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Epidermal Nevi, Neoplasms, and Cysts Part II Rick Lin, D.O., MPH, Dermatology Resident Texas Division of KCOM Dermatology Residency Program Basal Cell Carcinoma lBasal Cell Epithelioma lBasalioma lRodent ulcer lJacobis ulcer lRodent carcinoma BCC: What are they? lPEARLY PAPULES OR NODULES lROLLED BORDER lTELANGIECTASES lCENTRAL ULCER lCRUSTING lBLEED EASILY BCC: Where are they? lHEAD, NECK 85% lNOSE, 30% lFOREHEAD lEARS lCHEEKS lUPPER TRUNK BCC: When? lOFTEN 1/3 OF ALL CA IN USA. lChronic UVB, X-ray lImmunosuppression lRenal Transplant lGenetics BCC: Who? lELDERLY & MIDDLE AGED lAges 40-79 lANGLO-SAXON Blue Eyes, Fair Skin lX-Ray Exposure, ie Physicians, Dentists, Technicians, Workers BCC: How? lArise from immature pluripotential cells. lMutations in the HEDGEHOG pathway (genes which controls cell growth) lPATCHED (tumor suppressor) inactivated. lHEDGEHOG and SMOOTHENED (cell growth inhibitors) activated. lP53 and RAS mutations also play a role. BCC look-alikes: SGH BCC look-alikes: KA BCC Look alikes: SCC BCC: Variants lSUPERFICIAL BCC lMORPHEAFORM BCC lPIGMENTED BCC lCYSTIC BCC lBASAL CELL NEVUS SYNDROME (GORLINS SYNDROME) SUPERFICIAL BCC lPSORIASIFORM lTRUNK lLIMBS lFLAT GROWTHS lYOUNGER PATIENTS MORPHEAFORM BCC lRESEMBLES LOCALIZED SCLERODERMA lALMOST ALWAYS ON THE CHEEKS OR FOREHEAD lMOHS SURGERY lAGGRESSIVE PIGMENTED BCC lDARK SKINNED PATIENTS lLATIN AMERICANS lJAPANESE lNOT BLACKS (we have one black patient in the clinic though) lARSENIC INGESTION l6% OF ALL BCC BCC CYSTIC/SOLID lDOME SHAPED lBLUE GRAY lCYSTIC NODULES l4-8% OF ALL BCCS Fibroepithelioma of Pinkus lPremalignant fibroepithelial tumor lElevated, skin-colored sessile lesions on the lower trunk lHistology: interlacing basocellular sheets that extend downward from surface to form an epithelial meshwork enclosing a hyperplastic mesodermal stroma lA composite scan power view showing anastomosing bands of epithelium separated by large amounts of stroma. The stroma accounts for over 50% of the total volume of the tumor. lAnother composite scan power view. This is from a section taken parallel to the one above. The strands of epithelium are generally more delicate than those seen above. lPeripheral palisading of nuclei is associated with a cleft between the epithelium and the delicately fibrillar, slightly basophilic stroma. lThis clefting resembles that seen in basal cell carcinomas. lAmyloid (AMY) is seen below an area wherein parallel, coarse collagen fibers (VC) are oriented perpendicular to the interface of the epithelium and stroma. BCC TREATMENT lEXCISION lFULGURATION AND CURETTAGE lIONIZING RADIATION lCRYOSURGERY lTOPICAL 5-FU lLASER lMOHS MICROGRAPHIC 99% CURE Solitary Basal Cell Carcinoma in Young Persons lSolitary Basal Cell Carcinoma in Young Persons lThese lesions usually located in the region of embryonal clefts in the face lDeeply invasive lDeep surgical excision is much safer than curettage for their removal NEVOID BCC SYNDROME lJAW CYSTS lPALMAR PITS lSKELETAL DEFECTS lFRONTAL BOSSING lCALCIFICATION OF FALX CEREBRI lMOHS SURGERY Jaw Cysts l70% of the patient. lBoth Mandible and Maxilla lMandibular involvement twice as often lJaw pain, unable to close mouth, tenderness lFirst decade onset, maybe the first presentation Pits of hands and feet l87% of patients lSecond Decade of life Skeletal Defects lSpinal Bifida lDeformed ribs lScoliosis and Kyphosis lShorten metacarpal and metatarsal bones lDimple on the fourth metacarpophlangeal joint (Albrights sign) CNS disorders lCalcification of: falx cerebri, falx cerebelli, and dura or basal ganglia Intraepidermal Epithelioma lTan-brown, keratotic scaly, flat, someimes verrucous lesions. Clinically resembles Seborrheic keratosis. lSimple excision or EDC lAlso Known as: Borst Jadassohn epithilioma Intraepidermal epithelioma of Jadassohn Intradermal Nests of Basaloid Cells Squamous cell carcinoma lSquamous cell carcinoma (SCC) is a malignant neoplasm of keratinocytes with many features one of which is the production of keratin. lSCC can be categorized histologically into in situ (intraepidermal) or invasive (penetrating the dermal-epidermal junction). lSome examples of in situ SCC include Bowens disease and erythroplasia of Queyrat. Squamous cell carcinoma lSquamous cell carcinoma is the second most common skin cancer after basal cell carcinoma. lIt typically occurs on sun-exposed areas of the body and is more common in light- skinned men greater than 55 years. lThe incidence of SCC increases closer to the equator. Predisposing factors for SCC lfamily history of skin cancer lprecursor lip lesions from smoking lactinic keratosis lold burn scars lImmunosuppression lultraviolet radiation lradiation therapy lchemical carcinogens such as soot and arsenic Squamous cell carcinoma lLesions on the lower lip (13.7%), or in a scar (37.9%), have up to a 40% probability of metastasizing. lDesmoplastic SCC are 6 times more likely for metastasis lLesions on sun-damaged skin have a 2% tendency to metastasize. lMetastasis is primarily by way of the lymphatics, generally first to regional lymph nodes. Treatment lTreatment choice is dependent on lesion type, size, location, depth of penetration and the patients age and general health. lTreatment modalities include excisional surgery, curettage and electrodessication, cryosurgery, radiation therapy, Mohs surgery, and laser surgery. SCC with cutaneous horn Here is a cutaneous horn, overlying a tumor which on biopsy proved to be a squamous cell carcinoma. The presence of cutaneous horn is grounds for a biopsy of the underlying lesion. Encrusted squamous cell carcinoma Another firm tumor on the abdomen, this time with both scale and crust. Biopsy of this tumor revealed squamous cell carcinoma. Chronic sun exposure and squamous cell carcinoma This gentleman was in his 60s when he presented to the clinic because of the frequent development of skin cancers. You can see his scarred skin from the multiple previous procedures. On the superior aspect of the left breast is a crusted lesion which to palpation is firm. Biopsy confirms SCC. Squamous cell carcinoma of the lip Sun damage on the lower lip can result in actinic cheilitis and even squamous cell carcinoma as shown here. Squamous cell carcinoma of the scalp In his 30s when he presented to the clinic, this engineer had spent some years in Saudi Arabia and had neglected a growth on the top of his head at the site of a burn. At the time of presentation the tumor had been present for about 2 years. Biopsy revealed SCC and a workup revealed distant metastases. Shortly after presentation, he died from this tumor. Squamous cell carcinoma of the scalp Crusted and eroded tumor of the scalp in this elderly man was histologically SCC. Actinic keratosis These are scaly papules which occur on exposed skin of older, fairer- skinned, persons resulting from chronic overexposure to ultraviolet light from the sun. A small percentage of these lesions do develop into invasive squamous cell carcinoma. Actinic keratosis Here on the top outer edge of the ear is a palpably rough area, an actinic keratosis in one of the more common presentation sites for men. (In women, the ear is often protected from excess sunlight by the hair). Marjolins Ulcer lSCC arise in chronic ulcers, sinuses, and scars of various etiologies lBurns are most common cause Acantholytic SCC lFast growing tumor lOral cavity and conjunctiva may also be involved lAcantholysis with adenoid preliferation lSurgical excision is preferred treatment. Verrucous Carcinoma lSlow-growing lesion and very invasive lMay invade the bony structures around the tumor lBulbous rete ridges that are topped by an undulating keratinized mass. lExcision or Mohs Verrucous carcinoma can occur on the foot, in the groin, or in the mouth. It is a low grade tumor that seldom metastasizes. Note the destruction of normal structures in this verrucous carcinoma of the toe. Verrucous carcinoma of the groin. Note the destruction of the penis. Verrucous carcinoma is very low grade and has almost no atypia on histologic examination. Diagnosis is made by the extent of invasion. It is important to get a large, deep biopsy when one suspects this type of tumor. Bowens Disease lSCC in situ lStains for mucin is negative for Bowens but positive for Pagets lNo dyskeratosis in Pagets lWind blown pattern in histology lTinea circinata must be considered as well as Pagets Erythroplasia of Queyrat lBowens located on glans penis lTreat with 5-FU is effective because of the absence of follicles lResemble Zoons Balanitis Balanitis Plasmacellularis (ZOON) lZoons Balanitis is a condition found on the glans penis and/or inner surface of the prepuce of the uncircumcised, middle-aged to older male. lpresents most often as a solitary, glistening, red or cayenne pepper-colored, persistent plaque on the glans penis or inner surface of the prepuce of the uncircumcised male Balanitis Plasmacellularis lHistologically, the epidermis appears thinned, often showing an absence of the upper layers lThe upper dermis demonstrates a lichenoid infiltrate with copious plasma cells Balanitis Plasmacellularis lTreatments start with topical therapies. lMild topical steroids are the initial treatment of choice, however, recurrence upon their discontinuation is the rule. lCircumcision is curative in nearly all cases. Close follow-up is recommended. Pseudoepitheliomatous Keratotic and Macaceous Balanitis lRare condition lUlceration, cracking, and fissuring on surface of glans lPhimosis will develop in adult life lMany believe it to be a form of verrucous carcinoma lRequire Mohs or 5-FU Pagets Disease of the Nipple lUnilateral sharply defined eczema caused by epidermal metastases from underlying ductal adenocarcinoma of the breast lPresence of padget cells lCEA and apocrine epithelial antigen usually positive lBilateral lesions suggests neurodermatitis, contact, or nummular. Involvement of the epidermis by malignant adenocarcinoma cells. The cells are large with abundant clear cytoplasm and large anaplastic nuclei with prominent nucleoli. Extramammary Pagets lpresents clinically as an erythematous plaque, often several centimeters in dimension, and such lesions are sometimes pruritic. lDelay in diagnosis is common as many of these cases are erroneously treated for dermatitis or superficial fungus infection prior to the establishment of the real diagnosis. Low power view from one part of the biopsy.Low power view from one part of the biopsy. Medium power view of above. The cells are large and have a Medium power view of above. The cells are large and have a rather bland appearance in this area. Some are found singly in rather bland appearance in this area. Some are found singly in a a pagetoidpagetoid distribution, and others are in clusters. The distribution, and others are in clusters. The intervening keratinocytes are free of ervening keratinocytes are free of atypia. High power view of above. This solitary focus of High power view of above. This solitary focus of lumen production was found after examining lumen production was found after examining numerous sections. numerous sections. Composite high power . These cells are cytologically malignant. Some have vacuolated cytoplasm. Trabecular Carcinoma (Merkel Cell Carcinoma) lRapid growing nodule lHead and neck (44%) leg (28%) arm (16%) and buttock (9%) lRegion nodal metastases is 53% lDistant metastases is 75% Trabecular Carcinoma (Merkel Cell Carcinoma) lLocal recurrence 26% to 44% l5 Year survival rate 30% to 64% lPrognosis is worse than Melanoma lMohs excision, some recommended 3 cm margin Merkel cell carcinoma with formation of lobular structures in dermis and prominent lymphocytic infiltration Nevus Sebaceus (Organoid Nevus) lAKA Nevus Sebaceus of Jadassohn lPresent at birth, usually near vertex of scalp lBCC may develop from the lesion 5-10% of the time lDeletion of Patched gene has been identified and may be responsible for development of BCC Nevus Sebaceus (Organoid Nevus) lMay be associated with development of intracranial masses, seizure, MR, skeletal abnormalities, ocular lesions, hamartomas of the kidney lExcision recommended if possible. lPatient with BCC on scalp during the inspection. There are no large, anagen phase hair follicles in most of the field, and there are no fibrous tracks of the type that follow a telogen phase follicle. This is characteristic of nevus sebaceus of Jadassohn. The variety and degree of proliferation of follicular components varies from lesion to lesion. Sebaceous Neoplasms lSpectrum of sebaceous neoplasms lSebaceous Hyperplasia Sebaceous Adenoma Sebaceoma Sebaceous Epithelioma Sebaceous Carcinoma Sebaceous Hyperplasia lAKA senile sebaceous hyperplasia and senile sebaceous adenoma lProliferation of mature sebaceous glands lGerminative layer 1 cell thick lLobules may be grouped around a central dilated duct Sebaceous Adenoma lSebaceous adenoma presents as a yellow circumscribed nodule located either on face or scalp. lHistologically sebaceous adenoma is a multilobulated tumour sharply demarcated from the surrounding tissue. lTwo types of cells are present in the lobules. lThe large mature sebaceous cells (sebocytes) are present at the centre. Smaller,undifferentiated basaloid cells in the periphery Sebaceous Adenoma lProliferation of sebaceous glands lGerminative layer comprise to to 50% of lobules lRetains lobular architecture lThe cellular lobules contain ductal structures with holocrine secretion. Sometimes lobules contain cystic spaces in the center due to disintegration of mature sebaceous cells. lVery low power (direct scan of glass slide) view. The tumor communicates with the surface in multiple points, and holocrine secretion is prominent along the surface. Low power view. Note the holocrine secretion along the surface. lHigh power view. Most of the tumor cells have well- differentiated sebaceous cytology. Sebaceous Epithelioma Sebaceoma lCircumscribed, symmetric lobules lLarger lobules extending into deeper dermis l50% germinative cells lSame morphol
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