Keywords
neonatal ovarian cyst - serous cystadenoma - torsion
Introduction
The occurrence of ovarian cyst in a neonate is rare, and the reported incidence is 1 in 2,625 pregnancies.[1]
[2] The first case was reported in 1889 as an autopsy finding in a premature stillborn fetus.[1] The most frequent and severe complication is torsion, followed by hemorrhage, autoamputation, rupture, hydrops, dystocia, and pressure on adjacent organs.[2] The recommended treatment for symptomatic cases is surgical excision. Histologically, the neonatal ovarian cyst (NOC) has flattened to the single layer of cuboidal lining. Nevertheless, nearly 20% of cases turn out as serous cystadenoma, a benign neoplasm.[2]
[3]
[4]
[5] Awareness of serous cystadenoma in the neonate is essential for the pathologists to diagnose and the clinicians to treat it.
Therefore, this study aims to analyze retrospectively the specimens received in this part of the country for a better patient outcome.
Materials and Methods
All reported cases of NOCs received were retrieved from the archives of the department of pathology and analyzed for clinical presentation and pathological diagnosis over 4 years from January 2016 to December 2020. The blocks and slides were retrieved, sections cut, stained with hematoxylin and eosin, and analyzed.
Results
We retrieved 10 cases from the archives, and the results are shown in [Tables 1] and [2].
Table 1
The clinical and ultrasound features of 10 NOC cases
|
Case no.
|
Age at USG diagnosis (wk)
|
Side
|
Birth weight
(kg)
|
USG diagnosis
|
Material risk factor or comorbidities
|
Clinically mass palpable
|
Mode of delivery
|
Term of delivery
|
Associated
other
congenital anomalies
|
Consanguinity
|
|
1
|
32
|
Right
|
4
|
Duplication/Ovarian cyst
|
Nil
|
No
|
Emergency LSCS due to fetal distress
|
Term
|
Nil
|
Nonconsanguineous
|
|
2
|
30
|
Left
|
3.5
|
Ovarian cyst
|
Nil
|
No
|
NVD
|
Term
|
Nil
|
Nonconsanguineous
|
|
3
|
33
|
Left
|
2.9
|
Mesenteric/duplication/ovarian cyst
|
Nil
|
Yes 6 × 6 cm
|
NVD
|
Term
|
Nil
|
Consanguineous
|
|
4
|
35
|
Left
|
2.6
|
Mesenteric/duplication/ovarian cyst
|
Nil
|
Yes 5 × 3 cm
|
NVD
|
Term
|
Nil
|
Nonconsanguineous
|
|
5
|
32
|
Left
|
3.9
|
Ovarian dermoid
|
Hypothyroidism
|
Yes 5 × 5 cm
|
NVD
|
Term
|
Nil
|
Nonconsanguineous
|
|
6
|
34
|
Right
|
2.8
|
Enteric duplication cyst
|
Nil
|
No
|
NVD
|
Term
|
Nil
|
Nonconsanguineous
|
|
7
|
38
|
Left
|
2.6
|
Complex ovarian cyst/enteric duplication cyst
|
Nil
|
No
|
NVD
|
Term
|
Nil
|
Nonconsanguineous
|
|
8
|
32
|
Right
|
2.9
|
Duplication cyst
|
Rh incompatibility
|
No
|
NVD
|
Term
|
Nil
|
Consanguineous
|
|
9
|
36
|
Bilateral
|
2.6
|
Ovarian cyst
|
Nil
|
Yes 5 × 5 cm
|
LSCS
|
Term
|
Nil
|
Nonconsanguineous
|
|
10
|
32
|
Right
|
3.3
|
Ovarian cyst
|
Nil
|
Yes 4 × 3 cm
|
NVD
|
Term
|
Nil
|
Consanguineous
|
Abbreviations: LSCS, lower segment caesarean section; NOC, neonatal ovarian cyst; NVD, normal vaginal delivery; USG, ultrasonogram.
Table 2
The surgical management and pathological features of 10 NOC cases
|
Case no.
|
Age at cyst excision (d)
|
Gross cyst size (GD in cm)
|
Length of the pedicle (cm)
|
Mode of cyst excision
|
Complication evidenced on clinical and/or pathological examination
|
Type of cyst on microscopy
|
|
1
|
8
|
4
|
0.5
|
Laparoscopic excision after cyst aspiration
|
Torsion and pressure effects
|
Simple ovarian cyst with extensive infarction
|
|
2
|
35
|
4.8
|
0.3
|
Laparoscopic excision after cyst aspiration
|
Torsion
|
Ovarian cyst with no viable epithelial lining and with areas of calcification and infarct
|
|
3
|
5
|
5.5
|
0.4
|
Laparoscopic excision after cyst aspiration
|
Nil
|
Follicular cyst
|
|
4
|
30
|
4
|
0.6
|
Laparoscopic cyst excision
|
Torsion
|
Ovarian cyst with no viable epithelial lining and torsion induced changes
|
|
5
|
33
|
4.5
|
0.8
|
Laparoscopic cyst excision after aspiration
|
Torsion and pressure effects
|
Simple ovarian cyst with features of torsion and extensive calcification
|
|
6
|
10
|
4
|
0.4
|
Laparoscopic cyst excision after aspiration
|
Torsion
|
Simple ovarian cyst with torsion induced changes
|
|
7
|
20
|
5
|
0.5
|
Laparoscopic cyst excision after aspiration
|
Torsion
|
Serous cystadenoma with torsion induced changes
|
|
8
|
30
|
5
|
0.7
|
Laparoscopic cyst excision after aspiration
|
Torsion
|
Simple ovarian cyst with torsion induced changes
|
|
9
|
17
|
4 (Right)
1.8 (Left)
|
0.5
|
Right – cyst excision only
Left – only cyst fluid aspiration
|
Torsion
|
Simple ovarian cyst with torsion induced changes
|
|
10
|
11
|
3.5
|
0.6
|
Laparoscopic excision
|
Torsion and autoamputation
|
Serous cystadenoma with torsion induced changes
|
Abbreviation: NOC, neonatal ovarian cyst.
Cyst excision in the neonates was from 5 to 35 days with a mean age of 20 days. Ninety percent of the excision was for torsion (9/10 cases). All cases were antenatally diagnosed between 32 and 36 weeks of gestation by ultrasonogram. The neonates were diagnosed with intra-abdominal cyst between 30 and 38 weeks of gestational age by antenatal ultrasonography. There was no side predilection for the cyst occurrence, with one neonate having bilateral cysts. The size of the cyst varied from 1.8 to 5.5 cm in the greatest dimension. The most common complication of NOC was torsion (90%), two cases (20%) had pressure effects, and one case (10%) had autoamputation.
Histologically, simple cyst was found in 5 neonates (50%), serous cystadenoma in 2 neonates (20%), with no viable lining in 2 cysts (20%), and 1 case (10%) was of follicular origin ([Table 2], [Figs. 1] and [2]). These cysts most often had hemorrhage, calcification, and necrosis ([Fig. 3]).
Fig. 1 Histological lining of some cases of NOC studied in our case series (A) Photomicrograph of a simple ovarian cyst wall with single layer of flattened to cuboidal benign epithelial lining (H & E stain, 10x). (B) Photomicrograph of a benign serous cystadenoma ovarian cyst wall with single layer of benign columnar epithelial lining with cilia (H&E stain, 40x). (C) Photomicrograph of a simple ovarian cyst wall with denuded lining epithelium (H&E stain, 40x). (D) Photomicrograph of the cyst wall which has fibrocollagenous stroma with few lymphocytes and capillaries (H&E stain, 40x).
Fig. 2 A case of follicular neonatal ovarian cyst. (A) Photomicrograph of a cyst wall with cyst lined by several layers of cells in low power (hematoxylin and eosin [H&E] stain, 40 × ). (B) Cyst wall lining composed of an inner layer of granulosa cells and an outer layer of luteinized theca cells (H&E stain, 10 × ). (C) Theca cells contains eosinophilic to clear cytoplasm and round nuclei with prominent central nucleoli (H&E stain, 10 × ).
Fig. 3 Histological changes seen due to complications in neonatal ovarian cyst. (A and B) Photomicrograph of a cyst wall with hemorrhage and necrosis (hematoxylin and eosin [H&E] stain, 10 × ). (C) Photomicrograph of a cyst wall with focal calcification (H&E stain, 40 × ).
Discussion
NOC is frequently diagnosed in the third trimester by antenatal ultrasonography. The first case was described in 1889 as an autopsy finding in a premature stillborn fetus.[1] In an autopsy study of 233 neonatal deaths and stillborn by deSa,[6] small ovarian cysts were found in 34%. The age at diagnosis by ultrasonography ranged from 24 to 39 weeks of gestation in a study by Monnery-Noché et al.[7] The literature search shows the early age of diagnosis by ultrasonography at 19 weeks[3]
[8] of gestation. The cyst size is variable, and the largest cyst found in the literature measured 20 cm in diameter.[8] The cysts are predominately unilateral, and bilateral cysts are rare, with only a few cases reported.[5]
[8]
[9]
The etiopathogenesis of NOC remains unknown. The stimulus for the cyst formation is attributed to increased placental human chorionic gonadotrophin (HCG)[1]
[3]
[8]
[9]
[10]
[11] in conditions such as maternal diabetes, toxemia of pregnancy, and Rh incompatibility causing hyperstimulation of the normal ovarian follicle to undergo cystic change. In our study, one mother had Rh incompatibility, and one mother had hypothyroidism. The presence of fetal prematurity, congenital adrenal hyperplasia, and hypothyroidism[1]
[9]
[10] are also found to cause NOC as they are associated with greater sensitivity to HCG. Literature survey revealed NOC also occurs in healthy mothers often.[11]
Though serous cystadenomas constitute approximately 40% of all ovarian tumors occurring in the reproductive age group,[12] it is infrequent in the fetus. From the literature search, only three studies[3]
[4]
[5] have reported serous cystadenoma among the NOC. The etiological reasons for the occurrence of serous cystadenoma in neonates are not known. Our study found the incidence of serous cystadenoma to be 20%, similar to other studies. The pathologist should also consider the diagnosis of the surface epithelial tumor as a differential diagnosis of NOC.
The most common dreaded complication of these NOCs is torsion (50–78%)[2]
[9]
[13]
[14] and are found to occur most frequently during fetal life than in the postnatal period,[9] which is also evidenced in this case series. Certain studies[9] points out the risk of torsion, which depends on the cyst's size (frequently seen in cysts of size > 4 cm) and the length of the pedicle.[3] At the same time, there are studies[7]
[15] that state that the risk of developing torsion is not dependent on the size of the cyst or age of the neonate. Among the 10 cases presented here, 9 cases had features of torsion. Our study also infers that the torsion of the ovarian cysts in neonates is not dependent on the cyst size.
The other complications[2]
[9] of NOC are rupture, hemorrhage, perforation, intestine and urinary tract obstruction, pulmonary hypoplasia, dystocia, autoamputation,[10] adhesion, calcification of cyst wall,[3] and sterile necrosis of the ovary. We had two neonates with intestinal and urinary obstruction symptoms and an intraoperative observation of an autoamputation of an ovarian cyst. There were 9 out of 10 cases with microscopic evidence of calcification as noted in other studies.[1]
[4]
[14]
Controversy prevails regarding the management of NOC. Since most of them regress in the postnatal period, conservative management is suggested even for cysts with sizes of 5 cm.[3]
[8] Intrauterine ovarian cyst aspiration[3]
[16] can be performed, but it has complications such as rupture, hemorrhage, and infection. Laparoscopy excises cyst with complications or large size. From our study, we infer that 20% of cases are neoplastic, and therefore removal of NOC should be considered at the earliest with a follow-up even though there are no complications.
As surgery is the primary treatment for ovarian tumors, ovarian salvage with fertility preservation in the unilateral cyst is important since single ovary presence renders them a short reproductive life span.[7] Minimally invasive surgical technique is essential for the cysts occurring in a neonate. Follow-up using serial ultrasonography[14] is vital to look for regression or development of complications such as torsion.
A study by Gaspari et al[17] points out the occurrence of NOC as an early manifestation of McCune–Albright syndrome, which enables early diagnosis in such cases.
In summary, NOC is commonly diagnosed nowadays due to the increased usage of antenatal ultrasonography. The etiology is variable. The common complication is torsion. Histologically, the most common is a simple cyst with 20% incidence of serous ovarian cystadenoma. Increased awareness of the occurrence of serous cystadenoma is vital for pathologists to be able to consider it as a differential diagnosis in NOC. The management of the NOC is controversial, mainly by laparoscopy excision.
Limitation
The limitation of this study is further clinical follow-up of the neonates could not be done since they have not come for further follow-up at the center.
