Ghazi AA, Rotondo F, Kovacs K, et al. "Treatment of invasive silent somatotroph pituitary adenoma with temozolomide. Report of a case and review of the literature." Endocr. Pathol.. 2015;26(2):135-9. Abstract

Improved imaging techniques have contributed to increased diagnosis of pituitary tumors. These tumor types can be microadenomas or macroadenomas and can either be functional or non-functional. Atypical or aggressive pituitary adenomas are tumors that rapidly increase in size and may invade into the suprasellar or parasellar regions. They are characterized by a Ki-67 nuclear labeling index greater than 10 %. Management of these tumors is difficult, and many recur after surgery. Temozolomide, a second generation alkylating agent, has been showing promising results in the treatment of these tumors. The patient was a 39-year-old male diagnosed with an invasive silent somatotroph pituitary macroadenoma treated with temozolomide after surgery. We present the case along with the review of the literature of the therapeutic effects of temozolomide in somatotroph macroadenomas.

Fathalla H, Cusimano MD, Di Ieva A, et al. "Endoscopic versus microscopic approach for surgical treatment of acromegaly." Neurosurg Rev. 2015;38(3):541-8; discussion 548-9. Abstract

Transsphenoidal surgery in the setting of acromegaly is quite challenging due to increased soft tissue mass, bony overgrowth, and bleeding. There is a debate on the endoscopic versus microscopic approach for these patients. The purpose of our study is to compare the outcomes for acromegaly after transsphenoidal surgery using both techniques. Retrospective review of 65 acromegalic patients who underwent transsphenoidal surgery in our department. Clinical remission was defined as resolution of typical acromegalic symptoms. Radiological resection was defined by volumetric criteria, and biochemical remission was defined as by the 2010 consensus on the criteria for remission of acromegaly. There was no significant difference in age, preoperative endocrine status, percent of macro adenomas, suprasellar, or infrasellar extension between both groups. Patients were assigned to both groups based on our existing referral pattern. Endoscopic approach was performed in 42 patients, while the microscopic approach was performed in 23 patients. No significant difference in remission rates was found between both groups (45.2 vs. 34.7 %, p = 0.40). The endoscopic group, however, had a significantly higher rate of gross total resections (61 vs. 42 %, p = 0.05). There was also a trend towards higher rates of gross total resections when cavernous sinus was present (48 vs. 14.2 %, p = 0.09). Postoperative diabetes insipidus occurred more in microscopic patients (34.7 vs. 17 %, p = 0.05), otherwise there was no significant difference in rates of complications. The median follow-up period was 56.6 months (range 6-156, mean 66.1). There is no significant difference in the rates of biochemical remission between the endoscopic and microscopic techniques. The endoscope technique, however, seems to be superior in achieving gross total resection especially with tumors invading the cavernous sinus.

Fathalla H, Cusimano M, Di Ieva A, Karamchandani J, Fung R, Kovacs K. "Osteomalacia-Inducing Tumors of the Brain: A Case Report, Review and a Hypothesis." World Neurosurg. 2015;84(1):189.e1-5. Abstract

BACKGROUND: Osteomalacia-inducing tumors (OIT) are mesenchymal tumors that characteristically secrete fibroblast growth factor 23, resulting in a paraneoplastic syndrome of hypophosphatemic osteomalacia. These tumors are known to occur in soft tissues and bones in various sites. It is very unusual for OITs to occur intracranially, with only 10 reported intracranial cases since their discovery in 1959. The most common intracrainal OITs are phosphaturic mesenchymal tumors and hemangiopericytomas. We report a case of hypophosphatemic osteomalacia caused by a tumor in the right anterior cranial fossa. We also hypothesize, based on our review of the literature, that this entity is underdiagnosed.

CASE DESCRIPTION: A 49-year-old woman had a history of a nonhealing ankle fracture that required repeated surgery over 3 years. She subsequently was found to have severe hypophosphatemia and evidence of osteomalacia together with multiple occult fractures. A diagnosis of tumor-induced osteomalacia was suspected. An elevated serum fibroblast growth factor 23 level confirmed the diagnosis. An octreotide scan that was performed to locate the responsible tumor revealed an area of avid uptake in the right frontal lobe. Magnetic resonance imaging showed a large right anterior fossa extra-axial mass. The patient was referred for surgical intervention and was cured clinically after surgical removal of the tumor. Pathologic examination revealed a phosphaturic mesenchymal OIT. Her phosphate levels returned to normal 3 weeks after surgery.

CONCLUSIONS: The diagnosis of OIT should be considered in a case of severe hypophosphatemia and metabolic bone disease that is not explained by any other metabolic or hereditary disease. These tumors can occur intracranially and may be confused with a meningioma or a hemangiopericytoma. Taking OIT into consideration in such cases could lead to a shorter time to diagnosis and management, which in our case took 4 years.

Sameh S, Azzazi A, ElSayed A, Fathalla H. "Surgical Management of Penetrating Intracranial Bullet Injuries." Neurosurgery Quarterly. 2014.
Di Ieva A, Fathalla H, Cusimano MD, Tschabitscher M. "The indusium griseum and the longitudinal striae of the corpus callosum." Cortex. 2015;62:34-40. Abstract

In the eighteenth century, Lancisi described the indusium griseum (IG) and the longitudinal striae (LS) of the corpus callosum. The IG is a thin neuronal lamina above the corpus callosum, covered on each side of the midline by the medial and lateral LS. The medial LS (nerves of Lancisi) and lateral LS are two pairs of myelinated fiber bands found in the gray matter of the IG on the dorsal aspect of the corpus callosum. Embryologically, the IG and LS are dorsal remnants of the archicortex of the hippocampus and fornix and thus they are considered components of the limbic system. Recent studies using immunohistochemistry reported that acetylcholine, dopamine, noradrenaline, 5-hydroxytryptamine and GABA neurons innervate the IG. Newer imaging techniques, such as high field MRI and diffusion tensor imaging, provide new tools for studying these structures, whose true function remains still unclear. The present paper reviews the history of the discovery of the IG and LS of the corpus callosum, with a holistic overview on these interesting structures from the anatomical, embryological, neurochemical, radiological and clinical perspective.

Di Ieva A, Laiq S, Nejad R, et al. "Chordoid meningiomas: Incidence and clinicopathological features of a case series over 18 years." Neuropathology. 2014. Abstract

Chordoid meningioma (CM) is a rare subtype of meningioma, classified as grade II, which exhibits a high rate of recurrence following subtotal resection. We retrospectively examined nine cases of chordoid meningioma over a case series of 1743 meningiomas (0.52%) operated upon at our institution from 1995 to 2013. All the reported clinicopathological findings were analyzed. Two hundred and twenty-one CM cases have been published to date worldwide and few single-center large case series have been issued. Seventy-five percent of the cases that underwent subtotal resection at our institution had recurrence within 1 year. Total resection of the tumor should be the major objective of surgery to reduce the possibility of tumor recurrence. The percentage of chordoid features within the tumor specimen could assist in predicting the pathogenesis of the lesion. The correlation of the index of proliferation to recurrence rate is still controversial. Much debate exists with regard to the role of adjuvant radiotherapy in CM cases. Immunohistochemical, cytological and ultrastructural studies should be used in combination to assure a correct diagnosis of CM. Owing to the rare occurrence of this meningioma subtype, larger case series are required to assist in providing a reference for diagnosis and to improve the therapeutic management of CM.

Fathalla H, Cusimano MD, Alsharif OM, Jing R. "Endoscopic transphenoidal surgery for acromegaly improves quality of life." Can J Neurol Sci. 2014;41(6):735-41. Abstract

BACKGROUND: Acromegaly has important effects on quality of life (QOL). This is the first study to measure QOL in acromegalic patients after endoscopic transsphenoidal surgery (ETSS).

METHODS: We prospectively collected the RAND-36, Center for Epidemiologic Studies Depression (CES-D), and Pituitary QOL validated questionnaires and patients' demographics, clinical presentation, endocrine laboratory results, radiological studies, development of complications and remission rates from 20 consecutive acromegalic patients who had undergone endoscopic transphenoidal surgery.

RESULTS: The eleven females and nine males had an average age of 42 years; 90 percent had macroadenomas and 70% had cavernous sinus invasion on their preoperative imaging. Ninety percent had improved symptoms post-operatively and 80% stated that treatment improved their QOL. Biochemically, 35% were cured, 35% had discordant results and 30% were not cured, while pan-hypopituitarism occurred in 4 patients. Physical health subscales and pituitary-related symptoms were similar to norms. "Social," "emotional health," and "energy levels" were significantly lower than norms. Seventy percent stated that their relationship with their physician "very much so" affected their quality of life. Pan hypopituitarism and adjuvant therapy were the most significant predictors of lower QOL subscale scores.

CONCLUSION: Transsphenoidal surgery improves QOL in acromegaly. Attempts to achieve a cure, avoidance of surgically induced pan-hypotpituitarism and adjuvant therapy, will improve quality of life. Our study demonstrates the important role of the patient-physician relationship to QOL and the need to measure QOL in addition to the traditional measures of outcome.

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