Domenico Billeci

Clinical presentation and outcome of pituitary adenomas in teenagers

objective Pituitary adenomas rarely occur in childhood and adolescence, but their mass effect and endocrine abnormalities can compromise both quality and length of life. In this study we evaluated the symptoms at onset and the long‐term consequences induced in teenagers by functioning or nonfunctioning pituitary adenomas.

Symptomatic Rathke's Cleft Cysts: A Radiological, Surgical and Pathological Review

Rathkes cleft cyst is a non neoplastic lesion remnant of the Rathkes poutch. Symptomatic cysts must be surgically treated. There are no specific neuroradiological features for this lesion. We present a series of 14 symptomatic Rathkes cleft cysts treated with transphenoidal endoscopic technique, and we correlate the MRI intensity images with the intraoperative findings, the pathological analysis, the growth pattern and the recurrence tendency, to understand wether there is a better way to recognize these lesions, especially the asyntomatic ones, that, despite other neoplastic cysts, colud only be observed with a MRI follow up.

Long-term effects of octreotide on markers of bone metabolism in acromegaly: Evidence of increased serum parathormone concentrations

The effects of octreotide on biochemical markers of bone turnover were evaluated in patients with active acromegaly. Serum GH, IGF-I and serum and urinary markers of bone metabolism were measured before and after 4 months of treatment in 27 patients (short-term treatment) and after 12 and 24 months of treatment in 15 patients (long-term treatment). In the short-term, octreotide significantly decreased the levels of serum GH, IGF-I, calcium, osteocalcin, carboxyterminal propeptide of type I collagen and alkaline phosphatase plus urinary excretion of calcium. Short-term treatment significantly increased serum parathormone levels (before treatment 30.1 ±9.57 and at 4 months 46.1 ±24.98 ng/L, p<0.001) and urinary excretion of phosphate; urinary excretion of hydroxyproline was unchanged. The same results were observed during long-term treatment, except that there was no significant difference of serum calcium and alkaline phosphatase levels before and after treatment. Parathormone concentrations were still higher at 24 months compared with those prior to treatment (before treatment 31.9±9.74 and at 24 months 44.9±21.18 ng/L, p<0.05). The changes of most bone markers during octreotide therapy can be explained by the decrease of serum GH and IGF-I concentrations. On the other hand, the rise of parathormone concentrations suggests that octreotide has ulterior and long-standing actions on calcium homeostasis: intestinal malabsorption of calcium due to the octreotide could contribute to this secondary hyperparathyroidism. The clinical consequences of these alterations of bone metabolism need to be further clarified.

Pituitary adenomas in childhood and adolescence. Clinical analysis of 10 cases

Pituitary adenomas in childhood and adolescence constitute 2–6% of all operated pituitary adenomas. We report the clinical features, treatment and follow-up of 10 pediatric patients affected by pituitary adenomas. All patients underwent clinical evaluation, endocrine tests, magnetic resonance imaging and visual field assessment. Follow-up ranged from 8 to 132 months (median 52.6). All patients were older than 10 years of age; 60% were males. In 50% the initial complaints were headache and/or visual impairment, all except one had clear evidence of endocrine dysfunction. Ninety percent were macroadenomas. According to hormone measurements and immunostaining 50% were prolactinomas, 20% were pure GH-se-creting and 30% were non-functioning adenomas. Prolactinomas in two females were successfully treated with cabergoline. The other patients un-derwent surgery: three prolactinomas are still being treated with dopamine agonists and a GH-secreting adenoma is being treated with octreotide LAR and cabergoline. Two patients were also treated with conventional radiotherapy. Treatments were completely successful in 50% of patients: these have normal hormone secretion, full pubertal development, no significant tumor mass and normal visual field. Hypersecretion of prolactin persists in two cases; partial or complete hypopituitarism is present in four, relevant tumor remnant in another four and impairment of visual field is present in two cases. In conclusion, pediatric adenomas occur mostly in pubertal age, are prevalently macroadenomas and clinically functioning. Medical therapy should be preferred for secreting adenomas, but in some cases, notably prolactinomas in males, surgery and eventual radiotherapy may be needed.

111Indium-pentetreotide pituitary scintigraphy and hormonal responses to octreotide in acromegalic patients

GH secreting pituitary adenomas are frequently visualized by scintigraphy with the somatostatin analogue 111Indium-pentetreotide. We studied 111Indium-pentetreotide scintigraphy and hormonal responses to octreotide in 12 acromegalic patients. Nine patients with active acromegaly were studied before pituitary adenomectomy; 6 of these and 3 other patients were studied after operation. GH was measured after a single sc dose of 100 µg of octreotide (acute test). The patients were preoperatively treated with 100 µg sc tid octreotide for 3 months as were patients who had been unsuccessfully operated; GH and IGF-I were measured at the end of this period (chronic treatment). A decrease of the hormones higher than 50% of basal values was considered a positive response in both acute test and chronic treatment. Eight/nine unoperated patients had a pituitary adenoma visualized by scintigraphy and a positive response to both the acute test and chronic treatment; one patient had no evidence of tumor at scintigraphy and he did not respond to octreotide. Scintigraphy was negative in all of the three patients cured by surgery. Six patients still had active disease after adenomectomy: scintigraphy was positive only in one case, although GH responded to octreotide treatment in all patients. Conclusions. 111In-pentetreotide scintigraphy frequently visualizes pituitary adenomas and predicts GH responses to octreotide in unoperated acromegalic patients. In unsuccessfully operated patients scintigraphy is infrequently positive and does not predict which patients will respond to octreotide. These data and the cost of 111In-pentetreotide scintigraphy do not warrant its extensive clinical use in acromegaly.

Pituitary metastasis of Merkel cell carcinoma

Merkel cell carcinoma (MCC) is a malignant neuroendocrine tumor of the skin that demonstrates a remarkable tendency to metastasize. However, only a few cases of MCC brain metastases have been reported in the literature. We here present a unique case of a pituitary metastasis of MCC in a 65-year-old patient with a history of pituitary adenoma. This case is particularly novel due to the fact that the primary site of the MCC is unknown.

Spindle cell oncocytoma: Report of two cases with massive bleeding and review of the literature

Spindle cell oncocytoma (SCO) is a rare pituitary tumor, classified as a WHO grade I neoplasm. Due to its rarity, SCO is often preoperatively misdiagnosed as a pituitary macroadenoma. In the present study we report two recent cases of SCO, a 61-year-old male and a 65-year-old female presenting at Treviso General Hospital between March 2014 and April 2015. Tumor resection was achieved by endoscopic transsphenoidal approach but massive hemorrhagic events hampered surgery, endangering the patients life in both cases. Both tumors featured fascicles of spindle cells with eosiniphilic cytoplasm expressing vimentin, S-100 and thyroid transcription factor-1 (TTF-1). The diagnosis of SCO was confirmed by second opinion in both cases. Extensive review of available literature, about 30 cases from 2002 to 2015, provided valuable clinical data for preoperative diagnosis and surgical removal of SCO tumors.

Banked Fascia Lata in Sellar Dura Reconstruction after Endoscopic Transsphenoidal Skull Base Surgery.

Objectives Cerebrospinal fluid (CSF) leakage is an undesirable complication of transsphenoidal skull base surgery. The issue of the most appropriate sellar dura repair remains unresolved, although a multilayer technique using autologous fascia lata is widely used. We describe the novel application of a homologous banked fascia lata graft as an alternative to an autologous one in the reconstruction of sellar dura defects in endoscopic transsphenoidal surgery. Design The clinical records of patients who underwent endoscopic transsphenoidal surgery at our department from June 2012, when we started using homologous fascia lata, up to July 2014 were reviewed retrospectively. The data concerning diagnosis, reconstruction technique, and surgical outcome were analyzed. Results We treated 16 patients successfully with banked fascia lata. Twelve patients presented intraoperative CSF leakage, and four patients were treated for postoperative rhinoliquorrhea. Banked fascia lata was used in a single-to-multilayer technique, depending on the anatomical features of the defect and of the sellar floor. No complications or failures in sella reconstruction occurred. Conclusion A banked fascia lata graft proved reliable and safe in providing an effective sellar dura reconstruction. Used in a multilayer strategy, it should be considered a viable alternative to an autologous fascia lata graft.