BACKGROUND: Nevoid basal cell carcinoma syndrome (NBCCS, Gorlin syndrome) is a rare autosomal dominantly inherited disorder that is characterized by multisystem disorder such as basal cell carcinomas, keratocystic odontogenic tumors and skeletal abnormalities. Bilateral and/or unilateral ovarian fibromas have been reported in individuals diagnosed with NBCCS.
METHODS: A 22-year-old female, presented with low back pain, and was found to have bilateral giant adnexal masses on pelvic ultrasonography, which had been suspected to be malignant ovarian tumors. Positron emission tomography/computed tomography showed multiple intracranial calcification and skeletal abnormalities. The left adnexa and right ovarian tumor were resected with laparotomy, and pathology revealed bilateral ovarian fibromas with marked calcification. We recommended the patient to receive genetic testing and dermatological examination. No skin lesion was detected. Germline testing identified pathogenic heterozygous mutation in PTCH1 (Patched1).
CONCLUSIONS: The possibility of NBCCS needs to be considered in patients with ovarian fibromas diagnosed in an early age. Skin lesions are not necessary for the diagnosis of NBCCS. Ovarian fibromas are managed with surgical excision with an attempt at preserving ovarian function. Follow-up regime and counseling on options for future fertility should be offered to patients.

Yolk sac tumor (YST) is a malignant tumor derived from germ cells and usually occurs in the gonads. Extra-gonadal YST is most commonly seen in the vagina of children, but rarely in the cervix, vulva and endometrium. Primary YST of endometrium was extremely rare, standard treatment was still controversial and no guideline was established so far. The aim of the present study was to provide a comprehensive understanding and systematic thought for the management of primary YST of endometrium.
A systematic research of the literature was conducted in Scopus, PubMed database and Cochrane Library, including case reports and case series. We summarized clinical characteristics, treatments and prognosis of all collected cases. We collected data regarding patients, serum AFP level, initial symptoms, surgical information, postoperative chemotherapy and radiotherapy. A new case was also discussed.
We found only 26 cases have been reported previously. We reported a new case of primary endometrial YST in a 27-year-old woman, and in this case, we creatively performed bilaterally ovarian preservation and used DC (docetaxel and carboplatin) regimen of postoperative chemotherapy, we achieved a relatively good prognosis during the follow-up period of 14 months.
Primary YST of endometrium, kind of highly malignant germ cell tumors, was extremely rare, of which initial symptom is usually abnormal vaginal bleeding. Standard treatment was still controversial and no guideline was established so far. Surgery combining with postoperative chemotherapy was considered effective for treatment of primary endometrial YST. Decision on whether to preserve ovaries in young patient with early stage needs careful consideration, comprehensive preoperative assessment and full communication. Intraoperative biopsy and strict postoperative follow-up are recommended. However, standard chemotherapy regimen and feasibility of postoperative radiotherapy remains to be discussed.

BACKGROUND: Ovarian small cell carcinoma is a rare, aggressive neoplasm that occurs in young women and has a poor long-term prognosis. Treatment involves surgical resection and chemotherapy. The required radicality of surgery is uncertain, balancing cytoreduction with fertility preservation. Various chemotherapy regimens are utilized due to confusion regarding the neoplasm\'s lineage. Case We describe an adolescent with small cell carcinoma, hypercalcemic type, stage IA. Surgery included left salpingo-oopherectomy, left pelvic/paraaortic lymphadenectomy, omentectomy and peritoneal biopsies. She received four cycles of bleomycin, etoposide and cisplatin, similar to high-risk germ cell cancers. She has received no further therapy and is eleven years from diagnosis without evidence of disease.
CONCLUSIONS: This is the first long-term juvenile survivor managed with both fertility-sparing surgery and BEP (bleomycin, etoposide, cisplatin).

BACKGROUND: Gynandroblastoma is an extremely rare ovarian sex cord tumor with malignant potential.
METHODS: An 18-year-old adolescent experienced intermittent vaginal bleeding. A year later, a right adnexal mass with a heterogeneous imaging appearance was identified. Laparoendoscopic single-site ovarian tumorectomy was performed. A histopathological examination showed gynandroblastoma composed of juvenile granulosa and Sertoli-Leydig cells. Because the tumor was upstaged to stage Ic because of cyst rupture during surgery, three cycles of adjuvant chemotherapy with carboplatin and paclitaxel were added. Three years after surgery, no signs of recurrence have been noted.
CONCLUSIONS: The present findings can help clinicians make an accurate preoperative imaging diagnosis of gynandroblastoma with a juvenile granulosa cell component and plan an adequate treatment strategy for this rare, potentially malignant neoplasm.