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Adenocarcinomas of the vulva are distinctly uncommon, especially when compared to squamous cell carcinoma of the vulva. These rare tumors include extrammmary Paget's disease.


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Mammary Type Tubulolobular Carcinoma of the Anogenital Area: Report of a Case of a Unique Tumor Presumably Originating in Anogenital Mammarylike Glands.

*Sikl's Department of Pathology, Charles University Medical Faculty Hospital, Pilsen, Czech Republic daggerDepartment of Dermatology, Medical Military Academy, Saint-Petersburg, Russia.


Am J Surg Pathol. 2006 Sep;30(9):1193-1196. Abstract quote

We present a case of an unusual tumor that occurred in the perianal area of a 64-year-old woman. Clinical investigation revealed no tumor elsewhere. The lesion was removed and the patient is alive without signs of metastasis or recurrence 5.5 years after surgery.

Histopathologically, the neoplasm was composed of single-cell cords of uniform round to ovoid cells intermixed with round to elongated tubules showing decapitation secretion at the luminal border. The tubules were mainly composed of a single cell layer, but focally multilayered epithelium (without evidence of myoepithelial cell differentiation) was seen as well as discrete cribriform structures and intraluminal bridges. Overall, the cell cord component slightly dominated over the tubular component, and the two were intermixed. A vague targetoid arrangement of the cell cords was seen focally. Immunohistochemically, the tumor cells in both components reacted positively for E-cadherin, 34betaE12, estrogen receptors and progesterone receptor and were negative for HER2/neu (c-erbB-2). There was no evidence of myoepithelial cell differentiation with calponin.

We believe that the present case is best classified as mammary type tubulolobular carcinoma and, given the location, the origin in anogenital mammary-like glands most likely.
Adenocarcinoma of Mammary-Like Glands of the Vulva: Report of a Case and Review of the Literature.

Abbott JJ, Ahmed I.

*Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota daggerDepartment of Dermatology, Mayo Clinic, Rochester, Minnesota.

Am J Dermatopathol. 2006 Apr;28(2):127-133. Abstract quote  

Mammary-like glands are a poorly recognized structure of normal vulvar skin. Adenocarcinomas arising from these glands are rare and represent a distinct clinicopathologic entity that must be distinguished from metastatic tumors.

We reviewed 19 cases reported in the literature and describe a 51-year-old patient with a long-standing nodule on her right interlabial sulcus. Excisional biopsy and Mohs micrographic surgery demonstrated an infiltrating adenocarcinoma of the mammary-like glands involving the dermis and subcutis.

On review of all 20 cases, the mean age at diagnosis was 59.6 years, the labia majora were involved in 13 cases (65%), and the mean lesional size was 2.5 cm. The tumor's histologic patterns varied greatly, although the histomorphologic similarity among this neoplasm, sweat gland carcinomas, and invasive extramammary Paget disease suggested a morphologic spectrum among these tumors. Criteria for establishing the diagnosis of adenocarcinoma of mammary-like glands include identifying transition zones between normal mammary-like glands and adenocarcinomatous areas. This tumor is best categorized as a locally aggressive neoplasm with a low risk for widespread metastasis.

Use of aggressive surgical therapeutic regimens, particularly in the case of tumors localized to the skin, must be reassessed, especially given the likelihood of long-term morbidity with such regimens. In our patient, successful Mohs micrographic surgery suggests that alternate management may be adopted for tumors localized to the skin, especially in elderly patients.
Primary "breast-like" cancer of the vulva: a case report and critical review of the literature.

Fracchioli S, Puopolo M, De La Longrais IA, Scozzafava M, Bogliatto F, Arisio R, Micheletti L, Katsaros D.

Gynecologic Oncology Unit, S. Anna Hospital, Turin, Italy.

Int J Gynecol Cancer. 2006 Jan-Feb;16 Suppl 1:423-8. Abstract quote  

Ectopic mammary gland tissue in the vulva is an uncommon clinical or pathologic finding. Such ectopic tissue can be the site of the same physiologic and pathologic processes found in the normal breast. However, the occurrence of adenocarcinoma is very rare, the first case being reported by Greene in 1935.

We here report the 16th case of primary "breast-like" cancer arising in the vulva, together with a critical review of the literature, in order to highlight the dilemmas of a clinical approach to this neoplasm. Clear guidelines for diagnosis and therapy are still unavailable. The main diagnostic criteria suggested by the authors of previous reports are discussed together with our own findings.

The therapeutic approach to this rare malignancy is also critically reviewed. In our opinion, when diagnosis of breast-like vulvar cancer is finally confirmed, treatment and follow-up should be the same as that would be chosen in a case of orthotopic breast neoplasm.
Ductal carcinoma in-situ arising in mammary-like glands of the vulva.

Castro CY, Deavers M.

Department of Pathology (KB 728), University of Alabama at Birmingham, 619 19th Street South, Birmingham, Alabama 35249-7331, USA.

Int J Gynecol Pathol. 2001 Jul;20(3):277-83. Abstract quote  

Recently a variant of cutaneous glands has been recognized in the anogenital region that combines the morphologic and immunohistochemical features of eccrine, apocrine, and mammary glands, so-called 'mammary-like glands of the vulva'. Carcinoma arising in mammary-like tissue of the vulva is a rare occurrence.

So far, there have been 11 cases of primary, mammary-type invasive carcinoma and one case of in-situ carcinoma reported in the vulva. We describe an unusual case of ductal carcinoma in-situ without invasion arising in mammary-like glands of the vulva. A 57-year old woman presented with a 1-year history of a 1 cm nodule in the right labium majus.

Excision showed ductal carcinoma in-situ with cribriform and papillary morphology in an adenosis-like lesion associated with mammary-like glands. No invasion into the stroma was identified. Immunostains were positive for gross cystic disease fluid protein 15 (GCDFP-15) and estrogen and progesterone receptors. An extensive survey including bilateral mammograms was negative. One year postoperatively, the patient shows no evidence of disease.

To our knowledge, this represents the second case of DCIS associated with mammary-like glands of the vulva reported in the English literature.
Adenocarcinoma of the mammary-like glands of the vulva: a concept unifying sweat gland carcinoma of the vulva, carcinoma of supernumerary mammary glands and extramammary Paget's disease.

van der Putte SC, van Gorp LH.

Department of Pathology, University Hospital Utrecht, The Netherlands.

J Cutan Pathol. 1994 Apr;21(2):157-63. Abstract quote  

An unusual case of primary adenocarcinoma of the vulva is described. It combined features of the three different types of adenocarcinoma of the skin of the vulva which are currently recognized, i.e. sweat gland carcinoma, adenocarcinoma derived from supernumerary mammary glands, and extramammary Paget's disease (EMPD).

Central in this tumor was a recently recognized type of cutaneous gland which appeared special for the anogenital region and was distinguished because it combined morphological features of eccrine, apocrine and mammary glands. As it most resembles mammary glands, it is named "mammary-like gland".

On the basis of the case presented and of a critical review of the literature, it was concluded that, with the exception of a few sweat gland carcinomas similar to those elsewhere in the skin, adenocarcinomas of the skin of the vulva form a single category of neoplasms with a variable expression of features reminiscent of eccrine, apocrine and mammary gland carcinomas.

The data strongly suggested a common derivation from the mammary-like gland or, in cases of EMPD, its related germinative cells in the epidermis.
Primary-adenocarcinoma of Bartholin's gland: a case report.

Kokcu A, Cetinkaya MB, Aydin O, Kandemir B.

Department of Obstetrics and Gynecology, School of Medicine, University of Ondokuz Mayis, Samsun (Turkey).

Eur J Gynaecol Oncol. 2004;25(5):651-2. Abstract quote  

Up to now, about 300 primary adenocarcinoma carcinomas of Bartholin's gland have been reported in the literature.

A new case of a 57-year-old woman with primary carcinoma of Bartholin's gland is reported.

The patient underwent radical vulvectomy and has been healthy for 18 months.




Unusual Hyperplasia of
Anogenital Mammary-Like Glands.

Kazakov DV, Hugel H,
Vanecek T
, Michal M.

*Sikl's Department of Pathology, Charles University Medical, Faculty Hospital, Pilsen, Czech Republic dagger
Dermatohistopathologische Gemeinschaftspraxis, Friedrichshafen, Germany.

Am J Dermatopathol. 2006 Apr;28(2):134-137. Abstract quote  

Anogenital mammary-like glands, although known to exist (as ectopic breast tissue) as early as 1872, have only recently been thoroughly studied and suggested to be the possible origin for various neoplastic (benign and malignant) and reactive conditions that show a striking homology with lesions in the breast.

Reported herein is an unusual benign lesion involving anogenital mammary-like glands in the perineal area of a 41-year-old woman. The lesion is unique in that it does not fully fit into any of the previously recognized conditions affecting anogenital mammary-like glands nor has it an apparent well-defined mammary counterpart.

Microscopically, at first glance the lesion appeared as a cluster of extended anogenital mammary-like glands of variable complexity and a lobular architecture, which probably resulted from a compact proliferation of closely situated ducts with small, round lumens (when cut transversally). When cut longitudinally, some of these tiny ducts appeared to bud from larger ductal structures, resembling so-called satellitosis in the breast and this gave a fibroadenomatous appearance to the areas. In addition, there were adenosis-like areas.

This lesion extends the spectrum of changes seen in anogenital mammary-like glands.
Salivary Gland-Type Basal Cell Adenocarcinoma of Presumed Bartholin's Gland Origin: A Case Report

Ana Félix, M.D.; Jose F. Moura Nunes, M.D.; Jorge Soares, M.D., Ph.D.

From the Departamento de Patologia Morfológica, Instituto Português de Oncologia de Francisco Gentil, Portugal.

Int J Gynecol Pathol 2002;21:194-197 Abstract quote

Salivary gland-type carcinomas arising in Bartholin's gland are rare neoplasms.

We report the case of a 75-year-old female who presented with a vulvar tumor with morphological and immunophenotypical features identical to those of salivary gland basal cell adenocarcinomas. We believe that the tumor was most likely of Bartholin's gland origin, although no Bartholin's gland tissue was found adjacent to the neoplasm.

This type of malignancy with myoepithelial differentiation has not been previously reported to arise in the Bartholin's gland.

Endometrial carcinoma metastatic to the vulva: a case report and review of the literature.

Giordano G, Gnetti L, Melpignano M.

Department of Pathology and Medicine of Laboratory, Section of Pathology, Parma University, Parma, Italy.
Pathol Res Pract. 2005;201(11):751-6. Epub 2005 Oct 19. Abstract quote  

In this paper, we report a new case of metastatic endometrial carcinoma to the vulva and describe the clinical and pathological features.

We reviewed the literature to determine the frequency and to evaluate the prognostic significance of this rare disease. Moreover, we discuss the criteria for the differential diagnosis of endometrial carcinoma metastatic to the vulva, the primary vulvar adenocarcinomas, and other metastatic adenocarcinomas. The patient, previously diagnosed to have endometrial adenocarcinoma with squamous differentiation at III C stage (according to the FIGO system) and T2N1M0 stage (according to the TNM system), presented with a small plaque on the vulvar mucosa 8 months after endometrial primary carcinoma had been diagnosed.

The histological evaluation of excisional vulvar biopsy revealed a neoplasm with pathological features of endometrial carcinoma. Thus, the final diagnosis was metastatic endometrial carcinoma to the vulva. A total body computed tomography scan (CT) and an echotomography with contrast medium revealed a second metastatic lesion at the 8th segment of the liver. No other metastatic lesions developed, nor was a reduction in the size of liver metastasis observed after 3 months of hormonic treatment with progesterone. Fourteen months after the diagnosis of primary endometrial carcinoma, the patient died of disseminated metastatic lesions.

In conclusion, metastatic endometrial carcinoma to the vulva, although rare, might develop and could appear within a few months after the diagnosis of primary tumor. Moreover, in the presence of metastatic endometrial carcinoma to the vulva, it is necessary to verify if other visceral metastases are present.

Metastatic tumors of the vulva: a clinicopathologic study of 66 cases.

Neto AG, Deavers MT, Silva EG, Malpica A.


Am J Surg Pathol. 2003 Jun;27(6):799-804. Abstract quote

Metastatic tumors involving the vulva are rare, with only a few series and case reports published in the English literature to date. In this study, we present the clinicopathologic features of 66 cases of metastatic tumors of the vulva seen at the University of Texas M. D. Anderson Cancer Center from 1944 to 2001.

The patients' age ranged from 18 to 84 years (mean 54.8 years). The most common presentations were vulvar nodules or a mass (39 cases), pain (7 cases), and ulceration (5 cases). In 46.9% of cases, the primary tumor was of gynecologic origin, whereas in 43.9% of cases the primary tumor was of nongynecologic origin. The remainder had unknown primaries. The site most frequently involved by metastasis was the labium majus (44 cases: 18 on the right, 13 on the left, 6 bilateral, and 7 unspecified side). Thirty percent of the patients received chemotherapy as treatment for the metastasis, 27% received radiotherapy, and the rest received some combination of chemotherapy, radiotherapy, and surgery. Of the 60 patients with available follow-up, 52 died of disease within 1-81 months (median 7.5 months) from diagnosis of the metastasis.

Metastatic tumors of the vulva are rare; however, the diagnosis of these tumors is facilitated by the knowledge of a preexistent malignancy and the lack of a mucocutaneous intraepithelial lesion.


Prognostic Factors


Adenocarcinoma of mammary-like glands in the vulva successfully treated by weekly paclitaxel.

Tanaka H, Umekawa T, Nagao K, Ishihara A, Toyoda N.

Department of Obstetrics and Gynecology, Mie Prefectural Shima Hospital, Shima, Japan
Int J Gynecol Cancer. 2005 May-Jun;15(3):568-71. Abstract quote  

An 87-year-old was referred for gynecologic evaluation of a lesion involving the left labia majus noted 3 years earlier. Fine-needle aspiration cytology revealed clusters with an acinous structure or glandular formation. The tumor appeared as cell clusters with linear arrangements.

Histologic examination showed the same morphologic findings as scirrhus type of primary breast carcinoma. Examinations of the breasts and axillary lymph nodes were normal. This disease was diagnosed as an adenocarcinoma arising in mammary-like glands of the vulva. Bone scan showed multiple foci in the sternum, costa, and vertebrae, consistent with metastatic disease.

We administered five courses of weekly paclitaxel chemotherapy, which achieved a partial response. There were no severe adverse effects. In our case, the fine-needle aspiration cytology was a rapid and minimally invasive method of diagnosis, and the findings were extremely similar to those of the scirrhus type of primary breast carcinoma. Rapid and accurate diagnosis made with this technique might contribute to a good prognosis in the early-staged cases.

Weekly paclitaxel chemotherapy may be one of the safe and effective treatments for this disease with distant metastases, even in extremely aged patients (over 80 years).

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Last Updated September 14, 2006

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