Disorders of sex development -A complex puzzle for the doctor and the patient-A rare case report
Journal Title: IP Archives of Cytology and Histopathology Research - Year 2018, Vol 3, Issue 4
Abstract
Disorders of Sex Development DSD are referred to a group of disorders in which at least two out of gonadal sex genetic sex phenotypic sex and genital tract sex show discordance between them Among the three main categories of DSD 46XY DSD is a rarer type which we encountered in our case A 14 year old female presented with abdominal pain and lump since 10 days On clinical examination she had external sexual characters of a female Karyotyping revealed a 46XY type Intraoperatively there was a normal uterus with a tumor mass in the location of the right gonad which was removed along with a biopsy taken from the contralateral gonad both intraabdominal in location Histopathologic examination of the tumor mass revealed a malignant mixed germ cell tumour GCT comprising of dysgerminoma and yolk sac tumour and the contralateral gonad was found to be testis with evidence of intratubular germ cell neoplasm unclassified IGCNU Patient underwent 4 cycles of chemotherapy after the histopathologic diagnosis but was later lost to followup The relative ambiguity of these disorders with their propensity to develop into type II GCTs makes these cases unique perplexing and of utmost importanceKeywords 46XY DSD Mixed GCT IGCNU
Authors and Affiliations
Vivek Parameshwar, Kusum Jashnani, Sneha D. Sirodkar
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