Typing of inflammatory lesions of the pituitary

Inflammatory pituitary lesions account for 1.8% of all specimens from the German Pituitary Tumor Registry. They occure in 0.5% of the autoptical specimens and in 2.2% of the surgical cases. Women are significantly more often affected than men and are often younger when first diagnosed. In general, primary and secondary inflammation can be distinguished, with secondary types occurring more frequently (75.1%) than idiopathic inflammatory lesions (15.4%). In primary inflammation, the lymphocytic type is more common (88.5%) than the granulomatous type of hypophysitis (11.5%). The most common causes of secondary inflammation are Rathke’s cleft cysts (48.6%), followed by tumors (17.4%) such as the craniopharyngioma (9.1%), adenoma (5.5%) or germinoma (2.0%). More causes are tumor-like lesions (7.1%) such as xanthogranuloma (3.5%) or Langerhans histiocytosis (3.5%), abscesses (5.5%), generalized infections (5.1%), spreaded inflammations (4.7%) and previous surgeries (4.0%). In 1.6% of all specimens the reason for the inflammation remains unclear. The described classification of hypophysitis is important for specific treatment planning after surgery.

Contrast-free MRI Protocol for Pituitary Assessment in Children with Growth or Puberty Disorders: A Practical Approach.

Objectives Most of the pituitary MRI examinations in children with growth or puberty disorders (GPD) might not require gadolinium-based contrast agent (GBCA) administration. Methods Retrospective re-analysis of contrast-enhanced 567 pituitary MRIs of children with GPD. Two sets of sequences were created from each MRI examination: Set1 - common sequences without contrast administration and Set2 - common pre- and post-contrast sequences. The differences in the visibility of pituitary lesions between sets were statistically analyzed. Results The overall frequency of Rathke's cleft cysts was 11.6%, ectopic posterior pituitary 3.5% and microadenomas 0.9%. Lesions visible without contrast administration accounted for 85% of cases, while lesions diagnosed only after contrast injection accounted for 0.18% of all patients. Statistical analysis showed the advantage of antero-posterior (AP) pituitary dimension over other criteria in determining the appropriateness of using contrast in pituitary MRIs. The AP dimension was the most significant factor in logistic regression analysis: OR=2.23, 95%CI, 1.35-3.71, p-value=0.002 and in ROC analysis: AUC:72.9% with cut-off value 7.5 mm, with sensitivity/specificity rates: 69.2%/73.5%. Conclusions In most cases, the use of GBCA in pituitary MRI in children with GPD is unnecessary. The additional advantages of GBCA omission include: shortening the time of MRI examination and of general anesthesia.

Rathke’s cleft cyst with xanthogranulomatous change: A case report and review of the literature

Background: Rathke’s cleft cysts (RCCs) are benign, typically asymptomatic sellar lesions found incidentally in adults, with a dramatically lower incidence in pediatric patients (<18 years). We present a case of RCC with xanthogranulomatous change (XGC) – an even less common subtype of RCC – treated by endoscopic endonasal surgical resection. This is the second reported instance of an RCC with XGC occurring in a pediatric patient. Case Description: The patient is a 17-year-old male with delayed puberty who presented with bitemporal hemianopsia and was found to have a 2.6 cm lesion, initially thought to be a craniopharyngioma. He subsequently underwent uncomplicated transsphenoidal endoscopic endonasal resection. Histology confirmed the diagnosis of RCC and demonstrated marked degenerative XGCs with squamous metaplasia. The patient tolerated the procedure well with improvement in visual symptoms. Conclusion: RCC with XGC is a very rare pathology, particularly in the pediatric population. These lesions, while benign, can manifest clinically with significant symptoms. While treatment paradigms are not fully established with a small cohort of cases, endoscopic endonasal approaches have made surgical resection of these lesions a safe and effective treatment strategy, even in the pediatric population.

Endonasal Endoscopic Surgery for Pediatric Sellar and Suprasellar Lesions: A Systematic Review and Meta-analysis

Objectives The advent of endonasal endoscopic skull base surgery (ESBS) has redefined the management of pediatric sellar and suprasellar lesions. To date, the outcomes of these procedures have not been systematically reviewed. This study performed a systematic review with meta-analysis of surgical outcomes for pediatric patients undergoing ESBS for sellar and suprasellar lesions. Data Sources PubMed (National Library of Medicine, National Institutes of Health), Scopus (Elsevier), and Cochrane Library (Wiley). Review Methods Articles reporting on pediatric patients undergoing ESBS for craniopharyngiomas, pituitary adenomas, and Rathke’s cleft cysts were reviewed. The primary outcome was postoperative cerebrospinal fluid (CSF) leak. Secondary outcomes included endocrine, visual, and other complications. Results Twenty-five articles reporting on 554 patients were included. Overall postoperative CSF leak rate was 8.6%, with tumor-specific rates of 10.6% in craniopharyngiomas, 6.5% in pituitary adenomas, and 7.2% in Rathke’s cleft cysts ( P > .05). Older studies demonstrate higher postoperative CSF leak rates as compared with more recent studies (12.5% vs 6.1%, P = .0082). Younger children (8.9-12.6 years old) experienced a higher rate of postoperative CSF leaks as compared with older children (13.0-16.6 years old; 12.9% vs 4.9%, P = .0016). Additional postoperative complications included diabetes insipidus (26.7%), hypopituitarism (46.6%), visual deficits (2.6%), meningitis (3.4%), and weight gain (3.4%). Conclusion ESBS for pediatric sellar and suprasellar lesions is overall an effective management approach with an increasingly favorable risk-benefit profile. Younger children may be more susceptible to postoperative CSF leak as compared with older pediatric patients. Tumor type does not appear to be an independent risk factor for postoperative CSF leak in this population.

Successful application of stereotactic radiosurgery for multiply recurrent Rathke’s cleft cysts

OBJECTIVERathke’s cleft cysts (RCCs) are benign lesions of the sella turcica that usually come to neurosurgical attention due to compression of the optic apparatus (OA) and headaches. Treatment options for these lesions include observation, aspiration of cyst contents, or open resection of the cyst with the cyst wall. All of these options involve the potential for cyst recurrence or enlargement. In this study the authors report on a potential new therapeutic option for RCCs, i.e., stereotactic radiosurgery (SRS).METHODSA retrospective review was conducted of 5 patients with histologically confirmed, multiply recurrent RCCs who were treated with single-fraction SRS at a tertiary referral academic medical center.RESULTSThe total cohort consisted of 5 female patients with an average age of 31.8 years. The most common presenting symptom was headache followed by blurry vision. The symptoms were present on average for 7 months before intervention. The median number of surgeries prior to radiosurgery was 2. The average volume of lesion treated was 0.34 cm3. The median SRS dose was 12.5 Gy prescribed to the 50% isodose line with an average prescription coverage of 96.6%. The median dose to the OA was 5 Gy. At last follow-up, 3 of 5 cysts had completely regressed, 1 had regressed by more than 50% but was still present, and 1 was stable, with an overall mean follow-up duration of 34.2 months. There were no neurological, endocrinological, or visual complications attributable to SRS during the follow-up period.CONCLUSIONSRCCs can be a challenging clinical entity to treat, especially when they are multiply recurrent. In patients with an average of 2 previous surgeries for resection, a single SRS session prevented recurrence universally, with an average follow-up of almost 3 years. These results indicate that further investigation of the treatment of RCCs with SRS is indicated.