Sebaceous carcinomas of the ocular adnexa: A clinicopathologic study of 104 cases, with five-year follow-up data

Extramammary Paget's disease is a rare cutaneous adenocarcinoma, usually of epidermal origin and glandular differentiation and frequently associated with an underlying adnexal carcinoma and perhaps with underlying internal malignancy. One hundred ninety-seven cases of extramammary Paget's disease (196 cases reported in the English literature from 1962 to 1982 and one case of my own) are reviewed. It remains a rare cutaneous malignancy that occurs primarily in elderly people. It is seen more frequently in women than in men and occurs predominantly in vulvar and perianal locations. Twenty-six percent of patients with this disease will ultimately die of it or an associated internal malignancy. Twenty-four percent of patients with the disease have an associated underlying cutaneous adnexal adenocarcinoma. These patients have a higher mortality rate—46%—than patients with extramammary Paget's disease without underlying cutaneous adnexal adenocarcinoma. Twelve percent of patients with extramammary Paget's disease have an associated concurrent underlying internal malignancy. The location of the underlying internal malignancy appears to be closely related to the location of the extramammary Paget's disease—i.e., a perianal location is associated with adenocarcinoma of the digestive system, a penile-scrotal-groin location with genitourinary malignancy, etc. A directed internal malignancy search may be of benefit in patients who are diagnosed as having extramammary Paget's disease.

https://www.jaad.org/article/S0190-9622(85)70254-X/pdf

Extramammary Paget's disease : prognosis and relationship to internal malignancy

Perianal Paget's disease is rare, and its relationship to an associated internal regional cancer has been ill defined. We analyzed the histologic and immunohistochemical features of perianal Paget's disease in 11 patients to determine the frequency and relationship of associated regional internal carcinoma and to gain insight into its histogenesis. Of five patients with documented rectal adenocarcinoma, it was discovered synchronously with the Paget's disease in four and, subsequently, in one. Paget's cells of signet ring type predominated in four cases. Intraepithelial glands with intraluminal dirty necrosis were present in four cases. The immunophenotype in four cases studied was cytokeratin (CK)7+/CK20+/gross cystic disease fluid protein- (GCDFP) in both the intraepithelial Paget's cells and the invasive rectal adenocarcinoma. Six patients did not have documented rectal carcinoma. The Paget's cells in four were CK7+/CK20-/GCDFP15+. Three of these had purely intraepithelial Paget's disease, and invasive or metastatic disease developed in none after wide local excision. Bilateral inguinal lymph node metastases developed in the fourth patient, and the patient died 8 months after diagnosis of Paget's disease. In two patients, the Paget's cells were CK7+/CK20+/GCDFP15-. Recurrent intraepithelial perianal Paget's disease developed in one patient at 7 months; the patient was alive without disease at 24 months, and the other patient had several intraepithelial recurrences of perianal Paget's disease, and, subsequently, a large perianal tumor of uncertain cell type developed at 108 months, which led to the patient's death. We conclude that there are two types of perianal Paget's disease. One type has endodermal differentiation with gastrointestinal-type glands containing intraluminal dirty necrosis, numerous signet ring cells, CK20 positivity, and GCDFP15 negativity. Such cases are especially likely to be associated with synchronous or metachronous rectal adenocarcinoma. The other type is a primary cutaneous intraepithelial neoplasm in which the Paget's cells display sweat gland differentiation, including GCDFP15 positivity; it generally lacks gastrointestinal-type glands, intraluminal dirty necrosis, and CK20 positivity. The CK7 is a sensitive, albeit nonspecific, marker for Paget's cells.

Perianal Paget's Disease: A Histologic and Immunohistochemical Study of 11 Cases With and Without Associated Rectal Adenocarcinoma

Sebaceous carcinoma of the ocular adnexa.

Paget's disease of the vulva: pathology, pattern of involvement, and prognosis.

https://acsjournals.onlinelibrary.wiley.com/doi/pdf/10.1002/1097-0142%28196303%2916%3A3%3C387%3A%3AAID-CNCR2820160314%3E3.0.CO%3B2-0

Elson B. Helwig

Papers

Anogenital (extramammary) Paget's disease. A clinicopathological study