Giant (>4 cm) pituitary macroadenomas often require surgery to decompress the optic nerves. Compared with traditional open or transsphenoidal microscopic methods, the extended endoscopic endonasal transsphenoidal approach offers the potential for aggressive resection via a minimal access corridor. We conducted a systematic review of the literature to further our understanding of the role of endoscopy in the management of these challenging lesions. MEDLINE search of the modern literature (1995-2010) to identify surgical series for pediatric and adult pituitary adenomas >4 cm in maximal diameter. Patient and tumor characteristics, resection, morbidity and visual outcome were compared by approach. Chi-square and Fisher's exact tests with post-hoc Bonferroni analysis were used for statistical analyses. Sixteen studies (478 patients) were included. Compared with the open cohort, the endoscopic cohort had higher rates of gross total resection (47.2% vs. 9.6%; P < 0.003) and improved visual outcome (91.1% vs. 45.7%; P < 0.003). The microscopic transsphenoidal cohort had lower rate of total resection and worse visual outcomes than the endoscopic group. There were no instances of postoperative CSF leak reported in the endoscopic group. The transcranial group had a higher rate perioperative mortality compared to the transsphenoidal group (P = 0.004). In select cases, the endoscopic endonasal approach is safe and effective for the treatment of giant pituitary adenomas, with the potential for gross total resection and improved visual outcome. CSF leak, which is a major limitation of the endonasal approach, may be avoided using meticulous multi-layer closure and vascularised nasoseptal flaps.

Endoscopic endonasal compared with microscopic transsphenoidal and open transcranial resection of giant pituitary adenomas

The aim of this study was to determine the role of octreotide administration in acromegalic patients as a preparation for selective adenomectomy using transsphenoidal route. Octreotide was administered for 3 to 6 weeks before surgery in 12 patients and for 4 to 39 months in 25 patients. The clinical response was judged as excellent or good in 10 of 12 patients from group I and in 23 of 25 patients from group II. Marked reduction (ie, greater than 50% of initial values) in serum growth hormone (GH) levels was seen in all patients, with levels to less than 5 micrograms/L in 68% of patients and less than 2 micrograms/L in 27%. Insulin-like growth factor 1 (IGF-1) levels decreased to within normal limits in half the cases. During long-term treatment, an escape phenomenon could be seen. Varying degrees of tumor shrinkage were seen in more than 50% of cases. During surgery, with regard to the relative ease or difficulty in removing the tumor, the consistency of the tumor and the separation of normal from pathological tissue, no significant difference was observed between patients given octreotide and those from control series. Morphological changes in adenomatous tissue were rather small. The surgical outcome was similar in the pretreated series as in the control series, except in enclosed adenomas, which showed a tendency to a higher success rate. Since octreotide improves both the clinical condition and hormonal parameters and induces varying degrees of tumor shrinkage, it is potentially useful as an adjunct to surgery. Morphological data suggest that octreotide exercises a functional inhibitory effect on GH release.

Presurgical octreotide treatment in acromegaly

Craniopharyngiomas are biologically benign lesions (WHO Grade 1) of the sellar and suprasellar region, associated with a serious morbidity. About 50% of these tumors become clinically apparent during childhood. Clinical symptoms include headaches, chiasm syndrome, hydrocephalus, pituitary insufficiencies, and obesity. Growth arrest is a typical symptom in children. The treatment of craniopharyngiomas includes surgery as well as radiotherapy. The goal of surgery varies according to the tumor location and extension and may range from complete resection to biopsy. Surgical complications are well known and cause constant evaluation of surgical strategies. Diencephalic obesity is related to surgical manipulation of hypothalamic tissue. Therefore, a classification system for craniopharyngiomas based on preoperative MRI is suggested by the authors. Recurrences are frequent in craniopharyngiomas, even after complete or gross-total resection. Radiotherapy is therefore recommended to patients with incomplete resections. However, the ideal time for radiotherapy after surgery is under discussion. The treatment of craniopharyngiomas requires an interdisciplinary and multimodal approach. Each patient should receive an individually tailored treatment. Surgically, different approaches as well as different degrees of resection can be considered, depending on tumor location and tumor extension.

/pdf/surgical-strategies-in-childhood-craniopharyngioma-3xuyh78dda.pdf

Surgical strategies in childhood craniopharyngioma.

To investigate the presence and possible function of ether-a-go-go-related gene (erg) K+ channels in human lactotroph cells (HERG channels), primary cultures were prepared from human prolactinoma tissue. In almost all primary cultures, HERG currents could be recorded in identified prolactin cells using an external high-K+ solution. The antiarrhythmic agent E-4031, a specific blocker of erg channels, served to isolate HERG currents as the drug-sensitive currents. In cells of two tumours tested, thyrotropin-releasing hormone significantly reduced the amplitude of the HERG currents. The potential dependence of HERG current availability and the deactivation kinetics differed significantly even between prolactin cells derived from one adenoma. For comparison, corresponding values were obtained for heterologously expressed rat erg1, erg2 and erg3 channels. The expression of the three HERG channel subunits was investigated in nine human adenomas using RT-PCR. Transcripts for HERG1 were present in all adenomas and although transcripts for HERG2 and HERG3 were also detected, their expression level was more variable. The results demonstrate the functional expression of HERG channels in human prolactin-secreting tumours and are compatible with a physiological role for these channels in the control of prolactin secretion, as has been shown in normal rat lactotroph cells.

HERG K(+) currents in human prolactin-secreting adenoma cells.

Context: Pediatric somatotropinoma is uncommon but usually more aggressive than in adults, creating therapeutic challenges. No treatment guidelines are available. Objective

/pdf/clinical-features-and-treatment-of-pediatric-somatotropinoma-3kop8yjufm.pdf

Clinical features and treatment of pediatric somatotropinoma: case study of an aggressive tumor due to a new AIP mutation and extensive literature review.

The management of pituitary macroadenomas which lead to gigantism may require multiple therapeutical approaches, including medical treatment, surgery, and radiation therapy. Transsphenoidal surgery (TSS) during early childhood that achieves total removal of a growth hormone (GH) secreting tumor is rarely reported. The surgeon is confronted with special problems regarding the infantile anatomy. In this case, a 3.5 year old child, the youngest successfully treated by TSS so far, suffered from a GH- and prolactin (PRL) secreting macroadenoma of the pituitary gland. The girl initially presented with an increasing growth rate, later with breast development, and finally, at the age of 2.8 years, with galactorrhea and secretion of blood from the nipples. Increased levels of GH [122 μg/l], insulin-like growth factor (IGF-1) [830 μg/l], insulin-like growth factor binding protein 3 (IGFBP-3) [8.6 mg/l] and PRL [590 μg/l] were found. MRI scans revealed a macroadenoma of 2.7 cm diameter. An eight-week trial of relatively low dose dopamine agonists led to a reduction of PRL, while the GH- and IGF-1 levels remained unchanged; the tumor showed only little shrinkage. Since there was chiasma compression, we opted for early TSS. A complete tumor removal was achieved despite the difficulties of a narrow approach. After TSS, low levels of GH, IGF-1, and PRL documented a complete tumor removal, but persistent diabetes insipidus and anterior lobe deficits resulted from surgery. In summary, if primary medical therapy alone is unable to adequately reduce hormone hypersecretion and tumor size in early childhood, TSS is recommended. Thus, radiation therapy may be reserved for surgical failure.

Transsphenoidal surgery for pituitary gigantism and galactorrhea in a 3.5 year old child.

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Transsphenoidal surgery for pituitary gigantism and galactorrhea in a 3.5 year old child.