A rare case of acquired ptosis in a child secondary to Pituitary macroadenoma

Pituitary adenomas are rare neoplasms with a propensity for unusual presentations and an aggressive clinical course. An 8 years old female child presented to Department of Ophthalmology at SVS Medical College and Hospital Mahabubnagar with the chief complaints of mild ptosis of RE since 1month, associated with fever, headache, vomiting and diplopia since 2 days. On ocular examination, BCVA in BE - 6/9 and anterior segment examination suggested RE mild ptosis with hypotropia and adduction restriction. Fundus examination was normal. There was no palpable mass/ bruit in the orbit. MRI revealed lobulated enhancing soft tissue lesion in sella and parasellar region extending into basifrontal region causing pressure over optic chiasm, 3rd ventricle and cavernous sinuses suggestive of pituitary macroadenoma. Hormonal essays revealed elevated prolactin, beta HCG levels and abnormal thyroid profile. On the basis of clinical examination and investigations a clinical diagnosis of pituitary macroadenoma was made. Then the case was referred to department of neurosurgery where transphenoidal resection under GA was planned and after 2days of admission she had a respiratory arrest and could not be resuscitated.

Apoplejía hipofisaria, presentación de dos casos clínicos y revisión del tema / Pituitary apoplexy, presentation of two clinical cases and review of the subject

Antecedentes: la apoplejía hipofisaria es un síndrome clínico agudo, potencialmente fatal, que acontece como complicación de un adenoma hipofisario; presenta una complejidad diagnóstica, y puede imitar numerosos procesos intracraneales. Objetivo: realizar la revisión bibliográfica del tema, y describir dos casos clínicos, dada la poca frecuencia de presentación de la enfermedad y su complejidad diagnóstica. Desarrollo: caso 1, paciente masculino, de 52 años con antecedentes patológicos de hipertensión arterial y macroadenoma hipofisario no funcionante. Durante su seguimiento por Neurocirugía se detectó hiperprolactinemia, por lo que se inició tratamiento con bromocriptina a dosis progresiva, lo cual desencadenó cuadro agudo no definido. Tres meses después presentó nuevo cuadro agudo, que se diagnosticó como apoplejía hipofisaria; caso 2, paciente masculino, de 49 años, con antecedente de hipertensión arterial con tratamiento irregular. Fue ingresado en servicio de terapia intermedia con diagnóstico presuntivo de meningoencefalitis, y por estudios complementarios se llega al diagnóstico de apoplejía hipofisaria. Ambos casos llevaron tratamiento quirúrgico, con evolución favorable. Conclusiones: la apoplejía hipofisaria es una enfermedad que se presenta con poca frecuencia, por lo tanto, es importante la sospecha diagnóstica para una temprana identificación y tratamiento de la enfermedad(AU)

Background: pituitary apoplexy is an acute clinical syndrome, potentially fatal, that occurs as a complication of a pituitary adenoma. It presents a diagnostic complexity, and it can mimic numerous intracranial processes. Objective: to carry out the bibliographic review of the subject, and to describe two clinical cases given the infrequency of presentation of the disease and its diagnostic complexity. Development: case 1, male patient, 52 years old with pathological history of arterial hypertension and non-functioning pituitary macroadenoma. During follow-up by Neurosurgery, hyperprolactinemia was detected, so treatment with bromocriptine was started with a progressive dose, which triggered undefined acute symptoms. Three months later he presented new acute symptoms, which was diagnosed as pituitary apoplexy. Case 2, male patient, 49 years old, with a history of arterial hypertension under irregular treatment. He was admitted in the intermediate therapy service with a presumptive diagnosis of meningoencephalitis, and by complementary studies the diagnosis of pituitary apoplexy was reached. Both cases led to surgical treatment, with favorable evolution. Conclusions: pituitary apoplexy is a disease that occurs infrequently, therefore, diagnostic suspicion is important for early identification and treatment of the disease(AU)

Pituicytoma with Significant Tumor Vascularity Mimicking Pituitary Macroadenoma

A 19-year-old man presented with bitemporal hemianopsia and was found to have a large sellar and suprasellar tumor, resembling a pituitary macroadenoma. Emergency transsphenoidal approach was attempted because of rapid visual deterioration with headache. However, the approach was complicated and stopped by uncontrolled hemorrhage from the tumor. After conventional cerebral angiography and recognition of an unusual pathology, transcranial approach was achieved to prevent permanent visual loss. The final pathological diagnosis was pituicytoma with epithelioid features. Pituicytoma is a rare low-grade tumor (WHO Grade I) of pituicytes involving the sellar and suprasellar region, and originating from special glial cells of the neurohypophysis. Because of the high vascularity, the firm consistency, and invasion to surrounding neurovascular structures, a pituicytoma should be included in the differential diagnosis of a mass in the sellar and suprasellar area if the tumor shows high enhancement with vascular components. We report a case of rare pituicytoma mimicking a pituitary macroadenoma with massive hemorrhage to disturb surgery.

Factors Influencing Disconnection Hyperprolactinemia and Reversal of Serum Prolactin after Pituitary Surgery in a Non-Functioning Pituitary Macroadenoma

Background: To investigate factors influencing disconnection hyperprolactinemia, including tumour volume, degree of pituitary stalk displacement and extent of tumour growth based on a modified Wilson-Hardy classification in a non-functioning pituitary macroadenoma and to confirm reductions in serum prolactin levels after endoscopic transphenoidal surgery. Methods: This prospective, descriptive study was conducted in the Department of Neurosurgery, General Hospital Kuala Lumpur from Jan 1, 2011 to Jan 1, 2013. Forty patients fulfilling the inclusion criteria were enrolled. All patients underwent endoscopic transphenoidal resection of non-functioning pituitary macroadenoma. Pituitary stalk angle, tumour volume and extent of tumour growth were measured from Magnetic Resonance Imaging (MRI) pre- and post-operatively. These variables were compared to serum prolactin levels measured pre and post operatively. SPSS 21 was used to perform statistical analyses. Results: In 40 patients, the mean tumour volumes were 10.58 cm3 (SD 7.81) pre-operatively and 3.1 cm3 (SD 3.45) post-operatively. There was a 70% reduction in tumour volume post-operatively (P < 0.01). The mean serum prolactin was 457 mIU/L (SD 66.93) pre-operatively and 297 mIU/L (SD 6.73) post-operatively. There was a 65% reduction in prolactin serum levels after surgery (P < 0.01). The mean pituitary stalk angles were 93.45 ± 3.89 degrees pre-operatively and 51.45 ± 1.46 degrees post-operatively (P = 0.01). The mean pituitary stalk angle in the control group was 50.4 ± 8.80 degrees. Hence, there was a 98% reduction in pituitary stalk angle after surgery (P < 0.01). This study showed a linear correlation between the pre-operative and post-operative tumour volumes and serum prolactin levels (P = 0.01 pre-and post-operative) and between serum prolactin levels and pituitary stalk angle (P = 0.20 pre-operative; P = 0.01 post-operative). Conclusion: Tumour volume and pituitary stalk angle displacement have positive predictive values for disconnection hyperprolactinemia in non-functioning pituitary macroadenoma. However, a larger sample size and further objective studies are needed to confirm these findings.

Analysis of MR findings of misdiagnosed cases with pituitary macroadenoma / 实用放射学杂志

Objective To investigate MR findings and analyze the misdiagnosed cases of pituitary macroadenoma.Methods MR features of 1 90 patients of pituitary macroadenoma confirmed by operation and pathology were reviewed.Results 1 6 cases were mis-diagnosed as craniopharyngioma,chordoma,or meningioma.Among the sixteen cases,eight cases with cyst degeneration and hemor-rhage were misdiagnosed as craniopharyngioma;five cases with clival and sphenoid sinus destruction were misdiagnosed as chordo-mas;three cases with suprasellar and anterior cranial fossa extension were misdiagnosed as meningiomas.Conclusion Craniophar-yngioma,chordoma and meningioma should be considered in the differential diagnosis of atypical pituitary macroadenoma.The com-prehensive analysis should be based on a variety of signs.

Primary hypoparathyroidism and non-functioning pituitary adenoma: An incidental coexistence? / Journal of the ASEAN Federation of Endocrine Societies

@#Primary hypoparathyroidism is caused by a group of heterogeneous conditions in which hypocalcemia and hyperphosphatemia occur as a result of deficient parathyroid hormone (PTH) secretion. The most common cause is surgical excision and damage to the parathyroid gland(s). Nonetheless, autoimmune endocrine disorder of primary hypothyroidism has been well-described in polyglandular autoimmune syndromes (PAS).1 Its association with pituitary lesion may be autoimmune lymphocytic hypophysitis as the cause for pituitary disorder. In this report, we encountered a patient with primary hypoparathyroidism who had a non-functioning pituitary tumour. It was confirmed as pituitary adenoma rather than lymphocytic hypophysitis from the histopathological examination. To our knowledge, this is the first reported case of non-functioning pituitary macroadenoma and primary hypoparathyroidism.

Large Intracranial Aneurysm after Transsphenoidal Surgery for Pituitary Macroadenoma

Uncontrolled cerebrospinal fluid (CSF) leakage after transsphenoidal surgery (TSS) for pituitary adenoma can lead to meningitis. Intracranial mycotic pseudoaneurysm is a rare complication in central nervous system infection. Large single pseudoaneurysm is more uncommon. Most mycotic aneurysms occur due to endocarditis. The present patient had no heart problem and was infected by CSF leakage after transsphenoidal surgery. We present a case of large ruptured mycotic pseudoaneurysm as a complication of cerebral infection after TSS for pituitary macroadenoma.

Inappropriate secretion of thyroid stimulating hormone in a Filipino patient with a pituitary macroadenoma / Journal of the ASEAN Federation of Endocrine Societies

Pituitary tumors producing thyrotropin are very rare. We report a case of a 38-year-old, male admitted for seizure, preceded by 1 week history of generalized weakness, fatigue, anorexia, nausea and vomiting, with a 5 year history of recurrent headache followed by blurring of vision, who was found to have persistently elevated serum free thyroid hormones and non-suppressed thyroid stimulating hormone (TSH) levels, with tumor residual after craniotomy with excision of the pituitary macroadenoma. To our knowledge, this is the first reported case of TSH-producing adenoma in the Philippines.

The Volume of Tumor Mass and Visual Field Defect in Patients with Pituitary Macroadenoma

PURPOSE: We used the Swedish interactive threshold algorithms (SITA) standard strategy of Humphrey perimetry, to analyze the pattern of visual field (VF) defects and evaluate the quantitative correlation between the tumor volume and severity of VF defects in patients with pituitary macroadenoma. METHODS: We reviewed 50 patients with pituitary macroadenoma who received VF test and 11 patients were excluded. VF analysis was performed with Humphrey perimeter using the SITA standard strategy. The tumor volume was assessed radiologically via brain magnetic resonance images and was calculated using Cavalieri's principle. We used the mean deviation (MD) and pattern standard deviation (PSD) of the Humphrey parameter to measure VF defect severity, and then analyzed the correlation of tumor volume with VF defects. RESULTS: Twenty nine patients (74%) showed abnormal VF and bitemporal field changes, which were the most common field defects on presentation. Seven patients (18%) had unilateral VF defects, 22 patients (56%) had bilateral VF defects. The tumor volume of the patients with VF defects was significantly larger than that of patients with normal VF (p = 0.006). The tumor volume exhibited significant negative correlation with MD (r = -0.693; p < 0.001) and significant positive correlation with PSD (r = 0.589; p < 0.001). CONCLUSIONS: In patients with pituitary macroadenoma, there was a variety of VF defects and a high correlation between the tumor volume and the severity of VF defects. SITA standard strategy can be a fast and quantitative method for evaluating central VF defects.

Comparison of clinical and MRI characteristics of pituitary macroadenoma between different genders / 中国医学影像技术

Objective To compare the clinical and MRI characteristics of pituitary macroadenoma between male and female patients. Methods Two hundred and thirty patients with pathologically diagnosed pituitary macroadenomas (104 mlaes and 126 females) were divided into two groups according to different gender. Patient age, the ratio of intratumoral hemorrhage and cavernous sinus invasion, and tumor volume were calculated in each group and compared between the two groups. The constitution of different pathological types was also compared. Results The age, ratio of intratumoral hemorrhage and cavernous sinus invasion, and tumor volume was (44.8±13.2) years old, 21.15% (22/104), 50.00% (52/104), and 6100.48 (mm3, median) in the male group and (44.8±11.9) years old, 28.57% (36/126), 48.41% (61/126) and 5037.05 (mm3, median) in the female group, respectiveluy. There was no statistical difference between the two groups regarding to patient age, the ratio of intratumoral hemorrhage and cavernous sinus invasion, tumor volume, and constitution of pathological types (P=0.972, P=0.197, P=0.811, P=0.189, P=0.093, respectively). Conclusion Pituitary macroadenomas show no gender preference in clinical and MRI characteristics.

Panhipopituitarismo secundario a macroadenoma hipofisario no funcionante en la adolescencia

Objetivo: Presentar el caso poco frecuente de una adolescente con un macroadenoma hipofisario no funcionante que le produjo un hipopituitarismo. Se hace una revisión de la literatura. Caso Clínico: Adolescente femenina de 16 años 2 meses de edad quien presenta poco progreso en talla y ausencia de caracteres sexuales secundarios; refiere concomitantemente cefaleas ocasionales. Al examen físico presenta talla y peso por debajo del pc 3, velocidad de crecimiento 2,8 cm/año, cubitus valgus, paladar ojival, cuarto metacarpiano corto, no tiromegalia. Genitales, mamas, vello axilar y púbico Tanner I. Se hace diagnóstico de talla baja patológica y retraso puberal y se indican exámenes de paraclínicos. Los resultados de laboratorio muestran un déficit de hormona de crecimiento (GH) y un hipocortisolismo, con función tiroidea conservada. Edad ósea de 11 años para una edad cronológica de 15 años. Cariotipo 46,XX. Rx lateral de cráneo muestra silla turca amplia y excavada. Us pélvico: útero en anteversion, central, con longitud de 20 mm; no se visualizan línea endometrial ni ovarios. La RMN muestra el piso de la región selar deformado, excavado, con imagen compatible con LOE de contornos más o menos definidos con señal intermedia baja y dishomogénea en T1, con realce hipertenso dishomogéneo tras la administración del medio de contraste, de aproximadamente 2,9 x 1,36 cm. de diámetro, con compromiso de los elementos supraselares, ejerciendo efecto compresivo sobre el infundíbulo el cual deforma el quiasma óptico. Se hace diagnóstico de Panhipopituitarismo secundario a Macroadenoma Hipofisario no Secretante, con Déficit de GH, Hipogonadismo Hipogonadotropo e Insuficiencia Suprarrenal Secundaria (Déficit de ACTH). Se indica tratamiento con hidrocortisona por vía oral. Se refiere para resolución quirúrgica por vía transesfenoidal ya que en nuestro centro no se realiza. Se desconoce el reporte histológico del tumor y la evolución de la paciente. Conclusiones: Los adenomas hipofisarios representan menos del 2-3% de todos los tumores intracraneales y sus manifestaciones clínicas dependen de la suma de un efecto masa, que causa alteraciones neurológicas, y la afectación de la secreción hormonal, ya sea por exceso o por defecto. Es importante realizar un diagnóstico temprano, así como un tratamiento efectivo y seguimiento a largo plazo.

Objective: To present the uncommon case of a teenager with a non-functioning pituitary macroadenoma that resulted in a hypopituitarism. A literature review is done. Case Report: Female adolescent of 16 years 2 months of age who presents little progress in stature and absence of secondary sexual characters; concomitantly refers occasional headaches. Physical examination: height and weight below the 3 pc, growth velocity 2.8 cm/year, cubitus valgus, ojival palate, short fourth metacarpal, no thyroid enlargement. Genitals, breasts, axillary and pubic hair, Tanner I. The diagnosis of pathological short stature and delayed puberty is done and paraclinical examinations are indicated. Laboratory results show a deficit of growth hormone (GH) and a hypocortisolism with preserved thyroid function. Bone age of 11 years for a chronological age of 15 years. Karyotype 46, XX. Rx of the skull shows a wide and excavated sella turcica. Pelvic Us: central uterus, in anteversion, 20 mm in length and the endometrium and ovaries were no visible. NMR shows the floor of the sellar region warped, carved, with a image compatible with tumour of defined contours, with low-intermediate and dishomogenea signal on T1, with hypertensive and heterogeneou enhancement after administration of contrast medium, approximately 2.9 x 1.36 cm. in diameter, with involvement of the supraselares elements exerting compressive effect on the infundibulum, which deforms the optic chiasm. The diagnosis of panhypopituitarism secondary to pituitary non functioning macroadenoma is done, with GH deficiency, hypogonadotropic hypogonadism and secondary adrenal insufficiency (ACTH deficiency). Treatment with oral hydrocortisone is indicated. The patient is referred for resolution by transsphenoidal surgery. The histological report of the tumor and the evolution of the patient are unknown. Conclusions: Pituitary adenomas account for less than 2-3% of all intracranial tumours, and their clinical manifestations depend on the sum of a mass effect, which causes neurological disorders, and the disruption of hormonal secretion, either up or down. It is important to make an early diagnosis, an effective treatment and long-term monitoring.

A Case of Pituitary Macroadenoma Accompanied with CRH Deficiency / 대한내분비학회지

Pituitary tumor can be accompanied with various pituitary hormone abnormalities. Pituitary tumors can be divided into functioning or nonfunctioning tumors. A functioning pituitary tumor, via the oversecretion of pituitary hormones, causes diverse clinical features. A nonfunctioning pituitary tumor can be accompanied with pituitary dysfunction and this may be due to compression or destruction of normal pituitary tissue, suppression of the pituitary portal system or direct damage to the hypothalamus. Corticotropin-releasing hormone (CRH) deficiency, which is caused by defects in the synthesis or release of CRH, is a cause of secondary adrenocortical insufficiency. The clinical presentations are hypoglycemia, weight loss, anemia, weakness, nausea, vomiting and hyponatremia. Acquired CRH deficiency has also been suggested to occur based on a lack of adrenocorticotropic hormone (ACTH) response to insulin-induced hypoglycemia, but there is a normal ACTH response to exogenous CRH. We experienced a case of a woman with pituitary macroadenoma accompanied with CRH deficiency. We report here on this case with the review of the literature.

A Case of Normal Vaginal Delivery in a Hyperprolactinemia Patient with Pituitary Macroadenoma / 대한산부인과학회지

Prolactin-secreting adenomas are the most common pituitary tumors resulting in hyperprolactinemia which is one of the most important causes of female infertility with amenorrhea or galactorrhea. Although it is reported that spontaneous pregnancy occured in these patients using bromocriptine treatment or ovulation induction, it is very rare to find a pituitary tumor during a pregnancy by having neurological symptoms of headache, diplopia or visual disturbance. We experienced a case of normal delivery after symptom improvement and maintenance of pregnancy by administration of bromocriptine in a 27 year-old primigravida with diplopia and visual disturbance due to prolactin-secreting pituitary macroadenoma, which is diagnosed by brain MRI scan and serum prolactin level. A brief review of related literature was done.

Transsphenoidal microsurgery for pituitary macroadenomas / 中国微创外科杂志

85%) in 18 patients (21.4%) and a partial resection (

MRI follow-up after transsphenoidal surgery in patients with nonfunctional pituitary macroadenoma / 中国微创外科杂志

Objective To study the optimal time of MRI follow-up after transsphenoidal surgery in patients with nonfunctional pituitary macroadenoma. Methods MRI records of 50 patients before surgery,within 1 postoperative week (early stage),at 3 months (intermediate stage) and 1 year after surgery (late stage),respectively,were retrospectively analyzed.Dynamic changes in the sella turcica were observed and the degrees of tumor excision were studied before and after MRI T 1-weighted enhanced scans at different postoperative stages. Results In the early stage MRI images showed that the size of sellar contents decreased by 8%～32%,tumor disappeared in 22 cases,and suspected residual tumor was found in 28 cases.At 3 months after surgery,MRI scans revealed that sellar contents decreased by 11%～85% in size.On coronal position MRI scans,the size of sellar contents decreased more than 50% in 11 cases,30%～50% in 9 cases,less than 30% in 8 cases.Out of the 28 cases that were suspected of residual tumor in the early stage,confirmation of residual tumor in the sella turcica was made in 23 cases.MRI findings 1 year after surgery showed no change in the sella turcica in 46 cases and continuing to decrease in 4 cases. Conclusions Re-examination of MRI in intermediate stage after transsphenoidal surgery in patients with nonfunctional pituitary macroadenoma facilitates the detection of residual tumor and recurrence.

A Case of Thyrotropin - Secreting Pituitary Adenoma with Normal alpha-subunit / TSH Molar Ratio / 대한내과학회지

Thyrotropin(TSH)-secreting pituitary adenoma is a rare disorder causing hyperthyroidism, which is one of the syndrome of inappropriate secretion of TSH. It is characterized by high serum T4, T3 as well as elevated serum TSH. Generally serum free alpha-subunit concentration is also increased and alpha- subunit/TSH molar ratio is more than 1. This alpha- subunit/TSH molar ratio is a clue of diagnosis as well as a useful marker of therapeutic response. We experienced a case of 29-years old man with hyperthyroidism due to TSH-secreting pituitary adenoma. He was underwent 1.5cm sized pituitary tumor removal via transsphenoidal approach in our neurosurgery department. In immunohistochemical stain monotonous tumor cells showed strong positive reaction to antihuman TSH antibody and equivocal reaction to ACTH antibody. After operation, goiter size was progressively decreased and also serum T4, T3 and TSH were decreased in nearly normal range. However, he showed elevated serum T4, T3 and TSH after 1 month due to residual tumor. So he received radiation therapy thereafter. In this case the alpha-subunit and alpha-subunit/TSH molar ratio were not increased. So we report a case of TSH-secreting pituitary macroadenoma which had low alpha-subunit/TSH molar ratio with a literature review.

Clinical Analysis of Pituitary Macroadenoma by Transsphenoidal Approach

The authors report the results of transsphenoidal approach(TSA) for pituitary macroadenoma performd over a 2-year period.This series consisted of 15 patients:ten for nonfunctioning, two for prolactinoma, two of cushing's disease, one of acromegaly. The presenting symptom & sign was hyperfunctioning endocrinopathy in 5 patients, headache in 11 patients, visual symptom in 11 patients. TSA was performed without lumbar catheterization and fluoroscopic mornitoring. Success rates were as follows;normalization of endocrinopathy was achieved in 100% of cases;improvement of visual field in 100% of cases. There were no surgical mortality & some complication with three transient diabetes inspidus one hypopituitarysm. Eight of 15 patients who were removed tumor subtotally were received radiotherapy. Althrough there were short-term follow up(mean 19 months), there was no recurrence. This review reconfirms that the transspenoidal approach is safer and more effective procedure than craniotomy for pituitary macroadenomas.