iant extradural dermoid cyst in the right parietal-occipital region with obliteration of the transverse sinus

The study objective – to report a case of giant extradural dermoid cyst in a 48‑year-old woman; the cyst caused extensive erosion of the occipital bone and lower portions of the right parietal bone, which is quite rare.Case report. A 48‑year-old female patient was admitted to the Department of Neurosurgery, A. Ya. Kozhevnikov Clinic of Nervous System Diseases, University Clinical Hospital No. 3, I. M . Sechenov First Moscow State Medical University on 28.07.2020. In 2002, the patient noted the emergence of a 1‑cm subcutaneous formation in the right occipital region. The formation was slowly growing. On 17.12.2015, she underwent magnetic resonance imaging of the brain. It revealed a 2,5 × 4,4 × 4,0 cm extradural formation with clear contours and erosion of the occipital and right parietal bones. On 12.03.2020, she had contrast-enhanced magnetic resonance imaging, which demonstrated that the tumor was growing (its size was 3,0 × 6,4 × 5,5 cm). Magnetic resonance imaging scans also showed extensive erosion (6,4 × 5,4 cm) of the occipital and right parietal bones and obliteration of the right transverse sinus. On 28.07.2020, the patient underwent elective surgery, namely excision of the extradural dermoid cyst in the right parietooccipital region with simultaneous reconstruction of the calvarial bones using a titanium plate. The cyst capsule and its contents were completely removed. The capsule was tightly attached to the dura mater, but did not invade it. To remove small fragments of the capsule that could potentially invade the bone, we performed small marginal resection of the calvarial bones at the depth of 2–3 mm from the defect margin. After the removal of cyst capsule, well-defined furrow could be visualized on the dura mater in the projection of the obliterated transverse sinus. Five days postoperatively, the patient was discharged from hospital in satisfactory condition.Conclusion. Surgical treatment of extradural dermoid cysts implies radical removal of the tumor. Since the cyst capsule gets tightly attached to the edges of the bone defect along with bone erosion, the removal of such cysts as a single block is almost impossible. The risk of severe erosion of the skull bones caused by a dermoid cyst with extradural (and sometimes intradural) growth increases with age. Therefore, it is recommended to remove such tumors as early as possible. Patients with skull bone defects also require cranioplasty.

Evolution of treatment for children with esthesioneuroblastoma (1969–2019)

Introduction. Esthesioneuroblastoma is a rare neuroectodermal malignant tumor. The incidence of this tumor in children under 15 years of age is 0.1 per 100,000 pediatric population. The information on incidence, disease course, and treatment of esthesioneuroblastoma in children vary significantly and depend on the follow-up period and statistical methods.Objective – to assess risk factors for esthesioneuroblastoma and evaluate treatment strategies used in children and their prospects.Materials and methods. This study included 29 patients aged 2 to 17 years diagnosed with esthesioneuroblastoma and treated at the Research Institute of Oncology and Hematology, N.N. Blokhin Russian Cancer Research Center, Ministry of Health of Russia between 1969 and 2019. The median age upon diagnosis was 10 years. Six patients (20 %) developed the disease early in their life (being under 3 years of age). Esthesioneuroblastoma was equally common in boys and girls. More than half of the patients (n = 15; 51 %) had stage IV disease. The primary tumor invaded the maxillary sinus (n = 25; 86 %), orbit (n = 10; 34 %), ethmoid sinus (n = 18; 62 %), and cranial cavity (n = 12; 41 %). The majority of children (n = 28; 96 %) received pharmacotherapy and radiotherapy (n = 27; 93 %); the total focal dose was 50 Gy in case of primary tumor. The affected cervical lymph nodes were irradiated in 10 (35 %) patients (total focal dose 40 Gy). Ten (35 %) patients have undergone surgery.Results. The follow-up period varied between 3 months and 50 years. Nine (34 %) patients are still alive. Fourteen patients (48 %) died due to tumor progression; 1 (3 %) patient died due to complications from special treatment. Five (17 %) patients were lost to follow-up at different stages.Conclusion. The following risk factors were associated with poor prognosis in children with esthesioneuroblastoma: disease onset in early childhood, no adequate diagnosis of distant metastases prior to specific treatment initiation, disseminated disease, and high Ki-67 index. In patients with advanced cancer, surgeries were effective only in combination with chemoradiotherapy. Long-term treatment outcomes in children with esthesioneuroblastoma are poor due to high risk of recurrence and tumor dissemination.

Malignant tumors of the nose and paranasal sinuses: a retrospective review of a Portuguese cancer center’s experience

Introduction. Although uncommon from a population-based perspective, there is considerable morbidity and mortality associated with malignant tumors of the nose and paranasal sinuses.The objective of this study was to characterize the presentation, risk factors, management and survival of patients with these tumors treated at a single institution.Materials and methods. We retrospectively reviewed the clinical records of patients with malignancies of the nose and paranasal sinuses diagnosed between January 2010 and December 2014 at a tertiary cancer center. Univariate and multivariate analysis were performed.Results. Ninety patients were included in the study. Mean age at diagnosis was 62.8 years (range, 2–95 years) and mean follow-up was 44.5 months (range, 2–113 months). The maxillary sinus (33.3 %) and the nasal cavity (32.2 %) were the most frequent sites of origin. Squamous cell carcinoma (36.7 %), mucosal melanoma (15.6 %) and adenoid cystic carcinoma (10 %) were the most common histologic subtypes. Surgery was the primary treatment for 86.7 % of patients. Recurrence occurred in 45 patients (50 %). The overall 5‑year survival was 39.3 % and disease free-survival was 45.9 %. Survival was significantly decreased in non-smokers (p = 0.022), T3–4 tumors (p = 0.007), positive lymph nodes (p <0.001), nonepithelial tumors (p = 0.036) and positive margins (p = 0.032). Survival was not affected by surgical approach between endoscopic, open and combined approaches (p = 0.088).Conclusion. Prognosis is poor, with high recurrences and low survival, but clearly histology, location and stage-dependent. Sound oncologic principles, with complete resections and negative margins, result in a better outcome.

Induction chemotherapy with docetaxel and doxorubicin followed by proton beam radiation therapy for platinum-resistant unresectable sinonasal undifferentiated carcinoma

Sinonasal undifferentiated carcinoma is a rare and aggressive tumor with an extremely poor prognosis. In the vast majority of cases, this tumor can not be resected due to its rapid local growth. Correct morphological diagnosis is impossible without a thorough differential diagnosis between sinonasal undifferentiated carcinoma and a number of lowgrade tumors of the nasal cavity and paranasal sinuses. Very few case reports and retrospective studies on sinonasal undifferentiated carcinoma have been published so far. No unified widely accepted guidelines on sinonasal undifferentiated carcinoma treatment are currently available due to the lack of statistically significant data from randomized clinical trials. The optimal treatment strategy should be based on an aggressive multimodal approach involving radical surgery, precision radiation therapy, and intensive chemotherapy. The benefits of systemic targeted therapy for patients with sinonasal undifferentiated carcinoma are still unclear. The best results can be achieved by employing tailored treatment approaches preferably in multidisciplinary cancer centers, where healthcare professionals experienced in managing patients with head and neck tumors can be involved. In this article, we report a case of complete radiological response after induction chemotherapy with docetaxel and doxorubicin and proton radiation therapy for the primary tumor area in a 53‑year-old female patient with non-resectable platinum-resistant sinonasal undifferentiated carcinoma.

Prevention of neutropenia as an important factor in successful chemotherapy for head and neck cancer

Despite the advances in supportive care for cancer patients, they often develop such a serious complication of chemotherapy as febrile neutropenia. This disorder is the main cause of reduced treatment efficacy because of the lower doses of cytostatics or even withdrawal of myelosuppressive therapy in some cases. The mortality rate from infectious complications of febrile neutropenia currently reaches 10 %.Presence of risk factors in combination with aggressive chemotherapy necessitates prevention of febrile neutropenia to reduce potential risks of complications. Synthetic granulocyte colony-stimulating factors can be used to address this issue.Among patients with head and neck tumors, the most vulnerable population includes individuals receiving TPF or DCF regimen or chemoradiotherapy. Such patients require preventive administration of granulocyte colony-stimulating factors. Patients with grade III–IV neutropenia require prevention of febrile neutropenia with pegylated forms of granulocyte colony-stimulating factors (such as empegfilgrastim). This will ensure optimal treatment outcomes.

Periodontal pathogens as a risk factor for oral squamous cell carcinoma

Introduction. The associative role of the bacterial factor in the development of both primary malignant tumors of the oral mucosa and relapses is being actively discussed today. In the article published earlier, we have already described the possible mechanisms of action of periodontopathogenic microflora and its connection with the development of squamous cell carcinoma of the oral mucosa through cell proliferation, intracellular accumulation of pathogen, DNA replication and affect the signaling pathways of MARK (mitogen-activated protein kinase).Objective – to analyze the impact of periodontal pathogens on the risk of oral squamous cell carcinoma and its recurrence, as well as to evaluate the role of polymerase chain reaction and bacterial culture in the diagnosis of squamous cell carcinoma.Materials and methods. This study included 35 patients with stage Т3–4 squamous cell carcinoma, whose tumor tissue samples were tested for periodontal pathogens using culture and PCR. We analyzed 5 paraffin-embedded and 30 frozen tissue blocks from newly diagnosed (n = 15) and re-treatment (n = 15) patients.Results. We found that PCR was more sensitive than culture for the detection of possible etiological agents and predictors of squamous cell carcinoma (including Porphyromonas gingivalis, Tannerella forsythia, and Treponema denticola) (р <0.001). For example, Tannerella forsythia and Treponema denticola were detected only using PCR, whereas all cultures were negative. However, conventional culture proved to be more effective than PCR for the detection of Prevotella intermedia. Between 3 % and 100 % of newly diagnosed patients tested positive for Treponema denticola and some other periodontal pathogens (PCR) developed relapses, whereas among re-treatment patients, this proportion was 66.6 %.Conclusion. The combination of the culture method and the polymerase chain reaction method in the study of periodontopathogenic microflora has shown high efficiency in identifying possible predictors of squamous cell carcinoma of the oral mucosa and in preventing the development of chronic infectious periodontal diseases.

Results of comprehensive treatment of locally advanced oral cancer using regional intra-arterial chemotherapy

Introduction. Head and neck cancers are a significant healthcare and social problem worldwide with an estimated 377,713 new cases and 177,757 deaths registered annually. The incidence of head and neck cancer in Russia is 4.12 per 100,000. Despite all the achievements of current oncology, approximately 50–60 % of patients with locally advanced head and neck cancer develop relapses within the first 2 years after treatment completion.The study objective is to analyze the effectiveness of regional intra-arterial polychemotherapy in the combined and comprehensive treatment of locally advanced oral cancer.Materials and methods. This prospective study included 29 patients with oral squamous cell carcinoma treated and followed-up at N.N. Blokhin National Cancer Research Center between 2017 and 2020. The majority of patients (82.8 %) had stage III–IV disease. We evaluated clinical response to neoadjuvant regional intra-arterial chemotherapy, pathomorphosis of the primary tumor after surgery, treatment toxicity, overall survival, and relapse-free survival.Results. The objective response rate assessed for the primary oral tumor after regional intra-arterial chemotherapy was 100 %; complete regression of the primary tumor was achieved in 82.8 % of patients. More than one-third of participants (35.5 %) had complete regression of metastases in regional lymph nodes. In 73.5 % of patients who had under gone neoadjuvant regional intra-arterial chemotherapy followed by surgery, we observed grade IV therapeutic pathomorphosis of the primary tumor. The 2-year relapse-free survival rate was 100 % in individuals with stage II–III disease and 92.9 ± 6.9 % in patients with stage IV disease.Conclusion. The administration of regional intra-arterial chemotherapy as an induction stage ensures high treatment efficacy and high rate of complete pathomorphological responses. This suggests the opportunity of conservative treatment for patients with locally advanced oral cancer, which is currently considered impossible for such patients. Moreover, this method demonstrated moderate systemic toxicity, which expands the indications for its use and increase treatment compliance.

The use of a “chimeric” autotissue complex from the subscapularis vascular basin for maxillary reconstruction after malignant tumors resection

The objective of this work – to evaluate the results of reconstructive interventions using free revascularized tissue complexes of the scapular region in patients after maxillary resection for malignant tumors. Materials and methods. Between 2014–2020 the post-resection maxillary defects were replaced with free blood-sup plied flaps of the scapular region in 19 patients. In Group 1 (n = 10), the defect was eliminated after total maxillectomy with preservation of the eyeball using a “chimeric” tissue complex, parts of which were positioned in several planes. In Group 2 (n = 9), total and subtotal defects of the hard palate and alveolar process were replaced using a free flap with the inclusion of the scapula angle, which was placed horizontally. The functional and aesthetic results of the reconstructions, the degree of morphological correspondence of the reconstructed structures, as well as the incidence of postoperative complications were assessed. Results. In Group 1 total necrosis of the flap was noted in 2 cases (20 %), in 1 (10 %) case – necrosis of the skin fragment). In Group 2 graft necrosis was observed in 1 (11 %) patient. Satisfactory and excellent aesthetic and functional results were achieved in 6 (60 %) patients in Group 1 and 8 (89 %) patients in Group 2. Conclusion. The scapular flap has a number of advantages for microsurgical reconstruction, including the presence of a long vascular pedicle with large vessels, inclusion of different tissues, possibility of harvesting a “chimeric” version (with significant mobility of parts), low rate of vascular lesions in the area, and most importantly, morphologically close location of bone tissue of the scapula to the maxilla, which ensures successful application of this flap for maxillary repair in patients with advanced cancer of the upper jaw.

New way of closure of upper jaw defects

Article describes new original way of one-stage closure of upper jaw defects by using cutaneous-adiposal flap. The method is patented (patent of the Russian Federation for invention No. 2489096). We propose to use cutaneous-adiposal flap, mobilized in nasolabial furrow area, with axial pattern blood supply from angular artery and vein, by rotating to defect through buccal tonnel. This method allows to reliably eliminate upper jaw defect, improve functional and aesthetic results of treatment with minimal injuries caused by operation.Objective is to introduce new way of closure of upper jaw defects.

A clinical case of treatment of a patient with laryngeal granular cell (Abrikossoff) tumor

The study objective – present a clinical case of radical surgical treatment of a laryngeal granular cell tumor with invasion of cricoid cartilage and thyroid gland and show the importance of pathomorphological diagnosis.Case report. A 47‑year-old woman of European origin complained of progressive hoarseness that lasted for 8 months. We analyzed her clinical, laboratory, radiological, and pathomorphological data. At the preoperative stage, the tumor was classified as well differentiated squamous cell carcinoma (stage cT4aN0M0). Endoscopic examination revealed the exophytic part of the tumor with an infiltrative base and tuberous surface, pinkish, with signs of dyskeratosis and tumor invasion to the cartilage. Pathomorphological examination of biopsy specimens demonstrated well differentiated keratinizing squamous cell carcinoma. The patient has undergone laryngectomy, thyroidectomy, and selective cervical lymph node dissection. Pathomorphological and immunohistochemical examinations of surgical material verified granular cell tumor of the larynx (Abrikosov’s tumor) that was excised within healthy tissues (R0). Conclusion. The presented clinical case of such a rare pathology as a granular cell tumor of the larynx shows the importance of pathomorphological diagnosis and the use of additional immunohistochemical research methods for making an accurate diagnosis and differential diagnosis of squamous cell carcinoma and Abrikossoff tumor.