Molecular imaging and radionuclide therapy of pheochromocytoma and paraganglioma in the era of genomic characterization of disease subgroups.

In recent years, advancement in genetics has profoundly helped to gain a more comprehensive molecular, pathogenic, and prognostic picture of pheochromocytomas and paragangliomas (PPGLs). Newly discovered molecular targets, particularly those that target cell membranes or signaling pathways have helped move nuclear medicine in the forefront of PPGL precision medicine. This is mainly based on the introduction and increasing experience of various PET radiopharmaceuticals across PPGL genotypes quickly followed by implementation of novel radiotherapies and revised imaging algorithms. Particularly, 68Ga-labeled-SSAs have shown excellent results in the diagnosis and staging of PPGLs and in selecting patients for PRRT as a potential alternative to 123/131I-MIBG theranostics. PRRT using 90Y/177Lu-DOTA-SSAs has shown promise for treatment of PPGLs with improvement of clinical symptoms and/or disease control. However, more well-designed prospective studies are required to confirm these findings, in order to fully exploit PRRT's antitumoral properties to obtain the final FDA approval. Such an approval has recently been obtained for high-specific-activity 131I-MIBG for inoperable/metastatic PPGL. The increasing experience and encouraging preliminary results of these radiotherapeutic approaches in PPGLs now raises an important question of how to further integrate them into PPGL management (e.g. monotherapy or in combination with other systemic therapies), carefully taking into account the PPGLs locations, genotypes, and growth rate. Thus, targeted radionuclide therapy (TRT) should preferably be performed at specialized centers with an experienced interdisciplinary team. Future perspectives include the introduction of dosimetry and biomarkers for therapeutic responses for more individualized treatment plans, α-emitting isotopes, and the combination of TRT with other systemic therapies.

Update in the classification and the role of intra-arterial stenting in the management of carotid body paragangliomas.

BACKGROUND: To review the Shamblin classification of carotid body paragangliomas (CBPs) and the role of intra-arterial stenting in their surgical management. METHODS: Retrospective case series of 20 patients with 28 CBPs that were surgically resected at our center. Intra-arterial stenting was performed in Shamblin II and II classes. RESULTS: The mean follow-up was 47.8 months. Five (17.9%) tumors were Shamblin class I, 15 (53.6%) were class II, and 8 (28.6%) were class III. Thirteen (68.4%) CBPs were associated with other paragangliomas. The internal carotid artery (ICA) was stented preoperatively in eight (28.6%) cases and occluded in four (14.3%) cases. The tumor extended to the jugular foramen in six cases (21.4%). Intraoperatively, there was an ICA injury in one case of Shamblin II CBP in the present era. CONCLUSIONS: The proposed classification enables the clinician to plan the management of the ICA and the right approach. Stenting of the ICA gives a chance for complete tumor removal with arterial preservation.

Clinical significance of functional and anatomical classifications in paraganglioma of the urinary bladder.

OBJECTIVES: Paragangliomas of the urinary bladder (PUBs) are challenging catecholamine-producing neuroendocrine tumors. We aimed to facilitate their diagnosis and treatment by functional and anatomical classifications. MATERIALS AND METHODS: Between April 2007 and September 2017, 31 cases from 2 centers were retrieved, in which the patients were pathologically diagnosed with PUB. Besides classifying them into functional and nonfunctional PUBs, functional PUBs were further subclassified into typical functional PUB (with typical symptoms and elevated catecholamines/metabolites levels) and atypical functional PUB. Anatomically, they were classified into submucosal, intramural, and subserosal PUBs. RESULTS: Functionally, these cases comprised 17 (54.8%) functional and 14 (45.2%) nonfunctional PUBs. Functional PUBs had significantly larger diameters than nonfunctional PUBs (P < 0.01). Of the 17 functional PUB cases, 8 were further subclassified into typical functional PUB, of which 4 were diagnosed without cystoscopy. Anatomically, these cases comprised 14 (45.2%) submucosal, 13 (41.9%) intramural, and 4 (12.9%) subserosal PUBs. Intramural and subserosal PUBs had significantly larger diameters and were more likely to be functional than submucosal PUBs (P < 0.05). Cystoscopy failed to detect the tumor in all patients with subserosal PUB. Besides all patients with intramural or subserosal PUB, 1 patient with submucosal PUB underwent partial cystectomy. The remaining 13 patients with submucosal PUB underwent transurethral resection of bladder tumor, 5 of whom required extra surgical intervention. CONCLUSIONS: By functional classification, omitting cystoscopy is feasible in the diagnosis of typical functional PUBs. By anatomical classification, intramural, and subserosal PUBs tend to be large and functional. Moreover, negative cystoscopic findings are not sufficient to exclude subserosal PUBs. Finally, not all submucosal PUBs are amenable to transurethral resection of bladder tumor.

Nonmosaic somatic HIF2A mutations associated with late onset polycythemia-paraganglioma syndrome: Newly recognized subclass of polycythemia-paraganglioma syndrome.

BACKGROUND: Somatic mutations in hypoxia-inducible factor 2α (HIF2A) are associated with polycythemia-paraganglioma syndrome. Specifically, the classic presentation of female patients with recurrent paragangliomas (PGLs), polycythemia (at birth or in early childhood), and duodenal somatostatinomas has been described. Studies have demonstrated that somatic HIF2A mutations occur as postzygotic events and some to be associated with somatic mosaicism affecting hematopoietic and other tissue precursors. This phenomenon could explain the development of early onset of polycythemia in the absence of erythropoietin-secreting tumors. METHODS: Correlation analysis was performed between mosaicism of HIF2A mutant patients and clinical presentations. RESULTS: Somatic HIF2A mutations (p.A530V, p.P531S, and p.D539N) were identified in DNA extracted from PGLs of 3 patients. No somatic mosaicism was detected through deep sequencing of blood genomic DNA. Compared with classic syndrome, both polycythemia and PGL in all 3 patients developed at an advanced age with polycythemia at age 30, 30, and 17 years and PGLs at age 34, 30, and 55 years, respectively. Somatostatinomas were not detected, and 2 patients had ophthalmic findings. The biochemical phenotype in all 3 patients was noradrenergic with 18 F-fluorodopa PET/CT as the most sensitive imaging modality. All patients demonstrated multiplicity, and none developed metastatic disease. CONCLUSION: These findings suggest that newer techniques need to be developed to detect somatic mosaicism in patients with this syndrome. Absence of HIF2A mosaicism in patients with somatic HIF2A mutations supports association with late onset of the disease, milder clinical phenotype, and an improved prognosis compared with patients who have HIF2A mosaicism.

Two Extraordinary Sellar Neuronal Tumors: Literature Review and Comparison of Clinicopathologic Features.

Objectives: The list of tumors involving the pituitary gland has been expanded to include a variety of neuronal and paraneuronal tumors in the 2017 World Health Organization tumor classification of endocrine organs. All the entities included in this category are distinctly rare, with limited case reports in the literature. Methods: We illustrate two extraordinary sellar tumors with neuronal differentiation: a sellar paraganglioma and a sellar neurocytoma, with thorough literature review and comparison of the clinicopathologic features of these entities. Results: Both entities are exceptionally rare and tend to be misdiagnosed as pituitary adenoma preoperatively. Both entities demonstrate frequent clinical recurrence compared with pituitary adenoma, as well as the rare occurrence of metastatic disease. Conclusions: In evaluating a sellar tumor with an uncommon morphology and neuroendocrine differentiation, an increased awareness of the unusual entities that may involve the sellar region and a judicious panel of immunohistochemical studies should improve the diagnosis.

New Insights into the Nuclear Imaging Phenotypes of Cluster 1 Pheochromocytoma and Paraganglioma.

Pheochromocytomas and paragangliomas (PPGLs) belong to the family of neural crest cell-derived neoplasms. In up to 70% of cases they are associated with germline and somatic mutations in 15 well-characterized PPGL driver or fusion genes. PPGLs can be grouped into three main clusters, where cluster 1 includes PPGLs characterized by a pseudohypoxic signature. Although cluster 1 tumors share several common features, they exhibit unique behaviors. We present here unique insights into the imaging phenotypes of cluster 1 PPGLs based on glucose uptake, catecholamine metabolism, and somatostatin receptor expression. Recent data suggest that succinate is a major player in the imaging phenotype of succinate dehydrogenase-deficient PPGLs. This review emphasizes the emerging stromal cell-succinate interaction and highlights new perspectives in PPGL theranostics.

Endoscopic Management of Middle Ear and Temporal Bone Lesions.

Tantamount to the management of temporal bone neoplasms is the ability to visualize the pathology and its relationship with the numerous critical structures housed therein. Transcanal endoscopic ear surgery provides the surgeon with an unparalleled view of the entire middle ear. This article presents the latest information on the usefulness of transcanal endoscopic ear surgery in the management of middle ear and temporal bone neoplasms.

Pheo-Type: A Diagnostic Gene-expression Assay for the Classification of Pheochromocytoma and Paraganglioma.

CONTEXT: Pheochromocytomas and paragangliomas (PPGLs) are heritable neoplasms that can be classified into gene-expression subtypes corresponding to their underlying specific genetic drivers. OBJECTIVE: This study aimed to develop a diagnostic and research tool (Pheo-type) capable of classifying PPGL tumors into gene-expression subtypes that could be used to guide and interpret genetic testing, determine surveillance programs, and aid in elucidation of PPGL biology. DESIGN: A compendium of published microarray data representing 205 PPGL tumors was used for the selection of subtype-specific genes that were then translated to the Nanostring gene-expression platform. A support vector machine was trained on the microarray dataset and then tested on an independent Nanostring dataset representing 38 familial and sporadic cases of PPGL of known genotype (RET, NF1, TMEM127, MAX, HRAS, VHL, and SDHx). Different classifier models involving between three and six subtypes were compared for their discrimination potential. RESULTS: A gene set of 46 genes and six endogenous controls was selected representing six known PPGL subtypes; RTK1-3 (RET, NF1, TMEM127, and HRAS), MAX-like, VHL, and SDHx. Of 38 test cases, 34 (90%) were correctly predicted to six subtypes based on the known genotype to gene-expression subtype association. Removal of the RTK2 subtype from training, characterized by an admixture of tumor and normal adrenal cortex, improved the classification accuracy (35/38). Consolidation of RTK and pseudohypoxic PPGL subtypes to four- and then three-class architectures improved the classification accuracy for clinical application. CONCLUSIONS: The Pheo-type gene-expression assay is a reliable method for predicting PPGL genotype using routine diagnostic tumor samples.

The Differential Diagnosis of Sinonasal/Nasopharyngeal Neuroendocrine/Neuroectodermally Derived Tumors.

CONTEXT: The differential diagnosis of neuroendocrine neoplasms arising in the sinonasal tract is broad and includes lesions of epithelial, mesenchymal, and neuroectodermal origin. OBJECTIVE: To review the differential diagnosis of sinonasal neuroendocrine and neuroectodermally derived tumors. DATA SOURCES: The current literature was reviewed to provide updated information regarding the differential diagnosis and means for diagnosing neuroendocrine tumors including sinonasal neuroendocrine carcinoma, olfactory neuroblastoma, malignant melanoma, paraganglioma, pituitary adenoma, and Ewing family of tumors. CONCLUSIONS: The differential diagnosis of neoplasms with neuroendocrine differentiation in the sinonasal tract is broad, and diagnosis often includes not only histologic review but also immunohistochemical or molecular analysis.

[Gastrointestinal stromal tumor (GIST): advances in 2013]. / Gastrointestinální stromální tumor (GIST): pokroky do roku 2013.

Gastrointestinal stromal tumors (GIST) are currently regarded as a heterogenous group of tumors sharing common histological appearance, KIT immunopositivity and supposed origin from tissue progenitor cells capable of differentiation into the phenotype of Cajal interstitial cells. GISTs can be divided according to immunoexpression of the beta subunit of mitochondrial enzyme succinate dehydrogenase (SDHB) to SDHB-positive (encompassing KIT, PDGFRA and NF1 mutated GISTs), and SDHB-deficient GISTs (including Carney-Stratakis syndrome, Carney triad, sporadic pediatric GISTs, and a small subset of sporadic adult GISTs). The individual molecular subtypes differ in biological behavior and in their response to systemic targeted therapy, which is indicated in metastatic GISTs or in tumors with high risk of recurrence. Although several risk-stratification classifications have been developed, strictly defined criteria to identify patients at risk are still lacking. Pharmacogenomics have been successful in designing drugs to overcome not only the primary resistance of GISTs to the action of imatinib (e.g. GISTs with a substitution of Asp842Val in exon 18 PDGFRA or SDHB-deficient GISTs), but also the secondary resistance caused by secondary mutation of a gene encoding either the receptor tyrosine kinase or other molecules involved in the respective signalling cascade. Future directions concentrate on rational molecular targeting for systemic therapy based on complex genetic investigation of the tumor. Peripheral blood is planned to be used as a source of information for genetic events responsible for the secondary resistance of metastatic tumors.

The management of head-and-neck paragangliomas.

Paragangliomas (PGLs) are tumours originating from neural crest-derived cells situated in the region of the autonomic nervous system ganglia. Head-and-neck PGLs (HNPGLs) originate from the sympathetic and parasympathetic paraganglia, most frequently from the carotid bodies and jugular, tympanic and vagal paraganglia, and are usually non-catecholamine secreting. Familial PGLs are considered to be rare, but recently genetic syndromes including multiple PGLs and/or phaeochromocytomas have been more thoroughly characterised. Nowadays, genetic screening for the genes frequently implicated in both familial and sporadic cases is routinely being recommended. HNPGLs are mostly benign, generally slow-growing tumours. Continuous growth leads to the involvement of adjacent neurovascular structures with increased morbidity rates and treatment-related complications. Optimal management mostly depends on tumour location, local involvement of neurovascular structures, estimated malignancy risk, patient age and general health. Surgery is the only treatment option offering the chance of cure but with significant morbidity rates, so a more conservative approach is usually considered, especially in the more difficult cases. Radiotherapy (fractionated or stereotactic radiosurgery) leads to tumour growth arrest and symptomatic improvement in the short term in many cases, but the long-term consequences are unclear. Early detection is essential in order to increase the chance of cure with a lower morbidity rate. The constant improvement in diagnostic imaging, surgical and radiation techniques has led to a safer management of these tumours, but there are still many therapeutic challenges, and no treatment algorithm has been agreed upon until now. The management of HNPGLs requires a multidisciplinary effort addressing the genetic, surgical, radiotherapeutic, oncological, neurological and endocrinological implications. Further progress in the understanding of their pathogenesis will lead to more effective screening and earlier diagnosis, both critical to successful treatment.

Pheochromocytoma and paraganglioma: understanding the complexities of the genetic background.

Pheochromocytomas and paragangliomas (PCC/PGL) are tumors derived from the adrenal medulla or extra-adrenal ganglia, respectively. They are rare and often benign tumors that are associated with high morbidity and mortality due to mass effect and high circulating catecholamines. Although most PCCs and PGLs are thought to be sporadic, over one third are associated with 10 known susceptibility genes. Mutations in three genes causing well characterized tumor syndromes are associated with an increased risk of developing PCCs and PGLs, including VHL (von Hippel-Lindau disease), NF1 (Neurofibromatosis Type 1), and RET (Multiple Endocrine Neoplasia Type 2). Mutations in any of the succinate dehydrogenase (SDH) complex subunit genes (SDHA, SDHB, SDHC, SDHD) can lead to PCCs and PGLs with variable penetrance, as can mutations in the subunit cofactor, SDHAF2. Recently, two additional genes have been identified, TMEM127 and MAX. Although these tumors are rare in the general population, occurring in two to eight per million people, they are more commonly associated with an inherited mutation than any other cancer type. This review summarizes the known germline and somatic mutations leading to the development of PCC and PGL, as well as biochemical profiling for PCCs/PGLs and screening of mutation carriers.

Classification and management of cervical paragangliomas.

INTRODUCTION: Cervical paragangliomas are slow-growing tumours that eventually cause lower cranial nerve palsies and infiltrate the skull base. Surgical treatment may cause the same deficits and, in some, risks more serious neurological deficits. We describe a classification used to guide investigation, consent and management of cervical paragangliomas based on extensive experience. METHODS: The case notes of patients managed by the senior author at a tertiary referral skull base unit between 1987 and 2010 were reviewed retrospectively. A total of 87 cervical paragangliomas were identified in 70 patients (mean age: 46 years, range: 13-77 years). Of these, 35 patients had 36 vagal paragangliomas, 43 patients had 50 carotid body paragangliomas and 8 had both. One cervical paraganglioma arose from neither the carotid body nor the nodose ganglion. The main outcome measures were death, stroke, gastrostomy and tracheotomy. RESULTS: All tumours were classified pre-operatively based on their relationship to the carotid artery, skull base and lower cranial nerves. Type 1 tumours were excised with a transcervical approach, type 2 with a transcervical-parotid approach and type 3 with a combined transcervical-parotid and infratemporal fossa approach. Type 4 patients underwent careful assessment and genetic counselling before any treatment was undertaken. There were no peri-operative deaths; two patients had strokes, one required a long-term feeding gastrostomy and none required a tracheotomy. CONCLUSIONS: The use of a pre-operative classification system guides management and surgical approach, improves accuracy of consent, facilitates audit and clarifies which patients should be referred to specialised centres.

Literature survey on epidemiology and pathology of gangliocytic paraganglioma.

BACKGROUND: Although gangliocytic paraganglioma (GP) has generally been regarded as a neuroendocrine tumor, its origin remains unclear. We therefore aimed to investigate the details of this disease by carefully analyzing and extracting common features of the disease as presented in selected publications. METHODS: We searched for English and Japanese cases of GP using the PubMed and IgakuChuoZasshi databases on August 2010. We then extracted and sampled raw data from the selected publications and performed appropriate statistical analyses. Additionally, we evaluated the expression of hormone receptors based on our previously reported case. RESULTS: 192 patients with GP were retrieved from the databases. Patient ages ranged from 15 y to 84 y (mean: 52.3 y). The gender ratio was 114:76 (male to female, 2 not reported). Maximum diameter of the tumors ranged from 5.5 mm to 100 mm (mean: 25.0 mm). The duodenum (90.1%, 173/192) was found to be the most common site of the disease. In 173 patients with duodenal GP, gastrointestinal bleeding (45.1%, 78/173) was found to be the most common symptom of the disease, followed by abdominal pain (42.8%, 74/173), and anemia (14.5%, 25/173). Rate of lymph node metastasis was 6.9% (12/173). Our statistical analysis indicated that significant differences were found for gender between GP within the submucosal layer and exceeding the submucosal layer. Furthermore, our immunohistochemical evaluation showed that both epithelioid and pancreatic islet cells showed positive reactivity for progesterone receptors. CONCLUSIONS: Our literature survey revealed that there were many more cases of GP exceeding the submucosal layer than were expected. Meanwhile, our statistical analyses and immunohistochemical evaluation supported the following two hypotheses. First, vertical growth of GP might be affected by progesterone exposure. Second, the origin of GP might be pancreatic islet cells. However, it is strongly suspected that our data have been affected by publication bias and to confirm these hypotheses, further investigation is required.

[S100 protein positive sustentacular cells in pulmonary carcinoids and thoracic paragangliomas: differential diagnostic and prognostic evaluation]. / S100-Protein-positive Sustentakularzellen in pulmonalen Karzinoiden und thorakalen Paragangliomen : Differenzialdiagnostische und prognostische Bedeutung.

Paragangliomas have a classical histomorphology comprising a so-called "Zellballen" or nesting pattern with surrounding S100 protein positive sustentacular cells (SC) which form a meshwork with a wire-fence appearance. In adrenal and extra-adrenal paragangliomas the prevalence of SC is inversely associated with the patients' outcome. In order to get more insight into the prevalence as well as the prognostic and differential diagnostic value of this cell population in pulmonary carcinoids, we investigated a panel of 26 tumorlets, 147 typical and atypical pulmonary carcinoids and ten thoracic paragangliomas immunohistochemically. We were able to demonstrate that S100 protein positive cells are similarly distributed in both thoracic paragangliomas and pulmonary carcinoids. Hence, the presence and distribution of these cells does not appear to represent a reliable criterion in differential diagnosis. Moreover, all pulmonary carcinoid patients with a worse outcome had low numbers of or no S100 protein positive cells in their tissue specimens. Thus, the prevalence of these cells may potentially aid in prognostic assessment of pulmonary carcinoids, especially in biopsies.

[Neurogenic tumors of the mediastinum in adults]. / Tumeurs nerveuses du médiastin de l'adulte.

In adults, mediastinal neurogenic tumours constitute the third group of mediastinal tumours, after thymomas and lymphomas. If the group of neurogenic tumour is frequent, each type of tumour is relatively unusual in everyday's clinic. Among them, nerve sheath tumours are the more frequent, followed by tumour of the autonomic system. Askin tumour remains uncommon. Treatment of this tumour requires complete preoperative work-up, including standard radiography, CT-scan, MRI, and sometimes nuclear imaging. In most cases, the treatment is based on surgical resection, and may be associated with radiotherapy or chemotherapy in case of malignant tumour or incomplete resection. Better understanding of these tumours, including their molecular abnormalities, may lead to new changes in their classifications, and to their management.

[Classification of head and neck paragangliomas]. / Clasificación de los paragangliomas cervicocefálicos.

The terms glomus or chemodectoma arose with knowledge of the histological structure of these tumors; their paraganglionic cells, together with autonomic ganglion cells, form the paraganglia, and consequently the most appropriate term to describe these tumors is paraganglioma. Classification of these tumors varies according to the parameter chosen: patient age, secretory capacity or biochemical behavior, whether the tumor is isolated or syndromic, or benign or malignant, etc. Moreover, there are other classifications based on other features such as localization, extension, the recommended surgical approach, immunohistochemical characteristics of the tumor, whether the tumor is hereditary, etc. None of these criteria have been universally accepted. Thus, in the present article, the most important criteria will be described. By paying attention to their localization, extension and the recommended surgical approaches, the classification of these tumors has progressed and, at the same time, diagnostic imaging tests and surgical techniques have improved. This type of classification is of great interest from the clinical-surgical point of view and consequently the present article describes them in detail, especially in temporal and carotid body paragangliomas.

Clasificación de los paragangliomas cervicocefálicos / Classification of head and neck paragangliomas

Los términos glomus o quemodectoma surgen con el conocimiento de la estructura histológica de estos tumores; sus células paraganglionares, junto con las células autonómicas ganglionares, forman los paraganglios, por lo que el término paraganglioma resulta el más adecuado para referirse a ellos. La clasificación de estos tumores varía en función del parámetro elegido para su realización: edad del paciente, capacidad secretora o funcionalidad desde el punto de vista bioquímico, si son tumores aislados o sindrómicos, si son benignos o malignos, etc. Además, existen otras clasificaciones basadas en otros aspectos, como localización, extensión, abordajes quirúrgicos recomendados, características inmunohistoquímicas del tumor, carácter hereditario, etc. Ninguna de ellas ha sido aceptada de forma universal, así que procederemos a describir las más importantes en el siguiente capítulo. Atendiendo a la localización, extensión y abordajes quirúrgicos recomendados, la forma de clasificar estos tumores ha ido evolucionando, a la vez que han mejorado las técnicas diagnósticas de imagen y las técnicas quirúrgicas. Este tipo de clasificaciones es de gran interés desde el punto de vista clinicoquirúrgico, y éste es el motivo por el que serán más detalladas durante la exposición, sobre todo en los paragangliomas temporales y carotídeos (AU)

The terms glomus or chemodectoma arose with knowledge of the histological structure of these tumors; their paraganglionic cells, together with autonomic ganglion cells, form the paraganglia, and consequently the most appropriate term to describe these tumors is paraganglioma. Classification of these tumors varies according to the parameter chosen: patient age, secretory capacity or biochemical behavior, whether the tumor is isolated or syndromic, or benign or malignant, etc. Moreover, there are other classifications based on other features such as localization, extension, the recommended surgical approach, immunohistochemical characteristics of the tumor, whether the tumor is hereditary, etc. None of these criteria have been universally accepted. Thus, in the present article, the most important criteria will be described. By paying attention to their localization, extension and the recommended surgical approaches, the classification of these tumors has progressed and, at the same time, diagnostic imaging tests and surgical techniques have improved. This type of classification is of great interest from the clinical-surgical point of view and consequently the present article describes them in detail, especially in temporal and carotid body paragangliomas (AU)

A importância da embolização pré-operatória no tratamento do tumor do corpo carotídeo: relato de caso e revisão da literatura / The importance of preoperative embolization for the treatment of the carotid body tumor: case report and review of the literature

Os tumores do corpo carotídeo são neoplasias raras, que se originam dos pequenos órgãos quimio e barorreceptores localizados na adventícia da bifurcação da artéria carótida comum. Constituem-se uma doença de grande interesse para o cirurgião vascular, na medida em que crescem aderidos à adventícia dos vasos que compõem essa bifurcação. Por isso, sua cirurgia requer não só o conhecimento anatômico da região, mas também perfeito reconhecimento das técnicas de reconstrução vascular. Representam um problema especial quanto a seu manejo, devido à sua rica vascularização e intimidade com estruturas nobres da região cervical, como nervos e grandes vasos. Neste caso, apresentamos um homem com um tumor de corpo carotídeo aderido à carótida direita, diagnosticado por punção biópsia e tratado em dois tempos, sendo o primeiro por tratamento endovascular, realizando embolização percutânea do tumor, e, no segundo, a ressecção cirúrgica do mesmo, o que evidencia o tratamento combinado, segundo atual literatura.

Carotid body tumors are rare neoplasms originating from the small chemo- and baroreceptors located in the adventitia of the common carotid artery bifurcation. They are a disease of great interest for vascular surgeons, given that they grow adhered to the adventitia of vessels comprising this bifurcation. For that, their surgery requires not only anatomical knowledge of the region, but also perfect familiarization with vascular repair techniques. Carotid body tumors are a particular problem as to their management, due to rich vascularization and intimacy with important structures of the cervical region, such as nerves and large vessels. We report on a male patient with carotid body tumor adhered to the right carotid artery, diagnosed by puncture biopsy and treated at two different time periods: first by endovascular treatment, with percutaneous embolization of the tumor; and later by surgical resection, which represents the combined treatment suggested in the current literature.