Paget disease of the vulva an analysis of 24 cases

Paget disease is a complex disorder that can be identified in the breast (mammary Paget disease) or in other locations (extramammary Paget's disease) such as ano-genital skin (Paget disease of the vulva -PVD). This condition is associated with low mortality, but a late diagnosis and recurrence can negatively impact the prognosis. Therefore, the main objective of this study is to evaluate if the human epididymis protein 4 (HE4) and cancer antigen125 (CA125) can promote recognition of PVD in early stages and during the relapses. we have conducted a prospective, observational and laboratory-based study, that included 50 patients, whose 25 healthy women represented the control group and 25 PVD patients, which have been operated in our Oncology Institute, from May 2017 to September 2019. Both in the control group and in PVD patients, the CA-125 and HE4 were evaluated before surgery and after 6 months. Finally, a comparison of markers serum level, both between before/after surgery and with control group, and a ROC (Receiver Operating Characteristic) curve were performed. Dosing the markers in PVD patients, 3/25 (12%) showed a higher value of CA125 and 11/25 (44%) an increased HE4. In addition, after surgical treatment there were no statistically significant difference between levels of CA-125 ( P = .3) and HE4 ( P = .19). On the other hand, comparing HE4 in PVD patients with the control group, a statistically significant difference was found ( P -value = .0036). Contrary, comparing CA-125 in PVD patients with the control group ( P -value= .1969), no statistically significant difference was evidenced. Moreover, ROC (Receiver Operating Characteristic) curve showed low sensitivity and specificity for CA125 with area under curve (AUC) = 0.5608. Instead, the ROC curve of HE4 revealed a sensitivity and specificity of 76% and 88% respectively (AUC = 0.7408) using a cut-off at 90 pmol/L. Despite the limited cases, our data showed that CA125 is not a sensitive marker for PVD. On the other hand, in 44% of PVD we’ve seen an increase in HE4. So, this could be a starting point for further research that could confirm the possibility to use this marker in order to support PVD early identification.

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Section: Discussionmentioning

confidence: 99%

A strengthening the reporting of observational studies in epidemiology (STROBE)

Dellino

Gargano

Tinelli³

et al. 2021

Medicine

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“…The tumor's histogenesis is uncertain; it could represent a sweat gland carcinoma, arising from the acrosyringium, a carcinoma arising from stem cells residing in the infundibulum‐sebaceous unit of the hair unit of the hair follicle and other adnexal structures 2 . A recent case‐series published by Loiacono et al 20 included 24 patients with a median age of 69.3 years old; synchronous cancer was identified in two patients, and two other patients had metachronous disease. Four patients had invasive disease at the time of diagnosis and eight had recurrent disease (33%).…”

Section: Discussionmentioning

confidence: 99%

Non‐human‐papillomavirus‐related malignancies of the vulva: A clinicopathological study

et al. 2020

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Background: Malignant tumor of the vulva is the fourth gynecological malignancy in frequency. Close to 70% of all vulvar malignancies are related to high-risk human papillomavirus (HPV) infection. Methods: A search for non-HPV-related malignant tumors of the vulva was performed in the last 20 years (2000-2020) in the pathology database of a single tertiary institution. We aim to estimate the prevalence of non-HPV-related malignancies in our population, describe clinicopathological features of these tumors and investigate the expression of some potential therapeutic targets. Results: A total of 71 patients were recovered; 26 patients (36%) had the diagnosis of extramammary Paget disease, 17 patients (24%) had basal cell carcinomas, 17 patients (24%) had primary melanomas, 10 patients (14%) had metastatic disease to the vulva and one patient (1%) had a primary dermatofibrosarcoma protuberans. Fifty-four percent of patients with extramammary Paget disease had a secondary malignancy and 12.5% had invasive disease. Programmed death-ligand 1 (PDL-1) was positive in seven out of nine primary melanomas and Her2/neu was overexpressed in six out of seven extramammary Paget disease. Conclusion: Non-HPV-related malignancies are important differential diagnoses in patient with vulvar lesions. Additional research is necessary to further understand these complex malignancies and potential new therapeutic targets.

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“…PVD can remain undiagnosed for several years, so frequently it is recognized as an extensive vulvar lesion which needs the use of demolition surgery and subsequent plastic-reconstruction [21]. Therefore, the search for serological markers to assist the early detection of PVD, would allow the identi cation of limited and non-invasive forms and the use of alternative approaches such as imiquimod and photodynamic treatment (currently off label) [22].…”

Section: Discussionmentioning

confidence: 99%

Are HE4 and CA 125 suitable to detect a Paget disease of the vulva?

Dellino

Gargano

Carriero

et al. 2020

Preprint

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Background: Paget disease is a rare neoplasia, most commonly diagnosed in postmenopausal women and which can be identified in the breast (mammary Paget disease) or in other locations (extramammary Paget’s disease) such as ano-genital skin (Paget disease of the vulva -PVD). This condition is associated with low mortality, but a late diagnosis and recurrence can negatively impact the prognosis. Therefore, the main objective of this study is to evaluate if the human epididymis protein 4 (HE4) and cancer antigen125 (CA125) can promote recognition of PVD in early stages and during the relapses.Materials and Methods: we have conducted a prospective, observational and laboratory-based study, that included 50 patients, whose 25 healthy women represented the control group and 25 PVD patients, which have been operated in our Oncology Institute, from May 2017 to September 2019. Both in control group and in PVD patients, the CA-125 and HE4 were evaluated before surgery and after 6 months. Finally, a comparison of markers serum level, both between before/after surgery and with control group, and a ROC (Receiver Operating Characteristic) curve were performed.Results: Dosing the markers in PVD patients, 3/25 (12%) showed a higher value of CA125 and 11/25 (44%) an increased HE4. In addition, after surgical treatment there were no statistically significant difference between levels of CA-125 (p= 0.3) and HE4 (p=0.19). On the other hand, comparing HE4 in PVD patients with the control group, a statistically significant difference was found (p-value= 0.0036). Contrary, comparing CA-125 in PVD patients with the control group (p-value= 0.1969), no statistically significant difference was evidenced. Moreover, ROC (Receiver Operating Characteristic) curve showed low sensitivity and specificity for CA125 with area under curve (AUC) =0.5608. Instead, the ROC curve of HE4 revealed a sensitivity and specificity of 76% and 88% respectively (AUC= 0.7408) using a cut-off at 90 pmol/L.Conclusions: Despite the limited cases, our data showed that CA125 is not a sensitive marker for PVD. On the other hand, in 44% of PVD we’ve seen an increase in HE4. So, this could be a starting point for further research that could confirm the possibility to use this marker in order to support PVD early identification.

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Paget disease of the vulva an analysis of 24 cases

Cited by 14 publications

References 21 publications

A strengthening the reporting of observational studies in epidemiology (STROBE)

A strengthening the reporting of observational studies in epidemiology (STROBE)

Non‐human‐papillomavirus‐related malignancies of the vulva: A clinicopathological study

Are HE4 and CA 125 suitable to detect a Paget disease of the vulva?

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