Ulisses do Prado Aguiar & João Luiz Vitorino Araujo &

José Carlos Esteves Veiga & Milton Hikaro Toita &

Guilherme Brasileiro de Aguiar

Abstract

Purpose Congenital intracranial tumors are extremely rare and the most common is teratoma. Craniopharyngioma is a rare neonatal tumor with only eight cases reported. The management of this tumor in the neonatal period is still controversial, with the best results obtained when radical resection is performed. We present the case of a patient who received the diagnosis of a suprasellar tumor during the prenatal period and reviewed literature regarding the management. Methods We report a case of neonatal craniopharyngioma treated surgically. Results The routine ultrasound at 29 weeks of gestation showed a suprasellar echogenic image measuring 44 mm in diameter with polyhydramnios and macrocephaly. The patient was born at 38 weeks of gestation and underwent a surgical treatment on its 32nd day of life and the excision of almost 80 % of the lesion was achieved. He developed a subdural hygroma and on the 51st day of life, a subduroperitoneal shunt was installed to treat it. This patient died at 8 months of life due to complications of a shunt infection. Conclusion The present case is the ninth diagnosed during the prenatal period and the literature is controversial on the management of this rare tumor. The complete excision of the lesion using the microsurgical technique is the gold standard treatment for these patients; however, there are many factors that limit this approach in neonates. Usually, the resected tumors were smaller than 6 cm. Tumors larger than 8 cm have a worse prognosis, with a short survival time.

Introduction

Congenital intracranial tumors are extremely rare, accounting for 0.5 to 1.9 % of all brain tumors in children [1–7]. Teratomas are the most frequently diagnosed lesions in the neonatal period [1–4, 7]. Craniopharyngiomas account for approximately 6–9 % of all intracranial tumors diagnosed in childhood, constituting the most common non-glial tumors [2, 8]. However, there have been only eight cases diagnosed in the prenatal period reported in the literature [2, 9]. The management of this tumor in the neonatal period is still controversial, with the best results obtained when radical resection is performed [2–4]. Craniopharyngiomas are benign lesions for which the first-line treatment is surgical resection [2, 4, 6, 8]. As they are located in a complex anatomical area, they are difficult to resect, being associated with high morbidity and mortality [2, 8]. We present the case of a patient who received the diagnosis of a suprasellar tumor during the prenatal period and underwent neurosurgical treatment. The histopathological analysis was compatible with adamantinomatous craniopharyngioma. This study aims to report a rare case of craniopharyngioma diagnosed in the prenatal period and perform a review of the literature, discussing aspects related to the management.

Case report

The patient was a male child born by Caesarean section, to a primiparous mother, with no evidence of disease or comorbidities in the prenatal period. The ultrasonography (USG) at 29 weeks of gestation showed a suprasellar echogenic image measuring 44 mm in diameter, which promoted dilation of the lateral cerebral ventricles (Fig. 1a). Polyhydramnios and macrocephaly were identified throughout the remainder of the prenatal period. The patient was born at 38 weeks of gestation, according to the USG by means of an uneventful Caesarean section. At the initial physical examination, his weight was 3,645 g, the Apgar score was 8 in the first minute and 9 after 5 min, his length was 47 cm, his head circumference was 40.5 cm, with a wide and tense fontanel, and he presented good reactions and tonus, micropenis, and topical testes. The examination of the umbilical cord disclosed a single umbilical artery and vein. The pupils were isochoric and photoreactive. A head MRI was performed on the third day of life and showed sellar and suprasellar lesion with isointense signal on T1 and hyperintense on T2, with homogeneous contrast uptake, measuring about 68×66×62 mm, extending into the third ventricle and causing dilation of the lateral ventricles (Fig. 1b-f). On the 14th day of life, the patient underwent a parietal craniotomy using the transcortical transventricular approach, planning the radical resection of the tumor, obtaining biopsy material for diagnosis, and treating the obstructive hydrocephalus.

During surgery, the tumor was observed to be quite vascularized, resulting in bleeding during handling. Due to the large size of the tumor, the patient presented hemodynamic instability, which forced us to leave the radical resection to be performed in a subsequent surgery. The patient developed postoperative diabetes insipidus requiring the use of desmopressin. Histopathological examination showed that it was an adamantinomatous craniopharyngioma (Fig. 2). On the 32nd day of life, the patient underwent a new surgical procedure through the same access used before and the excision of almost 80 % of the lesion was performed (Fig. 3). He subsequently developed a subdural hygroma and on the 51st day of life, a subduroperitoneal shunt was installed to treat it. The neonate was discharged after his 63rd day of life with desmopressin. He died at 8 months of life due to complications of a shunt infection.

Discussion

Neonatal tumors are rare, representing approximately 0.5 to 1.9 % of all brain tumors diagnosed in childhood [1–7]. The most common tumors of the central nervous system diagnosed in the prenatal period are teratomas [1, 2, 7]. There are approximately 22 reported cases of prenatal and neonatal craniopharyngiomas, with only eight of them having been diagnosed during the prenatal period [2–6, 9]. The first craniopharyngioma present at birth was reported in the literature in 1952 by Iyer [5]. Craniopharyngiomas are benign neoplasms of epithelial origin, arising from epithelial vestiges along the migration of the adenohypophysis, known as the craniopharyngeal duct or Rathke’s pouch [2, 4, 8]. This structure extends from the pharynx to the sella turcica [8]. Most craniopharyngiomas are located in the sellar and suprasellar region, but they

can appear along the entire embryological craniopharyngeal trajectory [8]. The effectiveness of noninvasive diagnostic methods such as ultrasound and magnetic resonance imaging (MRI), associated with improvement in the quality of prenatal care, has allowed for the early diagnosis of neonatal tumors [2, 9–11]. The routine use of prenatal ultrasound is responsible for most diagnoses of antenatal tumors reported in the literature [1–3, 11, 12]. The presence of calcifications within suprasellar tumors can lead to the identification of this neoplasia, albeit in the absence of specific findings [1–4, 7, 9–12]. However, due to the rarity of reported cases, this diagnosis is rarely recalled. The MRI after birth can provide more information about the extension and exact location of the tumor, assisting in therapeutic decisionmaking. Although it is histologically benign, the sellar location in close proximity to the optic chiasm, hypothalamus, pituitary gland, the carotid arteries, and the ventricular system makes the management of this neoplasia difficult to achieve [2, 8]. This lesion progressively grows when untreated, leading to serious disorders associated with the compression of adjacent structures [2, 8]. The complete excision of the lesion using the microsurgical technique is the gold standard treatment for these patients; however, there are many factors that limit this approach in neonates [2–4, 8]. Freeman et al. [6], in 1988, observed that the radical excision of these lesions was not possible due to the complex parasellar anatomy and the hemodynamic lability of the newborn, while Hurst et al. [13] recommended early surgery in order to prevent complications associated with tumor growth. As they are largesized tumors, there have been reports in the literature of surgical excisions in different periods [14, 15]. The surgical results reported in the literature can be seen in Table 1. The evaluation of the hormonal profile associated with electrolyte measurement and investigation of possible visual deficits, according to the possibility of each age range, should be performed preoperatively, to evaluate the previous impairment caused by the tumor [2, 3, 8, 11]. There have been four cases reported in the literature in which total excision of the tumor was possible, with the best survival results found [2]. Arai et al. [4] report that a smaller tumor size is a common characteristic to all neonatal craniopharyngiomas possible of being resected. Usually, the resected tumors were smaller than 6 cm [4]. Tumors larger than 8 cm have a worse prognosis, with a short survival time [4]. In spite of the good results obtained with radical resection, postoperative morbidity is significant, reports of panhypopituitarism decreased visual acuity, and future psychological disorders are commonly reported [2–4]. The recurrence of neonatal craniopharyngiomas, as well as others, according to Müller-Scholden et al. [3], is best treated by surgical reoperation, as radiation therapy is extremely harmful for children younger than 3 years of age. The prognosis for children diagnosed with congenital brain tumors is generally unfavorable [2–4, 6, 9]. Craniopharyngiomas remain a challenge for surgeons dealing with the treatment of these lesions at any age [8]. The limitations imposed by the physiology of the newborn, associated with the large size that these tumors can attain, make neon

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