Incidentalomas o no incidentalomas: ¿cuál es la relevancia de los adenomas hipofisarios en el adulto? / Incidentalomas or Non-Incidentalomas: What is the Relevance of Pituitary Adenomas in the Adult?

Introducción: desde el punto de vista anatómico, los adenomas hipofisarios (AH) se observan en el 10% de la población. Son en su mayoría pequeños y no funcionantes. La mayoría de los incidentalomas descubiertos en estudios de imágenes con alta resolución pedidos en situaciones clínicas frecuentes, como el traumatismo craneoencefálico, el accidente cerebrovascular y las demencias, corresponden a AH indolentes. Nos preguntamos cuál es la relevancia clínica de los adenomas hipofisarios. Desarrollo: los AH clínicamente relevantes son tumores en su mayoría benignos que conllevan, en diferentes proporciones, aumento en la morbilidad y/o mortalidad de los pacientes por mecanismos relacionados con la hipersecreción hormonal, la insuficiencia hormonal y/o los efectos de masa ocupante. La prevalencia de los AH clínicamente relevantes es mayor de la que se suponía hace 20 años. Afecta aproximadamente a 1/1000 habitantes. Los más prevalentes son los prolactinomas y los adenomas no funcionantes. La acromegalia, la enfermedad de Cushing y los tumores agresivos se traducen en pacientes complejos con mayor morbimortalidad. El diagnóstico temprano y el tratamiento multimodal proveen una razonable mejoría de la sobrevida. El estudio epidemiológico de los AH clínicamente relevantes es importante para la estimación del impacto en los sistemas de salud. Conclusiones: los estudios por imágenes de mejor resolución continuarán señalando incidentalomas hipofisarios. Una evaluación cuidadosa de los pacientes podrá identificar aquellos AH clínicamente relevantes. (AU)

Introduction: from the anatomical point of view, pituitary adenomas (HA) are observed in 10% of the population. They are mostly small and non-functioning. Most incidentalomas discovered in high-resolution imaging studies ordered in frequent clinical situations, such as head trauma, stroke and dementia, correspond to indolent HA. We wonder what is the clinical relevance of pituitary adenomas. Development: clinically relevant HAs are mostly benign tumors that lead, in different degrees, to an increased morbidity and/or mortality in patients by mechanisms related to hormone hypersecretion, hormone insufficiency and/or occupying mass effects. The prevalence of clinically relevant HA is higher from what was assumed 20 years ago. It affects approximately 1/1000 of the population. The most prevalent are prolactinomas and non-functioning adenomas. Acromegaly, Cushing's disease and aggressive tumors make for complex patients with increased morbidity and mortality. Early diagnosis and multimodal treatment provide a reasonable improvement in survival. Epidemiological study of clinically relevant HAs is important for estimating the impact on health systems. Conclusions: Higher-resolution imaging studies will continue to highlight pituitary incidentalomas. Careful evaluation of patients will identify clinically relevant HAs. (AU)

Huge Sellar Plasmacytoma as Differential Diagnosis of Invasive Pituitary Adenoma: A Case Report

Sellar plasmacytomas are rare tumors arising from plasma cells. They are often misdiagnosed as adenomas.We report the case of a 63-year-old woman with headache, cranial nerve III palsy and decreased visual acuity. Imaging revealed an extensive lesion centered on the clivus, extending to the cavernous sinus bilaterally and into the sphenoid sinus. The hormonal tests were compatible with panhypopituitarism and mild hyperprolactinemia. The first hypothesis was invasive pituitary adenoma. Partial resection was achieved, and the immunohistochemical evaluation was compatible with plasmacytoma. After a few weeks, she developed lumbar and hip pain, and the imaging confirming osteolytic lesions. The final diagnosis was multiple myeloma.

Apoplexy in sporadic pituitary adenomas: a single referral center experience and AIP mutation analysis

ABSTRACT Objective: To analyze the clinical, laboratory, and radiological findings and management of patients with clinical pituitary apoplexy and to screen for aryl hydrocarbon receptor-interacting protein (AIP) mutations. Subjects and methods: The clinical findings were collected from the medical records of consecutive sporadic pituitary adenoma patients with clinical apoplexy. Possible precipitating factors, laboratory data, magnetic resonance imaging (MRI) findings and treatment were also analyzed. Peripheral blood samples were obtained for DNA extraction from leukocytes, and the entire AIP coding region was sequenced. Results: Thirty-five patients with pituitary adenoma were included, and 23 (67%) had non-functioning pituitary adenomas. Headache was observed in 31 (89%) patients. No clear precipitating factor was identified. Hypopituitarism was observed in 14 (40%) patients. MRI from 20 patients was analyzed, and 10 (50%) maintained a hyperintense signal in MRI performed more than three weeks after pituitary apoplexy (PA). Surgery was performed in ten (28%) patients, and 25 (72%) were treated conservatively with good outcomes. No AIP mutation was found in this cohort. Conclusion: Patients with stable neuroophthalmological impairments can be treated conservatively if no significant visual loss is present. Our radiological findings suggest that hematoma absorption lasts more than that observed in other parts of the brain. Additionally, our study suggests no benefits of AIP mutation screening in sporadic patients with apoplexy.

Two Cases of Hypovascular Pituitary Metastases and Literature Review / 中国医学科学院学报

Pituitary metastasis,a rare kind of intracranial malignant tumor,is characterized by metastasis from all parts of the body to the pituitary.The common tumors metastatic to pituitary are abundant with blood supply,located in the posterior pituitary lobe and/or the pituitary stalk.The lesion shows infiltrative growth,and mainly demonstrates low signal on T1 weighted imaging and high signal on T2 weighted imaging.It is usually enhanced significantly after iodinated contrast administration.The metastatic tumor with poor blood supply is similar to pituitary macroadenoma on magnetic resonance image,which makes it difficult to be differentiated.We reported two cases of pathologically diagnosed pituitary metastasis with poor blood supply and reviewed related papers,aiming to provide the imaging differentiation points of hypovascular pituitary metastasis.

Non-functioning pituitary adenomas and pregnancy: one-center experience and review of the literature

SUMMARY The usual clinical presentation of non-functioning pituitary adenoma (NFPA) consists of symptoms of mass effect and hypopituitarism. NFPA is a rare condition in young women and an uncommon complication during pregnancy. We present the outcome of three patients with NFPA during pregnancy. Case 1: a 38-year-old woman was referred at 32nd week of spontaneous pregnancy because of diagnosis of a pituitary macroadenoma discovered in the context of progressive visual loss. Hormonal deficiency and hypersecretion were ruled out. Prolactin levels were high as expected. She developed diplopia and severe headache despite the use of dopamine agonists and corticosteroids, so pregnancy was interrupted at 34th week. After an uncomplicated delivery of a healthy newborn, transsphenoidal surgery was performed. The pathology was consistent with a gonadotroph adenoma. She recovered visual field, and remained with normal pituitary function. Postsurgical tumor remnant increased in size during the follow-up. Case 2: a 34-year-old woman was referred due to secondary amenorrhea and galactorrhea. A macroadenoma with suprasellar extension was discovered. Transsphenoidal surgery confirmed a gonadotroph adenoma. Two years after surgery she had a normal pregnancy. Six years after surgery a small tumor recurrence occurred. Case 3: a 23-year-old woman was referred due to a microincidental pituitary adenoma. Laboratory testing was normal. No findings on physical examination. A wait and see approach was decided. Two years after diagnosis, the patient got pregnant without complications. Image remained stable. This article may contribute new cases and provides an extensive review of NFPA during pregnancy.

Remisión espontánea de acromegalia y enfermedad de Cushing en adultos mayores: casos clínicos / Spontaneous remission of acromegaly and Cushing's disease in older patients: report of two cases

Pituitary diseases such as acromegaly and Cushing's disease require surgical or medical therapy. In some functioning pituitary tumors, a spontaneous remission of hormonal hypersecretion is observed, mainly associated to apoplexy or pituitary infarction. We report the evolution of two female patients older than 70 years at the time of diagnosis, with multiple comorbidities. In case 1, acromegaly was diagnosed at 74 years of age. Sellar CT scan showed a 10-mm adenoma. During her follow-up, IGF1 levels remained normal. Nine years later, a magnetic resonance (MR) showed a 7-mm adenoma. In case 2, clinical and biochemical diagnosis of Cushing's disease was done being 71 years old. Sellar MR showed a 6-mm adenoma. Three years later, urinary cortisol normalized with no changes in adenoma at MR. Seven years later, she remains without clinical or biochemical signs of hypercortisolism. In both cases, no signs of hemorrhage were observed at MR.

Long-term follow-up of patients with elevated IGF-1 and nadir GH > 0 4 µg/L but < 1 µg/L

ABSTRACT Objective To report the results of initial investigation and after 5 years of patients with a suspicious clinical scenario for acromegaly, elevated IGF-1, and nadir GH during an oral glucose tolerance test (OGTT) > 0.4 µg/L but < 1 µg/L. Subjects and methods Seventeen patients who had elevated IGF-1 (outside puberty and pregnancy) in two measurements and GH between 0.4 and 1 µg/L during OGTT were selected. Results During initial assessment, only one patient had microadenoma on magnetic resonance imaging (MRI) of the pituitary. In this patient, IGF-1 returned to normal spontaneously after 5 years. In the remaining 16 patients, spontaneous normalization of IGF-1 was observed in four and IGF-1 continued to be elevated in 12 after 5 years. None of the latter patients developed a phenotype of acromegaly, changes in physiognomy or increase in IGF-1 and no tumor was detected by imaging methods. Two patients had nadir GH < 0.4 µg/L, while the nadir GH remained between 0.4 and 1 µg/L in 10 patients. Conclusion In patients (notably young adult or adult women) without a typical phenotype in whom IGF-1 is measured due to a suspicious clinical scenario and is found to be slightly elevated, even if confirmed and in the absence of other causes, a nadir GH cut-off value of 0.4 µg/L instead of 1 µg/L in the OGTT might be inadequate for the diagnosis.

Resonancia magnética en pacientes con tumores más frecuentes en la región selar / Magnetic resonance in patients with more frequent tumors in the selar region

La resonancia magnética constituye el examen ideal en pacientes con presunción clínica de tumores en la región selar, pues no emite radiaciones ionizantes y debido a su visión multiplanar y discriminación tisular, posibilita adoptar una conducta terapéutica adecuada y un seguimiento sin riesgos en estos casos. Se describen las imágenes obtenidas con esa técnica sobre los principales tumores selares, correspondientes a macroadenomas hipofisarios, meningioma intraselar, craneofaringioma supraselar y como caso interesante perteneciente a esta región: el cordoma del clivus, por poseer en la actualidad mejores posibilidades diagnósticas que en décadas anteriores

Magnetic resonance constitutes the ideal exam in patients with clinical presumption of tumors in the selar region, because it doesn't emit ionizing radiations and due to its multiplanar vision and tissular discrimination, it facilitates the adoption of an appropriate therapeutic behavior and a follow up without risks in these cases. The images obtained with that technique on the main selar tumors are described, corresponding to hypophysary macroadenomas, intraselar meningioma, supraselar craneopharyngioma and as an interesting case belonging to this region: the clivus chordoma, for having better diagnostic possibilities at the present time than in previous decades

Incidentalomas endocrinos, más que una imagen / Endocrine incidentalomas, more than an image

Endocrine incidentalomas are nodular lesions located in endocrine glands, diagnosed serendipitously by different image techniques requested for non-endocrine reasons. They can be located in many sites, but this review describes those that compromise pituitary, adrenal and thyroid gland. The main diagnostic challenges of endocrine incidentalomas are discrimination between benign and malignant lesions, and their functional or non-functional endocrine activity. The relevance of adequate image interpretation and associated hormonal study is discussed. (AU)

Prolactinomas may have unusual presentations resulting from massive extrasellar tumor extension / Prolactinomas podem ter apresentações incomuns resultantes de maciça extensão tumoral extrasselar

ABSTRACT The purpose of this case series is to report eight patients with giant prolactinomas emphasizing presentations and a treatment complication. The study group included six men and two women. The median age was 29 years (18–54 years); median serum prolactin level was 4,562 ng/ml (1,543–18,690 ng/ml); three patients (37.5%) had panhypopituitarism; median tumor diameter was 50 mm (41–60 mm). Five patients (62.5%) had visual field defects and three had improvement during treatment; six patients (75%) reached prolactin normalization, with a median time of 10.5 months (7–84 months) and median dose of 2.0 mg/week (1.0 to 3.0 mg/week). One patient presented as a true incidentaloma. One patient presented a cerebrospinal fluid leakage during medical treatment and refused surgery, however this resolved with conservative measures. This case series illustrate a rare subtype of macroprolactinomas, the importance of considering unusual presentations at the diagnosis, the effectiveness of pharmacological treatment and its possible complications.

RESUMO O objetivo desta série de casos é relatar oito pacientes com prolactinomas gigantes enfatizando as formas de apresentação e uma complicação do tratamento. O estudo incluiu seis homens e duas mulheres. A mediana de idade foi 29 anos (18–54); e dos níveis de prolactina foi 4.562 ng/ml (1.543–18.690); três pacientes (37,5%) apresentaram pan-hipopituitarismo; a mediana do máximo diâmetro tumoral foi 50 mm (41–60 mm). Cinco pacientes (62,5%) apresentaram alterações no campo visual e três tiveram melhora durante o tratamento; seis pacientes (75%) alcançaram normalização da prolactina em 10,5 meses (7–84) com dose mediana de cabergolina de 2,0 mg / semana (1,0 a 3,0). Um paciente se apresentou como um verdadeiro incidentaloma. Um paciente apresentou uma fistula liquórica durante o tratamento medicamentoso e recusou correção cirúrgica. No entanto a fistula foi resolvida com medidas conservadoras. Esta série de casos ilustra um subtipo raro de macroprolactinomas, a importância de considerar apresentações incomuns no diagnóstico, a eficácia do tratamento farmacológico e suas possíveis complicações.

Pituitary macroadenoma: analysis of intercarotid artery distance compared to controls / Macroadenoma de hipófise: análise da distância intercarotídea comparada a controles

ABSTRACT Objective To evaluate the intercarotid distance (ICD) of patients with pituitary macroadenoma and compare to heatlhy controls. Method We retrospectively reviewed contrast-enhanced MRI images from twenty consecutive patients diagnosed with non-functioning pituitary macroadenoma, measured the ICD at two different levels (petrous segment – ICD1 and horizontal cavernous segment – ICD2) and compared to twenty paired controls. Results There was no statistically significant difference of the mean ICD1 between the groups and subgroups. For the ICD2 there was statistically significant difference between the case and controls. However, there was no significant difference between the patients with smaller adenomas and the controls. In contrast, the patients with giant adenomas showed statistically significantly higher ICD2 than the controls. Conclusion The ICD at the horizontal segment of the cavernous carotid tends to be wider in patients with giant pituitary adenomas than in healthy individuals or patients with smaller adenomas.

RESUMO Objetivo Avaliar a distância intercarotídea (DIC) de pacientes com macroadenoma de hipófise e comparar com controles saudáveis. Método Foram analisados retrospectivamente imagens de ressonância magnética com contraste de vinte pacientes consecutivos com diagnóstico de macroadenoma hipofisário não-funcionante, medidas as DIC em dois níveis diferentes (segmento petroso – DIC1 e segmento cavernoso horizontal – DIC2) e comparados com vinte controles pareados. Resultados Não houve diferença estatisticamente significativa da DIC1 média entre os grupos e subgrupos. Para a DIC2 houve diferença estatisticamente significativa entre os casos e controles. No entanto, não houve diferença significativa entre os doentes com adenomas menores e os controles. Entretanto, os pacientes com adenomas gigantes tiveram estatisticamente significativamente DIC2 que os controlos. Conclusão A DIC no segmento horizontal da carótida cavernoso, tende a ser mais larga em doentes com adenomas hipofisários gigantes do que em indivíduos saudáveis ou de pacientes com adenomas menores.

Pituitary magnetic resonance imaging experience in Jordan

To assess the pituitary findings as demonstrated on MRI and to compare the results with the data published in the literature. One thousand, one hundred and thirty-eight pituitary MRI's with and without intravenous contrast media [gadolinium] were performed over 6 years from 2001 to 2007 in the Department of Diagnostic Radiology, Jordan University Hospital, Amman, Jordan. The patients were referred from various departments and were evaluated for pituitary, other sellar, and juxtasellar abnormalities. The results were compared with those in the published literature. Four hundred and eight-three normal scans were excluded from the study. The remaining 655 were abnormal, pituitary adenoma was detected in 327 [49.9%], microadenoma was present in 213 [32.5%], and macroadenoma in 114 [17.4%]. Partial empty sella was seen in 157 [24%], diffuse pituitary gland enlargement in 98 [14.9%], ectopic pituitary posterior lobe in 13 [2%], and other findings in 31 [4.7%]. The incidence of pituitary adenoma was equal in both genders; however, microadenoma was more common, affected a younger age group, and was predominately seen in females. The other parameters showed agreement with the published literature

HRCT of pituitary fossa correlation of clinical and CT findings

Purpose of the study was to correlate the clinical features with HRCT [High Resolution Computed Tomography] findings of pituitary fossa. Findings in a series of 30 patients, [22 female and 8 male] who underwent CT scanning of the pituitary fossa are reported majority of them had clinical suspicion of prolactinoma rest have other clinical signs of pituitary disease. Results showed 13 [out of 22] female patients with positive scan and 4 [out of 8] male patients with positive scan. In conclusion, this study conforms the usefulness of high resolution CT scanning of the sellar region in a specific settings

Bilateral fronto-orbito-zygomatic craniotomy--a combined extended frontal and orbitozygomatic approach.

In extensive skull base lesions involving the spheno-ethmoido-clival region and extending into both the cavernous sinuses and infratemporal regions, a combination of approaches is usually required, either in the same operation or at a second stage. The bilateral fronto-orbito-zygomatic craniotomy described in this report is a combination of an extended frontal approach and fronto-orbito-zygomatic craniotomy. This gives a wide exposure of the spheno-ethmoido-clival regions of both the cavernous sinuses and both the infratemporal regions. The exposure is thus greatly improved with minimal frontal lobe retraction. The single bone piece can be speedily replaced obviating the need for a complicated reconstruction technique and gives a superior cosmetic result. The operative technique is described in detail.