Unusual Manifestations of Vulval Paget Disease – Cotinis the genus of scarab beetles

Vulval extramammary Paget disease (vEMPD) is an uncommon epithelial malignancy that may arise within the vulva (primary vEMPD) or represent vulval skin involvement by a noncutaneous carcinoma (secondary vEMPD). Primary vEMPD is most often an in situ carcinoma arising within the epidermis but may be associated with dermal invasion (invasive vEMPD) or represent intraepidermal spread of an adenocarcinoma originating in vulval skin adnexa or anogenital mammary-like glands.

The latter, termed mammary gland-like adenocarcinoma (MGLA), exhibits morphologic, immunohistochemical, and molecular features of various breast carcinomas but, as far as we are aware, the metaplastic variant of MGLA has not been reported on the vulva.

We report 2 cases of metaplastic MGLA of the vulva with associated Paget disease and postulate that some cases of vulval MGLA may arise from Paget disease rather than originating in mammary-like glands.

We also report a unique case of secondary vEMPD resulting from spread of urothelial carcinoma in situ that subsequently progressed to invasive urothelial carcinoma within the vulva.

Fibroadenoma in vulval ectopic breast tissue in a patient with PTEN Hamartoma Tumour Syndrom

PTEN is a tumour suppressor gene involved in regulating cell division. Pathogenic germline variants in PTEN predispose to benign and malignant growths of numerous organs, including of the breast.

In the following report, we describe the first documented case of a fibroadenoma developing in ectopic breast tissue of the vulva in a patient with a germline pathogenic variant in PTEN.

This highlights the risk of hyperplasia developing in any breast tissue, including rare ectopic sites, particularly in patients with underlying germline variants in cancer susceptibility genes.

Keywords: Cowden syndrome; Ectopic breast tissue; PTEN; PTEN hamartoma tumour syndrome; Vulval fibroadenoma.

Metastatic Vulval Crohn’s Disease With Good Outcome on Ustekinumab

Vulval Crohn’s disease is a rare manifestation of Crohn’s disease. Although it is usually caused by a fistulating process, it may be a result of a metastatic disease process as well, the exact mechanisms of which are not well understood yet.

Vulval Crohn’s disease may occur before the onset of bowel symptoms and may continue to be active while bowel symptoms are quiescent. Coupled with the fact that most vulval Crohn’s lesions are similar to other disease processes, it can prove to be a diagnostic challenge.

Due to its rarity, no guidelines for treatment exist; however, most reports agree that when symptoms are not controlled with antibiotics and topical steroids, immunosuppressive medications such as oral steroids and biological agents are the way forward.

Although surgery may be an option, data suggest that its use is scarce. We report the case of a 47-year-old Caucasian woman with bilateral metastatic vulval Crohn’s disease from a district hospital in the United Kingdom who responded well to ustekinumab. This is one of the first few cases reporting good clinical outcomes with the agent.

Invasive Vulval Paget’s disease treated with primary radiotherapy: A rare case report and literature review

Extramammary Vulval Paget’s Disease (VPD) is rare neoplasm of post-menopausal women. In relatively young perimenopausal patients, it can cause both diagnostic dilemma and therapeutic challenges. Majority of VPD is of non-invasive variety.

Among invasive Paget’s disease, only 20% cases show invasion more than 1 mm. The present report describes a unique case of an invasive extra-mammary VPD with depth of invasion more than 4 mm presenting at a relatively young perimenopausal lady.

Only 14 cases of VPD has been treated with primary radiotherapy in literature till date. We report this case to be the 15th case where radiotherapy was solely used to treat an invasive VPD.

Invasive cervical adenocarcinoma arising from extension of recurrent vulval Paget’s disease

An 83-year-old woman with a long-standing history of both invasive and vulval extramammary Paget’s disease (EMPD) was referred to a tertiary gynaecological oncology service for suspicion of contiguous extension to the vagina and cervix.

Vaginal biopsies confirmed EMPD; however, a loop excision of the cervix demonstrated invasive adenocarcinoma arising from Paget’s disease. The patient subsequently underwent laparoscopic radical hysterectomy, bilateral salpingo-oophorectomy and radical upper vaginectomy, confirming FIGO stage 1B1 cervical adenocarcinoma. She was recommended but declined to have adjuvant pelvic external beam radiotherapy.

To our knowledge this is the second case of invasive cervical adenocarcinoma arising from vulval Paget’s disease in the literature.

Vulval Intestinal/Enteric Heterotopia in a Patient with Crohn’s Disease.

Intestinal/enteric heterotopia of the vulva is an extremely rare disease with only 3 cases described in the literature. We report here an unusual case of this disease occurring in a 26-year-old patient in a context of Crohn’s disease.

To the best of our knowledge, such type of association has not been previously described. The potential origins of these lesions including metaplastic transformation, dysontogenetic changes, or epithelial colonic displacement/implantation are discussed.

The Vulval Disease Quality of Life Index in women with vulval lichen sclerosus correlates with clinician and symptom scores.

The Vulval disease Quality of Life Index (VQLI) is a new tool that assesses the burden of vulval disease on quality of life (QoL). Our objective was to assess the correlation between VQLI score and clinician-rated severity scores, overall patient itch/discomfort, disease duration, sexual activity, and age, in vulval lichen sclerosus (VLS) at a vulval disorders clinic.

A retrospective case note review, including consecutive women with VLS who attended the clinic between April and October 2018. Outcome measures include the VQLI score, clinician-rated severity score, and patient symptom score.A total of 109 women with VLS were included.

On multivariable analysis, there was evidence of a positive relationship between VQLI scores and the total clinician-rated score (mean increase in VQLI score per unit increase in clinician score 1.34, 95% confidence interval [CI] 0.31, 2.38; P = 0.01); the relationship was stronger for the cutaneous component.

There was little evidence for relationships of the VQLI with the patient’s age, sexual activity or time since onset of symptoms.

There was strong evidence for a positive relationship between VQLI score and overall itch/discomfort score (mean increase 2.38, 95% CI 1.88, 2.88; P < 0.001).

New and follow-up data were obtained on sequential visits for 12 women, among whom the VQLI score dropped a mean -2.75 points between visits (95% CI -6.05, 0.55; P = 0.094).The clinician-rated severity correlates with the impact of VLS on QoL. The VQLI captures information included in a patient itch/discomfort score, which can be easily incorporated into routine assessment.

‘Primary gingival and later primary vulval carcinomas arising in lichen planus: report of a case and clinical suggestions for diagnosis of a neglected disease‘.

Lichen planus (LP) is a chronic immune-mediated dermatosis mainly affecting skin, oral, and genital mucosa. The heterogeneous clinical presentation, spectrum of symptoms depending on subtype and overlap with other vulval and cutaneous disorders can lead to challenging in diagnosis.

We report an unusual case of vulval SCC arising within a patient with initial oral mucosal lichen planus who later developed lichen planus of the vulva.

Discussion of this case is important as it typifies the difficulties in diagnosis of vulvo-vaginal disorders and potential complications. Evidence is available that lichen planus may be potentially precancerous condition and is associated with SCC development.

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