Extramammary Paget's disease

Extramammary Paget's disease
Micrograph of extramammary Paget's disease, H&E stain
Classification and external resources
Specialty oncology, dermatology
ICD-10 C44 (ILDS C44.L75)
ICD-O M8542/3
MeSH D010145
Orphanet 2800

Extramammary Paget’s disease (EMPD), also extramammary Paget disease, is a rare, slow-growing, usually noninvasive intraepithelial (in the skin) adenocarcinoma outside the mammary gland and includes Paget's disease of the vulva and the extremely rare Paget's disease of the penis.[1]

Signs and symptoms

Symptoms are not specific; most patients report itching, burning, and soreness. A small subset of patients may be asymptomatic. Presence of vulvar pain, bleeding, and tumor formation are reported to be more common in patients affected by invasive disease.Signs and symptoms are skin lesions, often mistaken as eczema, that may be itchy or painful.

Pathophysiology

Micrograph of extramammary Paget's disease, H&E stain

Vulvar Paget disease presents as a variety of clinical lesions that may occur over a protracted course. Initially it is velvety, soft, and red or bright pink with scattered white islands of hyperkeratosis. (a strawberry and cream appearance) The lesions become erythematous, plaque like, and desquamating especially when located in dry areas. Rarely the appearance is ulcerated. The borders appear irregular, slightly elevated, and sharply demarcated. The visible borders of vulvar Paget disease are often misleading as Paget cells may spread along the basal layers of normal appearing skin with multicentric foci. Involvement may be extensive including the perianal region, genitocrural, and inguinal folds. Clinical examination should determine the presence of periurethral and perianal lesions. In these cases an involvement of the skin by a noncutaneous internal neoplasm may occur.[2]

Diagnosis

It is important to exclude that the lesion is associated with another cancer. A biopsy will establish the diagnosis. The histology of the lesion is the same as for Paget's disease of the breast.

Classification

Paget's disease of the vulva, a rare disease, may be a primary lesion or associated with adenocarcinoma originating from local organs such as the Bartholin gland, the urethra, or the rectum and thus be secondary. Patients tend to be postmenopausal.[3]

Paget's disease of the penis may also be primary or secondary, and is even rarer than genital Paget’s disease in women. At least one case has been misdiagnosed as Bowen's disease. Isolated Paget's disease of the penis is extremely rare.[1]

Treatment

Primary disease is usually treated by surgical excision.

Prognosis

Extramammary Paget's disease is usually seen in isolation and is associated with an underlying invasive malignancy about 12% of the time. It is associated with an underlying adnexal malignancy about 24% of the time. Paget's disease of the breast is almost always associated with an underlying invasive malignancy, i.e. breast cancer (e.g. mammary ductal carcinoma).[4]

History

James Paget described Paget's disease of the nipple in 1874. Radcliffe Crocker reported the first case of EMPD in 1889, when he described a patient with a skin lesion affecting the penis and scrotum, the findings of which were identical to those described by Paget.[5]

References

  1. 1 2 Ekwueme, Kingsley C; Zakhour, Hani D; Parr, Nigel J (1 January 2009). "Extramammary Paget's disease of the penis: a case report and review of the literature". Journal of Medical Case Reports. 3 (1): 4. doi:10.1186/1752-1947-3-4.
  2. ↑ Vulvar Paget Disease: One Century After First Reported Mario Preti, MD,1 Leonardo Micheletti, MD,1 Marco Massobrio, MD,1 Shin-ichi Ansai, MD, PhD,2 and Edward J. Wilkinson, MD3© 2003, American Society for Colposcopy and Cervical Pathology Journal of Lower Genital Tract Disease, Volume 7, Number 2, 2003, 122–135
  3. ↑ Fanning J, Lambert L, Hale TM, Morris PC, Schuerch C (1999). "Paget's disease of the vulva: prevalence of associated vulvar carcinoma, invasive Paget's disease, and recurrence after surgical excision". Am J Obstet Gynecol. 180: 24–7.
  4. ↑ Chanda, JJ. "Extramammary Paget's disease: prognosis and relationship to internal malignancy.". Journal of American Academy of Dermatology. 13 (6): 1009–14. PMID 3001158.
  5. ↑ Marques-Costa, JC; Cuzzi, T; Carneiro, S; Parish, LC; Ramos-e-Silva, M (May–Jun 2012). "Paget's disease of the breast.". Skinmed. 10 (3): 160–5. PMID 22779098.
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