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2017-07-20  Craniopharyngioma  © Mahmoud Alalfy  www.TheFetus.net

Craniopharyngioma
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 “wet” 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.

Definition:
 benign midline tumor containing fat, calcification and cystic components.

Etiology:
 unknown.

Pathogenesis:
 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.

Prognosis:
 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 .

Management:
 excision.
 

Background 

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.
 
Embryology

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] 

Discussion

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 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 Tweighted 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 age
7. 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.
 
 
References
1. Jooma R, Kendall BE. Intracranial tumors in the first year of life. Neuroradiology 23:267274, 1982.
2. Sauerbrei E, Cooperberg P. Cystic tumors of the fetal and neonatal cerebrum: ultrasound and computed tomographic evaluation. Radiology 147:689692,1983.
3. Radkowski MA, Maidich TP, Tomita T, et al. Neonatal brain tumors: CT and MR findings. J Comput Assist Tomogr 12:1020, 1988.
4. Arnestein LH, Boldrey E, Naffzinger MC. A case report and survey of brain tumors during the neonatal period. J Neurosurg 8:315319, 1951.
5. Lipman SP, Pretorus DH, Rumack CM et al: Fetal intracranial tetatoma: US diagnosis of three cases and a review of the literature. Radiology 157:491494, 1985.
6. Takaku JA, Kodama N, Ohara H, et al. Brain tumors in newborn babies. Childs Brain 4:365375, 1978.
7. Hurst RW, McIlhenny J, Park TS, et al. Neonatal craniopharyngioma: CT and ultrasonographic features. J Comput Assist Tomogr 12:85861, 1988.
8. Majd M, Farkas J, LoPresti JM, et al. A large calcified craniopharyngioma in the newborn. Radiology 99:399400, 1971.
9. Ueyama Y, Kuratsuji T, Lee JY, et al. Congenital giant craniopharyngioma. Acta Pathol Jpn 35:12731277, 1985.
10. Tabaddor K, Shulman K, Dal Canto MC. Neonatal craniopharyngioma. Am J Dis Child 128:381383, 1974.
11. Freeman TB, Abati AD, Topsis J, et al. Neonatal craniopharyngioma. NY State J Med 88:8183, 1988.
12. Cushing H. Intracranial tumors: notes upon a series of two thousand verified cases with surgicalmortality percentages pertaining thereto. Springfield, Ill,Charles C Thomas 1932:9298.
13. Erdheim J. Uber hypophysenganggeschwalste und hirncholesteotome:sitzungsberichte der mathematisch. Naturwiss Klasse Kaiserl Akad Wiss 1904;113, Abt LLL:537726.
14. Goodrich JT, Post KD, Duffy P. Cilated craniopharyngioma. Surg Neurol 24:105111, 1985.
15. Akimura T, Kameda H, Abiko S, et al. Infrasellar craniopharyngioma Neuroradiology 31:180183, 1989.
16. James HE, Edwards MS. Systemic staging of supratentorial extraaxial brain tumors in children. Cancer 56:18001803, 1985.
17. Kang JK, Song JU. Results of the management of craniopharyngioma in children. Childs Neru Syst 4:135138, 1988.
18. Young SC, Zimmerman RA, Nowell MA, et al. Giant systic craniopharyngiomas. Neuroradiology 29:468473, 1987.
19. Zimmerman RA. Magnetic resonance imaging of midline pediatric cerebral neoplasms. Acta Neurochirurgica 35:6064, 1985.
20. Pusey E, Kortman KE, Flannigan BD. MR of craniopharyngiomas: tumor delineation and charactenization. AJR 149:383388, 1987.
21. Yaçsargil MG, Curcic M, Kis M, et al. Total removal craniopharyngiomas: approaches and longterm results in 144 patients. J Neurosurg 73:311, 1990.
22. Fischer EG, Welch K, Belli JA, et al. Treatment of craniopharyngiomas in children 19721981. J Neurosurg 62:496501, 1985.
23. Amendola BE, Gebaski SS, Bermudez AG: Analysis of treatment results in craniopharyngioma. J Clin Oncol 3:2528, 1985.
24. Sorva R, Heiskanen O: Craniopharyngioma in Finland. A Study of 123 cases. Acta Neuroch Wien 81:859, 1986
25. Strom EH, Tangsrud SE: Craniopharyngioma in a boy with centronuclear (myotubular) myopathy. Clin Neuropath 5:877, 1986.
26. Lau YL, Milligan DW: Atypical presentation of craniopharyngioma associated with Moyamoya disease. J R Soc Med 79:2367, 1986.
27. Adamson TE, Wiestler OD, Kleihues P et al: Correlation of clinical and pathological features in surgically treated craniopharyngiomas. J Neurosurg 73:127, 1990.
28. Fernandes VS, Bisi H, Camargo RY et al: Retrospective analysis of the incidence of midline supratentorial neoplasms in children and young patients: craniopharyngiomas, hypophyseal and pineal neoplasms. Rev Paul Med 108:717, 1990.
29- Arey L The craniopharyngeal canal reviewed and reinterpreted. Anat Record 1950; 106:1-16
30- Currarino G, Maravilla KR, Salyer KE. Transsphenoidal  canal ( large craniopharyngeal canal ) and its pathologic implications. Am J Neuroradiol 1985; 6:39-43.
31- O’Rahilly R. Müller F. Human Embriology and Teratology. Second Edit. Wiley Liss,Inc. USA. 1996:317-20.
32-[According to  S. Sartoretti-Schefer et al.Am J Neuroradiol 18:77–87, 1997  . © American Society of Neuroradiology.]
33-[  O. P. Eldevik et al.: Am J Neuroradiol 17:1427–1439, 1996  .© American Society of Neuroradiology.]
34-Petito CK, De Girolami U, Earle K 1976 Craniopharyngiomas. A clinical and pathological review. Cancer 37:1944–1952
35. Graziani N, Donnet A, Bugha TN, Dufour H, Figarella-Branger D, Grisoli F 1994 Ectopic basisphenoidal craniopharyngioma: case report and review of the literature. Neurosurgery 34:346–349
36. Akimura T, Kameda H, Abiko S, Aoki H, Kido T 1989 Infrasellar craniopharyngioma. Neuroradiology 31:180–183
37. Cooper PR, Ransohoff J 1972 Craniopharyngioma originating in the sphenoid bone. J Neurosurg 36:102–106
38. Jiang RS, Wu CY, Jan YJ, Hsu CY 1998 Primary ethmoid sinus craniopharyngioma: a case report. J Laryngol Otol 112:403–405
39. Duff TA, Levine R 1983 Intrachiasmatic craniopharngioma. J Neurosurg 59:176–178
40. Sohn CH, Bail SK, Kim SP, Kim IM, Sevick RJ 2004 Craniopharyngioma in the temporal lobe: a case report. Korean J Radiol 5:72–74
41. Solarski A, Panke ES, Panke TW 1978 Craniopharyngioma in the pineal gland. Arch Pathol Lab Med 102:490–491
42. Bashir EM, Lewis PD, Edwards MR 1996 Posterior fossa craniopharyngioma. Br J Neurosurg 10:613–615
43. Altinors N, Senveli E, Erdogan A, Arda N, Pak I 1984 Craniopharyngioma of the cerebellopontine angle. J Neurosurg 60:842– 844
44. Waga S, Morikawa A, SakakuraM1979 Craniopharyngioma with midbrain involvement. Arch Neurol 36:319–320
45. Davies MJ, King TT, Metcalfe KA, Monson JP 1997 Intraventricular craniopharyngioma: a long-term follow-up of six cases. Br J Neurosurg 11:533–541
46-Crotty TB, Scheithauer BW, Young WF, Davis DH, Shaw EG, Miller GM, Burger PC 1995 Papillary craniopharyngioma: a clinico-pathological study of 48 cases. J Neurosurg 83:206–214
47-Sartoretti-Schefer S, Wichmann W, Aguzzi A, Valavanis A 1997MR differentiation of adamantinous and squamous-papillary craniopharyngiomas. Am J Neuroradiol 18:77–87
48-Kahn EA, Gosch HH, Seeger JF, Hicks SP 1973 Forty-five years experience with the craniopharyngiomas. Surg Neurol 1:5–12
49-. Gorlin RJ, Chaudhry AP 1959 The ameloblastoma and the craniopharyngioma;
the similarities and differences. Oral Surg Oral Med Pathol 12:199–205]
50-M. Cassart ,N. Bosson ,C. Garel ,D. Euri ,F. Avni  Eur Radiol (2008) 18: 2060–2066 DOI 10.1007/s00330-008-0999-5.

 





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