There are several types of plasma cell neoplasms. These diseases are all associated with a monoclonal (or myeloma) protein (M protein). They include monoclonal gammopathy of undetermined significance (MGUS), isolated plasmacytoma of the bone, extramedullary plasmacytoma, and multiple myeloma.
Incidence and Mortality
Estimated new cases and deaths from multiple myeloma in the United States in 2012:
New cases: 21,700.
Clinical Presentation and Evaluation
Estimated new cases and deaths from vulvar cancer in the United States in 2013:
New cases: 4,700.
The vulva is the area immediately external to the vagina, including the mons pubis, labia, clitoris, Bartholin glands, and perineum. The labia majora are the most common site of vulvar carcinoma involvement and account for about 50% of cases. The labia minora account for 15% to 20% of vulvar carcinoma cases. The clitoris and Bartholin glands are less frequently involved. Lesions are multifocal in about 5% of cases. About 90% of vulvar carcinomas are squamous cell cancers. This evidence summary covers squamous cell cancers and vulvar intraepithelial neoplasias (VIN), some of which are thought to be precursors to invasive squamous cell cancers.
Survival is dependent on the pathologic status of the inguinal nodes and whether spread to adjacent structures has occurred. The size of the primary tumor is less important in defining prognosis. In patients with operable disease without nodal involvement, the overall survival (OS) rate is 90%; however, in patients with nodal involvement, the 5-year OS rate is approximately 50% to 60%.
Risk factors for lymph node metastasis include the following:[5,6,7,8,9]
Clinical node status.
Degree of differentiation.
Depth of stromal invasion.
Presence of capillary-lymphatic space invasion.
Overall, about 30% of patients with operable disease have lymph nodal spread.
In many cases, the development of vulvar cancer is preceded by condyloma or squamous dysplasia. The prevailing evidence favors human papillomavirus (HPV) as a causative factor in many genital tract carcinomas. The HPV-related basaloid and warty types are associated with VIN. About 75% to 100% of basaloid and warty carcinomas harbor HPV infection. In addition to the much higher prevalence of HPV in these subtypes than in the keratinizing subtypes, the basaloid and warty subtypes also share many common risk factors with cervical cancers, including multiplicity of sex partners, early age at initiation of sexual intercourse, and history of abnormal Pap smears. HPV-associated VIN (termed usual-type VIN when high-grade 2 and 3) is most common in women younger than 50 years, whereas non-HPV VIN (termed differentiated-type VIN when high-grade 3) is most common in older women. The former lesion-type VIN grade 1 is no longer classified as a true VIN.[12,13]
The pattern of spread is influenced by the histology. Well-differentiated lesions tend to spread along the surface with minimal invasion, whereas anaplastic lesions are more likely to be deeply invasive. Spread beyond the vulva is either to adjacent organs such as the vagina, urethra, and anus, or via the lymphatics to the inguinal and femoral lymph nodes, followed by the deep pelvic nodes. Hematogenous spread appears to be uncommon.
American Cancer Society.: Cancer Facts and Figures 2013. Atlanta, Ga: American Cancer Society, 2013. Available online. Last accessed March 13, 2013.
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Sedlis A, Homesley H, Bundy BN, et al.: Positive groin lymph nodes in superficial squamous cell vulvar cancer. A Gynecologic Oncology Group Study. Am J Obstet Gynecol 156 (5): 1159-64, 1987.
Binder SW, Huang I, Fu YS, et al.: Risk factors for the development of lymph node metastasis in vulvar squamous cell carcinoma. Gynecol Oncol 37 (1): 9-16, 1990.
Homesley HD, Bundy BN, Sedlis A, et al.: Prognostic factors for groin node metastasis in squamous cell carcinoma of the vulva (a Gynecologic Oncology Group study) Gynecol Oncol 49 (3): 279-83, 1993.
Hampl M, Sarajuuri H, Wentzensen N, et al.: Effect of human papillomavirus vaccines on vulvar, vaginal, and anal intraepithelial lesions and vulvar cancer. Obstet Gynecol 108 (6): 1361-8, 2006.
Schiffman M, Kjaer SK: Chapter 2: Natural history of anogenital human papillomavirus infection and neoplasia. J Natl Cancer Inst Monogr (31): 14-9, 2003.
Pepas L, Kaushik S, Bryant A, et al.: Medical interventions for high grade vulval intraepithelial neoplasia. Cochrane Database Syst Rev (4): CD007924, 2011.
Sideri M, Jones RW, Wilkinson EJ, et al.: Squamous vulvar intraepithelial neoplasia: 2004 modified terminology, ISSVD Vulvar Oncology Subcommittee. J Reprod Med 50 (11): 807-10, 2005.