Résumé
@#Intracranial lipomas are benign tumors that may occasionally be found in the suprasellar cistern while pituitary adenomas are far more common brain tumors. Pituitary adenomas may rarely coexist with other intracranial tumors in the sellar-suprasellar region. We share a unique case of a patient with coexisting non-functioning pituitary adenoma and sellar-suprasellar lipoma presenting with blurring of vision. We report a 55-year-old male presenting with a two-year history of blurring of vision with findings of a 2.7 x 3.0 x 3.2 cm homogeneously enhancing lobulated isointense mass on the sellar-suprasellar region. Hormonal workups revealed low cortisol and mildly elevated prolactin. He initially underwent endonasal transsphenoidal excision of the tumor which revealed to be a lipoma on histopathology. Due to minimal improvement of vision from the subtotal excision, he underwent repeat surgery through the transcranial approach which in turn showed a pituitary adenoma. The co-occurrence of two sellar-suprasellar tumors with different histology is rare, as most of the evidence is based on only a handful of case series. Intracranial lipomas result from persistence and abnormal differentiation of the meninx primitiva during the development of the subarachnoid cisterns. On the other hand, pituitary tumorigenesis is still largely unclear but appears to involve multiple tumor suppressor genes, oncogenes, cell cycle deregulation factors, and miRNAs. Given the differing pathogenesis of each tumor type, the coexistence may only be coincidental. The best surgical approach in this situation is unknown but the focus is on complete excision of the adenoma.
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Tumeurs de l'hypophyseRésumé
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)
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Humains , Mâle , Femelle , Adulte , Jeune adulte , Tumeurs de l'hypophyse/épidémiologie , Acromégalie/épidémiologie , Prolactinome/épidémiologie , Adénomes/épidémiologie , Résultats fortuits , Hypersécrétion hypophysaire d'ACTH/épidémiologie , Tumeurs de l'hypophyse/anatomopathologie , Tumeurs de l'hypophyse/imagerie diagnostique , Adénomes/anatomopathologie , Adénomes/imagerie diagnostique , Pertinence cliniqueRésumé
Objective: To investigate the clinicopathological characteristics of plurihormonal PIT1-lineage pituitary neuroendocrine tumors. Methods: Forty-eight plurihormonal PIT1-lineage tumors were collected between January 2018 and April 2022 from the pathological database of Sanbo Brain Hospital, Capital Medical University. The related clinical and imaging data were retrieved. H&E, immunohistochemical and special stains were performed. Results: Out of the 48 plurihormonal PIT1-lineage tumors included, 13 cases were mature PIT1-lineage tumors and 35 cases were immature PIT1-lineage tumors. There were some obvious clinicopathological differences between the two groups. Clinically, the mature plurihormonal PIT1-lineage tumor mostly had endocrine symptoms due to increased hormone production, while a small number of immature PIT1-lineage tumors had endocrine symptoms accompanied by low-level increased serum pituitary hormone; patients with the immature PIT1-lineage tumors were younger than the mature PIT1-lineage tumors; the immature PIT1-lineage tumors were larger in size and more likely invasive in imaging. Histopathologically, the mature PIT1-lineage tumors were composed of large eosinophilic cells with high proportion of growth hormone expression, while the immature PIT1-lineage tumors consisted of chromophobe cells with a relatively higher expression of prolactin; the mature PIT1-lineage tumors had consistently diffuse cytoplasmic positive staining for keratin, while the immature PIT1-lineage tumors had various expression for keratin; the immature PIT1-lineage tumors showed more mitotic figures and higher Ki-67 proliferation index; in addition, 25.0% (12/48) of PIT1-positive plurihormonal tumors showed abnormal positive staining for gonadotropin hormones. There was no significant difference in the progression-free survival between the two groups (P=0.648) by Kaplan-Meier analysis. Conclusions: Plurihormonal PIT1-lineage tumor belongs to a rare type of PIT1-lineage pituitary neuroendocrine tumors, most of which are of immature lineage. Clinically increased symptoms owing to pituitary hormone secretion, histopathologically increased number of eosinophilic tumor cells with high proportion of growth hormone expression, diffusely cytoplasmic keratin staining and low proliferative activity can help differentiate the mature plurihormonal PIT1-lineage tumors from the immature PIT1-lineage tumors. The immature PIT1-lineage tumors have more complicated clinicopathological characteristics.
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Humains , Tumeurs neuroendocrines , Tumeurs de l'hypophyse/anatomopathologie , Hormones hypophysaires , Hormone de croissance/métabolisme , KératinesRésumé
INTRODUCTION@#Spontaneous regression of pituitary adenomas is a rare occurrence that is thought to be due to pituitary apoplexy. We would like to review the demograhic data, imaging findings, and neurologic, endocrinologic, and radiologic outcomes of patients who exhibited this unusual phenomenon.@*METHODS@#We present a case of non-functioning pituitary adenoma (NFPA) in a 66-year-old man that underwent spontaneous regression. We also performed a systematic literature review on cases of pituitary adenomas that exhibited spontaneous regression, in accordance with the Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) guidelines.@*RESULTS@#There have been only 27 reported cases of spontaneous regression observed in all pituitary adenomas, 16 of which are NFPAs. Headache was the most common symptom while decreased visual acuity and oculomotor nerve palsy were the most common sign. Pituitary hemorrhage was seen in two-thirds of cases, while findings suggestive of pituitary infarct, such as enhancement of the sphenoid sinus mucosa. were seen in one-third. Complete radiographic remission was observed in 62% of cases, with an 11% recurrence rate seen at least two years after the initial imaging. Neurologic recovery was reported in 88% and endocrinologic recovery from hypopituitarism was 40%.@*CONCLUSION@#Most patients exhibited neurologic and endocrinologic improvement concomitant with the regression of the pituitary adenoma. However, these tumors may recur; thus, regular and long- term neuro-ophthalmologic and radiologic follow-up is advised.
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Tumeurs de l'hypophyse , Rémission spontanéeRésumé
The Ly-6 and uPAR (LU) domain-containing proteins represent a large family of cell-surface markers. In particular, mouse Ly-6A/Sca-1 is a widely used marker for various stem cells; however, its human ortholog is missing. In this study, based on a systematic survey and comparative genomic study of mouse and human LU domain-containing proteins, we identified a previously unannotated human gene encoding the candidate ortholog of mouse Ly-6A/Sca-1. This gene, hereby named LY6A, reversely overlaps with a lncRNA gene in the majority of exonic sequences. We found that LY6A is aberrantly expressed in pituitary tumors, but not in normal pituitary tissues, and may contribute to tumorigenesis. Similar to mouse Ly-6A/Sca-1, human LY6A is also upregulated by interferon, suggesting a conserved transcriptional regulatory mechanism between humans and mice. We cloned the full-length LY6A cDNA, whose encoded protein sequence, domain architecture, and exon-intron structures are all well conserved with mouse Ly-6A/Sca-1. Ectopic expression of the LY6A protein in cells demonstrates that it acts the same as mouse Ly-6A/Sca-1 in their processing and glycosylphosphatidylinositol anchoring to the cell membrane. Collectively, these studies unveil a novel human gene encoding a candidate biomarker and provide an interesting model gene for studying gene regulatory and evolutionary mechanisms.
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Humains , Protéines membranaires/génétique , Tumeurs de l'hypophyse/génétique , Marqueurs biologiquesRésumé
Male patients with prolactinomas usually present with typical hyperprolactinemia symptoms, including sexual dysfunction and infertility. However, clinical factors related to sexual dysfunction and surgical outcomes in these patients remain unclear. This study aimed to investigate the outcomes of male patients with prolactinomas after transsphenoidal surgery and the risk factors affecting sexual dysfunction. This study was conducted on 58 male patients who underwent transsphenoidal surgery for prolactinomas between May 2014 and December 2020 at the First Affiliated Hospital of Sun Yat-sen University, Guangzhou, China. We evaluated the sexual function of patients before and after surgery through International Index of Erectile Function-5 scores, libido, and frequency of morning erection. Of the 58 patients, 48 (82.8%) patients had sexual intercourse preoperatively. Among those 48 patients, 41 (85.4%) patients presented with erectile dysfunction. The preoperative International Index of Erectile Function-5 scores in patients with macroprolactinomas were significantly higher than those in patients with giant prolactinomas (17.63 ± 0.91 vs 13.28 ± 1.43; P = 0.01). Postoperatively, the incidence of erectile dysfunction was 47.9%, which was significantly lower than that preoperatively (85.4%; P = 0.01). Twenty-eight (68.3%) patients demonstrated an improvement in erectile dysfunction. Tumor size and invasiveness were significantly correlated with the improvement of erectile dysfunction. Preoperative testosterone <2.3 ng ml-1 was an independent predictor of improvement in erectile dysfunction. In conclusion, our results indicated that tumor size and invasiveness were important factors affecting the improvement of sexual dysfunction in male patients with prolactinoma. The preoperative testosterone level was an independent predictor related to the improvement of erectile dysfunction.
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Humains , Mâle , Prolactinome/chirurgie , Dysfonctionnement érectile/étiologie , Études rétrospectives , Troubles sexuels d'origine physiologique/complications , Testostérone , Tumeurs de l'hypophyse/anatomopathologieRésumé
The pituitary adenomas comprise the largest part of pituitary neoplasms, representing between 9 and 12% of primary brain tumors. Currently, the treatment of election is the surgical, the trans-sphenoidal endonasal path has provided a broader panoramic view, being feasible more delicate and safe procedures. The objective of this study was to describe the management of pituitary adenomas by endoscopic surgery trans-sphenoidal endonasal path. This is a study in a series of cases, with a sample of 17 patients who met the selection criteria and were attended at the José Carrasco Hospital Arteaga, Cuenca - Ecuador, period 2018 - 2019. All patients presented informed consent for the performance of the surgery and the subsequent dissemination of the results. The 53% of the population belonged to the male gender, 47% were patients between 40 and 60 years old, 65% were detected with macroadenomas, and 29% presented complications such as nasal obstruction, sphenoiditis and headache, the residual tumor stage was due 24%.Endoscopic surgery trans-sphenoidal endonasal path is a safe, minimally invasive tool, a surgical alternative with a high rate of tumor excision, better control and fewer complications, which reduces the morbidity of patients.
Los adenomas de hipófisis comprenden la mayor parte de neoplasias hipofisiarias, representando entre el 9 y 12% de los tumores cerebrales primarios. En la actualidad el tratamiento de elección es el quirúrgico, la vía endonasal transesfenoidal ha proporcionado una visión panorámica más amplia, siendo factibles procedimientos más delicados y seguros. El objetivo de este estudio fue describir el abordaje de adenomas de hipófisis mediante cirugía endoscópica vía endonasal transesfenoidal. Diseñamos un estudio de corte longitudinal, con una muestra de 17 pacientes que cumplieron con los criterios de selección y fueron atendidos en el Hospital José Carrasco Arteaga de la ciudad de Cuenca - Ecuador, periodo 2018 - 2019. Todos los pacientes presentaron consentimiento informado para la realización de la cirugía y la posterior difusión de los resultados. El 53% de la población pertenecieron al sexo masculino, 47% fueron pacientes entre 40 y 60 años, al 65% se le detectó macroadenomas, el 29% presentaron complicaciones como obstrucción nasal, esfenoiditis y cefalea, la tasa de tumor residual fue del 24%. La cirugía endoscópica vía endonasal transesfenoidal es una herramienta segura, mínimamente invasiva, una alternativa quirúrgica con una elevada tasa de exéresis tumoral, mejor control y menos complicaciones, que reduce la morbilidad de los pacientes.
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Humains , Mâle , Femelle , Adulte , Adulte d'âge moyen , Tumeurs de l'hypophyse/chirurgie , Adénomes/chirurgie , Endoscopie/méthodes , Os sphénoïde/chirurgie , Études longitudinales , Microchirurgie , Fosse nasale/chirurgieRésumé
Introduction Mucoepidermoid carcinoma (MEC) is a tumor originated from the epitheliumof the glandular excretory ducts and has highly variable biological potential. It is the most prevalent cancer of the salivary glands. The present report aims to describe a case of nasal mucoepidermoid carcinoma that developed after adjuvant radiotherapy (RT) treatment of a recurrent pituitary macroadenoma. Case Report Male patient, 62 years old, presented with recurrent nasal epistaxis on the right, associated with intense pulsatile headache, visual analogical scale (VAS) 10/10, with improvement only with the use of opioids andmorphine. After undergoing oncological screening and study by imaging exams, the presence of an expansive seal lesion with suprasellar extension was seen, involving the medial wall of the cavernous segment of the right carotid artery and the anterior cerebral artery, as well as the presence of a new expansive lesion in the right nasal cavity, with ethmoid bone invasion superiorly and medial orbit wall invasion laterally, compressing the ipsilateral optic nerve canal. Discussion Sinonasal neoplasms represent a small portion of all malignancies of the upper aerodigestive tract, accounting for<5% of these neoplasms. The development of MEC involves risk factors such as occupational issues, history of trauma and surgery involving the nasal area, and radiation exposure, as in previous RT. Conclusion Mucoepidermoid carcinoma is an uncommon neoplasia and can be associated with RT treatment, as used in cases of recurrent pituitary macroadenoma. In general, surgical resection to obtain free margins of neoplastic tissue is the aimed treatment, seeking better prognosis.
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Humains , Mâle , Adulte d'âge moyen , Tumeurs de l'hypophyse/radiothérapie , Tumeurs du nez/chirurgie , Carcinome mucoépidermoïde/chirurgie , Fosse nasale/chirurgie , Récidive , Tumeurs du nez/anatomopathologie , Carcinome mucoépidermoïde/anatomopathologie , Carcinome mucoépidermoïde/imagerie diagnostique , Fosse nasale/anatomopathologieRésumé
RESUMO A hidrocefalia é definida como a dilatação ventricular pelo aumento da pressão intraventricular e intracraniana quando não tratada ou por insucesso do tratamento. Muitas vezes, leva ao dano das vias ópticas, podendo causar atrofia óptica, devido à proximidade dessas vias com o ventrículo lateral quando ocorre a dilatação. Assim como a hidrocefalia pode levar à atrofia óptica, outras patologias também podem. Tumores hipofisários compartilham desse mesmo sinal, além de causar hemianospsia bitemporal quando o tumor comprime quiasma óptico. Ademais, a hemianopsia bitemporal é o distúrbio visual mais comum encontrado em pacientes com tumor de hipófise. Os tumores de hipófise, por exemplo, geram manifestações clínicas que podem estar relacionadas à disfunção da glândula ou aos efeitos mecânicos da expansão tumoral. Sinais e sintomas visuais estão mais ligados ao efeito mecânico do tumor. Assim, muitas vezes, o paciente procura o oftalmologista antes do endocrinologista. Neste caso, analisaremos uma paciente portadora de hidrocefalia que apresentava, concomitantemente, um tumor hipofisário, e a investigação oftalmológica fez toda a diferença no tratamento da paciente.
ABSTRACT Hydrocephalus is defined as ventricular dilation caused by increased intraventricular and intracranial pressure when untreated or due to treatment failure. Optical pathways can often cause optic atrophy due to the proximity to the lateral hazard when dilation occurs. Hydrocephalus can lead to optic atrophy, as well as other pathologies. Pituitary tumors share this same sign, in addition to causing bitemporal hemianopia when it compresses the optic chiasm. In addition, bitemporal hemianopia is the visual disturbance most commonly found in patients with pituitary tumors. Pituitary tumors, for example, have clinical manifestations that may be related to gland dysfunction, or to mechanisms of tumor expansion. Visual signs and symptoms are more linked to the mechanical effect of the tumor. Therefore, the patient usually seeks the ophthalmologist before the endocrinologist. In this case, we analyzed a patient with hydrocephalus who presented, at the same time, a pituitary tumor, and the ophthalmological investigation made all the difference in the treatment of the patient.
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Humains , Femelle , Adulte , Tumeurs de l'hypophyse/complications , Atrophie optique/étiologie , Hémianopsie/étiologie , Hydrocéphalie/complications , Chiasma optique , Nerf optique/anatomopathologie , Tumeurs de l'hypophyse/chirurgie , Spectroscopie par résonance magnétique , Acuité visuelle , Champs visuels , Atrophie optique/diagnostic , Syndromes de compression nerveuseRésumé
Objective: To investigate the clinicopathological features and treatment strategies of pituicytoma. Methods: Twenty-one cases of pituicytoma were collected at the First Affiliated Hospital of Nanjing Medical University and Jinling Hospital, Nanjing, China from 2009 to 2020. The clinical data of 21 pituicytoma patients was retrospectively analyzed, and the relevant literature was reviewed. Results: Twenty-one patients aged 4 to 68 years, including 8 males and 13 females. All patients underwent surgical treatment. Histologically, the tumor was consisted almost entirely of elongate, bipolar spindle cells arranged in a fascicular or storiform pattern. Mitotic figures were rare. Immunohistochemically, tumor cells were diffusely positive for S-100 protein (21/21), vimentin (15/15) and TTF1 (14/14), while they were weakly or focally positive for GFAP (13/16) and EMA (6/12). CKpan was negative in all cases and Ki-67 proliferation index was low (<5%). Among the 18 patients with follow-up, all survived and 2 relapsed after surgery. Conclusions: Pituicytoma is a rare low-grade glioma of the sellar area. It is easily confused with other sellar tumors. Preoperative diagnosis is difficult. It needs to be confirmed by histopathology and immunohistochemistry. Microsurgery is the main treatment method at present.
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Adolescent , Adulte , Sujet âgé , Enfant , Enfant d'âge préscolaire , Femelle , Humains , Mâle , Adulte d'âge moyen , Jeune adulte , Craniopharyngiome , Gliome/anatomopathologie , Immunohistochimie , Tumeurs de l'hypophyse/anatomopathologie , Études rétrospectivesRésumé
Objective: To investigate the related factors and treatments of delayed cerebrospinal fluid rhinorrhea (CFR) after invasive pituitary adenoma (IPA) surgery. Methods: One hundred and forty-two patients with IPA treated in Tianjin Huanhu Hospital from January 2014 to January 2019 were analyzed retrospectively, including 62 males and 80 females, aging from 38 to 67 years. The clinical data of patients before and after operation were collected. All patients with postoperative CFR underwent endoscopic CFR repair. During the operation, residual or recurrent pituitary adenomas were resected, the dura around the leak was enlarged and the necrotic tissue was removed. For those who still had fluid leakage after repair, the necrotic tissue was cleaned up, the leakage was filled and reinforced under endoscopy. Endoscopic rhinorrhea repair was performed if necessary. The cerebrospinal fluid leak was repaired with multi-layer materials. The related risk factors of delayed CFR after operation were analyzed. SPSS 19.0 software was used for statistical analysis. Results: Among the 142 patients in this group, 64 cases underwent total tumor resection and 78 cases underwent non-total tumor resection. They were followed up for 6 to 72 months. Thirty-one cases had delayed CFR, with an incidence of 21.83%, and occurred between 1 and 5 years postoperatively, with an average of 2.4 years. All 31 patients with delayed CFR underwent endoscopic CFR repair. The nasal endoscopy was rechecked at 2 weeks, 1 month, 3 months and 6 months after operation. Twenty-eight patients were repaired successfully after 1 operation, while 2 patients after 2 operations and 1 patient after 3 operations. These patients were followed up for 6 to 60 months, and no CFR occurred again. Univariate analysis showed that the degree of tumor resection, recurrence, size, texture, postoperative radiotherapy and operator experience were the risk factors of delayed CFR (all P<0.05). Multivariate analysis showed that the degree of tumor resection and recurrence were the highest independent risk factors for postoperative CFR, and tumor size, texture, postoperative radiotherapy and operator experience were the independent risk factors in this study. Conclusions: Delayed CFR after IPA is related to the degree of tumor resection, recurrence, size, texture, postoperative radiotherapy and the operator experience. It is necessary to completely remove the tumor under endoscope, to expand resection of the dura and necrotic tissue around the leak, to repair the defect with multi-layer materials, to follow-up closely and to repair timely after operation.
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Adulte , Sujet âgé , Femelle , Humains , Mâle , Adulte d'âge moyen , Adénomes/chirurgie , Fuite de liquide cérébrospinal , Rhinorrhée cérébrospinale/chirurgie , Tumeurs de l'hypophyse/chirurgie , Études rétrospectivesRésumé
@#A collision tumor involving metastasis to a pituitary adenoma is rare. We describe a case of a 68-year-old Bidayuh woman with underlying treatment-responsive lung adenocarcinoma, who presented with mass effect, panhypopituitarism and polyuria. Her initial imaging study reported pituitary macroadenoma, and she was treated with hormone replacement therapy. She then underwent transsphenoidal tumor debulking surgery with subsequent histopathological findings of a collision tumor of an adenocarcinoma with metastasis to a non-functioning pituitary adenoma.
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Tumeurs de l'hypophyseRésumé
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.
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Humains , Femelle , Adulte d'âge moyen , Tumeurs de l'hypophyse/thérapie , Plasmocytome/chirurgie , Myélome multiple/diagnostic , Myélome multiple/anatomopathologie , Tumeurs de l'hypophyse/imagerie diagnostique , Plasmocytome/anatomopathologie , Plasmocytome/imagerie diagnostique , Adénomes/anatomopathologie , Diagnostic différentiel , Myélome multiple/chirurgieRésumé
Introduction The endoscopic endonasal transsphenoidal approach (EETA) is routinely used to treat sellar and suprasellar tumors. It provides safe and direct access to tumors in these locations, with wide visualization of anatomical landmarks and great surgical results. With the COVID-19 pandemic, despite the high risk of transmission involved, various surgical procedures cannot be postponed due to their emergency. Case Report A 62-year-old female presented in the previous two months with headaches, followed by bilateral severe visual loss. In 2016, she was submitted to subtotal resection of a non-secretorymacroadenoma. Because of the progressive visual deficits, the EETA was used to the resect the pituitary adenoma. Technical Note We developed a low-cost adaptation to the surgical fields, covering the patient's head and superior trunk with a regular surgicalmicroscope bag with a tiny slit to enable the endoscope and surgical instruments to enter the nose, thus protecting the personnel in the operating room from the aerosolization of particles. This makes surgery safer for the surgical team and for the patient. Conclusion In view of the lack of literature on this subject, except for some reports of experiences from some services around the world, we describe the way we have adjusted the EETA in the context of the COVID-19 pandemic.
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Humains , Femelle , Adulte d'âge moyen , Tumeurs de l'hypophyse/chirurgie , Adénomes/chirurgie , Chirurgie endoscopique par orifice naturel/méthodes , COVID-19/transmission , Hypophyse/chirurgie , Adénomes/complications , Adénomes/imagerie diagnostique , COVID-19/prévention et contrôleRésumé
Introduction Hemangioblastomas of the pineal region or pituitary stalk are extremely rare. Only two cases of hemangioblastomas involving the pineal region have been reported, and four involving the pituitary stalk. The purpose of the present manuscript is to describe an unusual case of supposed hemangioblastoma found concomitantly in the pineal region and pituitary stalk of a patient diagnosed with Von Hippel-Lindau (VHL) disease. Case Report A 35-year-old female patient with a previous diagnosis of VHL complaining of occipital headaches and balance disturbances for three weeks, who previously had a cerebellar hemangioblastoma resected. The visual characteristics of the tumor suggested a friable vascular lesion with a reddish-brown surface, and an incisional biopsy was performed. The tumor consisted of a dense vascular network surrounded by fibrous stroma abundant in reticulin and composed by both fusiform and dispersed xanthomatous cells; the immunohistochemistry was immunopositive for neuronspecific enolase and immunonegative for epithelial membranous antigen. The patient has been monitored closely for 2 years, and the supratentorial masses have not presented any volume alteration. Conclusion This rare association must be taken into account in patients with VHL disease, or at least be suspected in patients who present a thickening of the pituitary stalk and a pineal-region mass. We believe a biopsy of our asymptomatic patient could have been dangerous due to inherent complications like intraoperative bleeding. We recommend close observation of asymptomatic lesions with MRIs every six months or until the lesions become symptomatic. If the pineal-region tumor does become symptomatic, gross resection via a transcallosal approach would be ideal.
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Humains , Femelle , Adulte , Glande pinéale/chirurgie , Pinéalome/chirurgie , Hypophyse/chirurgie , Hémangioblastome/chirurgie , Glande pinéale/malformations , Pinéalome/imagerie diagnostique , Hypophyse/malformations , Tumeurs de l'hypophyse/chirurgie , Hémangioblastome/imagerie diagnostique , Continuité des soins , Maladie de von Hippel-LindauRésumé
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.
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Humains , Tumeurs de l'hypophyse/génétique , Tumeurs de l'hypophyse/imagerie diagnostique , Apoplexie hypophysaire/étiologie , Apoplexie hypophysaire/génétique , Adénomes/génétique , Adénomes/imagerie diagnostique , Orientation vers un spécialiste , Dépistage génétique , Protéines et peptides de signalisation intracellulaire/génétique , MutationRésumé
Las lesiones selares son una patología con una incidencia de 3,2 a 4 / 100,000 y una prevalencia de 78 a 94 / 100,000. Un 10% son incidentalomas en la población adulta. Se cree que su prevalencia en el orden mundial actualmente va en aumento.En relación a las manifestaciones clínicas, cabe destacar que es una de las pocas enfermedades que pueden manifestarse tanto por signos y síntomas neurológicos (por ejemplo: hemianopsia bitemporal, síndrome de hipertensión endocraneana debido a hidrocefalia, entre otros), como también por síndromes endocrinológicos (por ejemplo: síndrome de Cushing, acromegalia, amenorrea-galactorrea, infertilidad).Todo paciente debe presentar un estudio clínico-radiológico completo, lo que permitirá un correcto diagnóstico y categorización del mismo.El objetivo del presente trabajo es proporcionar al neurocirujano en formación los conceptos claves que servirán de sustento para el manejo preoperatorio de un paciente con adenoma hipofisario.
Sellar lesions are a pathology with an incidence of 3.2 to 4 / 100.000 and a prevalence of 78 to 94 / 100.000. Normally, 10% of them are incidentalomas and adult patients are in the highest risk group. Because it Ìs prevalence in the world is currently increasing, it is of extremely importance to study and understand this pathology. In relation to the clinical manifestations, it should be noted that it is one of the few diseases that can manifest through neurological signs and symptoms like bitemporal hemianopsia, endocranial hypertension syndrome due to hydrocephalus, as well as endocrinological syndromes like Cushing's, acromegaly, amenorrhea-galactorrhea and infertility. One of the most important things to notice is that the treatment success in this pathology comes with the correct diagnosis and characterization of it, for what all patients should have a complete clinical-radiological evaluation.In this study, we establish a guide with concepts and key tools to support the medical personal during a pre-surgical preparation of patients with pituitary adenoma.
Sujets)
Adénomes , Tumeurs de l'hypophyse , Prolactine , Hormone de croissance , Hydrocéphalie , Manifestations neurologiques , NeurochirurgieRésumé
Objectif :Décrire le profil épidémiologique et remnologique des pathologies retrouvées chez les patients adressés pour IRM hypophysaire dans le service d'imagerie médicale du CHU-B. Patients et Méthode :il s'agissait d'une étude transversale descriptive, réalisée au service d'imagerie médicale du CHU de Brazzaville de janvier 2014 à décembre 2018. Tous les patients adressés pour IRM hypophysaire étaient inclus. Les examens ont été réalisés sur une IRM de haut champ de 1,5 Tesla sans et avec injection de produit de contraste. Les données épidémiologiques, l'indication et les résultats retrouvés ont été traitées avec le logiciel Microsoft Excel 2013. Résultats :Cinquante-huit IRM étaient retenues durant la période d'étude. L'âge moyen des patients était de 38,24 ± 11,52 ans avec des extrêmes allant de 12 à 74 ans et un âge médian de 36 ans. Le sexe ratio (F/H) était de 4,8 avec 48 femmes (82,76%). La galactorrhée était le motif de consultation le plus fréquemment retrouvé (21,88% des cas), suivie des aménorrhées (18,75%). L'adénome hypophysaire était la pathologie la plus fréquente devant les selles turciques vides et les kystes de la poche de Rathke. Conclusion :La pathologie hypophysaire est l'apanage du sujet jeune de sexe féminin. Son mode de révélation le plus fréquent est celui de troubles gynécologiques et/ou endocriniens. Les adénomes constituent la quasi-totalité de la pathologie hypophysaire avec une nette prédominance de macroadénome. L'IRM apporte des informations essentielles au diagnostic de cespathologies malgré son accessibilité et sa disponibilité qui restent encore limité.
Sujets)
Humains , Mâle , Femelle , Adulte , Adulte d'âge moyen , Sujet âgé , Tumeurs de l'hypophyse , Imagerie par résonance magnétique , Kystes du système nerveux central , Galactorrhée , Hypophyse , CongoRésumé
El gen AIP (proteína moduladora de la actividad del receptor de aril hidrocarburos) se localiza en la región 11q13.2 y codifica para una proteína de 330 aminoácidos que interactúa con el factor de transcripción AhR (receptor para aril hidrocarburos). Las mutaciones en este gen se han asociado con adenomas pituitarios aislados de tipo familiar (APAF). Se caracterizan por una presentación temprana (alrededor de 20 años), por lo regular producen hormona de crecimiento y/o prolactina, tienen un comportamiento clínico agresivo y poca respuesta a análogos de somatostatina.
The AIP gene (aryl hydrocarbon receptor interacting protein) is located on chromosome 11q13.2 and encodes a 330 amino acid protein which interacts with the aryl hydrocarbon receptor (AHR) transcription factor. Mutations in the AIP gene have been associated with familial isolated pituitary adenomas (FIPA). They characterize by an early-onset (around the age of 20 years old) and for being aggressive, growth hormone and/or prolactin-secreting tumors, with poor response to somatostatin analogues.