SKIN / SOFT TISSUE Flashcards
Most common soft tissue sarcoma
Malignant fibrohystiosarcoma
also called
pleomorphic sarcoms
Malignant fibrohystiosarcoma with multiple metastases - what is the management
Can do multiple metasectomy
Soft tissue sarcomas most important predictors of prognosis are
mitotic index
and
amount of necrosis.
Chemotherapy is effective with what type of sarcoma
CAREFUL radiation therapy most effective for sarcomas overall
Ewing’s Sarcoma, that show survival benefits with neoadjuvant chemotherapy. (KIDS, ONION PEAL)
staging of sarcoma
High-grade histologic findings
I-III
deep Fascia
> 5 cm
CAREFUL - nodes considered advanced disease
Staging classifies lesions as:
-T1 - 5 cm
-N1 - regional node involvement
-G1 - well-differentiated -G2 - moderately differentiated -G3 - poorly differentiated -G4 - undifferentiated.
Staging is based on T, N, M and G classification.
Stage IV disease is classified as any G or T with regional nodal involvement (N1) OR evidence of metastatic disease (M1).
Li-Fraumeni syndrome associated cancer
Colon cancer
colonoscopy every 2-3 years beginning
breast pre-menopausal
annual breast MRI and twice annual clinical breast examination beginning at age 20-25 years.
adrenocortical carcinoma (ACC)
soft tissue sarcoma,
osteosarcoma,
brain tumors,
leukemias.
most common site of metastasis of sarcomas.
The lung is the most common
hematogenous metastasis
Staging of sarcoma should include
CHEST CT - rule out lung metastasis, especially for high grade, large (> 5 cm) sarcomas.
NOT mandatory to do CT of the abdomen and pelvis with extremity sarcoma,
MAY consider CT abd/pelv if: myxoid liposarcoma, epitheliod, angiosarcoma synovial sarcoma, which commonly metastasize to the abdomen...
when would Imaging alone be considered diagnostic (without bx)
well-differentiated liposarcoma
(also known as atypical lipomatous tumors).
what sarcoma need adjuvant
almost all need xrt
may not need radiation therapy, provided that they can be reliably followed:
small (1 cm)
nonneoplastic tissue or biologic barrier (fascia)
breast sarcoma
Primary angiosarcoma
breast parenchyma young women.
The only potentially curative therapy is surgery.
patients should be offered a simple mastectomy instead of lumpectomy (don’t need nodes in sarcoma)
Partial resection of pectoralis major may be necessary to achieve negative margins!
The skin may be closed primarily.
staging with:
CT chest, abdomen, and pelvic
MRI of the breast!
contralateral breast may be a site for metastatic disease, but to date, a contralateral prophylactic mastectomy has no proven benefit.
in general responsive to systemic chemotherapy - unlike other sarcomas (besides Eweing)
However, once chemotherapy is stopped, the disease tends to regrow.
AND NO proven survival benefit from systemic therapy.
non-op tx of desmodis tumors
first choice
NSAIDS
Tomxafen
maybe: cytotoxic intravenous chemotherapy with different agents and schedules, oral tyrosine kinase inhibitors, GLEVAC?
Dermatofibrosarcoma protuberans
locally aggressive
slow, infiltrative growth.
low to intermediate grade.
minimal metastatic potential
tends to recur locally after surgical excision.
appear range from plaque-like to elephant nose
commonly on the trunk and proximal extremities.
Diagnosis punch biopsy.
Excisional biopsy should be avoided because reexcision will be required to obtain wide negative margins.
Other than routine chest x-ray, no preoperative metastatic work-up is indicated.
node exam on physical only
wide, pathologically negative margins as the optimal treatment for primary or recurrent DFSP.
For tumors occurring on the head, face, or other areas where wide local excision is not cosmetically acceptable, Mohs micrographic surgery may be a reasonable alternative.
Radiation therapy is typically provided as adjuvant treatment.
What lab do you need with every melanoma workup
LDH
When do you give interferon
Positive lymph node
Locally advanced disease
Metastatic disease
What are the adjuvant therapy options for melanoma
Yervoy
Zelb B-raf - targets B-raf
must have b - raf pos
Merkel cell
Presentation and treatment
This can be deep sub-cu and mimic a lipoma!
2 cm margin
Sentinel lymph node
If node pos:
XRT!
And
chemo (Cis-Platnum)
If node neg:
Chemo
Cis-platinum
Platinum treatment for a special non lipoma lesion
Tumor markers for melanoma
and path fetures to request
LDH
Other histopathologic features have prognostic implications and should be routinely described on the pathology report:
regression, microsatellitosis, vertical growth phase, angiolymphatic invasion, tumor-infiltrating lymphocytes
(b-raf if considering brafanimb)
“Duh” B-raf enib
mitoses/ mm2.
Removed nodes are then carefully evaluated using hematoxylin and eosin staining and immunohistochemistry techniques with antibodies to one or more melanoma marker epitopes such as:
S100,
HMB45,
MART-1/ Melan-A.
Chemotherapy for melanoma
Interferon
“Duh” B-raf enib
(if B-raf pos marker)
yervoy
Treatment of melanoma by thickness
Less than one mm
And no ulceration
1 cm wide local excision
No sentinel left on biopsy
1 cm or greater
2 cm wide local excision
Sentinel lymph node biopsy
Satellite lesions
In transit lesions
Node stage for melanoma
N1 1
a: micrometastases*
b: macrometastases¶
N2 2-3
a: micrometastases*
b: macrometastases¶
c: in-transit met(s)/satellite(s) without metastatic lymph nodes
N3 4 or greater
or
matted lymph nodes, or in-transit met(s)/satellite(s) with metastatic lymph node(s)
T stage melanoma
T1 ≤1.0 mm
a: without ulceration and mitoses 4.0 mm
a: without ulceration
b: with ulceration
Patients with clinical stage I and II (localized) melanoma are those with
no clinical or radiologic evidence of regional or distant metastases.
Stage I and II are node negative just like colon
Clinical stage III patients are those with regional metastases, manifesting as enlarged lymph nodes or satellite or in-transit disease or both.