Long-Term Outcomes of Patients With Carotid and Aortic Body Tumors.

Chemodectomas are tumors derived from parasympathetic nonchromaffin cells and are often found in the aortic and carotid bodies. They are generally benign but can cause mass-effect symptoms and have local or distant spread. Surgical excision has been the main curative treatment strategy. The National Cancer Database was reviewed to study all patients with carotid or aortic body tumors from 2004 to 2015. Demographic data, tumor characteristics, treatment strategies, and patient outcomes were examined, split by tumor location. Kaplan-Meier survival estimates were generated for both locations. In total, 248 patients were examined, with 151 having a tumor in the carotid body and 97 having a tumor in the aortic body. Many variables were similar between both tumor locations. However, aortic body tumors were larger than those in the carotid body (477.80 ± 477.58 mm vs 320.64 ± 436.53 mm, p = 0.008). More regional lymph nodes were positive in aortic body tumors (65.52 ± 45.73 vs 35.46 ± 46.44, p <0.001). There were more distant metastases at the time of diagnosis in carotid body tumors (p = 0.003). Chemotherapy was used more for aortic body tumors (p = 0.001); surgery was used more for carotid body tumors (p <0.001). There are slight differences in tumor characteristics and response to treatment. Surgical resection is the cornerstone of management, and radiation can often be considered. In conclusion, chemodectomas are generally benign but can present with metastasis and compressive symptoms that make understanding their physiology and treatment important.

Gastric glomus tumor: A rare etiology of upper gastrointestinal bleed.

The glomus tumor of stomach is an unusual submucosal mesenchymal tumor of the gastrointestinal tract. We describe a 42-year-old female who presented with chronic anemia and an episode of painless hematemesis. A preoperative diagnosis of the probable gastric gastrointestinal stromal tumor was made. Post-surgical histopathological examination of the specimen demonstrated a glomus tumor of the stomach confirmed on immunohistochemistry. The present case highlights the importance of morphology and immunohistochemistry in differentiating the subepithelial tumors of the stomach and one must consider glomus tumor in differential diagnosis of these gastric lesions.

An aortic body carcinoma with sarcomatoid morphology and chondroid metaplasia in a French Bulldog.

An 11-year-old female French Bulldog was presented with a mass at the base of the heart, detected by X-ray and echocardiography. Clinical abnormality included abdominal retention by ascites. Radiation therapy was performed for 5 weeks. The mass volume didn't change during the radiotherapy. The condition became worse and the dog died 6 months after the initial presentation and necropsy was performed. Grossly, the mass, 12.5 × 6.5 × 6.0 cm in size, was found at the base of the heart. Histopathological examination revealed that cardiac mass was composed of alveolar, bundle and diffuse proliferation of neoplastic cells. Most of the neoplastic cells showed a spindle morphology; in some areas small round or polyhedral neoplastic cells were observed. Occasional cartilage metaplasia was seen multifocal in the mass, and it was surrounded by the sarcomatoid proliferation. Electron microscopy revealed a few neuroendocrine granules in the cytoplasm of spindle and polyhedral neoplastic cells. Metastatic cells in the lungs which had not irradiated demonstrated typical morphology of aortic body tumors. Based on these findings, the case was diagnosed as an aortic body carcinoma with sarcomatoid morphology and chondroid metaplasia.

Cross-Sectional Imaging and Thoracoscopic Resection of Functional Intrathoracic Paraganglioma of the Aortic Body.

Paragangliomas are extra-adrenal tumors that are derived from neuroendocrine chromaffin cells. The rare disease has a variable presentation depending upon its anatomic location and functionality. We describe the case of a 56-year-old female patient who had an incidental mass found on imaging. The patient underwent biochemical testing and a mediastinal biopsy due to the nonspecific imaging findings. The mediastinal mass was resected through hemisternotomy and found to be a functional paraganglioma. This case highlights the importance of radiologists to consider the prospect of paragangliomas in the differential diagnosis of an intra-thoracic mass.

Metastatic neuroendocrine carcinoma of aortic body origin in a cat.

An 8-year-old, female spayed Domestic Shorthair cat was presented to the Auburn University Emergency and Critical Care service for evaluation of pleural effusion and a suspected intrathoracic mass. Computed tomography was performed which confirmed the presence of a large intrathoracic mass, likely heart-based. Fine-needle aspirates were obtained and a cytologic diagnosis of a neuroendocrine tumor was made. Treatment with toceranib phosphate was briefly attempted at home by the owners. The cat died at home approximately 6 weeks after diagnosis. Necropsy and subsequent histopathologic examination revealed a metastatic neuroendocrine carcinoma of aortic body origin. Aortic body tumors are extremely rare in cats and to the authors' knowledge, a neuroendocrine carcinoma of aortic body origin with distant metastases has not yet been reported in a cat.

Estrogen regulates energy metabolic pathway and upstream adenosine 5'-monophosphate-activated protein kinase and phosphatase enzyme expression in dorsal vagal complex metabolosensory neurons during glucostasis and hypoglycemia.

The ability of estrogen to shield the brain from the bioenergetic insult hypoglycemia is unclear. Estradiol (E) prevents hypoglycemic activation of the energy deficit sensor adenosine 5'-monophosphate-activated protein kinase (AMPK) in hindbrain metabolosensory A2 noradrenergic neurons. This study investigates the hypothesis that estrogen regulates A2 AMPK through control of fuel metabolism and/or upstream protein kinase/phosphatase enzyme expression. A2 cells were harvested by laser microdissection after insulin or vehicle (V) injection of E- or oil (O)-implanted ovariectomized female rats. Cell lysates were evaluated by immunoblot for glycolytic, tricarboxylic acid cycle, respiratory chain, and acetyl-CoA-malonyl-CoA pathway enzymes. A2 phosphofructokinase (PFKL), isocitrate dehydrogenase, pyruvate dehydrogenase, and ATP synthase subunit profiles were elevated in E/V vs. O/V; hypoglycemia augmented PFKL and α-ketoglutarate dehydrogenase expression in E only. Hypoglycemia increased A2 Ca(2+) /calmodulin-dependent protein kinase-ß in O and reduced protein phosphatase in both groups. A2 phospho-AMPK levels were equivalent in O/V vs. E/V but elevated during hypoglycemia in O only. These results implicate E in compensatory upregulation of substrate catabolism and corresponding maintenance of energy stability of A2 metabolosensory neurons during hypoglycemia, outcomes that support the potential viability of molecular substrates for hormone action as targets for therapies alleviating hypoglycemic brain injury.

Histopathological and immunohistochemical evaluation of malignant potential in canine aortic body tumours.

In order to verify the malignant potential of aortic body tumours (ABTs) in dogs, 13 cases of canine ABT were studied histopathologically and immunohistochemically. The cases were divided into two groups according to the presence or absence of metastases to other organs at necropsy examination (metastasis group [n = 9] and non-metastasis group [n = 4]). The mean tumour weight:body weight ratio (TW:BW; g/kg) in the metastasis group (9.3 ± 6.7) was significantly higher than that in the non-metastasis group (1.5 ± 1.7) (P <0.05). In both groups, the neoplastic cells had malignant features including pleomorphism, anisocytosis and anisokaryosis, and mononuclear giant cells were present, showing invasion through the capsule and into the vascular lumen and other adjacent tissues. The mitotic index (MI), mean nuclear area (NA) for size value and coefficient of variation of the nuclear area (CVNA) for anisonucleosis did not differ significantly between the two groups. These findings show that anaplastic characteristics are present regardless of the tumour size or the presence or absence of metastases, suggesting that these tumours are generally malignant or potentially malignant. Immunohistochemical analysis using neuroendocrine markers including neuron-specific enolase, chromogranin A and S100 revealed no obvious differences in labelling intensity of neoplastic cells related to the presence or absence of metastases or associated with the mean TW:BW, MI, NA or CVNA value, indicating that immunohistochemistry has no practical value for determining the tumour grade of canine ABTs.

A case of synchronous hepatocellular carcinoma and aortic body chemodectoma in a dog - pathological case report.

The authors describe a case of synchronously occurring (double) tumours, i.e. primary hepatocellular carcinoma and aortic body chemodectoma in a 14-year-old mixed-breed male dog. The tumours were identified during necropsy, following euthanasia. In the last months of its life, the dog showed signs of weakness, anorexia, apathy, inactivity, and abdominal palpation elicited a painful reaction. The primary liver cancer emerged in the left lateral lobe without evidence of any distant metastases. Histopathological and immunohistochemical investigations revealed a well-differentiated, trabecular, claudin-7-, claudin-5- and pancytokeratin-negative hepatocellular carcinoma. The Ki-67 proliferation index was 33%. During necropsy, a synchronously occurring benign, grade I type aortic body chemodectoma was also detected in the dog. This neuroendocrine tumour showed chromogranin-, synaptophysin-, neuron-specific enolase- and S100 protein-positivity, and the Ki-67 proliferation index was 2%. The authors believe that this is the first description of synchronously occurring hepatocellular carcinoma and aortic body chemodectoma in a dog.

Clinical, histopathologic, and radiographic indicators of malignancy in head and neck paragangliomas.

OBJECTIVE: The goal of this study is to review our series of head and neck paragangliomas to identify factors that may help in predicting malignancy. STUDY DESIGN: Case series with chart review. SETTING: Academic medical center. SUBJECTS AND METHODS: Subjects with head and neck paragangliomas at our institution from 1976 to current were reviewed. In addition to statistical comparisons of epidemiologic factors, pathologic and radiographic characteristics were reviewed. RESULTS: Of the 84 subjects, there were seven malignant paragangliomas (8%). Age was found to be significantly different between the benign and malignant subgroups, with an average age of 54 ± 16 and 40 ± 12 years, respectively (P = 0.02). Pain was a presenting complaint in five patients with benign disease (6%), and five of the seven malignant patients (71%) presented with pain, showing a significant association between pain and disease type (P < 0.0001). The odds ratio for a patient with pain having a malignant tumor was 36 (95% CI: 5.5-234). Enlarging neck mass was noted in all cases of malignant disease, but only in 31 percent of cases of benign disease (P < 0.0001). In a secondary analysis of carotid body tumors alone, enlarging neck mass was not found to be significant between benign and malignant disease (P = 0.14). However, pain continued to be significantly different, with 67 percent of malignant lesions demonstrating pain, compared with only 11 percent of benign lesions (P = 0.01). CONCLUSION: This study suggests that pain, a rapidly enlarging neck mass, and younger age are predictive factors of underlying malignancy, which should prompt one to consider an aggressive diagnostic and management approach.

Aortic body tumor in full-sibling English bulldogs.

A 10-year-old, neutered male English bulldog died acutely from respiratory distress after a short history of progressive dyspnea. Less than 2 months later, a spayed female full sibling of that dog died suddenly during a nail trim. An aortic body tumor was the cause of death in both dogs based on postmortem and histological examinations. A pheochromocytoma was also diagnosed in the neutered male. Neither dog had a history of brachycephalic airway syndrome, and the implication for a genetic predisposition toward the development of paraganglioma is discussed. This is the first case report of aortic body tumors in sibling dogs, although the condition may not be an uncommon phenomenon.

Simultaneous aortic body tumor and pulmonary histiocytic sarcoma in a flat-coated retriever.

A case of multiple primary tumors observed in the heart base and in the lung of a 7-year-old intact female, flat-coated retriever was reported. Morphological differences between both tumors and detailed immunohistochemical study revealed that the cardiac neoplasm was as a malignant aortic body tumor and the lung tumor was a pulmonary histiocytic sarcoma. The occurrence of aortic body tumor with other primary neoplasms has been previously reported in animals suggesting that this might be a common presentation in dogs.

Cervical paragangliomas: is SDH genetic analysis systematically required?

To evaluate the prevalence of succinate dehydrogenase (SDH) B, C, and D germline mutations in a surgical series of cervical paragangliomas and to precise the characteristics of patients presenting with familial form. Among 29 patients operated on cervical paragangliomas (carotid or vagal body) at our institution between 1994 and 2007, 23 could be asked for a genetic analysis and a familial study. Clinical characteristics of patients harboring a germline SDH mutation were studied and compared with those presenting without mutation. Mutations were found in 8/23 (35%) patients, mostly in SDHD gene (6 cases), and in SDHB and SDHC gene, respectively, in one case each. Mean age at onset was significantly lower for patients with mutation (34 vs. 51.5 years, P = 0.01). In patients presenting with a mutation, 50% had a family history of paraganglioma compared with 0% for others (P = 0.008) and 87.5% had a multifocal form of paragangliomas versus 0% for others (P = 0.001). No difference was found concerning malignant forms between the two groups (12.5 vs. 13.3%). In the 16 patients who had an apparently sporadic paraganglioma, 6% had mutations in the SDH gene. A positive family history of paraganglioma and/or the presence of bilateral or multiple paragangliomas and/or an early age of onset are the main parameters associated with SDH mutations. Genetic testing should be considered for all patients with a cervical paraganglioma, even for those presenting with an apparently sporadic tumor as familial form may be such identified in 6% of cases.

Paragangliomas de cabeza y cuello: revisión de 89 casos en 73 pacientes / Head and neck paragangliomas: revision of 89 cases in 73 patients

Introducción: Los paragangliomas son tumores poco frecuentes de origen neuroectodérmico. Se los considera tumores benignos, pero en algunas ocasiones tienen un comportamiento biológico similar a los tumores malignos (< 10 %). Las mutaciones germinales en los genes SDHB, SDHC y SDHD, que codifican las subunidades del mismo nombre en el complejo enzimático mitocondrial de la succinato deshidrogenasa, tienen un papel importante en la patogenia. Material y método: Se trata de un estudio retrospectivo en el que se revisa a 73 pacientes intervenidos en nuestro servicio con un total de 89 paragangliomas, ya que 8 pacientes presentaban paragangliomas múltiples. Los paragangliomas se distribuyeron de la siguiente forma: 33 yugulares, 17 timpánicos, 26 carotídeos y 13 vagales. Todos estos pacientes tuvieron un seguimiento mínimo de 1 año. Se evaluaron las vías de abordaje en función de la localización tumoral, las secuelas acaecidas y su ulterior evolución, así como las recurrencias y su relación con la localización del tumor primario. Resultados: El tratamiento fue quirúrgico, utilizando la radiocirugía como tratamiento complementario en un paciente. En los paragangliomas yugulares se realizó un abordaje infratemporal tipo A, en los carotídeos y vagales el abordaje fue cervical y en los timpánicos, transmeatal o transmastoideo. De los 73 pacientes con paragangliomas intervenidos que componen nuestra población en estudio, hubo 11 recurrencias, que aparecieron en los paragangliomas yugulares, que en 2 casos fueron paragangliomas múltiples. Las secuelas postoperatorias fueron sobre todo la parálisis de nervios craneales (VII, IX, X, XI y XII), junto con las fístulas de líquido cefalorraquídeo en el 14 % de los paragangliomas yugulares. Conclusiones: Con este artículo pretendemos reflejar nuestra experiencia en el tratamiento de este tipo de tumores. El tratamiento quirúrgico consigue un excelente control de la enfermedad con una morbilidad aceptable en pacientes de mediana edad o jóvenes. Para disminuir las probabilidades de parálisis facial en los paragangliomas yugulares, debe evitarse la transposición del facial en el abordaje infratemporal de la fosa yugular

Introduction: Paragangliomas (PGL) are uncommon neuroectodermal tumours. PGL are usually clinically benign tumours, although metastasis has been reported and invasive growth can occur in adjacent tissues (<10 %). Mutations in SDHB, SDHC, and SDHD, which encode sub-units of mitochondrial complex II (succinate dehydrogenase), play an important role in the pathogenesis of these tumours. Material and method: Retrospective review of 73 patients with 89 paragangliomas who had undergone resection of the PGL in our hospital. There were 8 patients who displayed multiple PGL. PGL were distributed as follows: 33 were jugular, 17 tympanic, 26 carotid body tumours, and 13 vagal paragangliomas. All these patients had a follow-up time of at least a year. The surgical approach was evaluated in terms of tumour origin, sequelae, and subsequent evolution, as well as the relapses and their relation with location of the primary tumour. Results: The treatment was surgical, using complementary radiosurgery in just 1 patient. The type A infratemporal fossa approach was used in jugular paragangliomas, the approach was cervical in the carotid and vagal ones and, in the tympanics, a transmeatal or transmastoid approach was performed. In the 73 patients making up our study group, there were 11 recurrences which appeared in jugular paragangliomas (two of them in multiple PGL cases). The post-operative sequelae were mainly cranial nerve paralysis (VII, IX, X, XI, and XII), along with cerebrospinal fluid fistulas in 14 of the jugular PGLs. Conclusions: With this article we try to reflect our experience in the treatment of this type of tumour. Surgical treatment achieves excellent control of the disease with an acceptable morbidity in young or middle-aged patients. In order to diminish the probabilities of facial nerve paralysis in jugular PGL we must avoid the facial nerve transposition in the infratemporal approach

Use of s-100 and chromogranin a antibodies as immunohistochemical markers on detection of malignancy in aortic body tumors in dog.

To define the characteristics of malignancy we performed routine histology and an immunohistochemical study on seventeen aortic body tumors in dogs. We essayed tumors using a panel of immunohistochemical markers: neuron specific enolase (NSE), chromogranin A (CrA) and S-100. Among 17 cases, the neoplastic cells were positive for NSE (17 cases, 100%), S-100 (9 cases, 53%), and CrA (8 cases, 47%), respectively. The sustentacular cells density and chief cell staining intensity were both inversely related to tumor grade. The most relevant data was consistent with a negative staining of S-100 correlated with absence or decreased number of sustentacular cells in tumors grade III. This report indicates that the immunohistochemical panel has utility for the diagnosis of chemodectoma and the negative staining to CrA and S-100 markers in tumors grade III expresses an indication of malignant behaviour of the tumor.

Aporte de las imágenes en el diagnóstico de los paragangliiomas de cabeza y cuello / Radiologic support for the diagnosis of paragangliomas of the head and neck

Los paragangliomas de cabeza y cuello son tumores que derivan de las células neuroendócrinas de los paraganglios extrasuprarrenales y se extienden desde la base de cráneo hasta el cayado aórtico. El pronóstico es en general favorable, excepto los que se ubican en el hueso temporal. En el presente trabajo se describen las características en imágenes de las diferentes localizaciones y subtipos de este tumor, en conjunto con una revisión de la literatura hasta la actualidad. Los métodos por imágenes son muy útiles para poder arribar al diagnóstico. El diagnóstico diferencial es de vital importancia a la hora de evitar procedimientos invasivos riesgosos y contraindicados en estos pacientes, como la biopsia

Aporte de las imágenes en el diagnóstico de los paragangliiomas de cabeza y cuello / Radiologic support for the diagnosis of paragangliomas of the head and neck

Los paragangliomas de cabeza y cuello son tumores que derivan de las células neuroendócrinas de los paraganglios extrasuprarrenales y se extienden desde la base de cráneo hasta el cayado aórtico. El pronóstico es en general favorable, excepto los que se ubican en el hueso temporal. En el presente trabajo se describen las características en imágenes de las diferentes localizaciones y subtipos de este tumor, en conjunto con una revisión de la literatura hasta la actualidad. Los métodos por imágenes son muy útiles para poder arribar al diagnóstico. El diagnóstico diferencial es de vital importancia a la hora de evitar procedimientos invasivos riesgosos y contraindicados en estos pacientes, como la biopsia

Glomus tumors: therapeutic role of selective embolization.

The primary goals of preoperative embolization of glomus tumors are to reduce the blood loss in the surgical field, minimize the risk of operative complications, and prevent recurrence by contributing to complete resection. Obliteration of a vascular channel may provide additional help in symptomatic relief by decreasing the tumor volume. Selective transarterial embolotherapy outcomes of 17 patients, most of whom had inoperable tumors and were unable to have surgery/radiotherapy because of poor general health, were evaluated to investigate the therapeutic contributions, efficacy, and safety of embolization techniques in the treatment of different glomus tumors and to examine the role of the embolotherapy in the treatment algorithm of such lesions. The pattern of vascular supply of these tumors was also documented. Eleven glomus jugulotympanicum, 4 glomus caroticum, and 2 glomus vagale tumors were embolized for palliative or curative purposes between 1992 and 2000. Coils and combination of coils plus polyvinyl alcohol were used for embolization. Relief of symptoms and patient satisfaction were analyzed within 3 months after the intervention by a questionnaire combined with full ear, nose, and throat and neurological examinations. The results indicate that the use of embolotherapy in the treatment of glomus tumors must be basically preoperative but is not curative.