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Intraparenchymal infiltration of Rathke's cleft cysts manifesting as severe neurological deficits and hypopituitarism: 2 case reports.

Ogawa Y, Watanabe M, Tominaga T - BMC Res Notes (2016)

Bottom Line: Most infiltrated cells were lymphocytes and plasma cells, thought to indicate the involvement of long-term underling inflammatory processes in this phenomenon.Long-term subclinical inflammation may be the mechanism of this extraordinary aggressive clinical course.Postoperative steroid administration should be reduced prudently, and careful follow-up imaging is essential in cases of Rathke's cleft cyst with abnormal histological findings.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurosurgery, Kohnan Hospital, 4-20-1 Nagamachiminami, Taihaku-ku, Sendai, Miyagi, 982-8523, Japan. yogawa@kohnan-sendai.or.jp.

ABSTRACT

Background: Rathke's cleft cysts generally remain asymptomatic throughout life, but a few patients may suffer severe neurological and/or endocrinological deficits. The symptoms include visual disturbances caused by compression of the optic chiasm, and severe endocrinological deficits caused by repeated intracystic hemorrhage or leakage of cyst content. However, no case of Rathke's cleft cyst has infiltrated into neuroglial tissue with marked cerebral edema.

Case presentation: Two patients presented with non-infectious re-deterioration of Rathke's cleft cysts with intraparenchymal infiltration and marked cerebral edema, to ipsilateral hypothalamus in one case and to the bilateral frontal lobes in the other. Both patients were surgically treated by extended transsphenoidal surgery, and showed remarkable improvement with postoperative pulse-dose steroid therapy, including disappearance/shrinkage of abnormal enhanced lesion and cerebral edema on magnetic resonance imaging. Histological examination disclosed significant squamous metaplasia in epithelia and marked infiltration of inflammatory cells into the pituitary gland and neuroglial tissues. Most infiltrated cells were lymphocytes and plasma cells, thought to indicate the involvement of long-term underling inflammatory processes in this phenomenon.

Conclusion: Long-term subclinical inflammation may be the mechanism of this extraordinary aggressive clinical course. Postoperative steroid administration should be reduced prudently, and careful follow-up imaging is essential in cases of Rathke's cleft cyst with abnormal histological findings.

No MeSH data available.


Related in: MedlinePlus

Case 1. a Preoperative axial T1-weighted MR image with gadolinium revealing a multicystic sellar lesion extending from the suprasellar cistern to the left ambient cistern. b Preoperative sagittal T1-weighted MR image with gadolinium revealing infiltration of the lesion to the third ventricle floor. (c, d) Preoperative coronal T2-weighted MR images revealing an extensive irregular high intensity area in the left hypothalamus
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Fig2: Case 1. a Preoperative axial T1-weighted MR image with gadolinium revealing a multicystic sellar lesion extending from the suprasellar cistern to the left ambient cistern. b Preoperative sagittal T1-weighted MR image with gadolinium revealing infiltration of the lesion to the third ventricle floor. (c, d) Preoperative coronal T2-weighted MR images revealing an extensive irregular high intensity area in the left hypothalamus

Mentions: On admission her consciousness was clear, and body temperature was 36.4 °C. Peripheral white blood cell count was 5200/μl and C-reactive protein level was 0.08 mg/dl. All other clinical tests were negative for systemic inflammation or infection. Humphrey visual field analyzer examination showed temporal hemianopsia of the right eye and whole visual field defect of the left eye. Magnetic resonance (MR) imaging demonstrated a multicystic lesion compressing the optic chiasm upwards, and extending from the suprasellar cistern to the left ambient cistern. Part of the lesion had clearly infiltrated into the left hypothalamus, and extensive cerebral edema was present (Fig. 2).Fig. 2


Intraparenchymal infiltration of Rathke's cleft cysts manifesting as severe neurological deficits and hypopituitarism: 2 case reports.

Ogawa Y, Watanabe M, Tominaga T - BMC Res Notes (2016)

Case 1. a Preoperative axial T1-weighted MR image with gadolinium revealing a multicystic sellar lesion extending from the suprasellar cistern to the left ambient cistern. b Preoperative sagittal T1-weighted MR image with gadolinium revealing infiltration of the lesion to the third ventricle floor. (c, d) Preoperative coronal T2-weighted MR images revealing an extensive irregular high intensity area in the left hypothalamus
© Copyright Policy - OpenAccess
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4837620&req=5

Fig2: Case 1. a Preoperative axial T1-weighted MR image with gadolinium revealing a multicystic sellar lesion extending from the suprasellar cistern to the left ambient cistern. b Preoperative sagittal T1-weighted MR image with gadolinium revealing infiltration of the lesion to the third ventricle floor. (c, d) Preoperative coronal T2-weighted MR images revealing an extensive irregular high intensity area in the left hypothalamus
Mentions: On admission her consciousness was clear, and body temperature was 36.4 °C. Peripheral white blood cell count was 5200/μl and C-reactive protein level was 0.08 mg/dl. All other clinical tests were negative for systemic inflammation or infection. Humphrey visual field analyzer examination showed temporal hemianopsia of the right eye and whole visual field defect of the left eye. Magnetic resonance (MR) imaging demonstrated a multicystic lesion compressing the optic chiasm upwards, and extending from the suprasellar cistern to the left ambient cistern. Part of the lesion had clearly infiltrated into the left hypothalamus, and extensive cerebral edema was present (Fig. 2).Fig. 2

Bottom Line: Most infiltrated cells were lymphocytes and plasma cells, thought to indicate the involvement of long-term underling inflammatory processes in this phenomenon.Long-term subclinical inflammation may be the mechanism of this extraordinary aggressive clinical course.Postoperative steroid administration should be reduced prudently, and careful follow-up imaging is essential in cases of Rathke's cleft cyst with abnormal histological findings.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurosurgery, Kohnan Hospital, 4-20-1 Nagamachiminami, Taihaku-ku, Sendai, Miyagi, 982-8523, Japan. yogawa@kohnan-sendai.or.jp.

ABSTRACT

Background: Rathke's cleft cysts generally remain asymptomatic throughout life, but a few patients may suffer severe neurological and/or endocrinological deficits. The symptoms include visual disturbances caused by compression of the optic chiasm, and severe endocrinological deficits caused by repeated intracystic hemorrhage or leakage of cyst content. However, no case of Rathke's cleft cyst has infiltrated into neuroglial tissue with marked cerebral edema.

Case presentation: Two patients presented with non-infectious re-deterioration of Rathke's cleft cysts with intraparenchymal infiltration and marked cerebral edema, to ipsilateral hypothalamus in one case and to the bilateral frontal lobes in the other. Both patients were surgically treated by extended transsphenoidal surgery, and showed remarkable improvement with postoperative pulse-dose steroid therapy, including disappearance/shrinkage of abnormal enhanced lesion and cerebral edema on magnetic resonance imaging. Histological examination disclosed significant squamous metaplasia in epithelia and marked infiltration of inflammatory cells into the pituitary gland and neuroglial tissues. Most infiltrated cells were lymphocytes and plasma cells, thought to indicate the involvement of long-term underling inflammatory processes in this phenomenon.

Conclusion: Long-term subclinical inflammation may be the mechanism of this extraordinary aggressive clinical course. Postoperative steroid administration should be reduced prudently, and careful follow-up imaging is essential in cases of Rathke's cleft cyst with abnormal histological findings.

No MeSH data available.


Related in: MedlinePlus