Stop routine screening for associated malignancies in cutaneous noninvasive vulvar Paget disease?

Linden, M. van der; Schuurman, M.S.; Bulten, Johan; Massuger, Leon F.A.G.; IntHout, Joanna; Aa, M.A. van der; Hullu, Joanne A. de

doi:10.1111/bjd.16894

Cited by 18 publications

(11 citation statements)

References 17 publications

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“…Common sites of the associated malignancies were the colon, rectum, prostate, and bladder; therefore, consideration of screening for internal malignancies might be required when EMPD is diagnosed. However, a recent population study with age-matched control showed no increased risk of associated malignancies in noninvasive primary vulvar EMPD patients, indicating that risk of other malignancies have been overestimated in large elderly population of EMPD patients [89,90]. From this finding, the authors suggest that routine screening for other malignancies in patients diagnosed with primary noninvasive vulvar EMPD may not be needed.…”

Section: Patient Evaluationmentioning

confidence: 90%

Extramammary Paget’s Disease: Diagnosis, Pathogenesis, and Treatment with Focus on Recent Developments

Ishizuki

Nakamura

2021

Current Oncology

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Extramammary Paget’s disease (EMPD) is a rare neoplasm that usually develops in apocrine gland-bearing areas, such as the vulva, scrotum, and penis. EMPD may present with a focal, multifocal, or an ectopic lesion. Clinically, EMPD lesions often exhibit infiltrative erythema, which is sometimes similar to other skin disorders such as eczema. While primary EMPD arises as intraepithelial neoplasm of the epidermis, EMPD-like lesions may occur from epidermotropic spread of malignant cells or direct extension from an underlying internal neoplasm, known as secondary EMPD. Because treatment strategies differ for primary EMPD and secondary EMPD, accurate diagnosis based on detailed histopathological evaluation is required. In the early stages, EMPD usually shows indolent growth, and most cases are diagnosed as carcinoma in situ. However, invasive lesions may result in metastases, and deep invasion is associated with high incidence of metastases. Conventional chemotherapies have been used for EMPD treatment in patients with distant metastases, but the efficacy is not satisfactory, and the prognosis for such patients remains poor. Recent studies have provided various insights into the molecular pathogenesis of the development and advancement of EMPD, which may lead to novel treatment approaches for metastatic EMPD. This review addresses the diagnosis, pathogenesis, and treatment of EMPD with focus on recent progress in understanding this disease.

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Section: Patient Evaluationmentioning

confidence: 90%

Extramammary Paget’s Disease: Diagnosis, Pathogenesis, and Treatment with Focus on Recent Developments

Ishizuki

Nakamura

2021

Current Oncology

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“…Although the rate of local recurrence is high in all patients with EMPD (approximately 40%), the risk of other malignancies is limited to patients with invasive disease (8% of cases) and patients with secondary EMPD [8][9][10]. Most cases of EMPD are primary and non-invasive.…”

Section: Discussionmentioning

confidence: 99%

“…Immunohistochemical analysis may aid in identifying secondary EMPD. Primary lesions are CK7+/CK20-; lesions with intestinal phenotype are CK7 −/CK20+/CDX2+; lesions with urothelial phenotype are CK7+/CK20+/uroplakin+ [8]. EMPD of urothelial origin is the rarest type of secondary EMPD, and urothelial neoplasia can appear before or up to 13 yr after the onset of vulvar symptoms [11][12][13].…”

Section: Discussionmentioning

confidence: 99%

Vulvar Paget disease secondary to high-grade urothelial carcinoma with underlying massive vascular embolization and cervical involvement: case report of unusual presentation

Primo

Basílio

et al. 2019

Diagn Pathol

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Background Vulvar extramammary Paget disease is a rare chronic condition, that presents with non-specific symptoms such as pruritus and eczematous lesions. Because most of these lesions are noninvasive, the distinction between primary and secondary Paget disease is crucial to management. Case presentation We report an unusual case of vulvar Paget disease associated with massive dermal vascular embolization, cervicovaginal involvement and metastasis to inguinal and retroperitoneal lymph nodes. The intraepithelial vulvar lesion had a classical appearance and was accompanied by extensive component of dermal lymphovascular tumor emboli, similar to those observed in inflammatory breast carcinoma. Immunohistochemical analysis revealed that the lesion was secondary to high-grade urothelial cell carcinoma. The patient had a history of superficial low-grade papillary urothelial carcinoma of the bladder, which had appeared 2 years before the onset of vulvar symptoms. Conclusions Eczematoid vulvar lesions merit careful clinical examination and biopsy, including vulva mapping and immunohistochemistry. The information obtained may help to define and classify a particular presentation of Paget disease. Noninvasive primary lesions do not require the same aggressive approaches required for the treatment of invasive and secondary disease.

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“…Based on a recent study by Van der Linden [1], however, secondary EMPD does not increase the risk of malignancy compared with the general population. Up until now, not so many familial EMPD cases have been reported.…”

Section: Introductionmentioning

confidence: 94%

A Case Report of Familial Extramammary Paget's Disease in Female Siblings

Rutnumnoi

Leeyaphan²

2021

Case Rep Dermatol

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Extramammary Paget’s disease (EMPD) is a rare intraepithelial neoplasm that occurs in apocrine-bearing areas of skin. Most EMPD patients initially present with chronic pruritic eczematous lesions involving genitalia, perineum and perianal area. Familial form of EMPD is extremely rare. Several genetic mutations have been proposed but specific modes of inheritance are still unknown. This article reports two cases of familial extramammary Paget’s disease in female siblings.

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Stop routine screening for associated malignancies in cutaneous noninvasive vulvar Paget disease?

Cited by 18 publications

References 17 publications

Extramammary Paget’s Disease: Diagnosis, Pathogenesis, and Treatment with Focus on Recent Developments

Extramammary Paget’s Disease: Diagnosis, Pathogenesis, and Treatment with Focus on Recent Developments

Vulvar Paget disease secondary to high-grade urothelial carcinoma with underlying massive vascular embolization and cervical involvement: case report of unusual presentation

A Case Report of Familial Extramammary Paget's Disease in Female Siblings

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