Shahrir et al. Journal of Medical Case Reports 2010, 4:97 http://www.jmedicalcasereports.com/content/4/1/97 C A S E R E P O R T JOURNAL OF MEDICAL CASE REPORTS Open Access Extragonadal germ cell tumor presenting in a woman with systemic lupus erythematosus: a case report Mohd Shahrir*, Abdul Halim, Soehardy Zainudin, Rozita Mohamad, Loo C Yuen, Rashidi Saidin, Norella Kong Abstract Introduction: Germ cell tumor of the pituitary gland is a very rare occurrence. Case presentation: We describe the case of a 28-year-old Malaysian Malay woman with lupus nephritis who complained of a three month headache and blurring of vision. She was found to have a pituitary mass, which was later proven to be a germ cell tumor. As of writing this case report, her disease is in remission. Conclusion: The disruption of the pituitary gonad axis could affect the disease activity by reducing immunoregulatory control. Introduction Germ cell tumor of the pituitary gland is a very rare occurrence. This case report describes a 28-year-old Malaysian Malay woman with lupus nephritis who com- plained of headache and blurring of vision. She was later found to have a pituitary germ cell tumor. Pituitary germ cell tumor is considered as a type of extragonadal germ cell tumor. They represent 5% of germ cell tumors and typically arise in midline locations. The specific location of the tumor varies with the patient’s age [1]. The most common sites of origin in adults are the anterior mediastinum, the retroperito- neum, and the pineal and suprasellar regions of the brain. Meanwhile, in infants and young children, the sacrococcyx is the most common site of extragonadal germ cell tumors, followed by intracranial sites [2]. In contrast to primary gonadal germ cell tumors, the only known risk factor for extragonadal germ cell tumors is the Klinefelter syndrome (47XXY), which is associated with mediastinal nonseminomatous germ cell tumors [3]. The age of onset, site of origin, and histologic type of the tumor are important distinguishing characteristics for its prognosis and treatment. * Correspondence: drobiwan@gmail.com Department of Medicine, Faculty of Medicine, Universiti Kebangsaan, Malaysia Case presentation A 28-year-old Malaysian Malay woman presented in 1994 with musculoskeletal manifestation of systemic lupus erythematosus (SLE). In 1999, she developed nephrotic syndrome secondary to lupus nephritis (World Health Organization Class III). She was immedi- ately started on low-dose cyclophosphamide and myco- phenolate mofetil treatment. A year later, however, she complained of amenor- rhoea. Levels of follicle-stimulating hormone and lutei- nizing hormone were both lower than would have been expected. Nine months later, she complained of persis- tent headaches and blurring of vision. She was screened for chronic meningitis but had a normal brain computed tomography (CT) scan. However, we were not able to do a lumbar puncture as she refused this procedure. Her SLE was already in remission at that time. On examination, both her peri-orbital areas were swollen and red. Her conjunctivae were also injected. She had bilateral temporal hemianopia with left optic atrophy. Magnetic resonance imaging revealed a lobu- lated mass which extended into the suprasellar cistern and floor of her third ventricle, splaying the optic chiasm. She underwent pituitary resection and her histopatho- logical examination later revealed a germ cell tumor (Figure 1). She was referred subsequently for radiotherapy. © 2010 Shahrir et al; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.