Vulvar cancer pathophysiology

Vulvar cancer Microchapters
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Overview

Vulvar cancer, a malignant invasive growth in the vulva, accounts for about 4 % of all gynecological cancers and typically affects women in later life. It is estimated that in the United States in 2006 about 3,740 new cases will be diagnosed and about 880 women will die as a result of vulvar cancer. Vulvar carcinoma is separated from vulvar intraepithelial neoplasia (VIN), a non-invasive lesion of the epithelium that can progress via carcinoma-in-situ to squamous cell cancer, and from Paget disease of the vulva.

Types

Table 31-1. Histologic Subtypes of Vulvar Cancer

1. Vulvar carcinomas

• Squamous cell carcinoma
• Basal cell carcinoma
• Vulvar Paget disease
• Adenocarcinoma
• Transitional cell carcinoma
• Verrucous carcinoma
• Merkel cell tumors
• Verrucous carcinoma

2. Vulvar malignant melanoma

3. Vulvar sarcoma

• Leiomyosarcoma
• Malignant fibrous histiocytoma
• Epithelial sarcoma

Melanoma

About 5% of vulvar malignancy is caused by melanoma of the vulva. Such melanoma behaves like melanoma in other locations and may affect a much younger population. Contrary to squamous carcinoma, melanoma has a high risk of metastasis.

Basal cell carcinoma

Basal cell carcinoma affects about 1-2% of vulvar cancer is a slowly growing lesion and affects the elderly. Its behavior is similar to basal cell carcinoma in other locations that is it tends to grow locally with a low potential of deep invasion or metastasis.

Other lesions

Vulvar cancer can be caused by other lesions such as adenocarcinoma or sarcoma.

Vulvar carcinomas Subtype	Features on Gross Pathology	Features on Histopathological Microscopic Analysis
Squamous cell carcinoma of vulva	Most lesions originate in the labia, primarily the labia majora. Other areas affected are the clitoris, and fourchette, and the local glands. unifocal	Eosinophilia. Extra large nuclei/bizarre nuclei. Inflammation (lymphocytes, plasma cells). Long rete ridges. Numerous beeds/blobs of epithelial cells that seem unlikely to be rete ridges.
Basal cell carcinoma	Tan/reddish brown color Sharp borders Well-demarcated, dome-shaped papular/verruccous lesion	Basaloid cells - similar in appearance to basal cells: Moderate blue/grey cytoplasm. Dark ovoid/ellipsoid nucleus with uniform chromatin. Palisading of cells at the edge of the cell nests. Artefactual separation of cells (forming the nests) from the underlying stroma - key feature. Surrounded by blue (myxoid) stroma - key feature.
Vulvar melanoma	Brown/black color, but may include reddish brown or white Hyperkeratotic, diffused borders with no distinct demarcation Irregular and elevated	Epidermal acanthosis and hyperkeratosis (most characteristic feature) Malignant melanocytes spread along the basal layer Cells arranged in lentiginous and dycohesive pattern along the dermoepidermal junction May be any of round, epithelioid, spindle, or oval cells May have perineural or endoneural invasion
vulvar sarcoma	Brown/black color, but may include reddish brown or white Hyperkeratotic, diffused borders with no distinct demarcation Irregular and elevated	Epidermal atrophy and flattening and prominent dermal invasion (most charactersitic feature) Large, pleomorphic cells Cells arranged in lentiginous and dycohesive pattern along the dermoepidermal junction Preservation of retiform epidermis May be any of round, epithelioid, spindle, or oval cells Evidence of actinic damage of the dermal matrix May have perineural or endoneural invasion Positivity for CD133+ and CD34+
Non-cutaneous melanoma	Variable morphology depending on location of melanoma	Histopathologically similar to other subtypes of melanoma
Desmoplastic/Spindle cell melanoma	Skin colored and morphologically resembles scar tissue	Dermal, fibrotic nodule Ill-defined, variable spindle cells with irregular contours and stromal desmoplasia Highly infiltrative pattern Appearance of sclerotic collagen fibers Nuclear hyperchromasia Appearance of lymphoid aggregates Solar elastosis Involvement of endoneurium and perineurium (neurotropism) Possibly evidence of other melanoma subtypes (co-existing tumors, especially lentiginous melanoma)"
Nevoid melanoma	Morphologically similar to a melanocytic nevus	Dermal mitosis Hypercellular and monomorphous-appearing dermal melanocytes that have a characteristic sheet-like appearance Evidence of cytologic atypia (nuclear enlargement, pleomorphism, irregular nuclear membrane, hyperchromasia) Irregular basal infiltration Evidence of angiotropism
Spitzoid melanocytic neoplasm	Morphologically similar to a Spitz nevus	Appearance of melanocytic proliferation along with features of Spitz tumors (small diametes, well-demarcated, symmetric lesion with no ulceration, epidermal effacement, dermal mitosis, or involvement of the subcutaneous fat) May have features that are not typically characteristic of Spitz tumors (ulceration, poor demarcation) Vertically oriented spindled melanocytes Clefts between junctional melanocytes
Angiotropic melanoma	No gross morphological features that distinguish angiotropic melanoma from other subtypes of melanoma	Melanoma cells in close proximity to abluminal surfaces of blood and/or lymphatic channels No invasion within the vascular lamina itself
Blue nevus-like melanoma	Morphologically similar to a blue nevus	Asymmetric nodular/multinodular appearance Aggregates of melaninized, atpical spindle cells
Composite melanoma	Features of more than one subtype on gross pathology	Features of more than one subtype on microscopic analysis May be characterized by one of the following: Collision tumor: Collision of melanoma and another nearby malignant tumor Colonization: Colonization of melanocytes in a tumor Combined: Two distinct tumors appear to have mixed features of the melanoma and the other tumor Biphenotypic: One tumor that has features of melanoma and another epithelial malignancy