Carcinoma adrenal virilizante en una adolescente: reporte de caso / Virilizing adrenal carcinoma in an adolescent: case report

Los tumores suprarrenales virilizante son infrecuentes y representan 5-6% de los tumores de esas glándulas1. Pueden secretar diferentes andrógenos como dehidroepiandrosterona sulfato (DHEAS), androstenediona y testosterona. Las características clínicas dependen de la edad de presentación; en niños pueden determinar pubertad precoz y en mujeres en edad fértil ocasionar hirsutismo, amenorrea o ciclos oligomenorreicos y diversos grados de virilización2. Los carcinomas adrenocorticales son tumores raros y la incidencia es aproximadamente uno a dos por millón de habitantes/año3,4. Los exámenes de imagen como la tomografía o la resonancia confirman el origen suprarrenal, valoran la presencia de metástasis y definen la conducta terapéutica5. La presentación inicial en pacientes pediátricos mayoritariamente es con virilización6 y aproximadamente el 50% de los pacientes adultos con carcinoma adrenal tienen un estadio de la enfermedad relativamente avanzado7. El tratamiento de elección es la cirugía y sigue siendo la mejor esperanza para la supervivencia a largo plazo8. El pronóstico habitual para el carcinoma adrenocortical es generalmente malo con una supervivencia global a 5 años de 20 a 25%5 en adultos, pero en niños y adolescentes la supervivencia puede llegar al 100%9. Se presenta el caso de una paciente con fenotipo totalmente masculino con diagnóstico de carcinoma adrenal virilizante que comienza en la infancia y se diagnostica en la adolescencia.

Virilizing adrenal tumors are uncommon and represent 5-6% on tumors of these glands1. They can secrete different androgens such as dehydroepiandrosterone sulfate (DHEAS), androstenedione, and testosterone. Clinical features depend on the age of presentation; in children they can determine precocious puberty and in women of childbearing age cause hirsutism, amenorrhea or oligomenorrheic cycles and various degrees of virilization2. Diagnosis consists of clinical evidence of hyperandrogenism, accompanied by an increase in androgens in the blood, especially DHEAS, whose origin is mainly adrenal. Adrenocortical carcinomas are rare and the incidence is approximately one to two per million inhabitants/year3,4. Imaging tests such as tomography or resonance confirm the adrenal origin, assess the presence of metastases and define the therapeutic approach5. In initial presentation in most pediatric patients is with virilization6 and approximately 50% adult's patients with adrenal carcinoma have a relatively advanced stage of the disease7. The treatment of choice is surgery and is the best hope for long-term survival. The usual prognosis for adrenocortical carcinoma is generally poor with a 5-year overall survival of 20 to 25%5 in adults, but in children and adolescent's survival can reach 100%9. We present the case of a patient with a totally male phenotype diagnosed with virilizing adrenal carcinoma that begins in childhood and is diagnosed in adolescence.

Functioning adrenocortical carcinoma causing virilisation: a case report

Adrenocortical carticnoma (ACC) is an uncommon tumor with an incidence of 1-2 cases/million/year. It has two peak incidences; the first one in the first decade and the second one in the fourth decade. Most patients present with features of steroid hormone excess or abdominal mass effects, but about 15% of ACC are diagnosed incidentally. It is hormonally functional in 80 - 100% patients and the predisposing lesions include congenital adrenal hyperplasia and adenoma. ACC has significant syndromic and genetic association. Surgery offers the best chance of cure, especially in localized disease. Here, we present the case of virilization in a young female child secondary to a functioning ACC. The child had classical hormonal and imaging features of functioning ACC and underwent resection of the tumor (Adrenalectomy) with good outcome.

"Double Trouble": A case of precocious puberty with concomitant malignant gluteal mass

@#Precocious puberty is the appearance of physical and hormonal signs of pubertal development at an earlier age than is considered normal. The causes may range from a variant of normal development to pathologic conditions such as glands that function abnormally early in life. This is a case of a seven year old female with clitoromegaly, early menarche and a gluteal mass accompanied by elevated 17-hydoxyprogesterone and decreased level of cortisol which supports the diagnosis of congenital adrenal hyperplasia. Gluteal mass cytological features are consistent with a malignant round cell tumor.

Reduction Clitoroplasty with Preservation of the Dorsal Neurovascular Pedicle in a 6-Year-Old Female: A Case Report

A 6-year-old female presented to our hospital with clitoromegaly. On physical examination, she demonstrated evidence of precocious pubic hair and clitoromegaly. The relaxed clitoris measured 3.7 cm in length. Her uterus was undetectable on diagnostic transvaginal ultrasonography and abdomen-pelvis computed tomography (CT) with otherwise normal-appearing internal female genitalia. We planned reduction clitoroplasty with preservation of the dorsal neurovascular pedicle to maintain sensitivity of the glans clitoris. After surgery, the patient demonstrated good recovery. The parents of the patient were satisfied with the surgical results. The patient was discharged without complications on hospital day 6.

Reduction Clitoroplasty with Preservation of Dorsal Neurovascular Pedicles

Conventional reduction clitoroplasty often leaves the glans clitoris without tactile sensation. Several clitoroplasty methods have been reported, but only a few describes preservation of dorsal neurovascular bundles. We present the case of 20-year- old female with symptomatic clitoromegaly since puberty, who underwent a clitoral reduction designed to preserve the neurovascular supply of the glans clitoris. The cosmetic outcome was excellent on postoperative examination and the patient reported normal sensation 6 months later. Preservation of?dorsal vascular pedicles showed a satisfactory result in sexually mature women.

A Case of Clitoroplasty with Preserving Neurovascular Supply to the Clitoris for Clitoromegaly in Congenital Adrenal Hyperplasia / 대한산부인과학회잡지

Congenital adrenal hyperplasia, which is caused by the disorder of the enzyme for synthesizingadrenal hormones, results in hypertropy of the clitoris due to overporduction of adrenalandrogen. Diagnosis of the congenital adrenal hyperplasia can be obtained by the study ofsteroid levels in blood and urine. The blood levels of ACTH, testosterone and 17-hydroxyprogesteronewere increased and the concentration of 17-ketosteroid were decreased after the dexamethasonesuppression test. The internal genital organ was normal appearance on the pelvic CTscan. Clitoromegaly due to congenital adrenal byperplasia was corrected previously by the variousoperational procedures in which the clitoris was removed entirely for only physial appearance.However the sensitive function of the clitoris after the operative correction throughpuberty is to be preserved by the method "clitoroplasty" for preserving neurovascular supply tothe clitoris. The writer experienced a case of a 5-year-old girl with congenital adrenalhyperplasia and tried this clitoroplasty for surgical treatment of clitoromegly for preservation ofpostoperative sensitivity of the clitoris.