ABSTRACT
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.
Subject(s)
Humans , Male , Middle Aged , Pituitary Neoplasms/radiotherapy , Nose Neoplasms/surgery , Carcinoma, Mucoepidermoid/surgery , Nasal Cavity/surgery , Recurrence , Nose Neoplasms/pathology , Carcinoma, Mucoepidermoid/pathology , Carcinoma, Mucoepidermoid/diagnostic imaging , Nasal Cavity/pathologyABSTRACT
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.
Subject(s)
Adult , Aged , Female , Humans , Male , Middle Aged , Adenoma/surgery , Cerebrospinal Fluid Leak , Cerebrospinal Fluid Rhinorrhea/surgery , Pituitary Neoplasms/surgery , Retrospective StudiesABSTRACT
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.
Subject(s)
Adolescent , Adult , Aged , Child , Child, Preschool , Female , Humans , Male , Middle Aged , Young Adult , Craniopharyngioma , Glioma/pathology , Immunohistochemistry , Pituitary Neoplasms/pathology , Retrospective StudiesABSTRACT
@#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.
Subject(s)
Pituitary NeoplasmsABSTRACT
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.
Subject(s)
Humans , Female , Adult , Pituitary Neoplasms/complications , Optic Atrophy/etiology , Hemianopsia/etiology , Hydrocephalus/complications , Optic Chiasm , Optic Nerve/pathology , Pituitary Neoplasms/surgery , Magnetic Resonance Spectroscopy , Visual Acuity , Visual Fields , Optic Atrophy/diagnosis , Nerve Compression SyndromesABSTRACT
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.
Subject(s)
Humans , Pituitary Neoplasms/genetics , Pituitary Neoplasms/diagnostic imaging , Pituitary Apoplexy/etiology , Pituitary Apoplexy/genetics , Adenoma/genetics , Adenoma/diagnostic imaging , Referral and Consultation , Genetic Testing , Intracellular Signaling Peptides and Proteins/genetics , MutationABSTRACT
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.
Subject(s)
Humans , Female , Middle Aged , Pituitary Neoplasms/therapy , Plasmacytoma/surgery , Multiple Myeloma/diagnosis , Multiple Myeloma/pathology , Pituitary Neoplasms/diagnostic imaging , Plasmacytoma/pathology , Plasmacytoma/diagnostic imaging , Adenoma/pathology , Diagnosis, Differential , Multiple Myeloma/surgeryABSTRACT
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.
Subject(s)
Humans , Female , Middle Aged , Pituitary Neoplasms/surgery , Adenoma/surgery , Natural Orifice Endoscopic Surgery/methods , COVID-19/transmission , Pituitary Gland/surgery , Adenoma/complications , Adenoma/diagnostic imaging , COVID-19/prevention & controlABSTRACT
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.
Subject(s)
Humans , Female , Adult , Pineal Gland/surgery , Pinealoma/surgery , Pituitary Gland/surgery , Hemangioblastoma/surgery , Pineal Gland/abnormalities , Pinealoma/diagnostic imaging , Pituitary Gland/abnormalities , Pituitary Neoplasms/surgery , Hemangioblastoma/diagnostic imaging , Continuity of Patient Care , von Hippel-Lindau DiseaseABSTRACT
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.
Subject(s)
Adenoma , Pituitary Neoplasms , Prolactin , Growth Hormone , Hydrocephalus , Neurologic Manifestations , NeurosurgeryABSTRACT
SUMMARY Acromegaly caused by ectopic growth hormone-releasing hormone (GHRH)-secreting tumor is exceedingly rare. We report a case of acromegaly secondary to GHRH secretion by an incidentally diagnosed pulmonary neuroendocrine tumor (NET) and review 47 similar cases in literature. A 22-year-old male patient presented with symptoms of pituitary apoplexy. Magnetic resonance imaging (MRI) showed apoplexy of a pituitary adenoma. Routinely prior to surgery, a chest radiography was performed which revealed a mass in the left lung. During investigation, the patient was diagnosed with metastatic GHRH-secreting pulmonary NET. In retrospect, it was noted that the patient had pituitary hyperplasia 20 months prior to the MRI which showed the presence of a pituitary adenoma. The histological findings confirmed somatotroph hyperplasia adjacent to somatotropinoma. This case suggests that GHRH secretion can be associated with pituitary hyperplasia, which may be followed by pituitary adenoma formation.
Subject(s)
Humans , Male , Adult , Young Adult , Pituitary Neoplasms , Acromegaly , Adenoma/complications , Adenoma/diagnostic imaging , Carcinoma, Neuroendocrine , Growth Hormone-Releasing Hormone , HyperplasiaABSTRACT
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.
Subject(s)
Pituitary Neoplasms/genetics , Intercellular Signaling Peptides and Proteins , Intercellular Signaling Peptides and Proteins/metabolism , Pituitary Diseases/genetics , Pituitary Diseases/metabolism , Pituitary Neoplasms/metabolism , Adenoma/genetics , Adenoma/metabolismABSTRACT
Tuberculosis is an infectious disease that involves any organ. However, the primary pituitary tuberculosis is an extremely rare disease. Intracranial tuberculomas account for 0.15-5% of intracranial space-occupying lesions, of which, pituitary as the primary site is unusual, and easily misdiagnosed as pituitary adenoma. In this setting, the late diagnosis can result in permanent endocrine dysfunction. We report the case of a 50-year-old woman who presented to the neurosurgery outpatient department with complaints of progressively increasing headache and diminished vision over the last year. On the clinical examination, the patient was conscious and oriented. The routine hematological and biochemical workup showed an increased erythrocyte sedimentation rate (ESR) and increased prolactin levels. The radiological working diagnosis was consistent with pituitary macroadenoma. No other radiological and/or clinical clue that could elicit the suspicion of pulmonary or extrapulmonary lesions of tuberculosis was found. The transsphenoidal endonasal tumor excision was done. The histopathology showed numerous epithelioid cell granulomas, Langhans giant cells along with scant necrosis. Ziehl Neelsen staining demonstrated acid-fast bacilli, and the final diagnosis of pituitary tuberculoma was made. We report this rare case of pituitary lesion that may be included in the differential diagnosis of sellar lesions to avoid unnecessary surgical interventions, especially in regions where the disease is endemic.
Subject(s)
Humans , Female , Middle Aged , Pituitary Gland/pathology , Pituitary Neoplasms , Tuberculosis/pathology , Adenoma/pathology , Epithelioid Cells , Giant Cells, Langhans , Rare Diseases , Diagnosis, Differential , Granuloma/pathologyABSTRACT
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.
Subject(s)
Humans , Male , Female , Adult , Middle Aged , Aged , Pituitary Neoplasms , Magnetic Resonance Imaging , Central Nervous System Cysts , Galactorrhea , Pituitary Gland , CongoABSTRACT
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.
Subject(s)
Humans , Contrast Media , Magnetic Resonance Imaging , Pituitary Diseases , Pituitary Gland , Pituitary Neoplasms/diagnostic imagingABSTRACT
Resumo Os tumores de hipófise representam aproximadamente 15% de todos os tumores cerebrais e dependendo do tamanho, pressionam o quiasma óptico, resultando em comprometimento da função visual que se manifesta como defeitos no campo visual, diminuição da acuidade visual e da visão das cores. O objetivo do presente estudo foi relatar um caso de macroadenoma de hipófise com compressão do quiasma óptico e defeito no campo visual, tratado inicialmente como glaucoma, levando a um diagnóstico e tratamento tardio.
Abstract Pituitary tumors represent approximately 15% of all brain tumors and depending on size, pressure optic chiasma, resulting in impaired visual func-tion that manifests itself as defective in the visual field, decreased acuity visual and color vision. The ob-jetive of the present study was to report a case of pitui-tary macroadenoma with compression of optical chiasma and visual field de-fect, initially treated as glaucoma, leading to a late diagnosis and treatment.
Subject(s)
Humans , Male , Adult , Optic Chiasm/physiopathology , Pituitary Neoplasms/diagnosis , Visual Acuity , Visual Fields , Adenoma/diagnosis , Color VisionABSTRACT
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.
Subject(s)
Humans , Female , Pregnancy , Infant, Newborn , Adult , Young Adult , Adenoma/surgery , Galactorrhea , Hypopituitarism/etiology , Pituitary Neoplasms/surgery , Pituitary Neoplasms/diagnostic imaging , Neoplasm Recurrence, LocalABSTRACT
Introducción: El cáncer de tiroides es la neoplasia endocrina más frecuenteyel Carcinoma Papilar de tiroides representa el 80% de los casos. Por otro lado, la acromegalia es un trastorno poco diagnosticado con una incidencia estimada anual de 4 casos por millón de personas. Presentamos un caso en el que se presentan las dos entidades al mismo tiempo en un paciente y la discusión del posible nexobio químico. Caso clínico: Mujer de 23 años, con masa nodular pétrea de 3 centímetros, en polo superior de lóbulo derecho de tiroides; sometida a tiroidectomía radical y linfadenectomía cervical bilateral, reporte histopatológico de carcinoma papilar. Evoluciona con cambios visuales en ojo izquierdo, cefalea, amenorrea de 1 año, galactorrea negativa, características morfo-anatómicas de acromegalia, campimetría hemianopsia homónima izquierda. IRM de cerebro lesión sellar y suprasellar hiperintensa en T2 en relación a macroadenoma hipofisario. Bioquímicos: Prolactina 131.20, GH 2.7, ACTH 18.5, IGF1 434, IGFBP 35.8, TTOG para GH que se informa: GH 0 min: 10.7, GH 30 min: 9.24, GH 60 min: 7.9. Evolución: La paciente fue sometida a hipofisectomía transesfenoidal endoscópica parcial. En tratamiento con cabergolina 0.5 mg bisemanal, levotiroxina 225 mcg día y octreótide 20 mg mensual. Conclusión: Se concluye que siendo la acromegalia una enfermedad rara se asocia a cáncer de tiroides, implicando posibles mutaciones en la subunidad α de la proteína G.
Introduction:Thyroid cancer is the most frequent endocrine neoplasia and Papillary thyroid carcinoma represents 80% of cases. On the other hand, acromegaly is a poorly diagnosed disorder with an estimated annual incidence of 4 cases per million people. We present a case in which the two entities occur at the same time in a patient and the discussion of the possible biochemical link. Clinical case: 23-year-old woman, with a 3 cm stone nodular mass in the upper pole of the right thyroid lobe; submitted to radical thyroidectomy and bilateral cervical lymphadenectomy, histopathological report of papillary carcinoma. It evolves with visual changes in the left eye, headache, 1-year amenorrhea, negative galactorrhea, morpho-anatomical characteristics of acromegaly,left homonymous hemianopia campimetry. T2 hyperintense seal and suprasellar lesion brain MRI in relation to pituitary macroadenoma. Biochemicals: Prolactin 131.20, GH 2.7, ACTH 18.5, IGF1 434, IGFBP 35.8, TTOG for reported GH: GH 0 min: 10.7, GH 30 min: 9.24, GH 60 min: 7.9. Evolution: The patient underwent partial endoscopic transsphenoidal hypophysectomy. In treatment with cabergoline 0.5 mg twice weekly, levothyroxine 225 mcg day and octreotide 20 mg monthly. Conclusion: It is concluded that, being acromegaly a rare disease, it is associated with thyroid cancer, involving possible mutations in the α subunit of the G protein.
Subject(s)
Humans , Thyroid Gland , Acromegaly , Thyroid Cancer, Papillary , Pituitary Neoplasms , Case Reports , Thyroid NeoplasmsABSTRACT
La mayoría de los adenomas hipofisarios son esporádicos, pero un 3-5% puede ocurrir en un contexto familiar y hereditario. Este es el caso de la neoplasia endocrina múltiple de tipo 1 (NEM1), complejo de Carney (CNC) y adenomas hipofisarios aislados familiares (FIPA). El FIPA es una condición infrecuente, que ocurre en un contexto familiar, no asociada a NEM t ipo1 ni CNC. Los FIPA pueden ser homogéneos (todos los adenomas tienen el mismo fenotipo) o heterogéneos (diferente fenotipo tumoral). Describimos una familia congolesa en la que dos hermanas y una prima fueron diagnosticadas a los 29, 32 y 40 años, respectivamente, con un prolactinoma (FIPA homogéneo). Las pacientes presentaron macroadenomas no invasivos al momento del diagnóstico, con buena respuesta biológica y tumoral al tratamiento con cabergolina hasta una dosis máxima de 1.5 mg/semanal. De las dos hermanas, una cursó un embarazo sin complicaciones. Durante el seguimiento de 12 años, ninguna de ellas presentó elementos clínicos o biológicos compatibles con NEM1 o CNC, por lo que dichos genes no se estudiaron. El análisis genético en dos de las pacientes permitió descartar la posibilidad de una mutación germinal del gen aryl hydrocarbon receptor interacting protein (AIP). Se considera que el 80% de los pacientes con FIPA no presentan mutación del gen AIP, por lo que se requieren futuros estudios en este tipo de familias, para poder determinar otros genes afectados involucrados en su fisiopatología.
Most pituitary adenomas are sporadic, but 3-5% can occur in a family and hereditary context. This is the case of multiple endocrine neoplasia type 1 (MEN1), Carney complex (CNC) and familial isolated pituitary adenomas (FIPA). FIPA is an infrequent condition that occurs in a family context, not associated with MEN type1 or CNC. FIPA kindred can be homogeneous (all adenomas affected in the family having the same tumor phenotype) or heterogeneous (different tumor phenotypes in the affected members). We describe a Congolese family in which two sisters and a cousin were diagnosed with a prolactinoma (homogenous FIPA) at the ages of 29, 32 and 40 years, respectively. The patients presented with macroadenomas at the time of diagnosis, non-invasive tumors and good biological response to cabergoline treatment (maximum dose of 1.5 mg/weekly). Of these two sisters, one went through a pregnancy without complications. Because no MEN1 and CNC clinical and biochemical features were detected during the 12-year follow-up, these genes were not investigated. The genetic analysis of the aryl hydrocarbon receptor interacting protein (AIP) was normal. As nearly 80% of patients with FIPA do not have a mutation in the AIP gene, future studies in these families are required to identify other affected genes involved in their physiopathology.