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Infrasellar craniopharyngioma
Clinical Neurology and Neurosurgery 114 (2012) 112–119
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Infrasellar craniopharyngioma Xin Yu ∗ , Rui Liu, Yaming Wang, Hongwei Wang, Hulei Zhao, Zhaohun Wu Department of Neurosurgery, Navy General Hospital, Beijing, China
a r t i c l e
i n f o
Article history: Received 4 May 2011 Accepted 21 September 2011 Available online 21 October 2011 Keywords: Infrasellar Craniopharyngioma Clinical manifestation Therapy Prognosis
a b s t r a c t Object: Infrasellar craniopharyngioma (IC) is a rare tumor. This study aimed to investigate the clinical manifestations, treatment methods, and prognosis of IC, which invades the cranial base, nasal sinuses, nasopharynx and clivus. Methods: Eleven consecutive cases of IC who received treatment in People’s Liberation Army Navy General Hospital from 1988 to 2007 were retrospectively analyzed, and the clinical manifestations of IC were summarized. At the same time, literature pertinent to IC was reviewed. These patients consisted of six males and five females with an average age of 28.5 years (range 7–52 years old). Among them, nine cases were identified as simple IC and two cases as suprasellar and IC. Clinical manifestations included headache (seven cases), nasal obstruction (four cases), polydipsia and polyuria (four cases), visual disorder (five cases), delayed sex organ development (three cases), menstrual disorder (one case) and no symptoms (one case). Tumor invasion regions included sellar bottom, ethmoidal sinus, maxillary sinus, sphenoidal sinus, infrasellar region, clivus, nasopharynx and nasal cavity. Solid craniopharyngioma was observed in three cases, cystic craniopharyngioma in seven cases, and mixed cystic and solid type in one case. Four cases underwent craniotomy for tumor resection (three cases also received adjuvant external beam radiation therapy), two cases underwent transnasal approach tumor resection under endoscope guidance (one case simultaneously received adjuvant interstitial brachytherapy) and four cases underwent stereotactic interstitial radiation (radioisotope 32 P brachytherapy). Results: All cases were followed up for an average of 22.5 years (range 9–98 months). Imaging results showed that tumors disappeared in one case, were clearly reduced in eight cases and were stable in two cases. Clinical symptoms disappeared in three cases, and improved in seven cases. No symptoms appeared in the case presenting with no symptoms. All patients were able to resume work, study and daily tasks. Conclusions: IC is rare (the present cases account for 0.23% of all retrieved cases). Its chief clinical manifestations include headache, nasal obstruction, polydipsia and polyuria, and visual disorder. Lesions include solid, cystic, and mixed cystic and solid types. It is very difficult to resect the whole diseased region because this disease invades the cranial base, nasal sinuses and nasopharynx. Individualized treatments should be used according to lesion characteristics and invasion range, for example, stereotaxic interstitial brachytherapy. Radical resection or partial resection plus external beam radiation therapy produces better prognosis in IC than intracranial craniopharyngioma. © 2011 Elsevier B.V. All rights reserved.
1. Infrasellar craniopharyngioma Craniopharyngioma (CP) is a rare benign epithelial tumor in the cranium and accounts for 1.5–1.6% of all intracranial tumors [1,2]. It arises within the sella turcica, predominantly in the suprasellar region and occasionally in the intrasellar region and third ventricle, but it rarely arises in the intrasellar region or invades the nasopharynx [3–5]. The present study reports 11 cases of infrasellar
∗ Corresponding author at: Neurosurgical Department, Navy General Hospital, 6, Fucheng Road, Beijing 100048, China. Tel.: +86 10 13701050134; fax: +86 10 68780891. E-mail address: [email protected] (X. Yu). 0303-8467/$ – see front matter © 2011 Elsevier B.V. All rights reserved. doi:10.1016/j.clineuro.2011.09.010
craniopharyngioma (IC), proposes a precise definition and classification based on retrieved literature, and discusses the clinical manifestation, treatment methods and prognosis of IC that invades the cranial base, nasal sinuses, nasopharynx and clivus.
2. Patient and methods Eleven consecutive cases of IC who received treatment in PLA Navy General Hospital from 1988 to 2007 were retrospectively analyzed. These cases accounted for 0.52% of all cases of CP admitted concurrently. Imaging results revealed that there were nine cases presenting tumors in or below the sellar floor, or the simple infrasellar type of tumor, and two cases presenting suprasellar and
Table 1 Clinical characteristics in 11 cases with infrasellar craniopharyngioma. Gender
Age (y)
Clinical manifestation
Invasion regions
Tumor type
Surgical approach
Tumor section
Other treatments
Follow-up time (months)
Imaging characteristics
Clinical outcomes
1
Male
18
S, I, ES,SS, C, NF, NPX
Massive resection
Adjuvant external-beam radiation therapy (50 GY)
28
Reduced
Improved clinical symptoms, resume physical labor
Male
30
Extradural anterior cranial fossa approach
Massive resection
Adjuvant external-beam radiation therapy (55 GY)
10
Obviously reduced
Improved clinical symptoms, resume housework
3
Female
21
Primarily solid type, massive calcification, normal blood supply, hard and tough texture Primarily solid type, massive calcification, normal blood supply, hard and tough texture Simply cystic type, intact cystic wall, cholesterol crystal
Extradural anterior cranial fossa approach
2
Headache, nasal obstruction, polydipsia and polyuria, visual disorder, delayed development Headache, nasal obstruction, polydipsia and polyuria, visual disorder, delayed development Headache, nasal obstruction
Stereotactic
Interstitial brachythera
None
10
Obviously reduced
Absent clinical symptoms, resume daily work
4
Male
7
Polydipsia and polyuria, visual disorder
I, S, SS, C
Double cystic type, cholesterol crystal
Frontal base stereotactic approach
None
12
Stable
Improved clinical symptoms, resume physical labor
5
Male
48
Headache, nasal obstruction
I, S, SS, C
Extradural anterior cranial fossa approach
Adjuvant external-beam radiation therapy (55 Gy)
88
Obviously reduced
Absent clinical symptoms, resume daily work
6
Male
52
Headache
I, C
Primarily solid type, hard and tough texture, abundant blood supply, Cystic type
Partial resection, interstitial brachytherapy Massive resection
Interstitial brachytherapy
None
9
Obviously reduced
Absent clinical symptoms, resume daily work
7a
Female
33
No symptoms
I, C
Cystic type
Clinical observation
None
10
No change
No new symptoms, resume daily work
8
Female
15
Headache, menstrual disorder, lactation
I, C
Mixed cystic and solid type
Radical resection
None
10
Disappeared
Improved clinical symptoms, resume learning
9
Male
24
I, C
Cystic type, oval shaped
Interstitial brachytherapy
None
10
Reduced
Improved clinical symptoms, resume daily work
10
Female
16
Polydipsia and polyuria, delayed sex organ development Decreased visual acuity
Transnasal approach under endoscope guidance Stereotactic
I, C
Primarily cystic type
Stereotactic
Interstitial brachytherapy
Gamma knife radiosurgery
28
Reduced
Improved clinical symptoms, resume learning
11
Female
15
Headache, decreased visual acuity
SS, ST, C, S
Mixed cystic and solid type
Transnasal approach under endoscope guidance,
Subtotal resection, interstitial brachytherapy
None
12
Reduced
Improved clinical symptoms, resume learning
S, I, ES, SS, C, NF, NPX, MS
I, ES, SS, C, NPX, NF
Stereotactic
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Case no.
NPX, nasopharynx; SS, sphenoid sinus; ST, sella turcica; S, suprasellar; I, infrasellar; ES, ethmoid sinuses; MS, maxillary sinus; NF, nasal fossa; C, clivus. a Imaging diagnosis. 113
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Fig. 1. Case 2: A 30-year-old male suffered decreased visual acuity over the course of 1 year. He also had headaches, nasal obstruction and right eye blindness over a period of 6 months. Physical examination demonstrated poorly developed genital system, right eye blindness, left visual acuity lost and bilateral optic atrophy. CT examination revealed mixed density lesion with an eggshell-like calcified edge on the bottom of the anterior and middle cranial fossa and in the sella region and the sellar sclerotin was obviously destroyed. The nasopharynx, right ethmoidal sinus, right maxillary sinus, right nasal cavity and sphenoidal sinus (A) were involved. MRI revealed long T1 and T2 signals in the above-mentioned region (B). He received subtotal resection of craniopharyngioma through frontal extradural approach. After surgery, the visual acuity of the left eye recovered to 0.1, and adjuvant external-beam radiation therapy (55 Gy) was performed. Ten months later, MRI showed that a large amount of tumor tissue was resected (C), and that the patient could resume his duties.
infrasellar tumor, which was determined by tumor body (≥80% of tumor volume) below the sellar diaphragm and a small part of the tumor components (<20% of tumor volume) able to penetrate the sellar diaphragm and develop towards the suprasellar region. The clinical manifestation and treatment methods of these 11 cases of IC were retrospectively analyzed. An average of 22.5 months (range 9–98 months) of clinical observation and follow up examination were performed. Retrieved literature was also used to discuss this disease.
3. Results The clinical manifestation, imaging characteristics, treatment methods, and follow-up results of 11 cases are shown in Table 1. The present patients consisted of six males and five females, and averaged 28.5 years of age (range 7–52) years. Eight cases were above 15 years of age, and three cases below 15 years old. Clinical manifestations included headache (7 cases), visual disorder (5 cases), nasal obstruction (4 cases), polydipsia and polyuria (4 cases), delayed sex organ development (3 cases), menstrual disorder (1 case), and no symptom (1 case). Tumor invasion regions included sellar bottom, ethmoidal sinus, maxillary sinus, sphenoidal sinus, infrasellar region, clivus, nasopharynx and nasal cavity. Solid CP was observed in three cases, cystic CP in seven cases (single cystic 5 cases and multiple cystic 2 cases), and mixed cystic and solid type in one case (with intact cystic wall, as determined by imaging and gross observation). Diagnosis evidence included pathological findings (six cases), cholesterol crystal hydatid fluid (four cases) and imaging data (one case).
Three cases of solid tumor and one case of double cystic tumor received the extradural anterior cranial fossa approach. The solid tumors were hard and tough and showed abundant blood supply. Massive section and adjuvant external-beam radiation therapy (one tumor at 50 Gy and the remaining two at 55 Gy) were performed. Following surgery, these three solid tumor cases were followed up for 10, 28 and 88 months. Results showed that the tumors were greatly reduced. Cystic tumor patients did not receive external-beam radiation therapy. Twelve-month follow-up results showed that the cystic tumors were stable. Two cases of cystic-solid tumor received tumor resection through a transnasal approach under endoscope guidance. Radical tumor resection was performed in one case, and the tumor disappeared 10 months later. Subtotal resection followed by stereotactic interstitial radiation (radioisotope 32 P brachytherapy) was performed in the other case, and the tumor was greatly reduced 12 months later. Four cases of cystic tumor underwent stereotactic interstitial radiation (radioisotope 32 P brachytherapy). Gamma knife radiosurgery was also used in one case to treat the solid tumor part. Tumor volume was obviously reduced within the follow-up period. No obvious changes were observed in the case presenting no clinical manifestations during the 10-month follow-up period. An average of 22.5 months (9–88 months) of follow-up results is shown as follows: the tumor disappeared in one case, was obviously reduced in eight cases and was stable in two cases following imaging. Clinical symptoms disappeared in three cases and improved in seven cases. No new symptoms were observed in the case presenting no symptoms. All patients were able to resume their work, study and normal everyday activities.
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Fig. 2. Case 3: A 21-year-old female presented with a gradually worsening vague pain in the anterior frontal region and nasal obstruction over a course of 6 months. Physical examination showed bilateral rhinostenosis. Cranial CT showed the space-occupying lesion of the anterior and middle cranial fossa. The tumor invaded the entire sphenoidal sinus, ethmoidal sinus, clivus, nasopharynx and nasal cavity. The edge of the lesion tissue was clear and the peripheral sclerotin was compressed and thinned (A). MRI revealed a cystic lesion (B). She received stereotactic radioisotope 32 P brachytherapy. After surgery, all symptoms disappeared. Six months later, MRI revealed evidently reduced lesions and good pituitary gland structure (C and D).
Illustrative Cases were seen in Figs. 1–4. 4. Discussion This report is the first to report a group of IC cases, comprising 11 cases that account for 0.52% of the 2 130 cases of CP concurrently admitted. CP primarily occurs in the sella turcica and suprasellar region (90–94%), and occasionally develops towards the anterior (2–5%) and lateral (2%) region of sella turcica or posterior cranial fossa (1–4%) [4]. It rarely originates from other regions, such as the nasopharynx, sphenoid bone, third ventricle, pineal body region, lateral fissure or pons, and IC is extremely rare [5]. From then Bock first reported IC in 1924 [6], to May 2009, approximately 50 cases of IC have been reported worldwide [2,7–37]. Benitez [7] classified IC into two types according to possible origin regions: (1) a typical CP, originating in the sella turcica and developing downward to the sphenoidal sinus; (2) originating from sphenoid bone or the derivate of residual embryo craniopharyngeal canal in the nasopharynx, which originates from sphenoidal sinus or nasopharynx. The tumor epidurally grows upward but does not destroy the sella turcica. However, large tumors cannot be classified based on these criteria. Chen [8] collated 27 cases of IC reported before 2000, in which 18 cases were simply IC, and the remaining nine cases were infrasellar and suprasellar CP. In this report, there
were nine cases that were confirmed suffering from simple IC due to the classification criteria: tumor originating in or below the sellar floor, and intrasellar structure, which was demonstrated by imaging; there were two cases presenting suprasellar and infrasellar tumors, which was determined by tumor body (≥80% of tumor volume) below the sellar diaphragm and a small part of tumor components (<20% of tumor volume) able to penetrate the sellar diaphragm and develop towards the suprasellar region. In this report, male and female patients were roughly equal in proportion, and 73% (8/11) of patients were over 15 years of age, which is similar to a previous report [8]. The IC reported here is also called infrasellar adamantinoma [9,10], pharyngeal CP [11], nasopharyngeal CP [12], or sphenoid sinus CP [13]. 4.1. Embryonic development Several theories regarding the origin of CP are related to adenohypophysis during embryogenesis [14–17]. Tumors possibly originate from the residual fraction of the pharyngeal pituitary. At week 4 of embryogenesis, a Rathke’s cyst forms on the top of the stomodeum and before the oropharyngeal membrane. The cyst then extends through the sphenoid bone development region until it reaches the sella turcica, then converges with the neuroectoderm of the neurohypophysis, which develops downward from the top
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Fig. 3. Case 8: A 15-year-old female suffered from headache over a course of 3 months, as well as menstrual disorder and lactation prior to admission. MRI (A) revealed that the tumor, cystic predominant, was primarily located in the sphenoidal sinus and superior clivus. A CT scan (B) revealed that the sclerotin of the sclerotin of sphenoidal sinus lateral wall and clivus were compressed and thinned. Craniopharyngioma was resected transnasally. At 10 months after surgery, MRI (C) revealed that the tumor disappeared and showed a clear anatomical structure of sella turcica. The patient explained that her headache disappeared and menstruation recovered.
of the third ventricle of the embryonic brain, and finally differentiates into the adenohypophysis. In the path passed by Rathke’s cyst, a solid cell cord forms to connect the adenohypophysis and the stomodeum ectoderm. During later embryogenesis, this cell cord resides between two centers of chondrification, which develop into the sphenoid bone body and wings, and differentiate into a craniopharyngeal canal. This passage extracranially extends from the anterior part of the sphenoidal hypophysial fossa up to the juncture between the posterior nasal septum and the palatum. The adult pharyngeal pituitary develops from the chordal tail. 4.2. Origin theory In 1899, Mott [38] first proposed that CP possibly originated from the pharyngeal pituitary. In 1904, Erheim [39] reported that the pharyngeal pituitary resided in the posterior part of the pearshaped bone and first proposed the theory that CP originated from the craniopharyngeal canal during embryogenesis. In 1931, Carmichael [40] found that 32.7% of the craniopharyngeal canal resided in the nearby region of hypophysis during autopsy. Later, McGrath [41] confirmed that the blood of the adult functional pharyngeal pituitary came from the hypothalamic–hypophyseal portal system and transferred to the vascular bed of the pharyngeal pituitary via the sphenoid bone. CP possibly occurs in any region
along the craniopharyngeal canal and even the functional pharyngeal pituitary [43]. Therefore, tumors can develop along a pathway including the posterior part of the pear-shaped bone above the juncture of the soft palate and the nasal septum, sphenoid bone, sellar floor and up to the infrasellar regions, such as nasopharynx. There is another viewpoint that CP originates from the squamous epithelium tissue of adenohypophysis. However, squamous cell nests are rarely seen in this region of children. Therefore, it is possible that CP originate from prehypophysial adult cells rather than the squamous cell metaplasia in children. Erdheim’s theory of adenohypophyseal embryogenesis has been presently supported by several studies. In 2001, Chen [8] reported a case of CP. Imaging examination showed that both the suprasellar region and sphenoidal sinus were invaded by tumors, and successive CT scans revealed that space-occupying lesions terminated in the juncture of the soft palate and nasal septum, further confirming Erdheim’s theory. The regions involved in IC include the nasopharynx, sphenoidal sinus, ethmoidal sinus, maxillary sinus and nasal cavity, in which, the sphenoidal sinus is most vulnerable to IC. 4.3. Clinical manifestations Compared with suprasellar and intrasellar CP, IC is diversified in clinical manifestations, and its symptoms and physical signs are
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Fig. 4. Case 9: A 24-year-old man was admitted owing to 10 years of delayed development of sex organs caused by polydipsia and polyuria. He underwent craniotomy twice for partial resection of tumor. Postoperative MRI revealed an infrasellar oval cystic space-occupying lesion invaded the sphenoidal sinus and clivus. The intrasellar components were shifted upwards (A and B). Stereotactic radioisotope 32 P brachytherapy was performed. At 6 months postoperatively, MRI revealed that the reduced lesion (C), and the patient could resume his daily work.
closely related to tumor region, size, and space-occupying compression to, and influence on, peripheral structures [33]. Clinical manifestations are classified into six kinds: (1) nasopharyngeal CP clinically present frontal headache, nasal obstruction, epistaxis, space-occupying lesions in the nasopharynx and nasal cavity, therefore, cases are first admitted to the otorhinolaryngology department [5,6,13,15,33]. There were seven cases of nasopharyngeal CP in this report; (2) sphenoidal sinus CP clinically present with headache and cavernous sinus syndrome [11,13], and there were six such cases; (3) intrasellar CP clinically present with secondary prehypophysial hypofunction, such as diabetes mellitus, amenorrhea, decreased sexual desire and general body deterioration [7], and there were four such cases; (4) suprasellar CP clinically present with damaged visual acuity, including diplopia, defect of visual field (mostly bitemporal hemianopsia) and loss of sight, and there were five cases; (5) hypothalamus damage clinically present with diabetes insipidus, hypoevolutism and intelligence changing [42,43], and there were four cases; and (6) in the late period of IC, intracranial pressure increases, resulting in headache accompanied by vomiting, vertigo and an impaired cranial nerve [4,42]. There was one case presenting no symptoms in this report.
its symptoms and physical signs lack specificity. Thus when this disease is finally determined, tumor volume is relatively large. Cranial plain films, tomography and CT images are commonly used [11] because they can display nasal sinus changes, tumor range, cystic space-occupying lesions in expanded sella turcica, sclerotin corrosion and tumor calcification. CT scans [7,16,17] can detect calcified cystic solid CP tissue and can help determine whether tumors are multicystic, the cyst position and changes in intensity. Multiple calcified tissues can be observed in tumor tissue, especially in young patients, but rarely in adult patients. MRI [7,17,44] can clearly show tumor range, the solid or cystic part, enhanced scanning range and the relationship to adjacent structures. Although CT scans are more specific, owing to their ability to display calcified tissue, MRI scans can provide information on the surgical approach required by precisely measuring tumor range. Through the use of the above-mentioned imaging techniques, it is not hard to diagnose typical IC, but for the non-typical position and imaging manifestations, IC should be identified from other lesions, and a biopsy can be used if necessary. Early diagnosis of IC can help enhance therapeutic effects. 4.5. Treatment
4.4. Diagnosis Diagnosis of IC at early stages is difficult because lesioned tissue is primarily located in the anterior-middle cranial fossa and
Previous methods for treatment of IC include hydatid fluid resorption or cystic fistulation [17], which can relieve intracystic pressure and improve symptoms, however, they have a high
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relapse rate. In most cases, surgical resection is the first option. The tumor can be resected via a transcranial or transsphenoidal approach. Some rhinolaryngologists also select the transpalatal approach, transnasal septum approach, and nasal side incision approach. The transnasal septum approach in conjunction with the transsphenoidal approach, based on tumor site and range, is also used. However, total resection of the tumor is the ideal method [45], as it provides the greatest opportunity of long-term survival for patients. However, total resection of tumors can also increase mortality and mutilation rates in some patients because of wide tumor range and may involve adjacent structures. Subtotal resection in conjunction with postoperative radiology therapy would be a relatively good selection. CP is sensitive to radiology therapy, which can prevent relapsed tumors and improve patients’ survival period [46]. Evidence exists that postoperative radiology therapy can reduce tumor relapse [45–47]. Therefore, postoperative radiotherapy has become an important factor to prevent tumor relapse and improve patients’ survival. For cystic or primarily cystic CP, stereotactic interstitial radiation is a selectable effective therapeutic method [46,47]. Due to great differences in tumor size, position and the possible involvement of adjacent structures, individualized treatment should be selected. For example, stereotactic interstitial radiation should be selected for simple cystic tumors; stereotactic interstitial radiation in combination with gamma knife radiosurgery or transnasal resection of tumor under the guide of endoscope should be used for cystic and solid tumors; total or subtotal resection of tumors in conjunction with adjuvant external-beam radiation therapy should be used for solid type of tumors. Clinical observation is selected for cases who present small lesion volume and no symptoms. An average of 22.5 months of follow-up demonstrates that all cases exhibited absent or improved clinical symptoms and all patients could resume their work, study and everyday activities. Imaging results revealed that tumors disappeared in one case, reduced in eight cases and were stable in two cases. 4.6. Prognosis Compared with suprasellar and intrasellar CP, the brain tissue structures invaded by IC are not important, so the patients with IC can acquire a better prognosis. 5. Conclusion IC is extremely rare and usually occurs in the sphenoidal sinus. IC comprising solid, cystic, or cystic mixed with solid types, can occur in any region of the degenerative craniopharyngeal canal pathway, including sphenoid bone, ethmoid bone, and nasopharynx. Clinical manifestations depend on invaded structure, but headache and nasal obstruction are common, and secondary prehypophysial hypofunction, impaired visual acuity and hypothalamus damage are commonly seen. Diagnosis primarily depends on CT or MRI imaging. Individualized treatment should be selected. For example, stereotactic interstitial radiation should be selected for simple cystic tumors, stereotactic brachytherapy combination with gamma knife radiosurgery or transnasal resection of tumors for mixed tumors, total or subtotal resection of tumors in conjunction with adjuvant external-beam radiation therapy for solid type tumors and clinical observation for cases who present small lesion volume and no symptoms. IC has better prognosis than suprasellar and intrasellar CP. References [1] Bunin GR, Surawicz TS, Witman PA, Preston-Martin S, Davis F, Bruner JM. The descriptive epidemiology of craniopharyngioma. J Neurosurg 1998;89:547–51.
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Clinical Neurology and Neurosurgery journal homepage: www.elsevier.com/locate/clineuro
Infrasellar craniopharyngioma Xin Yu ∗ , Rui Liu, Yaming Wang, Hongwei Wang, Hulei Zhao, Zhaohun Wu Department of Neurosurgery, Navy General Hospital, Beijing, China
a r t i c l e
i n f o
Article history: Received 4 May 2011 Accepted 21 September 2011 Available online 21 October 2011 Keywords: Infrasellar Craniopharyngioma Clinical manifestation Therapy Prognosis
a b s t r a c t Object: Infrasellar craniopharyngioma (IC) is a rare tumor. This study aimed to investigate the clinical manifestations, treatment methods, and prognosis of IC, which invades the cranial base, nasal sinuses, nasopharynx and clivus. Methods: Eleven consecutive cases of IC who received treatment in People’s Liberation Army Navy General Hospital from 1988 to 2007 were retrospectively analyzed, and the clinical manifestations of IC were summarized. At the same time, literature pertinent to IC was reviewed. These patients consisted of six males and five females with an average age of 28.5 years (range 7–52 years old). Among them, nine cases were identified as simple IC and two cases as suprasellar and IC. Clinical manifestations included headache (seven cases), nasal obstruction (four cases), polydipsia and polyuria (four cases), visual disorder (five cases), delayed sex organ development (three cases), menstrual disorder (one case) and no symptoms (one case). Tumor invasion regions included sellar bottom, ethmoidal sinus, maxillary sinus, sphenoidal sinus, infrasellar region, clivus, nasopharynx and nasal cavity. Solid craniopharyngioma was observed in three cases, cystic craniopharyngioma in seven cases, and mixed cystic and solid type in one case. Four cases underwent craniotomy for tumor resection (three cases also received adjuvant external beam radiation therapy), two cases underwent transnasal approach tumor resection under endoscope guidance (one case simultaneously received adjuvant interstitial brachytherapy) and four cases underwent stereotactic interstitial radiation (radioisotope 32 P brachytherapy). Results: All cases were followed up for an average of 22.5 years (range 9–98 months). Imaging results showed that tumors disappeared in one case, were clearly reduced in eight cases and were stable in two cases. Clinical symptoms disappeared in three cases, and improved in seven cases. No symptoms appeared in the case presenting with no symptoms. All patients were able to resume work, study and daily tasks. Conclusions: IC is rare (the present cases account for 0.23% of all retrieved cases). Its chief clinical manifestations include headache, nasal obstruction, polydipsia and polyuria, and visual disorder. Lesions include solid, cystic, and mixed cystic and solid types. It is very difficult to resect the whole diseased region because this disease invades the cranial base, nasal sinuses and nasopharynx. Individualized treatments should be used according to lesion characteristics and invasion range, for example, stereotaxic interstitial brachytherapy. Radical resection or partial resection plus external beam radiation therapy produces better prognosis in IC than intracranial craniopharyngioma. © 2011 Elsevier B.V. All rights reserved.
1. Infrasellar craniopharyngioma Craniopharyngioma (CP) is a rare benign epithelial tumor in the cranium and accounts for 1.5–1.6% of all intracranial tumors [1,2]. It arises within the sella turcica, predominantly in the suprasellar region and occasionally in the intrasellar region and third ventricle, but it rarely arises in the intrasellar region or invades the nasopharynx [3–5]. The present study reports 11 cases of infrasellar
∗ Corresponding author at: Neurosurgical Department, Navy General Hospital, 6, Fucheng Road, Beijing 100048, China. Tel.: +86 10 13701050134; fax: +86 10 68780891. E-mail address: [email protected] (X. Yu). 0303-8467/$ – see front matter © 2011 Elsevier B.V. All rights reserved. doi:10.1016/j.clineuro.2011.09.010
craniopharyngioma (IC), proposes a precise definition and classification based on retrieved literature, and discusses the clinical manifestation, treatment methods and prognosis of IC that invades the cranial base, nasal sinuses, nasopharynx and clivus.
2. Patient and methods Eleven consecutive cases of IC who received treatment in PLA Navy General Hospital from 1988 to 2007 were retrospectively analyzed. These cases accounted for 0.52% of all cases of CP admitted concurrently. Imaging results revealed that there were nine cases presenting tumors in or below the sellar floor, or the simple infrasellar type of tumor, and two cases presenting suprasellar and
Table 1 Clinical characteristics in 11 cases with infrasellar craniopharyngioma. Gender
Age (y)
Clinical manifestation
Invasion regions
Tumor type
Surgical approach
Tumor section
Other treatments
Follow-up time (months)
Imaging characteristics
Clinical outcomes
1
Male
18
S, I, ES,SS, C, NF, NPX
Massive resection
Adjuvant external-beam radiation therapy (50 GY)
28
Reduced
Improved clinical symptoms, resume physical labor
Male
30
Extradural anterior cranial fossa approach
Massive resection
Adjuvant external-beam radiation therapy (55 GY)
10
Obviously reduced
Improved clinical symptoms, resume housework
3
Female
21
Primarily solid type, massive calcification, normal blood supply, hard and tough texture Primarily solid type, massive calcification, normal blood supply, hard and tough texture Simply cystic type, intact cystic wall, cholesterol crystal
Extradural anterior cranial fossa approach
2
Headache, nasal obstruction, polydipsia and polyuria, visual disorder, delayed development Headache, nasal obstruction, polydipsia and polyuria, visual disorder, delayed development Headache, nasal obstruction
Stereotactic
Interstitial brachythera
None
10
Obviously reduced
Absent clinical symptoms, resume daily work
4
Male
7
Polydipsia and polyuria, visual disorder
I, S, SS, C
Double cystic type, cholesterol crystal
Frontal base stereotactic approach
None
12
Stable
Improved clinical symptoms, resume physical labor
5
Male
48
Headache, nasal obstruction
I, S, SS, C
Extradural anterior cranial fossa approach
Adjuvant external-beam radiation therapy (55 Gy)
88
Obviously reduced
Absent clinical symptoms, resume daily work
6
Male
52
Headache
I, C
Primarily solid type, hard and tough texture, abundant blood supply, Cystic type
Partial resection, interstitial brachytherapy Massive resection
Interstitial brachytherapy
None
9
Obviously reduced
Absent clinical symptoms, resume daily work
7a
Female
33
No symptoms
I, C
Cystic type
Clinical observation
None
10
No change
No new symptoms, resume daily work
8
Female
15
Headache, menstrual disorder, lactation
I, C
Mixed cystic and solid type
Radical resection
None
10
Disappeared
Improved clinical symptoms, resume learning
9
Male
24
I, C
Cystic type, oval shaped
Interstitial brachytherapy
None
10
Reduced
Improved clinical symptoms, resume daily work
10
Female
16
Polydipsia and polyuria, delayed sex organ development Decreased visual acuity
Transnasal approach under endoscope guidance Stereotactic
I, C
Primarily cystic type
Stereotactic
Interstitial brachytherapy
Gamma knife radiosurgery
28
Reduced
Improved clinical symptoms, resume learning
11
Female
15
Headache, decreased visual acuity
SS, ST, C, S
Mixed cystic and solid type
Transnasal approach under endoscope guidance,
Subtotal resection, interstitial brachytherapy
None
12
Reduced
Improved clinical symptoms, resume learning
S, I, ES, SS, C, NF, NPX, MS
I, ES, SS, C, NPX, NF
Stereotactic
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Case no.
NPX, nasopharynx; SS, sphenoid sinus; ST, sella turcica; S, suprasellar; I, infrasellar; ES, ethmoid sinuses; MS, maxillary sinus; NF, nasal fossa; C, clivus. a Imaging diagnosis. 113
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Fig. 1. Case 2: A 30-year-old male suffered decreased visual acuity over the course of 1 year. He also had headaches, nasal obstruction and right eye blindness over a period of 6 months. Physical examination demonstrated poorly developed genital system, right eye blindness, left visual acuity lost and bilateral optic atrophy. CT examination revealed mixed density lesion with an eggshell-like calcified edge on the bottom of the anterior and middle cranial fossa and in the sella region and the sellar sclerotin was obviously destroyed. The nasopharynx, right ethmoidal sinus, right maxillary sinus, right nasal cavity and sphenoidal sinus (A) were involved. MRI revealed long T1 and T2 signals in the above-mentioned region (B). He received subtotal resection of craniopharyngioma through frontal extradural approach. After surgery, the visual acuity of the left eye recovered to 0.1, and adjuvant external-beam radiation therapy (55 Gy) was performed. Ten months later, MRI showed that a large amount of tumor tissue was resected (C), and that the patient could resume his duties.
infrasellar tumor, which was determined by tumor body (≥80% of tumor volume) below the sellar diaphragm and a small part of the tumor components (<20% of tumor volume) able to penetrate the sellar diaphragm and develop towards the suprasellar region. The clinical manifestation and treatment methods of these 11 cases of IC were retrospectively analyzed. An average of 22.5 months (range 9–98 months) of clinical observation and follow up examination were performed. Retrieved literature was also used to discuss this disease.
3. Results The clinical manifestation, imaging characteristics, treatment methods, and follow-up results of 11 cases are shown in Table 1. The present patients consisted of six males and five females, and averaged 28.5 years of age (range 7–52) years. Eight cases were above 15 years of age, and three cases below 15 years old. Clinical manifestations included headache (7 cases), visual disorder (5 cases), nasal obstruction (4 cases), polydipsia and polyuria (4 cases), delayed sex organ development (3 cases), menstrual disorder (1 case), and no symptom (1 case). Tumor invasion regions included sellar bottom, ethmoidal sinus, maxillary sinus, sphenoidal sinus, infrasellar region, clivus, nasopharynx and nasal cavity. Solid CP was observed in three cases, cystic CP in seven cases (single cystic 5 cases and multiple cystic 2 cases), and mixed cystic and solid type in one case (with intact cystic wall, as determined by imaging and gross observation). Diagnosis evidence included pathological findings (six cases), cholesterol crystal hydatid fluid (four cases) and imaging data (one case).
Three cases of solid tumor and one case of double cystic tumor received the extradural anterior cranial fossa approach. The solid tumors were hard and tough and showed abundant blood supply. Massive section and adjuvant external-beam radiation therapy (one tumor at 50 Gy and the remaining two at 55 Gy) were performed. Following surgery, these three solid tumor cases were followed up for 10, 28 and 88 months. Results showed that the tumors were greatly reduced. Cystic tumor patients did not receive external-beam radiation therapy. Twelve-month follow-up results showed that the cystic tumors were stable. Two cases of cystic-solid tumor received tumor resection through a transnasal approach under endoscope guidance. Radical tumor resection was performed in one case, and the tumor disappeared 10 months later. Subtotal resection followed by stereotactic interstitial radiation (radioisotope 32 P brachytherapy) was performed in the other case, and the tumor was greatly reduced 12 months later. Four cases of cystic tumor underwent stereotactic interstitial radiation (radioisotope 32 P brachytherapy). Gamma knife radiosurgery was also used in one case to treat the solid tumor part. Tumor volume was obviously reduced within the follow-up period. No obvious changes were observed in the case presenting no clinical manifestations during the 10-month follow-up period. An average of 22.5 months (9–88 months) of follow-up results is shown as follows: the tumor disappeared in one case, was obviously reduced in eight cases and was stable in two cases following imaging. Clinical symptoms disappeared in three cases and improved in seven cases. No new symptoms were observed in the case presenting no symptoms. All patients were able to resume their work, study and normal everyday activities.
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Fig. 2. Case 3: A 21-year-old female presented with a gradually worsening vague pain in the anterior frontal region and nasal obstruction over a course of 6 months. Physical examination showed bilateral rhinostenosis. Cranial CT showed the space-occupying lesion of the anterior and middle cranial fossa. The tumor invaded the entire sphenoidal sinus, ethmoidal sinus, clivus, nasopharynx and nasal cavity. The edge of the lesion tissue was clear and the peripheral sclerotin was compressed and thinned (A). MRI revealed a cystic lesion (B). She received stereotactic radioisotope 32 P brachytherapy. After surgery, all symptoms disappeared. Six months later, MRI revealed evidently reduced lesions and good pituitary gland structure (C and D).
Illustrative Cases were seen in Figs. 1–4. 4. Discussion This report is the first to report a group of IC cases, comprising 11 cases that account for 0.52% of the 2 130 cases of CP concurrently admitted. CP primarily occurs in the sella turcica and suprasellar region (90–94%), and occasionally develops towards the anterior (2–5%) and lateral (2%) region of sella turcica or posterior cranial fossa (1–4%) [4]. It rarely originates from other regions, such as the nasopharynx, sphenoid bone, third ventricle, pineal body region, lateral fissure or pons, and IC is extremely rare [5]. From then Bock first reported IC in 1924 [6], to May 2009, approximately 50 cases of IC have been reported worldwide [2,7–37]. Benitez [7] classified IC into two types according to possible origin regions: (1) a typical CP, originating in the sella turcica and developing downward to the sphenoidal sinus; (2) originating from sphenoid bone or the derivate of residual embryo craniopharyngeal canal in the nasopharynx, which originates from sphenoidal sinus or nasopharynx. The tumor epidurally grows upward but does not destroy the sella turcica. However, large tumors cannot be classified based on these criteria. Chen [8] collated 27 cases of IC reported before 2000, in which 18 cases were simply IC, and the remaining nine cases were infrasellar and suprasellar CP. In this report, there
were nine cases that were confirmed suffering from simple IC due to the classification criteria: tumor originating in or below the sellar floor, and intrasellar structure, which was demonstrated by imaging; there were two cases presenting suprasellar and infrasellar tumors, which was determined by tumor body (≥80% of tumor volume) below the sellar diaphragm and a small part of tumor components (<20% of tumor volume) able to penetrate the sellar diaphragm and develop towards the suprasellar region. In this report, male and female patients were roughly equal in proportion, and 73% (8/11) of patients were over 15 years of age, which is similar to a previous report [8]. The IC reported here is also called infrasellar adamantinoma [9,10], pharyngeal CP [11], nasopharyngeal CP [12], or sphenoid sinus CP [13]. 4.1. Embryonic development Several theories regarding the origin of CP are related to adenohypophysis during embryogenesis [14–17]. Tumors possibly originate from the residual fraction of the pharyngeal pituitary. At week 4 of embryogenesis, a Rathke’s cyst forms on the top of the stomodeum and before the oropharyngeal membrane. The cyst then extends through the sphenoid bone development region until it reaches the sella turcica, then converges with the neuroectoderm of the neurohypophysis, which develops downward from the top
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Fig. 3. Case 8: A 15-year-old female suffered from headache over a course of 3 months, as well as menstrual disorder and lactation prior to admission. MRI (A) revealed that the tumor, cystic predominant, was primarily located in the sphenoidal sinus and superior clivus. A CT scan (B) revealed that the sclerotin of the sclerotin of sphenoidal sinus lateral wall and clivus were compressed and thinned. Craniopharyngioma was resected transnasally. At 10 months after surgery, MRI (C) revealed that the tumor disappeared and showed a clear anatomical structure of sella turcica. The patient explained that her headache disappeared and menstruation recovered.
of the third ventricle of the embryonic brain, and finally differentiates into the adenohypophysis. In the path passed by Rathke’s cyst, a solid cell cord forms to connect the adenohypophysis and the stomodeum ectoderm. During later embryogenesis, this cell cord resides between two centers of chondrification, which develop into the sphenoid bone body and wings, and differentiate into a craniopharyngeal canal. This passage extracranially extends from the anterior part of the sphenoidal hypophysial fossa up to the juncture between the posterior nasal septum and the palatum. The adult pharyngeal pituitary develops from the chordal tail. 4.2. Origin theory In 1899, Mott [38] first proposed that CP possibly originated from the pharyngeal pituitary. In 1904, Erheim [39] reported that the pharyngeal pituitary resided in the posterior part of the pearshaped bone and first proposed the theory that CP originated from the craniopharyngeal canal during embryogenesis. In 1931, Carmichael [40] found that 32.7% of the craniopharyngeal canal resided in the nearby region of hypophysis during autopsy. Later, McGrath [41] confirmed that the blood of the adult functional pharyngeal pituitary came from the hypothalamic–hypophyseal portal system and transferred to the vascular bed of the pharyngeal pituitary via the sphenoid bone. CP possibly occurs in any region
along the craniopharyngeal canal and even the functional pharyngeal pituitary [43]. Therefore, tumors can develop along a pathway including the posterior part of the pear-shaped bone above the juncture of the soft palate and the nasal septum, sphenoid bone, sellar floor and up to the infrasellar regions, such as nasopharynx. There is another viewpoint that CP originates from the squamous epithelium tissue of adenohypophysis. However, squamous cell nests are rarely seen in this region of children. Therefore, it is possible that CP originate from prehypophysial adult cells rather than the squamous cell metaplasia in children. Erdheim’s theory of adenohypophyseal embryogenesis has been presently supported by several studies. In 2001, Chen [8] reported a case of CP. Imaging examination showed that both the suprasellar region and sphenoidal sinus were invaded by tumors, and successive CT scans revealed that space-occupying lesions terminated in the juncture of the soft palate and nasal septum, further confirming Erdheim’s theory. The regions involved in IC include the nasopharynx, sphenoidal sinus, ethmoidal sinus, maxillary sinus and nasal cavity, in which, the sphenoidal sinus is most vulnerable to IC. 4.3. Clinical manifestations Compared with suprasellar and intrasellar CP, IC is diversified in clinical manifestations, and its symptoms and physical signs are
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Fig. 4. Case 9: A 24-year-old man was admitted owing to 10 years of delayed development of sex organs caused by polydipsia and polyuria. He underwent craniotomy twice for partial resection of tumor. Postoperative MRI revealed an infrasellar oval cystic space-occupying lesion invaded the sphenoidal sinus and clivus. The intrasellar components were shifted upwards (A and B). Stereotactic radioisotope 32 P brachytherapy was performed. At 6 months postoperatively, MRI revealed that the reduced lesion (C), and the patient could resume his daily work.
closely related to tumor region, size, and space-occupying compression to, and influence on, peripheral structures [33]. Clinical manifestations are classified into six kinds: (1) nasopharyngeal CP clinically present frontal headache, nasal obstruction, epistaxis, space-occupying lesions in the nasopharynx and nasal cavity, therefore, cases are first admitted to the otorhinolaryngology department [5,6,13,15,33]. There were seven cases of nasopharyngeal CP in this report; (2) sphenoidal sinus CP clinically present with headache and cavernous sinus syndrome [11,13], and there were six such cases; (3) intrasellar CP clinically present with secondary prehypophysial hypofunction, such as diabetes mellitus, amenorrhea, decreased sexual desire and general body deterioration [7], and there were four such cases; (4) suprasellar CP clinically present with damaged visual acuity, including diplopia, defect of visual field (mostly bitemporal hemianopsia) and loss of sight, and there were five cases; (5) hypothalamus damage clinically present with diabetes insipidus, hypoevolutism and intelligence changing [42,43], and there were four cases; and (6) in the late period of IC, intracranial pressure increases, resulting in headache accompanied by vomiting, vertigo and an impaired cranial nerve [4,42]. There was one case presenting no symptoms in this report.
its symptoms and physical signs lack specificity. Thus when this disease is finally determined, tumor volume is relatively large. Cranial plain films, tomography and CT images are commonly used [11] because they can display nasal sinus changes, tumor range, cystic space-occupying lesions in expanded sella turcica, sclerotin corrosion and tumor calcification. CT scans [7,16,17] can detect calcified cystic solid CP tissue and can help determine whether tumors are multicystic, the cyst position and changes in intensity. Multiple calcified tissues can be observed in tumor tissue, especially in young patients, but rarely in adult patients. MRI [7,17,44] can clearly show tumor range, the solid or cystic part, enhanced scanning range and the relationship to adjacent structures. Although CT scans are more specific, owing to their ability to display calcified tissue, MRI scans can provide information on the surgical approach required by precisely measuring tumor range. Through the use of the above-mentioned imaging techniques, it is not hard to diagnose typical IC, but for the non-typical position and imaging manifestations, IC should be identified from other lesions, and a biopsy can be used if necessary. Early diagnosis of IC can help enhance therapeutic effects. 4.5. Treatment
4.4. Diagnosis Diagnosis of IC at early stages is difficult because lesioned tissue is primarily located in the anterior-middle cranial fossa and
Previous methods for treatment of IC include hydatid fluid resorption or cystic fistulation [17], which can relieve intracystic pressure and improve symptoms, however, they have a high
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relapse rate. In most cases, surgical resection is the first option. The tumor can be resected via a transcranial or transsphenoidal approach. Some rhinolaryngologists also select the transpalatal approach, transnasal septum approach, and nasal side incision approach. The transnasal septum approach in conjunction with the transsphenoidal approach, based on tumor site and range, is also used. However, total resection of the tumor is the ideal method [45], as it provides the greatest opportunity of long-term survival for patients. However, total resection of tumors can also increase mortality and mutilation rates in some patients because of wide tumor range and may involve adjacent structures. Subtotal resection in conjunction with postoperative radiology therapy would be a relatively good selection. CP is sensitive to radiology therapy, which can prevent relapsed tumors and improve patients’ survival period [46]. Evidence exists that postoperative radiology therapy can reduce tumor relapse [45–47]. Therefore, postoperative radiotherapy has become an important factor to prevent tumor relapse and improve patients’ survival. For cystic or primarily cystic CP, stereotactic interstitial radiation is a selectable effective therapeutic method [46,47]. Due to great differences in tumor size, position and the possible involvement of adjacent structures, individualized treatment should be selected. For example, stereotactic interstitial radiation should be selected for simple cystic tumors; stereotactic interstitial radiation in combination with gamma knife radiosurgery or transnasal resection of tumor under the guide of endoscope should be used for cystic and solid tumors; total or subtotal resection of tumors in conjunction with adjuvant external-beam radiation therapy should be used for solid type of tumors. Clinical observation is selected for cases who present small lesion volume and no symptoms. An average of 22.5 months of follow-up demonstrates that all cases exhibited absent or improved clinical symptoms and all patients could resume their work, study and everyday activities. Imaging results revealed that tumors disappeared in one case, reduced in eight cases and were stable in two cases. 4.6. Prognosis Compared with suprasellar and intrasellar CP, the brain tissue structures invaded by IC are not important, so the patients with IC can acquire a better prognosis. 5. Conclusion IC is extremely rare and usually occurs in the sphenoidal sinus. IC comprising solid, cystic, or cystic mixed with solid types, can occur in any region of the degenerative craniopharyngeal canal pathway, including sphenoid bone, ethmoid bone, and nasopharynx. Clinical manifestations depend on invaded structure, but headache and nasal obstruction are common, and secondary prehypophysial hypofunction, impaired visual acuity and hypothalamus damage are commonly seen. Diagnosis primarily depends on CT or MRI imaging. Individualized treatment should be selected. For example, stereotactic interstitial radiation should be selected for simple cystic tumors, stereotactic brachytherapy combination with gamma knife radiosurgery or transnasal resection of tumors for mixed tumors, total or subtotal resection of tumors in conjunction with adjuvant external-beam radiation therapy for solid type tumors and clinical observation for cases who present small lesion volume and no symptoms. IC has better prognosis than suprasellar and intrasellar CP. References [1] Bunin GR, Surawicz TS, Witman PA, Preston-Martin S, Davis F, Bruner JM. The descriptive epidemiology of craniopharyngioma. J Neurosurg 1998;89:547–51.
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