Soft tissue /connective tissue tumors lecture 20 printed Flashcards
List 5 types of soft tissues and connective tissue
Fibrous connective tissue Adipose tissue muscle (smooth and skeletal vessels nerves
What is the etiology of soft and connective tissue tumors?
- Vause is unknown and sporadic
- Some are associated with genetic syndromes eg neurofibromatosis type 1
- Some show specific genetic derangements/mutations and chromosomal abnormalities eg synovial sarcoma
- Some are associated with radiation therapy, chemicals, thermal burns and trauma, viruses
Discuss the presentation of soft tissue tumors
- sex
- age
- sizes
- Benign / malignant
- common sites
>Occurs more in Males >Incidence increase with age but 15% occur in children and 4th common malignancy in pediatrics >Mass can grow to an enormous size causing disfigurement and may be painful >BT more common than MT >Common sites *40% lower limb *30% trunk *20%upper limb *20% head and neck
List the 4 ways soft tissue tumors are diagnosed
> Fine needle aspiration
- Good for recurrence /metastases
- sitological assessment
- Not for primary diagnosis
Incisional biopsy
*Preferred when core biopsy is not representative
> Core biopsy
- First line for most tumors
- May not be fully represented in the case of sarcoma
> Excisional biopsy
*Only is small (<3-5 cm ) and superficial
T/F; Histological diagnosis provides valuable information for treatment and prognosis
t
What are the common histological features we look out for?
- Growth pattern and architecture (storiform, palisading, and herringbone )
- Mitotic activity
- Pleomorphism
- Necrosis
- Behaviour
What other diagnostic tools are used to diagnose tumors
- immunohistochemistry
- cytogenesis
- molecular analysis
- electron microscope
Prognosis depends on :
4 things
- Accurate histological classification of the tumor
- Histological grading
a. Tumor differentiation
b. Percentage of tumor necrosis
c. Mitoses - Stage
- location: superficial tumors have a better prognosis than deep tissue tumors because they are easy to discover and excisie
How can tumors be treated
- Surgery
* enucleation for BT only - Chemotherapy
- Radiation
Give an example of superficial fibromatosis
Fibromatosis of superficial tissue includes palmar, plantar, and penile (Peyronies disease )
Dupuytren’s contracture: overgrowth of fibrous tissue around tendon resulting in flexion deformity.
it is benign
Give 3 subtypes of deep-seated fibromatosis and common sites
- Extra-abdominal: shoulders, chest wall, back, and thighs
- Abdominal: Desmoid tumors (anterior abdominal wall )
- Intra-abdominal (mesenteric, pelvic wall )
Tend to recur but do not metastasize
Discuss the frequency, sites, and appearance of fibrosarcoma
- Rare
- Retroperitoneum ,thigh ,knee and distal extremities
- necrosis ,hemorrhage
Histologically: spindle cells growing in a herringbone pattern, mitoses, pleomorphic and necrosis
T/F: Fibrohistolytic tumors are deep-seated in tissue
False: Usually superficial and range from benign to locally aggressive malignant
Discuss the lipoma
- benign
- common in adults
- can grow to a large size
- soft mobile and painless except for for angiolipoma
- cured by local excision
Discuss the liposarcoma
- Malignant
- arise in deep soft tissues of the proximal extremities and retroperitoneum
- Arise de novo and not from malignant transformation of lipoma
> has 4 types
- well differentiated
- myxoid
- pleomorphic
- dedifferentiated
Discuss the rhabdomyosarcoma
- MT of the skeletal muscle
- Common soft tissue sarcoma in child(<20)
- common sites(head ,neck and genitourinary tract )
- The diagnostic cell is a rhabdomyoblast
What are the 3 types of rhabdomyosarcoma
- Embryonal
- alveolar
- pleomorphic
Discuss the embryonal rhabdomyosarcoma and list their subtype
The ERS is common in children <10 yrs and occurs in nasal cavity ,orbital ,prostate ,middle ear
> Subtype : Sarcoma botryoides
- Resembles a branch of grapes
- Grows in the wall of hallow mucous lined structures eg bladder ,vagina and nasopharynx
Discuss the alveolar rhabdomyosarcoma
- Commonly arise in the deep muscles of the extremities
- Accounts for 10% RMS
- network of fibrous septae divides cells into clusters similar to alveolae ; cells in the center are discohesive
PLeomorphic rhabdomyosarcoma
- Least common
- occurs more in adults
- arises in deep soft tissue
- Characterised by large ,multinucleated ,bazarre eosinophilic cells
Tumors of the smooth muscles
leiomyoma
leiomyosarcoma
Leiomyoma :
common site and effect
> Arises commonly in the uterus, it can be multiplied and grow to enormous sizes
it is associated with dysfunctional uterine bleeding and infertility
> c: Erector pili muscle in skin, labia, and scrotum
e: Less frequent in deep soft tissue and wall of the gut
Leiomyosarcoma
- not common in soft tissue sarcoma
- occur in adults
- F>M
- c: skin tumors ,deep tissue of the extremities ad retroperitoneum
- painless and firm
- with/ without abdominal symptoms
Vascular tumors
haemangioma
kaposi sarcoma
angiosarcoma
Discuss the haemangioma
- BT
- present from birth and exapand with growth of child
- 7% occur in infancy and childhood
- C: head and neck ,some internal and others liver
Main variants’ :
capillary hemangioma
Cavernous hemangioma
t/f: Haemangioma are greatly capsulated
false ,hey are poorly capsulated
Discuss the Kaposi sarcoma
rare type of tumor
- intermediate malignancy
- initially presents as a skin lesion
- may disseminate to involve viscera: lungs and git
*Caused by HHV8
List the 4 forms of Kaposi sarcoma
- Classic ( associated with immunosuppression but not HIV)
- African(endemic; associated with immunosuppression but not HIV)
- transplant-associated
- AIDS-associated
Histology
*haemosiderin deposition and plasma cells
Angiosarcoma
- C: May occur anywhere especially liver and breast
- Highly malignant tumors ,locally aggressive and readily metastasize
*histologically (anastomosing channels in other words blood lakes and proliferation of endothelial cells
Peripheral nerve sheath tumors
> Neurofibroma
Schwannoma
Granular cell tumor
Malignant peripheral nerve sheath tumor
Neurofibroma
> It is associated with neurofibromatosis type 1
*it is neurofibroma , patches of skin pigmentation and lish nodules
Histologically :
*mast cells and shredded carrot collage and un-encapsulated proliferation of spindle cells
Discuss the schwannoma
- solitary tumors (arise to one side of nerve root )
- Can rise anywhere
Histologically
*palisading
t/f: Schwannoma are problematic when in relation to the sheath of the auditory nerve intracranially for they can compromise hearing
true
Malignant peripheral nerve sheath tumors
- affects adults and children
- 50% associated with neurofibroma
- Bulky deep-seated tumors arise from major nerves
summary
Soft tissue tumours are diverse
Commonly present as masses of the extremities, retroperitoneum, trunk or head and neck region
Clinically, difficult to determine whether benign or malignant
Definitive diagnosis requires histology core or incisional biopsy
Treatment depends on the nature of disease (whether benign or malignant), grade and stage
Prognosis depends on multiple factors, but is generally poor for malignant lesions
