Dermatology Online Journal

Localized blanching erythema in a patient with vulvar carcinoma
Jennifer Hau¹ BA, Lucia Diaz² MD, Taraneh Paravar³ MD, Susan Chon² MD
Dermatology Online Journal 18 (5): 8

1. The University of Texas Medical School at Houston, Houston, Texas
2. Department of Dermatology, The University of Texas Medical School at Houston / MD Anderson Cancer Center, Houston, Texas
3. Division of Dermatology, University of California, San Diego, San Diego, California

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Abstract

Mammary-like carcinoma arising in the vulva is a rare type of vulvar malignancy. Cutaneous metastasis of vulvar carcinoma is uncommon and the majority of cases have been reported in patients with squamous cell carcinoma of the vulva. We describe a 69-year-old woman with mammary-like carcinoma of the vulva with cutaneous metastasis presenting as asymptomatic localized blanching erythema.

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Introduction

Vulvar cancers comprise five percent of female gynecologic malignancies [1]. Carcinoma of the vulva arising from distinct mammary-like anogenital glands is a rare variant [2]. Vulvar carcinoma typically metastasizes first to regional lymph nodes. Common sites of distant metastasis include lung, liver, and bone [1, 3]. Metastatic spread of vulvar carcinoma to the skin is uncommon and the majority of cases have been reported in patients with vulvar carcinoma of the squamous cell type. Cutaneous metastasis of vulvar carcinoma can present as erythematous nodules, plaques, or telangiectatic lesions that may be tender or pruritic [4]. We describe a case of mammary-like carcinoma of the vulva with cutaneous metastasis presenting as asymptomatic localized blanching erythema.

Case report

Figure 1
Figure 1. Confluent blanching erythema on the right flank, lateral abdomen, buttock, and thigh

A 69-year-old woman with history of toe porocarcinoma, celiac disease, and newly diagnosed mammary-like carcinoma of the vulva and bilateral inguinal lymph nodes was evaluated for a progressive asymptomatic rash for one week. Clinical examination revealed confluent blanching erythema on her right flank, lateral abdomen, buttock, and thigh (Figure 1). The patient was afebrile and basic laboratories were normal. She had recently received two cycles of cisplatin as well as radiation to the vulva and bilateral inguinal nodes. She denied trauma, arthropod bites, travel, new contacts, or medications.

Figure 2	Figure 3
Figure 2. Metastatic poorly-differentiated mammary-like vulvar carcinoma presenting as extralymphatic tumor cells in the dermis. Immunohistochemistry was negative for D2-40. (H&E, low power) Figure 3. Metastatic poorly-differentiated mammary-like vulvar carcinoma in the dermis. (H&E, high power)

Histopathological examination showed metastatic mammary-like carcinoma in the dermis (Figures 2 and 3). This histology was similar to the poorly differentiated carcinoma with mammary-like pattern seen in prior biopsies from the labia majora and inguinal lymph nodes.

Discussion

Mammary-like carcinoma of the vulva is rare [2]. Primary vulvar lesions resembling breast tumors originally were considered to arise from supernumerary breast tissue. However, there are distinct anogenital glands of the vulva with morphological similarities to mammary glands. Cancers arising in these glands may demonstrate estrogen or progesterone receptor expression. Diagnosis may also be aided by characteristic location in the interlabial sulcus or by the presence of adjacent benign mammary-like glands on microscopic examination [2, 5].

Cutaneous metastasis of any vulvar carcinoma is uncommon, with the majority of reported cases occurring in patients with SCC [1, 3, 4, 5]. The destruction of lymphatic channels related to disease, lymph node dissection, and radiation therapy may interrupt the usual lymphatic outflow of vulvar cancer with resultant lymphedema promoting the cutaneous spread of cancer cells [3, 4]. Cutaneous involvement is usually limited to the groin, thighs, or abdomen [1, 5].

Mammary-like vulvar carcinomas are considered locally aggressive and often treated primarily with radical or hemivulvectomy [2]. Chemotherapy, radiotherapy, or hormonal therapy has been used alone or as adjunctive treatment for adenocarcinoma of mammary-like glands of the vulva. The resemblance to breast cancers has prompted treatment approaches similar to that for breast carcinoma treatment [6]. The relative rarity and short follow-up period of cases, however, limit full understanding of treatment response and prognosis [2].

The presence of skin metastasis is rare and may indicate late-stage disease in vulvar malignancy [1]. In the few reported cases of squamous cell carcinoma or apocrine carcinoma of the vulva, death followed within 12 months of diagnosis [1, 5]. Management strategies include excision, chemotherapy, radiotherapy, electron beam radiotherapy, and topical immunomodulators [3, 4, 5]. No treatment modality has proven successful and treatment, hence, should be considered palliative [3, 4].

References

1. Wang AR, O’Brien M, Ross R, et al. Epidermotropic metastasis from vulvar squamous cell carcinoma: a rare cutaneous manifestation. J Am Acad Dermatol. 2009;63:1088-1091. [PubMed]

2. Kazakov D, Spagnolo D, Kacerovska D, et al. Lesions of anogenital mammary-like glands: an update. Adv Anat Pathol. 2011;18:1-28. [PubMed]

3. Ghaemmaghami F, Modares M, Behtash N, et al. Multiple, disseminated cutaneous metastases of vulvar squamous cell carcinoma. Int J Gynecol Cancer. 2004;14:384-387. [PubMed]

4.Tjalma WAA, Watty K. Skin metastases from vulvar cancer: a fatal event. Gynecol Oncol. 2003;89:185-188. [PubMed]

5. Kiyohara T, Kumakiri M, Kawami K, et al. Apocrine carcinoma of the vulva in a band-like arrangement with inflammatory and telangiectatic metastasis via local lymphatic channels. Int J of Dermatol. 2003;42:71-74. [PubMed]

6. Abbot JJ, Ahmed I. Adenocarcinoma of mammary-like glands of the vulva: report of a case and review of the literature. Am J Dermatopathol. 2006;28:127-133. [PubMed]

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