For other diseases named after Paget, see Paget's disease (disambiguation).
| Extramammary Paget's disease |
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Micrograph of extramammary Paget's disease, H&E stain
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| Classification and external resources |
| Specialty |
oncology, dermatology |
| ICD-10 |
C44 (ILDS C44.L75) |
| ICD-O |
M8542/3 |
| MeSH |
D010145 |
Extramammary Paget’s disease (EMPD), also extramammary Paget disease, is a rare, slow-growing, usually noninvasive intraepithelial (in the skin) adenocarcinoma outside the mammary gland and includes Paget's disease of the vulva and the extremely rare Paget's disease of the penis.[1]
Contents
- 1 Classification
- 2 Signs and symptoms
- 3 Pathophysiology
- 4 Diagnosis
- 5 Treatment
- 6 Prognosis
- 7 History
- 8 References
- 9 External links
Classification
Paget's disease of the vulva, a rare disease, may be a primary lesion or associated with adenocarcinoma originating from local organs such as the Bartholin gland, the urethra, or the rectum and thus be secondary. Patients tend to be postmenopausal. [2]
Paget's disease of the penis may also be primary or secondary, and is even rarer than genital Paget’s disease in women. At least one case has been misdiagnosed as Bowen's disease. Isolated Paget's disease of the penis is extremely rare.[1]
Signs and symptoms
Symptoms are not specific; most patients report itching, burning, and soreness. A small subset of patients may be asymptomatic. Presence of vulvar pain, bleeding, and tumor formation are reported to be more common in patients affected by invasive disease.
Signs and symptoms are skin lesions, often mistaken as eczema, that may be itchy or painful.
Pathophysiology
Micrograph of extramammary Paget's disease, H&E stain
See also: Paget's disease of the breast § Pathophysiology
Vulvar Paget disease presents as a variety of clinical lesions that may occur over a protracted course. Initially it is velvety, soft, and red or bright pink with scattered white islands of hyperkeratosis. (a strawberry and cream appearance) The lesions become erythematous, plaque like, and desquamating especially when located in dry areas. Rarely the appearance is ulcerated. The borders appear irregular, slightly elevated, and sharply demarcated. The visible borders of vulvar Paget disease are often misleading as Paget cells may spread along the basal layers of normal appearing skin with multicentric foci. Involvement may be extensive including the perianal region, genitocrural, and inguinal folds. Clinical examination should determine the presence of periurethral and perianal lesions. In these cases an involvement of the skin by a noncutaneous internal neoplasm may occur.[3]
Diagnosis
It is important to exclude that the lesion is associated with another cancer. A biopsy will establish the diagnosis. The histology of the lesion is the same as for Paget's disease of the breast.[citation needed]
Treatment
Primary disease is usually treated by surgical excision.[citation needed]
Prognosis
Extramammary Paget's disease is usually seen in isolation and is associated with an underlying invasive malignancy about 12% of the time. It is associated with an underlying adnexal malignancy about 24% of the time. Paget's disease of the breast is almost always associated with an underlying invasive malignancy, i.e. breast cancer (e.g. mammary ductal carcinoma).[4]
History
James Paget described Paget's disease of the nipple in 1874. Radcliffe Crocker reported the first case of EMPD in 1889, when he described a patient with a skin lesion affecting the penis and scrotum, the findings of which were identical to those described by Paget.[5]
References
- ^ a b Ekwueme, Kingsley C; Zakhour, Hani D; Parr, Nigel J (1 January 2009). "Extramammary Paget's disease of the penis: a case report and review of the literature". Journal of Medical Case Reports 3 (1): 4. doi:10.1186/1752-1947-3-4.
- ^ Fanning J, Lambert L, Hale TM, Morris PC, Schuerch C. Paget’s disease of the vulva: prevalence of associated vulvar carcinoma, invasive Paget’s disease, and recurrence after surgical excision. Am J Obstet Gynecol 1999;180:24–7.
- ^ Vulvar Paget Disease: One Century After First Reported Mario Preti, MD,1 Leonardo Micheletti, MD,1 Marco Massobrio, MD,1 Shin-ichi Ansai, MD, PhD,2 and Edward J. Wilkinson, MD3© 2003, American Society for Colposcopy and Cervical Pathology Journal of Lower Genital Tract Disease, Volume 7, Number 2, 2003, 122–135
- ^ Chanda, JJ. "Extramammary Paget's disease: prognosis and relationship to internal malignancy.". Journal of American Academy of Dermatology.
- ^ Marques-Costa, JC; Cuzzi, T; Carneiro, S; Parish, LC; Ramos-e-Silva, M (May–Jun 2012). "Paget's disease of the breast.". Skinmed 10 (3): 160–5. PMID 22779098.
External links
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Glandular and epithelial neoplasms (ICD-O 8010-8589)
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| Epithelium |
Papilloma/carcinoma
(8010-8139) |
- Small cell carcinoma
- Combined small cell carcinoma
- Verrucous carcinoma
- Squamous cell carcinoma
- Basal cell carcinoma
- Transitional cell carcinoma
- Inverted papilloma
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| Glands |
Adenomas/
adenocarcinomas
(8140-8429) |
| Gastrointestinal |
- tract: Linitis plastica
- Familial adenomatous polyposis
- pancreas
- Insulinoma
- Glucagonoma
- Gastrinoma
- VIPoma
- Somatostatinoma
- Cholangiocarcinoma
- Klatskin tumor
- Hepatocellular adenoma/Hepatocellular carcinoma
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| Urogenital |
- Renal cell carcinoma
- Endometrioid tumor
- Renal oncocytoma
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| Endocrine |
- Prolactinoma
- Multiple endocrine neoplasia
- Adrenocortical adenoma/Adrenocortical carcinoma
- Hurthle cell
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| Other/multiple |
- Neuroendocrine tumor
- Adenoid cystic carcinoma
- Oncocytoma
- Clear cell adenocarcinoma
- Apudoma
- Cylindroma
- Papillary hidradenoma
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Adnexal and
skin appendage (8390-8429) |
- sweat gland
- Syringocystadenoma papilliferum
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Cystic, mucinous,
and serous (8440-8499) |
| Cystic general |
- Cystadenoma/Cystadenocarcinoma
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| Mucinous |
- Signet ring cell carcinoma
- Mucinous cystadenoma / Mucinous cystadenocarcinoma
- Mucoepidermoid carcinoma
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| Serous |
- Ovarian serous cystadenoma / Pancreatic serous cystadenoma / Serous cystadenocarcinoma / Papillary serous cystadenocarcinoma
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Ductal, lobular,
and medullary (8500-8549) |
| Ductal carcinoma |
- Mammary ductal carcinoma
- Pancreatic ductal carcinoma
- Comedocarcinoma
- Paget's disease of the breast / Extramammary Paget's disease
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| Lobular carcinoma |
- Lobular carcinoma in situ
- Invasive lobular carcinoma
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| Medullary carcinoma |
- Medullary carcinoma of the breast
- Medullary thyroid cancer
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| Acinar cell (8550-8559) |
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| Other |
| Complex epithelial (8560-8589) |
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- See also
- Template:Epithelium and epithelial tissue
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- Tumors: male urogenital neoplasia (C60–C63/D29, 185–187/222)
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| Internal |
| Testicles |
Sex cord-
gonadal stromal |
- Sertoli-Leydig cell tumour
- Sertoli cell tumour
- Leydig cell tumour
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| Germ cell |
| G |
- Seminoma
- Spermatocytic seminoma
- Intratubular germ cell neoplasia
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| NG |
- Embryonal carcinoma
- Endodermal sinus tumor
- Gonadoblastoma
- Teratoma
- Choriocarcinoma
- Embryoma
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| Prostate |
- Adenocarcinoma
- High-grade prostatic intraepithelial neoplasia
- Small-cell carcinoma
- Transitional cell carcinoma
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| External |
| Penis |
- Carcinoma
- Extramammary Paget's disease
- Bowen's disease
- Bowenoid papulosis
- Erythroplasia of Queyrat
- Hirsuties coronae glandis
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Index of reproductive medicine
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| Description |
- Anatomy
- Physiology
- Development
- sex determination and differentiation
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| Disease |
- Infections
- Congenital
- Neoplasms and cancer
- male
- female
- gonadal
- germ cell
- Other
- Symptoms and signs
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| Treatment |
- Procedures
- Drugs
- benign prostatic hypertrophy
- erectile dysfunction and premature ejaculation
- sexual dysfunction
- infection
- hormones
- androgens
- estrogens
- progestogens
- GnRH
- prolactin
- Assisted reproduction
- Birth control
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