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Journal of Pediatric Surgery Aug 2000The authors report a case of a virilizing adrenal tumor that developed in a 2-year-old child with Beckwith-Wiedemann syndrome (BWS). He had a fetal diagnosis of... (Review)
Review
The authors report a case of a virilizing adrenal tumor that developed in a 2-year-old child with Beckwith-Wiedemann syndrome (BWS). He had a fetal diagnosis of omphalocele and a history of neonatal adrenal cysts. The importance of prenatal diagnosis of BWS and postnatal follow-up of tumors is discussed. The differential diagnosis of adrenal pathologies occurring in BWS also is reviewed.
Topics: Adrenal Cortex Neoplasms; Beckwith-Wiedemann Syndrome; Child, Preschool; Follow-Up Studies; Humans; Male; Prenatal Diagnosis; Time Factors; Virilism
PubMed: 10945711
DOI: 10.1053/jpsu.2000.8771 -
Progress in Pediatric Surgery 1983In the treatment of AGS, the pediatric surgeon is faced with the task of correcting the form and appearance of the virilized female genitalia as early as possible. For... (Review)
Review
In the treatment of AGS, the pediatric surgeon is faced with the task of correcting the form and appearance of the virilized female genitalia as early as possible. For severe forms, a pull-through operation of the vagina and closure of the urethral fistula is necessary. At the same time, the hypertrophied clitoris should be corrected by a reduction plasty or backward displacement of the crura. The prospects for cure after treatment of adrenal cortical or ovarian tumours are usually poor. The tumours are malignant in over 85% of cases and metastasize early. The secondary sexual characteristics produced by the tumours regress only partially. When bony development is advanced, these patients inevitably remain small after growth ceases. Cortisone substitution under the guidance of the pediatric endocrinologist is essential at all surgical procedures.
Topics: Adrenal Cortex Neoplasms; Adrenal Hyperplasia, Congenital; Child; Clitoris; Female; Genitalia, Female; Humans; Infant, Newborn; Infant, Newborn, Diseases; Ovarian Neoplasms; Virilism
PubMed: 6348885
DOI: No ID Found -
American Journal of Clinical Pathology Apr 1972
Topics: Edema; Female; Humans; Hyperplasia; Ovarian Diseases; Polycystic Ovary Syndrome; Torsion Abnormality; Virilism
PubMed: 5012952
DOI: 10.1093/ajcp/57.4.545 -
The American Journal of Surgical... Nov 1986We describe the third report testosterone-producing, virilizing, adrenal Leydig cell adenoma, which was identified in a oophorectomized postmenopausal female patient....
We describe the third report testosterone-producing, virilizing, adrenal Leydig cell adenoma, which was identified in a oophorectomized postmenopausal female patient. Light- and electron-microscopy demonstrated typical Leydig cell differentiation, including numerous intracytoplasmic and intranuclear Reinke crystals and rice-like bodies (elementary tubular inclusions). Testosterone production by the adenoma was demonstrated by the immunoperoxidase technique. We propose that adrenal Leydig cell adenomas arise as a result of ovarian gonadal stromal metaplasia associated with elevated follicle-stimulating hormone and luteinizing hormone in the postmenopausal female patient. Adrenal Leydig cell lesions must be considered in the virilized woman with elevated testosterone levels and normal levels of the adrenal androgens and their 17-ketosteroid metabolites.
Topics: Adenoma; Adrenal Gland Neoplasms; Female; Humans; Leydig Cells; Male; Microscopy, Electron; Middle Aged; Testosterone; Virilism
PubMed: 3022613
DOI: 10.1097/00000478-198611000-00009 -
Rassegna Internazionale Di Clinica E... Jun 1972
Topics: Adult; Female; Humans; Ovarian Neoplasms; Sertoli-Leydig Cell Tumor; Virilism
PubMed: 4340717
DOI: No ID Found -
Journal of Pediatric and Adolescent... Jun 2009The clinical observation of virilization is a rare finding that has a number of possible explanations. Overall, ovarian tumors causing virilization are exceedingly rare...
BACKGROUND
The clinical observation of virilization is a rare finding that has a number of possible explanations. Overall, ovarian tumors causing virilization are exceedingly rare and mostly occur in post-menopausal women. In fact, there are no reported cases of virilization from a testosterone-producing ovarian dermoid in the adolescent female age group. The most frequent germ cell tumor derived from the ovaries is the benign cystic teratoma (dermoid) which accounts for 25% of all ovarian neoplasms. Teratomas consist of tissues that recapitulate the ectoderm, endoderm, and mesoderm. Usually the tumors are asymptomatic, but they occasionally can cause severe pain if there is torsion or if sebaceous material perforates the cyst wall, leading to reactive peritonitis.
CASE
A 12-year-old female was found to have a large 3 5 x 19 x 12 cm ovarian mature cystic teratoma arising from her right ovary. The patient also displayed evidence of masculinization demonstrated by a deepening voice and clitoromegaly. The dermoid was producing large amounts of testosterone from a nest of Leydig cells found pathologically in the mass.
CONCLUSION
Benign cystic teratomas can produce active hormones, albeit rarely. This is a finding important to consider when ovarian cystectomy is performed for removal of a benign cystic teratoma.
Topics: Child; Dermoid Cyst; Female; Humans; Leydig Cells; Male; Ovarian Neoplasms; Testosterone; Virilism
PubMed: 19539195
DOI: 10.1016/j.jpag.2008.05.012 -
Journal of Clinical Research in... Mar 2017Congenital adrenal hyperplasia (CAH) is classified as classical CAH and non-classical CAH (NCCAH). In the classical type, the most severe form comprises both... (Review)
Review
Congenital adrenal hyperplasia (CAH) is classified as classical CAH and non-classical CAH (NCCAH). In the classical type, the most severe form comprises both salt-wasting and simple virilizing forms. In the non-classical form, diagnosis can be more confusing because the patient may remain asymptomatic or the condition may be associated with signs of androgen excess in the postnatal period or in the later stages of life. This review paper will include information on clinical findings, symptoms, diagnostic approaches, and treatment modules of NCCAH.
Topics: Adrenal Hyperplasia, Congenital; Child; Diagnosis, Differential; Female; Genetic Testing; Humans; Male; Mutation; Steroid 21-Hydroxylase; Virilism
PubMed: 27354284
DOI: 10.4274/jcrpe.3378 -
Journal of Korean Medical Science Jul 2010Functioning adrenocortical oncocytomas are extremely rare and most reported patients are 40-60 yr of age. To our knowledge, only 2 cases of functioning adrenocortical...
Functioning adrenocortical oncocytomas are extremely rare and most reported patients are 40-60 yr of age. To our knowledge, only 2 cases of functioning adrenocortical oncocytomas have been reported in childhood. We report a case of functioning adrenocortical oncocytoma in a 14-yr-old female child presenting with virilization. She presented with deepening of the voice and excessive hair growth, and elevation of plasma testosterone and dehydroepiandrosterone sulfate. She had an adrenalectomy. The completely resected tumor composed predominantly of oncocytes without atypical mitosis and necrosis. A discussion of this case and a review of the literature on this entity are presented.
Topics: Adenoma, Oxyphilic; Adolescent; Adrenal Cortex Neoplasms; Adrenalectomy; Adult; Female; Humans; Male; Middle Aged; Virilism
PubMed: 20592902
DOI: 10.3346/jkms.2010.25.7.1077 -
Virchows Archiv : An International... Aug 1997Three further cases of mature benign cystic teratomas of the ovary associated with virilization are added to the three previously reported in the literature. They were...
Three further cases of mature benign cystic teratomas of the ovary associated with virilization are added to the three previously reported in the literature. They were found in postmenopausal, obese, diabetic women aged 52, 61, and 67 years. The patients presented with hirsutism and voice changes and clitoromegaly was present in one. Testosterone and androstenedione levels were elevated but promptly regressed after removal of the tumours. Histologically, sheets of stromal luteinized cells were found peripherally at the interface between the neoplasm and ovarian tissue. Luteinization of ovarian stroma induced by an unknown factor related to diabetes mellitus is the origin of the virilization.
Topics: Aged; Androstenedione; Female; Humans; Middle Aged; Ovarian Neoplasms; Postmenopause; Teratoma; Testosterone; Virilism
PubMed: 9293897
DOI: 10.1007/s004280050081 -
Nihon Rinsho. Japanese Journal of... Feb 2004Aromatase, a key enzyme in estrogen synthesis, is tissue-specifically regulated in various tissues and plays an important role through endocrine and intracrine estrogen... (Review)
Review
Aromatase, a key enzyme in estrogen synthesis, is tissue-specifically regulated in various tissues and plays an important role through endocrine and intracrine estrogen production in various physiological functions. Therefore, aromatase deficiency caused crucial impairments of physiological functions in the gonadal tissues as well as extra-gonadal tissues. Because aromatase is protective for androgenic exposure by catabolizing, virilization of a pregnant mother and pseudohermaphroditism of a baby girl consequently result from the deficiency. Similarly, because aromatase is productive for a multifunctional physiological factor, estrogens, impaired metabolisms of bone, carbohydrate, and fat etc. result from the deficiency. We discuss the etiology, clinical symptoms, and therapeusis by classifying it into two types of complete and incomplete aromatase deficiencies.
Topics: Androgens; Aromatase; Disorders of Sex Development; Estrogen Replacement Therapy; Estrogens; Female; Humans; Infant, Newborn; Male; Metabolic Diseases; Placenta; Pregnancy; Pregnancy Complications; Urogenital Surgical Procedures; Virilism
PubMed: 14968547
DOI: No ID Found