Vulva, vagina & female urethra

Table 1: Features distinguishing vulvar nevi, melanosis and melanoma (adapted from J Am Acad Dermatol 2014;71:1241)

Reassuring features suggestive of a benign process	Concerning features for possible malignancy
Clinical
Patient age < 50 years, symmetric, diameter < 1 cm, uniform pigmentation, regular border, macular or papular, associated with genodermatoses	Patient age > 50 years, irregular coloration, asymmetric borders, diameter > 7 mm, elevated lesion, associated bleeding, pruritus or discharge
Dermoscopy
Globular, cobblestone, ring-like, reticular-like, homogeneous, parallel or mixed	Gray, white or blue color plus structureless zones, irregular dots or globules, atypical vessels
Reflectance confocal microscopy
Hyperrefractive cells around the papillae, ringed or draped polycyclic papillae, sparse dendritic cells	Increased cellularity, atypical cells, disturbed architecture

Table 2: Clinical morphology for pigmented vulvar lesions (adapted from Dermatol Ther 2010;23:449)

Papules and macules	Patches and plaques
Pigmented nevi (nevocellular nevi)	Physiologic hyperpigmentation
Dysplastic nevi	Postinflammatory hyperpigmentation
Malignant melanoma	Vulvar melanosis (vulvar lentiginosis)
Anogenital warts	Acanthosis nigricans
Vulvar intraepithelial neoplasia
Seborrheic keratosis
Basal cell carcinoma
Angiokeratoma

Table 3: Genodermatosis syndromes with vulvar melanosis (adapted from J Am Acad Dermatol 2014;71:1241)

Genodermatosis	Areas affected	Key features	Genetic mutation
Peutz-Jeghers syndrome	Lips, oral mucosa, nostrils, perianal area, hands and feet, genitalia	Hamartomatous polyps in the gastrointestinal tract (GIT); increased risk of breast, ovarian, pancreatic and GIT cancers	STK11
Carney complex	Lips, eyelids, conjunctiva, oral and genital mucosa	Myxomas (especially cardiac), endocrine tumors, psammomatous melanotic schwannoma, blue nevus	PRKAR1A
Multiple lentigines syndrome (LEOPARD syndrome)	Face, neck, trunk, genitalia	ECG abnormalities, ocular hypertelorism, pulmonic stenosis, abnormal genitalia, delayed growth, sensorineural deafness	PTPN11, RAF1, BRAF
Bannayan-Riley-Ruvalcaba syndrome	Face, genitalia	Macrocephaly, intestinal hamartomatous polyposis, lipomas, hemangiomas, intellectual and motor deficiencies, joint hyperextensibility, pectus excavatum, scoliosis	PTEN
Dowling-Degos syndrome	Axillae, groin, flexural folds, neck, genitalia	Reticulated hyperpigmentation, follicular papules, comedone-like lesions, perioral scar, hypopigmented or erythematous macules	KRT5

Table 4: Clinical and histologic differences between atypical genital nevus and vulvar melanoma
(adapted from Hoang: Melanocytic Lesions - A Case Based Approach, 1st Edition, 2014)

Proposed diagnosis	Atypical genital nevus of special anatomic site	Vulvar melanoma
Age	Premenopausal, young adult	Postmenopausal
Size	< 1 cm	> 1 cm
Delineation	Well circumscribed	Infiltrative
Symmetry	Present	Absent
Lateral extension of junctional component	Focal	Present
Lentiginous junctional component	Focal	Present
Junctional nests	Dyscohesive	Confluent
Retraction artefact	Present	Absent
Ulceration	Absent or due to trauma	Often present
Pagetoid upward spread	Focal, central, inconspicuous	Prominent
Cytologic atypia	Superficial, mild - moderate	Deep, moderate to severe
Dermal mitosis	Rare and superficial	Conspicuous, atypical, deep
Dermal maturation	Present	Absent
Melanin pigmentation	Coarse, uniform	Fine, irregular
Dermal fibrosis	Broad zone of superficial coarse dermal fibrosis	Regression type

Contributed by José Alberto Fonseca Moutinho, M.D.



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Contributed by Emory University School of Medicine

A 19 year old patient presented with a 1.2 cm dark brown to black papule with irregular borders on the left labia minora. Histologic examination showed nearly contiguous large oval nests with striking atypia, nearly obscuring the dermal epidermal junction. What would be the most concerning feature?

1. Adnexal spread
2. Dense fibrosis in papillary dermis
3. Lack of a mature dermal component
4. Pagetoid spread to granular layer in the center of the lesion

C. Lack of a mature dermal component. Atypical melanocytic nevus of genital type is usually a symmetric, well delineated lesion that can be associated with a common dermal component. Answers B and D are incorrect because atypical melanocytic nevus is characterized by dense fibrosis in the papillary dermis and focal pagetoid spread to the granular layer is usually present in the center of the lesion. Answer A is incorrect because adnexal spread and mature dermal component are also common features of atypical melanocytic nevus of genital type. Hence, the lack of maturation is the most concerning feature in this patient.

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Reference: Pigmented vulvar lesions

A 65 year old woman presented with a dark brown macule with irregular borders measuring 0.7 cm in the largest dimension, located at the mucosal junction of right labia majora (left image). Shave biopsy is shown (right image). Which of the following is true for this patient's condition?

1. HPV test will help to diagnose the lesion
2. p63 will highlight neoplastic cells
3. PRAME will highlight an in situ component
4. SOX10 may demonstrate slight increase in density of the isolated melanocytes at the dermal - epidermal junction

D. SOX10 may demonstrate a slight increase in density of isolated melanocytes at the dermal epidermal junction. Melanocytic macules with hyperpigmentation in basal keratinocytes may show a slight increase in melanocytes at the dermal - epidermal junction, with no confluence. Hence, SOX10 may demonstrate a slight increase in density of isolated melanocytes at the dermal - epidermal junction. Answer C is incorrect because PRAME is typically positive in melanoma. Answer B is incorrect because p63 is positive in squamous epithelial cells. Answer A is incorrect because pigmented HPV associated lesions demonstrate koilocytic changes, hyperplastic squamous epithelium and a lack of basal layer maturation.

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Reference: Pigmented vulvar lesions