Finch et al. Journal of Medical Case Reports 2012, 6:148 http://www.jmedicalcasereports.com/content/6/1/148 Minor criteria – Macrocephaly – Congenital malformations such as cleft lip or palate, frontal bossing, coarse face, or hypertelorism – Other skeletal abnormalities such as Sprengel’s deformity, marked pectus deformity, or syndactyly – Radiologic abnormalities such as bridging of the sella turcica, vertebral anomalies (for example, hemivertebrae, fusion or elongation of the vertebral bodies), modeling defects of the hands and feet, or flame-shaped lucencies of the hands or feet – Ovarian fibroma – Medulloblastoma Additionally, linkage analysis or polymerase chain re- action for direct mutation detection can be used [6]. Al- though the number of basal cell carcinoma lesions of the skin can be very high, it is important to note that the patient does not require multiple skin lesions for diagnosis, and that 10% of patients over the age of 30 years do not have these lesions [1,6]. The commonest gynecological manifestation is bilat- eral ovarian fibromas, however endometrial adenocar- cinoma and ovarian fibrosarcoma have been described [1,7,8]. Ovarian fibromas are neoplasms of ovarian stro- mal cells which most commonly arise after puberty and constitute approximately 5% of ovarian neoplasms. Ovarian fibromas do not elaborate hormones and, in nonsyndromic cases, are usually unilateral with an aver- age size of 6 cm. Cases of ovarian fibromas arising be- fore puberty have been reported both in association with Gorlin syndrome and sporadically [8,9]. Ovarian fibro- mas were first reported to occur in conjunction with Gorlin syndrome in 1963 [8]. Syndromic fibromas are most often bilateral and calcified [1]. We discuss a uni- lateral ovarian fibroma in a patient with Gorlin syn- drome in the absence of any basal cell carcinoma skin lesions. Case presentation Our patient was a Caucasian woman, 22 years of age and born by Cesarean section following an unremarkable gestation. She was described as having normal develop- ment and unremarkable schooling. Our patient had a history of recurrent dental infections and had multiple odontogenic keratocysts removed at ages 7, 13 and 20 from her maxilla and mandible (Figure 1A,C,D). At age 13, our patient was admitted to hospital for suspected viral meningitis. During this admission, a computed tomography scan of our patient’s head revealed calcifica- tion of her cerebral falx (Figure 1B). She required cor- rective lenses as a child and was diagnosed with a right optic nerve coloboma at the age of 13 years. Page 2 of 5 A diagnosis of Gorlin syndrome was initially suggested in 2009 when our patient was 20 years old, following the third excision of odontogenic keratocysts. At that time, investiga- tions were also made for our patient’s irregular menses, with a pelvic ultrasound. The investigation revealed a 10 cm complex mass in the midline pelvis, suspected to be arising from the right adnexa (Figure 1F). The features of the mass were unclear and suggestive of ovarian neoplasm. An urgent computed tomography scan with intravenous contrast was performed, which demonstrated a multilocu- lated complex mass measuring 10.4 cm in largest dimen- sion that represented either unilateral or bilateral ovarian masses (Figure 1E). There were solid and cystic areas with calcifications. The differential diagnosis at this point included a teratoma, other ovarian malignancies and chronic recurrent partial ovarian torsion. Our patient was scheduled for a right salpingo-oophorectomy with possible conversion to a bilateral salpingo-oophorectomy if there was intraoperative suspicion of malignancy. Given that she was nulliparous and desired to have children in the future, our patient was offered egg retrieval and embryo cryo- preservation if the surgery was bilateral. Our patient under- went a successful right salpingo-oophorectomy with an intraoperative note of a 1 cm simple cyst of the left ovary and a Meckel’s diverticulum. The gross specimen received from the right salpingo- oophorectomy consisted of a 12 × 10.5 × 7 cm cystic mass with a partial solid component measuring 4.5 × 4 × 3 cm (Figure 2A). An attached fallopian tube was identified measuring 7 × 0.7 × 0.6 cm. The cut sur- face of the cystic component of the mass revealed a dif- fuse gelatinous appearance. Approximately 10 mL of pale straw-colored fluid was extruded. The cut surface of the firm component revealed a partly solid white-tan whorled appearance with surrounding areas of suspected normal ovarian parenchyma. On microscopic examination, the specimen demon- strated compressed ovarian tissue with secondary edema, follicular cysts and corpora lutea. The lesion was well- circumscribed with a variably cellular spindle cell prolif- eration arranged in intersecting bundles with abundant myxoid stroma. Areas of cystic degeneration and calcifica- tion were present. There was no cellular atypia and the mitotic rate did not exceed three per 10 high power fields (Figure 2B-D). Immunohistochemical staining was per- formed using an automated immunostainer (Ventana Benchmark XT, Ventana Medical Systems Inc., Tuscon, AZ, USA). Antibodies against smooth muscle actin (1A4, prediluted; Cell Marque, Rocklin, CA, USA), desmin (DE- R-11, prediluted; Ventana Medical Systems Inc.), vimentin (V9, prediluted; Ventana Medical Systems Inc.), calretinin (polyclonal, prediluted; Cell Marque), and inhibin (R1, 1:50; Dako North America Inc, Carpinteria, CA, USA) were used. Immunohistochemical staining of the lesion