1/40. A case of atypical McCune-Albright syndrome requiring optic nerve decompression.McCune-Albright syndrome (MAS) is a disease of noninheritable, genetic origin defined by the triad of cafe-au-lait pigmentation of the skin, precocious puberty, and polyostotic fibrous dysplasia. This syndrome, which affects young girls primarily, has also been reported with other endocrinopathies, and rarely with acromegaly and hyperprolactinemia. The fibrous dysplasia in MAS is of the polyostotic type and, apart from the characteristic sites such as the proximal aspects of the femur and the pelvis, the craniofacial region is frequently involved. A male patient with MAS presented with juvenile gigantism, precocious puberty, pituitary adenoma-secreting growth hormone and prolactin, hypothalamic pituitary gonadal and thyroid dysfunction, and polyostotic fibrous dysplasia causing optic nerve compression. Visual deterioration and its surgical management are presented.- - - - - - - - - - ranking = 1keywords = pituitary (Clic here for more details about this article) |
2/40. A case of pituitary adenoma associated with McCune-Albright syndrome.A 11-year-old boy presented with right temporal hemianopsia and was evaluated of a possible pituitary adenoma. At the age of six, he underwent surgery for facial deformities due to fibrous dysplasia. On admission, he had acromegalic features, was 170 cm tall, weighing 66 kg. The left side of his face was slightly deformed, and a cafe-au-lait spot was found on his right face. Endocrinologic examination revealed elevated basal level of serum GH (103.6 ng/ml, normal < 3 ng/ml) and PRL (259.1 ng/ml, normal < 30 ng/ml). Other endocrine functions were normal. CT showed hyperostosis of the right frontal, occipital, sphenoidal and maxillary bones. magnetic resonance imaging (MRI) revealed a pituitary macroadenoma with intraadenomatous cyst. On the basis of physical, endocrinologic and neuroradiologic examination, our diagnosis was pituitary adenoma with McCune-Albright syndrome. Surgery was performed by subfrontal approach. By light microscopy, the pituitary tumor represented a typical acidophilic adenoma. Immunoreactivity for GH and PRL were evident in most of the adenoma cells. Double immunostaining for GH and PRL demonstrated the co-existence of the two hormones in a few adenoma cells. However the majority of cells expressed only one hormone. After surgery the right temporal hemianopsia improved. Postsurgical endocrinologic examination revealed reduction in basal serum GH and PRL levels. Administration of bromocriptine decreased blood PRL levels but it had a limited action on GH hypersecretion.- - - - - - - - - - ranking = 4keywords = pituitary (Clic here for more details about this article) |
3/40. "cafe-au-lait spots" caused by vitiligo in McCune-Albright syndrome.cafe-au-lait spots, fibrous dysplasia of bone, and endocrine gland hyperactivity are the principal features of McCune-Albright syndrome (MAS). cafe-au-lait spots appear at, or soon after, birth. We illustrate "cafe-au-lait spots" acquired during middle age in a patient with MAS that are an illusion caused by vitiligo. This 64-year-old woman is the oldest patient reported with this disorder.- - - - - - - - - - ranking = 0.00047155307559951keywords = gland (Clic here for more details about this article) |
4/40. Clinical, endocrinological and radiography features in a child with McCune-Albright syndrome and pituitary adenoma.McCune-Albright syndrome is a rare syndrome presenting with polyostotic dysplasia, cafe-au-lait spots and multiple endocrinopathies that is very often combined with precocious puberty. We examined the clinical, endocrinological and radiological features in a boy with McCune-Albright syndrome and pituitary adenoma. x-rays, magnetic resonance (MRI) scan, whole body scintigraphy, single photon emission computer tomography (SPECT) and 3D-reconstruction from bone SPECT was performed to evaluate clinical improvement after treatment with sandostatin and pamidronic acid. After a six-month period of treatment with sandostatin and pamidronate, bone scintigraphy revealed significantly reduced activity. Treatment with bromocriptine and methimazole led to normalization of prolactin and thyroid hormone levels. Mobility of the patient improved. A significant improvement as a result of treatment with sandostatin and pamidronic acid was found in this patient with generalized fibrous dysplasia. So far, this condition has been treated with pamidronate only in adults, but severely affected children also benefit from this treatment regimen.- - - - - - - - - - ranking = 2.5keywords = pituitary (Clic here for more details about this article) |
5/40. McCune-Albright syndrome: growth hormone dynamics in pregnancy.Excess GH secretion has a well recognized association with McCune-Albright syndrome. Although there have been a number of reported pregnancies in uncontrolled acromegaly, none has been described in the McCune-Albright syndrome. We have studied the GH and insulin-like growth factor i (IGF-I) profiles in a patient with confirmed McCune-Albright syndrome and GH hypersecretion throughout a successful pregnancy and postpartum period. Prepregnancy, IGF-I was 60.6 nmol/L (normal, 18.0--43.1), and the daytime GH profile measured using assay A was 9.6--14.0 mU/L. At 13 weeks gestation there was a decline of IGF-I to 33.9 nmol/L and in the daytime GH profile (assay A) to 5.4--6.8 mU/L. At 24 weeks, IGF-I had risen to 51.6 nmol/L. A simultaneous daytime GH profile at this time using assay A revealed levels between 21.3--22.1 mU/L, but only 2.1--3.0 mU/L with assay B. Assay A has significant cross-reactivity with human placental lactogen (HPL), unlike assay B. At 36 weeks, IGF-I was still elevated at 56.6 nmol/L, with a daytime GH profile of 16.6--17.7 mU/L using assay A and 1.5--3.9 mU/L with assay B. At 12 weeks postpartum, IGF-I was 71.4 nmol/L, and the daytime GH profile with assay B was 5.6--8.6 mU/L. These data support a picture of GH suppression during pregnancy in acromegaly associated with McCune-Albright syndrome, shown best with assay B, which discriminates between GH and HPL. These results contrast with previous reports of pregnancy in uncontrolled acromegalics, in whom pituitary GH levels were unaffected by pregnancy, and total GH and IGF-I levels were noted to be elevated. These data suggest that GH secretion in a pregnant acromegalic with the McCune-Albright syndrome may not be entirely autonomous, as seen in classic acromegaly, but may be associated with a degree of negative feedback control that could be exerted by a circulating factor of placental origin, probably HPL or placental GH.- - - - - - - - - - ranking = 0.5keywords = pituitary (Clic here for more details about this article) |
6/40. Atypical McCune-Albright syndrome associated with growth hormone-prolactin pituitary adenoma: natural history, long-term follow-up, and SMS 201-995--bromocriptine combined treatment results.A 35-yr-old woman is described as having atypical McCune-Albright syndrome, associated with acromegaly and hyperprolactinemia due to pituitary adenoma. The patient did not present sexual precocity, but primary amenorrhea. After transphenoidal adenomectomy, the GH plasma levels returned to normal, whereas the PRL values decreased; bromocriptine therapy normalized PRL levels and induced ovulatory menses. After 4 uneventful yr the patient developed relapse of active acromegaly that did not recover after a second neurosurgical exploration. bromocriptine treatment maintained normal PRL levels but did not significantly reduce GH ones; the association with long-acting somatostatin analog SMS 201-995 by continuous sc pump infusion induced definitive control of GH and somatomedin-C secretion. These results suggest an additive inhibitory effect on GH secretion exerted by the two drugs.- - - - - - - - - - ranking = 2.5keywords = pituitary (Clic here for more details about this article) |
7/40. acromegaly with fibrous dysplasia: McCune-Albright syndrome -- clinical studies in 3 cases and brief review of literature--.The McCune-Albright syndrome (MAS) is characterized by a triad of poly/monostotic fibrous dysplasia, cafe-au-lait macules and hyperfunctioning endocrinopathies including growth hormone (GH) excess. Polyostotic bone lesions and cafe-au-lait macules are common while monostotic bone lesions are rare. Similarly, acromegaly as a manifestation of endocrine hyperfunction with MAS is uncommon and in most of the instances somatotropinoma has not been documented. We report 3 patients, two of them had monostotic lesion, none had cafe-au-lait macules and all had GH secreting pituitary macroadenoma. All of them underwent transfrontal pituitary adenomectomy and had histopathological confirmation of GH secreting pituitary adenoma. A brief review of literature is also presented.- - - - - - - - - - ranking = 1.5keywords = pituitary (Clic here for more details about this article) |
8/40. McCune-Albright syndrome associated with pituitary microadenoma: patient report.McCune-Albright syndrome (MAS) is a rare disorder characterized by the classic triad of precocious puberty, polyostotic fibrous dysplasia and cafe-au-lait spots. Additional endocrine abnormalities may also be present, including hyperthyroidism, growth hormone excess and hyperprolactinemia. The most commonly encountered endocrine dysfunction is gonadal hyperfunction. Gonadotropin-independent precocious puberty is typically the initial manifestation of MAS in girls. ovarian cysts may be detected on pelvic ultrasound. Our patient was also found to have pituitary microadenoma, evidenced by dynamic magnetic resonance imaging.- - - - - - - - - - ranking = 2.5keywords = pituitary (Clic here for more details about this article) |
9/40. Ductal carcinoma in situ in a 27-year-old woman with McCune-Albright syndrome.McCune-Albright syndrome (MAS) is a sporadic disorder characterized by the triad of irregularly edged hyperpigmented macules (cafe au lait spots); a slowly progressive bone disorder, polyostotic fibrous dysplasia, usually involving the base of the skull and the long bones; and luteinizing hormone-releasing hormone (LHRH)-independent precocious puberty. This case is the first report of a 27-year-old woman with ductal carcinoma in situ (DCIS) and Paget's disease of the nipple associated with MAS. The discussion focuses on two endocrine manifestations of this syndrome including precocious puberty and excess growth hormone secretion. In our patient, both her early puberty and pituitary adenoma, in association with MAS, resulted in excess production and secretion of estrogen and growth hormone. Both of these hormones function to stimulate breast growth and development. We hypothesize they are responsible for this patient's DCIS and Paget's disease of the nipple so early in life.- - - - - - - - - - ranking = 0.5keywords = pituitary (Clic here for more details about this article) |
10/40. Paediatric management of endocrine complications in McCune-Albright syndrome.McCune-Albright syndrome was diagnosed at 2.5 years in a male, with a lower limb fracture and cafe-au-lait markings. Polyostotic fibrous dysplasia was treated with pamidronate for 4 years, with control of bone pain, improved quality of life, normal growth and visual fields monitored 4-6 monthly. Gonadotrophin independent precocious puberty occurred between ages 5-7 years but was slowly progressive, requiring no treatment. Bone age was 9 years at 7.5 years. From age 8-8.5 years, growth velocity increased further, with widening of spaces between teeth, acral enlargement, sweating and facial acne but no change in pubertal status (testes 6 ml, pubic hair Tanner stage I). Binasal field loss occurred. MRI demonstrated a bulging pituitary gland typical of adolescence, with no discrete tumour, with a markedly thickened calvarium and sphenoid. Bone age advanced from 9 to 12 years in 1 year. Basal growth hormone (GH) was 78 mIU/l, rising to 100 mIU/l with glucose tolerance test, basal IGF-I 2.4 IU/l, FSH <0.1 mIU/l, LH 0.1 mIU/l, testosterone 1.4 nmol/l. Treatment with octreotide LAR, flutamide and testolactone resulted in control of GH (12 mIU/l) and precocious puberty, slowing of growth, return to normal jaw size and tooth spacing, sweating, acne, facial appearance and restoration of full visual fields. Response demonstrated short-term efficacy of medical treatment and a remarkable capacity for facial remodelling in a child with acromegaly. Visual field change is attributed to reduction in soft tissue swelling around the optic foramina. Management issues concern long-term control of GH secretion, the impracticability of surgery in this patient and radiotherapy concerns in a young child.- - - - - - - - - - ranking = 0.5004715530756keywords = pituitary, gland (Clic here for more details about this article) |
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