Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0001430 (adenoma)
 21,222 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Maffucci's syndrome (association of multiple enchondromatosis and multiple hemangiomata) is rarely associated with other ectodermal tumors. The present case is the second in literature in which the Maffucci's syndrome is associated with the parathyroid adenoma resulting in primary hyperparathyroidism. Although the cystic lesions of the bone and the ectopic calcifications are common to both diseases, pathogenesis apparently not related. The association of parathyroid adenoma with Maffucci's syndrome suggests the potential involvement of mesenchymal organs in this ectodermal neoplastic disorder.
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PMID:A case of Maffucci's syndrome associated with primary hyperparathyroidism. 730 64

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.
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PMID:Pathology of osseous and genitourinary lesions of Proteus syndrome. 810 99

Maffucci syndrome is an extremely rare, sporadic, and nonhereditary disease characterized by enchondromatosis, bony abnormalities, and vascular malformations. We report a successful anaesthetic management of an adult patient with Maffucci syndrome scheduled for surgical excision of parathyroid adenoma, who had numerous enchondromas, haemangiomas, and skeletal deformities involving different parts of the body and posing significant challenge during positioning, securing intravenous access, and insertion of endotracheal tube. Awareness about this syndrome is of paramount importance because similar patients may be encountered with increasing frequency for incidental or corrective surgeries.
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PMID:Maffucci syndrome and anaesthesia: Case report. 3114 85