Клинико-морфологические наблюдения нейробластомы надпочечников у взрослых

Abstract

У статті наведено огляд літературних даних стосовно частоти, локалізації, гістологічної структури нейробластоми. Приведено два випадки спостереження нейробластоми надниркових залоз у дорослих, нерозпізнаних за життя. Наведені спостереження свідчать, що нейробластома наднирникових залоз не є виключно рідкісним захворюванням у дорослих. З іншого боку, прижиттєва діагностика даної пухлини представляє істотні труднощі, так як нейробластома маскується під інші захворювання; В статье приведен обзор литературных данных о частоте встречаемости, локализации, гистологической структуре нейробластомы. Приведено два случая наблюдения нейробластомы надпочечников у взрослых, нераспознанных при жизни. Приведенные наблюдения свидетельствуют, что нейробластома надпочечников не является исключительно редким заболеванием у взрослых. С другой стороны, прижизненная диагностика данной опухоли представляет существенные трудности, так как нейробластома маскируется под другие заболевания; Neuroblastoma (simpatogonioma) is 8-10 % of all malignant tumors in children younger than 15 years. More than 90 % of the tumors occur in children under 5 years old, the peak incidence occurs in the first year of life. Feature of neuroblastoma is its clinical heterogeneity, ranging from benign subtypes, which are capable of spontaneous regression or maturation in ganglioneuroma (2 to 5 %) to the aggressive prone to metastasize rapidly forms. Microscopically, in typical cases, neuroblastoma consists mainly of small cells, rounded, with a small, round or oval nuclei surrounded by a thin rim of cytoplasm that the visual examination by light-optical picture level creates “bare core”. Partly detected characteristic of neuroblastoma pseudorosets as beaters from the cells in the center of which is determined by gently fibrillar substance ( pseudo-rosettes of Homer-Wright). According to modern concepts, for morphological verification of this type of tumors, prediction of clinical course and choice of optimal therapy should be used immunohistochemical techniques for the study. Quite often localized neuroblastoma in the adrenal glands. In children, this localization of the tumor is observed in 32 % of cases and is the most common, which is connected apparently with the features and the development of adrenal germs in embryogenesis. We present two cases of neuroblastoma observation (siympathogonioma) adrenal adults without diagnosed during his lifetime. Patient B., 45 years old, was admitted to the City Hospital with suspected Wegener’s disease, the scourge of the larynx, and then with the diagnosis - glomerulonephritis. The patient was observed sustained reduction in blood pressure, which was corrected with corticosteroids. Subsequently, the patient appeared bronze color of the skin and increased liver. After 10 days of hospitalization, with symptoms of acute cardiovascular failure patient died. The final clinical diagnosis of Wegener’s disease: ulcerative laryngitis, acute glomerulonephritis. Gastric cancer with liver metastases. Toxic hepatitis, myocarditis, low-lobular pneumonia. At the post mortem examination of the corpse found both adrenal neuroblastoma with metastases to the liver and heart. The immediate cause of death of the patient - adrenal insufficiency. Patient T, 70 years old, admitted to the emergency department with suspected renal colic, acute cholecystitis. After 14 hours of hospitalization in the events of the fall in blood pressure patient died. At autopsy discovered adrenal neuroblastoma with metastases to the deep inguinal, retroperitoneal, cervical lymph nodes. The patient’s death occurred as a result of adrenal insufficiency. These observations indicate that the adrenal neuroblastoma is a very rare disease in adults. On the other hand, the lifetime diagnosis of this tumor is considerable difficulties as neuroblastoma masked by other diseases.