Adenocarcinoma Of Vulva Research Articles

A case report of HER 2 positive vulvar extramammary Рaget disease

The low incidence, nonspecific clinical picture and absence of pathognomonic symptoms of vulvar adenocarcinoma make primary diagnosis very difficult. It could take months from the appearance of first symptoms to the final diagnosis and the start of specific treatment.There are limited options of standard treatment for this pathology. Therefore, attempts to use therapy that has proven itself in the other malignant tumors, based on molecular biological characteristics, are promising.Overexpression of HER2, which plays an important role in the carcinogenesis processes, is often observed in cases of extramammary Paget’s disease. We report a case of extramammary Paget’s vulvar cancer treated with a combination of cytostatic and targeted anti-HER2 agents.

Characteristics and outcomes of women with adenocarcinoma versus squamous cell carcinoma of the vulva: A Japanese Gynecologic Oncology Group study

ObjectiveStudies on vulvar adenocarcinoma are lacking. Thus, we aimed to compare the characteristics and survival outcomes between vulvar adenocarcinoma and squamous cell carcinoma (SCC). MethodsThis was a preplanned sub-analysis of a previously organized nationwide retrospective observational study in Japan conducted between 2001 and 2010 (JGOG-1075S). Surgically treated women with stage I-IV vulvar invasive adenocarcinoma were compared to those with SCC. Multivariable analysis was performed to identify patient and tumor characteristics related to adenocarcinoma. Inverse probability of treatment weighting was used to balance the background differences, and a Cox proportional hazards regression model was fitted to estimate the effect of the histological type on survival. ResultsForty-eight women with adenocarcinoma were compared with 537 women with SCC. On multivariable analysis, women with adenocarcinoma were younger (median age, 64.5 vs. 70 years, adjusted odds ratio [OR] per age 0.975, 95% confidence interval [CI] 0.955–0.995, P = 0.016) and had higher positive surgical margin rates (31.2% vs. 18.4%, adjusted OR 2.376, 95% CI 1.188–4.754, P = 0.014) than those with SCC. However, according to the weighted model, the survival outcomes were comparable (hazard ratio for progression-free survival, 1.088, 95% CI 0.740–1.601, P = 0.667 and hazard ratio for overall survival, 1.008, 95% CI 0.646–1.573, P = 0.973). Similar associations were observed when the cohort was stratified by age (≤70 or >70 years), stage (I-II or III-IV), and surgical margin (negative or positive) (all, P > 0.05). ConclusionVulvar adenocarcinoma is characterized by a younger age at diagnosis and higher positive surgical margin rates than SCC, but the survival outcomes are comparable.

Clinical characteristics and risk factors of invasion in extramammary Paget's disease of the vulva.

This study aimed to evaluate the risk factors of recurrence and invasive disease in patients with extramammary Paget's disease of the vulva (EPDV). We performed a retrospective analysis of patients who were initially diagnosed with EPDV in Fudan University Shanghai Cancer Center between May 2006 and March 2019. Thirty-eight patients were initially diagnosed with EPDV in our institution. Among them, 29 had intraepithelial EPDV, 8 had intraepithelial EPDV with stromal invasion, and 1 had an underlying vulvar adenocarcinoma. In total, 8 (21%) patients had 12 recurrences. Of these eight patients, four had one recurrence, while other four had two recurrences. Intraepidermal EPDV recurred nine times, while intraepidermal EPDV with invasive disease recurred thrice. The first and second recurrence intervals were 62.1 (9-146) months and 22 (15-28) months, respectively. The rate of invasive disease was 23.7% (9/38) for primary EPDV and 25% (3/12) for recurrent ones. We determined that the presence of invasive disease was associated with a history of more than 10years (p = 0.02) and inverselycorrelated with positive margins (p = 0.037), However, invasive disease had no statistical relations with age (p = 0.438), recurrence (p = 0.642), and lesion diameter (p = 0.08). EPDV with a history of more than 10years was associated with invasive disease. Close and long-term follow-up are recommended to identify those who require further treatment.

Primary adenocarcinomas of the vulva and related structures: An enigmatic and diverse group of tumors✰.

Approximately half of adenocarcinomas that involve the vulva are secondary, either through direct extension or metastases from elsewhere. Primary vulvar adenocarcinomas are rare and encompass a diverse array of neoplasms that are nominally classified based on the presumed tissue or organ of origin, the tumoral phenotype, or both. In this review, we summarize the clinicopathologic features of adenocarcinomas that originate from the vulva and related structures, including the terminal urethra. Adenocarcinomas of this region encompass lesions that are defined by their primary site (such as adenocarcinomas of the Bartholin gland, which by definition must be in the region of the Bartholin gland), histomorphology and immunophenotype (such as clear cell carcinoma and adenocarcinoma of intestinal [cloacogenic] type), or both (such as adenocarcinoma of skene gland origin, which is associated with that specific organ but which also displays a distinctive phenotype that is similar to the phenotype of high grade prostatic adenocarcinoma). Other types, such as mammary-type adenocarcinomas, are presumed to originate from the putative mammary-like glands of the vulva and display a spectrum of pathologic features that are similar to their mammary counterparts. Similarly, vulvar carcinomas of sweat gland origin are pathologically similar to their counterparts in the non-vulvar skin and include a variety of cutaneous adnexal-type malignancies such as apocrine adenocarcinoma and eccrine adenocarcinoma. Some tumors, such as adenoid cystic carcinoma, may represent a Bartholin gland adenocarcinoma, a carcinoma of sweat gland origin, or a carcinoma arising from extramammary Paget disease (EMPD), depending on the context. Invasive carcinomas of various types have been reported in 7-12.7% of EMPD, and these are likely the most common primary glandular malignancy of the vulva. Occasional vulvar adenocarcinomas have been reported to be HPV-associated, although this association has not been established for the broader group of vulvar adenocarcinomas. Rare adenocarcinomas are not classifiable by the aforementioned nosologic scheme, and are designated as vulvar adenocarcinoma NOS.

The diagnostic challenge of primary adenocarcinoma of the vulva: a case report

The diagnosis and classification of primary adenocarcinoma of the vulva is a complicated and understudied area, as this is a rare histological subtype of vulvar cancers. The differential diagnoses include extramammary Paget disease with invasion, adenocarcinoma arising from mammary-like glands, skin adnexal structures as well as Bartholin’s glands in the vicinity. We report a case of extramammary Paget disease which first presented as primary vulvar adenocarcinoma. The patient was a 71-year-old woman who presented with a swelling at the external genitalia. Clinical examination revealed a right vulvar lump, with eczematous features on the overlying skin. The lump was biopsied and was called Paget disease with underlying adenocarcinoma. Subsequently, the patient underwent radical vulvectomy and bilateral groin and pelvis dissection. The resection specimen was reported as extramammary Paget disease, with dermal invasion forming a poorly differentiated adenocarcinoma showing strong HER-2 expression on immunohistochemistry. This case illustrates the diagnostic challenge in the classification of primary vulvar adenocarcinoma. Positivity for HER-2 has been reported in Paget-associated vulvar adenocarcinoma and might offer potentially actionable targets on the selection of therapy, especially in advanced metastatic disease.

A Rare Case of Primary Invasive Papillary Adenocarcinoma of Vulvar Lesion [4J

INTRODUCTION: Vulvar cancer accounts for approximately 5% of all female genital malignancies and is commonly recognized as a disease of postmenopausal women. The vast majority of vulvar carcinomas are of the squamous cell type (90%) with primary vulvar adenocarcinomas ranking among the rare gynecological malignancies, comprising less than 0.1% of the cases. This case report is a primary vulvar adenocarcinoma and discusses the diagnosis and the treatment of this unusual and uncommon neoplasm. METHODS: The clinical case was analyzed followed by a literature review on papillary adenocarcinoma of the vulvar. RESULTS: A 53-year-old postmenopausal African-American female presented with 1-year history of enlarging, otherwise asymptomatic left vulvar mass. A flesh colored 3 × 2 cm mobile, nodular lesion was noted on left anterior labia majora. An excisional procedure was completed. Final pathology revealed the skin to be benign with cyst wall and contents revealing moderately differentiated invasive papillary adenocarcinoma. Stains of the neoplastic cells were positive for p53 immunostain. Follow-up clinical evaluation did not demonstrate evidence of metastatic disease and the recommendations of the monthly multidisciplinary meeting was for ipsilateral lymph node dissection and modified radical vulvectomy of tumor bed. The patient declined diagnosis and recommended treatment. CONCLUSION: Adenocarcinomas of the vulva are exceedingly rare, making diagnosis and management difficult for pathologists and clinicians. As a result literature is very limited. This report will allow for further advancement in regards to the diagnosis and treatment of this unusual and uncommon neoplasm.

Adenocarcinoma of the Vulva Arising in Ectopic Breast Tissue: A Case Series and Literature Review

Abstract Background: Embryonic mammary tissue may fail to regress, resulting in ectopic breast tissue in areas outside the breasts, including within the vulva. In exceedingly few instances, this tissue may undergo malignant transformation. Cases: Three cases of vulvar adenocarcinoma arising in ectopic breast tissue are presented. Tissue biopsy confirmed the diagnosis. Surgical resection was performed for these patients. Adjuvant therapy was individually tailored to the metastatic spread and receptor positivity of the tumors, including chemotherapy, radiation therapy, and hormonal therapy. Results: Radiation therapy was tolerated well by these patients, without undue side-effects. All three patients are alive and well 3 and 4 years post treatment. Conclusions: Treating patients with this vulvar malignancy with the established guidelines for breast carcinoma appears to be a rational approach with successful outcomes as occurred in the 3 patients presented in this report. (J GYNECOL SURG 33:22)

Primary mucinous adenocarcinoma of the vulva: A case report and review of the literature.

Primary vulvar cancer is a rare disease with an incidence of 2-3 per 100,000 women. The vast majority of vulvar carcinomas are of the squamous cell type (90%). Primary vulvar adenocarcinomas rank among the rare gynecological malignancies. We herein present a case of a vulvar mass near the vaginal orifice, the biopsy of which revealed a mucinous adenocarcinoma. Local excision was performed, followed by postoperative chemotherapy. The patient was asymptomatic and developed no recurrence during the 2 years of follow-up after surgery and chemotherapy. We consider local excision, with or without chemotherapy, to be an effective therapeutic approach to this type of tumor. However, further studies are required to support our conclusions for early-stage vulvar mucinous adenocarcinoma.

Apocrine Adenocarcinoma of the Vulva: A Case Report and Review of the Literature.

Primary vulvar adenocarcinomas are very rare. We describe the rare case of primary vulvar apocrine adenocarcinoma, a histologically rare subtype of vulvar adenocarcinoma. A 57-year-old Japanese woman presented with an enlarging vulvar mass. A dark-red, hemorrhagic, ulcerated tumor was on the right side of the anterior labial commissure measuring approximately 3.5 × 3.5 cm. Preoperative biopsy showed poorly differentiated carcinoma with partial differentiation to adenocarcinoma. Systemic examination revealed lymph node metastases in both inguinal regions and no other primary source. We performed radical vulvectomy and bilateral inguinal and pelvic lymphadenectomy. Histopathologic diagnosis was apocrine adenocarcinoma of the vulva with inguinal lymph node metastases, pT1bN2bM0. Surgical margins were negative. The patient received no adjuvant chemotherapy or radiation. Inguinal lymph node recurrence occurred after six months. Reresection and adjuvant tomotherapy were performed. After a further 12 months of observation, no rerecurrence was observed. The patient is now on follow-up.

Apocrine Adenocarcinoma of the Vulva

Cutaneous vulvar carcinomas are predominantly of squamous cell carcinoma type. Primary vulvar adenocarcinomas are rare with a poorly understood histogenesis. They are classified into extramammary Paget’s disease, sweat gland carcinomas, and breast-like adenocarcinomas of the vulva. Adenocarcinomas, originating from Bartholin glands, can also present as vulvar adenocarcinoma. Rare adenocarcinomas with apocrine features have been described in the literature. The origin of these neoplasms from the native apocrine sweat glands or from anogenital mammary-like glands is still debatable. We report herein a case of a 67 year old female with a rare primary apocrine carcinoma of the vulva.

An Unexpected Complete Remission of Advanced Intestinal-Type Vulvar Adenocarcinoma after Neoadjuvant Chemotherapy: A Case Report and a Literature Review

Vulvar cancer represents approximately 3%–5% of all gynecological malignancies. Squamous cell carcinoma is the most frequent histotype, whereas melanomas, adenocarcinomas, basal cell carcinomas, and sarcomas are much less common. Intestinal-type adenocarcinoma is a rare variant of vulvar carcinoma with only few cases found in the literature. The origin of this neoplasia is still much debated, but the most reliable hypothesis is the origin from cloacal remnants that may persist in the adult in different organs. Because of its extremely low incidence, the optimal management of this kind of vulvar cancer is still debated. We report the case of a woman affected by advanced intestinal-type vulvar adenocarcinoma, who achieved a complete clinical and pathological response after neoadjuvant chemotherapeutic treatment with platinum and paclitaxel.

Primary adnexal adenocarcinoma of the vulva: A diagnosis of exclusion based on location, immunohistochemistry, and pattern of spread

► 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.

Metastatic adenocarcinoma of mammary‐like glands of the vulva successfully treated with surgery and hormonal therapy

Vulvar cancer is a rare malignancy; most tumors are squamous cell type while adenocarcinomas are rare. Primary adenocarcinomas of the vulva predominantly include extramammary Paget's disease and sweat gland carcinomas. Greene first described a rare form of adenocarcinoma in 1936, which was called adenocarcinoma of mammary-like glands of the vulva because of its morphologic and immunohistochemical resemblance to breast adenocarcinomas. In the management of this entity, varying combinations of surgery, radiation therapy, systemic chemotherapy and/or hormonal therapy may be used, as in patients with orthotopic breast carcinoma. However, hormonal therapy leads the way in patients with positive hormonal receptors, where other therapies cannot be used due to comorbidities or advanced age. We present the first reported case of an elderly patient with metastatic vulvar adenocarcinoma arising from mammary-like glands, successfully treated with a combination of surgery and hormonal therapy.

Adenocarcinoma in the Vulva

Background: Adenocarcinoma in the vulva is uncommon, and can be primary or metastatic. Metastatic vulvar adenocarcinoma can derive from a genital primary site: cervix, endometrium, or ovary; or an extragenital site: colorectal, breast, or urinary tract. Cases: In this article 2 patients with metastasis of adenocarcinoma to the vulva are described, with emphasis on differential diagnosis and histology. One patient had a metastasis from a previously unknown and asymptomatic colorectal carcinoma, the other patient had a known cervical adenocarcinoma. In both patients, a metastasectomy was performed. Results:Case 1 was followed-up for more than 2 years and Case 2 for 1 year; no evidence of disease was found in both patients. Conclusions: Clinicians should be aware of the possibility of vulvar metastasis in patients with known primary tumors; new vulvar lesions in these patients should be biopsied to rule out metastatic disease. Second, this article demonstrates that immunohistochemistry can have a role in establishing the primary site when there is no known primary tumor elsewhere. (J GYNECOL SURG 28:43)

Extramammary Paget’s Disease: Summary of Current Knowledge

Extramammary Paget's disease (EMPD) is a rare cutaneous malignancy accounting for approximately 1% of vulvar cancers. The rarity of this disease has caused difficulties in its characterization. Controversies exist in the literature regarding many aspects of this condition including the prevalence of concurrent vulvar adenocarcinoma or invasive EMPD, association with regional and distant cancers, and recurrence rates following surgical excision. This extensive review takes a closer look at what is known about EMPD and the conclusions that have been drawn from this information. This article also provides a practical approach to patients with EMPD.

Vulvar apocrine adenocarcinoma: A case with nodal metastasis and intranodal mucinous differentiation

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.

Shedding of epidermal growth factor receptor is a regulated process that occurs with overexpression in malignant cells

Soluble isoforms of the epidermal growth factor receptor (sEGFR) previously have been identified in the conditioned culture media (CCM) of the vulvar adenocarcinoma cell line, A431 and within exosomes of the keratinocyte cell line HaCaT. Here, we report that the extracellular domain (ECD) of EGFR is shed from the cell surface of human carcinoma cell lines that express 7×105 receptors/cell or more. We purified this proteolytic isoform of EGFR (PI-sEGFR) from the CCM of MDA-MB-468 breast cancer cells. The amino acid sequence of PI-sEGFR was determined by reverse-phase HPLC nano-electrospray tandem mass spectrometry of peptides generated by trypsin, chymotrypsin or GluC digestion. The PI-sEGFR protein is identical in amino acid sequence to the EGFR ECD. The release of PI-sEGFR from MDA-MB-468 cells is enhanced by phorbol 12-myristate 13-acetate, heat-inactivated fetal bovine serum, pervanadate, and EGFR ligands (i.e., EGF and TGF-α). In addition, 4-aminophenylmercuric acetate, an activator of metalloproteases, increased PI-sEGFR levels in the CCM of MDA-MB-468 cells. Inhibitors of metalloproteases decreased the constitutive shedding of EGFR while the PMA-induced shedding was inhibited by metalloprotease inhibitors, by the two serine protease inhibitors leupeptin and 3,4-dichloroisocoumarin (DCI), and by the aspartyl inhibitor pepstatin. These results suggest that PI-sEGFR arises by proteolytic cleavage of EGFR via a mechanism that is regulated by both PKC- and phosphorylation-dependent pathways. Our results further suggest that when proteolytic shedding of EGFR does occur, it is correlated with a highly malignant phenotype.

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

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.

Skull metastasis in primary vulvar adenocarcinoma of the Bartholin's gland: A case report

Background. Metastatic bone involvement in vulvar squamous carcinoma is very rare. Here, we present a case of primary adenocarcinoma of the Bartholin's gland giving rise to isolated skull metastasis. Case. A 74-year-old woman was referred with a diagnosis of primary vulvar adenocarcinoma of the right labium minus. A right hemivulvectomy was performed with ipsilateral inguinofemoral lymphadenectomy, revealing a primary adenocarcinoma of the Bartholin's gland and metastatic involvement in 6 out of 13 lymphnodes. During chemotherapy administration, the patient started complaining of a worsening of right hypoacousis. CT scan and MRI of the brain showed the presence of a metastatic lesion on the right side of the skullbone. The diagnosis was metastatic poorly differentiated adenocarcinoma. Conclusion. In adenocarcinoma of the Bartholin's gland at an advanced stage of disease, a bone scan or survey in the preoperative as well as follow up staging procedures could be useful.

Primary Vulvar Adenocarcinoma of Cloacogenic Origin

A 43-year-old woman who had a vulvar mass associated with mild discomfort was found to have a rare primary vulvar adenocarcinoma of probable cloacal origin. The tumor was contiguous with the surface epithelium of the vulva and was a well to moderately differentiated adenocarcinoma of colonic type. Stains of the neoplastic cells were positive for both acid and neutral mucin, and periodic acid-Schiff (PAS) was positive after diastase reaction. The neoplastic cells were strongly positive for carcinoembryonic antigen, broad spectrum cytokeratin, and p-53 antigen. Clinical evaluation failed to show any primary tumor in colon, lung, or breast. The patient was disease free 18 months after operation.