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Skin and soft tissue > Malignant skin and soft tissue lesions & tumours > Flashcards

Malignant skin and soft tissue lesions & tumours Flashcards

1
Q

what are the advantages of incisional biopsy compared to core needle biopsy for diagnosis of suspected soft tissue sarcoma?

A
  • lower/very low risk of insufficient tissue sample or inconclusive area of tumour
  • amount of sample provides pathologists w/ greater confidence in grading tumour
  • amount of sample allows for additional analyses to classify/type the sarcoma, influences treatment and prognosis
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2
Q

What is the mechanism for development of cutaneous malignancy?

A
  • Radiation or other stimuli induce electron excitation in absorbing atoms, which causes chemical damage to DNA
  • Genetic mutations (inherited, spontaneous) contribute: p53 (BCC, SCC), oncogenes ras & fos, PTCH (BCC), CDK2NA & CDK4 genes (MM)
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3
Q

define BCC

A
  • cutaneous malignancy originating from basal layer of keratinocytes in epidermis and epidermal appendages
  • doubling time 4 d (0.5cm / yr)
  • arise de novo; no precursor lesion
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4
Q

How do you classify BCC? Describe classification.

A
  • There are many histological subtypes (> 26)
  • Many (~ 40%) BCC are histologically a combination of > 1 subtype
  • Clinical subtypes:
    • Nodular / nodulo-ulcerative: 50-70% - firm, round skin coloured or lightly pigmented papule with defined borders, often pearly border and telangectasia. As grows larger will outstrip blood supply centrally and form characteristic central ulcer (rodent ulcer)
    • Superficial spreading: 10-20% - lightly pigmented erythemetous macule/patch often trunk/shoulder
    • Pigmented: 5% looks like pigmented nodular w/ similar behaviour; MM in ddx
    • Morpheaform/sclerosing: 2-3%, white/yellow/pink with central sclerosis, thick ropey looks like a scar and ill-defined borders; most likely to recur / have +ve margins
    • Others: infiltrative, desmoplastic, basosquamous, (all higher risk recurrence); cystic (central tumour degeneration), micronodular (higher recurrence than nodular; looks like collection little hair bulbs)
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5
Q

how do you diagnose and what is characteristic pathologic finding of bcc

A
  • diagnosis can be suspected on history and physical
  • definitive diagnosis by biopsy and pathologic review
  • charactertistic path finding is peripheral palisading of basaloid cells, basaloid cells in dermis
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6
Q

Describe bowen’s disease

A
  • Cutaneous SCC in situ; in anogenital region (esp glans) called Erythroplasia of Queryat, also oral mucosa, conjunctiva, nail bed (subungual epidermoid carcinoma)
  • sharply demarcated hyperkeratotic plaque on erythemetous sometimes indurated base
  • Pathology: WINDBLOWN appearance; list 6 features of pre-malignant change
  • prognosis: 5-10% risk malignant transformation/progression to SCC (higher for oral, anogenital, nailbed ~ 30%)
  • Biopsy to diagnose
  • Treat - medical: Imiquimod, PDT, consider XRT
  • Treat - surgical excision w 2-5mm margin (want negative margin), consider MOHS
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7
Q

List and describe non-surgical options for treatment of pre-malignant and malignant cutaneous lesions

A
  • Imiquimod (Aldara)
    • MOA: immune response modifier - act through TLR to amplify NK and B-cell activity
    • Uses - On-label: AK, superficial BCC, genital warts; Off-label: nodular BCC, Bowen’s disease, porokeratosis, Lentigo meligna, extra-mammary paget, Keloid, +ve margins
    • How to use: apply topical qhs (leave overnight) x wks (2-16; often ~ 6)
  • 5-FU (Effudex)
    • MOA: blocks DNA synthesis as a pyrimidine antagonist
    • Uses - On-Label: AK, superficial BCC; Off-label: Bowen, porokeratosis, extra-mammary paget
    • How to use: apply BID for 2-6 wks
  • Retinoids
    • MOA: inhibits hyperproliferating keratinocytes
    • Uses - all off-label: AK (probably most accepted), arsenic keratosis, lentigo meligna, superficial BCC
    • 0.5 - 2mg/kg/d PO x wks
  • Photodynamic Therapy
    • MOA: photodynamic activiation of a topical sensitizing agent leads to cytotoxic effect against oxygen free radicals
    • Uses - all investigational at present: AK, BCC, SCC in situ
  • Radiation
    • Uses: BCC, SCC, Merkel cell, Keloid scar, Kaposi sarcoma
    • Indications: non-operative candidate, refractory to other treatments, perineural or lymphovascular invasion, + margins, as adjuvant therapy when LN +
    • Contraindications: Gorlin disease, pregnancy, Lupus and some other CTD, Verrucous carcinoma
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8
Q
  • List and briefly describe different treatment modalities for primary BCC including indications and techniques
A

MEDICAL

  • Imiquimod & 5-FU - superficial BCC (off label nodular BCC) - apply at night x wks
    • disadvantages is can’t assess margins and difficult to measure response
  • PDT - in infancy, not mainstream
  • Radiation - 92% cure; for older patients, non-surgical candidate, recurrent, + margins, perineural or lymphovascular invasion, or as adjuvant for N+ or T3/4

SURGICAL

  • Cryotherapy - series of applications of -40’c
  • Electrodessication and curretage - debulk tumour, currette bed, dessicate bed
    • both above cannot assess margin status
    • for low-risk and small lesions with defined borders
  • Surgical excision
    • Margins - for low risk tumours
    • for high risk tumours or > 2cm then 1cm is reasonable.
  • MOHS
    • Indications: indistinct borders (morpheaform/sclerosing/infiltrative/some ss), sensitive areas (H&N, specifically eye, NL fold, nose, peri-oral), some say very large.
    • excise deem and rim at 45’ wtih small margin, map and section horizontally
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9
Q

List indications for MOHs

A
  • High risk BCC or SCC lesions
    • Size and location:
      • >=6mm H face (mask face), genitalia, hands, feet (H face is periorbital, nose, perioral, pre/postauricular, temple, ear)
      • >=10mm cheek, forehead, scalp, neck, pre-tibia
      • >= 20mm anywhere else
    • Indistinct borders
    • Rapidly progressive
    • Recurrent
    • Previous XRT of bed
    • Pathologic subtype
      • SCC, poorly or undifferentiated, adenocystic, adenosquamous, basosquamous
      • BCC, morpheaphorm/sclerosing, micronodular
    • Other pathologic features
      • LVI, PNI
      • depth > 2mm
  • Other lesions that can be treated by MOHs: KA, DFSP,
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10
Q

If MOHs is unavailable but indicated, what is an alternative?

A

excision of complete circumferential and deep margin and intra-operative frozen section assessent

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11
Q

How do you perform mohs?

A
  1. debulk central tumour
  2. excise deep and peripheral margins, with small margin of normal tissue, tangential at 45’
  3. divide into quadrants & map
  4. serial horizontal section, examine
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12
Q

List principles of incisional biopsy when considering soft tissue sarcoma

A
  1. pre-procedure MRI whenever possible, especially with high suspicion
  2. incision along axis of extremity (to be included in definitive WLE)
  3. incision provide direct access to tumour, consideration to non-central area to avoid necrosis
  4. do not elevate skin flaps or undermine tissue
  5. prophylactic and judicious hemostasis
  6. closed suction drain exit through incision
  7. simple suture closure
  8. specimen for permanent pathologic review
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13
Q

what soft tissue tumours are likely to metastasize to regional nodal basin (and therefore SLNB are appropriate?)

A

SCARE

Unclear evidence; investiagted in high risk types; not definitive

Risk in below is 11-44%

  • synovial sarcoma
  • clear cell sarcoma
  • angiosarcoma
  • rhabdomyosarcoma
  • epithelioid sarcoma
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14
Q

what are the consequences of unplanned positive margins after primary resection of known soft tissue sarcoma?

A
  • higher radiation doses
  • greater risk of complications related to radiation
  • higher incidence of metastasis
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15
Q
A
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16
Q

Compare considerations for limb-salvage & reconstruction of sarcoma in lower vs. upper extremity

A
  • Lower extremity values stability and weight-bearing, whereas upper extremity values functional mobility or range of motion
  • Post-op appearance is less important for lower extremity, because differences can be more easily hidden by clothes and the lower extremity is not as important for routien social function (ie shake hands)
  • Atherosclerosis and orthostatic venous pressure are more profound and LE vs UE, and this is exacerbated by need to bear weight and may influence reconstructive decisions and outcomes
  • Nerve regeneration is less successful overall, at any age, in LE vs UE
  • Wound healing is slower, and risk of post-p[perative comlications is higher in LE vs UE
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17
Q

List features that would make you concerned for sarcoma, and start a sarcoma work-up

A
  1. Lesions that are attributed to a trauma, but have not disappeared after 4 wks
  2. Subfascial, popliteal or groin location
  3. Continue to demonstrate growth or are symptomatic (pain, paresthesia, bleeding, change in function)
  4. Size of > 5cm
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18
Q

what is the imaging diagnostic mainstay of sarcoma workup?

A

Gadolinium contrast-enhanced MRI

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19
Q

How are soft tissue tumours classified?

A

4 categories

  • Benign: - rarely recur locally, if they do then cured by complete excision
  • Intermediate - locally aggressive: infiltrative and locally destructive, require WLE
  • Intermediate - locally aggressive and rarely metastasizes: locally aggressive w/ low risk for distant metastasis (< 2%), DFSP
  • Malignant: locally destructive, high risk for recurrence and distant mets
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20
Q

describe the pseudocapsule around a sarcoma

A

forms 2 zones

  • Compression zone: peripheral portion of tumor compressing surrounding normal soft tissue; potentially contains scattered tumor cells; may demonstrate neovascularity
  • Reactive zone: thin layer of inflammatory tissue surrounding the compression zone
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21
Q

List syndomes w/ a predisposition to sarcoma

A
  • NF 1 (MPNST)
  • Li-Frumeni - rhabdomyosarcoma, osteosarcoma
  • Gardner syndrome - desmoid tumours (also BCC)
  • Gorlin syndrome - rhabdomyosarcoma (also BCC)
  • Werner syndrome (Adult progeria) - STS, bone sarcoma
  • Retinoblastoma - STS, bone sarcoma
  • Beckwith-Wiedmann syndrome
  • Enchondromatosis syndromes
    • Olivier - chrondrosarcoma
    • Mafucci - angiosarcoma, chondrosarcoma (also hemangioma w/ enchondromas)
  • McCune Albright - osteosarcoma (endocrine abn + fibrous dysplasia)
    *
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22
Q

discuss the relationship btwn radiation and sarcoma

A
  • Dose-dependent (at 5000cGy or more); mutations in p53
  • Incidence rate estimated at 0.03-0.80%
  • Criteria to qualify as post-radiation sarcoma
    • Sarcoma in the irradiated field + Histologic confirmation + Latency of at least 3yr
  • Most common types: Undifferentiated pleomorphic sarcoma & extra-skeletal osteogenic; most are high grade
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23
Q

discuss the relationship between chronic lymphedema and sarcoma

A

Lymphangiosarcoma post radical lymphadenectomy/mastectomy (Stewart-Treves syndrome)

rare < 1%

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24
Q

Compare benign vs. malignant features of SOFT TISSUE TUMOURS

A

Benign

Malignant

99% superficial

Depth: Extremity: 1/3 superficial 2/3 deep

95% < 5cm diameter (only 5% benign masses exceed 5cm)

Large: extremity median diameter = 5cm (superficial) to 9cm (deep); retroperitoneal much larger

~ 3/4 extremities (thigh; 45% LE & 15% UE); 10% trunk; 10% retroperitoneum; 10% H&N; rest abdomen/chest wall

slightly M > F

increased age (median age 65yrs); but embryological rhabdosarcoma = children; synvoial sarcoma = young adults;

liposarcoma/leiomyosarcoma/pleomorphic high grade sarcoma (ie MFH) = elderly

Innocent presentation: painless, not usually causing functional impairment, accidentally observed,

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25
Q

DESCRIBE MRI FEATURES OF SARCOMA

A
  • Non-specific: > 5cm diameter; Necrotic and heterogeneous; Deep to investing fascia; Pseudocapsule; intra- vs. extra-compartmental invasion
  • Specific to MRI: Hypointense on T1, hyperintense T2 (usually fat bright T1, water bright T2)
  • UPS – calcifications, older pts
  • Synovial sarcoma – calcifications + hypervascular
  • Liposarcoma – foci of fat
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26
Q

List tumours that show calcification on MRI

A
  • Malignant: Liposarcoma; synovial sarcoma; epithelioid sarcoma
    • also breast
  • Benign: Pilomatrixoma; myositis ossifications; trichilemmal cyst; hemangioma
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27
Q

what concerning features on history or physical wound prompt you to get an MRI or other imaging modality prior to biopsy?

A
  • Deep to investing fascia
  • > 5cm, associated with pain or neurologic symptoms
  • rapid growth
  • lesions over NV structures or bone
  • (+/- not resolved w/i 4 wks)
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28
Q

List and describe the methods available for biopsy for sarcoma workup?

A
  • incisional biopsy
    • indication: size > 3-5cm, subfascial
  • excisional biopsy
    • indication: size < 3-5cm, suprafascial
    • leave fascia
  • core biopsy may be more appropriate for deep / subfascial lesions
    • TruCut needle retrieves thin core of tissue (+/- CT guided)
    • disadvantage: 1) sufficient sample for dx; 2) sufficient sample for additional tests to determine histologic grade and subtype (influence treatment and prognosis); 3) increase risk of sample inconclusive tumour site
  • FNA
    • consider for retroperitoneal or intraabdominal
    • disadvantage: high volume/experience required for interpretation, limited sampling
    • 85% accuracy vs open biopsy at 96%
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29
Q

describe principles of incisional biopsy for suspected sarcoma (soft tissue tumour)

A
  • incision oriented with long axis of limb limited to one compartment (or to facilitate easy excision on trunk/chest)
  • shortest incision directly over tumour
  • no surrounding tissue dissection and no flaps
  • sutures close to incision; no mattress sutures or external drain sites
  • meticulous hemostasis with pneumatic tourniquet but no exsanguination
  • no drain or use of closed suction drain through incision
  • permanent pathologic review (this is the principle, a hint is to send to frozen to ensure that you have tumour and not just necrotic tissue)
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30
Q

describe staging systems for sarcoma

A
  • Staging (regardless of system - GRADE, SIZE, DEPTH)
  • ACJJ staging following TNM + sarcoma grade (G) criteria
    • T (1=< 5 or 2=> 5; a=superficial or b=deep); N (rare); mets (lung); G (2 - 4 grade system)
  • Deep (b) – Located in the muscular compartment of the extremity (automatically includes H&N, intra-thoracic, intra-abdominal, retroperitoneal, visceral
  • Grade: degree of differentiation, mitotic activity, % tumour necrosis
  • Musculoskeletal tumour society - T1/T2 (intracompartmental vs extra compartmental), G1/2, M0/1
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31
Q

what are the most important pathologic features to capture during sarcoma evaluation

A

o histologic subtype, mitotic index, % tumour necrosis

32
Q

what are different treatment regimines available for sarcoma?

A
  1. neoadjuvant chemo —> surgery —> adjuvant chemo & radiotherapy
  2. neoadjuvant chemo with pre-op radiotherapy —> surgery —> adjuvant chemo
  3. neoadjuvant chemo —> pre-op radiotherapy —> surgery —> adjuvant chemo
33
Q

describe how you will take your margins during extirpation of biopsy proven sarcoma

A
  • PRINCIPLE highlighted by Dr. Ferguson: if the tumor can have wide local excision with ~ 2-3cm margin without loss of function, then surgery alone is probably sufficient.
  • If bone, NV structures or limb amputation would need to be sacrificed to obtain a clear WLE, then preoperative radiation is employed… then only a microscopic negative margin is required.
34
Q

what are goals of surgery in sarcoma surgery?

A

minimize local recurrence and distant metastasis,

preservation of function,

preservation of quality of life

35
Q

describe how margins are CLASSIFIED in sarcoma surgery

A
  • UICC classification
    • R0 - resection with microscopically tumor-free margin
    • R1 - resection with microscopically tumor-positive margins
    • R2 - resection with macroscopically tumor-positive margins
  • Enneking classification: 4 types of surgical margins
    • Curative wide margin – More than 5 cm outside reactive zone
    • Wide margin – Less than 5 cm (Adequate if greater than 2cm and inadequate if less than 1cm)
    • Marginal Margin – Margin is in the reactive zone
    • Intralesional Margin – Passes through the tumor
36
Q

what are the PRINCIPLES of sarcoma surgery?

A
  • wide excision with adequate margins (aim is always R0 resection)
  • 2-3cm of normal tissue with fascial layer as deep margin = wide margin (without sacrificing bone/nerves/vessels)
  • if cannot achieve this principle requires pre-op radiation and/or adjuvant tx
  • Dissection through normal tissue uncontaminated by tumor (close to major nerves/vessels –> resect adventitia or perineurium, consider pre-op XRT)
  • resection includes all previous skin incisions/biopsy sites, stitch marks, drain sites
  • bloodless field with pneumatic tourniquet (with no exsanguination)
  • surgical clips placed at periphery of excision
  • atraumatic no-touch technique with tumour (no retraction or manipulation)
  • preservation of uninvolved blood vessels/perforators (as potential microsurgical recipients)
  • Limb salvage surgery or functional limb preservation
37
Q

describe indications (considerations) for amputation vs. limb salvage

A
  • Amputation indications: (rarely required; < 5% extremity sarcoma) Ghert et al. Annals Surgical Oncology 2004
  • Unable to attain negative microscopic margins with surgical resection (even after pre-op radiation)
  • Complete resection would result in a non-functional limb
  • Prior unplanned excision with widespread contamination
  • Co-morbidities of patient preclude extensive reconstruction
  • Dr. Wunder – Vascular involvement, motor nerve involvement, infected tumour, poor soft tissue tumour, expected functional outcome worse than with amputation
38
Q

when do you consider pre-op and post-op radiation in sarcoma?

A
  • pre-operative: (Preferred) High grade sarcomas, anticipated close margins, unresectable/anticipated sig functional loss with resection
  • adv: lower XRT volume and dose, limb preservation, improved long-term functional outcome, easier resection (thick pseudo cap), less fibrosis and edema, less fracture, possibly lower local recurrence / higher survival
  • disadv: double wound healing problems (35% vs. 17%)

Post operative

  • adjuvant radiation for close margins, microscopically +ve margin on bone/nerve/vessel, extra muscular involvement / high grade / deep
  • Adv ; fewer wound healign prob
  • Disadv: reuqires higher dose of Rtx =>increaed toxicity (ORN, lymphedema, fracture)
39
Q

discuss principles of chemotherapy in sarcoma

A
  • consider neoadjuvant and adjuvant therapy for high-grade or > 5cm
  • · generally thought to be chemo-resistant

· chemo-sensitive tumours: Rhabdomyosarc, Ewings +/- synovial, liposarcoma

o Predictive factors for subpopulations benefitting from chemo include: Quality of resection, Sex (males), Age > 40, grade 3

40
Q

what are goals of reconstruction after sarcoma surgery?

A

o tension free closure

o durable wound coverage

o obliteration of dead space

o restoration of form (ie restore/reconstruct involved tissue/structures)

o restoration of function

41
Q

describe liposarcoma

A
  • 11 % of adult sarcomas; more common b/w 50-70yr
  • Large tumors commonly found in LE and RP
  • Pathology: 4 subtypes, common morphologic denominator is presence of a lipoblast
  • Dedifferentiated – malignant
  • Myxoid – malignant
  • Pleomorphic – malignant
  • Not otherwise specified - malignant
  • Treatment: radical or en bloc excision + Radiotherapy may improve survival (children and extremity lesions have better prognosis)
42
Q

discuss DFSP

A
  • Superficial low-grade, locally aggressive fibroblastic neoplasm
  • Higher grade fibrosarcomatous progression in 10-15% of lesions
  • Firm nodular raised lesion attached to overlying skin
  • Often on trunk and proximal extremities (b/w 20-40yr)
  • Bednar tumour (AKA pigmented DFSP): DFSP with presence of melanin (in DDx of melanoma)
  • Histology: spindle cells
  • Treatment: wide local excision with 2-4cm margins to deep fascia, potential indication for Moh’s surgery (tendency to recur, 20-50%) ; delay reconstruction until permanent margins clear
  • Positive margins – re-resection until clear; unable to obtain clear margins, consider RT or Imatinib Mesylate (Gleevec)
43
Q

discuss kaposi sarcoma

A
  • Vascular proliferative disorder found in patients with HHV8/Kaposi sarcoma associated herpesvirus infection
  • Usually limited to skin, may involve mucous membranes, visceral organs, lymph nodes
  • Epidemiology/types:
  • Classic – Eastern European/Mediterranean/Jewish descent, older man M:F 9:1
  • Endemic – African children and adults (KS most common tumor in African male!)
  • Immunosuppressed – Transplant (0.5 – 5% incidence) (aggressive)
  • Epidemic – 10% of patients with HIV/AIDS will show KS (aggressive)
  • Treatment – consider when symptomatic; refer to med onc +/- rad onc
  • Medical – modification of immune suppression (anti-retrovirals), RT (v. radiosensitive), intralesional chemo (vincristine)
    • For disemminated lesions, those outside of 1 radiation field
  • Radiation: multiple symptomatic lesions in single radiation field
  • Surgical – only for discomfort, over joints, etc.

Prognosis – commonly recur, slow progression

44
Q

what is merkel cell tumour?

A

· Arises in the dermis, usually adults/elderly, F>M (60:40), face & extremities most commonly

· Neural crest derived cell (Neuroendocrine tumor), very aggressive malignancy

45
Q

what is the other entity that merkel cell can be confused w/ on pathology? how would you differentiate?

A

· can be difficult to discern btwn primary MCC and metastatic small cell carcinoma lung

o Immune: CK-20 and thyroid transcription factor 1 (TTF-1)

46
Q

how would you treat clinically node negative merkel cell carcinoma? (include discussion of margins and consideration of adjuvant therapy)

A
  • WLE: 1-2cm margins to investing fascia or pericranium when feasible; after or with SLNB
    • Immediate reconstruction of defect, avoidance of delayed wound healing (i.e. avoid delay in adjuvant RT), avoidance of complex options until negative margins confirmed
    • XRT mono therapy if: WLE not possible, associated with significant morbidity, pt refusal
  • SLNB with immune panel: CK-20 and pancytokeratins
  • adjuvant XRT:
    • to primary site (not required if lesion < 1cm and no LVI or immune suppression)
    • to draining nodal basin if: high risk false positive (H&N lesions) or SLNB not performed or SLNB +
    • consider XRT to draining nodal basin if SLNB - and high risk (RF to increase chance of FN SLNB)
  • imaging: CT / PET / MRI to draining nodal basin, chest, abdomen, head if SLNB+
47
Q

discuss treatment of clinically node + merkel cell carcinoma

A
  • FNA or core biopsy
    • N+: 1st step to define distant mets: CT / PET / MRI: chest, abdomen, +.- brain; WLE + ELND; adjuvant XRT to primary +/- nodal basin
    • N-: open bx, then pathway for N+ or N0
48
Q

Define sarcoma and carcinoma

A

Sarcoma - Malignant neoplasm of cells derived from mesenchymal tissue

Carcinoma - malignant neoplasm derived from epithelial cell orgin

49
Q

How do you classify soft tissue tumors

A

WHO classification by malignancy nd tissue origin

  • benign - superficial, curable by complete excision
  • intermediate (locally aggressive) - require WLE
  • intermediate (rarely metastasize) - <2% risk mets
  • malignant - 20-100% recurrence/mets

Tissue origin

  • Adipocytic
  • Fibroblastic/myofibroblastic
  • Fibrohistiocytic
  • Smooth muscle
  • Skeletal muscle
  • Pericytic (perivascular)
  • Vascular
  • Nerve sheath
  • Gastrointestinal stromal
  • BONE
    • chondrogenic
    • osteogenic
    • fibrohistiocytic, fibrogenic
    • hematopoietic
    • osteoclastic
  • Tumor of uncertian differentiation
  • undifferentiated
50
Q

Describe epidemiology of sarcoma

A
  • 5th most common cancer in children, rhabdomyosarcoma most common
  • Site: extremity 60%, trunk 20% RP 15% H&N 10%
  • 5y survival 50%
  • 75% of sarcoma are
    • UPS - undiff pleomorphic sarcoma
    • liposarcoma
    • MPNST
    • leiomyosarcoma
    • myxofibrosarcoma
    • synovial sarcoma
51
Q

What are 2 zones of pseudocapsule surrounding sarcoma

A

Compression zone - adjacent ot tumor, may contain tumor cells

Reactive zone - inflammatory tissue

52
Q

List 10 genetic syndromes with associated soft tissue tumors

A
  • NF1 (AD, NF1 mut)
    • neurofibroma, MPNST, GIST, Rhabdomyosarc
  • NF2
    • schwannoma
  • Lifraumeni (AD, p53)
    • Rhabdomyosarc, osteosarc
  • Gardner (AD, APC)
    • CF osteoma, desmoid, fibroma
  • Gorlin (AD, PTCH1)
    • Rhabdomyosarc, cardiac fibroma, rhabdomyoma
  • Werner (AR, WRN) - progeria = premature aging
    • bone soft tissue sarcoma
  • Tuberous Sclerosis (AD, TSC)
    • fibroma, rhabdomyoma, angiomyolipoma
  • Beckwidth-wiedemann (sporadic AD)
    • rhabdomyosarc, myxoma, fibroma, hamartoma
  • enchondromatosis (ollier D, mafucci)
    • enchondroma, chondrosarcoma, angiosarcoma
  • McCune albright
    • osteosarcoma
53
Q

List risk factors for deevelopment of soft tissue tumor

A
  • Genetic mutation
  • Environmental carcinogen (asbestos, herbicide, arsenic)
  • Radiation
    • sarcoma occurs in radiated field with >3y delay
  • Lymphedema
    • lymphoangiosarcoma in post ALND/Mx = stewart Treves syndrome
  • Viral infection
    • HHV8 (Kaposi), EBV (SM sarcoma)
  • Trauma
    • rare, 2-50yr latency
  • Malignant transformation from benign
    • eg. neurofibroma ->MPNST (nearly always in NF1)
54
Q

Describe history/PE/investigations/w/u for patient presenting with lesion suspicious for sarcoma

A

History

  • lesion onset, growth, associated sx (pain pruritis, ulceration, neurogenic, trauma, early saiety)
  • constitutional sx
  • occupational exposure, Rtx
  • PMHx, FmHx (syndromes)

P/E

  • location, size, color, cosistency, shape of tumor, relative to other important structures
  • tenderness, trasnillumination,t ethered
  • NV assessment
  • regional distnat LN

Investigation

  • Imaging before biopsy
    • MR for extremity
    • CT for RP
    • CXR, CT lung,abdo,pelvis bone scn for mets
  • Biopsy -based on location and size
    • Core
    • Open
    • FNA
55
Q

What are features on mri of sarcoma

A
  • Mass >5cm, deep
  • Heterogenous, Necrotic
  • Calcifications (UPS, Synovial sarc)
  • Hypodense T1 hyperdense T2
  • pseudocapsule
  • foci of fat (liposarc)
56
Q

How do you biopsy lesion for ? sarcoma

A
  • Core biopsy vs ope biopsy - simialr histologic accuracy of grading
  • FNA - for RP, intra-abdo
  • Open Biopsy -> Incisional vs excisional
    • Excisional - only if <5cm and superficial
    • Incisional -
      • minimal tissue manipulation
      • incise directly over tumor
      • orient longitudinal along extremity
      • no flap/recon
      • avoid tumor dissemination - meticulous hemostasis, no drains
      • permanent section
      • suture close to incision
      • avoid local field blocks
57
Q

What are prgnostic factors?

A
  • Poor prognosis
    • age >60
    • recurrence
    • positive margins
    • tumor >5cm
    • high grade (histology, mitosis, NV invasion)
  • Mets at presentation - single most important predictor
58
Q

Describe a lipoma epidemiology, associated syndromes,Tx, recurrence

A
  • most common STT
  • adults >40yo, associated w obesity in trunk, neck, proximal extremities - rare in hand, face, leg
  • multiple lipoma rare, Associted with
    • NF
    • Bannayan syndrome (lipoma, hemangioma, macroceph)
    • MEN syndrome
  • Tx - excision, 1-4% recur
  • Liposarcoma deep vs lipoma superficial
59
Q

Describe liposarcoma

A
  • In legs, RP, age 50-70
  • 4 types
    • dediferentiated
    • myxoid
    • pleomorphic
    • NOS
  • Tx - radical excision +Rtx
60
Q

List and clasiffy fibroblastic/myofibroblastic STT

A

Fibromatoses = tumor with prolif of fibroblast/myofibroblast w infiltrative growth, excess collagen deposition, high recurrence but no metastases

Benign

  • Nodular Fasciitis
  • Proliferative Fasciitis/Myositis
  • Myositis ossificans
  • Fibromatosis colli
  • Fibroma of tendon sheath

Intermediate - locally aggressive

  • Superficial fibromatoses
  • Desmoid fibromatoses

Intermediate - rarely metastasize

  • DFSP
  • Solitary fibrous tumor
  • Infantile fibrosarcoma

Malignant

  • Fibrosarcoma
61
Q

Describe nodular fasciitis

A

Benign fibroblastic STT

  • rapidly grows, may regress, on volar arm, back, chest, 20-40yo
  • Tx - excision
62
Q

Describe proliferative myositis/fasciiits

A

Benign fibroblastic STT, self limiting

  • rapid scar like tissue growth in muscle or fascia, firm solitary nodule
  • Tx - excise, recurrence low
63
Q

Descrieb myositis ossificans

A

Benign fibroclastic STT, intramuscular lesion = get benign heterotopic ossification of osft tissue

  • young males, hx of trauma 6wks previous, surgery, burns
  • 2types
    • myositis ossificans circumscripta (localized)
    • myositis ossificans progressive
  • Tx: splint to decrease ROM, rest, NSAIDs, excise when quiescent
64
Q

Describe superfical fibromatoses

A

Intermediate lcoally aggressive fibroblastic STT

  • growth arising from fascia and early (proliferative) phase followed by late (collagenous, retractive) stage

4 types

  • Palmar fibromatoses (dupuytren)
  • Penile fibromatoses (Peyronie)
    • growth on dorsum b/w c. spongiosum and tunica albigunia
    • excise and STSG/flap
  • Plantar fibromatoses (Ledderhosen)
    • medial arch
    • Tx steroid or excise
  • Knuckle pads (Garrod)
    • on MCP or PIP
    • Tx steroid/excision
65
Q

Describe DFSP

A

Intermediate rarely metastasizing fibroblastic STT

  • low grade, locally aggressive
  • 10-15% progess to fibrosarcoma
  • firmly attached to skin on trunk and extremitys, 20-40yo
  • Bednar tumor - pigmented DFSP
  • Tx - WLE 2-4cm margin to deep fascia, delay recon until clear margin. if positive re-resect. If cant get clear, Rtx, or Imatinib mesylate
66
Q

Descrie infantile and adult fibrosarcoma

A

Intermediate infantile, Malignant adult fibroblastic STT

  • growth in extremities, painless, hard and fixed
  • Tx WLE, chemo. Rtx for adult
  • Poor prognosis for adult, <50% 5yr survival
67
Q

Describe Giant cell tumor of tendon sheath or Tenosynovial giant cell tumor

A

Benign fibrohistiocytic STT

  • Localized type - 2nd most common hand tumor
    • Tx - excision
  • Diffuse type - PVNS -pigmented villonodular synovitis - occurs near joint
    • locally aggressive,often recurs
68
Q

Describe rhabdomyosarcoma

A

Malignant Skeletal muscle STT

  • most common STT sarcoma in children
  • associated with lifraumeni
  • most develop in H&N, GU, RP despite little SK muscle in these areas, rest UELE
  • Types: spindle (best) embryonal, alveolar, pleomorphic (worst)
  • Tx - surgical resection + chemo/rtx
69
Q

Describe Kaposi Sarcoma

A

Intermediate rarely metastasizing vascular STT

  • occurs in pts with HHV8 viral infection
  • limited to skin but may involve mucous membranes
70
Q

Describe angiosarcoma

A

Malignant vascular STT

  • rare
  • types:
    • cutaneous, no ass. lymphedema
    • cutaneous, w ass. lymphedema
    • angiosarcoma of breast
    • angiosarcoma of deep soft tissue
  • deeply invasive, blue red macules
  • poor prognosis - difficult to resect, multiplicity of lesions
71
Q

Describe Nerve sheath tumors

A

Schwannoma (benign) = neurilemmoma

  • schwann cells proliferation
  • can preserve nerve origin, peel off, unlike NF

Neurofibroma (benign)

  • non -encapsulated, mutliple tumors if ass. NF1
  • malign degen rare if non NF pt
  • Indication for excision
    • pain
    • neurologic impairment
    • compression
    • rapid growth suggestie of degneration

MPNST = malignant schwannnoma

  • 50% associted with NF, rest de novo
  • Tx radiacal excision
72
Q

What is a atypical fibroxanthoma

A

Atypical rarely metastasizing tumor of uncertain differentiation

  • dermal based
  • occurs in elderly, H&N
  • red juicy dome, bleeding common
  • Tx: excision, low recurrence
73
Q

What is an epithelioid sarcoma

A

Malignant tumor of uncertain differentiation

  • occurs in hands/feet
  • misdiagnosed as ulcer, SCC, infected wart
  • tx - radical excision, rtx, chmo
  • high recurrence and mets
74
Q

WHat is a clear cell sarcoma

A

Malignant tumor of uncertain differentiation

  • young adults deep soft tissue of extremity/trucnk = melanoma of the soft parts
  • Tx - resection, high rte of nodal/distant mets
75
Q

What is an undifferentiated pleomorphic Sarcoma UPS

A

Formely known as MFH - malignant fibroud histiocytoma

  • fibroblst, myofibroblast, histiocytes
  • apepars like AFX, DFSP
  • in adults, LE, adjacent to orthopedic implants??
  • WLE may be curative
76
Q

What is merkel cell tumor?

A

Malignant tumor of NCC arising in the dermis

  • F>M, adult/elderly, fce/extremtiy most common
  • SLNB prior to WLE with 1-2cm to fascia
  • +/- rad chemo
  • AJCC staging
    • T: <2, 2-5cm, >5cm, invading deeper structures
    • N: mets regional, in transit
    • M: met beyong LN, met to lung, met to bone
  • Treatment
    • N0 clinically - WLE 1-2cm, SLNBx
      • SLNBx +: imaging, LND +/- Rt to nodes
      • SLBX -: Rt only if high risk
    • N+ clinically - FNA/core
      • +: imaging, LND or Rtx
      • -:consider open bx
    • M1 - chemo, rad, WLE
77
Q

What are 6 painful skin or subcutaneous nodules

A
  • angiolipoma
  • glomus tumor
  • angioleiomyoma
  • eccrine spiradenoma
  • neuroma
  • blue rubber bleb nevus

Skin and soft tissue (13 decks)

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