Somatostatin receptor expression and clinical outcome of multilineage pituitary tumours expressing PIT1 and SF1.

The application of transcription factor immunohistochemistry to pituitary neuroendocrine tumour (PitNET) assessment has allowed identification of tumours that do not conform to a single lineage. Multilineage pituitary transcription factor 1 (PIT1) and steroidogenic factor 1 (SF1) PitNETs are a rare and relatively newly described tumour subtype. These tumours express both transcription factors and may also express combinations of hormones corresponding to both lineages. Histological and clinical characteristics can vary, and overall clinical behaviour and prognosis is not known. We describe the clinical outcomes and somatostatin receptor status (SSTR) of a series of nine cases identified from our cohort of pituitary tumours at Westmead Hospital. Eight PitNETs (88.9%) expressed growth hormone and caused acromegaly at presentation. Of the seven macrotumours that caused acromegaly, one had cavernous sinus invasion. The Ki-67 labeling index score ranged from 0.6% to 3.6%. About 88% of tumours that secreted excess growth hormone exhibited strong immunostaining for SSTR 2 and all tumours displayed weak immunoreactivity for SSTR5. In 62.5% of patients with acromegaly, cure was achieved after surgical resection. Somatostatin receptor ligands resulted in clinical remission in cases where medical treatment was initiated. There was no new tumour recurrence or regrowth over an overall mean follow-up period of 62.5 months.

Antemortem histopathology and imaging findings in a case of Marchiafava-Bignami disease.

Marchiafava-Bignami disease (MBD) is a rare condition often associated with chronic alcohol abuse. Clinical presentation is diverse. Characteristic magnetic resonance imaging (MRI) changes in the corpus callosum are the mainstay of radiological diagnosis. We present a case of a 54-year-old man with chronic alcoholism and peripherally enhancing lesion in the body of the corpus callosum on MRI Brain. Open biopsy of the lesion showed necrosis and demyelination. He was diagnosed with Marchiafava-Bignami disease based on clinical, radiology and histopathology findings. Our case represents the only case in the literature with antemortem histopathology findings describing MBD.

An alternative technique for pituitary tumour removal: Sequential endoscopic-microscopic pituitary procedure (SEMPP).

Several authors have reported advantages of the purely endoscopic (PE) approach over traditional purely microscopic (PM) techniques for many sinus and anterior skull base procedures. However, in pituitary surgery, the PE approach carries a number of limitations. Experienced microscopically-trained neurosurgeons are required to master the endoscopic technique which has a steep learning curve due to its one-handed nature and optically-distorted 2-dimensional view. We describe our novel technique, the sequential endoscopic and microscopic pituitary procedure (SEMPP) which does not require microscopically-trained neurosurgeons to alter their technique. We compare SEMPP with the PE approach in terms of outcome and safety. Retrospective chart review of consecutive SEMPP cases performed at our institution between January 2010 and December 2013 was conducted. Operative time, gross total resection rate, resolution of endocrine and visual dysfunction, hospital length of stay, cerebrospinal fluid (CSF) leak rate and revision rates were recorded. 32 patients were identified (50% female, mean age 53.0 years), and 33 SEMPP cases. Mean operating time was 132 min (range 90-200). 69% of patients experienced gross total resection. Most patients (81.3%) with preoperative visual deficit either experienced complete resolution or improved symptoms. The remainder experienced no change in vision. 12.5% (n = 4) of patients experienced intraoperative CSF leaks. All were repaired intraoperatively or with conservative management. Two patients (6.3%) experienced epistaxis managed with conservative measures. The SEMPP technique demonstrates comparable outcomes, complication rates and operative time to PE and PM techniques described in the literature.

Primary nerve repair following resection of a neurenteric cyst of the oculomotor nerve.

Neurenteric cysts are rare congenital lesions of endodermal origin occurring in the spinal canal and infrequently in the posterior cranial fossa. The authors report the case of a 3-year-old child who presented with a recurrent third cranial nerve palsy. Magnetic resonance imaging showed a large cystic mass lesion in the ambient cistern on the right side, with compression of the anterolateral aspect of the brainstem. The patient underwent a craniotomy, complete excision, and a primary third cranial nerve repair. While there have been 3 reported cases of neurenteric cysts arising from the oculomotor nerve, this is the first documented case with a primary nerve repair.