An Extra-large Ovarian Mucinous Cystadenoma in a Premenarchal Girl and a Review of the Literature

doi:10.1016/j.jpag.2012.04.007

Journal of Pediatric and Adolescent Gynecology

Volume 26, Issue 1, February 2013, Pages 22-26

https://doi.org/10.1016/j.jpag.2012.04.007 Get rights and content

Abstract

Objective

Epithelial ovarian neoplasms are extremely uncommon in children. Ovarian mucinous cystadenoma is benign and an extremely rare presentation in the premenarchal period. We present a case of giant mucinous cystadenoma of the left ovary in a 13-year-old and a review of the supporting literature.

Case Report

The patient was admitted with a history of increasing abdominal distension and pain for approximately 3 months and a history of an ovarian mass for 3 years. An adnexal mass measuring 40 × 30 × 20 cm was detected by abdominal ultrasonography and computed tomography. The tumor markers CEA, CA 19-9, and CA-125 were elevated, although α-fetoprotein and human chorionic antigen levels were within the normal range. The patient underwent surgery. The smooth-surfaced mass filled the abdomen. A frozen section biopsy was performed, and the results indicated a benign mucinous cystadenoma. A unilateral oophorectomy with tumor removal was performed.

Conclusion

The ovarian mass was revealed by abdominal distension, and a diagnosis was established by frozen biopsy. Complete and careful surgical resection provides satisfactory results; however, careful follow-up is required.

Introduction

Although ovarian tumors are most commonly observed in adults, they relatively rarely occur in children.¹ Ovarian tumors in childhood account for 1%-1.6% of all childhood tumors and 6% of ovarian tumors in all ages.2, 3, 4, 5 Ovarian tumors frequently occur in the second decade of childhood, and most originate from germ cells.1, 2, 3, 4, 5 In premenarchal girls, ovarian masses are considered rare, and as a result, operations for these lesions are uncommon. The etiologies of ovarian masses in premenarchal girls include functional cysts and torsion, and they range from non-neoplastic up to highly aggressive, malignant tumors.⁶ The majority of ovarian masses encountered in the premenarchal or childhood stages are non-neoplastic lesions such as benign functional cysts. The clinical signs and symptoms of ovarian masses are usually nonspecific; therefore, making the correct diagnosis preoperatively is occasionally a challenge, and early management may be necessary to save the patient's life and preserve fertility.4, 6

An evaluation of the literature on epithelial ovarian tumors shows that these tumors make up 10%-28% of ovarian tumors in children and adolescents.1, 2, 3, 4 Epithelial tumors account for 8%-10% of all ovarian tumors and are histologically classified as mucinous or serous. The most common benign epithelial ovarian tumor is cystadenoma, of which 75% are serous and 25% are mucinous cystadenoma (MCA).4, 6 The present study evaluates the case of an large tumor of left ovarian origin in the premenarchal period, which was diagnosed as MCA during surgery in light of the literature.

Section snippets

Case Report

A 13-year-old female patient had a 3-year history of an ovarian mass without regular follow-up. The patient presented at the emergency room complaining of abdominal swelling, which had been increasing over the past 3 months. Her physical examination showed abdominal distension and a hard mass extending from the pelvis to the xyphoid process and filling the abdomen with palpable borders (Fig. 1, Fig. 2). Abdominal ultrasound revealed a giant cystic lesion with a thin septum and dense content

Discussion

The literature was searched using the MEDLINE database through PubMed. Titles and abstracts were searched for a combination of the terms mucinous cystadenoma, ovarian, premenarche, and children. No data limits were used. A total of 15 papers published in the English-language literature reporting premenarchal cases, including our case, were evaluated (Table 1).

Hernandez et al reported the first case of mucinous carcinoma in a 10-year-old girl in 1982.⁷ Morris et al next reported 6 cases of

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Cited by (29)

Large ovarian tumors in adolescents, a systematic review of reported cases, diagnostic findings and surgical management
2021, Taiwanese Journal of Obstetrics and Gynecology
Citation Excerpt :
The remaining 650 articles were evaluated as explained, and 104 were selected for closer inspection. In the end, 47 articles were included in this review summing 57 cases, to which we added 1 case from our institution [3,7–46]. The flowchart in Fig. 1 outlines this process.
The purpose of this study was to analyze the published cases regarding large ovarian masses in adolescents, in order to find useful clinical implications for the diagnosis and management of the condition. The methodology employed a systematic review of reported cases of large/giant ovarian tumor in adolescence. The main objective was to assess the imagery findings, histology of the tumor, and the type of surgery performed. Our study included 58 patients with the tumor diameters ranged between 11 and 42 cm, with a median value of 22.75 cm and a mean diameter of 24.66 ± 8.50 cm. The lesions were benign in 47 cases (81%), borderline in 2 cases (3.4%), and malign in 9 cases (15.6%). We found no statistically significant difference (p > 0.05) between the size of the tumors and the ovarian markers levels. At the same time, a statistically significant difference was identified (p=<0,001) between elevated levels of the serum markers and the malignant tumor type (OR = 12.45; CI₉₅ = 2.55–94.77). The main types of surgery performed were cystectomy (35 cases), oophorectomy (4 cases), salpingo-oophorectomy (18 cases). Open laparotomy was performed in 46 cases on tumors with a median diameter of 25 ± 8.8 cm, while laparoscopy was done in 12 cases on tumors with a median diameter of 23.7 ± 7.6 cm. For optimal surgical management, it is advisable to assess first the imagery findings and tumor markers. Then, the laparoscopic approach should be considered as a feasible option, especially in cases where there are no predictive factors for a malign disease.
Giant ovarian mucinous tumour. Clinical case
2021, Clinica e Investigacion en Ginecologia y Obstetricia
El cistoadenoma mucinoso de ovario representa el 15% de las lesiones neoplásicas benignas del ovario, diagnosticándose por lo general entre los 20 y los 60 años de edad. Frecuentemente alcanza un gran tamaño ocasionando clínica compresiva. Presentamos a continuación el caso de una paciente joven que comenzó con clínica digestiva y distensión abdominal, siendo intervenida de un cistoadenoma ovárico gigante.
Mucinous ovarian cystadenoma accounts for 15% of benign neoplastic lesions of the ovary, and is usually diagnosed between the ages of 20 and 60. It often reaches a large size causing a compressive clinic. The case is presented of a young patient, whose first clinical manifestions were gastrointestinal with abdominal distension. A giant ovarian cystadenoma was found on being intervened.
A Giant Ovarian Cyst in an Adolescent
2018, Journal of Pediatrics
Postoperative Outcomes among Pediatric and Adolescent Patients Undergoing Minilaparotomy vs Laparoscopy in the Management of Adnexal Lesions
2017, Journal of Pediatric and Adolescent Gynecology
Citation Excerpt :
Although previous studies have shown the safety and efficiency of minilaparotomy in the management of adnexal masses, there are no data in the literature that compared laparoscopy with minilaparotomy in the pediatric and adolescent population.2,3,5 Additionally, a review of the literature in the pediatric population has shown that benign adnexal masses of 15 cm or greater continue to be managed with a larger laparotomy incision, which are associated with greater morbidity.6,7 In cases when laparoscopy is limited, many surgeons proceed with traditional larger laparotomy incisions, although minilaparotomy could offer a less invasive surgical approach.2
To compare institutional experience in postoperative recovery in children and adolescents who undergo laparoscopy vs minilaparotomy in the management of benign adnexal cystic lesions.
Retrospective cohort study.
Tertiary care children's hospital.
Patients ages 6-21 years who underwent surgical management for benign adnexal lesions.
Comparison of surgery times, size of lesions, pain scores, and patient length of stay after minilaparotomy vs laparoscopy.
Forty-four patients were identified. Of those, 59% (n = 26) had a laparoscopic procedure and 41% (n = 18) underwent minilaparotomy. Patients who underwent minilaparotomy were more likely to have a larger adnexal mass than those in the laparoscopy group with a median size of 15.5 cm vs 6.0 cm, respectively (P < .001). Postoperative length of stay and recovery were comparable in both groups.
Minilaparotomy offers a minimally invasive option for the management of benign adnexal lesions in the pediatric and adolescent age group with recovery comparable to that of laparoscopy. The minilaparotomy approach should be considered when laparoscopy is limited.
Laparoscopic Management of a Giant Ovarian Cyst in a Premenarchal Girl
2017, Journal of Minimally Invasive Gynecology
Mucinous Cystadenoma in Children and Adolescents
2017, Journal of Pediatric and Adolescent Gynecology
Citation Excerpt :
Reports suggest that they are usually unilateral but can grow large in size. The size of the tumor, although not indicative of malignancy, combined with the concern for recurrence, often leads the surgeon to perform an oophorectomy.1,2,19,28 Data from our case series review suggest that benign MCAs as large as 26 cm in children and adolescents can be managed conservatively with cystectomy and without an associated increased risk of recurrence.
Mucinous cystadenomas (MCAs) are benign epithelial ovarian tumors that occur rarely in children and adolescents. Because children and adolescents typically have their childbearing years ahead of them, conservative therapy is indicated. However, there is concern that ovarian cystectomy might be associated with significant recurrence risk in patients with MCA. Furthermore, guanine nucleotide binding protein, alpha stimulating (GNAS) gene mutations are associated with McCune-Albright syndrome, which is associated with cystic ovaries. We sought to evaluate the outcomes of children and adolescents with MCA treated conservatively. A subset of patients underwent GNAS gene testing.
After institutional board review approval, the pathology database of a large urban children's hospital was queried to identify adolescents with MCA between the years 2008 and 2014. Fourteen patients, aged 8-18 years (median, 14), were identified. A buccal swab for genetic testing was obtained from a subset of consenting patients.
MCA recurrence; ovarian return to normal size; GNAS gene variants.
Two patients underwent oophorectomies, and the remaining 12 underwent cystectomies. Follow-up ultrasound examination revealed slow return of ovary to normal size. Of the 10 patients with available follow-up data, there were no recurrences at a median of 225 days from surgery. Four patients consented to a buccal swab for genetic testing, and the GNAS gene was noted to have rare variants in 2 patients.
This series supports the use of ovary-sparing surgery in the treatment of MCA. Further research exploring possible genetic variants such as the GNAS gene in children and adolescents diagnosed with MCA is warranted.

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Original StudyAn Extra-large Ovarian Mucinous Cystadenoma in a Premenarchal Girl and a Review of the Literature

Abstract

Objective

Case Report

Conclusion

Introduction

Section snippets

Case Report

Discussion

J Pediatr Surg

J Pediatr Adolesc Gynecol

J Pediatr Adolesc Gynecol

Gynecol Oncol

J Pediatr Adolesc Gynecol

J Pediatr Adolesc Gynecol

Int J Gynaecol Obstet

Am J Obstet Gynecol

Mucinous cystadenoma of the ovary in perimenarchal girls

Pediatr Surg Int

Original Study
An Extra-large Ovarian Mucinous Cystadenoma in a Premenarchal Girl and a Review of the Literature