Cowden syndrome CS or multiple hamartoma syndromes MHSs is an uncommon condition and characterized by mucocutaneous lesions which may be associated with the lesions of breast, thyroid, gastrointestinal tract, skin, and often of central nervous system. A thorough evaluation of Cowden's disease is essential due to increased risk of malignancy in an organ or system of the affected patients. We are reporting a case of female patient who presented with multiple mucocutaneous papilloma associated with involvement of multiple organs and systems.
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Hereditary breast and ovarian cancer HBOC syndrome is an inherited cancer-predisposition syndrome. Affected individuals have a significantly greater risk of developing certain cancers, particularly breast cancer, in both men and women, and ovarian cancer in women. Affected individuals tend to develop cancer earlier in life as well, usually before the age of
This material supplements information provided by physicians, nurses, genetic counselors, and other members of your health care team about Cowden syndrome. It does not replace regular medical care or discussions with your health care team. If you see an unfamiliar term, please see the Glossary of Terms at the end of this material.
It is characterized by multiple hamartomatous lesions of ectodermal, mesodermal and endodermal origins. The organ system that most consistently manifests this syndrome is the skin. These signs precede the development of cancer by several years, and they serve as important clinical markers for identification of patients at high risk for malignancies of the breast or thyroid.
Skip to search form Skip to main content. The patient presented with lower abdominal pain. Her previous medical history was PJS and breast cancer.
Accessory breastsalso known as polymastiasupernumerary breastsor mammae erraticaeis the condition of having an additional breast. Extra breasts may appear with or without nipples or areolae. It is a condition and a form of atavism which is most prevalent in male humans, and often goes untreated as it is mostly harmless.
Cowden syndrome is an autosomal dominant disorder with a predisposition to multiple benign and malignant tumors. In our patient, in addition to breast and endometrial malignancies as well as facial trichilemmomas, she was noted to have multiple meningiomas, pancreatic lipomas and lung cysts. These latter lesions have been noted in previous Cowden syndrome case reports, but are not included in the diagnostic criteria at this time.
Fibrocystic breast changes lead to the development of fluid-filled round or oval sacs cysts and more prominent scar-like fibrous tissue, which can make breasts feel tender, lumpy or ropy. Fibrocystic breasts are composed of tissue that feels lumpy or rope-like in texture. Doctors call this nodular or glandular breast tissue.
Cowden's syndrome CSor multiple hamartoma syndrome, is an autosomal dominant disorder associated with benign skin tumors and an increased risk of breast cancer. In an effort to understand the basic mechanisms regulating the development of breast cancer in this patient population, as well as to define diagnostic aspects of the disorder, we describe for the first time the clinical and pathological spectrum of breast disease in CS. We obtained the clinical histories and examined the histopathology of 59 cases from 19 women with CS sent to us from a variety of institutions. The 19 women showed a spectrum of benign histopathological findings, including ductal hyperplasia, intraductal papillomatosis, adenosis, lobular atrophy, fibroadenomas, and fibrocystic change.