Paget's disease of the nipple a clinical review of 27 cases

Abstract

Fig. 575). Sections through this tumour showed a small focus of carcinoma, at an early stage of development. The story of this patient emphasizes the importance of keeping in touch with affected members even when they profess to be in good health, and illustrates the fact that malignant disease may arise in the absence of any symptoms due to the polyposis.Case 2.-The second patient was a boy, aged 17, marked by an arrow in the family pedigree (Fig. 576) in which 24 adult members are recorded. Nine of these 24 are known to have inherited polyposis and 5 have died from intestinal cancer.This family was first investigated by MI. Tom Rowntree in 1950, and the information available at that time was published (Rowntree, 1950), but in this earlier version of the family pedigree the patient now being reported was not included because at that time he had not been traced and his existence was unknown to us. We knew that his mother had died of rectal cancer at the age of 22, but only subsequently did we discover that she had had an illegitimate son who had been brought up by in-law relatives. This boy at the age of 17 began to have diarrhcea, which was investigated by barium meal, and polyposis coli was discovered. His doctor then referred him to this hospital, where the diagnosis was confirmed, and inquiry into his family connexions showed the linkage with the family previously investigated.When first examined at St. Mark's Hospital G. C. was 17 years of age. He appeared healthy and his only complaint was persistent diarrhoea for the last six weeks, unaccompanied by blood or excess of mucus. Sigmoidoscopic examination revealed large numbers of adenomatous polyps in the rectum, which were treated by fulguration in several sessions. Then the rectum having been cleared of polyps, colectomy and ileorectal anastomosis was carried out.The colon from this patient is illustrated in Fig. 577 and a close-up of one region in Fig. 578. It will be seen that the mucosa was thickly carpeted with rounded adenomatous tumours. In some regions more than thirty tumours were visible in a square inch. An estimate of the total number of tumours calculated from the average density and surface area gave a figure in excess of 5000. The histological picture was that of adenomas undergoing active epithelial proliferation but with no indication of malignancy. This second case illustrates the importance of keeping in touch with a O F T H E N I P P L E 48 1 GARDNER, E.

Large Mammary Paget Disease without Underlying Breast Carcinoma

“…The pathogenesis of disease is still inconclusive. Two widely accepted hypotheses that explain the origin and nature of the phenomenon are the epidermotropic theory 1 , 4 and the in situ malignant transformation theory. 1 , 5 The epidermotropic theory states that the cancer cells originate from the intraductal epidermis and migrate along the duct and spread through the lactiferous sinus to the nipple.…”

“…Two widely accepted hypotheses that explain the origin and nature of the phenomenon are the epidermotropic theory 1 , 4 and the in situ malignant transformation theory. 1 , 5 The epidermotropic theory states that the cancer cells originate from the intraductal epidermis and migrate along the duct and spread through the lactiferous sinus to the nipple. 4 Nevertheless, this rare case of mammary Paget disease appeared only within the nipple epidermis and was not associated with underlying malignancy.…”

“…An underlying invasive breast cancer or ductal carcinoma in situ is found in 80% to 90% of cases. 1 , 2 , 4 , 5 Only rare patients appeared primarily within the nipple epidermis. 2 , 3 , 5 …”

“…An underlying invasive breast cancer or ductal carcinoma in situ is found in 80% to 90% of cases. 1,2,4,5 Only rare patients appeared primarily within the nipple epidermis. 2,3,5 Mammary Paget disease may be confused with chronic eczema, dermatitis, psoriasis, Bowen disease, or malignant melanoma.…”

“…In 1874, Sir James Paget first described the clinical features of the disease as chronic eczematous skin of the nipple and areola which very often develops subsequent breast carcinoma within 1 to 2 years. 1 However, the etiology and treatment of this disease remain controversial.…”