THE CAROTID BODY TUMOR
paraganglioma in young female height
asymptomatic
selective carotid arteriography shows vascular paraganglioma at the carotid bifurcation
COMPARATIVE CLINICAL STUDY OF CAROTID BODY TUMORS
Mujer adulta con gran paraganglioma izquierdo en la serie de La Paz
arteriography showing carotid paraganglioma in bifurcacióm left in the series of La Paz
We discussed in a previous article of this blog: " Corpuscle CAROTID IN THE HEIGHT IS BIGGER AND HEAVY "already Arias Stella in 1973 had reported this finding and that just happens to hemoglobin increased over the years. Also that hypoxia may alter the histopathologic structure of this tissue to produce a hyperplastic response and perhaps tumors called chemodactomas, chemodeclarnas or paragangliomas associated with hypoxic stimulus height. So
these carotid body tumors called paragangliomas besides being very rare at sea level, so are in the height but 20 times more frequent.
This time, among many articles, we have chosen a published in Acta Andina 1995; 4 (1): 3-12 where the Bolivian Medical Hospital Dr. L. Obrero de Bolivia Wayllace and Belgian doctors Haot Jules, Jacques Rahier Catholic University of Leuven, Brucella, make an elegant review clinical aspects of the entity. We have dared to make a didactic summary of that article.
not touch the current aspects of diagnosis and nuclear magnetic resonance angiography (goldstandard examination for diagnosis), or the surgical aspects of the "Classification Shambin" because of specialty items, and lack of expertise not comment.
Dr. Achilles Monroy
these carotid body tumors called paragangliomas besides being very rare at sea level, so are in the height but 20 times more frequent.
This time, among many articles, we have chosen a published in Acta Andina 1995; 4 (1): 3-12 where the Bolivian Medical Hospital Dr. L. Obrero de Bolivia Wayllace and Belgian doctors Haot Jules, Jacques Rahier Catholic University of Leuven, Brucella, make an elegant review clinical aspects of the entity. We have dared to make a didactic summary of that article.
not touch the current aspects of diagnosis and nuclear magnetic resonance angiography (goldstandard examination for diagnosis), or the surgical aspects of the "Classification Shambin" because of specialty items, and lack of expertise not comment.
Dr. Achilles Monroy
asymptomatic
COMPARATIVE CLINICAL STUDY OF CAROTID BODY TUMORS
Luis Wayllace, Jules Haot, Jacques Rahier
SUMMARY.
parallel to the carotid body hyperplasia (CC), there is an increased incidence of tumors of this organ in height. This comparative study of tumors of the carotid body (TCC) of La Paz, Bolivia (3600 meters) and Brussels-Belgium (sea level) this frequency is 20 times higher in native high in patients originating from regions sea level.
The profile of patients with TCC is different altitude or sea level. The TCC in height are usually unilateral, occur in middle-aged individuals (51.1 + 11 years) with a female preponderance (89%), are exceptionally evil, are rarely associated with other paragangliomas and inheritance seems to play no role in pathogenesis. Among patients
sea level are two situations: there is a first group similar to the high TCC affecting middle-aged patients (mean 50 years) without known heredity for this type of injury or for other paragangliomas and the second group consists of younger individuals (mean 29.4 years) where the TCC developed in a context Hereditary neuroendocrine tumors. The frequency of malignant TCC is highest at sea level in height and is independent of the heredity of injuries. SUMMARY
. Closely
to carotid body hyperplasia, Carotid Body Tumor (CBT) Are frequently seen in high altitude regions. In this comparative clinical study CBT We Have Seen That in La Paz, Bolivia (3600 m) Are More Frequent Than 20 folds at sea-level (Belgium).
There Are Several clinical Difference between high altitude and sea-level CBTs. The Former group is Composed Mainly by middle aged (51 + 11 years) with Predominance female (89%). Tumors Are Usually unilateral, They Are Almost never Malignant and They Are Usually Not associated with other paragangliomas. Heredity seems not to play any significant etiopathogenic role.
In the sea-level CBT series there are two groups: the former resembles clinically to the high-altitude series: patients are middle aged (average 50 years) without hereditary background for CBT neither for paragangliomas. The second group is composed by younger patients (average 29.4 y.) where CBT grow within an hereditary background of neuroendocrine tumor.Malignant CBT are more frequently seen in the sea? level regions. Malignancy seems not to be hereditary-related.
INTRODUCCIÓN
El Tumor de Cuerpo Carotídeo (TCC), como el Cuerpo Carotídeo (CC) del que se origina, ha motivado controversias since its first description by March in 1891. The understanding of the nature of this injury has evolved over time, in fact, she was seen on vascular injury (Marchand 1891), a hamartoma (Oberndorfer 1905), a form of hyperplasia exaggerated chemoreceptor tissue (Saldaña et al. 1973) or finally as a true neoplasm provided with clinical and biological characteristics, genetic and epidemiological individuals (Parry et al. 1982).
Despite numerous reports in the literature, TCC is an uncommon injury, which has yet interesting epidemiological aspects. So, after work Arias-Stella (1969) and Arias-Stella and Valcarcel (1973) who observed hyperplasia of the carotid bodies in response to chronic stimulation by hypoxia in height, was that tumors arising in this tissue are more common chemoreceptor in high altitude regions in areas at sea level. These early works were followed by several studies of large series of TCC in the Andean regions of Peru (Saldaña et al. 1973), Bolivia (Rios and Wayllace Dalenz 1977; Aramayo et al. 1989) and Ecuador (Pacheco Ojeda et al. 1982) and Mexico (Krause-Senties 1971, Rodriguez Cuevas et al. 1986). Most of these reports consider to chronic hypoxic stimulation and the decisive factor for the occurrence of these injuries , considered by some as an exaggerated form of hyperplasia or tumors as true by others.
This study compared two sets of patients with carotid body tumors. The former consists of native high-altitude regions of Bolivia (3600 - 4000 m) and the second consists of people from areas located at sea level in Belgium. RESULTS
In the series of La Paz, about 29 628 pathology tests performed, we found 34 cases of carotid body tumors (CBT) lo que representa una prevalencia de 1/1000 y un solo caso de tumor del glomus yugular (TGY), mientras que en la serie de Bruselas, basado en 220 135 exámenes evaluados se encontraron 22 paragangliomas cervicales: 11 casos de TGY y 11 casos de TCC; éste último representando una prevalencia de 1/20 000. La proporción de los TCC es por lo tanto 20 veces más alta en La Paz que en Bruselas mientras que la proporción del TGY es a la inversa más alta en Bruselas que en La Paz.
SUMMARY.
parallel to the carotid body hyperplasia (CC), there is an increased incidence of tumors of this organ in height. This comparative study of tumors of the carotid body (TCC) of La Paz, Bolivia (3600 meters) and Brussels-Belgium (sea level) this frequency is 20 times higher in native high in patients originating from regions sea level.
The profile of patients with TCC is different altitude or sea level. The TCC in height are usually unilateral, occur in middle-aged individuals (51.1 + 11 years) with a female preponderance (89%), are exceptionally evil, are rarely associated with other paragangliomas and inheritance seems to play no role in pathogenesis. Among patients
sea level are two situations: there is a first group similar to the high TCC affecting middle-aged patients (mean 50 years) without known heredity for this type of injury or for other paragangliomas and the second group consists of younger individuals (mean 29.4 years) where the TCC developed in a context Hereditary neuroendocrine tumors. The frequency of malignant TCC is highest at sea level in height and is independent of the heredity of injuries. SUMMARY
. Closely
to carotid body hyperplasia, Carotid Body Tumor (CBT) Are frequently seen in high altitude regions. In this comparative clinical study CBT We Have Seen That in La Paz, Bolivia (3600 m) Are More Frequent Than 20 folds at sea-level (Belgium).
There Are Several clinical Difference between high altitude and sea-level CBTs. The Former group is Composed Mainly by middle aged (51 + 11 years) with Predominance female (89%). Tumors Are Usually unilateral, They Are Almost never Malignant and They Are Usually Not associated with other paragangliomas. Heredity seems not to play any significant etiopathogenic role.
In the sea-level CBT series there are two groups: the former resembles clinically to the high-altitude series: patients are middle aged (average 50 years) without hereditary background for CBT neither for paragangliomas. The second group is composed by younger patients (average 29.4 y.) where CBT grow within an hereditary background of neuroendocrine tumor.Malignant CBT are more frequently seen in the sea? level regions. Malignancy seems not to be hereditary-related.
INTRODUCCIÓN
El Tumor de Cuerpo Carotídeo (TCC), como el Cuerpo Carotídeo (CC) del que se origina, ha motivado controversias since its first description by March in 1891. The understanding of the nature of this injury has evolved over time, in fact, she was seen on vascular injury (Marchand 1891), a hamartoma (Oberndorfer 1905), a form of hyperplasia exaggerated chemoreceptor tissue (Saldaña et al. 1973) or finally as a true neoplasm provided with clinical and biological characteristics, genetic and epidemiological individuals (Parry et al. 1982).
Despite numerous reports in the literature, TCC is an uncommon injury, which has yet interesting epidemiological aspects. So, after work Arias-Stella (1969) and Arias-Stella and Valcarcel (1973) who observed hyperplasia of the carotid bodies in response to chronic stimulation by hypoxia in height, was that tumors arising in this tissue are more common chemoreceptor in high altitude regions in areas at sea level. These early works were followed by several studies of large series of TCC in the Andean regions of Peru (Saldaña et al. 1973), Bolivia (Rios and Wayllace Dalenz 1977; Aramayo et al. 1989) and Ecuador (Pacheco Ojeda et al. 1982) and Mexico (Krause-Senties 1971, Rodriguez Cuevas et al. 1986). Most of these reports consider to chronic hypoxic stimulation and the decisive factor for the occurrence of these injuries , considered by some as an exaggerated form of hyperplasia or tumors as true by others.
This study compared two sets of patients with carotid body tumors. The former consists of native high-altitude regions of Bolivia (3600 - 4000 m) and the second consists of people from areas located at sea level in Belgium. RESULTS
In the series of La Paz, about 29 628 pathology tests performed, we found 34 cases of carotid body tumors (CBT) lo que representa una prevalencia de 1/1000 y un solo caso de tumor del glomus yugular (TGY), mientras que en la serie de Bruselas, basado en 220 135 exámenes evaluados se encontraron 22 paragangliomas cervicales: 11 casos de TGY y 11 casos de TCC; éste último representando una prevalencia de 1/20 000. La proporción de los TCC es por lo tanto 20 veces más alta en La Paz que en Bruselas mientras que la proporción del TGY es a la inversa más alta en Bruselas que en La Paz.
Mujer adulta con gran paraganglioma izquierdo en la serie de La Paz Aspectos Clínicos en la Serie de La Paz
La totalidad of patients in this series were from regions between 3600 and 4000 meters above sea level (asl). The series consisted of 30 women and 4 men and the mean age was 51.1 years (25 - 79 years).
All patients except one had a single TCC . 16 with location on the left side, 17 on the right side and in one patient the location was bilateral. No patient had a family history of TCC. The duration of symptoms ranged from 5 months to 20 years. The most common initial symptom was the presence of a latero-cervical mass in most of the patients were painless, sometimes throbbing. Three patients reported discomfort at the tumor.
General symptoms, headache was noted in 4 patients, in one of them this was accompanied by dyspnea on moderate effort. Two patients experienced dizziness. Two patients were hypertensive and one had progressed to permanent drowsy sleep to perform a massage of the TCC.
On physical examination, peripheral cyanosis was recognized evident in three patients. Examination of the cervical region showed that most patients the tumor mass was painless except for 5 persons in whom manipulation caused pain. In 9 patients, TCC was adherent to the deep planes and in 3 people, the mass was mobilized laterally but not vertically. On palpation, 8 TCC were pulsating and showed an increase in local temperature. On auscultation, 3 tumors showed a systolic murmur. The rich vascularity of the tumor mass favored the angiographic diagnosis was possible in all cases (Fig. 2).
One patient who underwent partial resection of a large tumor, showed a significant recurrence 4 years after the first intervention. A woman died during the immediate postoperative neurological and pulmonary complications. The other patients had no recurrence or metastasis during follow-up period ranged from 2 to 7 years.
preoperative embolization was not performed in any patient's tumor.
La totalidad of patients in this series were from regions between 3600 and 4000 meters above sea level (asl). The series consisted of 30 women and 4 men and the mean age was 51.1 years (25 - 79 years).
All patients except one had a single TCC . 16 with location on the left side, 17 on the right side and in one patient the location was bilateral. No patient had a family history of TCC. The duration of symptoms ranged from 5 months to 20 years. The most common initial symptom was the presence of a latero-cervical mass in most of the patients were painless, sometimes throbbing. Three patients reported discomfort at the tumor.
General symptoms, headache was noted in 4 patients, in one of them this was accompanied by dyspnea on moderate effort. Two patients experienced dizziness. Two patients were hypertensive and one had progressed to permanent drowsy sleep to perform a massage of the TCC.
On physical examination, peripheral cyanosis was recognized evident in three patients. Examination of the cervical region showed that most patients the tumor mass was painless except for 5 persons in whom manipulation caused pain. In 9 patients, TCC was adherent to the deep planes and in 3 people, the mass was mobilized laterally but not vertically. On palpation, 8 TCC were pulsating and showed an increase in local temperature. On auscultation, 3 tumors showed a systolic murmur. The rich vascularity of the tumor mass favored the angiographic diagnosis was possible in all cases (Fig. 2).
One patient who underwent partial resection of a large tumor, showed a significant recurrence 4 years after the first intervention. A woman died during the immediate postoperative neurological and pulmonary complications. The other patients had no recurrence or metastasis during follow-up period ranged from 2 to 7 years.
preoperative embolization was not performed in any patient's tumor.
arteriography showing carotid paraganglioma in bifurcacióm left in the series of La Paz DISCUSSION
The carotid body tumor (CBT) is a relatively rare lesion since a comprehensive review completed by Zak and Lawson in 1982 estimated the total reported cases, including Soviet and American literature, between 900 and 1000. For our part, we have found 1033 extra cases in the literature during the period from 1982 to 1992.
All Latin American series, both Peruvian, Mexican, Ecuadorian and Bolivian indicate an increased rate of TCC at elevations above 1500 meters above sea level.
is noteworthy that the height increase in the number of tumors paraganglionic system appears to be limited to the carotid body (CB). Simultaneously with the TCC, we found a single case of TGY in La Paz. In Brussels, however they are more numerous than the CBT (11 patients had TCC TGY and 7 patients during the study period).
The female population is predominant in the high series where it ranges between 74 and 93% with an average of 89%. Most are unilateral without showing preference for one side. The bilateral TCC are exceptional in height (Saldaña et al. 1973). None of the patients had a hereditary predisposition.
The bilaterality of the TCC, the multicentricity of paragangliomas and association with neuroendocrine tumors are observed in parallel with a greater frequency of hereditary factors most often in the series from regions located at sea level. In the literature review conducted in 1982, Zak and Lawson found a relatively high rate of hereditary tumors, the order of 209 cases over 1000 TCC reviewed.
A striking fact is that on 180 cases of carotid body tumors in height, no author mentioned malignant tumors, while the plain series indicate malignancy rates ranging from 4 to 23% (Glenner and Grimley 1974, Zak and Lawson 1982, Parry et al. 1982, et al Klieve . 1989).
Our results of La Paz were not conclusive regarding the neuroendocrine role of the TCC, as it is a series where all but one of the TCC patients had unilateral and therefore retained a functional carotid body.
The carotid body tumor (CBT) is a relatively rare lesion since a comprehensive review completed by Zak and Lawson in 1982 estimated the total reported cases, including Soviet and American literature, between 900 and 1000. For our part, we have found 1033 extra cases in the literature during the period from 1982 to 1992.
All Latin American series, both Peruvian, Mexican, Ecuadorian and Bolivian indicate an increased rate of TCC at elevations above 1500 meters above sea level.
is noteworthy that the height increase in the number of tumors paraganglionic system appears to be limited to the carotid body (CB). Simultaneously with the TCC, we found a single case of TGY in La Paz. In Brussels, however they are more numerous than the CBT (11 patients had TCC TGY and 7 patients during the study period).
The female population is predominant in the high series where it ranges between 74 and 93% with an average of 89%. Most are unilateral without showing preference for one side. The bilateral TCC are exceptional in height (Saldaña et al. 1973). None of the patients had a hereditary predisposition.
The bilaterality of the TCC, the multicentricity of paragangliomas and association with neuroendocrine tumors are observed in parallel with a greater frequency of hereditary factors most often in the series from regions located at sea level. In the literature review conducted in 1982, Zak and Lawson found a relatively high rate of hereditary tumors, the order of 209 cases over 1000 TCC reviewed.
A striking fact is that on 180 cases of carotid body tumors in height, no author mentioned malignant tumors, while the plain series indicate malignancy rates ranging from 4 to 23% (Glenner and Grimley 1974, Zak and Lawson 1982, Parry et al. 1982, et al Klieve . 1989).
Our results of La Paz were not conclusive regarding the neuroendocrine role of the TCC, as it is a series where all but one of the TCC patients had unilateral and therefore retained a functional carotid body.
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