bubble_chart Overview

Malignant tumors of the vulva are predominantly primary, accounting for about 4% of female genital tract tumors and 1-2% of all tumors in women. The vast majority of vulvar cancers are squamous cell carcinomas, making up approximately 95% of vulvar malignancies, with an average onset age of 50-60 years, though cases can occur before the age of 40. Adenocarcinomas are less common. Sometimes, vulvar cancer is confined to the epithelium, spreading within the epithelial layer, known as carcinoma in situ, also referred to as vulvar intraepithelial carcinoma. There are two types of intraepithelial carcinoma: ① squamous cell carcinoma in situ (also called Bowen's disease); ② eczematoid intraepithelial carcinoma (also known as Paget's disease). Additionally, there are basal cell carcinomas and malignant melanomas.

bubble_chart Pathological Changes

1. Location of growth: Approximately two-thirds of the lesions occur on the labia majora, and one-third occur on the labia minora, clitoris, or vulvar commissure. Most lesions develop in the anterior part of the vulva, while a minority occur in the perineum or the lateral aspects of the labia majora. Intraepithelial carcinoma primarily arises on the labia majora. Adenocarcinoma occurs in the paraurethral glands or Bartholin's glands.

2. Gross appearance: ① Squamous cell carcinoma may present as a simple ulcer, white lesion, subcutaneous mass, or polypoid lesion. In the early stages, the epithelial rete ridges infiltrate the stroma, gradually forming subcutaneous nodules. These nodules may ulcerate or shrink, leading to misdiagnosis as inflammation. In advanced stages, they develop into cauliflower-like growths or ulcers. ② Bowen's disease appears as a dark red, rough patch with well-defined but irregular borders, covered with crusts. Removal of the crusts reveals granulation tissue and exudate. Paget's disease exhibits eczematoid changes, appearing red and slightly raised, accompanied by white lesions or small granules. Sometimes, shallow ulcers and crusting are observed.

3. Microscopic features: ① Squamous cell carcinoma is mostly well-differentiated, often showing epithelial pearl formation or keratinization. However, lesions on the clitoris or vestibule tend to be less differentiated. Additionally, impaired nucleic acid synthesis may be observed in adjacent normal tissues, indicating the necessity of resecting the entire vulva when treating vulvar cancer. ② Bowen's disease displays hyperkeratosis, parakeratosis, acanthosis, disordered cell arrangement, and nuclei with deep staining and atypia. The epidermal basement membrane remains intact. Deeper in the epidermis, typical Paget's cells may be found—large, round, oval, or polygonal cells with clear, vacuolated cytoplasm. Although the basement membrane is intact, tumor cells often extend beyond the visible lesion margins. When diagnosing Paget's disease, it is essential to assess for underlying sweat gland carcinoma. ③ Adenocarcinoma shows glandular hyperplasia, multilayered epithelium, disordered arrangement, and nuclei with deep staining and atypia.

bubble_chart Clinical Manifestations

The main symptoms are nodules and lumps in the vulvar area, often accompanied by a history of pain or itching. Some patients present with vulvar ulcers that do not heal for a long time. Advanced-stage patients may also experience increased purulent or bloody discharge, dysuria, and other discomforts.

Clinical staging: Vulvar cancer can be clinically divided into four stages.

Stage I: All lesions are confined to the vulva, with a maximum diameter of 2 cm or less, and no suspicious metastasis to the inguinal lymph nodes.

Stage II: All lesions are confined to the vulva, with a maximum diameter exceeding 2 cm, and no suspicious metastasis to the inguinal lymph nodes.

Stage III: Lesions extend beyond the vulvar area, with no metastasis or suspicious metastasis to the inguinal lymph nodes.

Stage IV: Any of the following conditions applies:

① Fixed or ulcerated inguinal lymph nodes, clinically confirmed as metastatic;

② Lesions invade the rectum, bladder, or urethral mucosa, or the tumor has become fixed to the bone.

③ Distant metastasis or palpable deep pelvic lymph nodes.

bubble_chart Diagnosis

The diagnosis is primarily based on clinical symptoms and pathological biopsy of living tissue. Detailed observation should be conducted for vulvar lesions. If persistent ulcers, papule-like warts, or white lesions with unclear treatment effects are found, a biopsy should be performed. Except for very early stages that resemble benign sexually transmitted diseases and are difficult to diagnose, the diagnosis is generally not difficult. However, it should be differentiated from papilloma, vulvar condyloma, hyperplastic dystrophy, basal cell carcinoma, and Paget's disease. Biopsy is the only reliable method for differentiation. Taking a biopsy from the non-decolorized area after toluidine blue staining can yield more accurate diagnostic results. If necessary, multiple biopsies from different sites may be required for a final diagnosis.

bubble_chart Treatment Measures

1. Treatment principles: surgical treatment is the preferred method, followed by radiation therapy.

2. Surgical treatment: Surgery is the primary treatment for vulvar cancer. Vulvar cancer rarely invades deep tissues, so even with larger tumors, curative or palliative surgical resection remains possible. Due to the growth characteristics of vulvar cancer—local infiltration is often extensive and may occur at multiple sites, with a high tendency for lymph node metastasis—the standard surgical approach for vulvar cancer includes radical vulvectomy and bilateral inguinal lymph node dissection. As for pelvic lymph node dissection, since pelvic lymph node metastasis only occurs after metastasis to the femoral lymph nodes, pelvic lymph node dissection is only performed in patients with femoral lymph node metastasis. Bowen's disease may be treated with simple vulvectomy, while Paget's disease often requires radical vulvectomy. For adenocarcinoma of the Bartholin's gland, partial vaginectomy and levator ani muscle resection may be necessary depending on the situation.

3. Radiation therapy: In recent years, improvements in radiation therapy equipment and techniques have reduced its side effects. Radiation therapy is now used for vulvar cancer, particularly in patients with contraindications to surgery or those with advanced-stage disease unsuitable for surgery, showing certain therapeutic efficacy.

bubble_chart Metastasis and Spread

The mode of spread is primarily local infiltration and lymphatic metastasis, with hematogenous spread being extremely rare.

1. Infiltration: The tumor in the vulva gradually enlarges but rarely invades the muscular fascia or adjacent structures such as the periosteum of the pubis. Once the vagina is involved, it quickly spreads to the levator ani muscle, rectum, urethral orifice, or bladder.

2. Lymphatic metastasis: The vulva has abundant lymphatic vessels, and the lymphatic capillary networks in the vulva are interconnected. Therefore, cancer from one side of the vulva can spread via bilateral lymphatic vessels, initially metastasizing to the superficial inguinal lymph nodes, then to the femoral lymph nodes located below the inguinal region, and from there to the external iliac, obturator, and internal iliac lymph nodes in the pelvis, ultimately reaching the para-aortic lymph nodes and left supraclavicular lymph nodes. Carcinoma of the clitoris may bypass the superficial inguinal lymph nodes and directly spread to the femoral lymph nodes, while carcinoma of the posterior vulva and lower vagina may skip the superficial inguinal lymph nodes and directly metastasize to the pelvic lymph nodes.