Malignant Cutaneous Neoplasms Flashcards
Morphoeaform (sclerosing, desmoplastic) and infiltrative, as well as lesions with micronodular or basosquamous histopathologic changes, are more aggressive
T-
JAAD Feb 2019 CME
although rare, perineural invasion in BCC indicates an aggressive variant with incr rates of metastasis and locoregional recurrence
T-
JAAD Feb 2019 CME
Nodular BCC accounts for 25-40% of lesions
F - Nodular BCC accounts for 50-80% of lesions
JAAD CME 2019
Micronodular BCC has no appreciable connection to overlying epidermis
T
Metastatic BCC = 0.003-0.005%
True
Lymph node metastasis followed by LUNG and BONE metastasis is the most common progression of metastatic BCC
True
What are high risk factors for SCC Mets?
- tumour thickness (2.1 - 6mm have 4% mets; >6mm have 16% mets)
- poor differentiation
- immunosuppression
- Location on lips or ear
- ## SCC’s in genital region are also more aggressive
SCC on highly sun damaged skin are more indolent and less likely to metastasise
True
What is Li-Fraumeni Syndrome
Rare familial cancer syndrome where germline mutations in TP53 genes play an important role
- syndrome is characterised by an autosomal dominant inheritance pattern and affected individuals display early onset of various tumours;
- breast cancer, brain tumours, osteosarcoma and leukaemia
- however, skin cancer does not occur as commonly as expected given that TP53 mutations are frequently observed in BCCs and SCCs
What is basal cell naevus syndrome ?
- Germline activating mutations in PTCH1 gene are found in vast majority of patients with this syndrome
- The most common inherited disorder associated with BCCs is BCN’s
- affected individuals have a wide range of developmental anomalies —> skeletal abnormalities, craniofacial dysmoprhism, macrocephaly
- multiple BCC’s with early onset are a hallmark of the syndrome, also featuring odontogenic keratolysis, palmoplantar pits, calcification of the falx cerebri
- also have incr risk of meningiomas, ovarian fibromas and ca, and cardiac fibromas
What is Bazex syndrome?
Rare genodermatoses characterised by follicular atrophoderma and early onset of multiple BCC’s
- responsible gene has been linked to Xq24-27 and there is no male-to-male transmission
- X-linked dominant disorder characterized by the triad of diffuse hypotrichosis, follicular atrophoderma, and BCCs
Milia cysts,hypohidrosis, trichoepitheliomas, and facial hyperpigmentation can also be seen
What is Rombo syndrome?
Rare autosomal dominant disorder presenting with acral erythema, facial vermiculate atrophoderma, multiple milia, telangiectasias, hypotrichosis and a tendency to develop milia and BCCs
Actinic keratosis risk factors:
- male
- elderly
- those with lighter skin photo types
- hx of chronic sun exposure
Lichenoid actinic keratosis can have associated pruritus or tenderness associated with onset of lichenoid infiltrate
T
Atrophic AK’s have minimal surface change
T
Actinic cheilitis does not resemble the classic form of AK
False
Arsenic induced SCC in situ resembles the classic variety clinically
True, also has a marked tendency to be multifocal + arise in sunprotected areas of the trunk
Ferguson-Smith KA’s are multiple + non-regressing
False - spontaneously regress
Grzybowski KA’s erupt in a generalised fashion
True
KA’s have been associated with chemical exposures, immunosuppression, BRAF inhibitors and HPV infection
True
Keratoacanthoma can reach several centimetres in diameter
True - eg. Keratoacanthoma centrifugum marginatum
KA can heal with prominent scarring
True
Grouped KAs often resolve more quickly than solitary KA’s
False - other way around
Ferguson-Smith syndrome is autosomal recessive with mutations in TGFBR1 which encodes TGF-B receptor type 1.
False - autosomal dominant
