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Tumors of the Vulva

Pathology

Vulvar cancer is a relatively rare malignancy, accounting for approximately 3 to 5% of cancers of the female genital tract. In Italy, about 1,200 new cases are diagnosed each year, most of them in postmenopausal women. Incidence increases with age, with around 90% of diagnoses occurring after the age of 60.

The vulva is the external portion of the female genital system and includes the labia majora, labia minora, vestibule (vaginal opening), clitoris, and perineum, the area of skin between the vagina and anus. Although vulvar cancer most commonly affects the labia majora, it can develop in any region of the vulva.

Vulvar cancer typically develops slowly over many years and is often preceded by pre-invasive lesions, which are cellular changes that are not yet malignant but may progress to cancer over time. These lesions are frequently associated with human papillomavirus (HPV) infection or with chronic vulvar dermatoses, such as lichen sclerosus.

Early detection is essential for a favorable prognosis. When identified at an early stage, vulvar cancer can often be successfully treated, with approximately 3 out of 4 women showing no signs of disease five years after diagnosis.

Types

  • Squamous cell carcinoma (85–90% of cases): arises from the squamous cells lining the vulva.
  • Malignant melanoma (2–5% of cases): a pigmented tumor resembling a dark mole.
  • Adenocarcinoma (1–3% of cases): develops from Bartholin’s glands.
  • Other rare tumors: include sarcomas and Paget’s carcinoma.

Symptoms

In the early stages, the tumor may be asymptomatic or mistaken for benign conditions. Initially, it may present as skin changes, which can appear thinner, redder, or darker than the surrounding tissue.

Over time, a white or red lesion with an irregular surface may develop, sometimes accompanied by itching, pain, burning, or small non-healing wounds.

Risk Factors

This type of neoplasm usually arises from precancerous lesions associated with human papillomavirus (HPV) infection or chronic inflammatory conditions, such as genital lichen sclerosus. The main known risk factors include:

  • Age (over 60 years)
  • Human papillomavirus (HPV) infection
  • Cigarette smoking
  • Presence of lichen sclerosus or other chronic inflammatory conditions (for example, Paget’s disease)

Additional factors that may increase the risk include:

  • Weakened immune system
  • Presence of precancerous lesions (dysplasia) or tumors in other sites, such as the cervix, vagina, or anus
  • Chronic granulomatous disease

Diagnosis and examination

The diagnostic pathway for vulvar cancer begins with a specialist gynecological examination, during which the physician collects the patient’s personal and family history, evaluates symptoms, and performs a careful inspection of the genital and inguinal regions, along with a complete gynecological examination.

If a suspicious lesion is identified, a biopsy is performed. This involves taking a small tissue sample for microscopic analysis and is essential to confirm or exclude the presence of cancer with certainty.

In some cases, particularly when the lesion is small or difficult to detect, the specialist may perform vulvoscopy, an examination that allows a more detailed visualization of the affected area and helps guide tissue sampling.

To assess the extent of the disease and possible involvement of the inguinal lymph nodes, imaging studies may be used, including:

  • Magnetic Resonance Imaging (MRI)
  • PET/CT
  • Computed Tomography (CT)

Early diagnosis and timely treatment are crucial, as identifying the disease at an early stage allows for more effective and targeted therapies.ease is identified, the more effective and targeted the available treatments can be.

Staging

Vulvar cancer is classified into four stages according to the criteria established by the International Federation of Gynecology and Obstetrics (FIGO):

  • Stage I: The tumor is confined to the vulva or perineum and has not spread to the lymph nodes.
  • Stage II: The tumor extends to nearby structures such as the lower urethra, vagina, or anus, without lymph node involvement.
  • Stage III: The disease involves inguinal or femoral lymph nodes and may also invade surrounding tissues.
  • Stage IV: The tumor is more advanced, may infiltrate deeper structures such as the bladder or rectum, attach to the pelvic bone, or give rise to distant metastases.

This classification is essential for determining the extent of the disease and guiding the selection of the most appropriate treatment strategy.

Therapies

To select the most appropriate treatment for this condition, it is essential to consider the patient’s age, pathological features, type of disease, as well as the location and extent of the tumor.

Pre-cancerous Lesions

In cases of precancerous lesions, a conservative approach is often sufficient. Treatment may include laser surgery, which allows for the removal or vaporization of altered cells while preserving surrounding healthy tissue.

Surgical Intervention

In cases of invasive vulvar cancer, surgery is the main treatment and serves both a therapeutic and symptomatic purpose. The extent of the procedure depends on the size and spread of the tumor. In many cases, the inguinal lymph nodes must also be removed, as they are the primary route of early metastatic spread.

In particular:

  • The sentinel lymph node is the first lymph node to receive lymphatic drainage from the tumor site. Its removal allows assessment of whether the cancer has spread, without the need to remove all regional lymph nodes, thereby reducing the risk of complications such as lymphedema.
  • Depending on the stage of the disease and the location of the tumor, it may be necessary to remove all inguinal lymph nodes, either on one side (unilateral) or both sides (bilateral).

Post-surgical Reconstruction

After a vulvectomy (partial or total removal of the vulva), vulvar reconstruction is of great importance, both for psychological well-being and for improving quality of life.

When a large amount of tissue has been removed, the involvement of a plastic surgeon can help achieve a more functional and aesthetically appropriate reconstruction.

Radiotherapy, Chemotherapy and Other Therapies

In some cases, surgery is combined with radiotherapy or chemotherapy, depending on the stage of the disease.

In addition, new targeted therapies, designed to act on specific molecular targets in cancer cells, and immunotherapy, which aims to stimulate the immune system against the tumor, are currently under investigation.

At present, these approaches are mainly available in experimental settings and may broaden future treatment options.

Ongoing Support

At the Candiolo Cancer Institute, we provide continuous support before, during, and after treatment, ensuring that each patient is guided and cared for throughout every stage of their treatment journey and recovery.

Psychological Support

A cancer diagnosis affects not only physical health but also emotional and psychological well-being. Coping with cancer is often a profoundly challenging experience that can give rise to feelings of anxiety, fear, anger, and depression.

At the Candiolo Cancer Institute, psychological support is an integral part of the care pathway. Alongside advanced medical treatments, patients have access to qualified psycho-oncological support designed to help them navigate the emotional challenges associated with diagnosis, treatment, and recovery.

Patients may also participate in psychological support groups, providing an opportunity to share experiences, receive mutual support, and connect with others who are facing or have faced similar challenges.

Management of Emergencies and Treatment-Related Side Effects

Cancer patients often require ongoing medical support throughout their treatment journey. When symptoms arise, whether related to the disease itself or to treatment-related side effects, it is important that they can promptly access specialist advice through a dedicated fast-track pathway.

For this reason, the Candiolo Cancer Institute provides a dedicated support service Monday through Friday, from 8:00 a.m. to 5:00 p.m. Patients can contact the Oncology Day Hospital Secretariat at +39 011 993 3775 to request an urgent consultation. Following the request, the patient is promptly contacted by their specialist physician for assessment and guidance.

Supportive and Palliative Care

Cancer patients often have complex needs that extend beyond the treatment of the disease itself and require comprehensive, multidisciplinary care.

At the Candiolo Cancer Institute, patients who need additional support have access to specialists from a range of disciplines, ensuring personalized management of cancer-related symptoms and associated conditions. Services may include nutritional counseling, physical rehabilitation, pain management, and support for other medical needs that may arise during the course of treatment and recovery.

The goal is to improve quality of life, promote overall well-being, and provide comprehensive care tailored to each patient’s individual needs.

Follow-up

After completion of treatment, follow-up is essential to monitor the patient’s health and allow for early detection of any recurrence. European gynecologic oncology guidelines (ESGO) recommend the following schedule:

  • Gynecological visits: every 3–6 months during the first two years, then every 6 months for the following three years.
  • Pap test: once a year to assess the health of cervical cells.
  • Instrumental and blood tests: such as ultrasound, CT scan, or MRI, performed only when clinically indicated by the physician, depending on symptoms and disease stage.

Personalized follow-up allows for early intervention in case of new findings and ensures safe, long-term monitoring of the patient.

Social Work Services

The Social Work Service at the Candiolo Cancer Institute provides information, guidance, and support to patients and their families regarding access to community services and the welfare and social security benefits available under current legislation.

During dedicated consultations, social workers assist with matters such as disability recognition, access to aids and prosthetic devices, employment-related benefits and leave, and other social support services.

The service is available on Wednesdays and Fridays from 9:00 a.m. to 1:00 p.m. and can be contacted at +39 011 993 3059.

Multi Disciplinary Team

Every cancer requires a multidisciplinary approach at all stages of disease management. At the Candiolo Cancer Institute, this is provided by a team of specialists from various clinical and surgical departments, known as the GIC, Interdisciplinary Care Group (or MDT). The GIC ensures that each patient is supported throughout the diagnostic and therapeutic process, including arranging and coordinating examinations and maintaining communication with the patient and their family.

For each patient, the GIC (MDT) defines and shares a personalized care pathway based not only on the type and stage of the tumor but also on the patient’s individual characteristics. The goal is to achieve the best possible outcomes both oncologically and functionally, while maintaining a high quality of life.

The Group also collaborates closely with the Institute’s researchers to provide patients with rapid access to the latest innovations in screening, diagnosis, and treatment.

Clinical Divisions

The diagnostic and therapeutic pathway for vulvar cancer at Candiolo involves several clinical divisions, including:

Clinical Studies

Researchers at the Canciolo Cancer Institute are currently involved in several national and international projects on gynecological cancers.

The main activities include clinical, translational, and molecular research, as well as numerous experimental clinical trials evaluating new chemotherapy drugs and immunotherapy approaches.

In addition, experimental surgical protocols are underway focusing on lymph node staging in cervical cancer, including the use of the sentinel lymph node technique.

Why Choose Us

At the Canciolo Cancer Institute, every patient with vulvar cancer is managed according to highly specialized standards, through the coordinated work of a dedicated Interdisciplinary Care Group (GIC).

Clinical Experience and Tailored Approach

Thanks to the large number of cancer cases treated each year, the Candiolo Cancer Institute is a national reference center for the care of this disease. This extensive experience allows us to manage even the most complex cases, always using a personalized approach, tailored to the clinical and individual profile of each patient.

Imaging Technologies and Advanced Diagnostics

Establishing a treatment plan always begins with an accurate and timely diagnosis. Patients have access to state-of-the-art imaging technologies, such as ultrasound, contrast-enhanced CT, MRI, and cholangio-RM, which are critical for accurately assessing the extent of the tumor.

Advanced laboratory tests, including molecular analyses, are also available to help define biological features of the disease and guide treatment choices.

Minimally Invasive Surgical Techniques and Multidisciplinarity

When appropriate, surgeries are performed using minimally invasive, laparoscopic or robotic techniques. These approaches involve the use of camera-equipped instruments introduced into the abdomen through small incisions, thus reducing surgical trauma. Benefits to the patient include shorter hospital stay times, faster recovery, and lower risk of complications compared with traditional open surgery.

Clinical Research and Access to Trials

As an IRCCS (Scientific Institute for Research, Hospitalization, and Healthcare), the Candiolo Cancer Institute combines clinical care with a strong focus on scientific research. Patients can be considered for participation in active clinical trials, offering access to innovative therapies not yet available in standard practice. This integration of care and research is a distinctive strength that translates into tangible benefits for patients.

Care and Support Every Step of the Way

The Interdisciplinary Care Group (GIC or MDT) supports the patient at every stage: from diagnosis, through treatment, to follow-up. Special attention is paid to nutritional support, psychological health and reintegration into daily life. The organization of checkups, examinations, and treatment is designed to ensure continuity, serenity, and a humane, caring approach to each patient’s needs.