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LETTER TO EDITOR
Year : 2014  |  Volume : 62  |  Issue : 1  |  Page : 100-103

Extracranial infrasellar craniopharyngioma


Department of Neurosurgery, Achantha Lakshmipathy Neurosurgical Centre, Voluntary Health Services Hospital, Taramani, Chennai, Tamil Nadu, India

Date of Submission02-Jan-2014
Date of Decision02-Feb-2014
Date of Acceptance03-Feb-2014
Date of Web Publication7-Mar-2014

Correspondence Address:
Shyam Sundar Krishnan
Department of Neurosurgery, Achantha Lakshmipathy Neurosurgical Centre, Voluntary Health Services Hospital, Taramani, Chennai, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.128356

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How to cite this article:
Senthilvel HP, Krishnan SS, Vasudevan MC. Extracranial infrasellar craniopharyngioma. Neurol India 2014;62:100-3

How to cite this URL:
Senthilvel HP, Krishnan SS, Vasudevan MC. Extracranial infrasellar craniopharyngioma. Neurol India [serial online] 2014 [cited 2020 Feb 26];62:100-3. Available from: http://www.neurologyindia.com/text.asp?2014/62/1/100/128356


Sir,

A 68-year-old male patient presented with epistaxis and frontal headache of 1 month duration. Neurologic examination was normal. Endocrine profile was normal. Magnetic resonance imaging (MRI) showed T1 iso to hypointense and T2 predominately hyperintense solid lesion enhancing intensely with contrast in both nasal cavities, extending into the ethmoidal sinuses and eroding sphenoid sinus, clivus and reaching up to sellar floor [Figure 1]a and b. He underwent endoscopic transnasal approach, the lesion was greyish white, moderately vascular, flaky and suckable in nature [Figure 2]a. The nasal part of the tumor was radically removed and the sphenoidal rostrum was visualized [Figure 2]b. The floor of the sphenoid sinus was eroded and occupied by the lesion and was eroding the clivus up to the level of choana with intact sellar floor. It was radically removed and confirmed on post-operative MRI [Figure 1]c. Post-operatively, the patient had improvement in pre-operative symptoms. Histopathology revealed squamous papillary type of craniopharyngioma [Figure 2]c.
Figure 1: T1 sagittal contrast imaging of the patient showing solid lesion enhancing intensely with contrast seen in nasal cavities eroding ethmoidalsinus, sphenoid sinus, clivus and reaching upto sellar floor; (a) T2 coronal imaging showing solid lesion seen in nasal cavities eroding ethmoidal sinus; (b) Post-operative magnetic resonance imaging brain sagittal contrast showing radical excision of lesion with post-operative changes seen (c)

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Figure 2: Intraoperative photo showing the lesion extending into the nasal cavity; (a) Intraoperative photo showing endoscopic excision of the sphenoid portion of the lesion; (b) Histopathological features showing keratin whorls (blue arrow) separated by fibroblaticstroma (c)

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Craniopharyngiomas commonly are sellar-suprasellar in location and seldom extend into the sphenoid or nasopharynx. [1] Infrasellar craniopharyngiomas are rare presentation. Among these, the isolated infrasellar craniopharyngiomas without sellar involvement are extremely rare, only ten cases have been reported [Table 1]. [2],[3],[4],[5],[6],[7],[8],[9],[10],[11]
Table 1: List of cases reported so far of infrasellar craniopharyngioma

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Craniopharyngiomas originate along the infundibulum at the floor of the third ventricle. As they enlarge, they extend along the paths of least resistance in the parasellar region into the sella inferiorly and/or elevate the floor of the third ventricle superiorly. This is the most common location of craniopharyngiomas. They do not extend into the infrasellar region due to the resistance offered by the sphenoid bone. Infrasellar location of craniopharyngioma is a rare variant they may involve nasopharynx, sphenoid, clivus and the ethmoids. Other locations are third ventricle, pineal gland, sylvian fissure and cerebellopontine angle. [1] Pure infrasellar craniopharyngiomas are significant from embryological point of view. Their origins from ectoderm stomodeum are similar to oral ameloblastomas, which have a similar pathological appearance to infrasellar craniopharyngiomas. The former from migration inferiorly into the oral cavity (enamel) and the latter from migration superiorly toward the hypophysis (pituitary). [7]

Aberrations in the sequence of origin during embryogenesis results in the formation of craniopharyngiomas and their variable location [Figure 3]. The glandular part arises from the out pouching of the ectodermal epithelium covering the roof of stomadeum called the Rathkes diverticulum. The neural or diencephalic part arises by out pouching of the floor of the third ventricle (from the neuroectoderm). The Rathkes diverticulum invaginates and penetrates the mesenchyme in the direction of the diencephalon and forms the Rathkes pouch. The cell of Rathkes pouch proliferate rapidly and gives rise to pars distalis, pars tuberalis and pars intermediata. By the 5 th week, the Rathkes pouch has elongated and becomes constricted at the oral epithelium by the pharyngo-hypophyseal stalk. A remnant of this stalk may persists and gives rise to pharyngeal hypophysis, which give rise to craniopharyngioma as postulated by Mott and Barret. [12] The Rathkes pouch involutes during the 7 th week of gestation. Persistent hypophyseal pharyngeal duct is noted in 33% of cases. The hypophyseal pharyngeal duct extends from the floor of the sella to the vomer. [10] The remnant cords of cells of the primitive Rathkes pouch anywhere along this tract gives rise to craniopharyngioma. Erdheim postulated that the craniopharyngioma originated from the remnants of this obliterated hypophyseal pharyngeal duct, suggesting that these tumors can arise anywhere along the tract of migration of Rathke's pouch from the vomer and from the roof of the nasopharynx to the floor of the sella, within or above it. [1] Craniopharyngioma in infrasellar location originate from this non involuted remnant along the posterior aspect of the vomer at the junction of the soft palate and nasal septum.

The symptomatology of suprasellar tumor is often characterized by visual field defects, pituitary insufficiencies and or features of raised intracranial pressure. The craniopharyngiomas located in the nasopharyngeal region presents with frontal headache, nasal obstruction, epistaxis, nasopharyngeal and/or nasal fossa masses. Infrasellar and intrasellar craniopharyngioma have similar radiological characteristics. These tumors are heterogeneous with solid and cystic components, calcification, lytic lesions and irregular enhancement on computed tomography. Magnetic resonance image reveals the cyst as high signal intensity on T1- and T2-weighted sequences indicating high water and protein content whereas the solid component have high signal intensity on T2 and variable intensity on T1-weighted images. Differential diagnosis on radiological basis for infrasellar craniopharyngiomas can be juvenile angiofibroma, rhabdomyosarcoma, lymphoma, squamous cell carcinoma and pharyngeal chordoma. [7],[10]
Figure 3: Embryology: (a) Anterioradenohypophysis (A) origin: Rathkes pouch (Pink ellipsoid) directing upwards from stomodeumectoderm. Posteriorneurohypophysis (N) origin: Diencephalic – outpouching downward from fl oor of third ventricle (Grey ellipsoid). (1) 5th week: Elongated Rathkes pouch constricted at the oral epithelium by the pharyngo-hypophyseal stalk (pharyngiohypophysialcannal [PHC]) (pink star) and detaches (2, 3). (b) Remanent of development PHC/ stalk (2); 1= suprasellar, 2 = sellar, 3 = sphenoid, 4 = nasopharyngeal. Craniopharyngioma locations (3)

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Craniopharyngiomas are most frequently cystic or solid or both, irregular and adherent to the surrounding structures. The cyst contains the classical "machine oil" fluid with shimmering cholesterol crystals. Histologically these are epithelial tumors, the cyst being lined by simple stratified squamous. [1] There are two clinicopathological variants: Adamantinomatous type seen mainly in children with poor overall outcome and a squamous papillary type which is common in adults with a better prognosis. Almost all cases of infrasellar craniopharyngiomas are of squamous papillary type. [11]

Treatment for a craniopharyngioma whether infrasellar or otherwise is radical excision whenever possible. Unlike sellar, suprasellar craniopharyngioma, where vital structures preclude complete excision, pure infrasellar craniopharyngioma can be removed completely. However inevitable micro-remanentimay results in recurrence. Subtotal resection will mandate re-surgery or adjuvant radiotherapy. The approach to these tumors can be microscopic or endoscopic as per the surgeons experience. [5],[7]

Infrasellar craniopharyngiomas should be differential diagnosis in patients with intranasal mass and epistaxis. In view of lack of vital structure adhesion we advocate radical excision as a goal and endoscopic endonasal approach as it provides excellent panoramic visualization.

 
  References Top

1.Falavigna A, Kraemer JL. Infrasellar craniopharyngioma: Case report. Arq Neuropsiquiatr 2001;59:424-30.  Back to cited text no. 1
    
2.Cooper PR, Ransohoff J. Craniopharyngioma originating in the sphenoid bone. Case report. J Neurosurg 1972;36:102-6.  Back to cited text no. 2
    
3.Lewin R, Ruffolo E, Saraceno C. Craniopharyngioma arising in the pharyngeal hypophysis. South Med J 1984;77:1519-23.  Back to cited text no. 3
    
4.Akimura T, Kameda H, Abiko S, Aoki H, Kido T. Infrasellar craniopharyngioma. Neuroradiology 1989;31:180-3.  Back to cited text no. 4
    
5.Byrne MN, Sessions DG. Nasopharyngeal craniopharyngioma. Case report and literature review. Ann Otol Rhinol Laryngol 1990;99:633-9.  Back to cited text no. 5
    
6.Jiang RS, Wu CY, Jan YJ, Hsu CY. Primary ethmoid sinus craniopharyngioma: A case report. J Laryngol Otol 1998;112:403-5.  Back to cited text no. 6
    
7.Deutsch H, Kothbauer K. Infrasellar craniopharyngiomas. Case report and review of the literature. Skull Base 2001;11:121-8.  Back to cited text no. 7
    
8.Arndt S, Wiech T, Mader I, Maier W. Entire infrasellar craniopharyngioma simulating clival chordoma. Otolaryngol Head Neck Surg 2007;137:981-3.  Back to cited text no. 8
    
9.Mohanty S, Balakrishnan S. Management of extra sellar craniopharyngioma masquerading as hypertrophied adenoid tissue in a 6-year-old boy. Int J Pediatr Otorhinolaryngol 2008;72:1441-4.  Back to cited text no. 9
    
10.Nourbakhsh A, Brown B, Vannemreddy P, Lian T, Nanda A, Guthikonda B. Extracranial infrasellar ectopic craniopharyngioma: A case report and review of the literature. Skull Base 2010;20:475-80.  Back to cited text no. 10
    
11.Magill JC, Ferguson MS, Sandison A, Clarke PM. Nasal craniopharyngioma: Case report and literature review. J Laryngol Otol 2011;125:517-9.  Back to cited text no. 11
    
12.Mott FW, Barret JO. Three cases of tumor of the third ventricle. Arch Neurol 1899;1:417-40.  Back to cited text no. 12
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]
 
 
    Tables

  [Table 1]

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