Fibrogenic tumours Flashcards Preview

Pathology FRCS > Fibrogenic tumours > Flashcards

Flashcards in Fibrogenic tumours Deck (17):
1

What is a calicifying aponeurotic fibroma?

  • A benign Fibrogenic mass that usually presents as a painless mass in hands/ feet
  • In children young adults 3-30 yrs

A image thumb
2

How does a calicifying aponeurotic fibroma present?

  • A painless growing mass in hands/feet

3

What is the imaging of calicifying aponeurotic fibroma?

  • A faint mass with stippling

A image thumb
4

What is calicifying aponeurotic fibroma histology?

  • fibrous tissue w central region of calcification and cartilage formation

5

What is the tx of calicifying aponeurotic fibroma?

Nonoperative

  • Lesion often resolves with maturity

Operative

  • Local excision
  • recurrence up to 50%

6

What is planatr Fibromatosis?

  • Aka Lederhosen disease
  • Benign tumour of the foot plantar fascia that consists mainly of  myofibroblasts and collagen
  • similar process to Dupuytren's in palmar fibromatosis

A image thumb
7

what is extra- abdominal desmoid tumour?

  • A Fibrogenic lesio that is mostly locally invasive of all benign soft tissue tumours
  • 15-40 yrs
  • F>M
  • location
    • shoulder, chest wall/back, thigh
    • >50% extra abdominal
    • may have multiple lesions
    • new lesion usually appears more proximal
  • Genetics
    • Patients with familial adenomatosis polyposis > risk of devloping  extra- abdominal desmoid tumour
    • Trisomy in chromosome 8 or 20

8

Name associated conditions of  extra- abdominal desmoid tumour?

  • Dupuytren's disease
  • Ledderhose disease

9

What is the prognosis of  extra- abdominal desmoid tumour?

  • High risk of recurrence
  • highly unpredictable lesions
  • occasional spontaneous regression
  • NO risk of malignant change or mets unless hx of radiation

10

What are the symptoms of  extra- abdominal desmoid tumour?

  • Usually an enlarging Painless mass
  • 'rock hard' on palpation

A image thumb
11

What is seen on imaging of  extra- abdominal desmoid tumour?

  • Xrays- not useful
  • MRI
    • best to dx
    • low signal on T1
    • Low to medium on T2
    • Gadolinium enhances appearance
    • may erode bone locally

A image thumb
12

what is seen on histology of  extra- abdominal desmoid tumour

  • marcoscopically
    • gritty, white, poor capsulated 
  • Histology
    • well differentiated fibroblasts
    • uniform spindle cells w elongated nuclei
    • abundant colagen
    • Immunohistochemistry
      • 100% positive for Estrogen receptor Beta

A image thumb
13

What is the tx of  extra- abdominal desmoid tumour?

Non operatively

  • Low dose Chemotherapy only- Tamoxifen
    • for inoperable lesions
    • tamoxifen works via oestrogen receptor blockage

Operative

  • wide surgical resection w radiotherapy
    • for symptomatic lesions/ recurrent lesions
    • local reoccurance is common- reduced with radtx
    • external beam radiotherapy up to 60Gy

14

What is nodular fascitis?

  • A common reactive lesion that usually occurs in upper extremities 
  • you people 15-35
  • half cases happen in Upper limbs
  • painless/painful enlarging mass
  • MRI- Inhomogenous mass, xrays normal
  • Tx is operative- marginal excision

A image thumb
15

What is undifferentiated pleomorphic sarcoma?

  • A malignant fibrogenic tumour 
  • 30-80 years
  • similar to fibrosarcoma in presentation/tx
  • PC- enlarging, painless mass
  • symptoms start when mass >10 cm
  • MRI- deep seated inhomogenous mass
  • cartwheel pattern of spindle & histiocytic cells
  • tx OPerative
    • Wide local resection & adjuvant Radiotherapy
    • add radiation if tumour >5cm
    • 5000cGy neoadjuvant , 2000cGy adjuvant

16

What is fibrosarcoma of soft tissue?

  • A malignant fibrogenic tumour 
  • pts 30-80 years
  • same as Malignant Fibrous Histiocytoma of soft tissue
  • PC usually painless enlarging mass (>10cm= symp)
  • MRI- deep seated inhomogenous mass
  • histology
    • spindle shaped cells
    • tissue organised in herringbone fashion

A image thumb
17

What is the tx of fibrosarcoma of soft tissue?


Operatively
 


  • wide local resection & periop Radiotherapy

    • most cases
       


    • Add radiation if >5cm

       
    • ( 5000 cGy neoadjuvant then 2000cGy post)
    • local adjuvant tx with phenol/cryotherapy