Amenorrhea-galactorrhea and craniopharyngioma

A 40-year-old woman had a 1-year history of amenorrhea-galactorrhea. She was found to have an intrasellar tumor with suprasellar extension and a prolactin level of 98.1 ng/mL. Subtotal resection of the tumor resulted in a return of the prolactin level to normal within 4 days of the operation and of the prolactin response to thyrotropin releasing hormone, accompanied by the disappearance of the galactorrhea. Histologic examination revealed a typical craniopharyngioma. These observations indicate that one important mechanism of hyperprolactinemia in patients with craniopharyngioma is direct encroachment on the hypothalamus and/or pituitary stalk by the tumor and interruption of prolactin inhibiting factor.     

Moyamoya disease associated with craniopharyngioma

A case of coexisting moyamoya disease and craniopharyngioma in an 8-year-old boy is reported. The patient had been suffering from recurrence of transient right hemiparesis with headache and vomiting during the three years before admission. After the diagnosis of both moyamoya disease and craniopharyngioma, evacuation of the cystic fluid of the craniopharyngioma, encephalomyosynangiosis, and internal irradiation through an Ommaya drainage system were carried out. The symptoms gradually improved after these procedures. The relation of moyamoya disease to craniopharyngioma is discussed. We consider that internal irradiation is one of the best means of treatment for a cystic craniopharyngioma associated with moyamoya disease.     

Ciliated craniopharyngioma

A tumor of Rathke's cleft origin was studied, which showed some histologic features consistent with craniopharyngioma, but also had ciliated and mucin-containing cells more commonly observed in Rathke's cleft cysts. The combination of features provides further evidence that craniopharyngiomas and Rathke's cleft cysts have a common embryonic origin.     

Surgical experience with craniopharyngioma in adults

It is proposed that the term craniopharyngioma of the adult be employed only for tumors presenting after the successful completion of puberty. The contrasts with the current practice of arbitrary distinction on the basis of age at the time of operation. Of 48 patients over 20 years of age at the time of operation, 38 fulfilled the criteria for "craniopharyngioma of the adult". Clinical characteristics of these 38 are analyzed. In contrast to generally accepted ideas, the course of patients with incompletely excised tumors is unfavorable, with frequent early and serious recurrences. A complete excision is therefore recommended for craniopharyngioma of the adult.     

Infrasellar craniopharyngioma

A case of infrasellar craniopharyngioma is reported. This must be the first report that presents computed tomographic findings of an infrasellar craniopharyngioma. A computed tomography scan demonstrated the characteristic features of a tumor at the base of the skull extending massively into the nasopharynx, prepontine cistern, and suprasellar region. Further, air-contrast computed tomographic cisternography suggested the extradural growth of the tumor. We confirmed these neuroradiologic findings at a transsphenoidal operation.     

Diencephalic syndrome of emaciation in an adult associated with a third ventricle intrinsic craniopharyngioma: case report

OBJECTIVE AND IMPORTANCE: Diencephalic syndrome of emaciation (DS) is seen almost exclusively in infants and young children, and only two cases of DS in adults have been reported previously. We describe a case of DS associated with a third ventricle intrinsic craniopharyngioma in an adult patient. CLINICAL PRESENTATION: A 54-year-old man presented with profound emaciation, disorientation, memory loss, and psychological disorders. Computed tomographic scanning and magnetic resonance imaging of his brain disclosed a tumor within the third ventricle. The preoperative endocrinological examination indicated an elevated growth hormone level and a decreased somatomedin C level. INTERVENTION: The patient underwent partial resection of the tumor, which was adherent to the floor of the third ventricle but not to the ventricle wall, by an interhemispheric-transcallosal approach. The histological examination revealed a squamous papillary-type craniopharyngioma. The patient received 50 Gy of radiotherapy, which resulted in 90 to 95% reduction of the tumor size within 6 months, as indicated by the magnetic resonance images presented. The psychological disorders, memory disturbance, and severe emaciation improved gradually thereafter. CONCLUSION: Although extremely rare, DS can occur in an adult harboring a tumor in the anterior hypothalamus.     

A case of prolactinoma associated with craniopharyngioma

A surgical case of prolactinoma associated with craniopharyngioma is reported. A 47-year-old man was admitted to some neurosurgical clinic on October 12, 1982, because of visual disturbance, general fatigue and impotence. Laboratory study revealed hyperprolactinemia (360 ng/ml) and slight enlargement of sella turcica indicated the pituitary adenoma. Transsphenoidal surgery was performed to remove the tumor on November 20, 1982. Histopathological examination revealed chromophobe adenoma, and prolactin was stained in the tumor cells by means of immunoperoxidase staining. Though the clinical symptoms had been improved after surgery, visual disturbance became worse about one month later. At that time empty sella syndrome was suspected and the second operation (interhemispheric approach) was performed on January 21, 1983. No pathological changes were observed at all. On July 13, 1983, he was transferred to our clinic, because his visual acuity was deteriorating. At this time we reviewed the previous CT scan and noticed a suprasellar mass. It was supposed that the lesion had been overlooked and was the cause of the visual disturbance. On August 1, 1983, a bifrontal craniotomy was performed and the suprasellar tumor was removed. Pathological examination of the tumor revealed craniopharyngioma. So it was supposed that pituitary adenoma and craniopharyngioma had been coexisting since onset. Except for cases with von Recklinghausen's disease, multiple primary intracranial tumors of different cell types are relatively rare. A review of literature revealed 94 cases until 1986. The most frequent combination of multiple tumors was meningioma and glioma. But we could not find any case of pituitary adenoma associated with craniopharyngioma in the literature.(ABSTRACT TRUNCATED AT 250 WORDS)     

A case of craniopharyngioma with intratumoral hemorrhage

A patient of craniopharyngioma associated with intratumoral bleeding was reported. A 44-year-old female was admitted to our department with sudden onset of headache suggesting pituitary apoplexy. On admission, she complained of mild bifrontalgia and neurological examination revealed no abnormality except bitemporal hemianopsia. Hormonal examination indicated hypopituitarism with masked diabetes insipidus and chronic thyroiditis. MRI showed a cystic 12 x 10 x 10 mm tumor with an enhanced rim at the intra- and suprasellar region. The MRI findings of the cyst suggested intratumoral bleeding with various stages. CT and plain tomography revealed plane calcification adjusting or aligning itself to the floor of the sellar. This calcification was difficult to distinguish from the sellar floor on MRI. Under the diagnosis of craniopharyngioma, almost all of the tumor was removed, using the transsphenoidal approach. The cyst consisted of old hematoma and cholesterin. The histological examination revealed a stratified squamous epithelia with numerous immature blood vessels. Such findings are unusual in cases of craniopharyngioma. Craniopharyngioma is rarely associated with intratumoral hemorrhage. The mechanism of this hemorrhage was discussed with reference to the literature on this subject.     

Recurrent ectopic craniopharyngioma

A 66-year-old woman developed an asymptomatic mass in the right frontal lobe 5 years after undergoing a right frontal craniotomy and removal of a craniopharyngioma. The mass progressively enlarged over the next 3 years, during which time it became multiloculated and partially cystic. Repeat craniotomy was performed 8 years after the original operation, at which time the mass was found to be an ectopic craniopharyngioma. The lesion probably resulted from seeding of tumour cells along the surgical tract at the time of the initial surgery.     

Ectopic clival craniopharyngioma

Summary.  Ectopic craniopharyngioma is a rare entity. The authors present a very rare case of an ectopic clival craniopharyngioma completely separate from the sella turcica.  A 44-year old woman presented with abducens palsy. A MR imaging study and a CT scan revealed a cystic clival lesion separate from the sella turcica. Surgical resection was performed successfully with flexible endoscope-assisted procedure using an endonasal transsphenoidal approach. No evidence of involvement of the sellar region was found according to radiological, intra-operative, and clinical findings.  A review of the literature revealed no other such cases. The discussion includes the formation of craniopharyngioma from the ectopic Rathke's pouch remnants and the surgical approach for clival lesions. We believe that our approach provides good results with minimal invasiveness for some clival lesions. Published online October 31, 2002 Correspondence: Takakazu Kawamata, M.D., Ph.D., Department of Neurosurgery, Neurological Institute, Tokyo Women's Medical University, 8-1 Kawada-Cho, Shinjuku-Ku, Tokyo 162-8666, Japan.     

Primary cerebellopontine angle craniopharyngioma in a patient with gardner syndrome. Case report and review of the literature

The authors report on the case of a craniopharyngioma arising in the cerebellopontine angle (CPA) in a patient with Gardner syndrome. Although familial adenomatous polyposis (FAP) is associated with intracranial neoplasms, the current case is only the third reported craniopharyngioma in a patient with Gardner syndrome. Two of these tumors, including that of the current case, originated in the CPA, an unusual location for craniopharyngiomas. The literature concerning FAP and its associations with intracranial neoplasia, as well as the pathogenesis of craniopharyngiomas in the posterior fossa, is discussed.     

De novo adamantinomatous craniopharyngioma with long-term pre-diagnostic imaging

Adamantinomatous craniopharyngioma has a bimodal age distribution occurring in children aged 5–15 years and less frequently in adults aged 45–60 years. The current embryogenetic hypothesis suggests that adamantinomatous craniopharyngioma (ACP) arises from epithelial remnants of the craniopharyngeal duct or Rathke's pouch. It is thought that this tumor exists early on during childhood but remains indolent, growing very slowly until it is diagnosed incidentally or due to symptoms. Recent reports of de novo development of ACP, however, have challenged this theory. Herein, we present a case of an incidentally discovered de novo adamantinomatous craniopharyngioma that was documented to arise de novo on serial MRIs performed for a different indication. To our knowledge, this is the first report of a middle-aged patient who is diagnosed with a de novo ACP documented with contrast-enhanced MRIs of the sella over a 16-year period. This case challenges our current understanding of the pathophysiology of adamantinomatous craniopharyngioma.     

Primary ectopic frontotemporal craniopharyngioma

IntroductionPrimary ectopic craniopharyngiomas have only rarely been reported. Craniopharyngiomas involve usually the sellar and suprasellar region, but can be originated from cell remnants of the obliterated craniopharyngeal duct or metaplastic change of andenohypophyseal cells. We present the first case of a primary ectopic frontotemporal craniopharyngioma.Presentation of caseA 35-year old woman presented with a one-year history of headache and diplopia. MRI showed a large frontotemporal cystic lesion. Tumor resection was performed with a keyhole endoscopic frontal lateral approach. The pathological features showed an adamantinomatous craniopharyngioma with a cholesterol granuloma reaction.DiscussionThere have been reported different localizations for primary ectopic craniopharyngioma. Our case presented a lobulated frontotemporal cystic mass formed by a dense eosinophilic proteinaceous material dystrophic calcifications and cholesterol crystals, with epithelial remnants. No tumor regrowth was observed in the magnetic resonance image 27 months postoperatively.ConclusionPrimary ectopic craniopharyngioma is a rare entity with a pathogenesis that remains uncertain. This is an unusual anatomic location associated with unique clinical findings.     

Recurrent infrasellar clival craniopharyngioma

Summary The patient is a 44-year-old man who underwent resection of a posterior nasopharynx tumor 12 years earlier via left lateral rhinotomy approach. The final pathological analysis indicated the tumor was a craniopharyngioma, and the patient subsequently underwent focal radiation. The patient returned to medical attention complaining of dysequilibrium. A neurologic exam was nonfocal. Magnetic resonance imaging revealed a clival mass, separate from the sella turcica, with imaging characteristics concerning for chordoma or primary bone tumor (Fig. 1). The lesion was resected via an endoscope-assisted endonasal transsphenoidal approach, with gross total resection achieved. Intraoperatively, the mass was noted to erode through the posterior nasopharynx, without extension superiorly into the sella or posteriorly through the clival dura (i.e., lesion was infrasellar). The final pathological results indicated the tumor was adamantinomatous craniopharyngioma.     

Ectopic recurrence of craniopharyngioma

A 27-year-old woman presented with ectopic recurrence manifesting as an asymptomatic mass in the left frontal convexity 5 years after subtotal removal of suprasellar craniopharyngioma through a right orbitozygomatic craniotomy. The mass had enlarged gradually over the next 5 years, so a second operation was performed for total removal. The histological diagnoses of both lesions were adamantinomatous type craniopharyngioma with MIB-1 indexes of 4.2% and 7.4%. The second lesion probably resulted from dissemination. Craniopharyngioma is a benign tumor, and ectopic recurrence is rare. Long-term clinical and neuroimaging follow up is recommended.     

Treatment of recurrent craniopharyngioma

Treatment results on 48 patients with "recurrent" craniopharyngioma treated by surgery or/and radiation are analyzed. Median relapse-free survival time was 43.6 months in patients treated initially with radiation and 22.2 months without. Operative death occurred in 17% of all patients and in 3 out of six patients after total removal. The five- and ten-year survival rates were 91.7% and 66.8%, respectively, for 14 patients treated with combined surgery and radiation therapy. For 26 patients treated with surgery, the survival rates were 20.3% and 10.1%. All of 6 patients, who had received both initial and later radiotherapy, were well 1/2 to 18 years later without clinical evidence of radiation injury. These results lead us to the following conclusions: 1) A radical surgery in recurrent cases has the higher risks of mortality and morbidity than that of the first radical surgery. 2) Radiation therapy improved the survival rate of patients with "recurrent" craniopharyngioma. 3) After initial radiation therapy, additional irradiation was allowed based on the scale of nominal standard dosage and the estimation of "decay factor".     

Malignant transformation in craniopharyngioma

Malignant transformation in a craniopharyngioma has not been described previously. A 49-year-old woman presented with recurrence of a suprasellar craniopharyngioma diagnosed 35 years previously. The patient had been treated surgically for recurrence on five occasions. Radiation therapy had been administered 7 years before the final presentation. Tissue obtained from the fifth operation revealed malignant degeneration in a typical craniopharyngioma.