Vulva Pathology Study Guide

Vulva

The skin and mucosa of the female genitalia external to the hymen, including the labia majora, labia minora, mons pubis, and vestibule, comprise the vulva.

The vulva is lined by squamous epithelium.

Vulva pathology includes:

Bartholin cyst
Condyloma
Lichen sclerosis
Lichen simplex chronicus
Vulvar carcinoma
Extramammary Paget disease

Vulva. Labeled image of a vulva, showing external and internal views OpenStax College - Anatomy & Physiology, Connexions Web site. - Not altered. CC BY 3.0
Vulva. Visible parts of the clitoris and the parts lying under the skin Sciencia58 - Not altered. CC0

Bartholin’s Gland Cyst

A fluid-filled lump near the vaginal opening is known as a Bartholin’s gland cyst.

Bartholin’s gland cysts can be painful if infected.

Each side of the vaginal canal has one Bartholin’s gland, which produces mucus-like fluid that drains into the lower vestibule via ducts.

Bartholin’s gland cyst develops as a result of inflammation and obstruction of the gland and is most common in women of reproductive age.

Bartholin’s gland cysts appear as a single, painful cystic lesion in the lower vestibule near the vaginal canal.

Bartholin Cyst. Bartholin's cyst of the right side Medimage. not altered. CC BY-SA 3.0
Bartholin Cyst. Newly Outflow Tract of Bartholin Duct Cyst After RFA Fistulization Surgery. A. 5 days postoperation. B. 30 days postoperation. C. 60 days postoperation. D. 90 days postoperation. Application of Radiofrequency Ablation Fistulization to Treat Bartholin Duct Cysts and Gland Abscesses: A Minimally Invasive Surgical Technique Xiao Shan Chai1 , Yong Jing Zhang1 , and Guang Shi Tao1 1Affiliation not available May 5, 2020. Not altered. CC.
Procedure with CO2 laser in the treatment of a Bartholin cyst. (A) Exposure of the cyst to the right, with 2% xylocaine infiltration. (B) Incision with the focused laser beam over the cyst. (C) Opening of the capsule with complete drainage of the content. (D) Repair of the borders with exposure of the internal surface of the capsule. (E) Vaporization of the internal capsule with unfocussed laser beam. Treatment of Bartholin gland cyst with CO2laser. Speck NM, Boechat KP, Santos GM, Ribalta JC - Einstein (São Paulo, Brazil) (2016 Jan-Mar). Not Altered. CC.

Condyloma

Condylomas are genital warts.

Condylomas are often large, soft, noncancerous growths that can form on the skin on the outside or inside of the vagina or anus, or inside the cervix.

Condylomas are typically caused by low risk HPV types which include:

HPV 6
HPV 11

Histologically, koilocytes, the hallmark of HPV-infected cells, are present in HPV-associated condylomas (technically known as condyloma acuminata).

Condyloma latum is known as secondary syphilis, and is a less frequent cause of condylomas.

Both condyloma acuminatum and condyloma latum are spread sexually.

Pigmented Seborrehic Keratosis of the Vulva. This was a single SK amid multiple condylomata acuminata removed at the same time. Ed Uthman. Not altered. CC BY 2.0
Condyloma. A woman afflicted with genital warts, also called condylomata acuminata, around the anus. These warts are caused by human papillomavirus (HPV). SOA-AIDS Amsterdam. not altered. CC BY-SA 3.0
Pathologic finding of giant condyloma acuminatum of anus. (A), (B) The epithelium shows considerable superficial hyperkeratosis with koilocytosis (hematoxylin and eosin staining magnification ×100). (C) There is distinct perinuclear clear vacuolization of the prickle cells (koilocytosis) (hematoxylin and eosin staining; magnification ×200). (D) Koilocytosis characteristic of HPV infection (hematoxylin and eosin; magnification ×400).Giant anal condyloma (giant condyloma acuminatum of anus) after allogeneic bone marrow transplantation associated with human papillomavirus: a case report. Hyun JS, Kim GB, Choi BS, Kim MS, Park SG - Journal of medical case reports (2015).Not Altered. CC.

Lichen Sclerosus

Lichen sclerosus is a localized rash lesion that often itches and is dry.

Lichen sclerosus manifests as a leukoplakia, a white area with vulvar skin that resembles parchment paper.

Postmenopausal women are most frequently affected by this lichen sclerosus, which may have an autoimmune cause.

The histology of lichen sclerosis shows thickening of the epidermis and fibrosis or sclerosus of the dermis are features of lichen sclerosus.

Although lichen sclerosus is regarded as benign, there is an increased risk of squamous cell carcinoma (SCC).

Lichen Sclerosus, Atrophic. Ed Uthman. Not altered. CC BY 2.0
Lichen Sclerosus, Hypertrophic. Ed Uthman. Not altered. CC BY 2.0
Lichen Sclerosus, Hypertrophic. Ed Uthman. Not altered. CC BY 2.0
Syringoma and Lichen Sclerosus, Vulva. Ed Uthman. Not altered. CC BY 2.0
Syringoma and Lichen Sclerosus, Vulva. Ed Uthman. Not altered. CC BY 2.0
Lichen Sclerosus. Micrograph of extragenital lichen sclerosus: epidermal atrophy, follicular plugging and basal vacuolization, and sclerosis with initial homogenization of collagen in the dermis. Anna Jędrowiak, Aleksandra Kobusiewicz, Ewa Trznadel-Grodzka, Andrzej Kaszuba - Not altered. CC BY 4.0
Lichen Sclerosis. Micrograph of extragenital lichen sclerosus: epidermal atrophy, follicular plugging and basal vacuolization, and sclerosis with initial homogenization of collagen in the dermis.[23] Anna Jędrowiak, Aleksandra Kobusiewicz, Ewa Trznadel-Grodzka, Andrzej Kaszuba - (2018). "Dermoscopic findings in extragenital lichen sclerosus". Our Dermatology Online 9 (2): 197–199. DOI:10. Not altered. CC BY 4.0
Lichen Sclerosus. High magnification micrograph of lichen sclerosus, also lichen sclerosus et atrophicus. H&E stain. Related images Low mag. Intermed. mag. High mag. Very high mag. Nephron. Not altered. CC BY-SA 3.0
(A, B) Decrease of elastic fibers in lichen sclerosus et atrophicus (elastic von Gieson, ×40, 100), (C, D) Normal limits of elastic fibers in morphea (elastic von Gieson, ×40, 100). Yasar S, Mumcuoglu CT, Serdar ZA, Gunes P. A case of lichen sclerosus et atrophicus accompanying bullous morphea. Ann Dermatol. 2011;23(Suppl 3):S354-S359. doi:10.5021/ad.2011.23.S3.S354. Not altered. CC.
A: Three months after the flap surgery, the hairy darker pigmented skin of the flap (arrowhead) is normal corresponding to its donor site (arrowhead). The hairless vulvar skin exhibits clinical signs of lichen sclerosus. B: Eight years later, the lichen of the hairless vulva skin had progressed involving parts of the anus as well. The hairy skin island (arrow) is completely diseased, whereas the skin of the donor region (arrowhead) remains normal. Inset: Microscopic features of LS on the hairy skin of the cutaneous flap: thinning of the epidermis with loss of the rete ridges and dermal hyalinisation (*) involving the superficial reticular dermis, accompanied by scattered infiltration of lymphocytes. Anogenital lichen sclerosus: Change of tissue position as pathogenetic factor. Gynecologic Oncology Reports. Not Altered. CC.

Lichen Simplex Chronicus

Lichen simplex chronicus is hyperplasia of the vulvar squamous epithelium.

Lichen simplex chronicus manifests as leukoplakia with thick, leathery vulvar skin and chronic itching and scratching.

Lichen simplex chronicus is regarded as benign.

There is no increased incidence of squamous cell cancer (SCC) with lichen simplex chronicus.

Lichen Simplex Chronicus. Very low magnification micrograph of lichen simplex chronicus, abbreviated LSC. H&E stain. Skin biopsy. Features: Acanthosis (epidermal thickening). Hyperkeratosis (thickened stratum corneum). Parakeratosis = retention of nuclei in the stratum corneum. +/- Spongiosis (epidermal intercellular edema -- cells appear to have a clear halo around 'em). Nephron. Not altered. CC BY-SA 3.0

Vulvar Carcinoma

On the external surface of the female genitalia, vulvar carcinoma is a particular form of cancer.

The urethra, vagina, clitoris, and labia are all covered by the vulva, which is the region of skin.

Vulvar carcinoma is rare, making up just a small portion of female genital cancers that manifest as leukoplakia.

The population of females effected by vulvar carcinoma tend to be 70-years-old on average.

To differentiate vulvar carcinoma from other kinds of leukoplakia, a biopsy may be necessary.

The etiology of vulvar carcinoma may be:

HPV related
non-HPV related

Risk factors for vulvar carcinoma are:

Increased number of sexual partners
Initial age of sexual contact

Vulvar carcinoma develops from vulvar intraepithelial neoplasia, a dysplastic precursor disease characterized histologically by altered koilocytic differentiation, disorganized cellular maturation, nuclear atypia, and elevated mitotic activity.

The most frequent cause of non-HPV associated vulvar cancer is chronic lichen sclerosus.

Vulvar Carcinoma. Stage 4A vulvar cancer Cancer Research UK - Not altered. CC BY-SA 4.0
Vulvar Carcinoma. Stage 3 vulvar cancer Cancer Research UK - Not altered. CC BY-SA 4.0
Vulvar Carcinoma. Stage 2 vulvar cancer Cancer Research UK -Not altered. CC BY-SA 4.0
Vulvar Carcinoma. Stage 1A and 1B vulvar cancer Cancer Research UK Not altered. CC BY-SA 4.0
Vulvar Carcinoma. Micrograph of vulvar intraepithelial neoplasia III (VIN III). H&E stain. See also Image:Vulvar_intraepithelial_neoplasia3_2.jpg - low magnification micrograph of VIN III Nephron. Not altered. CC BY-SA 3.0
Right minor labium infiltrated by LEL Carcinoma, H&E 100×. Lymphoepithelioma-like carcinoma of the vulva, an underrecognized entity? Case report with a single inguinal micrometastasis detected by sentinel node technique. Bösmüller H, Haitchi-Petnehazy S, Gruber C, Roithmeier F, Stummvoll W, Webersinke G - Diagnostic pathology (2011). Not altered. CC.
Immunohistochemistry of HMGA2 in vulva tumors. (A) HMGA2 immunostaining of case 5, an SCC (x50, magnification). (B) HMGA2 immunostaining of case 20, a vulvar MM (x50, magnification). Inset shows immunohistochemistry at a higher magnification (x200). SCC, squamous cell carcinoma; MM, malignant melanoma. HMGA2 expression pattern and TERT mutations in tumors of the vulva. Agostini A, Panagopoulos I, Andersen HK, Johannesen LE, Davidson B, Tropé CG, Heim S, Micci F - Oncology reports (2015). Not Altered. CC.
Macroscopic and microscopy findings at diagnosis: (a): vulvar bleeding vegetants lesions; (b): micropapillary pattern; (c): acantholysis and mitosis. Early invasive vulvar squamous cell carcinoma arising in a woman with vulvar pemphigus vulgaris and systemic lupus erythematosus. Bifulco G, Mandato VD, Piccoli R, Giampaolino P, Mignogna C, Mignogna MD, Costagliola L, Nappi C - BMC cancer (2010). Not Altered. CC.

Extramammary Paget Disease

Extramammary Paget disease is characterized by erythematous, itchy, and ulcerated vulvar skin, which is caused by malignant epithelial cells in the vulva’s epidermis.

In addition to this, extramammary Paget disease also represents carcinoma in situ, usually with no underlying carcinoma.

Note that mammary Paget disease effects the breast.

Extramammary (away from the breast) Paget disease is does not involve the breast.

Melanoma is on the differential for extramammary Paget disease, and special stains must be ordered differentiate the two.

Paget cells are PAS +, keratin +, and S100 -.

Melanoma is PAS -, keratin -, and S100 +.

Extramammary Paget Disease. H&E stained micrograph of Extramammary Paget's disease, showing Paget cells infiltrating the epidermis Nephron. Not altered. CC BY-SA 3.0
Extramammary Paget Disease. High magnification micrograph of extramammary Paget's disease, abbreviated EMPD. Vulvar biopsy. H&E stain. The images show the characteristic large cells with clear cytoplasm in the epidermis. The H&E slide is not definitive; the clear cells may represent: Benign Toker cell hyperplasia. Malignant melanoma. Bowen's disease. The definitive diagnosis is determined with immunohistochemical stains (S-100 -ve and HMB-45 -ve (both typically +ve in melanoma), CK7 +ve, CEA +ve (-ve in Bowen's disease, -ve in Toker cells). The histologic appearance of Paget's disease of the breast is identical to extramammary Paget's disease, i.e. it is not possible to say this is extramammary from the image alone. Related images Low mag. Intermed mag. High mag. Very high mag. Very high mag. Nephron. Not altered. CC BY-SA 3.0
Clinical appearance and histological findings of the biopsy specimen. Eczematoid nodules on the vulva was observed (A). Typical full thickness atypia (B), cellular atypia combined with basal Paget cells (C), and pagetoid distribution of Paget cells were observed (D) (H&E: ×100 and ×400). Primary Extramammary Paget's Disease Combined with Bowen's Disease in Vulva. Kim SA, Kwon JI, Jung HR, Lee KS, Cho JW - Annals of dermatology (2011). Not Altered. CC.