op stabilisatio leads to improved quality of life
- post op radiation
  - all pts unless death immunent or area has had previous radiation
  - begin radiation after surgery
  - area of irradiation should include entire fixation device
Prophylatic stabilisation of impeding fx ( entire bone - ie nail) allow immediate WB, post op rdx
preop embolization
- renal cell carcinoma/thyroid as v vascular

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What is the tx for metastatic spinal lesion?

Non operative

Palliative care
- if life expectancy< 6months

Operative

Neurological decompression, spinal stabilisation adn post op radiotherapy
- for met lesions to spine with neurological deficits in pts with life expectancy > 6 months
- preop embolisation included for renal ca to psine

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Are there any scoring systems to help decide prognosis of spinal mets and so tx?

Tokuhashi et al Spine 2009. published a scoring system to help develop a treatment algorithm for patients with metastatic spinal lesions.
They used a patient’s
- general condition
- number of extraspinal bone metastases
- the number of metastases in the vertebral body,
- the metastases to other major internal organs,
- the primary site of the tumor,
- degree of neurologic compromise to determine the life expectancy.
- They recommended against surgical intervention for patients with less than six months to live.

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What is a Giant cell tumour?

A Benign Agressive Tumour
typically found in Epiphysis of long bones but may airse in apophysis ( like chondroblastomas)
> females (cf most bone tumours)
Age 30-50 yrs
location
- 50% around knee
- **10% sacrum/vertebra body**
- distal radius
- phalanges also common
- may a

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Describe the malignancy of gct?

Primary malignant giant cell tumour
- mets to lung in 2-5%
- hand lesions have > chance of metastasis
Secondary malignant giant cell tumour
- occurs following radiation/multiple resections of GCT

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What are the symptoms of GCT?

Pain refered to involved joint

O/E

palpable mass
decreased rom of joint

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What is seen on imaging a GCT?

Xrays

An Eccentric Lytic Epiphyseal/Metaphsyeal lesion
often extends into the distal epiphysis and borders subchondral bone

Bone scan

Very hot

MRI

Demarcation on T1 image between fatty marrow and tumour- see pic

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Describe the histology of GCT?

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Neoplastic cell is the mononuclear stromal cell- see pic
Hallmark giant cells are numerous
secondary aneurysmal bone cysts not unusual

What is the tx of GCT?

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Non operative

Radiation alone
- only inoperable/multiple lesions
- 15%-> malignant transformation
Medical Mx
- Bisphosphonates
  - OC inhibitors- may decrease size of defect in GCT
- Denosumab
  - Monoclonal antibody against RANKL
  - trails= decrease size of defect in GCT

Operative

Extensive Currettage and Reconstruction ( w adj tx)
- lesions amenable to currettage
- hand lesions controversial
  - if no cortical defect- currettage + cement
  - if cortical defect- intercalary resection w fibular free graft vs amputation
  - need to preform extensive exterioration ( removal of large cortical window over lesion ) is required
- Outcome 10-30% recurrence vs 3% w adjuvant tx ( phenol, hydrogen peroxide)

What is Ewing’s sarcoma?

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A distinctive round cell bone sarcoma
5-25 yrs
2nd most common bone tumour in children
Uncommon in african americans/chinese
50% Diaphysis of long bones
locations
- Pelvis, distal femur, proximal tibia, femoral diaphysis, proximal humerus
Genetics
- t( 11:22) translocation - in all cases
- which leads to -> Fusion protein ESW-FLI1= identified w PCR

What is the prognosis of Ewing’s sarcoma?

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60-70% long term survival w isolated extremity disease
40% long term survival w pelvic lesions
15% long term survival if presents w mets

Poor prognostic factors

Spine & pelvic tumours
tumour >100cm3
<90% necrosis w chemo
elevated lactic dehydrogenase levels
p53 mutation in addition to T(11:22) translocation

What is the symptoms of Ewing’s sarcoma?

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Pain accompanied by fever
often mimics infection

O/E

Swelling and local tenderness

What are the signs of Ewing’s sarcoma on imaging?

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Xray

Large destructive lesion in Diaphysis/metaphysis with a Moth eaten appearance- see pic
Lesion maybe purely lytic or somereactive bone formation
Periosteal reaction= onion skin/sunburst appearance

Bone Scan

Hot lesion

MRI

often shows large soft tissue component

CT chest

Bony Mets

What is seen on lab results with Ewing’s sarcoma?

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Elevated WCC
Elevated ESR
Anaemia Common
Lactic Dehydrogenase
Bone marrow biopsy- to rule out mets in marrow

What is seen on histology with Ewing’s sarcoma?

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Gross- may have liquid consitency like pus
sheets of monotomous small round blue cells- see pic
prominent nuclei and minimal cytoplasm
may have pseudo-rosettes ( circle cells w necrosis in centre)
Immunostaining
- CD99 reactivity

Describe the tx for ewing’s sarcoma?

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Operative
- Chemotherapy and Limb Salvage Resection
  - standard most pts
  - preop chemo 8-12 wks then surgery then chemo 6-12 mo post
  - Curent trend away radiotx
  - only with resectable tumours/mets

What are the age differentials of small- round cell tumours?

* \<5 yrs= Neuroblastoma/ Leukaemia * 5-10 yrs= Esosinophilic Granuloma * 5-30 yrs= Ewing's sarcoma * \>30 yrs= lymphoma * \>50 yrs= myeloma

What is an Adamantioma?

* A **rare low grade Malignant tumour of unknown aetiology that is almost always located in the mid-tibia** * \<300 cases documented * young adults 20-40 yrs

Name associated conditions of adamantinoma?

* **Osteofibrous dysplasia** * _not a precusor_ of adamantinoma * a BENIGN, fibro-osseous lesion in the tibia cortex of young children * Tx by observation

What is the prognosis of adamantinoma?

* May metastatize to lungs **25%** * so _longterm follow up is recommended_ * reocurrance is uncommon with negative margin excision

What are the symptoms of adamantinoma?

* Pain of months- years duration O/E * **Bowing deformity** of tibia * **Palpable mass** of tibia

What is seen on imaging of adamantinoma?

* xray * **Multiple sharply circumscribed lucent lesions "soapy bubbles" with interspersed sclerotic bone in mid tibia** * some lesions mat destroy cortex * may see bowing of tibia * radiographic evolution of lesion helpful as lesions may continue to grow and erode cortex * No periosteal reaction

What is seen in histology of adamantiomas?

* Fibrous and epithelial tissue in gland like pattern * **nests of epithelial like cells** arranged in palisaing or **glandular pattern** * background of stroma

What is the tx of adamatiomas?

* Operative * **Wide margin surgical resection** * most pts * often requires intercallary resection w allograft /megaprosthesis reconstruction * **Chemo and Radiotx typically not used**

Unknown origin tumours Flashcards

Metastatic bone disease Giant cell tumour ewing's tumour Adamantioma (32 cards)