Combined Microsurgical, Endoscopic and Neuronavigation Assisted Transseptal-Transsphenoidal Resection of Pituitary Tumors

<p><strong>Objective. </strong>To describe the technical nuances of multimodal transseptal-transsphenoid surgery for pituitary tumors using a combination of microneurosurgery, neuroendoscopy, and electromagnetic neuronavigation.</p><p><strong>Materials and Methods. </strong>A transnasal approach to the sella is performed endoscopically and widely exposed by an otolaryngologic surgeon. Surgery is next performed by the neurosurgeon with microscope and neuronavigation for microsurgical resection of pituitary tumors. Neuroendoscope is also used at the end of surgery to confirm tumor resection and inspect operative site. During surgery, the patient’s head, angle and height of the microscope, and position of the table are repositionable to allow for multiple angle views. Abdominal fat harvested prior to the procedure is used to ensure cerebrospinal fluid seal.</p><p><strong>Results. </strong>The senior author (KIA) has used the combined approach with 84 consecutive patients. Radical resection was achieved in 66 patients, subtotal in 11, and partial in 7. There were no perioperative complications. Six patients experienced postoperative transient diabetes insipidus. The pituitary gland and stalk were preserved in all cases. Visual symptoms were improved in 78% and endocrinological symptoms in 56% of cases.</p><p><strong>Conclusion. </strong>This combined approach is safe and effective. It increases the efficacy and radicality of surgical resection, helps to preserve the pituitary gland, and improves and resolves preoperatively altered patient hormonal function and impaired vision. It also reduces complications, provides less postoperative pain and discomfort, reduces the surgery time, and enables a shorter hospital-stay.</p>

Xanthogranuloma of the sellar region accompanied by growth hormone deficiency: case report and literature review

Background Xanthogranuloma of the sellar region is a rare entity. Its pathology is controversial and it is difficult to strictly differentiate it from craniopharyngioma or Rathke’s cyst. Case presentation We report a case of xanthogranuloma accompanied by growth hormone deficiency in an 11-year-old girl. She did not show any other pituitary hormone deficiency or neurological symptoms before operation. The preoperative diagnosis was craniopharyngioma, but histological findings showed small areas of epithelium. Thus, the final diagnosis was xanthogranuloma. Xanthogranuloma is an important cause of growth delay. We reviewed 16 cases reported after 2000, and included our case, of xanthogranuloma in children. Conclusions Endocrinological symptoms are often regarded as one of the few apparent symptoms in xanthogranuloma compared with craniopharyngioma. Therefore, we should follow up carefully and accumulate cases.

A Histologically Diagnosed Case with Infantile Osteopetrosis Complicated by Hypopituitarism

Malignant infantile osteopetrosis is a rarely seen severe disorder which appears early in life with general sclerosis of the skeleton. It is caused by functionally defective osteoclasts which fail to resorb bone. Affected infants can exhibit a wide spectrum of clinical manifestations including impaired hematopoiesis, hepatosplenomegaly, visual impairment, and hypocalcemia. With the exception of secondary hyperparathyroidism, involvement of the endocrine system seems to be quite rare. Hypopituitarism is defined as underproduction of the growth hormone in combination with deficiencies of other pituitary hormones. Any lesion that damages hypothalamus, pituitary stalk, or anterior pituitary can cause secondary hypopituitarism. In this report, we presented a rare combination of malignant infantile osteopetrosis and secondary hypopituitarism in a newborn who presented predominantly with endocrinological symptoms. This is the first case report of malignant infantile osteopetrosis accompanied by hypopituitarism secondary to sclerosis of the sella turcica. On the other hand, this is a very interesting case which was diagnosed based on histological examination of bone marrow biopsy specimens despite lack of any clinical suspicion.

Variability of endocrinological dysfunction in 55 patients with X-linked adrenoleucodystrophy: clinical, laboratory and genetic findings

X-linked adrenoleucodystrophy (ALD) has been shown to be one of the most frequent causes of Addison's disease in men. It is characterized by an impaired peroxisomal beta-oxidation of very long chain fatty acids and is associated with mutations of the ALD gene resulting in a defective peroxisomal membrane transport protein. There is a striking variability of endocrinological and neurological symptoms in patients with ALD, with no clearly evident correlation between mutations of the ALD gene and the different neurological phenotypes. No data on endocrinological symptoms and the ALD genotype have been published so far. We report endocrinological, clinical, laboratory and molecular genetic data from 55 patients with ALD from 34 families. Endocrinological symptoms of adrenal insufficiency were observed in 33 patients, 20 of whom showed additional neurological symptoms of cerebral ALD or adrenomyeloneuropathy. Isolated neurological symptoms were seen in 12 patients; in nine patients there were neither endocrinological nor neurological symptoms. Mutations of the ALD gene (n = 28) were detected in 50 patients (including nine sets of brothers) from 32 families. No correlation was found between the ALD gene mutation and endocrinological dysfunction. However, we found that all sets of brothers were concordant for the endocrinological phenotype (cortisol synthesis was reduced in two sets and normal in seven sets), whereas four sets showed a discordant neurological phenotype. As yet unknown hereditary factors other than mutations within the ALD gene may interfere with the endocrinological phenotype more strongly than with the neurological phenotype of ALD.

Lipoma of the infundibulum

✓ Lipomas of the infundibulum are extremely rare and may not be recognized radiologically, even on computerized tomography (CT) scanning, if the tumor is less than 20 mm in diameter. A questionable CT diagnosis in the presence of endocrinological symptoms may justify an operation by microsurgical technique on the retrochiasmatic region. In the case reported, microsurgical removal of a small infundibular lipoma was successfully accomplished.