Surgery versus conservative care for Rathke's cleft cyst.

BACKGROUND: Rathke's cleft cysts are benign cystic lesions of the sellar region, which may cause headache, pituitary deficiencies and visual disturbances from mass effect. Their management is not standardized yet. This study is about establishing a consensus for medical care of RCC. MATERIAL AND METHODS: We performed a retrospective observational study of all patients that were diagnosed or followed for RCC between 2008 and 2018 (11 years), in the neurosurgical and the adult endocrine departments of our institution. The study's average time length of follow-up is 72.9 months (from 2 to 385 months). RESULTS: The 57 included patients were divided into 2 groups: group A, which included 39 patients that were conservatively managed and group B, which included 18 surgically treated patients. Group A showed either an improvement or a spontaneous resolution of headaches in 56.1% of the cases (P<0.01); a resolution of hyperprolactinemia in 70% of the cases (P=0.21); and of hypogonadism, ACTH deficiency, growth hormone deficiency in 100% of the cases. There was no spontaneous improvement of visual disturbances (P<0.01) or diabetes insipidus (P=0.29) during follow-up. Regarding group B, surgery allowed improvement or complete resolution of headaches in 60% of the cases; visual troubles in 100% of the cases (P<0.01); and hyperprolactinemia in 100% of the cases. Pituitary deficiencies were not improved by surgery. CONCLUSIONS: This study offers guidance in decision-making regarding the management of RCC patients. Surgery is particularly suitable for treating visual disturbances caused by RCC. Regular follow-up is more appropriate than surgery concerning headaches, hyperprolactinemia, endocrine disruptions and diabetes insipidus.

Outcome of diabetes insipidus in patients with Rathke's cleft cysts.

OBJECTIVES: It is well-known that Rathke's cleft cysts (RCCs) infrequently cause headache, endocrinological dysfunction, and visual disturbance, and in rare cases, cause diabetes insipidus (DI). Although surgical evacuation of the cyst content can result in high rates of symptomatic improvement, not only the treatment efficacy but also the pathophysiology of DI with RCC are undetermined. The aim of this study is to elucidate the underlying mechanisms and outcomes of DI associated with RCCs. PATIENTS AND METHODS: We retrospectively studied 109 patients with RCCs treated at Kanazawa University Hospital between 2000 and 2016. Their age, sex, symptoms, endocrinological status, DI, visual disturbance, neuroradiological findings, pathological appearances, and pre-/post-operative hormone levels and status of anti-diuretic hormone replacements were assessed. RESULTS: Among 109 cases of RCCs, five cases (4.6%, 2 males and 3 females) manifested with DI as initial presentation were included. These five cases could be divided into two types: the acute type and the chronic type, based on the onset and duration of symptoms. Three acute onset cases presented with not only strong thirst but also sudden headaches without pituitary dysfunction, whereas the two chronic onset cases presented with chronic headaches and hypopituitarism. Pathological examination in the acute type revealed inflammatory cell infiltration into only the posterior lobe of the pituitary and disruption of the cyst wall adjacent to the posterior lobe, which might suggest RCC rupture. In contrast, the chronic type showed inflammatory cell infiltration into both the anterior and posterior lobes of the pituitary and thickened fibrosis beneath the cyst wall. Postoperatively, two cases of the acute type could be controlled with a smaller amount of 1-deamino-8-D-arginine vasopressin (DDAVP) than that required preoperatively, whereas no change was observed in the cases of the chronic type. CONCLUSION: The cases of DI onset caused by RCCs could be divided into the acute type and the chronic type. In the chronic type, surgical treatment could not affect the status of DI. However, in acute type, urgent surgical intervention partially relieved DI.

Extended endoscopic endonasal approach for recurrent or residual symptomatic craniopharyngiomas.

OBJECTIVES: Removal of recurrent or residual symptomatic craniopharyngiomas is more challenging than the primary surgery. The extended endoscopic endonasal (EEE) approach has been proposed an alternative surgical route for removal of various suprasellar tumors including craniopharyngiomas currently. In this study, we summarized the operative experience and described the feasibility and advantages of this technique in recurrent or residual symptomatic craniopharyngiomas. PATIENTS AND METHODS: A retrospective review of 15 patients (9 males and 6 females) whom underwent EEE approach between April 2012 and February 2017, were included in this study. The lesions included 8 purely suprasellar craniopharyngiomas (2 extraventricular, 6 intraextraventricular), 3 both intra- and suprasellar craniopharyngiomas, and 4 intrasellar craniopharyngiomas. The mean preoperative (that is, EEE approach) tumor volume was 10.54 cm3. The mean follow-up period was 23.1 months (range, 8-54). All patients were analyzed in terms of the treatment effect, complictions and follow-up results. RESULTS: Total removal of tumors was achieved in 12 patients (80.0%) and subtotal removal in 3 cases (20.0%). The pituitary stalks were identified in 11 patients during operations and secured in 8 patients. Postoperative visual acuity was improved in 10 cases, and normalization of the impairment was achieved in 3 patients. There were no significant differences between pre and postoperative endocrine function, except in one patient with normal preoperative pituitary hormone function who suffered postoperative hypopituitarism. Postoperative diabetes insipidus (DI) occurred in 14 patients including 6 patients who had long-term DI and others reporting transient postoperative DI. No cerebrospinal fluid (CSF) leak was identified. There were no deaths or major complications. Obesity developed in 2 patients, with no deaths and recurrent cases during follow-up period. CONCLUSION: The pure EEE approach is a safe, effective alternative for treatment of recurrent or residual symptomatic craniopharyngiomas owing to its advantages including wide-angle view, close observation and elimination of brain retraction. Larger studies with further follow-up is needed to assess the long-term efficacy of this minimal access approach.

Xanthomatous Hypophysitis Presenting with Diabetes Insipidus Completely Cured Through Transsphenoidal Surgery: Case Report and Literature Review.

BACKGROUND: Xanthomatous hypophysitis (XH) is extremely rare. Only 27 cases have been reported in the literature. No XH patient presenting with diabetes insipidus (DI) has been completely cured through surgery. Here, we describe the first XH case of a DI patient whose pituitary function was normalized postoperatively, without hormone replacement therapy. CASE DESCRIPTION: A 41-year-old woman suffered from polydipsia, DI, headache, and breast discharge. Laboratory investigation revealed hyperprolactinemia. Pituitary magnetic resonance imaging showed a 2.0-cm × 1.4-cm × 1.6-cm lesion that demonstrated heterogeneous intensity on T1-weighted imaging and peripheral ring enhancement following contrast; the lesion was totally removed through transsphenoidal surgery. Histopathologic and immunohistochemical examinations confirmed the diagnosis of XH. At the 4- and 15-month follow-up visits, all pituitary-related hormones were normal, and the patient was not taking medication. A repeat pituitary magnetic resonance imaging showed no evidence of recurrence. CONCLUSIONS: To the best of our knowledge, this case is the first documented occurrence of XH with DI completely cured through surgery. If XH is suspected, total surgical resection of the lesion is recommended and normal pituitary tissue should be carefully protected intraoperatively.

Outcome of Radical Surgical Resection for Craniopharyngioma with Hypothalamic Preservation: A Single-Center Retrospective Study of 1054 Patients.

OBJECTIVE: A retrospective review of the surgical outcome for patients with craniopharyngioma (CP) treated in a single neurosurgical center with surgical resection using visualization to ensure hypothalamic preservation. METHODS: The study included 1054 patients. Before 2003, a pterional cranial approach was preferred for 78% of patients; after 2004, the unifrontal basal interhemispheric approach was performed in 79.1% of patients. RESULTS: Complete tumor resection was achieved in 89.6% of patients; vision improved in 47.1% of patients who had preoperative vision impairment. However, diabetes insipidus worsened in 70.4% of patients and new-onset diabetes insipidus occurred in 29.7% of the remaining patients. Pituitary stalk preservation occurred in 48.9% of cases. There were 89.6% of patients with total tumor removal; 13.3% of patients showed tumor recurrence within an average of 2.8 years. Of 69 follow-up patients with a subtotal or partial resection, 94.2% showed tumor recurrence within an average of 4.3 months. Of the total patients, 82.3% fully recovered. CONCLUSIONS: This study has shown that radical surgical resection of CP using microsurgical excision can be effective with a good patient outcome without more limitations on each individual tumor of distinct features despite the impact of recent endoscopic techniques on CP surgery. The surgical approach depends on a direct and wider visualization of CP located in the midline with preserving hypothalamic structures by identifying some hypothalamic landmark structures. After surgery, most patients can resume their normal activities even after aggressive tumor removal, although patients require postoperative hormonal replacement.

Bilateral Ossified Chronic Subdural Hematoma Presenting as Diabetes Insipidus-Case Report and Literature Review.

Calcified chronic subdural hematomas are an occurrence rarely seen in neurosurgical clinical practice. And when they occur bilaterally, the radiologic image they present is fascinating, as is the clinical presentation, but their management may be challenging. They have been reported to present with a multitude of neurologic deficits but never with diabetes insipidus, which is described here. Due to the rarity of this pathology, the management protocol is not well defined, though there have been quite a few papers on this condition. This review article gathers information published over the years on this rare entity to suggest a treatment protocol.

Surgical management of pituitary metastases.

BACKGROUND: Pituitary metastases are rare and commonly described in case reports or small case series. Due to its rarity this entity is not subject to standardized treatment guidelines, there is debate about typical initial symptoms that may lead to finding the correct diagnosis and information about the clinical course is also sparse. METHODS: We have conducted a retrospective analysis of patients with pituitary metastases who were surgically treated via a transsphenoidal procedure at our institution between 2006 and 2014. Underlying primary disease, clinical and surgical course as well as adjuvant radiotherapy and follow-up data are presented. RESULTS: 14 patients met the inclusion criteria (8 female, 6 male). Mean age was 61.5 years. Most patients became symptomatic with visual symptoms--both visual deterioration and/or diplopia (n = 13)--and anterior lobe insufficiency (n = 8). Surprisingly diabetes insipidus was only seen in three patients. All patients underwent transsphenoidal surgery initially, four patients had to undergo surgery for residual tumor or recurrence, two of them via a transcranial route. Breast cancer was the most common entity (n = 6), followed by prostate cancer (n = 3), nsclc (n = 2) and melanoma, thyroid cancer and renal cancer in one case each. Postoperative MRI showed gross total resection in four cases and residual disease in eight cases (subtotal resection, partial resection and biopsy), two patients files were incomplete regarding MRI-results. All patients underwent adjuvant radiotherapy. Survival after the initial diagnosis of cancer was 36 and 16 months after diagnosis of pituitary metastases. CONCLUSION: Our results indicate that transsphenoidal surgery is a safe method to resect pituitary metastases and that the extend of resection does not have an influence on survival time. Our results also indicate that diabetes insipidus may not be the most common initial symptom of pituitary metastases and lack thereof should not lead to making a wrong diagnosis and delaying appropriate therapy.

Pure Lymphocytic Infundibuloneurohypophysitis Caused by the Rupture of Rathke's Cleft Cyst: Report of 2 Cases and Review of the Literature.

The major symptoms that are caused by Rathke's cleft cysts (RCCs) are visual disturbances, headaches, and endocrine insufficiencies. Among these symptoms, the endocrine insufficiencies are thought to result from the spreading of inflammation that is induced by the cyst contents onto the pituitary gland or the compression of the gland and the pituitary stalk by RCCs. Here, we present 2 rare cases with lymphocytic infundibulohypophysitis with the sudden onset of headaches and subsequent diabetes insipidus (DI). Magnetic resonance imaging revealed remarkable swelling of the pituitary gland with a small mass that was located between the anterior and the posterior lobe of the pituitary gland. Transsphenoidal surgery was performed to remove the mass, and pathological examinations of the cyst wall demonstrated that the epithelial tissue of the RCC and the posterior lobe were affected by massive lymphocytic infiltration. The clinical courses and pathological results of these patients strongly suggested that the rupture of the RCC onto the posterior lobe caused the lymphocytic hypophysitis. Postoperatively, the DI could be controlled with a smaller amount of anti-diuretic hormone replacement compared to that required preoperatively.

Primary adult infradiaphragmatic craniopharyngiomas: clinical features, management, and outcomes in one Chinese institution.

OBJECTIVE: This study was designed to evaluate the clinical, radiologic, histologic, and surgical outcome characteristics of this disease treated in a single institution. METHODS: Sixteen adult patients underwent transsphenoidal microsurgery from October 2005 to December 2010 at Neurosurgical Center of Beijing Tiantan Hospital. The clinical, radiological, operative, and pathological findings of the patients were reviewed retrospectively. RESULTS: Pituitary dysfunction was presented in 12 patients, visual acuity and/or field deterioration in 11 patients, and headache in 8 patients. Hyperprolactinemia was presented in 7 of 9 female patients. All lesions were resected by transsphenoidal microsurgery as the primary procedure. A gross total resection was achieved in 3 of 16 patients, a radical subtotal resection in the remaining 13 patients. Nine cases were histologically classified as adamantinous subtype. After a mean follow-up of 50 months, 2 patients experienced recurrence. All female patients who had hyperprolactinemia experienced a gain of function postoperatively. Six patients experienced new diabetes insipidus. Visual field improved or normalized in 8 of 9 patients. Visual acuity improved in 1 case, and worsened in 1 patient. CONCLUSIONS: Primary adult infradiaphragmatic craniopharyngiomas are relatively rare lesions occurring in young adults. Pituitary dysfunction, visual acuity and/or field deterioration, and headache were the most common chief symptoms. Transsphenoidal surgery, including tearing the cyst walls off the diaphragma sellae and protecting normal pituitary tissue as much as possible, is recommended. Although at the risk of impairing the function of anterior pituitary, transsphenoidal surgery results in a high rate of both visual field and hyperprolactinemia improvement with a low associated risk of recurrence. In terms of pathological aspects, the adamantinous subtype was more common.

Individualized surgical strategies for Rathke cleft cyst based on cyst location.

OBJECT: An assessment regarding both surgical approaches and the extent of resection for Rathke cleft cysts (RCCs) based on their locations has not been reported. The aim of this study was to report the results of a large series of surgically treated patients with RCCs and to evaluate the feasibility of individualized surgical strategies for different RCCs. METHODS: We retrospectively reviewed 87 cases involving patients with RCCs (16 intrasellar, 50 intra- and suprasellar, and 21 purely suprasellar lesions). Forty-nine patients were treated via a transsphenoidal (TS) approach, and 38 were treated via a transcranial (TC) approach (traditional craniotomy in 21 cases and supraorbital keyhole craniotomy in 17). The extent of resection was classified as gross-total resection (GTR) or subtotal resection (STR) of the cyst wall. Patients were thus divided into 3 groups according to the approach selected and the extent of resection: TS/STR (n = 49), TC/STR (n = 23), and TC/GTR (n = 15). RESULTS: Preoperative headaches, visual dysfunction, hypopituitarism, and diabetes insipidus (DI) resolved in 85%, 95%, 55%, and 65% of patients, respectively. These rates did not differ significantly among the 3 groups. Overall, complications occurred in 8% of patients in TS/STR group, 9% in TC/STR group, and 47% in TC/GTR group, respectively (p = 0.002). Cerebrospinal fluid (CSF) leakage (3%), new hypopituitarism (9%), and DI (6%) were observed after surgery. All CSF leaks occurred in the endonasal group, while the TC/GTR group showed a higher rate of postoperative hypopituitarism (p = 0.7 and p < 0.001, respectively). It should be particularly noted that preoperative hypopituitarism and DI returned to normal, respectively, in 100% and 83% of patients who underwent supraorbital surgery, and with the exception of 1 patient who had transient postoperative DI, there were no complications in patients treated with supraorbital surgery. Kaplan-Meier 3-year recurrence-free rates were 84%, 87%, and 86% in the TS/STR, TC/STR, and TC/GTR groups, respectively (p = 0.9). CONCLUSIONS: It is reasonable to adopt individualized surgical strategies for RCCs based on cyst location. Gross-total resection does not appear to reduce the recurrence rate but increase the risk of postoperative complications. The endonasal approach seems more appropriate for primarily intrasellar RCCs, while the craniotomy is recommended for purely or mainly suprasellar cysts. The supraorbital route appears to be preferred over traditional craniotomy for its minimal invasiveness and favorable outcomes. The endoscopic technique is helpful for either endonasal or supraorbital surgery.

Rathke cleft cyst in seven-year-old girl presenting with central diabetes insipidus and review of literature.

Rathke cleft cysts (RCC) are benign cysts derived from remnants of Rathke cleft, and are rarely symptomatic in children. Symptoms due to RCC are associated with mass effect and pituitary hormone deficiencies. Slow growth rate of the cyst makes its incidence increase with aging. Here we report on a seven-year-old girl who presented with central diabetes insipidus (CDI). Her sella MRI revealed a lesion in the sellar region which grew rapidly in follow-up. She underwent microneurosurgical operation and the lesion was totally excised. Pathologic examination revealed RCC with degenerative changes. In her follow-up, growth hormone deficiency developed in addition to arginine vasopressin deficiency. Rapid growth of the cyst is not the usual course of RCC's. Mechanisms regarding the cyst growth are unclear as they are in this case. This is the youngest child to date presenting with central diabetes insipidus due to RCC. Rapid growth of RCC can cause CDI in young children.

Primary, non-exophytic, optic nerve germ cell tumors.

Tumors of the optic chiasm are relatively uncommon and usually associated with phakomatoses such as neurofibromatosis. Even more rare is the presentation of a primary, non-exophytic, isolated optic chiasm germ cell tumor (GCT). These tumors have imaging characteristics nearly indistinguishable from optic chiasmatic gliomas (OCGs). Herein we describe two cases of young men who presented with similar findings of progressive, painless visual loss and hypothalamic-pituitary-adrenal axis dysfunction including diabetes insipidus. Brain imaging was non-diagnostic and suggestive of an OCG. Pathology demonstrated GCTs in each case highlighting the importance of biopsy confirmation of the diagnosis. Both patients underwent a pterional craniotomy and sub-frontal approach to the optic chiasm. The chiasm was diffusely enlarged and discolored in each case without evidence of sellar, suprasellar or perichiasmatic pathology. Pathology demonstrated a malignant mixed GCT in the first patient and a germinoma in the second. This case series highlights the importance of tissue biopsy for patients with progressive symptoms from optic chiasm tumors. Furthermore, this is the first report of a primary, non-exophytic malignant mixed GCT. As the treatment regimens differ widely between optic chiasm GCTs and chiasm gliomas, tissue diagnosis is important.

Lymphocytic infundibulo-neurohypophysitis: An unusual cause of recurrent optic neuropathy in a child.

Lymphocytic infundibulo-neurohypophysitis (LINH), a rare autoimmune disease, is distinct from lymphocytic hypophysitis and is characterized by lymphocytic and plasma cell infiltration of the posterior lobe of the pituitary and the pituitary stalk.(1) We report the case of a young boy who presented with recurrent, steroid-responsive optic nerve dysfunction and diabetes insipidus. T1-weighted magnetic resonance imaging scans of the brain showed an isointense soft tissue mass within the sella turcica and a thickened pituitary stalk. Tissue specimen resected at transsphenoidal surgery revealed chronic inflammation with infiltration of lymphocytes and no granulomatosis or necrosis, establishing the diagnosis of LINH.(2) This is the first report of optic neuropathy in association with LINH in a child.

Keyhole surgery for isolated pituitary stalk metastatic tumors: a case report and review of the literature.

Solitary metastatic pituitary stalk tumors account for approximately less than 1% of all pituitary gland tumors and present difficulties in clinical diagnosis because most of them are clinically silent and usually too small to cause radiological changes. With the advance of microsurgical techniques, keyhole surgery is indicated to obtain a specimen for pathological diagnosis and possible removal of the tumor. Here, we reported a patient who has a history of advanced breast cancer and who complained of polyuria and polydypsia. Magnetic resonance images revealed a solitary tumor over the pituitary stalk. A right supraorbital craniotomy was performed and the pathological report confirmed the diagnosis of metastatic breast cancer. This is the first case report using keyhole surgery to confirm the pathology and improve the clinical symptoms. The relevant literature is also reviewed.

Panhipopituitarismo y diabetes insípida secundarios a hipofisitis linfocitaria en una paciente con trisomia 12p / Panhypopituitarism and diabetes insipidus secondary to a lymphocytic hypophysitis in one patient with trisomy 12p

Lymphocytic hypophysitis (LH) is an uncommon inflammatory disease of the hypophysis. It's female to male ratio of appearance is 9:1. Pregnant women are more affected during the third trimester of pregnancy or postpartum. Clinical and radiological presentation can simulate a hypophyseal adenoma. We report a nonpregnant 13 years old adolescent, with a trisomy 12p, with panhypopituitarism, diabetes insipidus and a selar tumor. It was necessary to differentiate between a germinoma and a LH. The latter was confirmed with the hypophyseal biopsy.

Spontaneous resolution of diabetes insipidus after pituitary stalk sectioning during surgery for large craniopharyngioma. Endocrinological evaluation and clinical implications for surgical strategy.

The mechanism of spontaneous resolution of diabetes insipidus (DI) was investigated after surgery for large craniopharyngioma. Twenty-two adult patients (mean age 48.9 years old), who underwent surgery via the anterior interhemispheric trans-lamina terminalis approach, were divided into three groups: Group I, the entire pituitary stalk was preserved (n = 2); Group II, the stalk was dissected distally from the tumor but ultimately sacrificed (n = 9); Group III, the stalk was not identified and was sacrificed (n = 11). All patients were discharged without neurological deficits 1 month after surgery. Four patients underwent gamma-knife treatment for residual tumor or recurrence. Postoperative endocrinological functions were normal in Group I, and no replacement therapy was required. Hormonal replacement for pan-hypopituitarism and DI was necessary in Groups II and III (mean follow-up period 5.9 years). DI resolved at 2.7 +/- 1.3 years after surgery in four patients in Group II, and a hypertonic saline infusion test revealed production of small amounts of intrinsic antidiuretic hormone (ADH). Urine osmolarity was high in the morning, and a significant increase in urinary osmolarity was noted after Pitressin injection. These results indicate induction of hypersensitivity of the distal renal tubules to small amounts of intrinsic ADH, resulting in decreased urinary output. Recovery from DI can be expected, despite permanent impairment of anterior pituitary function, if the pituitary stalk is dissected as distally as possible.

Resolution of diabetes insipidus following gamma knife surgery for a solitary metastasis to the pituitary stalk. Case report.

The authors present the case of a 58-year-old woman who presented with symptoms of diabetes insipidus (DI) 1 year after she was found to have a Stage 3 (of 4) estrogen receptor-positive infiltrating ductal adenocarcinoma of the left breast with pulmonary and bone metastases. Magnetic resonance images demonstrated a solitary site of metastasis in the patient's pituitary stalk, and gamma knife surgery (GKS) was performed to treat the lesion. Three months after GKS the patient was able to reduce the medication she required for the DI. There was no evidence of pituitary failure and no negative effect on her vision.

Refining the staging evaluation of pineal region germinoma using neuroendoscopy and the presence of preoperative diabetes insipidus.

Treatment strategies for CNS germinoma are currently evolving. Current approaches include reducing the volume and dose of radiation by adding pre-irradiation chemotherapy. Very accurate staging is necessary with such an approach to prevent failures. Eight consecutive patients with pineal germinoma at one institution underwent endoscopic surgery for tumor biopsy, direct visualization of the third ventricular region, and third ventriculostomy for those with hydrocephalus. All patients were treated with 4 cycles of chemotherapy. Conformal field radiation therapy followed, with the dose to the tumor bed dependent on the response to chemotherapy. Patients who had MRI, endoscopic, or cerebrospinal fluid evidence of multicentric or disseminated disease also received craniospinal radiation. Six patients had diabetes insipidus (DI) at presentation. All 6 had tumor studding the floor of the third ventricle on endoscopic visualization, while only 4 of those patients had MRI evidence of disease in that region. All patients have completed therapy and are alive, with no evidence of disease at median follow-up of 31.5 months from diagnosis. Direct endoscopic visualization of the third ventricular region may be more sensitive than MRI for evaluating the presence of suprasellar disease and appears to add important information. This parameter should be added to the staging evaluation when feasible. In this series, the presence of DI was 100% predictive of suprasellar disease, even when the MRI was negative for involvement of that region. Patients should be evaluated for DI as part of the initial staging, and if it is present, the patients should be treated for suprasellar disease regardless of MRI findings.