A case of metastatic suprasellar tumor with edema along the optic pathway

A rare case in presented of a patient with edema formation along the optic pathway, which was caused by metastatic suprasellar tumor mimicking craniopharyngioma. A 63-year-old female with visual disturbance, diabetes insipidus, and symptomatic panhypopituitarism was admitted to our hospital. CT images showed a suprasellar mass lesion. MR images demonstrated a heterogeneously enhanced tumor and edema along the optic nerve and tract. Although no cystic component is shown in radiological findings, these features are commonly observed in cases of craniopharyngioma. The patient underwent removal of the tumor by the transcranial approach. Since histological examination revealed adenocarcinoma, the tumor was diagnosed as metastatic brain tumor originating from non-recurrent rectal cancer operated on eight years previously. Conventional radiotherapy with a dose of 50GY was performed for the residual tumor. The tumor size was unchanged, but the edema had disappeared 7 months after the operation. Edema spreading along the optic tract was reported as a characteristic MR finding for diagnosis of craniopharyngiomas as distinguished from other common parasellar tumors. However, it is suggested that edema of the optic pathway seems to be caused not only by craniopharyngioma but also other suprasellar lesions. Even if there was a characteristic radiological finding for some specific diseases, metastasis should be kept in mind as a differential diagnosis, because of its various appearance in neuroimages.     

Cavernous malformation of the optic chiasm: case report

We report a 14-year-old boy with cavernous malformation of the optic chiasm. He had a 2-year history of gradually worsening visual disturbance. Computed tomography (CT) and magnetic resonance imaging (MRI) revealed a suprasellar mass, findings compatible with craniopharyngioma. The mass was biopsied and histological examination confirmed cavernous malformation. On the second day after the biopsy, he suffered chiasmal apoplexy due to intratumoural haemorrhage, lost visual acuity and developed a field cut. Cavernous malformations arising from the optic nerve and chiasm are extremely rare; only 29 cases have been reported to date. Most patients manifested acute visual acuity and visual field disturbances. Although MRI findings of cavernous malformations in the brain parenchyma have been reported, MRI findings on the optic nerve and chiasm may not be completely diagnostic. Of the 29 documented patients, 16 underwent total resection of the lesion without exacerbation of their preoperative symptoms; in some cases, resection was complicated by risk of damage to the surrounding neural tissue. As patients may suffer intratumoural haemorrhage after biopsy or partial removal of the lesion, the advisability of surgical treatment of cavernous malformations of the optic nerve and chiasm must be considered carefully.     

An elderly case of craniopharyngioma: treatment by limited surgery and radiation therapy

Craniopharyngioma accounts for approximately 3.5% of all intracranial neoplasm and may occur at any age. However craniopharyngioma in the elderly was rare. We report a rare case of craniopharyngioma in an elderly patient, who was treated by radical surgery successfully. An 80-year-old male was admitted to our hospital with a history of visual disturbance and bitemporal hemianopsia. CT scan of the brain revealed a hypodense cystic mass with calcification in the suprasellar area. MRI demonstrated the optic chiasm was displaced superiorly. Her symptom deteriorated day short time of her hospitalized life. We decided to removal tumor radically because of saving her visual function. Subtotal removal of the suprasellar lesion was performed, followed by radiotherapy to the residual lesion. A visual impairment was improved after surgery. No recurrences had been detected on MRI eighteen months after the initial operation. Based on previous report and the clinical course of our case, radical surgery is sometimes needed for saving visual function in spite of elderly patient. However, in this case, sparing hypothalamic and pituitary function is essential for good prognosis.     

Stereotactic radiosurgery. XVII: Recurrent intrasellar craniopharyngioma

Stereotactic radiosurgery for craniopharyngioma is usually a high risk procedure due to the intimate relationship of the tumour to the optic chiasm and conservative dosing has been advocated to reduce complication rates. In 2002, in a publication from Karolinska Hospital, Sweden, 13 out of 21 patients received only a marginal dose of 6 Gy (not considered a radical dose) and 11 out of 13 tumours progressed. This recent report must argue against single dose stereotactic radiosurgery as the primary radiation therapy modality in most cases. However, where the disease is 'away' from the optic apparatus, such constraints do not apply. We here report the successful treatment of three consecutive patients whose craniopharyngioma was confined to the pituitary fossa, and a finite distance from the optic pathways and in whom optimal dosing was employed.     

An elderly case of recurrent craniopharyngioma suffering from hemorrhage

A case of a 63-year-old female with craniopharyngioma is reported. She first suffered from occult pituitary apoplexy and had recurrent enlargement and characteristic changes of a tumor during short periods. This patient was hospitalized after suddenly developing bitemporal hemianopsia. An intra-and suprasellar hematoma was revealed on computed tomography (CT) and magnetic resonance imaging (MRI). At the first operation, the hematoma was removed totally by the transsphenoidal approach, but tumor tissues were not identified. During the following 12 months, operations were repeated three times due to the recurrence and/or enlargement of a tumor associated with visual symptoms. Pathological diagnosis was squamous-type craniopharyngioma without any malignancy. Microscopic appearance of the tumor apparently changed during the clinical course. The characteristic findings were revealed respectively on MRI and CT. On the first preoperative MRI, a lesion of diffuse high signal intensity was observed on both T1 and T2 weighted images. At the second operation, the lesion was also revealed as having high signal intensity on T1 and T2 weighted images but the tumor had a large cyst with serous-yellowish fluid contents. A differential diagnosis was made on CT. At the third and fourth operations, the tumor was solid and had atheromatous contents. The lesion was revealed as having low signal intensity on T1 image and high on T2 respectively. Occult pituitary apoplexy with intra and suprasellar hemorrhage is very rare in cases of craniopharyngioma and only nine cases have been reported until now. It is also interesting with this high-aged patient that repeated recurrence and/or enlargement of a tumor with different microscopic appearances occurred during such short periods.(ABSTRACT TRUNCATED AT 250 WORDS)     

The anterior pituitary lobe in patients with cystic craniopharyngiomas: Three cases of associated lymphocytic hypophysitis

Summary Specimens of the anterior pituitary lobe were investigated histologically in 28 craniopharyngioma patients operated on transsphenoidally. The pituitary glands in 3 patients revealed lymphocytic invasion giving a histological appearance typical of lymphocytic hypophysitis (incidence: 11%). At follow-up examination all three patients with associated lymphocytic hypophysitis had complete pituitary insufficiency, whereas only 36% of the craniopharyngioma patients without associated lymphocytic hypophysitis were in this poor postoperative endocrine state.The phenomenon of associated lymphocytic hypophysitis in craniopharyngioma patients has not been reported so far. This might be due to the fact that investigators have failed to systematically examine the anterior pituitary lobe in craniopharyngioma patients. The 60 cases of lymphocytic hypophysitis reported in the literature occurred, for the most part, in women during late pregnancy or shortly after delivery. An auto-immune origin is assumed in this type of inflammation. In contrast to this pathophysiological mechanism, we assume a local induction of inflammation resulting from the craniopharyngioma cyst in our 3 patients.Dedicated to Prof. Dr. H.-D. Herrmann on the occasion of his 60th birthday.     

Pituitary hyposecretion and hypersecretion produced by a Rathke's cleft cyst presenting as a noncystic hypothalamic mass

A 53-year-old man presented with polydipsia, polyuria, lightheadedness on standing, and syncope. Visual field deficits suggesting left optic tract compression and pituitary dysfunction consisting of diabetes insipidus, hyperprolactinemia, and anterior pituitary insufficiency were diagnosed. On the computed tomography scan, an isodense, ring-enhancing, parasellar mass was localized primarily in the suprasellar region and also extended into the sella. It was believed that the pituitary hormone hypersecretion and hyposecretion were due to hypothalamic dysfunction from the mass which was initially believed to be a craniopharyngioma. At surgery a Rathke's cleft cyst was resected. The unusual presentation of this Rathke's cleft cyst presenting as a hypothalamic lesion is discussed.     

Postoperative intracranial seeding of craniopharyngioma. Three case reports and a review of the literature

Metastasis of craniopharyngioma is uncommon. Only 10 cases have been reported in the literature. In this report, we describe 3 patients who presented metastases following operative treatment of suprasellar craniopharyngioma. All 3 patients (ages: 32, 11, and 9 years) underwent radical excision of a supradiaphragmatic, retrochiasmatic craniopharyngioma by the right frontopterional approach. Resection was considered as total in all cases. All patients required hormone replacement therapy. Local recurrence was observed in only one case. Metastasis occurred along the surgical route in all three patients. The interval between surgery and metastasis was 5 and 3 years in the first two cases. The third patient presented two metastases: one in the temporal space at 3 years and another in the frontal space at 10 years. Repeat surgery was performed in 2 patients. The first case involved a lesion located in the right frontal space, and the second involved local recurrence and metastasis along the surgical route. The third patient was treated by puncture and radiation therapy for the temporal lesion and surveillance for the frontal lesions. No recurrence has been observed at 2 and 7 years after re-operation. Although rare, metastasis of craniopharyngioma has been reported up to 21 years after resection of the primary tumor. Metastasis often occurs along the surgical route, as in our 3 cases, but spreading to distant locations via cerebrospinal fluid has been observed. Regular follow-up is necessary, even after supposedly total resection.     

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.     

Rapid enlargement and recurrence of a preexisting intrasellar craniopharyngioma during the course of two pregnancies. Case report.

Enlargement of preexisting pituitary adenomas during pregnancy is well documented, but this phenomenon is unusual for nonendocrine pituitary tumors such as craniopharyngiomas. Only six cases of craniopharyngioma have been reported as presenting during pregnancy. The authors describe a 19-year-old woman who presented with amenorrhea and galactorrhea caused by an intrasellar mass. Seven months later, when she was 20 weeks pregnant, the patient developed sudden visual dysfunction. Emergency transsphenoidal surgery was performed to restore visual function, and the tumor was found to be a craniopharyngioma. The patient had spontaneous labor and delivered a healthy infant at term. The tumor recurred 4 years later, during her second pregnancy, and was again entirely removed via a second transsphenoidal approach. She again had a normal term delivery. During the 5-year follow-up period she has demonstrated no endocrinological or visual dysfunction. Control magnetic resonance images have revealed no recurrence of the tumor. The transsphenoidal approach seems to be the safest procedure to use during pregnancy to achieve an immediate optic nerve decompression and to preserve pituitary function.     

Craniopharyngioma presenting a symptom of pituitary apoplexy and hyponatremia: a case report

We report a case of a 70-year-old woman admitted to our hospital with sudden headache, drowsiness and hyponatremia. MRI on admission showed an intrasellar mass with suprasellar extension. Serum examination revealed decreased sodium and hypopituitarism, but they were normalized gradually by compensation using hydrocortisone, thyroid hormone and salt. Afterwards, masked diabetes insipidus appeared and required aqueous pitressin for the control of urine volume. A removal operation was performed uneventfully using the transsphenoidal approach and histological examination confirmed squamous-papillary type craniopharyngioma. Intratumoral hemorrhage of the craniopharyngioma is extremely rare. These cases tend to occur after over 15 years of maturation and the squamous-papillary type tend to bleed more than the adamantinomatous type. However, it is very difficult to make a differential diagnosis between craniopharyngioma with intratumoral hemorrhage and pituitary apoplexy, judging only by symptoms or interventional radiology. The definite mechanism of hyponatremia associated with a parasellar lesion is still obscure, but compression to the anterior hypothalamus or pituitary gland by an enlargement of the parasellar tumor is generally hypothesized.     

Nasopharyngeal Extension of a Craniopharyngioma in a 4 Year Old Girl

Summary. Nasopharyngeal extension of a craniopharyngioma is very rare and usually presents with headache, nasal obstruction and visual disturbances. We present a case of a 4 year old girl, who became symptomatic with visual deterioration. MRI showed a huge supra – and infrasellar cystic craniopharyngioma with extension into the sphenoid sinus. Primary treatment was a transnasal puncture of the cyst followed by a subfrontal approach with removal of the tumour preserving the chiasm and optic nerves. The visual acuity postoperatively improved while she needed hormone replacement due to panhypopituitarism. Follow-up 12 months after the operation showed no recurrence. This is the youngest patient of about 27 patients reported so far in the literature.     

Oedema of the abductor digiti quinti muscle due to subacute denervation: Report of two cases

The clinical presentation of abductor digiti quinti (ADQ) denervation is often non-specific. The diagnosis is generally clinical and may be easily missed. This case report of two patients describes the magnetic resonance imaging (MRI) finding of unilateral oedema and fatty infiltration isolated to the ADQ. A 36-year old woman who presented with laterally located left foot pain was initially diagnosed as having plantar fasciitis. An MRI scan arranged due to the unusual site of the pain showed increased signal intensity within the ADQ muscle on T1 and T2 images indicating fatty infiltration. Short tau inversion recovery (STIR) images showed hyperintensity of the ADQ indicating oedema. The MRI scan of a 45-year old man who presented with a three month history of left heel pain revealed similar findings. These MRI appearances indicate subacute denervation, which, when involving solely the ADQ muscle suggests entrapment of the first branch of the lateral plantar nerve. Consideration of this imaging finding when examining MRI scans of patients with non-specific heel pain has the potential to facilitate diagnosis.     

Craniopharyngiomas with intratumoral hemorrhage--two case reports

Two cases of craniopharyngioma with intratumoral hemorrhage are reported. A 22-year-old male was admitted with meningitis. Lumbar tapping was performed twice. He subsequently developed reduced visual acuity and field deterioration due to intratumoral hemorrhage from an intra- and suprasellar tumor. He underwent emergency craniotomy and total extirpation of the tumor. A 29-year-old female underwent partial extirpation of an intra- and suprasellar cystic tumor via transsphenoidal surgery. Two months after the first operation, she suffered intratumoral hemorrhage necessitating emergency surgery and subsequent gamma-knife therapy. The histological diagnosis was craniopharyngioma in both cases. Hemorrhage is extremely rare in craniopharyngiomas and difficult to discriminate from that in pituitary adenoma, but both diseases require decompression by clot extirpation.     

Pituitary carcinoma with a single metastasis causing cervical spinal cord compression. Case report

Pituitary carcinoma is rare, with fewer than 100 cases having been reported in the English-language literature. The diagnosis of pituitary carcinoma requires the demonstration of cerebrospinal and/or systemic metastases rather than local invasion. The lesion carries a poor prognosis; fewer than 50% of patients survive beyond 1 year after diagnosis. In this report the authors describe the case of a 68-year-old man who had undergone transsphenoidal debulking surgery and pituitary radiotherapy 4 years earlier for a pituitary adenoma. He presented with cervical cord compression due to a single metastasis from pituitary carcinoma. The authors discuss the management of this entity and review the literature for current opinion on the pathogenesis of these tumors, factors resulting in malignant transformation, and the reliability of markers that predict future malignant behavior. Evidence for the various treatment modalities is also appraised.     

Isolated oculomotor nerve palsy following apoplexy of a pituitary adenoma.

An isolated oculomotor nerve palsy is very rarely the presenting sign of a pituitary adenoma. It may occur slowly due to mechanical compression or rapidly, secondary to pituitary apoplexy. Magnetic resonance imaging (MRI) with and without gadolinium DTPA enhancement provides excellent anatomical detail and is useful in the planning of the operative procedure. When correctly diagnosed and treated, the third nerve dysfunction appears to be reversible. We report a case of a pituitary adenoma presenting with an isolated, partial oculomotor nerve palsy in the setting of apoplexy. The pathophysiology, prognostic factors and MRI findings of this entity are discussed.     

Juvenile transient bone marrow oedema of the foot associated with Vitamin D deficiency: A case study and an overview of pathogenesis and treatment

Bone Marrow Oedema Syndrome during childhood is a rare benign transient condition without clear pathophysiology. It usually resolves after conservative treatment, but resolution may exceed up to 8 months. A 12-year-old child with bone marrow oedema of the left foot which was diagnosed by magnetic resonance imaging (MRI) is reported. She presented with a six week subacute pain and mild swelling on the dorsal surface of the foot. Routine plain radiographs, blood tests, biochemical and serological tests were normal with the exception of serum Vitamin D levels that were reduced. The management of the child included partial weight-bearing, administration of anti-inflammatory drugs and supplementation of Vitamin D due to insufficient Vitamin D intake. After six months the child did not have any clinical symptoms and MRI showed complete resolution of the oedema. This is the first report of a juvenile bone marrow oedema correlated with hypovitaminosis D that was successfully treated with Vitamin D administration.     

Pituitary adenoma associated with neurofibromatosis type 1: case report

A case of pituitary adenoma associated is neurofibromatosis type 1 is reported. On June 6, 2000, a 49-year-old man was admitted to the Department of Neurological Surgery, Okayama University Hospital, for bitemporal hemianopsia. Twenty-nine years previously, he had been operated on for a left inguinal tumor that proved to be a neurofibroma. Based on the presence of other manifestations, such as café-au-lait spots and subcutaneous nodules, he had been diagnosed with neurofibromatosis type 1, the same as his father, sister, and daughter. Computed tomography and magnetic resonance imaging demonstrated an intrasellar mass lesion with a cystic portion in the suprasellar region. Endocrinologically, almost all of his basic hormone levels were normal. A right front-temporal craniotomy was performed for a preoperative diagnosis of craniopharyngioma, and total intracapsular tumor extirpation was achieved. The histological diagnosis was clinically silent corticotroph pituitary adenoma. Neurofibromatosis is sometimes associated with neoplasms of the central nervous system, usually optic gliomas. Associations between pituitary adenomas and NF 1 are very rare and have been reported in only four cases, including the present case.     

Cortical seeding of a craniopharyngioma after craniotomy: Case report

BACKGROUND: Cortical seeding of a craniopharyngioma has been rarely reported. We present a case that ectopically recurred along the tract of a previous surgical route. METHODS: A 27-year-old woman presented earlier with a suprasellar craniopharyngioma. A left frontotemporal craniotomy was done with subtotal resection of the tumor because it was strongly adhered to the optic chiasm. Histopathology confirmed the diagnosis of craniopharyngioma. Six months after, the patient presented with decreased visual acuity and diplopia. She was reoperated through the previous craniotomy with a total resection. One year after the second surgery, the patient presented with seizures that were difficult to control. Magnetic resonance imaging revealed a contrast-enhancing tumor with cystic and solid components on the left temporal lobe cortex. The primary tumor bed was intact. The patient was reoperated, and the temporal lobe tumor was totally removed. Histologic studies showed an adamantinomatous craniopharyngioma. The patient was free of neurologic abnormalities, and no new lesion was found in the magnetic resonance imaging performed 1 year after the last surgery. CONCLUSIONS: Although craniopharyngiomas exhibit a benign histopathologic pattern, a total resection combined with careful inspection and irrigation of the surgical field is the optimal treatment for preventing local and ectopic recurrences. It is strongly recommended that the concerned patients have a long-term clinical and neuroimaging follow-up.     

Malignant optic glioma presenting radiologically as a "cystic" suprasellar mass: case report and review of the literature

The diagnosis of malignant glioma of the optic nerves and chiasm may be difficult because these rare lesions may mimic other suprasellar lesions. We report a case of glioblastoma multiforme of the optic chiasm in which the appearance of the lesion on computed tomographic (CT) scan was consistent with that of craniopharyngioma or cystic pituitary adenoma. We suggest that malignant optic glioma should be considered in the differential diagnosis of an adult with progressive visual loss of rapid onset, even when ophthalmological examination strongly suggests extrinsic compression of the anterior optic pathways and when the CT scan apparently indicates a cystic mass lesion.