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Erdheim–Chester disease (ECD) is a rare disease with a poor prognosis. The first two cases were reported by Jakob Erdheim and William Chester in 1930. The etiology and disease incidence are unknown. One of the main components of this disorder is a chronic uncontrollable inflammation. In 2016 ECD was classified as histiocytic neoplasm by the World Health Organization and was categorized as “tumors of histiocytes and dendritic cells”. More than half of patients testing positive for the BRAF mutation. The final diagnosis is made on the basis of histological finding. There are radiological markers indicating a possible association with the disease. This is a specific, almost pathognomonic scintigraphic picture of the skeleton, “coated” aorta, “hairy” kidney patterns on computer tomography. There is a Erdheim–Chester Disease Global Alliance (ECDGA), which try to unite and provide with information about diagnostics and treatment of this rare disease both patients and doctors. Today, there are 571 registered patients and only one from Russia. We demonstrate three clinical cases of patients with newly diagnosed ECD and the role of nuclear medicine methods in assessing the prevalence of this disease.

The study objective : using a clinical example to demonstrate possibilities of single photon emission computerized tomography (SPECT) in combination with computed tomography (CT) in identifying latent bone metastases, taking into account the dynamics growth of serum basal calcitonin. Materials and methods . Patient S., 60 years old, visited N.N. Blokhin National Medical Research Center of Oncology for consultation on multiple lung metastases of cancer of unknown primary. Results. Taking into account basal calcitonin level, immunohistochemistry, ultrasound investigation andfine-needle aspiration biopsy of the node in the right thyroid lobe a diagnose of medullary thyroid cancer was made. CT revealed multiple metastases in both lungs. Specialist erformed thyroidectomy with central lymphadenectomy and facial neck dissection on both sides. During next four months basal calcitonin level increased twice. Control contrast CT lung screening showed the growth of previously identified metastases and the appearance of multiple new ones. Bone scan showed focuses of increased radio-pharmaceutical accumulation in the area of 7 h left rib, left iliac wing, in the left bones of cranial vault, in C 7 , Th 6 , Th 9 , Th I2 vertebrae and right foot bones. Additional examination using SPECT/CT (from the skull base to the hip joints) revealed metastases in corpuses of Th 9 , Th I2 vertebrae and the left iliac wing, and suspicion for metastasis in 7 h left rib. Magnetic resonance imaging (MRI) confirmed metastasis in Th 9 , Th I2 , L 3 vertebral bodies and in the left iliac wing. Conclusion. Conclusion . In this clinical example, SPECT/CT allowed to correctly detect metastases in both Th 9 and Th I2 vertebrae while bone scan was questionable, and MRI showed an additional damage of L 3 vertebra. Changes in the 7 h left rib could not be verified using CT, although this changes may be an emerging metastasis. Obviously, extensive use of radiation methods does not guarantee complete identification of all pathological focuses, it therefore allows assessing the prevalence of the pathological process. It is crucially important to control calcitonin level in these patients, since its rapid growth allowed suspecting additional distant metastases.

Rosai–Dorfman disease or sinus histiocytosis is a rare entity which may affect various lymph node groups. Extranodal form is observed in 45 % of cases, facial bone involvement is especially rare. A 62-year old male presented with complaints on dumb sensation of the chin and lower lip. After clinical assessment and surgical exploration an intraosseous extranodal Rosai–Dorfman disease was diagnosed. The patient under-went free fibula osseous graft reconstruction. After 2 years the patient is recurrent free. The purpose of this publication is to describe a rare case of lower jaw involvement in an intraosseous form with significant bone destruction. The clinical and microscopic features of the process are discussed.

Introduction. The issue of selecting treatment strategy for сT2N0M0 cancer of the oral mucosa remains relevant. Induction chemotherapy (applied in order to evaluate the possibility of organ-preserving chemoradiotherapy) is widely used in clinical practice. In this case, surgical treatment is performed only when chemotherapy (CT) is ineffective or a residual tumor is detected after chemoradiotherapy (CRT). However, many specialists recommend surgical treatment on the first stage of treatment. Objective: evaluation of the induction chemotherapy feasibility in the treatment of T2N0M0 cancer of the oral mucosa. Materials and methods . A total of 122 patients with primary Т2N0М0 cancer of oral mucosa without regional metastases (according to clinical examination) were included in the study. Results . In order to determine the best treatment strategy, we have compared treatment outcomes in three groups of patients. The most favorable outcomes were observed in the group of patients receiving combined treatment according to a scheme «surgery + radiotherapy (RT)/ CRT, where the overall 5-year survival rate and the progression-free 5-year survival rate were 70.4 % and 68.5 % respectively. Survival rate in this group was significantly higher (р < 0.05) than that in the group «induction CT (ICT) ± RT ± surgery» where these parameters comprised 42.2 % and 36.2 % respectively. Conclusion . Combined treatment with a surgery on the first stage followed by RT or CRT ensures better outcomes of the cancer of the oral mucosa than the strategies that include ICT.

This review paper describes the possibilities of visualization of regional lymph nodes using contrast-enhanced ultrasound. Discussed is the experience of contrast-enhanced ultrasound implication for differential diagnosis of regional metastases of squamous cell carcinoma of the oral mucosa. The basics of the technique of contrast-enhanced ultrasound examination of the neck lymphatic basin are described.В данной работе представлено описание возможностей визуализации регионарных лимфоузлов с применением контрастно-усиленного ультразвука. Обсуждается опыт применения контрастно-усиленного ультразвука в дифференциальной диагностике регионарных метастазов плоскоклеточного рака слизистой полости рта. Описываются основы методики технического и клинического обеспечения выполнения контрастно-усиленного ультразвука для обследования лимфоаппарата шеи.

The paper describes properties of clinical visualization approaches which are applied for detection of sentinel lymph nodes in patients suffering from oral cavity squamous cell cancer. Diagnostic efficiency results and technological features of different imaging techniques are discussed.В данной статье представлена характеристика способов визуализации, применяемых при выявлении сторожевых лимфатических узлов у пациентов, страдающих плоскоклеточным раком слизистой оболочки полости рта. Обсуждаются результаты оценки диагностической эффективности и особенности технологического обеспечения биопсии сторожевых лимфатических узлов.

The paper presents literature review and clinical case of patient with fibromyxoid sarcoma of maxilla illustrating diagnostic difficulties and efficacy of multidisciplinary treatment approach.В статье представлен анализ данных литературы и клинический случай больной с фибромиксоидной саркомой верхней челюсти, иллюстрирующий трудности диагностики данного новообразования и необходимость междисцилинарного подхода к лечению.