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Extramammary Paget Disease

Extramammary Paget's Disease


Presentation

  • It is exceedingly rare for EMPD to present on the face, chest, or abdomen, and even more unusual for it to present as a pigmented lesion.[ncbi.nlm.nih.gov]
  • The clinical presentation of this disease is similar to the characteristics of Mammary Paget’s disease (MPD).[en.wikipedia.org]
Subcutaneous Nodule
  • A physical examination showed a pigmented vulvar, perianal erythematous plague, and a subcutaneous nodule in the left major labia.[ncbi.nlm.nih.gov]
  • By June 2011, a nonpainful, firm, occasionally hemorrhagic subcutaneous nodule was noted in the patient’s left upper scrotum. In September 2011, excision of the scrotal lesion revealed invasive empd.[current-oncology.com]
Scaly Rash
  • When an itchy or scaly rash is seen that persists, one should always have to to consider a malignant process, especially in older people.[ncbi.nlm.nih.gov]
Chronic Dermatitis
  • EMPD can occur in both benign or malignant form and is often confused with chronic dermatitis.[aboutcancer.com]

Workup

  • The diagnosis is based on clinical and histopathological findings and extensive workup to rule out an underlying primary carcinoma is crucial. Surgical excision is the gold standard.[omicsonline.org]
Lymphocytic Infiltrate
  • Histologically, EMPD exhibits abundant lymphocyte infiltration.[doi.org]

Treatment

  • More recently radiotherapy has been suggested as a suitable treatment when surgical excision or other modalities are not appropriate. We report a case of anogenital extramammary Paget disease and the clinical response to treatment with radiotherapy.[ncbi.nlm.nih.gov]

Prognosis

  • Prognosis is generally good, but factors such as depth of invasion and duration of disease need to be considered.[en.wikipedia.org]
  • These results indicate that therapies that target HER2 may be useful in treating metastatic EMPD, for which the prognosis is poor. However, there is limited information on the expression and gene amplification of HER2 in metastatic EMPD.[ncbi.nlm.nih.gov]

Etiology

  • The etiology of the disease is still unclear. We aimed to study the expression differences of vascular endothelial growth factor (VEGF) and basic fibroblast growth factor (bFGF) in EMPD tissues and corresponding adjacent normal tissues.[ncbi.nlm.nih.gov]
  • TERMINOLOGY Abbreviations • Extramammary Paget disease (EMPD) Definitions • Adenocarcinoma involving epidermis and commonly extending to epithelium of eccrine glands &/or hair follicles (adenocarcinoma in situ) • May involve dermis in minority of cases ETIOLOGY[basicmedicalkey.com]
  • Volume One; Volume Two; Basic Science Chapters; Therapeutic Ladders; Dermatology Website; Dedication; Acknowledgments; Section One Overview of Basic Science; 0 Basic Principles of Dermatology; Chapter Contents; Introduction to clinical dermatology; Etiologic[worldcat.org]

Epidemiology

  • OBJECTIVE: This study aimed to further characterize the epidemiology, clinical manifestations, pathology, immunopathology, outcome from therapy, and associated underlying malignancy in extramammary Paget disease (EMPD).[ncbi.nlm.nih.gov]
  • […] of cases – Genital EMPD is often associated with genitourinary carcinomas and perianal EMPD is more frequently associated with gastrointestinal carcinomas – Most frequent in perianal EMPD (underlying rectal carcinoma in 1/3 of cases) CLINICAL ISSUES Epidemiology[basicmedicalkey.com]
  • Historical and epidemiological aspects of vulvar Paget disease are summarized.[journals.lww.com]
Sex distribution
Age distribution

Pathophysiology

  • Pathophysiology The precise pathogenesis of EMPD is still the subject of controversy. The literature seems to indicate that extramammary Paget disease does not have a uniform histogenesis.[ncbi.nlm.nih.gov]
  • Its pathophysiology is still poorly understood. It is associated with synchronous or metachronous malignancies in 50% of cases [ 1 ].[omicsonline.org]
  • Please see the following for more information: Pathophysiology and Etiology Extramammary Paget disease (EMPD) arises as a primary cutaneous adenocarcinoma in most cases.[emedicine.medscape.com]
  • Pathophysiology MPD is largely believed to originate from underlying ductal carcinoma of the breast. Some, however, question whether Toker cells beget MPD, especially in the uncommon instances of MPD arising without underlying breast carcinoma.[dermatologyadvisor.com]
  • In triple or quadruple extra-mammary Paget’s disease, the racial and gender differences in incidence, the pathophysiology underlying simultaneous distant occurrence of three or four lesions, and their malignant potentials are the matters to be elucidated[medicaljournals.se]

Prevention

  • Awareness of these structures in vulvectomy specimens for EMPD is important to prevent misinterpretation of the syringoma-like lesions as an invasive component of the EMPD.[ncbi.nlm.nih.gov]
  • Unfortunately, the different treatment protocols chosen in these publications prevent from defining a clear protocol. Nevertheless, in our case, as in others [ 2 ], daily application was not well tolerated.[em-consulte.com]
  • Hence, exploring novel therapeutic strategies to prevent and treat metastatic EMPD is imperative. Table 1. Current systemic therapy for metastatic extramammary Paget’s disease in case studies and case reports.[doi.org]

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