1/18. Large bilateral adrenal incidentalomas complicating untreated 11B hydroxylase deficiency in the third decade of life. A case report.The occurrence of bilateral giant adrenal incidentalomas is reported in a 22-year-old man who was evaluated for urinary tract infection. The right gland measured 16 cm, the left one 6 cm at computed tomography. Height was 145 cm, blood pressure 190/100 mm Hg. Testes were not palpable. Laboratory investigations revealed elevated levels of 17 hydroxyprogesterone:>50 ng/ml (n<1,1); 11 desoxycortisol: 919 nmol/l (n<30); testosterone: 19 ng/ml (n<0.7) and ACTH: 1 402 ng/l (n<48). karyotype was 46 XX. The patient was a female pseudohermaphrodite with congenital adrenal 11 B hydroxylase deficiency. Adrenal masses responded to glucocorticoid therapy with marked reduction of their size after six months. We confirm previous recommendations that patients with adrenal incidentaloma should be checked for congenital adrenal hyperplasia.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
2/18. adrenocortical carcinoma in two female children.adrenocortical carcinoma is a rare tumor in children. This tumor is more likely to be hormonally active in children than in adults and tends to cause a variety of symptoms, which may mimic other benign endocrinopathies. These tumors are usually diagnosed at advanced stages and portend a dismal prognosis. We describe two cases of adrenocortical carcinoma. One child presented with Cushingoid symptoms secondary to hypercortisolism, including amenorrhea, hirsutism and weight gain. The other child presented with precocious puberty. Both children underwent resection of the tumors. We describe their presenting symptoms, postoperative course, adjuvant therapy and clinical course. Pertinent literature regarding the anatomy of the adrenal gland, pathology of adrenocortical carcinoma, factors influencing outcome, diagnostic modalities and treatment, are discussed.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
3/18. Endoscope-assisted microsurgery for treatment of a suprasellar craniopharyngioma presenting precocious puberty.Being the most common childhood tumor to involve the hypothalamus and pituitary gland, craniopharyngioma, a histologically benign tumor, is usually related to growth retardation and hypogonadism. The presentation of precocious puberty is very rare for a hypothalamic craniopharyngioma. Here, we report such a case. The female patient had presented with symptoms of menarche and breast development since she was 6 years old. Hormonal therapy with a gonadotropin-releasing hormone analogue was instituted to cease precocious puberty but was unsuccessful. magnetic resonance imaging of the sella showed a 1-cm tumor in the hypothalamic area. Through a pterional approach, the tumor was removed en bloc with endoscope-assisted microsurgery. After the operation, our patient's symptoms improved, so the hormone therapy was discontinued. This extremely unique association of craniopharyngioma and precocious puberty, the causes and mechanisms involved along with the advantages of endoscope-assisted microsurgery are discussed.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
4/18. testosterone-producing adrenocortical neoplasm in a 6-year-old boy.Adrenal tumours that predominantly secrete testosterone are virtually unknown in prepubertal male patients.We present the case of a 6-year-old boy with premature sexual development and markedly elevated serum testosterone, but normal urinary steroid levels. diagnostic imaging demonstrated a spherical tumour of the left adrenal gland. Surgical excision led to normalisation of testosterone levels, and postoperative serial low hormone measurements ruled out tumour recurrence.Although extremely rare, this case illustrates that testosterone-producing adrenal adenomas may be encountered in boys without urinary steroid elevation. Surgical excision promises a definite cure. testosterone is a useful tumour marker in these patients until the beginning of puberty.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
5/18. A child with pituitary gigantism and precocious adrenarche: does GH and/or PRL advance the onset of adrenarche?We describe a female child with pituitary gigantism and precocious adrenarche. From two years of age she showed unusual overgrowth, and at 5 years old she was 133.5 cm ( 5.5 SD) tall and weighed 40.5 kg. Her precocious manifestations were public hair, acne vulgaris, hirsutism, and advanced bone age. Endocrinological examination revealed markedly increased serum growth hormone (GH) and prolactin (PRL), which responded paradoxically to a TRH test. In addition, the concentrations of serum dehydroepiandrosterone (DHA) and its sulfate (DHAS) were increased to adult levels, moving in accordance with changes in ACTH, which suggested that these androgens were secreted from the adrenal glands functionally. These androgens seemed to be responsible for her partial precocity. Prior reports have suggested that GH and/or PRL overproduction might have played a role in the induction of adrenarche. Also, in previous reports of 9 gigantism patients under 10 years old, the manifestation of precocious adrenarche was suggested in 8. Further investigation of the influence of GH and PRL on adrenal androgen production in children with pituitary gigantism is required. On the other hand, in short children with normal GH secretion, attention should be paid to whether or not the GH therapy in early childhood induces precocious adrenarche.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
6/18. Precocious puberty associated with a pineal cyst: is it disinhibition of the hypothalamic-pituitary axis?Accelerated development of secondary sexual characteristics or sexual precocity is a well-known entity. Most authors recognize two groups of patients, those described as having central precocious puberty (CPP) and those with precocious pseudopuberty. CPP results from premature activation of the hypothalamic-pituitary-gonadal axis and pseudopuberty is caused by lesions that secrete gonadotropin-like substances or hormones. The onset of CPP is usually before age 8 in females and age 9 in males; however, there is contention that the age of onset is much earlier and also differs depending on the patients' race. Previously reported causes of CPP include intracranial neoplasm, infection, trauma, hydrocephalus and Angelman's syndrome. Pineal cysts are usually asymptomatic incidental findings, but have been associated with CPP. We present an interesting case of a patient with CPP and an associated pineal cyst. We review the literature on the pathogenesis of CPP and associated pineal cyst, the neuroendocrine relationship between the pineal gland and puberty and the neurosurgical role in these cases.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
7/18. 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 = 1keywords = gland (Clic here for more details about this article) |
8/18. Duplication of the hypophysis associated with precocious puberty: presentation of two cases and review of pituitary embryogenesis.Pituitary duplication is a rare malformation commonly associated with other major neural/craniofacial anomalies, easily shown by magnetic resonance imaging. The authors describe two girls with duplication of the pituitary gland and thickening of the hypothalamus, facial dysmorphism and precocious pubertal development. The pathogenesis of pituitary duplication and its relationship with precocious pubertal development are discussed.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
9/18. Primary hypothyroidism presenting as ovarian tumor and precocious puberty in a prepubertal girl.We report a case of a prepubertal girl with juvenile primary hypothyroidism presenting as ovarian cysts and precocious puberty. The 7-year-old female was referred to our clinic because of a pelvic/abdominal mass and vaginal bleeding. Besides these findings, on physical examination we noticed the thyroid gland globally increased and the presence of secondary sexual characteristics. Based upon the clinical profile and investigations, the patient was diagnosed with juvenile primary hypothyroidism due to autoimmune thyroiditis. The cysts and precocious puberty resolved spontaneously after the simple replacement of thyroid hormone. It is important to bear in mind hypothyroidism in cases of girls presenting ovarian cysts and precocious puberty in order to avoid unnecessary surgery on the ovaries.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
10/18. Adrenal steroid secretion in girls with pseudoprecocious puberty due to autonomous ovarian cysts.To evaluate the role of adrenal steroids in pseudoprecocious puberty due to large ovarian follicular cysts, we studied the serum 17-hydroxyprogesterone response to a combination of dexamethasone suppression followed by iv ACTH administration in two girls and compared the results to those in girls with premature thelarche, normal prepubertal girls, and a girl with true precocious puberty. Although basal serum 17-hydroxyprogesterone levels were normal in all subjects, there was incomplete suppression of 17-hydroxyprogesterone with dexamethasone in the two girls with pseudoprecocious puberty and large ovarian cysts. The 17-hydroxyprogesterone response to ACTH was much greater in these girls (360 and 540 ng/dl) than in the girls with other types of precocious puberty (mean /- SD, 71 /- 15 ng/dl) or in normal prepubertal girls (80 /- 20 ng/dl). The girls with large ovarian cysts had decreased gonadotropin responses to GnRH, which were reversed subsequent to removal of the cyst. Removal of the ovarian cysts also restored the dexamethasone suppressibility of serum 17-hydroxyprogesterone and abolished the progression of pubertal development. However, 17-hydroxyprogesterone responses to ACTH were still elevated (160 and 350 ng/dl). Preoperatively, both girls had increased levels of dehydroepiandrosterone sulfate, 16-hydroxydehydroepiandrosterone sulfate, and another unidentified steroid sulfate. These steroid sulfates were also found in the cyst fluid from the one patient from whom the fluid was obtained. These results suggest that steroid production by the adrenal gland may stimulate the development of small ovarian cysts (which may be present in normal prepubertal girls) into large ovarian cysts capable of causing gonadotropin-independent precocious puberty.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
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