Extramammärer Morbus Paget

Wagner, Gunnar; Sachse, Michael Max

doi:10.1111/j.1610-0387.2010.07581_suppl.x

Cited by 9 publications

(2 citation statements)

References 38 publications

(69 reference statements)

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“…Previous studies have shown that EMPD constitutes only 6.5% of all cases of Paget's disease (2). As the clinical manifestation of EMPD is similar to that of eczema or dermatitis, pathological examination may be delayed.…”

Section: Introductionmentioning

confidence: 99%

Human epidermal growth factor receptor 2 amplification as a biomarker for treatment in patients with lymph node‑metastatic penoscrotal extramammary Paget's disease

et al. 2019

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The role of human epidermal growth factor receptor 2 (HER2) amplification as a biomarker for treatment in patients with lymph node (LN)-metastatic penoscrotal extramammary Paget's disease (EMPD) was investigated in the present study. A total of 11 male patients with LN-metastatic penoscrotal EMPD were retrospectively reviewed. Positron emission tomography/computed tomography (PET/CT) was conducted prior to surgery. Immunohistochemistry and fluorescence in situ hybridization were used to evaluate HER2 gene amplification in LN samples. Sanger sequencing was used to investigate HER2 mutations. A literature review of the prevalence of HER2 amplification in EMPD and the efficacy of HER2-targeted therapy was also undertaken. PET/CT is effective in detecting metastatic sites. The sensitivity and specificity of PET/CT was 90.9 and 100.0% for inguinal LNs, and 85.7 and 80.0% for pelvic LNs, respectively. The median time from LN dissection to disease progression was 15.9±1.5 months. Of the 11 patients, 3 (27.3%) indicated HER2 amplification. Patients with HER2 amplification showed shorter median times from disease discovery to LN metastasis (HER2 amplification vs HER2 non-amplification; 15.6 vs. 10.0 months; P=0.50) and from LN dissection to disease progression (HER2 amplification vs. HER2 non-amplification, 16.2 vs. 13.6 months; P=0.11). However, the aforementioned observations were not indicated to be statistically significant. No HER2 mutations were identified. Trastuzumab, a HER2-targeted monoclonal antibody, was administered to 2 of the patients with HER2 amplification. A literature review of the prevalence of HER2 amplification in EMPD and the efficacy of HER2-targeted therapy showed similar results. Altogether, 485 cases of EMPD were reported, 35 of which had metastases. The results in the present study suggest that PET/CT should be used on all metastatic EMPD patients. EMPD may be effectively treated with trastuzumab. The present study and case reports from the literature provide evidence for the benefit of testing for HER2 amplification in this rare disease and highlight the requirement for a multicenter clinical trial to assess the impact of trastuzumab therapy in treating this disease.

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Section: Introductionmentioning

confidence: 99%

Human epidermal growth factor receptor 2 amplification as a biomarker for treatment in patients with lymph node‑metastatic penoscrotal extramammary Paget's disease

et al. 2019

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“…Zu den Prädilektionsstellen zäh-len anogenitale Lokalisationen und seltener die Achseln [7]. Klinisch finden sich hell-bis dunkelrote, scharf und umschrieben polygonal begrenzte Erytheme mit Erosionen und Krusten, pityriasiformer bis groblamellöser Schuppung sowie vereinzelt pflastersteinartig angeordneten Papeln oder Keratosen [25]. Die Therapie der Wahl des primären EMP ist die chirurgische Exzision.…”

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2011

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A 90-year-old woman presented with an extensive primary form of extramammary Paget disease localized in the anogenital region. An associated carcinoma could be excluded. Due to the age of the patient, the location and extent of the tumor as well as existing comorbidities, neither a surgical nor a radio-oncological treatment were advisable. A local treatment with imiquimod 5% cream applied 3 times weekly for 2 × 3 weeks led to a clinically and histopathologically complete remission. As also shown by other authors, imiquimod appears to be a treatment option for the primary form of extramammary Paget disease.

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Cutaneous Markers of Internal Malignancy

Emtestam

Sartorius

2016

Rook's Textbook of Dermatology, Ninth Edition

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Different types of skin change are associated with internal malignancy. Tumour may spread to the skin from adjacent tissues, or may be deposited in the skin by metastasis. More typically dermatologists are called upon to assess paraneoplastic phenomena involving the skin, or to comment on dermatoses which are known, in some cases, to be markers of internal malignancy. Certain rare, inherited conditions, which have skin manifestations, carry a risk for the development of internal neoplasia. In other paraneoplastic settings, dysfunction of physiological activities, such as flushing or clotting, can lead the dermatologist to suspect an associated cancer. Potential cutaneous markers of internal malignancy vary in their reliability for predicting underlying neoplasia. The extent and intensity of the investigations for malignancy should therefore be tempered by a general assessment of the patient.

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Extramammärer Morbus Paget – Klinik, Histogenese, Therapiemöglichkeiten

Cited by 9 publications

References 38 publications

Human epidermal growth factor receptor 2 amplification as a biomarker for treatment in patients with lymph node‑metastatic penoscrotal extramammary Paget's disease

Human epidermal growth factor receptor 2 amplification as a biomarker for treatment in patients with lymph node‑metastatic penoscrotal extramammary Paget's disease

Extramammärer Morbus Paget

Cutaneous Markers of Internal Malignancy

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