A case report and literature review of steroid cell tumours, not otherwise specified
DOI:
https://doi.org/10.18597/rcog.308Keywords:
ovarian steroid cell tumour, ovarian neoplasia, virilisationAbstract
Introduction: ovarian steroid cell tumours, not otherwise specified (NOS), is a not very frequently occurring ovarian sex chord neoplasia. It is characterised by becoming presented at any age and producing different endocrine manifestations due to the hormonal influx of patients suffering it. It has 50% potential malignancy.
Objective: reporting the case of a 50 yearold patient diagnosed by histology and immunohistochemistry studies as having malign steroid cell tumour NOS. Carrying out a literature review for the clinical presentation, genetics, diagnosis and treatment of this not very frequently occurring type of neoplasia.
Methodology: an electronic search of the literature we carried out using the following databases: MEDLINE, Embase, HINARI, Ovid, SciELO y BIREME. The search was limited to include the most outstanding original research and review articles from 1987 to 2008.
Results: 74 pertinent case reports were found. Clinical manifestations were variable and nonspecific according to the production of hormones such as testosterone, oestrogen or progestagen. Abdominal pain, severe ascites and Cushing’s syndrome were also presented. Differential diagnosis included other steroid tumours such as luteinizing thecoma, luteoma during pregnancy, primary clear cell carcinoma, metastatic renal cell carcinoma and hilar and non-hilar Leydig cell tumours. Treatment is by surgery accompanied by gonadotropin-releasing hormone (GnRH) agonist treatment.
Conclusion: steroid cell tumours occur infrequently and are associated with endocrine syndromes. Their management has not been standardised and must be individualised according to tumour histology, surgical state and the desire to become fertile. Studies are required for making advances in gaining knowledge about these tumours.
Author Biographies
Javier Andrés Ramírez
Óscar Suescún
María Cristina Palomino
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