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Kazunori Arita, Atsushi Tominaga, Kazuhiko Sugiyama, Kuniki Eguchi, Koji Iida, Masayuki Sumida, Keisuke Migita, and Kaoru Kurisu

Object

The increase in the incidental detection of asymptomatic pituitary adenomas, known as “pituitary inciden-talomas,” led the authors to conduct a survey of the natural course of these lesions.

Methods

Forty-two patients with clinically nonfunctioning pituitary adenomas who had manifested no neurological or endocrinological disorders were monitored with magnetic resonance imaging studies. The follow-up period ranged from 10.8 to 168.2 months (mean ± standard deviation, 61.9 ± 38.2 months). The mean initial tumor size was 18.3 ± 7 mm.

In 21 patients, the tumor increased by at least 10% of its measured size on detection. This increase was first detected between 8.4 and 58.8 months (mean 31.8 ± 17.6 months) after diagnosis. There was no correlation between the original tumor size, patient age, or the presence of intratumoral cysts and tumor growth. Symptoms were noted in 10 patients during follow up; in four, extensive tumor necrosis accompanied hemorrhage, leading to severe headache, acute ophthalmological symptoms, and panhypopituitarism, which was indicative of pituitary apoplexy. Transsphenoidal surgery was performed in 12 patients with enlarged tumors, including three with apoplexy. With the exception of one apoplectic patient, visual function was recovered in all who underwent surgery. All apoplectic patients continue to manifest hypopituitarism.

Conclusions

In the course of 4 years, the size of the incidentalomas increased in 40% of 42 patients and became symptomatic in 20%. During the 5-year follow up, pituitary apoplexy developed in 9.5%. These findings may justify early intervention, especially in young individuals with incidentally found macroadenoma.

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Kazunori Arita, Kaoru Kurisu, Atushi Tominaga, Kazuhiko Sugiyama, Fusao Ikawa, Hiroyuki Yoshioka, Masayuki Sumida, Yukari Kanou, Koji Yajin, and Ryusuke Ogawa

✓ The authors treated two patients with pituitary apoplexy in whom magnetic resonance (MR) images were obtained before and after the episode. Two days after the apoplectic episodes, MR imaging demonstrated marked thickening of the mucosa of the sphenoid sinus that was absent in the previous studies. The relevance of this change in the sphenoid sinus was investigated.

Retrospective evaluations were performed using MR images obtained in 14 consecutive patients with classic pituitary apoplexy characterized by acute onset of severe headache. The mucosa of the sphenoid sinus had thickened predominantly in the compartment just beneath the sella turcica, in nine of 11 patients, as ascertained on MR images obtained within 7 days after the onset of apoplectic symptoms. This condition improved spontaneously in all four patients who did not undergo transsphenoidal surgery. The sphenoid sinus mucosa appeared to be normal on MR images obtained from three patients at the chronic stage (> 3 months after onset). The incidence of sphenoid sinus mucosal thickening during the acute stage was significantly higher in the patients with apoplexy than that in the 100 patients without apoplexy. A histological study conducted in four patients who underwent transsphenoidal surgery during the early stage showed that the subepithelial layer of the sphenoid sinus mucous membrane was obviously swollen.

The sphenoid sinus mucosa thickens during the acute stage of pituitary apoplexy. This thickening neither indicates infectious sinusitis nor rules out the choice of the transsphenoidal route for surgery.

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Seiji Hama, Kazunori Arita, Takashi Nishisaka, Toshiyuki Fukuhara, Atsushi Tominaga, Kazuhiko Sugiyama, Hiroyuki Yoshioka, Kuniki Eguchi, Masayuki Sumida, Yuji Heike, and Kaoru Kurisu

Object. Rathke cleft cysts (RCCs) are composed of tall, well-differentiated, ciliated columnar epithelia. Their structures are altered by hyperplasia or squamous metaplasia, but their cause remains unknown.

Methods. The authors studied pathological findings and anterior pituitary function in 20 patients harboring RCCs. They classified RCC epithelium as either single (a single ciliated columnar cell lining or a flattened cuboidal cell lining) or stratified (a stratified ciliated columnar cell lining, basal cell hyperplasia, columnar cell hyperplasia, or squamous metaplasia). Inflammation was classified as acute, subacute, chronic, or end stage.

The epithelial cell lining was observed in 13 specimens obtained during surgery (six specimens contained single and seven contained stratified epithelia). Inflammation had penetrated the cyst epithelium or subjacent stroma in 10 patients, and the stage of inflammation correlated well with the type of epithelia group: early stages of inflammation in the single epithelium group and chronic or end-stage inflammation in the stratified epithelia (p = 0.0027). The adenohypophysis was identified in 21 surgical specimens. Postoperatively, growth hormone (p = 0.019), cortisol (p = 0.027), and thyroid-stimulating hormone (p = 0.039) responses significantly worsened as the inflammation progressed. The presence of diabetes insipidus correlated well with advanced stages of neurohypophysitis (p = 0.025).

Conclusions. Epithelial stratification in the RCC is caused by inflammation that may extend into the adjacent adenohypophysis or neurohypophysis and overwhelm the hypophysis, resulting in panhypopituitarism. Transsphenoidal excision may represent the best choice for treatment, at least for cases of RCC in which there is partial impairment of hypophysial function.

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Kazunori Arita, Fusao Ikawa, Kaoru Kurisu, Masayuki Sumida, Kunyu Harada, Tohru Uozumi, Shuji Monden, Jun Yoshida, and Yoshikazu Nishi

Object. Hypothalamic hamartoma is generally diagnosed based on its magnetic resonance (MR) imaging characteristics and the patient's clinical symptoms, but the relationship between the neuroradiological findings and clinical presentation has never been fully investigated. In this retrospective study the authors sought to determine this relationship.

Methods. The authors classified 11 cases of hypothalamic hamartoma into two categories based on the MR findings. Seven cases were the “parahypothalamic type,” in which the hamartoma is only attached to the floor of the third ventricle or suspended from the floor by a peduncle. Four cases were the “intrahypothalamic type,” in which the hamartoma involved or was enveloped by the hypothalamus and the tumor distorted the third ventricle.

Six patients with the parahypothalamic type exhibited precocious puberty, which was controlled by a luteinizing hormone—releasing hormone analog, and one patient was asymptomatic. No seizures or mental retardation were observed in this group. All patients with the intrahypothalamic type had medically intractable seizures, and precocious puberty was seen in one. Severe mental retardation and behavioral disorders including aggressiveness were seen in two patients. The seizures were controlled in only one patient, in whom stereotactically targeted irradiation of the lesion was performed.

This topology/symptom relationship was reconfirmed in a review of 61 reported cases of hamartoma, in which the MR findings were clearly described. The parahypothalamic type is generally associated with precocious puberty but is unaccompanied by seizures or developmental delay, whereas the intrahypothalamic type is generally associated with seizures. Two thirds of patients with the latter experience developmental delays, and half also exhibit precocious puberty.

Conclusions. Classification of hypothalamic hamartomas into these two categories based on MR findings resulted in a clear correlation between symptoms and the subsequent clinical course.