Table 2.
Clinical and surgical characteristics of pediatric patients with Gorlin syndrome undergoing surgical management of fibromas (N = 4).
| Case | Age (y) | Past medical history | Clinical symptoms | Imaging findings | Surgery (y) | Intraoperative findings | Pathology |
Recurrence | ||
|---|---|---|---|---|---|---|---|---|---|---|
| Largest size (cm) | No. of lesions | |||||||||
| 1 | a | 5 | None | Pain, concern for torsion | Lobulated 4.3 cm calcified midline mass with heterogenous vascular soft tissue | Laparotomy, RSO, appendectomy (2005) | Acute appendicitis; calcified right ovarian mass with necrotic adnexal torsion | 4.3 | 1 | Yes, see 1b |
| b | 17 | GS diagnosed after ovarian fibroma | Asymptomatic | Left 5.3 cm complex cystic-solid ovarian mass with scattered calcifications | Laparotomy, left ovarian cystectomy (2017) | Hard, 6 cm left ovarian mass | 4.5 | 9 | No | |
| 2 | 14 | Known GS; BCC x2, odontogenic cyst | Asymptomatic | Right 4.7 cm hypoechoic vascular paraovarian mass | Laparotomy, right ovarian cystectomy (2014) | Exophytic 6cm solid right ovarian mass | 6 | 1 | No | |
| 3 | 13 | Known GS; BCC, maxillofacial fibromas, medulloblastoma | Pain, concern for torsion | Multiple masses, largest 9.3 cm with hemorrhage and necrosis | Laparoscopic RSO, left ovarian cystectomy (2019) | Multilobulated 15 cm ovary; 2 cm left ovarian mass | 9 | 3 | Yes, multiple (largest 4 cm) | |
| 4 | 18 | Known GS; BCC x2, embryonal rhabdomyosarcoma | Asymptomatic | Right 7 cm paraovarian mass with enhancing fibrous tissue; left ovary with multiple <1 cm hyperechogenic lesions | Laparotomy, RSO (2022) | Solid, irregular right ovarian mass with no discrete borders; normal left ovary | 7.6 | 3 | No | |
Note: BCC = basal cell carcinoma; GS = Gorlin syndrome; RSO = right salpingo-oophorectomy.