Primary vulvar adenocarcinomas are rare tumors, and their histogenesis is not fully understood. They are classified into extramammary Paget's disease, sweat gland carcinomas, and "breast-like" adenocarcinomas of the vulva. The latter resemble adenocarcinomas arising in the breast morphologically and immunophenotypically. Rare cases of adenocarcinoma with apocrine features have been reported, and whether these neoplasms originate from the "native apocrine" sweat glands or from "anogenital mammary-like" glands are still debatable. The presence of normal mammary-like glands in the vicinity of the tumor, the transitional malignant morphological features from normal mammary-like glands and the tumor, the breast-like histological features of the tumor, and the expression of estrogen and progesterone receptors generally suggest an origin from anogenital mammary-like glands. Absence of these features points toward native apocrine sweat glands as the source of these neoplasms. In this report, we present a patient who was initially diagnosed with Paget's disease of the right vulva, which was treated by hemi-vulvectomy, and who later presented with primary vulvar apocrine adenocarcinoma with metastasis to the inguinal lymph nodes and intranodal mucinous/colloidal differentiation: a feature, to the best of our knowledge, not reported before. We also reviewed the histogenesis of the vulvar adenocarcinomas, with emphasis on the morphological features that separate the tumors arising from the anogenital mammary-like glands in the vulva from those arising from the native vulvar sweat glands.
"Immunohistochemically, apocrine adenocarcinomas are usually negative for estrogen and progesterone receptors, an exception being those originating from the breast. Vulvar adenocarcinomas of sweat gland origin have a propensity to spread through the local lymphatics, typically to ipsilateral inguinal lymph nodes (Alsaad et al., 2009). Several studies have looked at survival rates of apocrine adenocarcinomas in general based on metastasis to inguinal nodes, with one study recommending prophylactic lymphadenectomy, though most surgeons will only perform this for clinically apparent lymphadenopathy (Chintamani et al., 2003). "
[Show abstract][Hide abstract] ABSTRACT: ► Vulvar adenocarcinomas are rare comprising less than 0.1% of primary malignancies of the vulva. ► The diagnosis of primary vulvar adenocarcinomas remains a challenge due to its rarity, variation in histological appearance, and limited literature. ► The basis of the diagnosis includes morphology, immunohistochemistry, clinical history and pattern of spread.
"The cancer cells in the neoplasm usually stay "in situ" and only rarely invade into the dermis to be metastatic via the lymphatic system [4,5]. One has to differentiate neoplasms with Paget phenomenon from carcinomas metastasized from adjacent organs such as the urinary system and rectum . Here, we present an unusual case of EMPD with carcinoma cells invading into the dermis without lymph node infiltration in a Chinese woman. "
[Show abstract][Hide abstract] ABSTRACT: Extramammary Paget's disease (EMPD) remains a rare condition with only a limited number of cases reported in the literature. EMPD is mainly composed of intraepidermal Paget cells, and possesses variable clinical behaviors and histological appearances, leading to difficulty in the diagnosis of this disease.
We here report a case of primary EMPD with the appearance of a nodule on the background of erythema. Histological assessment showed Paget cell infiltration throughout the epidermis with dermal spread. Using immunohistochemistry, the expressions of CK7, CK19, CK20, GCDFP-15, CEA, S-100 protein and bcl-2 were examined to elucidate the cellular differentiation of the carcinoma.
According to the histological assessment, this case was diagnosed as primary EMPD with carcinoma cells invading into the dermis, but without lymph node infiltration.
BMC Cancer 08/2010; 10(1):405. DOI:10.1186/1471-2407-10-405 · 3.36 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: The aim of this article was to evaluate the clinical and pathologic characteristics, therapy, and prognostic factors of vulvar sweat gland carcinoma.
Clinical and pathologic data for 12 patients with vulvar sweat gland carcinoma treated at our institution from January 1958 to April 2009 were retrospectively analyzed. Of the 12 cases, 7 cases were vulvar sweat gland carcinoma, 3 cases were vulvar Paget disease with underlying sweat gland adenocarcinoma, 1 case was vulvar apocrine adenocarcinoma, and 1 case was adenoid cystic carcinoma of the vulvar sweat gland. Two patients were treated with simple vulvar tumor excision at other medical institutions without adjuvant therapy. Among the other 10 patients, 6 underwent radical vulvectomy; 3, wide local excision of the vulva; and 1, a simple vulvectomy. For 5 of the 12 patients, bilateral or unilateral inguinal lymph nodes excision and biopsy were performed. For 1 patient with bulky inguinal lymph nodes, only a biopsy was performed, and the patient received radiotherapy after vulvar surgery.
A follow-up for 11 patients was conducted until death or April 1, 2009. Five of the 11 patients had recurrences after primary treatment. For 2 of these patients, recurrence was local 6 and 48 months after treatment. For 3 patients, distant metastasis was found 18, 5, and 31 months after surgery at our institution. Five of 11 patients died, 1 of whom died of irrelevant disease and 4 of tumor progression. The total survival periods of the 4 patients who died of tumor progression were 24, 36, 44, and 203 months. The other 6 patients have survived for more than 5 years without local failure. In total, there are 7 patients who have survived for 5 years or more.
Vulvar sweat gland carcinoma is a very rare entity. Surgery is the primary treatment modality, and the function of radiotherapy and chemotherapy is uncertain. The vulvar tumor size and inguinal lymph nodes metastasis will influence the prognosis, with pathologic differentiation and surgical margin status being the probable prognostic factors.
International Journal of Gynecological Cancer 07/2010; 20(5):874-8. DOI:10.1111/IGC.0b013e3181e1c167 · 1.95 Impact Factor
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