Mahmoud Alalfy,M.S., Omaima Idris,M.D, Ibrahem magdy,M.D., walaa Hussen,M.Sc, Hassan Gaafar,M.D

Mahmoud Alalfy,M.S.c *, ,Omaima Idris,M.D**, Ibrahem magdy,M.D **** , walaa Hussen,M.Sc ****  ,  Hassan Gaafar,M.D ***
*Assistant Researcher, National Research Centre , Egypt and The senior fellow of Cairo fetal Medicine unit,Egypt
**Head of the cairo fetal medicine unit , Egypt
***Assistant professor, cairofetal medicine unit ,Egypt
****Senior fellow,Cairo fetal medicine unit ,Egypt


Case report

A 24-year-old primigravida woman, was referred for routine  second trimester scan. 
She had previously had a normal sonogram at 10 weeks.
A follow up exam at 18 weeks revealed a large head.

We found a large BPD and HC for GA, due to an echogenic intracranial mass with some areas of  cystic degeneration  with moderate vascularity in its periphery  
The mass was located in the midline, inferior to the corpus callosum, and superior to the sella turcica.
On 3D ultrasound evaluation the mass was found to be starting from below the level of corpus callosum and lateral ventricles  and extending downwards through the skull and continue in the anterior and middle cranial fossa then extending retroorbital, ethmoid sinus then paranasal and retronasal filling the nasopharynx and then in the oral cavity posterior to the tongue filling the oropharynx.  
The mouth was opened and tongue was seen protruded during our examination.
Ventriculomegally was evident  with dilated both lateral ventricles and third ventricle with frontal bossing  and also polyhydramnios  was noticed.
The diagnosis of fetal craniopharyngioma was made, with persistent craniopharyngeal canal .


Fetal  MRI was offered to the parents  and proper counseling  about  the poor prognosis was made but  the patient had preterm  premature rupture of membranes 2 days after our scan and had spontaneous abortion in the nearby hospital away from our unit. 

Fetopathology was done after abortion happened and revealed that the mass was an epithelial neoplasm consistent with craniopharyngioma (of the¬†Adamantinomatous type ), showed mixed type of solid and cystic components with calcifications and fat content.¬†The epithelium consists of palisaded basal layer of cells, intermediate stellate reticulum, and a layer of flattened, keratinized squamous cells. Nodules of ‚Äúwet‚ÄĚ keratin¬† are a distinctive feature and gliotic reaction in the surrounding parenchyma.

Images 1, 2 and 3: 2D and color Doppler images showing a large and heterogeneous mass with peripheral vascularity. The size of the mass was almost 10cm x 7cm, and showed solid and cystic components and calcifications.


Images 4 and 5: volume rendering  image  with a coronal cut section  showing  a  large   mass with mixed type showing a solid component  with partial cystic component and calcifications.   


Images 6, 7 and 8:   3D reconstructive HD live image  with a saggital and coronal  sections through the brain and face illustrating   the whole  craniopharyngioma with its start  intracranially  inferomedial to the lateral ventricle and  then  continue  retro orbital, retronasal and in oropharynx, ending in the upper hypopharynx.
LV(lateral ventricle ), M (mass) IC (intracranial portion ), RO (retroorbital portion ), RN (retronasal portion), OPH (oropharyngeal portion).


Prevalence: 25% of intracranial tumors, 50% of suprasellar tumors in children. Only 10 postnatal  cases have been reported with few cases diagnosed prenatally.

 benign midline tumor containing fat, calcification and cystic components.


 Cushing believed that the tumor arose from cell rests derived from the craniopharyngeal duct. 
Others suggest that these tumors arose from remnants of Rathke"s pouch.

Differential diagnosis:
 teratoma, astrocytomas, optic chiasm and hypothalamic glioma, primitive neuroectodermal tumors and lipoma of the corpus callosum.

 very poor with a fatal outcome: only one of 10 previously reported congenital cases survived beyond one year of age.
Recurrence risk. after complete removal: 7%, higher after partial resection.
Not known to be increased in subsequent gestations .



Craniopharyngioma  is  a benign congenital tumors
Persistence of the craniopharyngeal canal is a rare basal skull congenital defect with a range from small canals (in 0.42% of the asymptomatic population [29] ) to large basal cephalocele with major craniofacial defects. Craniopharyngeal canal represents a remnant of the stem of Rathke’s pouch that goes through the sphenoidal synchondrosis between the presphenoid and postsphenoid by a vertical conduct in the basisphenoid, extending from the floor of the sella turcica to the undersurface of this bone and connecting the pituitary fossa with the nasopharynx cavity.

The adenohypophyseal primordium is induced by the adjacent floor of the forebrain from which the neurohypophysis will develop. The primordium is situated immediately external to the oropharyngeal membrane and becomes a pocket, the adenohypophyseal pouch, at 24 days of gestation. The basement membrane of the pouch appears to be in contact with that of the diencephalon, but cells soon become visible between the two membranes and the pouch becomes discontinuous at its apex. Then over the apex, the floor of the forebrain form an envagination, the stem of the pouch becomes narrowed and loses its connection with the roof of the mouth during the sixth week of embryonic development. The craniopharyngeal canal is a channel formed during osteogenesis and which may persist as a passage through the sphenoid bone. It indicates approximately the course of the former stem of the adenohypophyseal pouch [31] 


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 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.  

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‚ÄĚ27. 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).

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.

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 masses2,5.The CT appearance is that of a heterogenous  suprasellar mass, often with nonuniform enhancement, and calcification is seen in 80% of cases18. The large size of these neonatal tumors is also a usual  CT  feature7. 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 T1weighted 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 intensity19. On T2 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‚Äď95%) has a suprasellar component (purely suprasellar, 20‚Äď41%; both supra and intrasellar, 53‚Äď75%) (40, 73, 74), while¬† the purely intrasellar tumors¬† resemble¬† the least common variety (5‚Äď6%) (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)


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).
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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