Работа выполнена в соответствии с плановой НИР отделения хирургической эндокринологии ГБУЗ МО «Московский областной научно-исследовательский институт им. М. Ф. Владимирского» « Разработка инновационных технологий в диагностике, лечении, реабилитации и мониторинге пациентов с эндокринными опухолями», тема № 38 в разделе «Онкология». Учреждением, финансирующим исследование, является Министерство Здравоохранения Московской области. Авторы гарантируют ответственность за объективность представленной информации. Авторы гарантируют отсутствие конфликта интересов и собственной финансовой заинтересованности. Рукопись поступила в редакцию 14.03.2018.
BACKGROUND: Adrenocortical carcinoma is a rare and aggressive disease. Tumor recurrence prevention is vital for increasing patients survival rate. Therefore, the identification of prognostic markers is of particular importance. AIM: To evaluate clinical, laboratory and chromatographic criteria for adrenocortical carcinoma recurrence using gas chromatography-mass spectrometry to optimize patients follow-up. MATERIALS AND METHODS: 40 patients [10 (25%) men and 30 (75%) women] with histologically confirmed adrenocortical carcinoma (according to the European Network for the Study of Adrenal Tumors stage I 3, II 29, III 8 patients at presentation) have been recruited. Measurement of 24-hour urinary steroid metabolite excretion has been carried out by gas chromatography-mass spectrometry (GCMS-ТQ8050, Shimadzu) in preoperative period. The survival distribution has been assessed according to the KaplanMeier method. Cox proportional hazards regression methods have been used to determine predictive factors on recurrence-free survival. RESULTS: The patients with early stages of disease (III versus III) had a significantly longer recurrence-free survival, overt hypercortisolism was associated with significantly shorter recurrence-free survival. A significant correlation between pregnenediol urinary excretion and tumor diameter, stage has been found. Increased urinary excretion of tetrahydro-11-deoxycortisol, pregnenediol was associated with a decreased recurrence-free survival in the patients with overt hypercortisolism. Increased urinary excretion of 16-OH-dehydroepiandrosterone was a marker of a shorter recurrence-free survival in the patients without Cushing syndrome. Increased urinary excretion of pregnenediol, pregnenetriol, 16-oxo-androstenediol in all the adrenocortical carcinoma patients was associated with a recurrence risk. In the multivariate analysis pregnenediol urinary excretion, stage and overt cortisol excess were significantly and independently associated with a shorter recurrence-free survival. CONCLUSIONS: Adrenocortical carcinoma recurrence prognostic markers have been found by gas chromatography-mass spectrometry. The application of these findings may improve patient-centered outcomes.
Работа выполнена в соответствии с плановой НИР отделения хирургической эндокринологии ГБУЗ МО «Московский областной научно-исследовательский институт им. М. Ф. Владимирского» «Разработка инновационных технологий в диагностике, лечении, реабилитации и мониторинге пациентов с эндокринными опухолями», тема № 38 в разделе «Онкология». Учреждением, финансирующим исследование, является Министерство Здравоохранения Московской области. Авторы гарантируют ответственность за объективность представленной информации. Авторы гарантируют отсутствие конфликта интересов и собственной финансовой заинтересованности. Рукопись поступила в редакцию 05.03.2018.
In the lecture on the basis of literature data and the results of our research deals with the basic aspects of insufficiently studied problems of metastatic adrenal tumors. Special attention is drawn to the modern algorithm of examination of these patients, the surgical treatment strategy and evaluation of long-term results. For the period from 1997 to 2016 in the surgical clinics of MONIKI operated on 56 patients with metastatic adrenal tumors (MAT). 22 MAT were synchronous, 36 — metachronous. From the 46 produced a «potentially radical» adrenalectomy with tumor (40 unilateral, 6 bilateral), 6 — partial removal of the MAT, in 4 open biopsy. 46 radically operated patients survival rates were analyzed in 37, the average follow-up period was 36.5 months. Overall and disease-free 5-year survival rate of patients with MAT was 46.8 % and 27.6 %, respectively. The most favorable results of surgical treatment of patients with isolated metastases of clear cell kidney cancer in the adrenal glands. Unilateral adrenalectomy is indicated for patients with solitary synchronous and metachronous tumors, total adrenalectomy — bilateral with isolated neoplastic lesions of the adrenal glands.
Relevance of research: аdrenalectomy remains the only method of potentially radical treatment of adrenocortical carcinoma (AСС), and the complete resection is considered one of the leading factors in the prognosis of disease. The question of the possibility and feasibility of using video- endoscopic technologies in the treatment of patients with ACC is still being discussed. Objective: the purpose of our study was to evaluate the effectiveness of laparoscopic adrenalectomy in the treatment of ACC patients. Material and methods: the study is based on the analysis of the results of diagnostics and surgical treatment of 12 patients who underwent laparoscopic adrenalectomy and verified ACC during histological and immunohistochemical studies. Results: stage I were verified (according to the ENSAT) in 4 cases, in 4 - II stage, and in 4 - III stage. The median of tumor size was 4.9 cm. Intraoperative and postoperative complications did not occur. In all cases, the R0-resection was confirmed by morphological examination. The median patient follow-up was 71 months (1; 141), during this period 11 patients were alive, 1 patient, who had surgery in stage III died of disease progression (distant metastases) 49 months after surgery. During the follow-up period tumor recurrence did not occur. Overall 5-year survival was 75% (95% CI; 30-95%). Conclusion: despite the small number of patients included in this study, satisfactory immediate results (absence of complications, R0-resection) and long-term results allow us to consider video- endoscopic surgery as an effective treatment for patients with ACC.
The diagnosis and treatment of multiple endocrine neoplasia type 2A syndrome or Sipple’s syndrome, as well as other endocrine hereditary syndromes, presents certain difficulties for several reasons. One of them is the rarity of this pathology and, as a result, the lack of awareness of physicians. In addition, the diagnosis requires a comprehensive examination and a high level of clinical, laboratory, radiation and morphological diagnostics. And finally, surgical treatment, which is essentially the only method, involves an individual approach to each patient. We present the clinical case of Sipple's syndrome in a 39-year-old man. The diagnosis was carried out of medical history, clinical examination, laboratory tests (24-hour urine excretion of metanephrines and normetanephrines; serum calcium, calcitonin and parathyroid hormone levels), neck ultrasound and abdominal CT scan and was confirmed by histopathological examination and genetic testing for RET mutation. The achievement of the endpoint in the form of normalization of blood pressure and the absence of adrenal insufficiency was due to radical surgical treatment.
Background. Adrenocortical carcinoma (ACC) is a rare malignant tumor characterized by an annual incidence of 0.5—2 cases per million population. Surgery is the-first line treatment for ACC. When total tumor resection is not possible due to its proliferation or progression, mitotane (o,p’DDD) is used. In this case, stabilization and partial response (as assessed by RECIST criteria) was observed only in 48.7% of cases, necessitating the search for new therapeutic targets. Objective — the study was aimed at assessing the somatostatin receptor expression in adrenocortical carcinomas and adrenal cortex tumors with uncertain malignant potential. Material and methods. Surgical material from adrenocortical tumors of 13 patients (4 males and 9 females aged from 28 to 68 years) was used. In all cases, the diagnosis was verified by morphological and immunohistochemical (IHC) studies: ACC was detected in 10 cases (including 1 case of ACC liver metastasis), oncocytic carcinoma — 1 case, oncocytoma with uncertain malignant potential — 2 cases. Morphological assessment of ACC was carried out according to Weiss criteria (for ACC tumors) and Lin—Weiss—Bisceglia criteria (for oncocytic neoplasms of the adrenal cortex). IHC study was carried out with antibodies to the spectrum of adrenal cortex-specific markers, as well as Ki-67 and somatostatin receptors 2 and 5 (SSTR2 and SSTR5). Results. The expression of SSTR2 and/or 5 was detected in 8 (61.5%) of 13 cases of ACC. Isolated SSTR2 expression was observed in 4 cases (4/13), while SSTR5 expression was observed in 6 cases (6/10). In 2 cases (2/10), co-expression of both receptor types was observed. SSTR expression was observed both in ACC and ACC liver metastasis, as well as in oncocytic ACC. Conclusion. SSTR2 and/or 5 expression in ACC tissue expands diagnostic and prognostic capabilities for this pathology.
Наиболее часто первичный гиперпаратиреоз (ПГПТ) в сочетании с раком (как правило, медуллярным) щитовидной железы (ЩЖ) встречается при синдромах множественных эндокринных неоплазий. Сочетание немедуллярных карци-ном ЩЖ и ПГПТ отмечается у 3% пациентов. Доля папиллярного рака (ПР) ЩЖ достигает 87% от всех ее злокачествен-ных опухолей. Атипическая аденома (АА) околощитовидной железы (ОЩЖ) -это новообразование, в котором отсут-ствуют достоверные признаки инвазивного роста, но есть морфологические критерии, подозрительные в отношении их злокачественного потенциала. Распространенность АА ОЩЖ как причины ПГПТ составляет около 0,5-4%. АА ОЩЖ относятся к опухолям неопределенного злокачественного потенциала. Клиническое значение и отдаленные результаты, а также объем оперативного вмешательства и продолжительность наблюдения за пациентами с АА ОЩЖ из-за редкости опухоли и отсутствия стандартов диагностики данной патологии не определены. В настоящей статье авторы сообщают о двух случаях сочетания АА ОЩЖ и ПР ЩЖ у 63-летней женщины и 57-летнего мужчины. У одного из пациентов, в отличие от второго, были классические симптомы ПГПТ, в том числе тяжелая остео-дистрофия и нефропатия. Предоперационный уровень кальция составил 3,48 и 4,1 (2,12-2,6) мкмоль/л, паратиреоидно-го гормона -1300 и 1533 (15-65) пг/мл соответственно. В обоих случаях ультразвуковое исследование ЩЖ не выявило достоверно злокачественных новообразований. Рак ЩЖ был заподозрен только во время интраоперационной ревизии, в связи с чем пациентам была проведена тиреоидэктомия и удаление опухоли ОЩЖ. Гистологическое исследование выявило папиллярную микрокарциному ЩЖ (в первом случае одностороннюю, во втором -двустороннюю) и АА ОЩЖ. Большая осведомленность специалистов о сочетании ПРЩЖ с атипической аденомой ОЩЖ позволит расширить насто-роженность эндокринологов и хирургов, своевременно оценить возможную патологию ЩЖ у пациентов с ПГПТ, тем самым применить адекватный объем вмешательства в ходе одной операции. Клю че вые сло ва: папиллярный рак щитовидной железы, атипичная аденома околощитовидной железы, первичный гипер-паратиреоз.Most clinicians are well aware of the coexistence of medullary thyroid cancer and hyperparathyroidism in hereditary and sporadic multiple endocrine neoplasia syndromes. Тhe reported incidence of nonmedullary thyroid carcinoma in patients with primary hyperparathyroidism (pHPT) is only approximately 3%. Papillary thyroid carcinomas (PTC) is a malignant epithelial tumour. PTC represent up to 87% of all thyroid carcinomas. Atypical parathyroid adenoma (APA) are a subset of parathyroid neoplasms that exhibit some of the features of parathyroid carcinoma but lack unequivocal invasive growth. APA represents about 0.5-4% of cases of PHPT. As a group, they may be considered tumors of uncertain malignant potential. The clinical importance, and long-term outcomes as well as appropriate operative management and surveillance are not well defined for APA probably due to the overall low prevalence as well as the lack of a standard definition of APA. We rep...
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