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Best Practice & Research. Clinical... May 2020Childhood adrenocortical tumors (ACTs) are rare, representing ∼0.2% of all pediatric malignancies and having an incidence of 0.2-0.3 new cases per million per year in... (Review)
Review
Childhood adrenocortical tumors (ACTs) are rare, representing ∼0.2% of all pediatric malignancies and having an incidence of 0.2-0.3 new cases per million per year in the United States, but incidences are remarkably higher in Southern Brazil. At diagnosis, most children show signs and symptoms of virilization, Cushing syndrome, or both. Less than 10% of patients with ACT exhibit no endocrine syndrome at presentation, although some show abnormal concentrations of adrenal cortex hormones. Pediatric ACT is commonly associated with constitutional genetic and/or epigenetic alterations, represented by germline TP53 mutations or chromosome 11p abnormalities. Complete tumor resection is required to achieve cure. The role of chemotherapy is not established, although definitive responses to several anticancer drugs are documented. For patients undergoing complete tumor resection, favorable prognostic factors include young age, small tumor size, virilization, and adenoma histology. Prospective studies are necessary to further elucidate the pathogenesis of ACT and improve patient outcomes.
Topics: Adenoma; Adrenal Cortex Neoplasms; Age of Onset; Antineoplastic Agents; Child; Child, Preschool; Female; Germ-Line Mutation; Humans; Incidence; Male; Prognosis; Prospective Studies; Virilism
PubMed: 32636100
DOI: 10.1016/j.beem.2020.101448 -
European Journal of Gynaecological... 2011A case is reported of a 27-year-old pregnant woman with ovarian tumors, measuring 12 cm and 11.5 cm in the greatest diameter, discovered during investigation for... (Review)
Review
A case is reported of a 27-year-old pregnant woman with ovarian tumors, measuring 12 cm and 11.5 cm in the greatest diameter, discovered during investigation for virilization symptoms. Termination of the pregnancy at the 22nd week of gestation and tumorectomy with both adnexa were performed, with the provisional diagnosis of arrhenoblastoma. Pathological examination of the tumors showed typical Krukenberg neoplasms and subsequent upper GI tract endoscopy revealed a gastric cancer that was excised. The pathological examination revealed a diffuse type gastric adenocarcinoma with signet ring morphology, similar to ovarian tumors. In any case of ovarian tumor with unusual hormonal manifestations, in addition to hormonally active sex cord-stromal neoplasms, metastatic ovarian tumors must be considered as well, especially in cases of bilateral tumors.
Topics: Abortion, Therapeutic; Adult; Female; Gastrectomy; Humans; Krukenberg Tumor; Ovarian Neoplasms; Pregnancy; Pregnancy Complications, Neoplastic; Stomach Neoplasms; Virilism
PubMed: 21797128
DOI: No ID Found -
Problemy Endokrinologii Dec 2019SertoliLeydig cell tumor is a rather rare type of ovarian neoplasms belonging to the group of sex cordstromal tumors. This malignancy is characterized by androgen...
SertoliLeydig cell tumor is a rather rare type of ovarian neoplasms belonging to the group of sex cordstromal tumors. This malignancy is characterized by androgen overproduction, which results in the so-called virilization and can be accompanied by various metabolic disorders such as abdominal obesity, disturbances of carbohydrate and protein metabolism, and high blood pressure. During differential diagnosis, it is important to identify the source of androgen overproduction. An androgen-secreting ovarian tumor needs to be differentiated from androgen-secreting adrenal tumor, ovarian stromal thecomatosis (hyperthecosis), and endogenous hypercorticism (the Cushings syndrome). In most cases, the SertoliLeydig cell tumor is associated with DICER1 mutation carriership. If a patient is found to carry the DICER1 mutation, patients relatives need to undergo genetic testing as the individuals with mutations in this gene have an elevated risk of developing a broad range of benign and malignant tumors (most of these tumors are relatively rare in the overall population). The awareness of this rare ovarian neoplasm among medical specialists (obstetriciansgynecologists, endocrinologists, and oncologists) is supposed to ensure timely diagnosis and adequate treatment of this disease.
Topics: DEAD-box RNA Helicases; Diagnosis, Differential; Female; Humans; Male; Mutation; Ovarian Neoplasms; Ribonuclease III; Sertoli-Leydig Cell Tumor; Virilism
PubMed: 32202730
DOI: 10.14341/probl10222 -
Postgraduate Medical Journal Mar 1960
Topics: Female; Humans; Syndrome; Virilism
PubMed: 14422652
DOI: 10.1136/pgmj.36.413.176 -
Ryoikibetsu Shokogun Shirizu 1993
Review
Topics: Female; Humans; Ovarian Neoplasms; Sertoli-Leydig Cell Tumor; Virilism
PubMed: 7757742
DOI: No ID Found -
Southern Medical Journal Mar 1960
Topics: Adrenal Cortex; Adrenal Cortex Neoplasms; Female; Humans; Medical Records; Neoplasms; Virilism
PubMed: 14408472
DOI: 10.1097/00007611-196003000-00006 -
Gynecological Endocrinology : the... Aug 2016Oncocytic tumors of the adrenal cortex are rare, mostly nonfunctioning and benign.
CONTEXT
Oncocytic tumors of the adrenal cortex are rare, mostly nonfunctioning and benign.
SETTING
Report virilizing oncocytic adrenocortical carcinoma in a 50-year-old woman.
PATIENT
She presented a recent and progressive virilization syndrome, associated with high blood pressure. Hormonal evaluation showed elevated serum testosterone and delta-4-androstenedione levels, normal urinary free cortisol level and incomplete suppression of cortisol at the 1 mg dexamethasone suppression test. CT scan of the abdomen revealed a 35 mm left adrenal mass.
INTERVENTION
The patient underwent a left adrenalectomy, and the histological study showed a 3 cm oncocytic adrenocortical carcinoma with signs of malignancy.
RESULTS
Immunohistochemical study revealed that tumor cells expressed the steroidogenic enzymes involved into androgen synthesis (3βHSD and P450c17α), P450 aromatase and luteinizing hormone (LH) receptors. Post-operatively, signs of virilization improved rapidly, serum testosterone and delta-4-androstenedione levels returned to normal, as did the dexamethasone suppression test. During follow-up CT-scan and 18-FDG PET/CT showed a right ovary mass, corresponding to a follicular cyst associated with hyperthecosis. The patient is alive with no recurrence 48 months after adrenal surgery.
CONCLUSION
Oncocytic adrenocortical carcinomas, although extremely rare, should be considered in women with a virilization syndrome. In this woman immunohistochimical studies revealed the presence of steroidogenic enzymes involved into androgen synthesis and aromatization, and LH receptors could be implicated in this pathology.
Topics: Adenoma, Oxyphilic; Adrenal Cortex Neoplasms; Adrenocortical Carcinoma; Female; Humans; Immunohistochemistry; Middle Aged; Virilism
PubMed: 26954035
DOI: 10.3109/09513590.2016.1149811 -
BMJ Case Reports Jun 2021Adrenocortical carcinoma (ACC) is a rare malignancy, with an estimated annual incidence of 0.7-2 cases per million and a median overall survival of 3-4 years....
Adrenocortical carcinoma (ACC) is a rare malignancy, with an estimated annual incidence of 0.7-2 cases per million and a median overall survival of 3-4 years. Hormone-secreting ACCs represent most cases; of these, only a small minority presents with virilisation alone. Early diagnosis is key to increase the chances of a better outcome. Here, we report a case of a 41-year-old woman who presented with menstrual irregularities, hirsutism and virilising symptoms, associated with abdominal discomfort and constitutional symptoms. On physical examination, there was a palpable mass in the right upper quadrant. Laboratory workup revealed elevated serum androgens. The imaging study showed a 163×110×122 cm right adrenal mass with features consistent with ACC and suggested potential hepatic invasion. Our patient underwent surgical resection, and the histopathological findings confirmed the diagnosis. She was referred to a specialised centre for follow-up and adjuvant therapy.
Topics: Adrenal Cortex Neoplasms; Adrenocortical Carcinoma; Adult; Androgens; Female; Humans; Liver; Virilism
PubMed: 34083198
DOI: 10.1136/bcr-2021-242895 -
Obstetrics and Gynecology Mar 1989A 71-year-old woman, 26 years post-menopause, presented with virilization. Facial hirsuties, non-hereditary frontal balding, voice changes, male escutcheon, and mild...
A 71-year-old woman, 26 years post-menopause, presented with virilization. Facial hirsuties, non-hereditary frontal balding, voice changes, male escutcheon, and mild clitorimegaly accompanied a right adnexal mass. Blood hormone studies showed testosterone 430 ng/dL, FSH 118 mIU/mL, and LH 210 mIU/mL. By ultrasound examination, the cystic adnexal mass involved the right ovary. An 18-cm, 1300-g, unicameral mass with 1200 mL of clear serous fluid and with smooth inner and outer surfaces was removed from the right broad ligament. Intraoperative testosterone levels were as follows: peripheral vein 285 ng/dL, left ovarian vein 301 ng/dL, and right ovarian vein 1635 ng/dL; tumor cystic fluid was 3032 ng/dL. Peripheral vein testosterone was 15 ng/dL 3 days postoperatively. Histopathologically, the tumor was a serous cystadenoma. No evidence of stromal luteinization, hyperplasia, or inflammation was found, and other virilizing lesions were not encountered in either ovary or in other tissues. Epithelial cells constituting the tumor may have been the source of the excess testosterone in this unique case of virilizing serous cystadenoma.
Topics: Aged; Cystadenoma; Female; Humans; Ovarian Neoplasms; Ovary; Testosterone; Virilism
PubMed: 2915883
DOI: No ID Found -
The Journal of Clinical Endocrinology... Feb 1947
Topics: Female; Humans; Neoplasms; Ovarian Neoplasms; Ovary; Virilism
PubMed: 20240742
DOI: 10.1210/jcem-7-2-115