I agree with Dr Tulio, the pictures looks-like well differentiated mammary carcinoma. In the last immage we see also a typical "intra cytoplasmatic lumen" inclusion.The literature includes in the list of epithelial vulvar lesions, a rare tumor with " breast-like " features, originating probably in anogenital adnexal glands.
1: Ann Pathol. 1999 Apr;19(2):124-7.
[Breast-like carcinoma of the vulva]
Erb-Gremillet S, Gunther M, Amiaux F, Parache RM.
Service d'Anatomie et de Cytologie Pathologiques, Centre Alexis-Vautrin (CRLCC),
Breast-like carcinomas of the vulva is a rare finding. Only 11 cases are
reported in the literature. This article reports a painful tumor of the left
vulvar labia in a 62 year-old woman. Excisional biopsy showed an infiltrating
adenocarcinoma, histologically similar to a breast tumor with positive hormonal
receptors. The origin of this tumor, ectopic mammary tissue or specific adnexal
genito-anal gland, is discussed. From data of the literature, we offer
guidelines for diagnosis, treatment and origin of this rare tumor.
PMID: 10349477 [PubMed - indexed for MEDLINE]
2: Eur J Gynaecol Oncol. 2002;23(4):350-2.
Breast-like cancer of the vulva: primary or metastatic?
A case report and review of the literature.
Pathology Department, General Clinic, Thessaloniki, Greece.
A 45-year-old white female presented a polypoid nodule in the vulva, one year
after she was operated on for breast cancer. Histologic examination showed a
poorly differentiated carcinoma that closely resembled the primary breast tumor.
Eight similar cases have been previously described in the literature. This very
rare event should be differentiated from primary adenocarcinoma of the
mammary-like glands of the vulva. The recognition of such a lesion as primary or
metastatic is very important, since it greatly influences management and
prognosis.PMID: 12214743 [PubMed - indexed for MEDLINE]
3: Wien Klin Wochenschr. 2000 Oct 13;112(19):855-8.
Primary breast cancer of the vulva: a case report and review of the literature.
Gorisek B, Zegura B, Kavalar R, But I, Krajnc I.
Clinical Department of Gynecology and Perinatology, Maribor Teaching Hospital,
Since 1872, 40 cases of ectopic mammary gland tissue in the vulva have been
reported in the literature. Out of these, 12 had a primary cancer in the ectopic
breast tissue. Seven metastases of an orthotopic breast cancer have been found
in this location. We are presenting the 20th case of cancerous breast tissue in
the vulva whom we classified as the 13th case of primary cancer based on
clinical and histopathological criteria of primary and metastatic malignant
disease. Because of the advanced age of the patient, wide local excision
followed by adjuvant hormonal therapy was opted for. Nineteen months after
surgery, there is no evidence of recurrent disease. Due to the rarity of this
entity, its management presents therapeutic dilemmas, and variable treatment
strategies are being found in the literature. In our opinion, the same basic
principles used for treatment of cancers of the orthotopic breast should be
applied in ectopic breast carcinoma.PMID: 11098538 [PubMed - indexed for MEDLINE]
4: Eur J Gynaecol Oncol. 2002;23(1):21-4.
Primary breast carcinoma of the vulva: case report and review of literature.
Piura B, Gemer O, Rabinovich A, Yanai-Inbar I.
Department of Obstetrics and Gynecology, Soroka Medical Center and Faculty of
Health Sciences, Ben-Gurion University of the Negev, Beer-Sheva, Israel.
The occurrence of ectopic breast tissue within the vulva is uncommon and the
development of breast carcinoma within vulvar ectopic breast tissue is very
rare. To date, only 12 cases of primary vulvar breast carcinoma have previously
been reported in the English literature. This paper presents the 13th reported
case of primary breast carcinoma of the vulva. The patient presented with a
vulvar ulcerated lump and the diagnosis was based on a morphologic pattern
consistent with breast carcinoma and the presence of estrogen and progesterone
receptors. Primary surgery consisted of radical vulvectomy and bilateral groin
dissection. The groin lymph nodes were involved bilaterally. Adjuvant therapy
consisted of systemic chemotherapy (4 cycles of adriamycin and cyclophosphamide
followed by 4 cycles of paclitaxel) and pelvic radiotherapy. Oral tamoxifen 20
mg/day was started for the next five years. It is concluded that the management
of primary breast carcinoma of the vulva should be modeled after that for
primary carcinoma of the orthotopic breast with primary surgery followed by
systemic chemotherapy and pelvic radiotherapy. Chemotherapy should be similar to
that employed for breast carcinoma. Tamoxifen should be prescribed for patients
whose tumors contain estrogen receptors.PMID: 11876386 [PubMed - indexed for MEDLINE]
5: Rev Med Chil. 2001 Jun;129(6):663-5.
[Mammary carcinoma ine ectopic breast tissue. A case report]
Pardo M, Silva F, Jimenez P, Karmelic M.
Unidad de Mastologia, Departamento de Obstetricia y Ginecologia, Hospital
Clinico de la Universidad de Chile, Av. Santos Dumont 999, Santiago-Chile.
Ectopic breast tissue, that includes supernumerary breasts and aberrant breast
tissue, develops along the mammary line. Malignancies rarely develop in this
tissue. We report a 44 years old female subjected to a resection of a 3 cm O
tumor located 2 cm below the right lower mammary sulcus. The pathological study
reported an infiltrating ductal carcinoma. The patient was operated again and a
metastatic carcinoma was detected in three resected axillary lymph nodes.
Adjuvant chemotherapy and radiotherapy was indicated.PMID: 11510208 [PubMed - indexed for MEDLINE]
I think that we need to make the differential diagnosis with “Microcystic adnexal carcinoma of the vulva” by immunohistochemistry study.
Microcystic adnexal carcinoma of the vulva
Gynecol Oncol. 2001 Sep;82(3):571-4.
Buhl A, Landow S, Lee YC, Holcomb K, Heilman E, Abulafia O.
Division of Gynecologic Oncology, Department of Obstetrics and Gynecology, State
University of New York Health Science Center at Brooklyn, 450 Clarkson Avenue,
Brooklyn, New York, 11203, USA.
BACKGROUND: Microcystic adnexal carcinoma (MAC) is a subset of sweat gland
carcinoma first described as a specific entity by D. J. Goldstein, R. J. Barr,
and D. J. Santa Cruz (Cancer 1982;50:566-72). We report the first case of MAC
occurring on the vulva and review the literature pertaining to this rare tumor.
CASE: A 43-year-old multiparous black woman presented initially to Kings County
Hospital Medical Center with a chief complaint of a vulvar lesion arising on the
left labia majora which she had noted for 4 years prior to presentation. Aside
from increasing paresthesia in the area, she denied any constitutional symptoms.
Her past medical history was significant only for hyperthyroidism and mild
hypertension and surgical history was noncontributory. Gynecologic history was
unremarkable, with sporadic care over the last 20 years. Physical examination
revealed a 1.5 x 2.0-cm raised, well-circumscribed, firm mobile lesion on the
left labia majora. It was noted to be yellow in color with the surrounding
tissue being unremarkable in character. The remainder of her gynecologic
examination and lymph node survey was unremarkable. Preoperative chest X ray was
negative as was the CAT scan of the abdomen and pelvis. All laboratory values
were within normal limits. A Pap smear done preoperatively was significant for
atypical squamous and glandular cells of undetermined significance. Subsequent
colposcopic examination of the cervix was remarkable for cervicitis and was
adequate, with the entire transformation zone visualized. Both endocervical
curettage and endometrial biopsy were normal. Initially, an excisional biopsy
was performed with final pathology demonstrating microcystic adnexal carcinoma
with positive surgical margins. She subsequently underwent a left radical
hemivulvectomy with bilateral inguinal groin lymph node dissection. At the time
of surgery, the left labia majora was noted to be well healed, with a residual
surgical scar easily discernible. No areas of discoloration were noted and
digital palpation of the area was unremarkable. Microscopic residual tumor was
noted; however, all surgical margins and lymph nodes were negative for tumor.
Her postoperative course was unremarkable. The patient has continued to do well
since the time of her surgery and is being followed conservatively. CONCLUSION:
Radical vulvectomy should be performed when MAC occurs in the vulva to secure
negative margins of resection. Groin dissection should be reserved for cases in
which the inguinal lymph nodes are clinically suspicious or in cases of tumor
recurrence. PMID: 11520158 [PubMed - indexed for MEDLINE]
I beg to be excused, by the long text…
Salvador Bahia Brasil.