Sec 22 Melanocytic Tumors Flashcards
Presence of melanocytic cells in epidermal nests, defined as three or more melanocytic cells in direct contact (also known as thèque), within the dermis, or in other tissues
Melanocytic neoplasia
Increased melanocytes confined to the basal layer of the epidermis
Melanocytic hyperplasia
Represent a developmental abnormality of normal melanocytic development due to a mutation that occurs in a progenitor cell that results in the abnormal extensive accumulation of melanocytic cells along migration pathways during normal development
Congenital Nevomelanocytic Nevus
Present at birth but large variety appear between 1 month and 2 years of life, grows relatively proportionally, associated with loss of pigmentation, halo depigmentation, and regression
Congenital Nevomelanocytic Nevus
Congenital Nevomelanocytic Nevus: Small Medium Large Giant
Small <1.5 cm
Medium 1.5–19.9 cm
Large (≥20 cm)
Giant - as large as the patient’s palm if on the head and neck (and twice that area for other anatomic sites), 30% of the body surface, or 900 cm2 in adults
Have smooth, regular, and sharply demarcated border, and skin markings distort the skin surface; some relatively hairless; some have coarse, long, darkly pigmented; have a smooth, pebbly, rugose, verrucous, cerebriform, or grossly lobular surface
Congenital Nevomelanocytic Nevi
CNNs of the head, neck, or posterior midline, and/or the presence of multiple satellite lesions associated with large CNN may be complicated
Cranial and/or spinal leptomeningeal melanocytosis
Associated with giant CNN
Neurofibromatosis
Associated with the relatively sudden appearance of a dermal or subcutaneous nodule, very dark pigmentation, itching, pain, bleeding, or ulceration
Malignant degeneration of large CNNs
Histopathology: presence of nevomelanocytes in the epidermis as well-ordered thèques and/or nevomelanocytes in the dermis, which are present as sheets, nests, cords, and/ or single cells
Congenital Nevomelanocytic Nevus
Histopathology: appearance may be identical to typical acquired nevi, with nevomelanocytes in the epidermis as well-defined thèques and/or nevomelanocytes in the papillary dermis as sheets, cords, or nests; nevomelanocytes in the lower two-thirds of the reticular dermis and to be associated with appendageal and neurovascular structures
Nevomelanocytic type of large CNN
Histopathology: melanocytic elements take on the appearance of Wagner-Meissner corpuscles (lames foliacée), a palisaded arrangement of cells around a cellular mass of homogeneous material (Verocay body), and sheathing of nerves by neuroid tissue (neuroid tubes); may be responsible for lobulation and redundancy of tissue (pachydermatous or cerebriform appearance) due to the production of connective tissue elements such as reticulin, collagen, and sometimes mucinous stroma; may take on the appearance of a pigmented neurofibroma
Neuroid type of giant CNN
Histopathology: dermis may be infiltrated in whole or in part by nests or sheets of epithelioid cells and/or spindle cells; may have atypical cellular and architectural features, large epithelioid cells may comprise superficial papillary zones of the nevus, and smaller nevomelanocytes may appear in the same lesion in deeper zones of the reticular dermis, with a grenz zone separating the two elements
Spindle cell and/or epithelioid cell type of giant CNN
Histopathology: appearance may be that of a giant blue nevus, or the lesion may have elements of blue nevus (either common or cellular type) with heavily pigmented spindle-shaped melanocytic cells alone or intermixed with nevomelanocytes in the reticular dermis or deeper tissues
Blue (dermal melanocytic) type of giant CNN
Treatment goal in CNN
To remove as much of the nevus while preserving function and improving cosmetic appearance
Presents as a circumscribed macule/patch of tan background pigmentation (<1 cm to >10 cm in diameter) with features consistent with lentigo or café-au-lait macules including scattered, more darkly pigmented nevomelanocytic (or more hyperplastic) macular and/or papular elements
Nevus spilus
Associated with large varieties of nevus spilus
Multiple granular tumors Nevus flammeus Muscle atrophy Neurological disorders Phacomatosis pigmentokeratotica
Histopathology: consists of increased numbers of melanocytes in a lentiginous epidermal pattern
Electron microscopy: demonstrate melanin macroglobules
Nevus spilus - tan background pigmentation
Histopathology: demonstrate foci of increased melanocytic hyperplasia or melanocytic dysplasia (architectural disorder and variable cellular atypia)
Nevus spilus - flat, dark elements
Histopathology: usually contain collections of nevomelanocytes in the epidermis and/or dermis
Nevus spilus - raised elements
Develop after birth, slowly enlarge symmetrically, stabilize, and after a period of time may regress with majority develop during the 2nd-3rd decades of life
Common acquired nevomelanocytic nevi
Spontaneous and concurrent development of scattered nevomelanocytic nevi often similar in appearance
Eruptive nevi
Phenomenon of spontaneous development of a zone of depigmentation around a preexisting nevus, indicating the onset of involution and subsequent regression
Halo nevus Leukoderma acquisitum centrifugum Sutton’s nevus Leukopigmentary nevus Perinevoid vitiligo Perinevoid leukoderma
Lesions have a homogeneous surface and coloration pattern, round or oval shape, regular outlines, and relatively sharp borders; may be papillomatous, dome-shaped, pedunculated, or flat-topped and are usually skin-colored, pink, or brown
Common acquired nevomelanocytic nevi