Extramammary Paget's disease natural history, complications and prognosis: Difference between revisions

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{{CMG}}{{AE}}{{Simrat}}
{{CMG}}{{AE}}{{Simrat}}
==Overview==
==Overview==
If left untreated, the disease always takes a progressive course with no spontaneous recovery. Common complications of extramammary Paget's disease include recurrence of the [[tumor]]. Depending on the extent of the [[tumor]] at the time of [[diagnosis]], the [[prognosis]] may vary. The [[prognosis]] for primary Extramammary Pagets's disease (EMPD) confined to the [[epidermis]] is excellent.  However, [[Invasive (medical)|invasive]] primary extramammary Paget's disease (EMPD) carries a poor [[prognosis]], particularly if [[Infiltration (medical)|lymphovascular invasion]] is present.
If left untreated, the [[disease]] always takes a progressive course with no spontaneous recovery. Common [[complications]] of extramammary Paget's disease include recurrence of the [[tumor]]. Depending on the extent of the [[tumor]] at the time of [[diagnosis]], the [[prognosis]] may vary. The [[prognosis]] for primary Extramammary Pagets's disease (EMPD) confined to the [[epidermis]] is excellent.  However, [[Invasive (medical)|invasive]] primary extramammary Paget's disease (EMPD) carries a poor [[prognosis]], particularly if [[Infiltration (medical)|lymphovascular invasion]] is present.


==Natural History==
==Natural History==
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*Because of their benign appearance, the [[Lesion|lesions]] are often neglected for several years before being presented for medical attention.
*Because of their benign appearance, the [[Lesion|lesions]] are often neglected for several years before being presented for medical attention.
*The duration to local or even [[metastatic]] recurrence can vary from months to years, and extramammary Paget's disease can be associated with other [[Cancer|cancers]] in a metachronous or synchronous fashion.
*The duration to local or even [[metastatic]] recurrence can vary from months to years, and extramammary Paget's disease can be associated with other [[Cancer|cancers]] in a metachronous or synchronous fashion.
*With no spontaneous recovery, the disease always takes a progressive course, if not treated early.  
*With no spontaneous recovery, the [[disease]] always takes a progressive course, if not treated early.  
*The average interval between the diagnosis of [[Epithelium|intraepithelial]] [[Vulva|vulvar]] extramammary Paget's disease and its sequential progression to [[Carcinoma|invasive carcinoma]] amounts approximatively to 11 years.<ref name="MorettoNair2013">{{cite journal|last1=Moretto|first1=P.|last2=Nair|first2=V.J.|last3=El Hallani|first3=S.|last4=Malone|first4=S.|last5=Belanger|first5=E.|last6=Morash|first6=C.|last7=Canil|first7=C.M.|title=Management of penoscrotal extramammary Paget disease: case series and review of the literature|journal=Current Oncology|volume=20|issue=4|year=2013|pages=311|issn=1718-7729|doi=10.3747/co.20.1353}}</ref>
*The average interval between the [[diagnosis]] of [[Epithelium|intraepithelial]] [[Vulva|vulvar]] extramammary Paget's disease and its sequential progression to [[Carcinoma|invasive carcinoma]] amounts approximatively to 11 years.<ref name="MorettoNair2013">{{cite journal|last1=Moretto|first1=P.|last2=Nair|first2=V.J.|last3=El Hallani|first3=S.|last4=Malone|first4=S.|last5=Belanger|first5=E.|last6=Morash|first6=C.|last7=Canil|first7=C.M.|title=Management of penoscrotal extramammary Paget disease: case series and review of the literature|journal=Current Oncology|volume=20|issue=4|year=2013|pages=311|issn=1718-7729|doi=10.3747/co.20.1353}}</ref>


==Complications==
==Complications==
Common complications of extramammary Paget's disease include:
Common complications of extramammary Paget's disease include:
*Recurrence of the disease after treatment
*Recurrence of the disease after treatment
:*May be multifocal, where local recurrences are common even when the margins of resection are negative
:*May be multifocal, where local recurrences are common even when the margins of [[resection]] are negative
:*The recurrence rate of extramammary Paget's disease (EMPD) is 30% even with margin control
:*The recurrence rate of extramammary Paget's disease (EMPD) is 30% even with margin control
:*The average time to recurrence is 2.5 years, with case reports of more than 10 years follow-up
:*The average time to recurrence is 2.5 years, with case reports of more than 10 years follow-up
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==Prognosis==
==Prognosis==
*The prognosis for primary extramammary Pagets's disease confined to the epidermis is excellent.
*The [[prognosis]] for primary extramammary Pagets's disease confined to the [[Epidermis (skin)|epidermis]] is excellent.
*However, invasive primary extramammary Paget's disease (EMPD) carries a poor prognosis, particularly if lymphovascular invasion is present.  
*However, [[Invasive (medical)|invasive]] primary extramammary Paget's disease (EMPD) carries a poor [[prognosis]], particularly if lymphovascular invasion is present.  
*Poor prognostic factors include the following:<ref name="MorettoNair2013">{{cite journal|last1=Moretto|first1=P.|last2=Nair|first2=V.J.|last3=El Hallani|first3=S.|last4=Malone|first4=S.|last5=Belanger|first5=E.|last6=Morash|first6=C.|last7=Canil|first7=C.M.|title=Management of penoscrotal extramammary Paget disease: case series and review of the literature|journal=Current Oncology|volume=20|issue=4|year=2013|pages=311|issn=1718-7729|doi=10.3747/co.20.1353}}</ref><ref name="pmid9331290">{{cite journal| author=Goldblum JR, Hart WR| title=Vulvar Paget's disease: a clinicopathologic and immunohistochemical study of 19 cases. | journal=Am J Surg Pathol | year= 1997 | volume= 21 | issue= 10 | pages= 1178-87 | pmid=9331290 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9331290  }} </ref><ref name="pmid9500217">{{cite journal| author=Goldblum JR, Hart WR| title=Perianal Paget's disease: a histologic and immunohistochemical study of 11 cases with and without associated rectal adenocarcinoma. | journal=Am J Surg Pathol | year= 1998 | volume= 22 | issue= 2 | pages= 170-9 | pmid=9500217 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9500217  }} </ref><ref>DeVita, Vincent T., Theodore S. Lawrence, and Steven A. Rosenberg. DeVita, Hellman, and Rosenberg's cancer : principles & practice of oncology. Philadelphia: Wolters Kluwer Health/Lippincott Williams & Wilkins, 2011. Print.</ref><ref name="pmid11064666">{{cite journal| author=Lloyd J, Flanagan AM| title=Mammary and extramammary Paget's disease. | journal=J Clin Pathol | year= 2000 | volume= 53 | issue= 10 | pages= 742-9 | pmid=11064666 | doi= | pmc=PMC1731095 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11064666  }} </ref><ref name="MannLavaf2012">{{cite journal|last1=Mann|first1=J.|last2=Lavaf|first2=A.|last3=Tejwani|first3=A.|last4=Ross|first4=P.|last5=Ashamalla|first5=H.|title=Perianal Paget disease treated definitively with radiotherapy|journal=Current Oncology|volume=19|issue=6|year=2012|issn=11980052|doi=10.3747/co.19.1144}}</ref>
*Poor [[Prognosis|prognostic]] factors include the following:<ref name="MorettoNair2013">{{cite journal|last1=Moretto|first1=P.|last2=Nair|first2=V.J.|last3=El Hallani|first3=S.|last4=Malone|first4=S.|last5=Belanger|first5=E.|last6=Morash|first6=C.|last7=Canil|first7=C.M.|title=Management of penoscrotal extramammary Paget disease: case series and review of the literature|journal=Current Oncology|volume=20|issue=4|year=2013|pages=311|issn=1718-7729|doi=10.3747/co.20.1353}}</ref><ref name="pmid9331290">{{cite journal| author=Goldblum JR, Hart WR| title=Vulvar Paget's disease: a clinicopathologic and immunohistochemical study of 19 cases. | journal=Am J Surg Pathol | year= 1997 | volume= 21 | issue= 10 | pages= 1178-87 | pmid=9331290 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9331290  }} </ref><ref name="pmid9500217">{{cite journal| author=Goldblum JR, Hart WR| title=Perianal Paget's disease: a histologic and immunohistochemical study of 11 cases with and without associated rectal adenocarcinoma. | journal=Am J Surg Pathol | year= 1998 | volume= 22 | issue= 2 | pages= 170-9 | pmid=9500217 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9500217  }} </ref><ref>DeVita, Vincent T., Theodore S. Lawrence, and Steven A. Rosenberg. DeVita, Hellman, and Rosenberg's cancer : principles & practice of oncology. Philadelphia: Wolters Kluwer Health/Lippincott Williams & Wilkins, 2011. Print.</ref><ref name="pmid11064666">{{cite journal| author=Lloyd J, Flanagan AM| title=Mammary and extramammary Paget's disease. | journal=J Clin Pathol | year= 2000 | volume= 53 | issue= 10 | pages= 742-9 | pmid=11064666 | doi= | pmc=PMC1731095 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11064666  }} </ref><ref name="MannLavaf2012">{{cite journal|last1=Mann|first1=J.|last2=Lavaf|first2=A.|last3=Tejwani|first3=A.|last4=Ross|first4=P.|last5=Ashamalla|first5=H.|title=Perianal Paget disease treated definitively with radiotherapy|journal=Current Oncology|volume=19|issue=6|year=2012|issn=11980052|doi=10.3747/co.19.1144}}</ref>
:*Deep invasion: less than 1 mm dermal invasion has more favorable prognosis than lesions showing deeper invasion
:*Deep [[Invasive (medical)|invasion]]: less than 1 mm [[Dermis|dermal]] [[Invasive (medical)|invasion]] has more favorable [[prognosis]] than [[Lesion|lesions]] showing deeper [[invasion]]
:*Lymphovascular involvement
:*Lymphovascular involvement
:*Presence of an underlying adnexal adenocarcinoma
:*Presence of an underlying [[adnexal]] [[adenocarcinoma]]
:*Primary anorectal extramammary Paget's diesease
:*Primary [[anorectal]] extramammary Paget's diesease
:*Distant metastasis
:*Distant [[metastasis]]
:*Older age
:*Older age
:*[[Radiotherapy]] as monotherapy
:*[[Radiotherapy]] as [[monotherapy]]
:*Perianal disease
:*Perianal disease


Latest revision as of 13:16, 10 April 2019

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Simrat Sarai, M.D. [2]

Overview

If left untreated, the disease always takes a progressive course with no spontaneous recovery. Common complications of extramammary Paget's disease include recurrence of the tumor. Depending on the extent of the tumor at the time of diagnosis, the prognosis may vary. The prognosis for primary Extramammary Pagets's disease (EMPD) confined to the epidermis is excellent. However, invasive primary extramammary Paget's disease (EMPD) carries a poor prognosis, particularly if lymphovascular invasion is present.

Natural History

  • Early clinical presentation of extramammary Paget's disease consists of multifocal or single dry, raised, erythematous, and slow-growing lesions.[1]
  • If left untreated, the lesions may evolve into crusted, eczematoid, ulcerated, or papillary lesions.[2][3][4][5][6][1]
  • Because of their benign appearance, the lesions are often neglected for several years before being presented for medical attention.
  • The duration to local or even metastatic recurrence can vary from months to years, and extramammary Paget's disease can be associated with other cancers in a metachronous or synchronous fashion.
  • With no spontaneous recovery, the disease always takes a progressive course, if not treated early.
  • The average interval between the diagnosis of intraepithelial vulvar extramammary Paget's disease and its sequential progression to invasive carcinoma amounts approximatively to 11 years.[1]

Complications

Common complications of extramammary Paget's disease include:

  • Recurrence of the disease after treatment
  • May be multifocal, where local recurrences are common even when the margins of resection are negative
  • The recurrence rate of extramammary Paget's disease (EMPD) is 30% even with margin control
  • The average time to recurrence is 2.5 years, with case reports of more than 10 years follow-up
  • Metastasis

Prognosis

The relationship between extent of the disease and five-year survival is shown below in a tabular form:

Extent of disease Five-year survival
Localized disease

95%

Regional disease

85%

Distant metastasis

52%

References

  1. ↑ 1.0 1.1 1.2 1.3 Moretto, P.; Nair, V.J.; El Hallani, S.; Malone, S.; Belanger, E.; Morash, C.; Canil, C.M. (2013). "Management of penoscrotal extramammary Paget disease: case series and review of the literature". Current Oncology. 20 (4): 311. doi:10.3747/co.20.1353. ISSN 1718-7729.
  2. ↑ 2.0 2.1 Goldblum JR, Hart WR (1997). "Vulvar Paget's disease: a clinicopathologic and immunohistochemical study of 19 cases". Am J Surg Pathol. 21 (10): 1178–87. PMID 9331290.
  3. ↑ 3.0 3.1 Goldblum JR, Hart WR (1998). "Perianal Paget's disease: a histologic and immunohistochemical study of 11 cases with and without associated rectal adenocarcinoma". Am J Surg Pathol. 22 (2): 170–9. PMID 9500217.
  4. ↑ DeVita, Vincent T., Theodore S. Lawrence, and Steven A. Rosenberg. DeVita, Hellman, and Rosenberg's cancer : principles & practice of oncology. Philadelphia: Wolters Kluwer Health/Lippincott Williams & Wilkins, 2011. Print.
  5. ↑ 5.0 5.1 Lloyd J, Flanagan AM (2000). "Mammary and extramammary Paget's disease". J Clin Pathol. 53 (10): 742–9. PMC 1731095. PMID 11064666.
  6. ↑ 6.0 6.1 Mann, J.; Lavaf, A.; Tejwani, A.; Ross, P.; Ashamalla, H. (2012). "Perianal Paget disease treated definitively with radiotherapy". Current Oncology. 19 (6). doi:10.3747/co.19.1144. ISSN 1198-0052.
  7. ↑ DeVita, Vincent T., Theodore S. Lawrence, and Steven A. Rosenberg. DeVita, Hellman, and Rosenberg's cancer : principles & practice of oncology. Philadelphia: Wolters Kluwer Health/Lippincott Williams & Wilkins, 2011. Print.
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