1/5. Nevoid basal-cell carcinoma syndrome.The nevoid basal-cell carcinoma syndrome is characterized by major manifestations, such as multiple basal-cell carcinomata, cysts of the jaws, and skeletal--specifically, rib--abnormalities. Findings in 53 patients and a review of the literature document both major and lesser-known manifestations of the disorder. The odontogenic keratocyst, which usually appears during adolescence, has a marked tendency to recur. In addition to the skin tumors, milia, epidermoid cysts, chalazia, comedones, and palmar and plantar pits are frequent. The skin tumors, originally thought to be independent of sun exposure, are more common in sun-exposed areas and are far less frequent and occur at a much later age in blacks than in whites. There is some evidence that radiation of the skin promotes the appearance of skin cancers in this disorder. Unilateral linear nevoid basal-cell carcinomas with comedones may represent postzygotic somatic mutation. A proclivity to other forms of neoplasia exists. patients with this syndrome have had medulloblastoma, meningioma, ovarian fibroma, ovarian fibrosarcoma, fibrosarcoma of the jaws, cardiac fibroma, fetal rhabdomyoma, and lymphatic or chylous cysts of the mesentery. In addition to the usual modalities of treatment, topical immunotherapy and topical 5-fluorouracil have been used with success. Oral synthetic retinoids, such as 13-cis-retinoic acid, have been used to prevent new lesions from appearing and to arrest the growth of older lesions by inducing differentiation. The independent observations of increased prostaglandin levels associated both with odontogenic keratocyst expansion and aggression of basal cell cancers merit further investigation both as a fundamental cellular mechanism and as a possible basis for treatment (e.g., with antiprostaglandins). The key to pre- and early postnatal diagnosis of the disorder--as well as to an understanding of the basic abnormality--lies in molecular genetics. Prime attention should be given to finding the site of the gene through the use of dna and other markers.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
2/5. Urogenital tract carcinoma metastatic to the head and neck.A 9-year review of our experience with head and neck metastases from 845 urogenital tract tumors of the kidney, prostate, bladder, testicle, spermatic cord, penis, urethra, and ureter was performed. Thirty-one (3.7%) of these tumors developed metastases to the cervical and supraclavicular lymph nodes, scalp, thyroid gland, thyroid cartilage, parotid gland, retroorbit, mandible, nasal cavity, and paranasal sinuses. Unusual cases and a review of the literature are presented. The frequency of such metastases to the head and neck from various primaries, diagnostic application of current immunohistochemical methodology, and therapeutic alternatives are emphasized.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
3/5. Genitourinary tumors in men with the muir-torre syndrome.The muir-torre syndrome is an autosomal dominant genodermatosis characterized by the occurrence of one or more sebaceous gland tumors (either adenoma, epithelioma, or carcinoma) and a single malignant internal neoplasm. To date, 133 cases of muir-torre syndrome have been described. Colorectal (49%) and genitourinary (21%) carcinomas are the most common initial neoplasms. The case of a man with a history of recurrent ocular sebaceous carcinoma who subsequently had seminoma is described, and previously published reports of men with muir-torre syndrome and genitourinary tumors are discussed. Including this report, an associated genitourinary tumor was the initial malignant internal neoplasm in 11 men with muir-torre syndrome. The detection of a genitourinary neoplasm preceded diagnosis of the patient's muir-torre syndrome-associated sebaceous gland tumor in five patients (45%). All patients with sebaceous gland tumors of the type associated with muir-torre syndrome warrant consideration of muir-torre syndrome and appropriate workup to detect asymptomatic malignant visceral neoplasms.- - - - - - - - - - ranking = 1.5keywords = gland (Clic here for more details about this article) |
4/5. Sebaceous gland adenoma of the palpebral conjunctiva in a patient with Muir-Torre-syndrome: a case report.BACKGROUND: muir-torre syndrome (MTS) is a hereditary genodermatosis associated with internal malignancies. methods: We report the histological case of an atypical sebaceous gland adenoma of the palpebral conjunctiva in a 42-year-old female patient. RESULTS: The combination of this adenoma with atypical adenomatous hyperplasia of the endometrium and a strong family history of gastrointestinal and urogenital cancers led to the diagnosis of MTS. CONCLUSION: We present a case where the diagnosis of an atypical solitary sebaceous gland adenoma of the palpebral conjunctiva combined with a conspicuous personal and family history led to the diagnosis of MTS.- - - - - - - - - - ranking = 3keywords = gland (Clic here for more details about this article) |
5/5. pathology of osseous and genitourinary lesions of proteus syndrome.A male patient followed from the age of 3 to 25 years was eventually diagnosed as having proteus syndrome. He was born with linear epidermal nevi of the neck and forearm and presented with macrodactyly of the right hand and progressive hemihypertrophy of the right lower limb recurring after multiple reduction operations. The bone ends showed disorderly overgrowth of hyaline and fibrocartilage mixed with collagen and bone, and early differential diagnoses included Ollier's disease. The child also had vertebral anomalies, scoliosis, a bony protrusion of the cranial vertex, and strabismus. In the second decade he developed gyriform swelling of the soles, retinopathy, bilateral papillary cysts of the epididymis, and a giant cyst of the left kidney with complex glandular foci. At 22 years a 3-cm meningioma containing adipose tissue was resected, and at 24 years a 3-cm cellular nodule of the rete testis with hyperchromatic foci, probably an adenoma, was removed. The features of proteus syndrome were those of hyperplasia and neoplasia of mostly mesodermal tissues. Unlike other reported cases, overgrowth of a finger recurred at 25 years.- - - - - - - - - - ranking = 0.5keywords = gland (Clic here for more details about this article) |