Treatment and management of duodenal gangliocytic paraganglioma: A case report.

Gangliocytic paraganglioma (GP) is a rare neuroendocrine tumor primarily found in the duodenum, most commonly in the second and third sections of the duodenum. Diagnosis of GP is based on its distinctive histopathological characteristics, which include three types of tumor cells in varying proportions: i) Epithelioid, ii) spindle-like and iii) ganglion-shaped cells. The distribution of the three tumor cell components varies from case to case and a patient may be easily misdiagnosed if one of the components is predominant. Endoscopic submucosal dissection (ESD) or surgical resection is the ideal treatment for duodenal GP (DGP); however, biotherapy, nuclide therapy, chemotherapy, targeted therapy and immunotherapy can be selected individually for patients with postoperative recurrence, metastasis or not suitable for surgery. In the present study, a male patient with DGP experienced recurrence after ESD surgery, and so received octreotide (Novartis; 30 mg/28 days) for 12 consecutive cycles. The patient had no further symptoms of gastrointestinal bleeding and no new lesions or metastases were observed after 47 months of follow-up.

Head and Neck Paragangliomas: 16-year Single-center Experience and Mini Review on Diagnosis, Treatment, and Follow-up.

Objective: To investigate head and neck paraganglioma cases treated at a tertiary center from 2007 to 2023. The research includes a thorough examination of published studies that have focused on long-term outcomes. The additional goal is to contribute to the existing knowledge on head and neck paraganglioma, with a particular emphasis on refining diagnostic algorithms, treatment selection, and follow-up procedures. Methods: A total of 44 patients were retrospectively analyzed, and 39 were included. Demographic information, symptoms, radiological examination results, types, stages, and postoperative complications were recorded. A review was conducted to select articles that reported single-center experiences with large cohorts, long follow-ups, and different treatment modalities since 2010. Results: The mean age of the patients was 54 years, and the female/male ratio was 3.55:1. Among the 39 cases examined, 18 and 19 were identified as cervical paraganglioma and 19 as temporal bone paraganglioma. All patients initially underwent surgical resection. The mean follow-up duration was 5.42 years. Four residual cases and two recurrences were identified postoperatively, and a Gamma Knife was used as additional treatment. Subsequently, 17 articles were selected and summarized, and then a flowchart was prepared showing the possible options for diagnosis, treatment, and follow-up. Conclusions: Preoperative staging is essential for surgical planning and predicting potential intraoperative complications. Based on our findings and review of the articles, we have prepared a flowchart that includes all possibilities depending on the tumor stage to help in the diagnosis, treatment, and follow-up of head and neck paragangliomas.

Progress in surgical approaches and outcomes of patients with pheochromocytoma and paraganglioma.

Significant advances have been made in the past few decades in surgical management and outcomes of patients with pheochromocytoma and paraganglioma. Improvements in preoperative hypertensive control with the implementation of alpha- and beta-adrenergic blockade has resulted in better intra-operative blood pressure control and less incidence of hypertensive crises, which had been a large source of morbidity in the past. Emphasis on anesthesia and surgical team communication has also assisted in minimizing intraoperative hypertensive events at critical points of the operation. Shifting away from open resection, the now standard-of-care laparoscopic and minimally invasive adrenalectomy offers less pain, shorter hospitalizations, and quicker recoveries. Patient underlying germline mutations can guide the timing, approach, and extent of surgery. Postoperative outcomes have significantly improved with recent advancements in perioperative care in addition to regimented biochemical and radiographic surveillance. Here, we highlight the recent advancements in surgical approaches and outcomes for patients with pheochromocytoma and paraganglioma.

Atypical Presentation and Postoperative Management of Vagal Nerve Tumors.

OBJECTIVES: Tumors involving the vagus nerve are often clinically silent. We offer a case series with different clinical presentations and distinctive post-surgical sequelae that highlight some of the challenges associated with managing cervical vagal nerve tumors. METHODS: Single-institution, retrospective review of patients with tumors involving the vagus nerve. We describe clinical presentations and postoperative sequelae of five patients who underwent surgical management of vagal nerve pathology with atypical presentation or subsequent clinical course. RESULTS: Here, we present five patients treated at our institution for vagal tumors. In four of the five patients, the presenting symptoms resolved after surgery. Two patients presented with intractable neurogenic cough, and another two presented with autonomic symptoms, one with syncope/palpitations and the other with intractable sweating. The final patient presented with a rapidly enlarging vagal paraganglioma and developed intractable cough after resection. We present two patients with novel approach to vagal paragangliomas that underwent ligation of feeding blood supply without removing the tumor, resulting in resolution of an intractable cough in one patient and resolution of severe nighttime sweating in the other. CONCLUSION: Management of tumors associated with the cervical vagus nerve that present with symptoms or rapid growth poses a clinical dilemma. Consideration of the tumor origin with either enucleation of schwannomas or ligation of feeding vessels may preserve function while addressing the presenting symptoms.

18F-FDG PET/CT and 68Ga-DOTATATE PET/CT Findings of Polycystic Kidney-derived Paraganglioma.

Paragangliomas (PGLs) are neuroendocrine tumors originating from the neural crest. They usually arise from the adrenal medulla and sympathetic or parasympathetic ganglions. Approximately 10% of PGLs are located in the extra-adrenal gland. Renal PGL is a rare condition. In this case report, we present the 18F-fluorodeoxyglucose positron emission tomography/computed tomography (PET/CT) and 68Ga-DOTATATE PET/CT findings of polycystic kidney-derived PGL.

Alterations and clinical value of peripheral lymphocyte subsets and inflammatory factors in paragangliomas.

OBJECTIVES: Given the pivotal role of immune and inflammatory responses in tumor patients, the present study aimed to explore alterations and the clinical value of peripheral lymphocyte subsets and inflammatory factors in pheochromocytomas/paragangliomas (PPGLs). METHODS: The clinical data of 327 patients, including 102 patients with metastatic PPGLs, were retrospectively analysed. Peripheral lymphocyte subsets were determined by flow cytometry. Relationships between immune and inflammatory parameters and clinicopathological characteristics were evaluated by intergroup comparisons and correlation analyses. Univariate and multivariate logistic regression analyses were employed to identify metastatic indicators. The corresponding nomogram was constructed and evaluated for discrimination and calibration. RESULTS: The median age at diagnosis was 45.0 years, and duration of follow-up was 3.0 years. Compared with those in younger patients (<45.0 years), most lymphocyte subsets were significantly reduced in older patients (≥45 years) (P<0.05). The count of lymphocytes, CD3+T cells and CD4+T cells were negatively correlated with 24h-urinary epinephrine and plasma metanephrine levels (R=-0.2∼-0.1, P<0.05). In addition, patients with lymph node (n=37) or bone metastases (n=41) had a lower percentage of CD4+T cells (P<0.05). Multivariate analysis revealed that CD3+T cell≥1446.50/µl, CD4+T cell%<39.95%, CD8+T cell%<24.95%, CD4+/CD8+T cell ratio<2.88, B cell%≥8.65%, TNF-alpha<12.45pg/ml, IL8<30.50pg/ml and PLT≥269.50*109/L were significant indicators of metastatic PPGLs. The area under the curve (AUC) of the nomogram was 0.800 (95% CI: 0.736-0.865). CONCLUSIONS: Immunesenescence, characterized by immune dysfunction with aging, was observed in PPGLs. Higher epinephrine and metanephrine levels might impair host's immune response. Monitoring changes in peripheral lymphocyte subsets and serum cytokines could indicate patients' conditions, especially the occurrence of metastasis.

Radioligand therapy in sympathetic-adrenal-medullary axis tumors: state of art and perspectives.

The approval in 2017 by the European Medicines Agency (EMA) and in 2018 by the US Food and Drug Administration (FDA) of radioligand therapy (RLT) led to its wide application in therapeutic management of neuroendocrine neoplasms (NENs). However, the indications are currently limited to certain specific histotypes belonging to the broader NEN's family, mainly advanced well-differentiated gastro-entero-pancreatic NENs. As a consequence, several other tumors of the NEN spectrum that can potentially benefit, due to their biological characteristics, from RLT are still ineligible and can be considered "RLT-orphans". Among those, the subgroup of NENs originating from the sympathetic-adrenal-medullary (SAM) axis can be listed. This paper discusses the state of art and perspectives of the theragnostic applications in pheochromocytomas and paragangliomas, considering both the traditional theragnostic model - with radiolabelled metaiodobenzylguanidine (MIBG) - and the innovative one with radiolabeled somatostatin analogs (SSAs), that will hopefully become available for these patients in the near future.

Laryngeal Paraganglioma with Neck Metastasis.

Paragangliomas are neoplasms of neural crest cells. They represent less than 1% of head and neck tumors. Laryngeal paragangliomas constitute a smaller fraction of head and neck paragangliomas. Malignant laryngeal paragangliomas with metastasis are extremely rare tumors. Definitive treatment includes complete surgical excision with maximum preservation of laryngeal function. Here, we report a rare case of a 55 year old woman who was diagnosed with recurrent laryngeal paraganglioma along with neck metastasis. A complete excision of mass with a lateral pharyngotomy approach along with neck dissection followed by chemotherapy was done.

Non-functional muscle-invasive bladder paraganglioma-a case report.

Background: Extra-adrenal pheochromocytoma (paraganglioma) of the urinary bladder is a rare tumor, accounting for 0.05% of bladder tumors and less than 1% of all paragangliomas. In the genitourinary tract, paragangliomas are most commonly found in the bladder. These tumors have aggressive malignant potential, so complete surgical resection for localized disease is important. Paragangliomas may be non-functional or functional with catecholamine secretions. Although these tumors are rare and difficult to distinguish from urothelial carcinoma (UC), intraoperative manipulation of these tumors may lead to a catecholamine surge and intraoperative complications. Preoperative or early intraoperative recognition of this tumor would facilitate appropriate alpha blockade to minimize morbidity. Case Description: Herein we report a rare non-functional paraganglioma arising from the bladder of a 46-year-old male. This case is notable for the location of the mass, requiring a 70-degree cystoscopic lens for complete visualization near the bladder neck, and for the identification of a golden-yellow sessile mass during the resection. Upon visualization of this mass, the operation should be paused for close hemodynamic monitoring and assess for signs of hypertensive crisis prior to continuing without alpha blockade. Conclusions: Suspected localized bladder paraganglioma cases should be optimized hemodynamically and managed surgically. Visualization of a sessile bladder mass on gross examination with golden-yellow tumor during the resection should prompt suspicion for a paraganglioma. Biochemical evaluation with serum or urine catecholamines, metanephrines, and normetanephrines should be performed to assess for tumor functionality.

Current views on the role of HIF-2α in the pathogenesis and syndromic presentation of pheochromocytoma and paraganglioma.

Pathogenic variants (PVs) in EPAS1, which encodes hypoxia-inducible factor-2α (HIF-2α), could be the underlying genetic cause of about 3%-6% of pheochromocytoma and paragangliomas (PPGLs). EPAS1-related PPGLs may occur as isolated tumors or as part of Pacak-Zhuang Syndrome (PZS) with two or more of a triad of PPGL, polycythemia, and somatostatinoma. HIF-2α plays a critical role in the regulation of the cellular hypoxia pathway. When a gain-of-function PV is acquired, HIF-2α evades steady-state hydroxylation by the prolyl hydroxylase type 2 (PHD2), which accelerates von Hippel-Lindau (VHL)-mediated proteasomal degradation. In this situation, HIF-2α is stabilized and can translocate to the nucleus, inducing the expression of several genes involved in tumorigenesis. This leads to the development of PPGL and other manifestations of PZS. EPAS1-related PPGLs usually occur in the second or third decade of life, more frequently in females, and are usually multiple, adrenal and extra-adrenal, and norepinephrine-secreting. In addition, these tumors carry an increased metastatic potential and have been reported with metastatic disease in up to 30% of cases. While polycythemia is fairly common in PZS, somatostatinomas are rare. It has been suggested that the character of the acquired PV in EPAS1, which affects its binding to PHD2, correlates with certain phenotypes in PZS. PVs in EPAS1 that have been found in related sporadic PPGLs have also been associated with hypoxic conditions including cyanotic congenital heart disease, hemoglobinopathies and high altitude. Understanding the hypoxia pathway and its role in the pathogenesis of PPGL may open a new avenue for developing effective therapies for these tumors. Indeed, one of these therapies is Belzutifan, a HIF-2α inhibitor that is being tested in the treatment of metastatic PPGLs.

Update on clinical characteristics in the evaluation of phaeochromocytoma and paraganglioma.

Pheochromocytomas and sympathetic paragangliomas (PPGL) are rare neuroendocrine tumors originating from chromaffin tissue of the adrenal medulla and extra-adrenal sympathetic paraganglia. Historically, many of these tumors were diagnosed postmortem, earning pheochromocytomas the moniker "great mimic" due to their diverse clinical manifestations that can resemble various other conditions. Over time, the clinical presentation of PPGL has evolved, with a shift from symptomatic or postmortem diagnoses to more frequent incidental discoveries or diagnoses through screening, with postmortem identification now being rare. The development of a clinical scoring system has improved the identification of patients at increased risk for PPGL. Notably, the proportion of PPGL patients with normal blood pressure ranges from 15 % to 40 %, varying based on the clinical context. Despite the tumor's reputation, PPGL is an exceedingly rare cause of resistant hypertension. Management of a pheochromocytoma crisis has advanced, with several classes of drugs available for treatment. However, PPGL during pregnancy remains a significant concern, associated with substantial maternal and fetal mortality rates. Additionally, PPGL can present as rare disorders, including catecholamine-induced cardiomyopathy, Cushing syndrome, and urinary bladder PGL. Given these varied presentations, heightened awareness and prompt recognition of PPGL are crucial for timely diagnosis and treatment, ultimately improving patient outcomes. In this article, we offer an in-depth analysis of the diverse clinical presentations of PPGL, highlighting their complexity and the associated diagnostic and treatment strategies.

Management of rTTS associated with PGL using VA-ECMO and surgical resection: a case report.

Background: Paraganglioma (PGL) is a neuroendocrine tumor located outside the adrenal gland that can secrete catecholamines. Clinical manifestations include headaches, hypertension, and, rarely, cardiomyopathy. Among these, reverse Takotsubo cardiomyopathy (rTTS) is a rare Takotsubo cardiomyopathy (TTS) associated with a surge in catecholamines. Case introduction: This article reports a case of a hypertensive patient admitted for recurrent dizziness and chest tightness. During treatment, the patient suddenly experienced chest tightness and shortness of breath, followed by refractory cardiogenic shock, and was eventually diagnosed with rTTS. The patient gradually recovered and was successfully discharged after receiving treatments, including tracheal intubation with mechanical ventilation, extracorporeal membrane oxygenation (ECMO), and surgery. Conclusion: The diagnosis of rTTS is significantly aided by the presence of free plasma metanephrines and specific changes observed in cardiac ultrasound. In the treatment of severe rTTS, ECMO can serve as a crucial life support technology. Under VA-ECMO support, early resection of the PGL after accelerated preoperative preparation may be a feasible approach.

Current Management of Head and Neck Paragangliomas: A Multicenter Series With Long-Term Follow-Up.

OBJECTIVE, STUDY DESIGN, SETTING, METHODS: To improve our knowledge about head and neck paragangliomas (HN-PGL), the clinical characteristics of all the patients with HN-PGL included in the Spanish Registry were analyzed, as well as the treatment modalities and their outcomes. RESULTS: A total of 202 patients, 67.8% women, aged 53.1 ± 17.4, with 264 HN-PGL, from 16 Spanish hospitals, were included. Tumors were located in the carotid body in 61%, jugular bulb in 20.5%, tympanic area in 10.2%, and along the vagal nerve in 6.4%. Multiple tumors developed in 20.8% and metastatic disease in 4%. A genetic study was done in 64.4% and showed a pathogenic variant in SDHx in 50%. These patients were younger, with no sex predominance, and had more multiple and metastatic tumors. Tumors were treated by surgery in 134 patients, radiotherapy in 33, and other treatments in 20, and were observed without active treatment in 41 patients, who were older, more often asymptomatic, and had smaller tumors. Tumors treated with radiotherapy were larger and more often in locations other than the carotid body. After a median follow-up of 80 months (interquartile range: 41-136), 6.9% had died; among the survivors, 48.4% were disease-free, 42.1% stable, and 9.4% had progressed. The rate of sequelae was similar among patients submitted to surgery, radiotherapy, or observation. We could not identify any prognostic factor for progression. CONCLUSION: Most HN-PGL are localized slow-growing tumors. Long-term survival is high, even in case of metastasis. Although surgery is the most common treatment, radiotherapy and active surveillance are safe approaches.

Personalized oncology in pheochromocytomas and paragangliomas: integrating genetic analysis with machine learning.

Pheochromocytomas and paragangliomas (PCCs/PGLs) are uncommon neuroendocrine tumors with a significant genetic tendency. Approximately 35-40% of these tumors are associated with genetic factors. The present study performed a thorough analysis using publicly accessible genetic and clinical data from the Cancer Genome Atlas (TCGA) to examine the involvement of six genes, namely GBP1, KIF13B, GPT, CSDE1, CEP164, and CLCA1, in the development of PCCs/PGLs. By employing multi-omics data, this study investigates the relationship between mutational patterns and the prognosis of tumors, focusing on the possibility of tailoring treatment methods to individual patients. The study utilizes Mutect2 to detect somatic mutations with high confidence in whole-exome sequencing data from PCCG samples. The study uncovers mild effects on protein function caused by particular mutations, including GBP1 (p.Cys12Tyr), KIF13B (p.Arg847Gly), and GPT (p.Gln50Arg). A random forest classifier uses mutational profiles to predict potential drug recommendations, proposing a focused therapy strategy. This study thoroughly analyzes the genetic mutations found in PCCs/PGLs, highlighting the significance of precision medicine in developing specific treatments for these uncommon types of cancer. This study aims to improve the understanding of the development of tumors and identify personalized treatment approaches by combining genetic data with machine learning analyses.

Large Neuroendocrine Neoplasms of the Duodenum: Description of Two Rare Subtypes and Technical Details on Surgical Treatment.

BACKGROUND: Duodenal neuroendocrine tumors (NETs) are uncommon, accounting for less than 4% of all gastrointestinal neoplasms. Prognosis is related to tumoral staging and grading, as well as to the specific subtype. In this article, we retrospectively describe the clinical presentation and surgical treatment of two rare large duodenal NETs: a high-grade G3 NET and a Gangliocytic Paraganglioma (GP). METHODS: Both patients presented with moderate-to-high-degree abdominal pain, without jaundice. Main vessel involvement and metastatic spread were excluded with imaging, while preoperative bioptic diagnosis was obtained via percutaneous needle citology and endoscopic ultrasound. RESULTS: The presence of a sessile large lesion contraindicated any conservative approach in favor of a pancreaticoduodenectomy (PD). The detection of soft pancreatic tissue and a narrowed main pancreatic duct led us to perform a pancreaticogastrostomy to restore proper pancreatic drainage and to minimize the risk of postoperative leakage. CONCLUSION: PD may be a favorable choice in these cases; this procedure is challenging, but it results in a safer and more favorable clinical outcome for our patients. Pancreaticogastrostomy may guarantee lower rates of postoperative leak and appears to be preferred in this subset of patients.

Primary Paraganglioma Arising From the Maxillary Bone.

We present a unique case of primary intraosseous paraganglioma (PGL) originating from maxillary bone. PGL is a neurosecretory neoplasm that arises from cells believed to originate from the neural crest. A 30-year-old woman presented with right facial pain and swelling, along with palpitations. Computed tomography (CT) imaging revealed a 3.3 × 3.1 × 2.3 cm mass in the anterior maxilla, and biochemical results showed elevated plasma dopamine, 3-methoxytyramine, and chromogranin A levels. Biopsy confirmed a PGL, with positive expression of synaptophysin, chromogranin A, and GATA-3. Whole-body positron emission tomography/computed tomography (PET/CT) scans showed avidity on 18F-fluorodopa (18F-FDOPA), 68Ga-DOTA(0)-Tyr(3)-octreotate ​​ (68Ga-DOTATATE), and 18F-fluorodeoxyglucose (18F-FDG). No other lesions (primary or metastatic) were found. Proton beam therapy was chosen over surgery due to potential complications and patient's preference. Following radiotherapy, she experienced symptom relief, with dopamine levels decreasing and chromogranin A normalizing, with the lesion remaining stable on 11-month follow-up imaging. This case highlights the rarity of primary bone PGLs and underscores the importance of comprehensive diagnostic approaches combining physical examinations, biochemical testing, functional imaging, and histopathological analysis properly guiding personalized treatment strategies. Additionally, proton beam therapy emerges as a highly suitable treatment option for head and neck paragangliomas (HNPGLs), offering effective tumor control with minimal complications.

Mouse developmental defects, but not paraganglioma tumorigenesis, upon conditional Complex II loss in early Sox10+ cells.

In humans, loss of heterozygosity for defective alleles of any of the four subunits of mitochondrial tricarboxylic acid cycle enzyme succinate dehydrogenase (SDH, also Complex II of the electron transport chain) can lead to paraganglioma tumors in neuroendocrine cells. With the goal of developing mouse models of this rare disorder, we have developed various SDH conditional loss strategies. Based on recent lineage tracing studies, we hypothesized that conditional SDHC loss in early embryogenesis during migration of primordial neural crest cells that form the susceptible chromaffin cells of the adrenal medulla might induce paraganglioma. We triggered low levels of detectable SDHC loss in Sox10+ cells at E11.5 of mouse development. We report that, rather than developing adrenal medulla paraganglioma (pheochromocytoma), offspring survived with evidence of neural crest cell dysfunction. Phenotypes included mild lower extremity gait anomalies suggestive of neural tube closure defects and patches of unpigmented fur consistent with neural crest-derived melanocyte dysfunction. These defects were not observed in mice lacking Sdhc knockout. Our results add to existing data suggesting that, unlike humans, even early embryonic (Sox10-driven) SDHx loss is inadequate to trigger paraganglioma in mice of the genetic backgrounds that have been investigated. Instead, low levels of tricarboxylic acid cycle-deficient neural crest cells cause mild developmental defects in hind limb and melanocyte function. This new model may be of interest for studies of metabolism during early neural crest cell development.

Multiple Primary Paragangliomas in a Pediatric Patient With von Hippel Lindau: A Diagnostic Dilemma.

Pheochromocytoma and paragangliomas (PPGLs) are rare chromaffin cell tumors arising from neural crest tissue. The majority of these tumors are nonmetastatic, with complete cure achieved through surgical resection. PPGLs have been associated with several hereditary cancer syndromes, including von Hippel-Lindau (VHL). We present the case of a 10-year-old patient with VHL and a history of 2 asynchronous pheochromocytomas requiring bilateral adrenalectomies who presented with a new 1.2 cm × 1.3 cm × 1.5 cm nodular structure between the superior pole of the right kidney and the intrahepatic inferior vena cava. The patient was noted to have hypertension but was otherwise asymptomatic. Positron emission tomography-DOTA-(Tyr)3-octreotate revealed a metabolically active retrocrural lymph node. Based on these imaging findings and laboratory studies showing elevated plasma normetanephrine, clinical suspicion was highest for metastatic pheochromocytoma. The patient underwent surgical resection of multiple abdominal tumors. Pathology ultimately favored a diagnosis of multiple primary paragangliomas rather than metastatic disease. With this shift in diagnosis, the patient was managed with surgery alone. One year later, he has no signs of disease recurrence. Long-term surveillance imaging and screening with fractionated plasma metanephrines is indicated to monitor for new tumors in the setting of VHL and 3 prior endocrine tumors.

Hereditary Pheochromocytoma as a Main Manifestation of von Hippel Lindau Disease (vHL) in Childhood - A Long-term Follow-up of 5 Patients with vHL from One Family.

Von Hippel-Lindau disease (vHL) is a hereditary, autosomal dominant syndrome manifested by a predisposition to the occurrence of benign and malignant neoplasms. The spectrum of vHL-related neoplasms includes: pheochromocytoma (PHEO), central nervous system and retinal hemangioblastomas, renal clear cell carcinoma, epididymal cystadenomas, pancreatic neuroendocrine tumors as well as visceral (renal and pancreatic) cysts. We report the family (5 patients) with genetically confirmed vHL in which every member had PHEO diagnosed during pediatric care. The presented family had a missense variant in the VHL gene (ex1 g.A451G gene, p. S80G) which is connected with an increased risk of PHEO. Performing screening laboratory and imaging tests in patients with genetically confirmed vHL disease can help to avoid the occurrence of disease symptoms and to perform an elective surgery in safe conditions. Due to the risk of coexisting pathologies and the complexity of the disease, patients with vHL require long-term care.

Tactics of surgical treatment of recurrent glomus tumors of the skull base: A clinical case.

AIM: The purpose of the study is to build effective tactics of surgical treatment of pathology, and to increase the effectiveness of surgical treatment of patients with this diagnosis by optimising preoperative preparation, and the correct sequence of actions that will help prevent relapse. METHODS: The leading approach to the study is the description of a clinical case, which will help to carefully consider this issue from all angles, analyse the methodology of patient examination, and build highly effective tactics of surgical intervention to eliminate tumour-like masses, minimising the risks of recurrence. The second clinical case was also considered, and a comparative analysis was carried out on certain parameters. RESULTS: The paper presents a clinical case with a practical aspect of surgical treatment of the pathology. The issue of detailed symptoms and manifestations of the disease is disclosed, and the course of surgical interventions is described in stages, in which no nerve is damaged, no complications are caused, and the desired result is achieved. The peculiarity of this case is the recurrence of the disease, and the ineffectiveness of previous treatment methods, including embolisation of the neoplasm and antromastoidotomy. CONCLUSIONS: The results obtained in the course of this study and the formulated conclusions are of great importance for surgeons who are faced with patients with the stated diagnosis.