Extramammary Paget's disease natural history, complications and prognosis
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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.[1][2][3][4][5][6]
Natural History
- Early clinical presentation of extramammary Paget's disease consists of multifocal or single dry, raised, erythematous, and slow-growing lesions.[6]
- If left untreated, the lesions may evolve into crusted, eczematoid, ulcerated, or papillary lesions.
- 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.[6]
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 prognosis for primary extramammary Pagets's disease confined to the epidermis is excellent.
- However, invasive primary extramammary Paget's disease (EMPD) carries a poor prognosis, particularly if lymphovascular invasion is present.
- Poor prognostic factors include the following:[6][1][2][7][4][5]
- Deep invasion: less than 1 mm dermal invasion has more favorable prognosis than lesions showing deeper invasion
- Lymphovascular involvement
- Presence of an underlying adnexal adenocarcinoma
- Primary anorectal extramammary Paget's diesease
- Distant metastasis
- Older age
- Radiotherapy as monotherapy
- Perianal disease
The relationship between extent of the disease and five-year survival is shown below in a tabular form:
| Extent of disease | Five-year survival |
|---|---|
|
95% |
|
85% |
|
52% |
References
- ↑ 1.0 1.1 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.
- ↑ 2.0 2.1 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.
- ↑ 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.
- ↑ 4.0 4.1 Lloyd J, Flanagan AM (2000). "Mammary and extramammary Paget's disease". J Clin Pathol. 53 (10): 742–9. PMC 1731095. PMID 11064666.
- ↑ 5.0 5.1 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.
- ↑ 6.0 6.1 6.2 6.3 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.
- ↑ 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.