Cases reported "Puberty, Precocious"

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1/30. sertoli-leydig cell tumor of the ovary, a rare cause of precocious puberty in a 12-month-old infant.

    We report a 12-month-old infant who presented with a 4-month history of isosexual precocious puberty secondary to an estrogenizing sertoli-leydig cell tumor of the ovary. Total serum immunoreactive inhibin and subunits A and B were markedly elevated before surgical resection and subsequently decreased 7 wk later into the normal prepubertal range. Twenty weeks following surgical removal, the patient presented again with central precocious puberty; inhibin B levels were raised on this occasion, a luteinizing releasing hormone stimulation test confirmed central precocious puberty. This is the youngest reported occurrence of this rare sex cord stromal neoplasm. The prognosis of this extremely rare tumor presenting at this early juvenile stage is uncertain. This report illustrates the usefulness of serum inhibin as a tumor marker during therapeutic suppression with leuprorelin acetate for central precocious puberty. Analysis of genomic and tumor dna revealed a normal nucleotide sequence for the LH receptor and the Galpha(s) gene. To understand the molecular pathogenesis of this tumor we analyzed mRNA levels for the inhibin A and B subunits, FSH receptor, LH receptor aromatase, steroidogenic factor-1 and the ER beta genes. Molecular characterization reveals the presence of genes specific for granulosa and leydig cells; the relative expression of these genes, in addition to its histologic characteristics, suggests that this tumor may result from a dysdifferentiation of a primordial follicle.
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ranking = 1
keywords = granulosa
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2/30. Precocious puberty in a female with proteus syndrome.

    proteus syndrome is a complex disorder comprising malformations and overgrowth of multiple tissues and characterized by its polymorphism and mosaicism. The syndrome is rare and sporadic. Oliveira M da C et al reported the first case of proteus syndrome associated with precocious puberty in a boy. We are reporting a case of a 7-month old girl with proteus syndrome who developed a juvenile granulosa cell tumor in one ovary causing precocious puberty. At our knowledge this is the first case of proteus syndrome with precocious puberty in a female.
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ranking = 2.777015377759
keywords = granulosa cell, granulosa
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3/30. Venous sampling can be crucial in identifying the testicular origin of idiopathic male luteinising hormone-independent sexual precocity.

    It has been recently shown that male LH-independent sexual precocity is caused by a somatic activating mutation in the luteinising hormone receptor (LHR) of Leydig cell tumours. In each of the patients described to date, the tumour was a well-defined, single encapsulated nodule. We present a 5.7-year-old boy with nodular Leydig cell hyperplasia, who harbours a somatic mutation of the LHRgene. The boy showed the clinical features of severe sexual precocity caused by LH-independent testosterone hypersecretion. Congenital adrenal hyperplasia, hCG- or androgen-secreting tumours, McCune-Albright syndrome, and familial male-limited precocious puberty (or testotoxicosis) were all ruled out as possible causes. A hypoechoic area was detected at the cranial pole of his right testis and a biopsy was performed. Histological examination revealed a lack of mature leydig cells. When dna from the affected tissue was isolated and sequenced, no somatic mutation of the LHR gene was found. To further determine the origin of the elevated testosterone levels, venous sampling was performed. blood samples taken from the right spermatic vein showed an elevated serum testosterone concentration of 259 nmol/l. Unilateral orchiectomy of the right testis was performed, and systemic testosterone concentrations normalised. Histological examination revealed nodular Leydig cell hyperplasia. dna analysis of the nodular tissue showed a heterozygous mutation in exon 11 of the LHR gene, which caused the replacement of aspartic acid at codon 578 by histidine. CONCLUSION: the somatic activating mutation (Asp578His) of the luteinising hormone receptor gene is not only present in Leydig cell adenomas, but can also be found in nodular Leydig cell hyperplasia. Venous sampling can play a vital role in determining the origin of elevated testosterone levels.
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ranking = 0.07512196378787
keywords = cell tumour
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4/30. Precocious pseudopuberty due to juvenile granulosa cell tumor.

    A case of precocious puberty occurring in a young girl is presented. Vaginal bleeding and secondary sexual characteristics had occurred at 7 years of age associated with an abdominal mass. These findings were due to a functional juvenile granulosa cell tumor in the right ovary. Right adenectomy was performed. Histopathology was confirmatory.
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ranking = 13.885076888795
keywords = granulosa cell, granulosa
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5/30. adult type granulosa cell tumor causing precocious pseudopuberty in a 6 year-old girl.

    Malignant ovarian tumors are responsible for 2-3% of all cases of precocious pseudopuberty (PP) in girls. The most common forms of ovarian tumors presenting as PP are the granulosa cell tumors (GCT). The clinical and pathological features of granulosa cell tumors that occur frequently in young girls, so-called juvenile granulosa cell tumors (JGCT), differ histologically from those occurring in adults. As a cause of PP in young girls, adult type granulosa cell tumors (AGCT) are extremely rare. We report a 6 year-old girl presenting with early breast development and vaginal bleeding due to a well encapsulated ovarian tumor. Microscopic features of the resected tumor were characteristic of AGCT.
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ranking = 22.216123022072
keywords = granulosa cell, granulosa
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6/30. Cystic adult granulosa cell tumor causing precocious pseudopuberty in a six-year-old girl.

    A very rare occurrence of adult granulosa cell tumor of the ovary (not of the juvenile type) causing precocious pseudopuberty in a six-year-old girl is described. An additional feature of interest was that the tumor appeared entirely cystic. To our knowledge, this condition in such a young premenarchal patient has not been reported previously.
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ranking = 13.885076888795
keywords = granulosa cell, granulosa
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7/30. Pseudo-precocious puberty caused by a juvenile granulosa cell tumour secreting androstenedione, inhibin and insulin-like growth factor-I.

    We report a female child who presented at age 3.92 years with a 2-year history of consonant pubertal development caused by a large right-sided ovarian juvenile granulosa cell tumour (JGCT). Although JGCTs causing pseudo-precocious puberty have been previously described, they remain rare and endocrine data are often incomplete. In this case the tumour was associated with raised serum oestradiol, androstenedione, inhibin and IGF-I. Histological changes were consistent with JGCT. Immunohistochemical studies revealed positive reactivity to MIC-2, inhibin, melan A, IGF-I and IGFBP-2.
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ranking = 36.986478119557
keywords = granulosa cell tumour, granulosa cell, granulosa, cell tumour
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8/30. Juvenile granulosa cell tumor in a 2-year-old infant: report of a case complicated with ascites and acute respiratory distress.

    A two-year-old girl with a juvenile granulosa cell tumor (JGCT) and with acutely progressive hydrothorax and ascites is presented. She had precocious pseudopuberty and an elevated level of serum estradiol. Sudden onset of respiratory distress, due to a pleural effusion and severe abdominal distention, led to an emergency laparatomy. Unilateral salpingo-oophorectomy induced a rapidly favorable course. Histological examination showed no evidence of invasion or peritoneal metastasis. This is the first case of JGCT associated with an acute respiratory emergency as a main clinical feature.
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ranking = 13.885076888795
keywords = granulosa cell, granulosa
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9/30. Juvenile granulosa cell tumour (JGCT) of the ovary in a 6-year-old girl: laparoscopic resection achieves long-term oncological success.

    Juvenile granulosa cell tumours (JGCT) represent a rare malignancy in childhood and their laparoscopic resection has not been advocated yet. We report on a 6-year-old girl with signs of precocious pseudo-puberty and an abdominal tumour. work-up revealed premature thelarche, vaginal discharge, elevated estrogen levels, and a solid tumour in the lower pelvis (6 x 4 x 3 cm in MRI). The girl underwent laparoscopy (3 ports, 5-mm instruments), during which a non-invasive, mobile tumour of the left ovary was found. Since all margins of resection could be clearly identified, salpingo-oophrectomy was performed, using the harmonic scalpel for dissection and 5-mm clips to ligate the fallopian tube. Histopathology revealed a JGCT with an intact capsule (FIGO 1 a) which required no further chemotherapy. Within 3 months postoperatively the girl's signs of precocious puberty had resolved and at present, after a follow-up of more than 3 years, there is no evidence of tumour recurrence. Minimally invasive surgery of solid ovarian tumours in children remains controversial. However in the present case, laparoscopic resection did not compromise the surgical and oncological safety.
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ranking = 36.986478119557
keywords = granulosa cell tumour, granulosa cell, granulosa, cell tumour
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10/30. Pseudo-precocious puberty in a Kenyan African child: a case report.

    A female Kenyan child with pseudoprecocious puberty due to juvenile granulosa cell tumour is presented. Clinical features in this patient included a mass in the lower abdomen, a growth spurt and appearance of pubic hairs as well as enlargements of the breasts. No similar case has been reported in a Kenyan child. The management and review of literature are discussed.
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ranking = 7.3972956239113
keywords = granulosa cell tumour, granulosa cell, granulosa, cell tumour
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