Rathke cleft cyst apoplexy: Hormonal and clinical presentation

Background: Rathke cleft cyst (RCC) apoplexy is an uncommon type of lesion that is challenging to diagnose without histopathological samples. Very few articles have been published describing the details of RCC apoplexy. We studied a good number of published articles to analyze its demographics, clinical and hormonal presentations, and outcomes. Methods: A literature review of English language publications about RCC apoplexy or pituitary apoplexy was conducted using Medline and EMBASE search engines. Thirty articles with histological diagnosis of RCC apoplexy were identified, the earliest of which was published in 1990 and the latest in 2019. We combined the findings of these articles with our own case report and then compared the demographics, clinical and hormonal presentations, and outcomes between RCC apoplexy and pituitary adenoma apoplexy. Results: Our data included 29 patients with RCC, with a mean age of 36.87 years (8–72) and a predominance of female patients (68%). The hemorrhagic type was most common, reported in 86%. Headache was the most common presenting symptom, being reported in 93% followed by hypogonadism (73%) and hormonal deficits (52%). All but three patients improved neurologically (90%); however, 45% of patients required long-term hormonal replacement, mostly thyroid hormone. No cases of worsening neurological or hormonal status were reported. Conclusion: RCC apoplexy presents with less severe neurological and hormonal abnormalities than pituitary adenoma apoplexy; it also has a better prognosis in endocrine functional recovery. We recommend applying current management guidelines of pituitary adenoma apoplexy to RCC apoplexy.

MR-Based Radiomics for Differential Diagnosis between Cystic Pituitary Adenoma and Rathke Cleft Cyst

Background. It is often tricky to differentiate cystic pituitary adenoma from Rathke cleft cyst with visual inspection because of similar MRI presentations between them. We aimed to design an MR-based radiomics model for improving differential diagnosis between them. Methods. Conventional diagnostic MRI data (T1-,T2-, and postcontrast T1-weighted MR images) were obtained from 215 pathologically confirmed patients (105 cases with cystic pituitary adenoma and the other 110 cases with Rathke cleft cyst) and were divided into training ( n = 172 ) and test sets ( n = 43 ). MRI radiomics features were extracted from the imaging data, and semantic imaging features ( n = 15 ) were visually estimated by two radiologists. Four classifiers were used to construct radiomics models through 5-fold crossvalidation after feature selection with least absolute shrinkage and selection operator. An integrated model by combining radiomics and semantic features was further constructed. The diagnostic performance was validated in the test set. Receiver operating characteristic curve was used to evaluate and compare the performance of the models at the background of diagnostic performance by radiologist. Results. In test set, the combined radiomics and semantic model using ANN classifier obtained the best classification performance with an AUC of 0.848 (95% CI: 0.750-0.946), accuracy of 76.7% (95% CI: 64.1-89.4%), sensitivity of 73.9% (95% CI: 56.0-91.9%), and specificity of 80.0% (95% CI: 62.5-97.5%) and performed better than multiparametric model ( AUC = 0.792 , 95% CI: 0.674-0.910) or semantic model ( AUC = 0.823 , 95% CI: 0.705-0.941). The two radiologists had an accuracy of 69.8% and 74.4%, respectively, sensitivity of 69.6% and 73.9%, and specificity of 70.0% and 75.0%. Conclusions. The MR-based radiomics model had technical feasibility and good diagnostic performance in the differential diagnosis between cystic pituitary adenoma and Rathke cleft cyst.

Intrasellar cysticercosis cyst treated with a transciliary supraorbital keyhole approach – A case report

Background: Neurocysticercosis is the most common parasitic disease affecting the central nervous system. Isolated sellar cysticercosis cysts are rare and can mimic other sellar lesion as cystic pituitary adenoma, arachnoid cyst, Rathke cleft cyst, or craniopharyngioma. The surgical resection is mandatory because the cysticidal drugs are ineffective, however, new microsurgical approaches are emerging to reduce complications and need to test in this condition. We present a patient with a sellar cysticercosis cyst treated by transciliar supraorbital keyhole approach. Case Description: A 45-year-old female with presented with chronic severe headaches, progressive deterioration of 6 months in visual acuity and bitemporal hemianopia. The pituitary hormonal levels were normal. Magnetic resonance findings showed a sellar and suprasellar cyst and underwent a microsurgical supraorbital transciliar keyhole approach for lesion resection. Pathologically, the lesion demonstrated a parasitic wall characterized by wavy, dense cuticle, and focal globular structure, surrounding inflammatory reaction with plasma cells. Postoperatively, the patient recovery fully neurologically. Conclusion: Intrasellar cysticercosis cyst causes significant neurological deficits due to its proximity to the chiasm, optic nerves, pituitary stalk, and the pituitary gland. Surgical section is an effective treatment. The supraorbital keyhole craniotomy offers satisfactory exposure, possibility of total resection with dissection of the supra and parasellar structures, short operative time, less blood loss, short hospital stay, and good overall surgical outcome.

Diabetes Insipidus After Endoscopic Transsphenoidal Surgery

BACKGROUND Diabetes insipidus (DI) is a recognized transient or permanent complication following transsphenoidal surgery (TSS) for pituitary tumors. OBJECTIVE To describe significant experience with the incidence of DI after TSS, identifying predictive characteristics and describing our diagnosis and management of postoperative DI. METHODS A retrospective analysis was performed of 700 patients who underwent endoscopic TSS for resection of pituitary adenoma (PA), Rathke cleft cyst (RCC), or craniopharyngioma. Inclusion criteria included at least 1 wk of follow-up for diagnosis of postoperative DI. Permanent DI was defined as DI symptoms and/or need for desmopressin more than 1 yr postoperatively. All patients with at least 1 yr of follow-up (n = 345) were included in analyses of permanent DI. Multivariable logistic regression models were constructed to identify predictors of transient or permanent postoperative DI. RESULTS The overall rate of any postoperative DI was 14.7% (103/700). Permanent DI developed in 4.6% (16/345). The median follow-up was 10.7 mo (range: 0.2-136.6). Compared to patients with PA, patients with RCC (odds ratio [OR] = 2.2, 95% CI: 1.2-3.9; P = .009) and craniopharyngioma (OR = 7.0, 95% CI: 2.9-16.9; P ≤ .001) were more likely to develop postoperative DI. Furthermore, patients with RCC (OR = 6.1, 95% CI: 1.8-20.6; P = .004) or craniopharyngioma (OR = 18.8, 95% CI: 4.9-72.6; P ≤ .001) were more likely to develop permanent DI compared to those with PA. CONCLUSION Although transient DI is a relatively common complication of endoscopic and microscopic TSS, permanent DI is much less frequent. The underlying pathology is an important predictor of both occurrence and permanency of postoperative DI.