Spontaneous rupture of craniopharyngioma cysts. A report of five cases and review of the literature

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Spontaneous Rupture of Craniopharyngioma Cysts . A Report of Five Cases and Review of the Literature

Hideki Satoh, M .D ., Tohru Uozumi, M .D ., Kazunori Arita, M .D ., Kaoru Kurisu, M .D ., Takuhiro Hotta, M .D ., Katsuzo Kiya, M .D ., Fusao Ikawa, M .D ., Junji Goishi, M .D ., and Takashi Sogabe, M .D . Department of Neurosurgery, Hiroshima University School of Medicine and Department of Neurosurgery, Matsuyama Red Cross Hospital, Japan

Satoh H, Uozumi T, Arita K, Kurisu K, Hotta T, Kiya K, Ikawa F, Goishi J, Sogabe T . Spontaneous Rupture of Craniopharyngioma Cysts. A Report of Five Cases and Review of the Literature . Surg Neurol 1993 ;40 :414-9 .

gioma cyst as well as diagnostic considerations . The relevant literature is reviewed .

Reports of spontaneous rupture of a craniopharyngioma cyst are extremely rare. Five cases of spontaneous rupture of a craniopharyngioma cysts are reported . Clinical symptoms included chemical meningitis in three patients, alleviation of headache in one, and improvement in a visual disturbance in one . Reduction in cyst size was confirmed by computed tomography or magnetic resonance imaging in three of five patients, and the histopathological diagnosis was confirmed histologically in four patients . Cerebrospinal fluid findings were abnormal in the three patients with chemical meningitis . Spontaneous rupture of craniopharyngioma cysts tended to occur more frequently in adult males . Computed tomography and magnetic resonance imaging were useful in diagnosing cyst rupture, and cerebrospinal fluid findings, especially the presence of cholesterol crystals and an elevated cholesterol concentration, are suggestive, even when no reduction in cyst size is observed radiologically .

Patients and Methods

KEY WORDS : Cerebrospinal fluid ; Computed tomography ; Craniopharyngioma ; Magnetic resonance imaging ; Meningitis

Spontaneous rupture of a craniopharyngioma cyst is reported to be rare . Only ten cases, one of which was our own [8], have been described . In all cases, the diagnosis was based on a reduction in cyst size . We now have treated five patients with spontaneous rupture of a craniopharyngioma cyst . This report discusses the clinical presentation of spontaneous rupture of a craniopharyn-

Address reprint requests to.' Hideki Satoh, M .D., Department of Neurosurgery, Hiroshima Prefectural Hiroshima Hospital, 1-5-54 Ujina-Kanda, Minami-ku, Hiroshima, 734, Japan. Received December 29, 1992 ; accepted March 8, 1993 . C 1993 by Elsevier Science Publishing Co ., Inc .

The patients included four males and one female, aged 17 to 64 years, who were suspected of having a craniopharyngioma cyst in the suprasellar region . All underwent lumbar puncture and computed tomography (CT) or magnetic resonance imaging (MRI) (Table 1) . Case I has been reported previously . In Case 2, the patient was referred to our hospital following rupture of the cyst. The only abnormality on CT was calcification in the suprasellar region . The diagnosis of spontaneous rupture of a craniopharyngioma cyst was based on the presence of cholesterol crystals in the cerebrospinal fluid (CSF) . The clinical presentation varied . Three patients developed chemical meningitis (Cases 2, 3, and 5), one experienced alleviation of headache (Case 1), and one patient noted improvement in her visual disturbance (Case 4) . The CSF contained cholesterol crystals in two cases (Cases 1 and 2), and the cholesterol concentration was increased in one patient (Case 5) . The CSF protein concentration and cell count were increased in the three patients with chemical meningitis but were within normal limits in the other two (Table 1) . CT or MRI was performed in four patients both prior to and following rupture . Reduction in the size of the cyst was confirmed by CT in two patients (Cases I and 3) and by MRI in one (Case 4) . No reduction in cyst size was detected by CT in Case 5, even immediately following rupture .

Illustrative Case Reports Case 3 A 36-year-old man developed headache and had been treated by his family doctor since early June 1985 . He 0090.3019/93/56.00



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4 15

Table 1 . Demographics and Clinical Data from Five Patients with Spontaneous Rupture of a Craniopharyngiomatous Cyst CSF findings Cholesterol crystals

Proteins (mgldL)

Cells (cells/mms)

Decrease in cyst size

Alleviation of headache

+

Normal

Normal

25/M 36/M

Headache, nausea Fever, neck stiffness

+

400 93

35 3070

4

64/F

Normal

Normal

5

54/M

Improvement in visual disturbance High fever, consciousness disturbance

+ (CT) + (CT) + (MRI) (CT)

Case no.

Age (yrs)/sex

1

17/M

2 3

Presenting signs and symptoms

(turbid)

(cholesterol concentration : 39mg/dL)

was referred to our hospital because his headache became worse, he began vomiting and became disoriented on September 5 . On physical examination, the patient was mildly disoriented . His visual fields were normal, but both optic discs were choked . No other neurologic abnormalities were detected . Routine biochemical studies were unremarkable . A craniogram was nondiagnostic, but a CT scan demonstrated a slightly low density mass spreading from the suprasellar region to the third ventricle with evidence of hydrocephalus (Figure 1 A) . Angiography showed unwinding of the anterior cerebral artery . Pituitary function studies were within normal limits . The patient became febrile on the 10th hospital day, exhibiting nuchal rigidity and Kernig's sign . A lumbar puncture yielded an opening pressure of 250 mmH,O and turbid CSF containing 3070 cells/mm 3 (neutrophils 75% and lymphocytes 25%), protein 93 mg/dL, glucose 45 mg/dL, and no cholesterol crystals . Bacteriologic culture of the CSF was negative . Repeat CT confirmed a reduction in cyst size and less severe hydrocephalus (Figure 1 B) . Chemical meningitis due to rupture of the cyst was suspected and corticosteroid therapy was started . Following resolution of symptoms and normalization of the CSF, a right frontal-temporal craniotomy with partial resection of the tumor was performed 23 days following rupture . Histopathological examination revealed a typical adamantinomatous craniopharyngioma . Local radiation with 50 Gy was administered postoperatively, and the patient was discharged in good condition .

Case 4 A 64-year-old woman presented with a visual disturbance in early June 1989 . Cataracts were diagnosed, and she underwent surgery but did not experience any improvement postoperatively . CT demonstrated a suprasellar lesion, and she was referred to our hospital . Physical examination was unremarkable except that visual acuity was 0 .3 on the right and 0 .08 on the left,

855

54

Histology Adamantinomarous Adamantinomarous Adamanrlnomarous Squamous cell

and a left upper quadrant anopsia was noted . The optic discs were atrophic bilaterally . Pituitary function and CSF examination were normal . A second CT scan demonstrated a cyst with partial wall calcification in the suprasellar region . MRI of the brain showed a low intensity signal on T,-weighted image (Figure 2 A) . Visual acuity improved suddenly following hospital admission . No signs of meningitis were observed, and repeat CSF examination was normal . MRI indicated shrinkage of the cyst (Figure 2 B) . Cisternography following CT did not demonstrate reflux of contrast into the cyst . The patient was initially managed conservatively, but subsequent cyst enlargement necessitated a partial resection of the tumor . Histology demonstrated a typical adamantinomatous craniopharyngioma . Postoperative radiation, 50 Gy, was administered .

Case 5 A 54-year-old man noted reduced libido beginning in 1982 and reduced right visual acuity beginning in 1984 . He had visited a local clinic one month prior to admission, complaining of headache and a gait disturbance . A suprasellar mass and hydrocephalus were detected by CT, and the patient was hospitalized . On physical examination, pubic and axillary hair were absent ; his skin was pale and dry, and the patient was disoriented . He showed little volition and was apathetic . Visual acuity was limited to perception of hand movement on the right and was 0 .8 on the left . Bitemporal hemianopsia was detected ; the optic discs were choked bilaterally . A mild left hemiparesis also was noted . Endocrinologically, baseline hormone concentrations were decreased : ACTH, 10 pg/ mL ; GH, 0 .2 ng/mL, and cortisol, 2 .4 µg/dL. Insulininduced hypoglycemia showed low cortisol and GH responses, suggesting hypopituitarism. Both CT and MRI showed a mass with small cysts and a parenchymatous portion that exhibited marked heterogeneous enhancement (Figure 3 A) . The patient suddenly developed a



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Satoh et al

B A Figure 1 . Radiographic documentation of spontaneous rupture ofa craniopharyngioma cyst by CT. (A) Initially, a large, slightly low-density cystic lesion (arrow is seen extending from the suprasellar region to the third ventricle . Note the presence of ventricular enlargement . (B) Postrupture, the cystic lesion has decreased in size and ventricular enlargement has resolved,

high fever and a further disturbance in consciousness . Repeat CT did not reveal any reduction in the size of the mass (Figure 3 B) . A lumbar puncture yielded thin, bloody CSF with an elevated cholesterol concentration, 39 mg/dL . Following resolution of the patient's meningitis, a combined approach, transphenoid for the intrasellar tumor and transcranial for the suprasellar tumor, was performed . Radiation therapy, 60 Gy, was administered for residual tumor . The final histologic diagnosis was squamous cell craniopharyngioma . Discussion Since the first desciption by Worster-Drought et al [151, ten cases of spontaneous rupture of craniopharyngioma cysts have been reported (Table 2) [2,4-6,9,11,12,14] . In four cases, the histologic diagnosis was made at au-

topsy or was unavailable because the patient did not undergo resection . Only three cases were documented to have experienced a reduction in cyst size by diagnostic imaging [2,12,14) . The mean age of these patients was 41 .6 years (range, 16 to 73 years), and seven of the ten were male . This is consistent with data from this series . The incidence of cystic craniopharyngioma is highest from ages 5 to 9, and 38 to 510 of patients are younger than 20 years old . The incidence is only slightly higher in males (M : F = 1,2 : 1) [1,13] . Thus, spontaneous rupture of craniopharyngioma cysts appears to occur in a specific population : adult men . No history of trauma was elicited in any patient in out series, although Russell and Pennybacker [11] have reported three cases of traumatic cyst rupture . We believe all our cases represent spontaneous rutpure . Chemical meningitis occurs in many cases with reexpansion of the cyst following shrinkage . Therefore, spontaneous absorption of the cyst contents is believed unlikely . It appears that the cyst wall becomes weak as the cyst expands, resulting in degenerative changes in the cyst wall [101 . However, no site of cyst rupture has ever been confirmed .



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A

Figure 2 . Radiographic documentation of spontaneous rupture ofa craniopharyngioma by MR1 . (A) A 0 .1 tesla, T,-weighted (inversion recovery) magnetic resonance image in the coronal plane demonstrates a suprasellar hypointense mass (arrow) . The third ventricle is deformed by extrinsic compression from the lower right side. (B) Postrupture, 0.5 tesla, . T,-weighted (spin echo) coronal MRI demonstrates a decrease in cyst size (arrow . The third ventricle is no longer compressed.

The symptoms and signs of cyst rupture included lessening intensity of a headache, chemical meningitis, and diminution of visual disturbance . The presence of chemical meningitis was variable . In previous reports, chemical meningitis was the dominant presentation (9 of 10 cases) . The only exception was a patient whose disturbance in consciousness cleared when cyst rupture relieved his obstructive hydrocephalus [14) . Prior to CT and MRI, diagnosing cyst rutpure and confirming cyst shrinkage when chemical meningitis was not present was extremely difficult . This may explain the paucity of reports on cyst rupture without chemical meningitis . CSF collected following cyst rupture contained cholesterol crystals in two of the five patients ; another patient had an elevated cholesterol concentration .

417

B

Worster-Drought et al [15] reported fat globules in the CSF, and Patrick et al [9] also described fat droplets and an elevated cholesterol concentration (175 mgldL) . Because cholesterol crystals and cholesterol are characteristics of craniopharyngioma, their presence in the CSF carries considerable diagnostic significance . If rupture of a craniopharyngioma cyst is suspected, the CSF cholesterol concentration should be measured, even when cholesterol crystals are not observed . Chemical meningitis may be associated with elevations of the CSF cell count and protein concentration during the acute phase . The cells in the CSF may be lymphocyte-dominent or polynucleocyte-dominant . Concentrations of glucose and C1 - are decreased slightly . The natural history of chemical meningitis may resemble tuberculous or fungal meningitis . Cholesterol crystals in the subarachnoid space can induce chemical meningitis . Most reported cases of meningitis due to rutpure of craniopharyngioma cysts were based on the association between cholesterol in the CSF and clinical symptoms . In our series, only three of the five patients developed chemical meningitis . Tokiwa et



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A B Figure 3 . Failure to detect any change in size of a craniopharyngioma cyst following rupture. (A) At diagnosis, CT shows a suprasellar isodense mass containing small cysts . (B) Computed tomography following rupture showing no change in the size of the mass .

Table 2 . Case Reports

of Spontaneous

Rupture of Craniopharyngioma Cysts CSF findings

Reference Worster-Drought et al [15] Russell and Pennybacker [11] Kaemmerer [2] Patrick et al (9]

Age (yrs)Isez

Symptoms

19/F

Chemical meningitis

67/F

Chemical meningitis

46/M 21/M

Chemical meningitis Chemical meningitis

Cholesterol crystals Far globules

Protein (mg/dL)

Decrease in cyst size

Histologic confirmation

642

Not done

Autopsy

500

Not done

Autopsy

189 7500

228 23700

CAG Not done

Not done Not done

280 520 1420

107 252 400

Not done Not done Not done

Operation Operation Operation

CT CT

Operation Operation

Not done

Operation

160 Not reported

Ft droplets

Cells (cells/mm')

(cholesterol concentration : 175 mg/dL) Kuzuhara et al [5] Lloyd [6]

42/M 16/M 29/F

Chemical meningitis Chemical meningitis Chemical meningitis

Scully et al [12] Tokiwa et al [14]

60/M 43/M

Krueger and Larson [4]

73/M

Chemical meningitis Improvement in level of consciosuness Chemical meningitis

(turbid)

Abbreviations : CAG, carotid angiography.

280 Normal

104 Normal

Normal

450



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al [14] have noted that the development of chemical meningitis is dependent on the amount of cholesterol dispersed into the subarachnoid space . Lunardi et al [7] have reviewed 35 cases of chemical meningitis caused by rupture of intracranial cysts . They found that congenital brain tumors, such as a cystic dermoid or epidermoid tumor, and malignant paraventricular cysts accounted for most cases . Only five cases were caused by craniopharyngioma . Interestingly, the reported incidence of chemical meningitis following surgery for epidermoid cysts is only 1 .6% {31-Thus, the frequency of chemical meningitis caused by rutpure of a cyst may be less than expected . The meninges themselves exhibit little inflammation around the cyst [151 . This finding differs markedly from typical infectious meningitis, and inflammatory cells associated with cyst rupture probably represent a nonspecific reaction to tumor cells . The widespread use of CT and MRI make it likely that cases of cyst rupture will be recognized more frequently . Depending on the composition of the cyst fluid, asymptomatic cyst rupture may be identified .

Conclusions Five cases of spontaneous rupture of a craniopharyngioma cyst are reported and the literature reviewed . Spontaneous rupture is more frequent in adult males, and it presents variously, ranging from chemical meningitis to improvement in neurologic abnormalities . The presence of cholesterol in the CSF strongly suggests the diagnosis . However, diagnostic imaging techniques are invaluable .

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