Paget's disease

Paget's disease most commonly occurs in the breast, where most patients have an underlying ductal carcinoma. It has also been reported from extramammary sites, most commonly the vulva but also the perianal region, perineum, groin, pubic area, scrotum, penis, thigh, buttock, axilla and eyelids. There is usually no underlying carcinoma. The histogenesis of extramammary Paget's disease is controversial. The mucin profile of vulval Paget's disease most closely matches that of the Bartholin's gland and of clear cell papulosis (described in children and characterised by multiple small maculopapules composed of clear cells resembling the Toker cells of the nipple and thought to represent ectopic eccrine cells¹)

Immunohistochemistry

		mucins			cytokeratins
		MUC1	MUC2	MUC5AC	CK7	CK20
breast	normal mammary gland	10/101	0/101	0/101	10/101	0/101
	Toker cells	5/51	0/51	0/51	5/51	0/51
	ductal carcinoma	13/131	0/131	0/131	13/131	0/131
	Paget's disease	13/131	0/131	0/131	13/131	0/131
GI tract	rectal mucosa	0/101	10/101	3/101	6/101	10/101
	anal glands	0/61	0/61	6/61	6/61	0/61
	perianal Paget's disease	1/31	3/31	1/31	1/31	3/31
vulva	eccrine glands	12/121	0/121	0/121	12/121	0/121
	apocrine glands	8/81	0/81	0/81	8/81	0/81
	mammary-like glands	6/61	0/61	0/61	6/61	0/61
	Bartholin's glands	7/71	0/71	7/71	7/71	0/71
	clear cell papulosis	1/11	0/11	1/11	1/11	0/11
	vulval Paget's disease	8/71	0/71	7/71	8/71	5/71
	vulval Paget's with underlying apocrine carcinoma	1/11	0/11	0/11	1/11	0/11
male genitalia	scroto-penile Paget's disease	2/21	0/21	2/21	2/21	0/21

Both cytokeratin 7 and MUC1 may be useful in the identification of Paget's cells.

References

¹ Kuan, S. F., Montag, A. G., Hart, J., Krausz, T., Recant, W. Differential Expression of Mucin Genes in Mammary and Extramammary Paget's Disease. Am J Surg Pathol 2001;25:1469-1477

² Liegl B, Horn LC,Moinfar F. Androgen receptors are frequently expressed in mammary and extramammary Paget's disease. Mod Pathol 2005; 18:1283-8

This page last revised 1.4.2006.

©SMUHT/PW Bishop