Fifteen Years of Experience with Surgical Management of Bilateral Non-familial Carotid Body Tumors.

BACKGROUND: Bilateral carotid body tumors (CBTs) clinical manifestation is infrequent. We conducted this work to describe our experience in the surgical treatment of bilateral CBT and to analyze our results. METHODS: A retrospective, observational study. We analyzed the totality of bilateral CBT resections that had been performed in our institution from January 2008 to September 2023. Data was obtained from medical records and anonymized, ethics approval was obtained from our institution committee. As the number of observations was less than those required by the central limit theorem our sample was considered nonparametric. Statistical analysis was performed on Stata 17. RESULTS: We evaluated 16 patients with a total of 32 CBT; surgical resection was performed in 28 cases (87.50%). Median age of the patients was 60 years (interquartile range [IQR] 46-64). Regarding the Shamblin classification, 9 CBTs (32.14%) were classified as Shamblin I, 11 (39.29%) as Shamblin II, and 8 (28.57%) as Shamblin III. The median Distance to the Base of the Skull (DTBOS) was 3.5 cm (IQR 2.7-5.1), and the median tumor volume was 11.25 cc (IQR 3.4-18.7). The median bleeding volume was 300 ml (IQR 200-500), and the median surgical time was 190 min (IQR 145-240). All surgeries were performed using the Retrocarotid Dissection technique. We documented 9 (32.14%) cases of nerve injuries, all of which were transitory. In the median regression a statistically significant association was found between DTBOS, Shamblin classification and tumor volume with intraoperative bleeding and length of stay. CONCLUSIONS: Surgical treatment remains safe and should be considered the gold standard for accurate histologic diagnosis. DTBOS and tumor volume, in addition to Shamblin classification, must be considered in preoperative planning to predict bleeding and hospital stay.

Retrocarotid dissection technique compared to the caudocranial approach for the surgical treatment of carotid body tumors: A 15-year experience.

INTRODUCTION: Carotid body tumors are rare neoplasms with malignant potential. We aim to follow up on our initial experience published in 2015 and compare the occurrence of complications and postoperative outcomes with the use of retrocarotid dissection (RCD) against the standard caudocranial (SCCD) technique. METHODS: This was an observational, case-control study in which we analyzed all of the carotid body tumor resections performed from 1986 to 2022. Parametric and nonparametric tests were used accordingly. Statistical analysis was performed on Stata 17. RESULTS: A total of 181 surgical procedures were included, mean age was 56 years (± 13.63), and 168 (93%) were performed in women. The mean medio-lateral diameter was larger in the RCD group (2.85 ± 1.57 cm vs 1.93 ±1.85 cm; p = 0.002) and presurgical embolization was more frequently performed in the SCCD group (27.5% vs 0.7%; p < 0.001). A total of 40 (22.09%) resections were performed using the SCCD technique. In contrast, in 141 (77.91%) procedures the RCD technique was used. The mean surgical time in the RCD group was lower (197.37 ± 70.56 min vs 232 ± 98.34 min; p = 0.01). No statistically significant difference was found between SCCD and RCD in terms of vascular lesions (n = 20 [11.04%], 15% vs 9%, respectively; p = 0.36), transient or permanent nerve injuries (25% vs 33%, respectively; p = 0.31), or mean intraoperative bleeding (SCCD: 689.95 ± 680.05 mL vs RCD: 619.64 ± 837.94 mL; p > 0.05). CONCLUSIONS: RCD appears to be a safe and equivalent alternative to the standard caudocranial approach in terms of intraoperative bleeding or vascular lesions, with a sustained, significant decrease in surgical time.

Paraganglioma cuerpo carotídeo: experiencia en 20 años y revisión de la literatura / Carotid body paraganglioma: 20-year experience and literature review

Resumen El paraganglioma carotideo es un tumor infrecuente, originado de las células de la cresta neural. Raramente son secretores y tienen un bajo potencial maligno. El diagnóstico es difícil y requiere una alta sospecha clínica, combinada con estudios imagenológicos. Su tratamiento está basado en la cirugía, con especial cuidado de las estructuras vasculonerviosas que se encuentran en intimo contacto. Se describe la casuística de paragangliomas de cuerpo carotídeo en Clínica Las Condes y compararla con una revisión de la literatura actualizada del tema.

Abstract Carotid paraganglioma is a rare tumor, originated from neural crest cells. Usually they lack hormone secretion function, and have a low malignant potential. Diagnosis is difficult, and requires high clinical suspicious, combined with image and pathologic findings. Its treatment is based on surgery, with special care of close anatomic relation with important vascular-nervous structures. Here, we present cases of carotid paragangliomas evaluated at Clinica Las Condes comparing them with an updated literature review.

Clinical Characteristics and Surgical Outcomes of Carotid Body Tumors: Data from the Carotid Paraganglioma Cooperative International Registry (CAPACITY) Group.

BACKGROUND: This study's objective was to conduct a multinational registry of patients with carotid body tumors (CBTs) and to analyze patients' clinical characteristics, treatments, and outcomes. METHODS: Retrospective study from the Carotid Paraganglioma Cooperative International Registry involving eleven medical centers in Bolivia, Ecuador, Mexico, and Spain, of all patients with a CBT who underwent resection between 2009 and 2019. RESULTS: A total of 1432 patients with a CBT surgically treated were included. Median patient age was 54 years (range: 45-63 years), and 82.9% (1184) of the study cohort were female. While at low altitude, the proportion of female-to-male cases was 2:1, at high altitude, this proportion increased to 8:1, with statistically significant differences (p = .022). Median operative time was 139 min (range: 110-180 min), while median operative blood loss was 250 ml (range: 100-500 ml), with statistically significant difference in increased blood loss (p = .001) and operative time (p = .001) with a higher Shamblin classification. Eight (0.6%) patients suffered stroke. Univariate analysis analyzing for possible factors associated with increased odds of stroke revealed intraoperative vascular lesion to present an OR of 2.37 [CI 95%; 1.19-4.75] (p = 0.001). In 245 (17.1%), a cranial nerve injury was reported. Seven (0.5%) deaths were recorded. CONCLUSION: The most common CBT type on this cohort was hyperplasic, which might be partially explained by the high altitudes where these patients lived. Increased blood loss and operative time were associated with a higher Shamblin classification, and the risk of stroke was associated with patients presenting transoperative vascular lesions.

Surgical Management of Bilateral Carotid Body Tumors.

BACKGROUND: Carotid body tumors (CBTs) are rare neoplasms located in the carotid bifurcation. The majority of these tumors are unilateral; bilateral CBTs represent approximately 5% of all affected patients, and the recommended treatment is to surgically remove them in staged-planned surgeries. We describe the experience, outcomes, and the surgical management of bilateral CBTs in our institution. METHODS: A retrospective review of CBTs patients was completed; patient demographics, comorbidities, lesion location, anatomic characteristics, surgical techniques, complications, reinterventions, and other factors that may influence outcomes were evaluated. RESULTS: A total of 109 patients with CBTs were treated surgically; of these, 8 had bilateral CBTs (7%); the mean age was 56 years, and 7 (87%) were females. Thirteen surgical resections were performed, and in 2 of the cases, the pathology report was malignant (15%). Five were classified as Shamblin I (31%), 5 as Shamblin II (31%), and remaining 6 as Shamblin III (38%). The mean time between the first and second procedure was of 10.7 months. Complications included one case of neck hematoma requiring evacuation and postoperative neurologic complications occurred in three patients (one patient with facial and two with vocal cord palsies). None of the studied individuals had a family history of CBT, and all of them lived in altitude areas higher than 2000 meters above mean sea level (mamsl). The mean tumor size was 3.55 cm and 2.75 cm for right and left CBTs, respectively. CONCLUSIONS: A better understanding of the clinical characteristics of patients with bilateral CBTs may lead to a more standardized and optimal management with fewer complications and a better quality of life afterward.

New predictors of complications in carotid body tumor resection.

OBJECTIVE: This study examined the relationship between two new variables, tumor distance to base of skull (DTBOS) and tumor volume, with complications of carotid body tumor (CBT) resection, including bleeding and cranial nerve injury. METHODS: Patients who underwent CBT resection between 2004 and 2014 were studied using a standardized, multi-institutional database. Demographic, perioperative, and outcomes data were collected. CBT measurements were determined from computed tomography, magnetic resonance imaging, and ultrasound examination. RESULTS: There were 356 CBTs resected in 332 patients (mean age, 51 years; 72% female); 32% were classified as Shamblin I, 43% as Shamblin II, and 23% as Shamblin III. The mean DTBOS was 3.3 cm (standard deviation [SD], 2.1; range, 0-10), and the mean tumor volume was 209.7 cm3 (SD, 266.7; range, 1.1-1642.0 cm3). The mean estimated blood loss (EBL) was 257 mL (SD, 426; range, 0-3500 mL). Twenty-four percent of patients had cranial nerve injuries. The most common cranial nerves injured were the hypoglossal (10%), vagus (11%), and superior laryngeal (5%) nerves. Both Shamblin grade and DTBOS were statistically significantly correlated with EBL of surgery and cranial nerve injuries, whereas tumor volume was statistically significantly correlated with EBL. The logistic model for predicting blood loss and cranial nerve injury with all three variables-Shamblin, DTBOS, and volume (R2 = 0.171, 0.221, respectively)-was superior to a model with Shamblin alone (R2 = 0.043, 0.091, respectively). After adjusting for Shamblin grade and volume, every 1-cm decrease in DTBOS was associated with 1.8 times increase in risk of >250 mL of blood loss (95% confidence interval, 1.25-2.55) and 1.5 times increased risk of cranial nerve injury (95% confidence interval, 1.19-1.92). CONCLUSIONS: This large study of CBTs demonstrates the value of preoperatively determining tumor dimensions and how far the tumor is located from the base of the skull. DTBOS and tumor volume, when used in combination with the Shamblin grade, better predict bleeding and cranial nerve injury risk. Furthermore, surgical resection before expansion toward the base of the skull reduces complications as every 1-cm decrease in the distance to the skull base results in 1.8 times increase in >250 mL of blood loss and 1.5 times increased risk of cranial nerve injury.

Management of Carotid Bifurcation Tumors: 30-Year Experience.

BACKGROUND: The carotid bifurcation can host a variety of tumors requiring complex surgical management. Treatment requires resection and, in some cases, vascular reconstruction that may compromise the cerebral circulation. The most frequent lesion at this location is the carotid body tumor (CBT). CBT are classified according to Shamblin in 3 types depending on the degree of carotid vessels encasement. Our main objective was to report our clinical experience managing carotid bifurcation tumors throughout the last 30 years. METHODS: Between 1984 and 2014, we treated 30 patients with 32 carotid bifurcation tumors. There were 21 women and 9 men (2.3:1), with a mean age of 45.5 years (18-75). The most frequent presentation was an asymptomatic neck swelling or palpable mass localized at the carotid triangle (86.7%). RESULTS: Thirty of 32 tumors were resected. Since 1994, computed tomography scan has been the most frequently used diagnostic imaging tool (80%), followed by magnetic resonance imaging. Angiography was used mainly during the first 10 years of the study period. Mean size of the tumor was 44.6 mm (20-73 mm). Nineteen (63%) were classified as Shamblin II and 6 (20%) as Shamblin's III. All specimens were analyzed by a pathologist; 28 tumors (93%) were confirmed as paragangliomas, 2 (7%) were diagnosed as schwannomas. Two patients underwent preoperative embolization of the CBT; 5 patients (17%) required simultaneous carotid revascularization, all of them Shamblin III. Mean hospitalization time was 4.5 days (1-35 days). Transient extracranial nerve deficit was observed in 7 patients (23.3%). Three patients (Shamblin III) required red blood cells transfusion. One patient (Shamblin III) underwent a planned en bloc excision of the vagus nerve. There was no perioperative mortality or procedure-related stroke. No malignancy or tumor recurrence were observed during follow-up. CONCLUSIONS: CBTs can be diagnosed on clinical grounds requiring vascular imaging confirmation. These infrequent lesions are generally benign. Early surgical removal by surgeons with vascular expertise avoids permanent neurologic and or vascular complications.

[Hypoplasia of internal carotid artery associated with carotid paraganglioma. A case report and review of the literature]. / Hipoplasia de arteria carótida interna asociado con paraganglioma carotideo. Reporte de un caso y revisión de la bibliografía.

BACKGROUND: Hypoplasia of the internal carotid artery is a rare congenital malformation. It has been mainly associated with aneurysms and other pathologies but not in association with paraganglioma. The incidence is 0.01% of all the anomalies of carotid vessels. Although the exact cause is unknown, it is thought to represent a sequel to an insult due to mechanical causes or hemodynamic stress but perhaps also involves aspects of molecular biology of embryonic development. CLINICAL CASE: We describe the case of a 37 year-old female patient with paraganglioma associated with hypoplasia of the internal carotid artery, which was found incidentally during surgery. Previous angiographic studies as well as other analyses were carried out, but we failed to detect hypoplasia of the internal carotid artery. Tumor was removed along with ligation of the external carotid artery due to injury. The hypoplastic internal carotid artery was left intact. CONCLUSION: Angiographic studies of the base of the skull are important as well as hemodynamic analysis in order to not overlook these anomalies. The patient had a satisfactory evolution without sequelae.

Antecedentes: la hipoplasia de la arteria carótida interna es una rara malformación congénita. Se le ha relacionado principalmente con aneurismas y otros padecimientos, pero no con paraganglioma. Su incidencia es menor de 0.01% de todas las anomalías de los vasos carotídeos. Aunque su origen exacto no se conoce, se cree que es una secuela de una lesión provocada por causas mecánicas o por estrés hemodinámico; sin embargo, también pudieran estar implicados aspectos de biología molecular del desarrollo embrionario. Caso clínico: se describe un caso raro de una mujer de 37 años de edad con paraganglioma concomitante con hipoplasia de la arteria carótida interna. En estudios angiográficos y análisis rutinarios previos no se había detectado alteración de la arteria carótida interna. En la intervención se extirpó el tumor y se ligó la arteria carótida externa dado que estaba involucrada en la lesión; se dejó intacta la arteria carótida interna hipoplásica. Conclusión: es importante realizar estudios angiográficos de la base del cráneo, así como análisis hemodinámicos para no pasar por alto estas anomalías. La evolución fue satisfactoria y sin secuelas.

[Carotid body paraganglioma in a teenager. Case report]. / Paraganglioma del cuerpo carotídeo en adolescente. Reporte de caso.

BACKGROUND: Paragangliomas of the head and neck are neuroendocrine tumors. They have a low incidence (0.6%), are generally benign, have a poorly defined etiology, and multiple factors have been associated with their origin. Humans and other species living at high altitudes (>2000 m above sea level) are subjected to a relatively chronic hypoxia and there is a high prevalence of the development of carotid body hyperplasia and eventually paragangliomas. This disease is usually seen in patients in their 50s and in their 30s if there is a family history. CLINICAL CASE: We present the case of a 16 year-old female with acute pharyngitis and growing tumor located on the left side of the neck, without symptoms. A duplex Doppler ultrasound showed a solid nodular lesion on the left carotid bifurcation. A left lateral cervicotomy was performed, finding a highly vascularized tumor of 4 × 3 × 3 cm involving the common carotid from its middle third, the internal carotid up to the cranial base, and the external carotid to its upper third, and intimately related to the trachea, esophagus and cervical spine. The tumor was completely resected and the histopathological analysis corroborated the presence of paragangliomas. CONCLUSIONS: The publication of this case is relevant and of clinical interest due to the uncommon age of presentation and the fact that it should be considered as a diagnostic possibility.

Antecedentes: los paragangliomas de cabeza y cuello son tumores neuroendocrinos de baja incidencia (0.6%), en su mayor parte benignos, en cuyo origen se han involucrado múltiples factores. Los seres humanos y otras especies que viven a grandes alturas (por arriba de 2000 metros sobre el nivel del mar) son propensos a sufrir hipoxia crónica relativa, hiperplasia del cuerpo carotídeo y, eventualmente, paragangliomas. Este padecimiento aparece en la quinta década de la vida y en la tercera, en caso de presentación familiar. Caso clínico: se comunica el caso de una adolescente de 16 años de edad que un año antes tuvo faringitis aguda con tumor localizado en la cara lateral izquierda del cuello, de crecimiento gradual, sin ningún síntoma. El ultrasonido Doppler dúplex de cuello mostró una lesión nodular sólida sobre el trayecto de la bifurcación carotídea izquierda. Fue intervenida quirúrgicamente mediante cervicotomía lateral izquierda, en la cual se encontró una tumoración de aproximadamente 4 × 3 × 3 cm, sumamente vascularizada, que afectaba la carótida común desde su tercio medio, la carótida interna hasta la base del cráneo y la carótida externa hasta el tercio superior, con íntima relación con la tráquea, el esófago y la columna cervical. La tumoración se resecó en su totalidad, el estudio histopatológico corroboró los paragangliomas. Conclusiones: la edad de presentación es poco común, por lo que se considera relevante y de interés clínico comunicar este caso, toda vez que debe considerarse como posibilidad diagnóstica.

Head and neck paragangliomas: clinical and molecular genetic classification.

Head and neck paragangliomas are tumors arising from specialized neural crest cells. Prominent locations are the carotid body along with the vagal, jugular, and tympanic glomus. Head and neck paragangliomas are slowly growing tumors, with some carotid body tumors being reported to exist for many years as a painless lateral mass on the neck. Symptoms depend on the specific locations. In contrast to paraganglial tumors of the adrenals, abdomen and thorax, head and neck paragangliomas seldom release catecholamines and are hence rarely vasoactive. Petrous bone, jugular, and tympanic head and neck paragangliomas may cause hearing loss. The internationally accepted clinical classifications for carotid body tumors are based on the Shamblin Class I-III stages, which correspond to postoperative permanent side effects. For petrous-bone paragangliomas in the head and neck, the Fisch classification is used. Regarding the molecular genetics, head and neck paragangliomas have been associated with nine susceptibility genes: NF1, RET, VHL, SDHA, SDHB, SDHC, SDHD, SDHAF2 (SDH5), and TMEM127. Hereditary HNPs are mostly caused by mutations of the SDHD gene, but SDHB and SDHC mutations are not uncommon in such patients. Head and neck paragangliomas are rarely associated with mutations of VHL, RET, or NF1. The research on SDHA, SDHAF2 and TMEM127 is ongoing. Multiple head and neck paragangliomas are common in patients with SDHD mutations, while malignant head and neck paraganglioma is mostly seen in patients with SDHB mutations. The treatment of choice is surgical resection. Good postoperative results can be expected in carotid body tumors of Shamblin Class I and II, whereas operations on other carotid body tumors and other head and neck paragangliomas frequently result in deficits of the cranial nerves adjacent to the tumors. Slow growth and the tendency of hereditary head and neck paragangliomas to be multifocal may justify less aggressive treatment strategies.

Head and neck paragangliomas: clinical and molecular genetic classification

Head and neck paragangliomas are tumors arising from specialized neural crest cells. Prominent locations are the carotid body along with the vagal, jugular, and tympanic glomus. Head and neck paragangliomas are slowly growing tumors, with some carotid body tumors being reported to exist for many years as a painless lateral mass on the neck. Symptoms depend on the specific locations. In contrast to paraganglial tumors of the adrenals, abdomen and thorax, head and neck paragangliomas seldom release catecholamines and are hence rarely vasoactive. Petrous bone, jugular, and tympanic head and neck paragangliomas may cause hearing loss. The internationally accepted clinical classifications for carotid body tumors are based on the Shamblin Class I-III stages, which correspond to postoperative permanent side effects. For petrous-bone paragangliomas in the head and neck, the Fisch classification is used. Regarding the molecular genetics, head and neck paragangliomas have been associated with nine susceptibility genes: NF1, RET, VHL, SDHA, SDHB, SDHC, SDHD, SDHAF2 (SDH5), and TMEM127. Hereditary HNPs are mostly caused by mutations of the SDHD gene, but SDHB and SDHC mutations are not uncommon in such patients. Head and neck paragangliomas are rarely associated with mutations of VHL, RET, or NF1. The research on SDHA, SDHAF2 and TMEM127 is ongoing. Multiple head and neck paragangliomas are common in patients with SDHD mutations, while malignant head and neck paraganglioma is mostly seen in patients with SDHB mutations. The treatment of choice is surgical resection. Good postoperative results can be expected in carotid body tumors of Shamblin Class I and II, whereas operations on other carotid body tumors and other head and neck paragangliomas frequently result in deficits of the cranial nerves adjacent to the tumors. Slow growth and the tendency of hereditary head and neck paragangliomas to be multifocal may justify less aggressive treatment strategies.

Mutation of SDHB is a cause of hypoxia-related high-altitude paraganglioma.

PURPOSE: Paragangliomas of the head and neck are neuroendocrine tumors and are associated with germ line mutations of the tricarboxylic acid cycle-related genes SDHB, SDHC, SDHD, and SDHAF2. Hypoxia is important in most solid tumors, and was directly implicated in tumorigenesis over 40 years ago when it was shown that dwelling at high altitudes increases the incidence of carotid body hyperplasia and paragangliomas. Although recent research has now elucidated several pathways of hypoxia in paragangliomas, nothing is currently known of the genetics or of gene-environment interactions in high-altitude paraganglioma. We postulated that SDH mutations might play a role in these tumors. EXPERIMENTAL DESIGN: Patients from a Mexican family, originating and resident in Guadalajara, were tested for mutations of SDHD, and subsequently, for mutations of SDHB followed by immunohistochemical confirmation of SDHB loss. RESULTS: Two patients, born and resident at altitudes of between 1,560 and 2,240 m, were found to have head and neck paragangliomas, including a remarkably aggressive recurrent tumor. Mutation analysis identified a pathogenic missense mutation in exon 7 of SDHB, c.689G>A, p.Arg230His, and loss of the SDHB protein was confirmed by immunohistochemistry. CONCLUSIONS: This is the first report of a SDH gene mutation in paraganglioma at high altitude. A rapidly recurrent head and neck paraganglioma is a very rare finding in an SDH mutation carrier, suggesting a gene-environment interaction. Neither patient showed evidence of sympathetic paraganglioma.

Tumor del corpúsculo carotídeo / Carotid corpuscle tumor

Los tumores del cuerpo carotídeo, paragangliomas o quemodectomas son neoplasias poco frecuentes que se originan a partir de los tejidos del paraganglio, son generalmente benignos, de crecimiento lento y curso indoloro, presentan un gran componente vascular en su estructura. Pueden manifestar un comportamiento locorregional agresivo a pesar que histológicamente no muestren signos de malignidad. Se observan frecuentemente en pacientes que habitan en regiones de elevada altitud. Pueden ser esporádicos o familiares y se asocian en ocasiones a paragangliomas en otros sitios. Presentamos un caso clínico tratado en nuestro servicio en el que se evidencia la agresividad local en su comportamiento

Carotid body tumors, paragangliomas or chemodectomas are uncommon neoplasms originating from paraganglion tissues, generally are benign, of slow growth and painless course, have a great vascular component in its structure. They may to show an aggressive locoregional behavior despite histologically haven't malignancy signs. Frequently are present in patients inhabit in high altitude regions. May to be sporadic or familial and occasionally are associated with paragangliomas in other sites. Present case was treated in our service, which demonstrates its local aggressive behavior

Nonresectable carotid body tumor: hybrid surgical procedure to achieve complete and safe resection.

BACKGROUND: Carotid body tumors of Shamblin class III without free internal carotid, between the tumor and skull base, are considered nonresectable. The objective of this work is to describe a surgical technique that combines traditional and endovascular strategies to achieve a safe surgical resection of the carotid body tumor of this class. METHODS AND RESULTS: A female patient with a nonresectable carotid body tumor underwent placement of an endoprosthesis going from the common carotid artery to the internal carotid beyond the skull base (to secure cerebral circulation) excluding the external carotid artery. Forty-five days later, the tumor was resected without vascular or cranial nerves injury, and bleeding amounted to 50 cm(3). At 6 months, the patient is tumor free and asymptomatic. CONCLUSION: This is the first known reported case that has been successfully resolved by combining endovascular and traditional strategies. The endoprosthesis maintained cerebral circulation, which otherwise would have been impossible; besides, the vascular supply from the external carotid artery was excluded leading to a decrease in tumor size and a recovery of the sub-adventitial dissection plane, allowing for a successful and safe tumor resection.

Tumor del cuerpo carotídeo: A propósito de 10 casos tratados / Carotid body tumors: Report of ten cases

Background: Carotid body tumors arise from a cellular conglomerate located at the carotid bifurcation. Progressive enlargement can involve the arterial wall and neighbor cranial nerves. Aim: To report a series of 10 patients treated of carotid body tumors and review national experience. Patients and methods: Between 1984 and 2006, we operated 8 women and 2 men, aged 19 to 75 years, with this type of tumor. Results: The most common cause for consultation was a cervical mass in 90 percent, with a mean evolution lapse of 13.2 months (range 3 to 126). In all cases, diagnosis was confirmed with angiographic imaging and histopathology. Ten tumors were surgically removed with no complications. Eighty percent of tumors were in stage II according to Shamblin classification. During long term follow up all patients have remained asymptomatic. Only 31 carotid body tumors have been reported in Chilean medical literature during a 43 year period. Conclusions: Paragangliomas of the carotid body can be diagnosed in clinical grounds, requiring vascular imaging. These infrequent lesions are generally benign, early surgical removal by surgeons with vascular expertise avoids neurological and or vascular complications.

[Carotid body tumors: report of ten cases]. / Tumor del cuerpo carotídeo: A propósito de 10 casos tratados.

BACKGROUND: Carotid body tumors arise from a cellular conglomerate located at the carotid bifurcation. Progressive enlargement can involve the arterial wall and neighbor cranial nerves. AIM: To report a series of 10 patients treated of carotid body tumors and review national experience. PATIENTS AND METHODS: Between 1984 and 2006, we operated 8 women and 2 men, aged 19 to 75 years, with this type of tumor. RESULTS: The most common cause for consultation was a cervical mass in 90%, with a mean evolution lapse of 13.2 months (range 3 to 126). In all cases, diagnosis was confirmed with angiographic imaging and histopathology. Ten tumors were surgically removed with no complications. Eighty percent of tumors were in stage II according to Shamblin classification. During long term follow up all patients have remained asymptomatic. Only 31 carotid body tumors have been reported in Chilean medical literature during a 43 year period. CONCLUSIONS: Paragangliomas of the carotid body can be diagnosed in clinical grounds, requiring vascular imaging. These infrequent lesions are generally benign, early surgical removal by surgeons with vascular expertise avoids neurological and or vascular complications.

Paragangliomas de cabeza y cuello / Parangangliomas of the head and neck

Los parangangliomas son tumores altamente vascularizados que se originan de células provenientes de la cresta neural. El tratamiento de elección es la resección quirúrgica completa, lo cual se ve dificultado por su abundante irrigación y por su estrecha relación anatómica con importantes estructuras vásculo-nerviosas. En el presente estudio se revisa retrospectivamenete la experiencia del Departamento de Otorrinolaringología de Clínica Las Condes en el manejo quirúrgico de estas neoplasias. Entre los años 1998 y 2003 se trataron 5 pacientes portadores de paragangliomas de cabeza y cuello: un glomus yugular (GY), 2 glomus carotídeo (GC) y 2 glomus timpánicos (GT). El estudio imagenológico consistió en tomografía computarizada (TC) para todos los casos, complementada con resonancia nuclear magnética (RNM) y/o angiografía en algunos pacientes. El abordaje quirúrgico fue el indicado para cada lesión: timpanotomía retroauricular (GT), cervicotomía (GC) y abordaje infratemporal tipo A de Fisch (GY). En todos se logró una resección tumoral completa, sin presentarse complicaciones perioperatorias de consideración ni recurrencias hasta la fecha. Contando con un adecuado estudio imagenológico y una cuidadosa planificación quirúrgica, la resección de los paragangliomas de cabeza y cuello puede realizarse con un bajo índice de complicaciones, reservando la radioterapia como alternativa terapéutica únicamente en aquellos casos inoperables.

Carotid body tumors: review of a 20-year experience.

Carotid body tumors (CBT) are a rare entity that should be considered in evaluating every lateral neck mass. The objective of the study was to compare demographic data, complications and evolution of patients treated at our institution. A retrospective study was made of 66 patients with 69 CBT that were treated at our institution between 1982 and 2002. We reviewed the demographic characteristics, clinical features, surgical approach and complications. Women significantly predominated (96.9%) with a female:male ratio of 31.2:1. Ages ranged from 18 to 94 (mean=50.2). Fifty-four per cent of the patients lived at altitudes higher than 2200 m above sea level. The most common chief complaint was a painless neck mass (78.7%). No patient had any malignant tumors or a familial history of CBT. Both sides were similarly affected. There were four tumors grouped in Shamblin's class I, 24 in class II, and 35 in class III. Six tumors were of undetermined Shamblin's class as inferred from their medical records. Fifty-three patients received treatment: 46 (86.8%), surgery; 6 (11.3%), radiotherapy; 1 (1.9%), radiotherapy following surgery. Three patients (6.3%) underwent vascular reconstruction. In 23 (49%) patients neurological deficit was observed after surgery. Minor complications occurred in five (10.6%) patients. Median follow-up was 38 months, one patient died from a cause not related to the CBT, and the rest remain disease-free and asymptomatic. We found an overwhelming predominance of women, which opens the possibility that we are dealing with a different disease in female Latin populations. Most of the tumors were of Shamblin's class III CBT. Early surgical management is recommended to avoid neurological deficit due to a Shamblin's class III tumor.

Malignant paraganglioma with vertebral metastasis: case report.

A paraganglioma is a rare tumor, composed of chromaffin cells, groups of cells associated to the autonomous system. When the tumor occurs in the adrenal gland, it is called pheochromocitoma. The malignant paraganglioma is a very rare presentation; it is diagnosed by local recurrence after total resection of the primary mass, or findings of distant metastases. We present a case report of a 29-year-old woman with cervico-brachial pain. In 1995 she underwent a carotid body tumor resection. Magnetic resonance imaging (MRI), plain X-rays and computerized tomography scan revealed multiple lesions in C5, T5 and T12. She underwent a surgical procedure to correct the cervical lesion. The histological and immunohistochemical assays revealed a malignant paraganglioma. She received adjuvant radiotherapy, showing clinical improvement after treatment, presenting no symptoms after one year. The therapeutic approach is based on the total resection of the tumor. The treatment of distant metastases can be made with adjuvant measures such as conventional radiotherapy, I 131-MIBG, or chemotherapy, especially in malignant pheochromocitomas.

Malignant carotid body tumors: report of three cases.

The aim of the current report was to describe 3 cases of malignant carotid body tumor (CBT) and to review the literature. My clinical records of 87 CBTs in 81 patients (6 bilateral) were reviewed, 79 of which were operated on. Three malignant cases were found. The first was in a 40-year-old man who presented with pulmonary metastases 6 years after resection of a CBT. He was treated with chemotherapy and interferon, but died with disseminated disease 2 years later. The second case was in a 56-year-old woman who had a 5-cm, fixed, hard mass in the upper aspect of the neck and a paralysis of the left vocal cord. This lesion was completely resected, and a shunt and reconstruction with a saphenous vein graft were performed. Pathology revealed a malignant chemodectoma with invasion to 2 of the 5 lymph nodes removed. Radiotherapy (50 Gy) was given after the operation. She is well and free of disease 68 months after the resection. The third case was in a 61-year-old woman who presented with an 8-cm nontender, hard, immobile mass in the left upper neck that displaced the left wall of the oropharynx toward the midline. A carotid arteriogram showed a CBT. On computed tomography, the tumor extended to the infratemporal fossa with no bone involvement. The lesion was embolized with a 40% reduction in vascularity. At surgical exploration, the tumor involved the sternocleidomastoid muscle and the lymph nodes at levels II and III, and the internal carotid artery could not be dissected free at the skull base, so only a partial resection was performed. This patient was lost to follow-up. These 3 cases are in agreement with the literature. Locoregional control is usually obtained with complete primary tumor resection and lymphadenectomy and eventual radiotherapy. Surgery with radiotherapy seems to be effective for isolated metastases. Current multidisciplinary treatments have been unsuccessful in controlling disseminated disease.