Vulva, Vagina, and Anus

Abdul‐Karim, Fadi W.; Somrak, Theresa M.; Yang, Bin

doi:10.1016/b978-141604208-2.10011-9

Cited by 1 publication

(8 citation statements)

References 85 publications

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“…These cells are arranged in single or small clusters of tissue in a background of neutrophilic debris. 2,3 It has also been reported in the literature that PCs can have a squamouslike appearance. However, in these reported cases, the tridimensionality, papillary or acinar morphology, cells with vacuolated cytoplasm, and eccentric nucleoli allowed the diagnosis to be reached.…”

Section: Discussionmentioning

confidence: 91%

“…Caucasian people and women are more commonly affected. [1][2][3][4] The vulva is the most frequently involved site (65% of the cases). [1][2][3][4] Diagnosis of EMPD of the vulva represents a diagnostic challenge because it needs to be differentiated from its mimickers (Table 1).…”

Section: Discussionmentioning

confidence: 99%

“…Occasional signet ring cell appearance can be identified. 3 Histologically, the epidermis in EMPD is acanthotic due to the absence of desmosomes in the tumour cells. Erosion or ulceration may be present.…”

Section: Discussionmentioning

confidence: 99%

“…EMPD represents 6.5% of all cases of cutaneous Paget’s disease and mainly affects patients between the fifth and eighth decades of life, with a peak age incidence of 65.3 years old. Caucasian people and women are more commonly affected 1‐4 1‐4 …”

Section: Discussionmentioning

confidence: 99%

“…However, it accounts for only 1% of all vulvar neoplasms. 3 EMPD of the vulva is extremely rare and is characterised by a high recurrence rate, ranging from 12%-61%. 4 The rate of underlying adenocarcinoma that has been reported in the largest case series is 4%.…”

Section: Introductionmentioning

confidence: 99%

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Cytomorphological features of extramammary Paget’s disease of the vulva in a patient with diagnoses of squamous cell carcinoma of the cervix status post‐chemoradiation, and high‐grade vulvar intraepithelial neoplasia: The importance of basic principles

González

Duhoki

2021

Cytopathology

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Background: Extramammary Paget's disease (EMPD) is a rare neoplasm that commonly affects post-menopausal women. It usually presents in the anogenital area where apocrine sweat glands are abundant, most commonly in the vulva. The disease is characterised by slow growth and high local recurrence rates. Clinically, EMPD presents as well-demarcated erythematous lesions or plaques that may be ulcerated.Microscopically, it shows a group of atypical cells with abundant clear cytoplasm and nuclear pleomorphism. Methods:We present the case of a 58-year-old female with a history of poorly differentiated squamous cell carcinoma of the cervix, status post-radical-hysterectomy with bilateral salpingo-oophorectomy, and chemoradiation in 2016. The patient also had a long-standing history of vulvar pain, skin changes on her left labia, and itching.Results: Punch biopsies of the vulva revealed acanthosis and acantholysis of the epidermis with an intraepidermal scattering of single, or clusters of, large cells with round/oval nuclei and abundant clear cytoplasm. The cells were positive for p16, CK19, CK7, and PAX8, supporting the diagnosis of EMPD without evidence of dysplasia. The concurrent Papanicolaou smear showed a hypercellular specimen composed of hyperchromatic tissue fragments with high nuclear-to-cytoplasmic ratios and apoptotic bodies. The presence of intracytoplasmic mucin and the tridimensionality of the fragments supported the diagnosis of adenocarcinoma. Conclusions:This study compares the histological and cytomorphological features of EMPD with high-grade squamous intraepithelial lesions, since the pathogenesis, precursor lesions, etiological associations, staging, clinical treatment, and prognosis differ substantially and may have a significant clinical impact on the patient's treatment.

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Section: Discussionmentioning

confidence: 91%