Transcript ADDITIONAL MALIGNANCIES OF THE SKIN

ADDITIONAL MALIGNANCIES OF
THE SKIN
Merkel Cell Carcinom
Once thought to be a variant of SCC, Merkel cell carcinomas are
actually of neuroepithelial differentiation.These tumors are
asso-ciatedwith a synchronous .
Due to their aggressive nature, wide local resection with 3-cm margins
is recommended.
Local recurrence rates are high, and distant
Metastases occur in one third of patients.
prophylactic regional LN dissection and adjuvant radiation therapy are
recommended.
Overall, the prognosis is worse than for malignant melanoma.
Merkel cell carcinoma
Merkel cell carcinoma
Merkel cell carcinoma
Merkel cell carcinoma
Merkel cell carcinoma
Merkel cell carcinoma
Kaposi's Sarcoma
Kaposi's sarcoma( KS) appears as rubbery bluish nodules that occur
primarily on the extremities but may appear any, where on the skin
and viscera .
The lesions are locally aggressive but undergo periods of remission.
A different variety of kS has been described for people with AIDS or
with immunosuppression from chemotherapy.
For reasons not yet understood AIDS-related KS occurs primarily in
Male homosexuals and not in IV drug abusers .
In this form of the disease the lesions spread rapidly
to the nodes, and the GI and respiratory tract often are lnvolved.
Sarcoma kaposi
Sarcoma kaposi
Sarcoma kaposi
Sarcoma kaposi
Sarcoma kaposi
Sarcoma kaposi
Sarcoma kaposi
Sarcoma kaposi
Angiosarcoma
Angiosarcomas may arise spontaneously mostly on the scalp,face,
and neck.They usually appear as a bruise that spontaneously bleeds
or enlarges without trauma.
Tumors also may arise in areas of prior radiation therapy or in the
setting of chronic lymphedema of the arm, such as after mastectomy
( Stewart-Treves syndrome).
Although total excision of early lesions can provide occasional cure,
the prognosis usually is poor,
Chemotheray and radiation therapy are used for palliation.
angiosarcoma
angiosarcoma
angiosarcoma
angiosarcoma
angiosarcoma
angiosarcoma
angiosarcoma
angiosarcoma
Stewart- treves syndrom
Stewart- treves syndrom
Stewart- treves syndrom
Stewart- treves syndrom
Dermatofibrosarcoma protuberans
Dermatofibrosarcoma protuberans( DFSp) accounts for I to 2% of
all soft-tissue sarcomas occurs most frequently in persons aged 20 to
50 years,and is more common in males.
The most common presenting location is on the trunk (50 to 60%) although
the proximal extremities (20 to 30 % o f cases),as well as head and neck
Also aref requently affected( 10 to 15%).
DFSP often appears as a pink, nodular lesion that may ulcerate and become
infected.
local recurrence remains frequent and mortality associated with metastasis
Relatively high
DFSP
DFSP
Fibrosarcoma
Fibrosarcomas are hard, irregular masses found
in the subcutaneous fat.
The fibroblasts appear markedly anaplastic with
Disorganized growth. If they are not excised
completely,metastasis usually develop.
The 5-year survival rate after excision is
approximately 60-7o%.
Fibrosarcoma
Fibrosarcoma
Fibrosarcoma
Fibrosarcoma
Liposarcoma
Liposarcomas arise in the deep muscle planes, and,
rarely, from the subcutaneous tissue.
They occur most commonly on the thigh.
An enlarging lipoma should be excised and
inspected to distinguish it from a liposarcoma.
Wide excision is the treatment of choice, with
radiation therapy reserved for metastatic
disease.
Liposarcoma
Liposarcoma
Liposarcoma
Liposarcoma
SYNDROMIC SKIN MALIGNANCIE
Several genetic syndromes are associated with
An increased incidence of skin malignancy.
Based on their respective genetic defects,
Syndromes associated with BCC, SCC, and
melanoma have all been identified and well
describe.
Basal cell nevus syndrom
(Gorlin's)
Basal c ell nevus syndrome is an autosomal
Dominant disorder characterized by the growth of
hundreds of BCC during young adulthood.
Palmar and plantar pits are a common physical
finding and represent foci of neoplasms.
Treatment is limited to excision of only aggressive
and symptomatic lesions.
Gorlin
Gorlin
Gorlin
Gorlin
Gorlin
Gorlin
Nevus sebaceus of jadassohn
Is a lesion containing several cutaneous
tissue elements that develops during
childhood.
This lesion is associated with a variety of
neoplasms of the epidermis, but most
commonly BCC.
Nevus sebaceous of jadassohn
Nevus sebaceous of jadassohn
Diseases associated with SCC
Skin diseases that cause chronic wounds, such as epidermolysis
bullosus and lupus erythematosus, are associated with a high
incidence of SCC.Epider_ modysplasia verruciformis is a rare
autosomal recessive disease associated with infection with HPV. Large
Verrucous lesions develop early in life and often progress to invasive
SCC in middle age.
Xeroderma pigmentosum is an autosomal recessive disease
associated with a defect in cellular repair of DNA damage. The
inability of the skin to correct DNA damage from UV radiation leaves
these patients prone to cutaneous malignancies.
SCCs are most frequent, but BCCs, melanomas, and even acute
leukemias are seen.
XP
XP
XP
XP
Familial
dysplastic nevus syndrome
Is an autosomal dominant disorder.
Dysplastic nevi are considered precursors to
melanoma .
Patients develop multiple dysplastic nevi, and
longitudinal studies have demonstrated an
almost 100% incidence of melanoma.
Much like familial polyposis coli and the association
with colon cancer, familial dysplastic nevus
syndrome is treated by close surveillance and
frequent biopsy of all suspicious lesions .
Similarly,the development of colon cancer can be
arrested with total proctocolectomy; unfortunately,
a similar solution is not possible in
patients with familial dysplastic nevi
dysplastic nevus syndrome
dysplastic nevus syndrome
dysplastic nevus syndrome
dysplastic nevus syndrome
Soft Tissue Sarcomas
lncidence
• Soft tissue sarcomas can occur throughout the body and
encompass more than 50 histologic lines of differentiation.
The most common histologic types of soft tissue sarcoma in adults
(excluding Kaposi's sarcoma) are malignant fibrous histiocytoma
(MFH, 28%o)leiomyosarcoma( l2o/o),
Liposarcoma ,synovial sarcoma( l0o/o) ,and malignant peripheral
nerve sheath tumors .
Rhabdomyosarcoma is the most common soft tissue sarcoma of
childhood.
Most primary soft tissue originate in an extremity (50 to 60%); the
next most common sites are the trunk, retroperitoneum, and head
and neck.
Epidemiology
Despite the variety of histologic subtlpes, sarcomas have many
Common clinical and pathologic features. Overall, the clinical behavior
of most soft tissue sarcomas is similar and is determined by
anatomic location (depth), grade, and size. The dominant pattern of
metastasisis hematogenous primarily to the lungs. Lymph node
Metastase are rare (<5%) except for a few histologic subtypes such
as epithelioid sarcoma, rhabdomyosarcoma, clear-cell sarcoma,
synovial sarcoma, MFH, and angiosarcoma.
Radiation Exposure
External radiation therapy is a rare but well-established risk factor for
soft tissue sarcoma. An eightfold to 50-fold increase in the incidence
of sarcomas has been reported among patients treated for cancer of
the breast,cervix, ovary testis,and lymphatic system.In a review
of 160 patients with postirradiation sarcomas, the risk of developing
a sarcoma increased with higher radiation doses, and the median
latency period was 10 years. Post irradiation sarcomas usually are
diagnosed at advanced stages and generally have a poor prognosis.
Occupational Chemicals
Exposure to some herbicides such as phenoxyacetic
acids and chlorophenols has been linked to an
Increase risk of soft tissue sarcoma.
Several chemical
carcinogens, including thorium oxide (Thorotrast),
vinyl chloride, and arsenic have been associated
with hepatic angiosarcomas.
Trauma
• No causal relationship has been established
Chronic Lymphedema
In 1948,Stewart and Treves first described the
association between chronic lymphedema after
axillary dissection and subsequent
lymphangiosarcoma.
Lymphangiosarcoma also has been reported as
occurring after filarial infections and in the lower
extremities of patients with congenital or heritable
lymphedema.
Clinical Presentation
Soft tissue sarcomas most commonly present as an asymptomatic
mass.
Soft tissue sarcomas often grow in a centrifugal fashion and
compress surrounding normal structures' Infrequently, their impinge.surronding on bone or neurovascular bundles produces pain, edema,
And swelling.
Retroperitoneal soft tissue sarcomas almost always Present as
large asymptomatic masses.
Less frequently, Patients present with obstructive GI synptoms or
Neurologic symptoms related to compression of lumbar or pelvic
nerves.
Diagnostic lmaging
Ultrasonogrophy
ComputedT omography; Contrast-enhanced
CT can assess the extent of soft tissue tumor burder
and the proximity of the tumor to vital structure
Magnetic Resonance Imaging;
MRI accurately delineates muscle groups and
distinguishes among bone, vascular structures, and
tumor
Biopsy Techniques
Fine-Needle Aspiration;
Fine-needle aspiration is an acceptable method of diagnosing most
soft tissue sarcomas particularly when the results Correlate closely
with clinical and imaging findings.
lncisional Biopsy;
Open biopsy is a reliable diagnostic method that allows adequate
tissue to be sampled for definitive and specific histologic identification
of bone or soft tissue sarcomas. When adequate tissue for diagnosis
cannot be obtained by fine-needle aspiration biopsy or core biopsy, an
incisional biopsy is indicated for deep tumors and for superficial soft
tissue tumors >3 cm.
Excisional Biopsy;
Excisional biopsy can be performed for easily accessible
(superficial) extremity or truncal lesions <3 cm. Excisional biopsy
should not be done for lesions involving the hands and feet,
because definitive re-excision may not be possible after the
biopsy.
Prognostic Factors
Histologic Grade;
Histologic grade remains the most important prognostic factor for
Patients with sarcomas.
The features that define grade are cellularity, differentiation,
pleomorphism, necrosis, and the number of mitoses.
Tumor grade has been shown to predict the development of metastas
and overall survival.The metastatic potentials Have been estimated at
5 to 10% for low-grade lesions,25 to 30%
for intermediate-grade lesions,and 50 to 60% for high-grade Tumors.
Tumor Size;
Tumor size has long been recognized to be an important
prognostic variable in soft tissue sarcomas. Sarcomas have
classically been stratified into two groups on the basis of size;
T1 lesions are <5 cm,
and T2 lesions are >5 cm.
Nodal Metostasis;
Lymph node metastases arising from soft tissue sarcomas are
rare.
A few histologic subtypes,including epithelioid sarcoma,
rhabdomyosarcoma, clear-cell sarcoma, synovial sarcoma, MFH,
and angiosarcoma, have a higher incidence of nodal
involvement.(stage 3)
Distant Metastasis;
Distant metastases occur most often in the lungs .
Selected patients with pulmonary metastases may survive for
long periods after surgical resection and chemotherapy. Other
potential Sites of metastasis include bone,the brain, and the
liver .
Visceral and retroperitoneal sarcomas have a higher incidence of
liver and peritoneal metastases.
rhabdomyosarcoma
rhabdomyosarcoma
rhabdomyosarcoma
rhabdomyosarcoma
rhabdomyosarcoma
rhabdomyosarcoma
MFH
MFH