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BACKGROUND: Crooke cell adenomas (CCAs) are a rare, aggressive subset of secretory pituitary corticotroph adenomas (sCTAs) found in 5%-10% of patients with Cushing disease. Multiple studies support worse outcomes in CCAs but are limited by small sample size and single-institution databases. We compared outcomes in CCA and sCTA using a multicenter, international retrospective database of high-volume skull base centers. METHODS: Patients surgically treated for pituitary adenoma from January 2017 through December 2020 were included. RESULTS: Among 2826 patients from 12 international centers, 20 patients with CCA and 480 patients with sCTA were identified. No difference in baseline demographics, tumor characteristics, or postoperative complications was seen. Microsurgical approaches (60% CCA vs. 62.3% sCTA) were most common. Gross total resection was higher in CCA patients (100% vs. 83%, P = 0.05). Among patients with gross total resection according to intraoperative findings, fewer CCA patients had postoperative hormone normalization of pituitary function (50% vs. 77.8%, P < 0.01) and remission of hypersecretion by 3-6 months (75% vs. 84.3%, P < 0.01). This was the case despite CCA having better local control rates (100% vs. 96%, P < 0.01) and fewer patients with remnant on magnetic resonance imaging (0% vs. 7.2%, P < 0.01). A systematic literature review of 35 studies reporting on various treatment strategies reiterated the high rate of residual tumor, persistent hypercortisolism, and tumor-related mortality in CCA patients. CONCLUSIONS: This modern, multicenter series of patients with CCA reflects their poor prognosis and reduced postsurgical hormonal normalization. Further work is necessary to better understand the pathophysiology of CCA to devise more targeted treatment approaches.
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Adenoma Hipofisario Secretor de ACTH , Adenoma , Neoplasias Hipofisarias , Humanos , Adenoma Hipofisario Secretor de ACTH/cirugía , Adenoma Hipofisario Secretor de ACTH/complicaciones , Estudios Retrospectivos , Adenoma/diagnóstico por imagen , Adenoma/cirugía , Adenoma/complicaciones , Neoplasias Hipofisarias/patología , Hipófisis/cirugía , Hipófisis/patología , Resultado del Tratamiento , Estudios Multicéntricos como AsuntoRESUMEN
SUMMARY: Pituitary infections, particularly with fungus, are rare disorders that usually occur in immunocompromised patients. Cushing's syndrome predisposes patients to infectious diseases due to their immunosuppression status. We report the case of a 55-year-old woman, working as a poultry farmer, who developed intense headache, palpebral ptosis, anisocoria, prostration and psychomotor agitation 9 months after initial diabetes mellitus diagnosis. Cranioencephalic CT scan showed a pituitary lesion with bleeding, suggesting pituitary apoplexy. Patient underwent transsphenoidal surgery and the neuropathologic study indicated a corticotroph adenoma with apoplexy and fungal infection. Patient had no preoperative Cushing's syndrome diagnosis. She was evaluated by a multidisciplinary team who decided not to administer anti-fungal treatment. The reported case shows a rare association between a corticotroph adenoma and a pituitary fungal infection. The possible contributing factors were hypercortisolism, uncontrolled diabetes and professional activity. Transsphenoidal surgery is advocated in these infections; however, anti-fungal therapy is still controversial. LEARNING POINTS: Pituitary infections are rare disorders caused by bacterial, viral, fungal and parasitic infections. Pituitary fungal infections usually occur in immunocompromised patients. Cushing's syndrome, as immunosuppression factor, predisposes patients to infectious diseases, including fungal infections. Diagnosis of pituitary fungal infection is often achieved during histopathological investigation. Treatment with systemic anti-fungal drugs is controversial. Endocrine evaluation is recommended at the time of initial presentation of pituitary manifestations.
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SUMMARY: Silent corticotroph adenoma (SCA) is an unusual type of nonfunctioning pituitary adenoma (NFA) that is silent both clinically and biochemically and can only be recognized by positive immunostaining for ACTH. Under rare circumstances, it can transform into hormonally active disease presenting with severe Cushing syndrome. It might often produce diagnostic dilemma with difficult management issue if not thoroughly investigated and subtyped accordingly following surgery. Here, we present a 21-year-old male who initially underwent pituitary adenomectomy for presumed NFA with compressive symptoms. However, he developed recurrent and invasive macroadenoma with severe clinical as well as biochemical hypercortisolism during post-surgical follow-up. Repeat pituitary surgery was carried out urgently as there was significant optic chiasmal compression. Immunohistochemical analysis of the tumor tissue obtained on repeat surgery proved it to be an aggressive corticotroph adenoma. Though not cured, he showed marked clinical and biochemical improvement in the immediate postoperative period. Anticipating recurrence from the residual tumor, we referred him for cyber knife radio surgery. LEARNING POINTS: Pituitary NFA commonly present with compressive symptoms such as headache and blurred vision. Post-surgical development of Cushing syndrome in such a case could be either drug induced or endogenous. In the presence of recurrent pituitary tumor, ACTH-dependent Cushing syndrome indicates CD. Rarely a SCA presenting initially as NFA can transform into an active corticotroph adenoma. Immunohistochemical marker for ACTH in the resected tumor confirms the diagnosis.
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BACKGROUND/AIMS: Alcoholic hepatitis (AH) is an acute hepatic inflammation associated with high morbidity and mortality. Treatment with steroids is known to decrease short-term mortality in severe AH patients. Hence, we hypothesize that adrenal insufficiency can be associated with severe AH and affects prognosis. The aim of this study was (1) to evaluate relative adrenal insufficiency (RAI) in patients with AH and (2) to Compare RAI with the severity of AH. METHODS: Newly diagnosed cases of AH hospitalized in SMS Medical College and Hospital, Department of Gastroenterology were, enrolled. All patients of AH were classified as mild and severe AH on the basis of Maddrey discriminant function (DF). After baseline serum cortisol, 25 IU ACTH (Adreno Corticotrophic Hormone) was injected intramuscularly and blood sample was collected after 1 h and assessed for serum cortisol. RAI was defined as <7 µg increase in the cortisol level from baseline. RAI was compared with severity of AH. RESULTS: Of 120 patients of AH, 58 patients fulfilled the inclusion criteria, in which 48 patients were diagnosed as severe AH and 10 patients were diagnosed as mild AH. In patients with severe AH, the baseline mean serum cortisol level was significantly high as compared with mild AH; 26 patients (54.16 %) of 48 patients with severe AH showed RAI (P ≤ 0.001).Whereas in patients with mild AH, none of patients showed RAI. RAI also showed negative correlation with DF. There was no difference in RAI with respect to acute kidney injury (AKI). CONCLUSION: RAI is a common entity in patients with severe AH, and it is related with the severity of disease.
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INTRODUCTION AND OBJECTIVE: The ability to predict recurrence of pituitary adenoma (PA) after surgery may be helpful to determine follow-up frequency and the need for adjuvant treatment. The purpose of this study was to assess the prognostic capacity of pituitary tumor transforming gene (PTTG), insulin-like growth factor 1 receptor (IGF1R), and Ki-67. MATERIALS AND METHODS: In this retrospective study, the normalized copy number (NCN) of PTIG and IGF1R mRNA was measured using RT-PCR, and the Ki-67 index was measured by immunohistochemistry in 46 PA samples. Clinical data, histological subtype, and radiographic characteristics were collected to assess associations between variables and tumor behavior. Progression of tumor remnants and its association to markers was also studied in 14 patients with no adjuvant treatment after surgery followed up for 46±36 months. RESULTS: Extrasellar tumors had a lower PTTG expression as compared to sellar tumors (0.065 [1st-3rd quartile: 0.000-0.089] NCN vs. 0.135 [0.105-0.159] NCN, p=0.04). IGF1R expression changed depending on histological subtype (p=0.014), and was greater in tumor with remnant growth greater than 20% during follow-up (10.69±3.84 NCN vs. 5.44±3.55 NCN, p=0.014). CONCLUSIONS: Our results suggest that the IGF1R is a more helpful molecular marker than PTTG in PA management. Ki-67 showed no association to tumor behavior. However, the potential of these markers should be established in future studies with standardized methods and on larger samples.
Asunto(s)
Biomarcadores de Tumor/análisis , Antígeno Ki-67/análisis , Proteínas de Neoplasias/biosíntesis , Neoplasias Hipofisarias/química , Receptor IGF Tipo 1/biosíntesis , Securina/biosíntesis , Adulto , Anciano , Antígenos de Neoplasias/análisis , Antineoplásicos Hormonales/uso terapéutico , Terapia Combinada , Supervivencia sin Enfermedad , Agonistas de Dopamina/uso terapéutico , Femenino , Estudios de Seguimiento , Regulación Neoplásica de la Expresión Génica , Humanos , Hipofisectomía , Masculino , Persona de Mediana Edad , Terapia Neoadyuvante , Invasividad Neoplásica , Proteínas de Neoplasias/análisis , Proteínas de Neoplasias/genética , Recurrencia Local de Neoplasia/epidemiología , Neoplasias Hipofisarias/clasificación , Neoplasias Hipofisarias/tratamiento farmacológico , Neoplasias Hipofisarias/patología , Neoplasias Hipofisarias/cirugía , Pronóstico , ARN Mensajero/análisis , ARN Mensajero/biosíntesis , ARN Neoplásico/análisis , ARN Neoplásico/biosíntesis , Receptor IGF Tipo 1/análisis , Receptor IGF Tipo 1/genética , Estudios Retrospectivos , Securina/análisis , Securina/genética , Somatostatina/análogos & derivados , Somatostatina/uso terapéutico , Resultado del TratamientoRESUMEN
Objetivos: En el presente trabajo se revisará nuestra serie de casos de Adenomas Corticotrópos silentes y revisar la literatura acerca de esta patología fascinante. Material y Método: Se revisaron las historias clínicas de 5 pacientes del Hospital Universitario Fundación Santa Fé de Bogotá en el periodo de enero de 1990 a enero de 2008, que se le realizó tratamiento para una masa sellar y se le realizó diagnóstico patológico de Adenoma Corticotrópos Silente. Discusión: Los adenomas de hipófisis corresponden entre el 17 por ciento de los tumores intracraneales. El 60 por ciento de estas lesiones se asocian a hipersecreción hormonal. Los adenomas corticotropos silentes son tumores hipofisarios que histológicamente muestran diferenciación corticotrópica con algún grado de secreción de ACTH pero sin la evidencia clínica de síndrome de Cushing. Conclusión: Aunque estos tumores sean clínicamente significativos y su histopatología fascinante, hay poca literatura y estudios clínicos con enfoque especial sobre estas lesiones. Estos tumores son muy importantes tenerlos en cuenta en la patología selar ya que su tratamiento requiere mayor dedicación y seguimiento a estos pacientes.
Objectives: In the present paper we want to review our series of silent corticotrofic adenomas and review the literature concerning this fascinating pathology. Material and Methods: we review the clinical history of 5 patients treated and diagnosed with Silent corticotrofic adenomas in University Hospital Fundacion Santa Fe de Bogota in the period of January 1990 till January 2008. Discussion: Pituitary adenomas count for 10 to 15 percent of intracranial tumors. In 60 percent of the cases there is a hormonal hypersecretory syndrome associated with these mass. Silent Corticotophic adenomas are pituitary tumors which histologically show a corticotrophic differentiation with secretion of ACTH but do not present with clinical evidence of Cushing disease. Although silent corticotophic adenomas are clinically significant and histologically fascinating there are only few clinical studies and medical literature with regard to these tumors. Conclusion: Although silent corticotophic adenomas are clinically significant and histologically fascinating there are only few clinical studies and medical literature with regard to these tumors. It is very important to take in regard this tumor in the pathology of the selar region since these patients demand a major dedication and a strict follow up.