General
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• Uncommon
• Diagnosis is fairly straightforward when a documented history of prior malignancy elsewhere is available
• Most metastases occur within a few months after diagnosis of the primary tumor, but late recurrences / metastases have been documented
• Rarely, tumors are of unknown origin, presenting as widely metastatic tumor, with their origin difficult to determine due to poor histologic differentiation
• >50% of metastases to vulva have gynecologic primaries, most commonly from uterine cervix, followed by ovary, endometrium and vaginal origin
• Non gyn primaries are most commonly from GI tract, followed by breast, melanoma, lung, lymphoma, bladder and kidney carcinomas

Epidemiology
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• 5-8% of all vulvar malignancies are metastases (Am J Surg Pathol 2003;27:799)
• Often seen in peri- or post-menopausal women
• Reported frequently in Caucasian women

Sites
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• Labium majus (frequently unilateral), followed by clitoris

Clinical features
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• The common presenting symptom is mass (single or multiple nodules), followed by pain, ulceration, bleeding, pruritus, erythema and swelling (Am J Surg Pathol 2003;27:799)
• Clitoromegaly

Diagnosis
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• CT and MRI of the abdominopelvic region will reveal the extent of tumor
• FDG PET scan for staging and recurrence
• Transvaginal ultrasonography for assessment of uterus and adnexae

Prognostic factors
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• The prognosis in most patients is poor and they frequently die of metastatic disease
• Rarely the vulva is the only site of metastasis; these cases may have a "fair" prognosis

Case reports
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• Bladder:
  • 78 year old woman with bladder urothelial carcioma and vulvar metastases (J Reprod Med 1999;44:729)
  • 84 year old woman with bladder squamous cell carcinoma presenting as vulvitis and cliteromegaly (Gynecol Oncol 2004;95:722)
• Breast:
  • 32 year old woman with breast cancer metastatic to vulva (Gynecol Oncol 2004;94:858)
  • 49 year old woman with breast cancer metastatic to vulva on a rectus abdominis myocutaneous flap (Eur J Gynaecol Oncol 2003;24:577)
  • 49 year old woman with malignant phyllodes tumor of breast metastasizing to vulva (Nucl Med Mol Imaging 2012;46:232)
  • 53 year old woman with lobular breast carcinoma metastatic to vulva (Gynecol Oncol 1998;69:84)
  • 68 year old woman with ductal breast cancinoma metastatic to clitoris (J Low Genit Tract Dis 2012;16:66)
  • 93 year old woman with lobular carcinoma of breast metastatic to vulva (J Egypt Natl Canc Inst 2010;22:57)
• Cervix:
  • 37 year old woman with cervical adenocarcinoma metastatic to vulva (Dermatol Online J 2013;19:18172)
  • 46 year old woman with squamous cell carcinoma of cervix and vulval metastases (Ann Dermatol 2011;23:S64)
  • 66 year old woman with cervical cancer and cutaneous metastatic disease including to vulva (J Obstet Gynaecol Can 2010;32:467)
  • Vulvar metastasis due to advanced cervical squamous cell carcinoma (Arch Gynecol Obstet 2010;281:761)
  • Clitoral metastasis from cervical carcinoma (J Obstet Gynaecol 2011;31:359)
  • Multiple skin metastases to vulva from carcinoma of cervical stump (Ginekol Pol 2010;81:140)
• GI tract:
  • 57 year old woman with isolated metastasis from sigmoid adenocarcinoma to vulva (Int J Clin Exp Pathol 2012;5:609)
  • Anal adenocarcinoma metastasizing to clitoris (Eur J Obstet Gynecol Reprod Biol 2009;144:182)
  • Appendix primary with metastasis presenting as vulvar swelling (J Obstet Gynaecol Can 2014;36:904)
• Kidney:
  • 75 year old woman with urothelial carcinoma of renal pelvis metastasizing to clitoris (Int Urol Nephrol 2004;36:331)
• Lung:
  • 57 year old woman with primary lung large cell carcinoma metastatic to vulva (Gynecol Oncol 2004;94:829)
  • 66 year old woman with lung carcinoma revealed by vulvar metastasis (Rep Pract Oncol Radiother 2013;18:182)
  • Metastatic lung cancer involving vulva (Aust N Z J Obstet Gynaecol 2011;51:175)
• Lymphoma:
  • 87 year old woman with vulvar metastasis as initial presentation of non-Hodgkin lymphoma (J Reprod Med 2007;52:1065)
• Uterus:
  • 46 year old woman with low grade endometrial stromal sarcoma metastatic to clitoris (Int J Gynecol Cancer 2007;17:290)
  • 63 and 83 year old women with endometrial carcinoma metastatic to vulva (J Low Genit Tract Dis 2007;11:118)
  • 79 year old woman with endometrial carcinoma metastatic to squamous cell carcinoma of vulva (tumor to tumor metastasis) (Arch Pathol Lab Med 2013;137:1825)
  • Early stage well differentiated endometrial cancer metastatic to vulva (J Minim Invasive Gynecol 2014;21:708)
  • Endometrial carcinoma metastatic to clitoris / vulva (Acta Med Iran 2013;51:652, Pathol Res Pract 2005;201:751)
• Thyroid:
  • 43 year old woman with medullary carcinoma metastatic to vulva (In Vivo 2010;24:791)

Treatment
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• In most patients, the treatment is palliative
• Surgical resection: tumor debulking or simple excision, wide local excision, vulvectomy
• Chemotherapy
• Radiotherapy

Clinical images
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CT scan, tumor of the vulva

Thickening of left vaginal wall

PET images

 

Vulvar mass before and after radiotherapy

Micro description
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• In most cases, the tumors can be readily identified as adenocarcinoma, squamous cell carcinoma, melanoma, lymphoma, etc
• However, presentation as poorly differentiated neoplasm is not uncommon, which necessitates examination of a panel of immunohistochemical markers to identify the primary organ of origin
• Mammary type carcinomas arising from ectopic breast tissue and other tumors arising primarily in vulva should be ruled out
• As with metastases to other sites, the tumors generally are well-circumscribed with mostly pushing or rare infiltrative tumor edges
• Though most are located within subcutaneous tissue or dermis, focal epidermotropism is seen rarely

Micro images
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Cervical squamous
cell primary

Sigmoid colon primary

     

Melanoma metastatic to vulva

Immunohistochemistry
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• The appropriate stains will depend on the suspected primary; the most commonly included stains for an unknown primary are:
  • Melanocytic markers: S100, MelanA/MART1, HMB45, pan-melanoma cocktail, SOX10
  • Pan-cytokeratin, CK7, CK20, CK5/6, p63, p40, CEA, TTF1, Naspin, GCDFP-15, mammaglobin
  • ER, PR, E-cadherin, CK903, Uroplakin-III, CDX2
  • Chromogranin, synaptophysin, NSE, calcitonin
  • CD45, CD3, CD20, PAX5, BCL2, BCL6
  • Desmin, smooth muscle actin, CD99, CD34

Differential diagnosis
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• Bartholin gland abscess
• Vulvar Crohn disease
• Vulvar infection

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