Growing Teratoma Syndrome Secondary to Ovarian Giant Immature Teratoma in an Adolescent Girl

Abstract

Growing teratoma syndrome (GTS) is a rare clinical entity first described by Logothetis et al in 1982. Although it is unusual for GTS to be located in the ovary, this report is of a case of an adolescent girl who underwent a complete surgical resection of the mass. Histopathology confirmed only an immature teratoma had originated from the ovary and so she received adjuvant chemotherapy with blemycin, etopside, and cisplatin over 4 cycles. Results from an abdominal enhanced CT (computed tomography) 9 years later revealed a giant mass had compressed adjacent tissues and organs. Laparotomy was performed and a postoperative histopathology showed the presence of a mature teratoma, and so the diagnosis of ovarian GTS was made. One hundred one cases of ovarian GTS from English literature published between 1977 and 2015 were collected and respectively analyzed in large samples for the first time.The median age of diagnosis with primary immature teratoma was 22 years (range 4–48 years, n = 56). GTS originating from the right ovary accounted for 57% (27/47, n = 47) whereas the left contained 43% (20/47, n = 47). Median primary tumor size was 18.7 cm (range 6–45 cm, n = 28) and median subsequent tumor size was 8.6 cm (range 1–25 cm, n = 25). From the primary treatment to the diagnosis of ovarian GTS, median tumor growth speed was 0.94 cm/month (range 0.3–4.3 cm/month, n = 21). Median time interval was 26.6 months (range 1–264 months, n = 41). According to these findings, 5 patients did have a pregnancy during the time interval between primary disease and GTS, making our patient the first case of having a pregnancy following the diagnosis of ovarian GTS. Because of its high recurrence and insensitiveness to chemotherapy, complete surgical resection is the preferred treatment and fertility-sparing surgery should be considered for women of child-bearing age.Anyhow GTS of the ovary has an excellent prognosis. Patients with GTS had no evidence of recurrence or were found to be disease free during a 40.3-month (range 1–216 months, n = 48) median follow-up. Moreover, regular follow-ups with imaging and serum tumor markers are important and must not be neglected.

Recurrence of immature ovarian teratoma as malignant follicular carcinoma with liver and peritoneal metastasis 22 years after completion of initial treatment

“…Despite usually being seen during chemotherapy, or shortly after, GTS has been reported up to 9 years following completion of treatment for NSGCT 5. On imaging, GTS tumours demonstrate cystic components with necrosis, although tumour growth is the only consistent feature of GTS.…”

“…With adequate treatment, GTS have a good prognosis with 5-year survival of 89% achieved 5. Completeness of surgical resection is important due to a high recurrence rate when resection is incomplete (72%–83%) compared with 0%–4% in those who undergo complete resection 9.…”

Pediatric Ovarian Growing Teratoma Syndrome

“…Most of the patients had abdominal symptoms, such as abdominal pain and distension when they first sought evaluation. The median age for the diagnosis of primary immature teratoma was 22 years (range 4–48 years, n = 56) [15]. Many cases of GTS metastasis spread in the peritoneal cavity and tend to occur in the pelvis, peritoneum, or retroperitoneum but have been located in other places such as the liver, pineal gland, and mediastinal/cervical lymph nodes [7, 14, 17, 18].…”

Recurrent ovarian immature teratoma in a 12-year-old girl: Implications for management

“…AFP [28][29][30][31]. GTS has been reported to occur in 12% of ovarian GCTs, most commonly in the retroperitoneum [32][33][34]. GTS can occur in patients with IT or with mixed malignant GCTs who receive chemotherapy.…”

“…The first and most plausible explanation is that chemotherapy transforms and differentiates more 'malignant' GCT cells into larger and more 'benign' teratomatous cells. The second is that the chemotherapy can only destroy malignant cells, and so it leaves chemoresistant teratoma behind [34], although this explanation does not necessarily fully explain why such lesions become larger. It is important to note that even if a patient is not exposed to chemotherapy, IT has the potential to convert into mature disease if post-surgical recurrence occurs [10].…”