Brain tumors in the early neonatal life are mainly supratentorial in position 13. Congenital brain tumors, described by Arnstein et al, are those tumors that produce symptoms in the first 2 months of life 4.
Teratomas are the most common congenital intracranial tumors 56.
Congenital craniopharyngiomas are less commonly diagnosed , and there are few reported cases of these neoplasms 710.
Only 2 previous in utero detection of a craniopharyngioma has been reported11.one of them was diagnosed by Marc J. Lacrampe, and Philippe Jeanty .
In a large study of midline supratentorial neoplasms from the University of Sao Paulo, of 1632 cases of intracranial neoplasms, 592 cases (36.2%) occurred in patients between 0 to 20 years. Of these, 3.5% were craniopharyngiomas, 1.35% hypophyseal neoplasms, and 6% pineal neoplasms28. Only 30% of craniopharyngiomas occur in children less than 16 years old24.
Pathology
Craniopharyngiomas are histologically benign tumors composed of bands of stratified squamous epithelium separated by connective tissue14 or an adamantinous or a squamous papillary structure.
In a around 100 craniopharyngiomas the following histopathology types were found. “The frequently solid (50%), always uncalcified squamous papillary tumor type was present in one third of the adult patients ( more than or around twenty years) but did not occur in children.
It was associated with a good functional postoperative outcome (84.6%). There were no cases of tumor recurrence in the squamous papillary group.
However, in the group with the adamantinous type of craniopharyngioma, the recurrence rate was 13% in adult patients and 9% in children. When compared to the adult adamantinous cases, the incidence of visual deficits was lower in the squamous papillary group (75% vs. 84%) but the incidence of endocrine abnormalities was higher (75% vs. 52%).
Thus, the preoperative, operative, and postoperative features of the two types of craniopharyngioma were found to be completely different in adults and children”27. They are usually suprasellar in position, but some infrasellar cases have been reported15. Because of their location, these tumors may compress the optic chiasm and optic tracts or cause hypothalamic or pituitary dysfunction16 and hydrocephalus (from obstruction of the third ventricular cerebrospinal fluid outflow).
Symptomatology
Signs and symptoms commonly produced by these tumors in older children include: headache, vomiting, visual loss and papilledema, endocrine disorders including short stature, obesity, hypogonadism, and diabetes insipidus17.
Diagnosis
The prenatal ultrasonographic features include a large, echogenic midline intracranial mass with calcification.
Hydrocephalus is usually
11. Polyhydramnios was reported in one of these cases and both hydrocephalus and polyhydramnios were seen in our case . So, polyhydramnios and hydrocephalus have been variable findings in other cases of prenatally diagnosed intracranial masses
2,5.The CT appearance is that of a heterogenous suprasellar mass, often with nonuniform enhancement, and calcification is seen in 80% of cases
18. The large size of these neonatal tumors is also a usual CT feature
7. Low density cystic areas are described in about 85% of cases, and these are frequently multilocular.The MRI findings were reported , primarily in older patients. On T
1weighted images the signal characteristics arenot the same , based on the amount of cholesterol, keratin, and methemoglobin in the lesion, and range from CSF to fatlike intensity
19. On T
2 weighted images, the masses are typically heterogeneous with high signal intensity. MRI, with the advantages of multiplanar imaging and high resolution, is the method of choice for assessing the degree of these tumors and the degree of vascularity
20.According to S. Sartoretti-Schefer
et al.,
MRIs can show craniopharyngiomas as a hypointense suprasellar tumor with peripherally enhancing cystic areas and an inhomogeneously enhancing solid tumor part.
(32
).
Associated anomalies
There have been no anomalies consistently reported in association with congenital craniopharyngiomas. In two cases lowset ears have been noted; polydactyly, lung hypoplasia 11, centronuclear (myotubular) myopathy25, or Moyamoya disease26 have documented . There is a mild female predisposition in the diagnosed cases, with male to female ratio of 4:7.
Differential diagnosis
The primary differential diagnostis of neonatal craniopharyngioma is a teratoma.This tumor occurs with greater frequency, is often suprasellar, large and contains calcification.
The differential diagnosis between craniopharyngioma and teratoma in neonates is very hard by US, CT or MRI and biopsy is indicated to confirm the diagnosis.
Other neonatal intracranial tumors represent astrocytomas, which may be suprasellar in site and inhomogeneous in echopattern 1 and optic chiasm and hypothalamic glioma. Primitive neuroectodermal tumors and lipoma of the corpus callosum should also be put into consideration.
Craniopharyngiomas may arise from many sites along the craniopharyngeal canal, but most of craniopharyngiomas are located in the sellar/parasellar location. The majority (94–95%) has a suprasellar component (purely suprasellar, 20–41%; both supra and intrasellar, 53–75%) (40, 73, 74), while the purely intrasellar tumors resemble the least common variety (5–6%) (73, 74). Sometimes , a suprasellar tumor may extend to the anterior (9%), middle (8%), or posterior (12%) fossa (34 ).
Other less common sites include the nasopharynx (35), the paranasal area (36), the sphenoid bone (37), the ethmoid sinus (38), the intrachiasmatic area (39), the temporal lobe (40), the pineal gland (41), the posterior cranial fossa (42), the cerebellopontine angle (43), the midportion of the midbrain (44), or completely within the third ventricle (45).
As regard consistency, the adamantinomatous type is predominantly cystic in 59% of the cases, mixed in 30%, and predominantly solid in 11% (45).
The rates for the papillary range is predominantly cystic between 12 and 27%, mixed between 27 and 53%, and predominantly solid between 35 and47%, respectively (48, 49)
Prognosis
Although these tumors are benign histologically, and can be cured if completely removed, the prognosis for neonatal craniopharyngioma is very poor. Only one of 10 previously reported cases survived beyond one year of age7. The usual massive size and critical location of these tumors makes complete surgically removal very difficult21 and accounts for this poor prognosis.
Total excision gives the best outcome but cannot always be done. When partial excision is done it is usually complemented by radiotherapy in older babies 22.
In lesions discovered during childhood and treated by surgery and radiation therapy, 5 and 10 year survival rates of 90% and 80% had been reported 23 but with a high morbidity (visual and endocrine deficits).
Management
Because of the poor prognosis, termination of pregnancy can be offered if the diagnosis is suspected early . The difficulty of making a differential diagnosis with teratoma is of little concern, as both tumors have very bad outcome . Cesarean section is not indicated even when the fetal head is large.
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