SOFT TISSUE TUMOR

DR MANOJ DAS Ortho resident

Institute Of Medicine , TUTH , Nepal

Intoduction

• Neoplastic condition arising from extra-skeletal mesoderm

-Fibrous tissue

-Smooth muscle

-Skeletal muscle

-Adipose tissue

-Blood and lymphatics vessels

-Peripheral nerves

Epidemology

-The annual clinical incidence (number of new patients consulting a doctor) of benign soft tissue tumours has been estimated as up to 3000/million population

-The annual incidence of soft tissue sarcoma is around 30/million i.e. less than 1 percent of all malignant tumours

-Benign mesenchymal tumours outnumber sarcomas by a factor of at least 100.

Classification

Classification…

Classification…

Aetiology• Unknown

• In rare cases, genetic and environmental factors, irradiation, viral infections and immune deficiency have been found associated with the development of usually malignant soft tissue tumours

Aetiology• Chemical carcinogens

-Several studies, many of them from Sweden, have reported an increasedincidence of soft tissue sarcoma after exposure to phenoxyacetic herbicides,chlorophenols, and their contaminants(dioxin) in agricultural or forestry work{607,608}.

-Other studies have not found this association. -One explanation for different findings may be the use of herbicides with different

dioxin contaminations {4,2333}.

Aetiology…Radiation

-incidence of post-irradiation sarcoma ranges from some few per thousand to nearly one percent.

-incidence estimates are based on breast cancer patients treated with radiation as adjuvant therapy. The risk increases with dose; most patients have received 50 Gy or more and the mediantime between exposure and tumour diagnosis is about 10 years.

-Patients with a germline mutation in the retinoblastomas gene (RB1) have a significantly elevated risk of developing post-irradiation sarcomas, usually osteosarcoma

Aetiology…- Viral infection and immunodeficiency Human herpes virus 8 plays a

key role in the development of Kaposi sarcoma and the clinical course is dependent on the immune status of the patient {2232}.

-Epstein-Barr virus is associated with smooth muscle tumours in patients with immunodeficiency {1368}.

-StewartTreves syndrome, development of angiosarcoma in chronic lymphoedema, particularly after radical mastectomy, has by some authors been attributed to regional acquired immunodeficiency

Aetiology…Genetic susceptibility

hereditary multiple lipomas (often angiolipomas)

Desmoid tumours occur in patients with the familial Gardner syndrome (including adenomatous polyposis, osteomas and epidermal cysts)

Neurofibromatosis (types 1and 2) is associated with multiple benign nerve tumours (and sometimes also nonneural tumours). In around 2% of the patients with neurofibromatosis type1 malignant peripheral nerve sheath tumours develop in a benign nerve sheath tumour

Li-Fraumeni syndrome {954} is a rare autosomal dominant disease caused by germline mutations in the TP53 tumour suppressor gene, which seems to be of importance for sarcomagenesis. Half of the patients have already developed malignant tumours at age 30, among them, in more than 30% of cases, soft tissue and bone sarcomas

.

Approach to diagnosis

History - Age- Symptoms Mass Pain Numbness - Duration- Trauma- Family History- Constitution Symptoms

Approach to diagnosis…Local Examination -site -Size -Depth -Consistency -Mobility -Pulsation -Tinel’s Sign -Neurovascular Status

• General Physical examination

- café-lauit spot - Freckles• Lymph Node

Examination

X -Ray

• Provide little information

• Fat density radiolucencies

• Phleboliths

• calcification

CT Scan

• Replaced by MRI in most institutions due to the superior soft tissue contrast achieved by MRI

• still be useful for imaging soft tissues near the chest wall that are subject to motion artifact with MRI, and in cases where MRI is not an option

MRI- modality of choice for detecting, characterizing,

and staging soft tissue tumours

- distinguish tumour tissue from adjacent muscle and fat

- define relationships to key neurovascular bundles

PET Scan• assess metabolic function and can be used

to diagnose, stage, and monitor treatment response for many cancers, and some soft tissue tumors.

• One specific application is in the evaluation of multifocal desmoid tumors, and more specifically, response to treatment.

“ Kasper et al. demonstrated that PET imaging might complement CT and improve the assessment of patients with desmoid tumors. Imatinib, a tyrosine kinase inhibitor, has been shown to successfully stabilize desmoid tumors, and PET imaging has been used to monitor response to imatinibtreatment in patients with multifocal desmoids [9]”

Biopsy

• open (incisional or excisional) or closed (needle or trephine)

Closed BiopsyAdvantage

- less invasive, -require smaller doses of anesthetic and analgesic agents -lower risk of tumor contamination, hemorrhage, and infection Disadvantage -Smaller amount obtained for pathologic review

Some rules for for Biopsy “One should always approach tissue biopsy with the assumption

that there is a possibility for malignancy, using a well-planned biopsy site”

- Perform the biopsy through a longitudinal incision.

-In the operative dissection, do not expose or contaminate important vascular or neural structures.

- Do not violate anatomic compartments other than those containing the tumor.

- Take special care around joints to prevent intraarticular contamination with tumor cells. - Biopsies of soft tissue should not involve major tendons (e.g., the patellar tendon) or their insertion -In a biopsy, do not use intramuscular or intranervous planes, as in more standard orthopaedic

approaches. Instead, use a direct approach with the smallest possible incision directly through skin, subcutaneous tissue, fascia, and muscle down to tumor

Staging of soft tissue tumor

Staging of soft tissue sarcomaAmerican joint committee on cancer for staging of soft tissue sarcoma

Treatment of Benign Soft tissue tumor Observation

- Asymptomatic - <5cm - Superficial to fascia -Reevaluation every 6-12 weeks followed by every 3-6

months for 1 yr to document lack of growth

Biopsy and Surgical Excision - not following natural history -Symptomatic

Treatment of Soft tissue SarcomaGoal of Treatment

-minimizing local recurrence -maximizing function -improving patient survival

Surgery- Mainstay of treatment

Limb Salvage Surgery VS Amputation

• Amputation rarely done nowadays

• Limb sparing procedure are performed in 90-95% of cases

“Rosenberg et al published a landmark article in 1982, a prospective randomized trial to evaluate the issue of amputation versus limb-sparing surgery plus radiation therapy in soft tissue sarcomas of the extremity. They found that although local recurrence was greater in the limb-sparing group, disease-free survival was no different”

Role of Radiotherapy in soft tissue sarcoma

• enhance local control• preserve function• achieve acceptable cosmesis by contributing to tissue preservation

“Dagan et al. performed a retrospective review to evaluate the local control and amputation-free survival in patients who received preoperative radiation therapy prior to undergoing a marginal resection for a soft tissue sarcoma of the extremity. The authors concluded that patients can expect excellent rates of local control and limb preservation regardless of whether they have a marginal, wide, or radical resection according to the classic Enneking margin definitions [37].”

Role of Chemotherapy in soft tissue Sarcoma

• Controversial and still investigated• palliative and used to treat unresectable or metastatic disease to try and slow

progression “ In 1997, the Sarcoma Meta-Analysis Collaboration published their results of

doxorubicin-based chemotherapy regimens combined with surgery for local control. Disease-free survival improved from 45 % for control patients to 55 % for chemotherapy patients, an advantage of only 10 %. The authors were also unable to show a statistically significant benefit to improved survival at 10 years, with survival of patients undergoing chemotherapy at 54 %, compared to the control at 50 %”

Favorable response to chemotherapy, -synovial sarcoma -Liposarcoma - pediatric rhabdomyosarcom

Surveillance Following Definitive Treatment

• For potential development of local recurrence or metastaticdisease

• 40–60 % of patients will develop local or distant recurrence, and mostrecurrences occur within 2 years of treatment of the primary tumor

• Current recommendations for surveillance include:- - clinical exam and chest radiograph or CT scan every 3 months for the first 2 years,

every 4 months for the third year, every 6 months for the following 2 years, and annually thereafter

• Local recurrence monitored with physical exams of the surgical site atroutine intervals

-If the patient is felt to be at high risk for local recurrence or thereis concern on physical exam findings, then an ultrasound or MRI with and without gadolinium should be obtained.

Some important soft tissue tumor

LIPOMA• the most common soft tissue lesions• AGE:- 5th to 7th decades of life without a clear gender predilectioN• prevalence:- 2.1 per 100 people• Site:-upper back, neck, proximal extremities, and

abdomen• Clinical Presentation -Painless mass -can be a/w local pain, limitation of range of motion, and nerve compression in approximately 25

% of patients -Usually Solitary

• On physical Examination Mobile Doughy in consistency usually S/C InvestgationsX-ray - Soft tissue radiolucencyMRI – Investigation of choice Isointense to s/c fat

LIPOMA…Tissue Diagnosis Gross -yellow to orange

color with a greasy appearance

Microscopic-mature adipocytes with uniform

eccentric nuclei

Lipoma…

Treatment Observation Excisional biopsy -increasing size -Symptomatic - Cosmetically undesirsble

Benign Peripheral Nerve Sheath TumorInclude -Schwannoma -Neurofiroma

Age:- 20-50yr Schwannoma 40-50 yr NeurofibromaA/W Neurofibromatosis Type 2

Clinical Presentation - Painless mas - Neurological symptomsOn Physical Examination -Firm - Mobile -Tender to touch and pressure ( Schannoma)

Benign Peripheral Nerve Sheath Tumor..

X-Ray- Soft tissue massMRI -Fusiform mass, often

displacing neurovascular bundle

Benign Peripheral Nerve Sheath Tumor…

Tissue Diagnosis Gross-cut surface of a schwannoma has a

yellow-graycolor, sometimes with cystic regions

Microscopic- Antoni A- Antoni BNeurofibromas-- not encapsulated- Histologically spindle-shaped cells in

a myxoid stroma, with some collagenfibers.

Benign Peripheral Nerve Sheath Tumor…

TreatmentObservation with Serial RadiographSurgical Excision

Risk for neurological deficit after surgical excision??? - secondary to iatrogenic fascicular injury “Kim et al. evaluated postoperative neurological deficits and potential risk factors

associated with this complication. They found an immediate neurological deficit in 76.7 % of their 30patients, and at final followup, residual deficits persisted in 36.7 % of patients. Therisk for deficits was highest in patients with larger tumors.”

Giant Cell Tumor of Tendon Sheath• the most common benign neoplasm of the hand, often found on the fingers• fourth to fifth decade of life• On physical examination

-firm -minimally mobile mass that runs along tendon -usually nontender

Radiographs -nonspecific -cortical erosion

Ultrasound -To distinguish this solid tumor from a ganglion cyst

MRI - circumscribed soft tissue mass adjacent to a tendon, and is isointense with muscle on T1-weighted images

Giant Cell Tumor of Tendon Sheath…

• On gross examination -dense capsule - C/S gray-white appearance with variegated

pink, brown, or yellow discoloration

• Histologically the predominant cells are mononuclear

cells, epithelioid histiocyte like cells, giant cells, and xanthomatous cells, hemosiderin-laden macrophages

Treatment -Excision Biopsy

Recurrence Rate 10-20 %

Hemangioma• benign vascular lesions that histologically resemble normal blood vessels• Common tumor in 1st decade of life• Sites - Skin - Subcutaneus tissue - Intramuscular -Intraarticular -BoneTypes - Capillary - Cavernous -Venous -Arteriovenous Malformation

Intramuscular Hemangioma

• More often larger than cutaneous hemangioma• Composed of morphologically benign vascular channels occuring within

muscle• Age – Adolescents and adult• Sites –Lower limb m/c, Head neck, upper limb• Clinical presentation - Slowly enlarging mass - painful -pain worse after exercise -Changes size according to position of limbX-Ray -Phlebolith -Pressure erosion of bone or extension in to bone( Channel like

radiolucencies )

Intramuscular Hemangioma…CT Scan

- poorly defined lesion attenuation similar to muscle

-Marked contrast enhancement of serpentine vascular channels

-Subtle phlebolith

MRI

- hypointense, or isointense to muscle on T1-weighted sequences

-hyperintense on T2-weighted sequences due to thevascular channels

Intramuscular Hemangioma…Gross- large, poorly demarcated,

yelowish- Vascular and hemorhagic areas

Microscopic- Mixed vessel type- Lined by normal appearing

epithelial cells- Diffusely infiltrate muscle and

entrap muscle cells- Prominent adipose component

Intramuscular Hemangioma…

Treatment -Observation -Surgical excision -Embolization -Injection with sclerosing agents

Desmoid Tumor• Benign , non metastasizing infiltrative fibrofroliferative neoplasm

• Composed of fibrocytes, fibroblasts, and myofibroblasts within a collagenenous to myxoid stroma

• Infiltration to muscle occur routinely

• High propensity for local recurrence

• A/W Familial Adenomatous polyposis

Desmoid Tumor…• Age 15-40• F>M• Sites – -shoulder girdle/upper arm(mc) -Buttock -Trunk -Head and Neck

Clinical Presentation - painless/painful mass -When in close proximity to a joint, range of motion may be limited -Neurological symptoms if the tumor compresses or invades peripheral motor or

sensory nerves.

Desmoid Tumor…

X-ray -Soft tissue mass -Cortical erosion

CT scan - Similar accentuation to muscle

MRI-low signal intensity on both T1

and T2 Sequence- Frequently enhance after i/v

contrast

Desmoid Tumor…Gross- Large tumor - Glistening with fibrous tissue- Infiltrates skeletal muscle at

periphery

Microscopic- Normal appearing sparse

fibroblastic cells within dense fibrous stroma

- Macrophage, lymphocyte, giant cells

Desmoid Tumor…

Treatment

- Observation for asymptomatic and stable- Aggressive treatment for symptomatic lesion Surgical excision+ Adjuvant Radiotherapy

Glomus Tumor• Benign lesion arising from glomus bodies• 2 % of soft tissue tumors• Sites- hand especially within the subungual skin, palm, wrist, forearm, or

foot• Age – adult• Clinical presentation: Triad of -hypersensitivity to cold - paroxysmal pain -pinpoint pain

• Love’ s Pin test- 100% sensitive• Hildreth’s test- 100% specific and & 79% sensitive

MRI-increased signal intensity on T2-

weighted images, and strong contrast enhancement

Gross- small blue red nodules

Microscopic- glomus cells , smooth cells, blood vessels

Treatment -Excision biopsy

Synovial chondromatosis• disorder in which cartilaginous nodules are formed within a synovial joint

• The nodules then detach from the synovium and may undergo calcification

• Age- third and fifth decades

• M:F 2:1

• Site- Usually monoarticular with the knee being the most common joint affected, but it can involve any synovial joint

• Clinical presentation -pain, swelling, stiffness, and sometimes locking of the involved joint.

On physical examination - effusion, tenderness, and decreased ROM.

Synovial chondromatosis…Radiograph -multiple intra-articular densities when

calcified - ‘‘ring-and-arc’’ chondroid mineralization

pattern

MRI -visualizing radiolucent nodules where they have low signal on T1-weighted images, and increased signal on T2-weighted images

-erosive changes of thearticular cartilage

-in late stages, there is joint space narrowing, osteophytes, andsclerosis much like an osteoarthritic joint

Synovial chondromatosis…

On gross inspection-the synovial nodules, which are composed of

hyalinecartilage, typically have a cobblestone appearance

histologic appearance -benign hyaline cartilage, with low to

moderate cellularity, and a synovial tissue lining outside

Treatment-Nodule removal and Synovectomy-Open or arthroscopic approach

Liposarcoma• 20 % of all soft tissue sarcomas in adults• Ages 50–65• Site-thigh and retroperitoneum

• 1) Well-differentiated liposarcomas (atypical lipomatous tumor) 2)Dedifferentiated liposarcomas• 3)Myxoid or Round cell liposarcomas• 4)pleomorphic

Rhabdomyosarcoma

• most common soft tissue sarcoma of infants and children, although they can occur in adults

• Site – in areas lacking skeletal muscle- head, neck and genitourinary tract• Embryonal rhabdomyosarcoma –most common type -head , neck and genitourinary tract

• Alveolar rhabdomyosarcoma - Aggressive type -arm, leg and trunk

Pleomorphic rhabdomyosarcoma -common in adults

- poor prognosisClinical presentation depends on location

TreatmentSurgery+ Chemotherapy+Radiotherpy

In summary…

Thank you