Vulval ca and vulval lymph

Vulval Ca

1. Vulval Lymphatics and Vulval Cancer DR. HEM NATH SUBEDI RESIDENT ,OBGYN COMSTH

2. LYMPHATICS OF THE VAGINA The intrinsic plexuses are situated in the mucosal and muscle layers. Upper two-thirds drain into the glands like those of the cervix. Lower one-third drains into inguinal and at times into external iliac nodes.

3. LYMPHATICS OF THE VULVA There are dense lymphatic plexuses in the dermis of the vulva, which intercommunicate with those of subcutaneous tissue .   The lymphatics of each side freely communicate with each of them.   The lymphatics hardly cross beyond the labiocrural fold.   The vulval lymphatics also anastomose with the lymphatics of the lower-third of the vagina and drain into external iliac nodes   Lymphatics from the deep tissues of the vulva accompany the internal pudendal vessels to the internal iliac nodes   Superficial inguinal lymph nodes are the primary lymph nodes that act as the sentinel nodes of the vulva. Deep inguinal lymph nodes are secondarily involved. It is unusual to find positive pelvic glands without metastatic disease in the inguinal nodes   Gland of Cloquet or Rosenmüller , which is the upper most deep femoral nodes is absent in about 50 percent of cases.

4. Labia majora (anterior half) Lymphatics intercommunicate with the opposite side in the region of mons veneris → Superficial inguinal nodes. Thus, there is bilateral and contralateral spread of metastasis in malignancy affecting the areas.

5. Labia majora (posterior half) Drains into → Superficial inguinal → Deep inguinal→ External iliac. Labia minora and prepuce of clitoris Intercommunicating with the lymphatics of the opposite side in the vestibule and drains into superficial inguinal nodes. Glans of clitoris: Drains directly into the deep inguinal and external iliac glands. Bartholin’s glands: The lymphatics drain into superficial inguinal and anorectal nodes.

6. Node of Cloquet It was previously thought to be the main relay node through which the efferents from the superficial inguinal nodes pass to the external iliac nodes. Recent study shows its insignificant involvement in vulval malignancy, and thus, it is not considered to be the relay node. The efferents from the superficial inguinal may reach the external iliac group bypassing the node of Cloquet .

7. VULVAL CARCINOMA Incidence The lesion is rare, about 1.7 per 100,000 females. The distribution varies from 3-5 percent amongst genital malignancies. Etiology The etiology remains unclear. But the following factors are often related. Usually occurring in postmenopausal women with a median age of 60. ™More common amongst whites. Increased association with obesity, hypertension, diabetes and nulliparity . ™Associated vulval epithelial disorders (lichen sclerosus ) specially of atypical type are the risk factors. ™Human papilloma virus (HPV) DNA (type 16, 18) has been detected in patients with invasive vulval cancer. ™ Vulval cancer may have a causal relation with condyloma accuminata (HPV 6, 11), syphilis and lymphogranuloma venereum . ™ Chronic pruritus usually preceds invasive vulval cancer. ™ Chronic irritation of the vulva by chemical or physical trauma associated with poor hygiene may be a predisposing factor. ™ Other primary malignancies have been observed in about 20 percent of cases with vulval cancer. Cervix is most commonly affected; other sites are breast, skin or colon.

8. ™ Vulval cancer may have a causal relation with condyloma accuminata (HPV 6, 11), syphilis and lymphogranuloma venereum . ™Chronic pruritus usually preceds invasive vulval cancer. ™Chronic irritation of the vulva by chemical or physical trauma associated with poor hygiene may be a predisposing factor. ™Other primary malignancies have been observed in about 20 percent of cases with vulval cancer. Cervix is most commonly affected; other sites are breast, skin or colon.

9. Pathology Sites: The commonest site is labium majus followed by clitoris and labium minus. Anterior two-third are commonly affected. Malignant ulcer on the contralateral side may be multifactorial . Naked Eye Ulcerative: The features are raised everted edges, sloughing base with surrounding induration . This is common. Hypertrophic: The overlying skin may be intact or it ulcerates sooner or later. This is rare.

10. Spread Direct Lymphatics Hematogenous

11. Clinical features Patient profile: The patients are usually postmenopausal, aged about 60 years often with obesity, hypertension and diabetes. signs „ Vulval inspection reveals an ulcer or a fungating mass on the vulva. The ulcer has a sloughing base with raised, everted and irregular edges and it bleeds to touch. Surrounding tissue may be edematous and indurated . „Associated vulval lesions mentioned earlier may be present. Inguinal lymph nodes of one or both the sides may be enlarged and palpable. The enlargement may also be due to infection. „Clinical examination of the pelvic organs, including the cervix, vagina, urethra and rectum must be done. This is due to the coexistance of other primary cancers in the genital tract.

12. Diagnosis The diagnosis is confirmed by biopsy. When a definite growth is present, the biopsy is to be taken from the margin. Cystourethroscopy , Proctoscopy CT/MRI scan (for nodes) may be needed. In cases of vulval dystrophy, the sites are from multiple areas usually from the persistent red areas or from stained areas following toluidine blue test.

13. DIFFERENTIAL DIAGNOSIS The lesion needs differentiation from: Condyloma accuminata Syphilitic ulcer Tubercular ulcer Lymphogranuloma venereum Soft sore

14. CAUSES OF DEATH „Uremia—from ureteric obstruction due to enlarged common iliac and paraaortic nodes. „Rupture of the femoral vessels by the overlying involved inguinal lymph glands. „Sepsis.

15. MANAGEMENT Prophylactic Adequate therapy for non- neoplastic epithelial disorders of the vulva. Adequate therapy for persistent pruritus vulvae in postmenopausal women. Frequent use of multiple biopsies in conservative treatment of VIN. Liberal use of simple vulvectomy in postmenopausal women with VIN where follow-up facilities are not available.

16. DEFINITIVE TREATMENT Microinvasive lesion There is increased incidence of lymph node involvement in lesion of more than 1 mm invasion. It is thus prudent to perform radical vulvectomy with bilateral groin node dissection in all cases of stromal invasion more than 1 mm. However, if the invasion is less than 1 mm, wide local excision with or without ipsilateral groin lymphadenectomy may be done with follow up. Generally, there is no lymph gland involvement.Tumor free surgical margin should be at least 1 cm to prevent local recurrence.

17. Frank invasion Radical vulvectomy with bilateral inguinofemoral lymphadenectomy is the ideal surgery. Three separate incision (one for radical vulvectomy and one each for groin node dissection) approach is currently preferred instead of en-block approach. Pelvic node metastases are rare unless inguinofemoral nodes are involved. Pelvic lymphadenectomy in cases of positive deep node involvement is omitted in preference to external radiation on the groin and pelvis—in the form of 4500 to 5000 cGy , usually 4–6 weeks after surgery.

18. Negative sentinel lymph node biopsy for Micrometastasis may avoid extensive lymphadenectomy . Radical vulvectomy is often associated with Major long-term morbidity, sexual dysfunction and loss of body image. Radical local excision of the vulva with wide margins (1–3 cm) is considered to be an alternative to radical vulvectomy with equal result.

19. If the general condition is poor and/or in presence of medical diseases The following principles may be adopted: Two stage operation is preferred. Total vulvectomy followed by at a later date, bilateral inguinofemoral lymphadenectomy . Total vulvectomy followed by full pelvic and groin irradiation (megavoltage therapy). Neoadjuvant chemotherapy—chemotherapy followed by surgery, radiotherapy or both. Technically inoperable or recurrent lesion − Chemotherapy ( cisplatin , bleomycin , 5-FU) can be used as radiation sensitizer. Chemoradiation therapy may be combined as primary therapy or following surgical excision of the tumor. Radiotherapy can be used as a primary therapy for advanced disease.

20. RESULTS With negative groin nodes, the 5 year survival rate for invasive carcinoma ranges from 90–100 percent. With positive groin nodes, the survival rate falls to 20–55 percent. With positive pelvic nodes, the survival rate falls even below 20 percent.

21. Prognostic factors for vulval squamous cell carcinoma Clinical stage of the disease. Site of the tumor . Depth of stromal invasion. Lymph node involvement ( inguinofemoral and pelvic). Tumor diameter and differentiation. DNA ploidy status.

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