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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Adrenocortical carcinoma (ACC) is a rare, often fatal disease, that may be seen sporadically or with hereditary predisposition syndromes. Patients with ACC are usually girls under the age of seven who present signs of excess production of adrenal glucocorticoids and androgens, with the diagnosis being confirmed by imaging. Here we reproduce and examine what we believe to be the first autopsy case report of a child with ACC, reported by Dr. Henry Sampson in Philosophical Transactions, published by The Royal Society of London in 1697. The paper describes the autopsy of a girl with severe virilization and profound signs of Cushing syndrome who died at age six, strongly suggesting ACC. She apparently had extensive pulmonary metastases, and may have had liver involvement. The report indicates her disease arose from her left kidney and there is no indication of an adrenal origin, perhaps because the adrenal gland was not generally known as a separate organ at that time. This classic example of an early case report is particularly instructive in the context of medical knowledge and understanding in the 17th century compared to current knowledge.
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PMID:Adrenocortical carcinoma in a 17th-century girl. 2696 Feb 2

In postmenopausal women presenting with virilization and elevated testosterone levels, laparoscopic salpingo-oophorectomy should be considered after exclusion of adrenal causes. A clinicopathological study was conducted among those women who presented with features of hyperandrogenism in our postmenopausal clinic over a period of 2 years. Relevant past medical and surgical histories were elicited. Basic hormonal evaluation and radiological imaging were done. Laparoscopic bilateral salpingo-oophorectomy was done. Six weeks postoperatively, serum testosterone was undetectable with significant clinical improvement. There was no recurrence of symptoms during the follow-up period of 2 years. Treatment of postmenopausal women with hyperandrogenism and virilization with laparoscopic bilateral salpingo-oophorectomy is effective if she has no pronounced ovarian enlargement or adrenal tumor on imaging. An extensive endocrine testing and a detailed search for metastatic disease may be unnecessary.
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PMID:Postmenopausal hyperandrogenism of ovarian origin: A clinicopathologic study of five cases. 2809 44

Adrenocortical carcinomas (ACCs) are rare and often aggressive with more than 50% of the cases already in stage III-IV (ENSAT) at the time of diagnosis. Nearly 60% of ACCs present with hormone overproduction syndromes (Cushing's syndrome and/or virilization), while the rest present with abdominal mass or incidental finding. Aggressive surgical resection is the mainstay of treatment usually followed by adjuvant mitotane monotherapy. For the advanced stage, adjuvant radiotherapy and combined chemotherapy with mitotane therapy can be added for survival benefit. Here, we would like to report a case of stage III high-grade ACC without syndromes of hormone overproduction, initially presented with pulmonary embolism. It was rapidly progressive with metastases to lungs, peritoneum and bone despite aggressive surgery followed by adjuvant mitotane monotherapy. However, after palliative radiotherapy to thoraco-lumbar spine for spinal cord compression, and adding chemotherapy (six cycles of EDP: etoposide, doxorubicin, cisplatin) to mitotane, a significant partial remission was achieved. He has had 24 months of progression-free survival, and is currently on mitotane monotherapy with cortisol replacement. Discussion will support multimodality therapy for stage III high-grade ACC with surgery immediately followed by adjuvant radiotherapy and combined chemotherapy with mitotane therapy to prevent local recurrence and distant metastases.
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PMID:Pulmonary Embolism as an Initial Presentation of Adrenocortical Carcinoma. 2914 95

About 1% of ovarian tumors that comprise testicular cell types can cause hyperandrogenism followed by characteristic virilization. Androgenic group of tumors originated mainly from sex-cord stromal ovarian tumors are including steroid cell tumors, Leydig tumors, granulosa cell tumors, Sertoli cell tumors, Sertoli-Leydig cell tumors, gonadoblastomas, and some other rare forms as ovarian metastases from neuroendocrine tumors. Germline or somatic mutations in some genes like DICER1, STK11, and FOXL2 are associated with the development of some sex cord-stromal ovarian tumors. Basal serum testosterone concentrations above 7 nmol/L could indicate an androgen-secreting tumor. Other ovarian and adrenal androgens should be determined and functional endocrine testing including low-dose dexamethasone suppression test, gonadotrophin-releasing hormone (GnRH) agonist test, imaging methods, and selective venous sampling should be performed. Surgery is the first-line treatment for most of the tumors. Women who are not good surgical candidates could benefit from use of GnRH agonist to control hyperandrogenism. In some cases, chemotherapy and/or radiation therapy is required while some tumors respond on antiangiogenic agents used alone or in combination with chemotherapy. Metabolic implications and long-term outcomes of ovarian androgen-secreting tumors are unknown and require more detailed follow-up in multicentric and longitudinal clinical studies.
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PMID:Androgen-Secreting Ovarian Tumors. 3149 93


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