Multiple cutaneous sebaceous adenomas are seen in:
Ans is 'd' i.e. Muir Torre syndrome Multiple sebaceous Neoplasms (adenomas) are characterstics of Muir Torre syndrome. Muir-Torre syndrome Muir-Tone syndrome is an autosomal-dominant familial cancer syndrome. The hallmark of Muir-Tone syndrome is presence of numerous sebaceous gland neoplasms, including carcinomas that may precede, follow, or coexist with visceral cancers. Visceral cancers associated with MTS are - GI tract adenocarcinoma of the colon or genitourinary tract, or lymphoma. The defining feature of this syndrome is the combination of sebaceous gland tumours and at least one visceral cancer. Typical skin tumours associated with this syndrome include sebaceous adenomas, epitheliomas and carcinomas. Keratoacanthomas and basal cell carcinomas with sebaceous differentiation also occur. All these sebaceous gland tumours are rare in the general population; the finding of such a tumour is a marker for MTS. Early recognition of the syndrome in patients with sebaceous gland tumours should facilitate early detection of subsequent malignancies if the patient is entered into appropriate screening programmes. Colorectal cancer is the commonest visceral neoplasm to occur in MTS. Cutaneous neoplasms Visceral malignancies * Sebaceous adenomas * GIT malignancies most Muir Torre * sebaceous epitheliomas commonly colorectal ca syndrome * sebaceous carcinomas * Genitourinary ca * Keratocanthoma * Basal cell carcinoma Other options: Cowden's disease or multiple hamaoma syndrome is an autosomal-dominant syndrome with numerous tumors of the hair follicles called tricholemmomas located on the face. These patients have very high risk of breast and thyroid carcinomas. Bilateral mastectomies may be recommended. Mammography and other exposure of the breast tissue to 0 radiation is contraindicated. Multiple Hamaomatous polyps occur anywhere in the GI tract. Gardner's syndrome is a variant of FAP; familial adenomatous polyposis, a colorectal cancer syndrome characterized by hundreds of adenomatous colorectal polyps, with an inevitable progression to colorectal cancer by age 40. Cutaneous manifestations may be the presenting sign and include numerous epidermal cysts and soft tissue tumors. Osteomas of mandible and skull are the most common facial lesions in Gardner syndrome. Gardner syndrome is characterized by: - Colorectal polyposis - Epidermoid cysts - Desmoid tumors - Osteomas - Thyroid neoplasms, and - Congenital hyperophy of the retinal pigment epithelium Turcot's syndrome is also a variant of FAP characterized by: - Colorectal polyposis - Brain tumors (Medulloblastoma, Glioblastoma multiforme) Also Know: Gorlin-Goltz syndrome or Nevoid basal cell carcinoma syndrome is characterized by the presence of multiple basal cell carcinomas, numerous bony abnormalities, and a strong predisposition for malignancy. The most common malignant tumor is medulloblastoma, but also astrocytoma, meningioma, and craniopharyngioma.
Cowden syndrome
Gardner syndrome
Turcot syndrome
Muir Torre syndrome