Paraganglioma carotídeo maligno. A propósito de un caso / Malignant carotid paraganglioma: a case report

Los tumores del cuerpo carotídeo son neoplasias poco frecuentes, de lento crecimiento, muy destructivos y vascularizados, que tienen un origen neuroectodérmico. Los tumores malignos representan el 5-13% de los casos y presentan un comportamiento agresivo local con infiltración de los ganglios linfáticos adyacentes o metástasis a distancia. Presentamos el caso de un varón de 60 años al que en el estudio por dolor y parestesias en miembro superior se le realiza una resonancia magnética donde se visualiza una gran tumoración en el espacio carotídeo izquierdo, la cual se biopsió obteniendo el diagnóstico histológico de paraganglioma. Al realizar el estudio de extensión se identificó la presencia de metástasis pulmonares y óseas, lo que confirma la malignidad del tumor

Tumors of the carotid body are uncommon neoplasms that originate in the neuroectoderm. These tumors are slow growing but well vascularized and very destructive. Between 5% and 13% of carotid body tumors are malignant, behaving aggressively at the local level, invading the adjacent lymph nodes, and resulting in remote metastases. We present the case of a 60-year-old man who was examined for pain and paresthesias in his arm. Magnetic resonance imaging showed a large tumor in the left carotid space. Histologic study of a biopsy specimen from the tumor revealed that it was a paraganglioma. Further studies to determine the extent of disease detected metastases in bone and lung, confirming the tumor's malignancy

Metastatic brain carotid body paraganglioma with endocrine activity: a case report and literature review.

A woman with hypertension and hyperglycemia was diagnosed a metastatic brain carotid body paraganglioma. Her blood pressure, glucose, and norepinephrine were normal after craniotomy. Although most carotid body tumors are benign, a few show distant metastasis. This is the first reported case of intracerebral metastases from a carotid body tumor.

Hipertensión arterial refractaria en paciente joven secundaria a tumor del cuerpo carotídeo bilateral productor de catecolaminas / Refractory arterial hypertension in a young patient with a bilateral catecholamine-secreting carotid body tumour

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Carotid Body Tumors: A Case Series and Review of the Literature.

BACKGROUND: Paragangliomas of the head and neck are rare vascular tumors derived from the paraganglia tissues originating from the neural crest. They are usually benign and hypervascularized. Diagnosis is relatively easy in condition to consider it in evaluating every lateral neck mass. METHODS: We made a retrospective study of the records of 10 patients who presented with carotid body tumors at the Department of Vascular surgery of the Military Hospital Avicenne in Marrakech during the period between 2008 and 2013. Epidemiologic, etiologic, diagnostic, and therapeutic features were analyzed. RESULTS: The average age of our patients was 35.4 years (26-55 years), with a male predominance (sex ratio = 2.33). We noted 7 cases of isolated carotid locations and 3 cases of multiple locations. A slow-growing neck mass was the main clinical presentation. Other signs were pain, dysphonia, dizziness, headache, and tinnitus. Physical examination showed, in most cases, a neck nontender mass with side to side mobility. Imaging techniques included Doppler ultrasound, computed tomography (CT) scan, magnetic resonance imaging, and catheter arteriography. Urinary analysis for metanephrine was carried out in 1 case. The clinical presentation and imaging results strongly suggested the diagnosis of carotid paraganglioma in all cases. Treatment was surgical excision in all cases associated with a preoperative embolization in 1 case and a postoperative radiotherapy in 2 cases. Pathology confirmed the diagnosis, and a lymph node metastasis was suspected of malignity in 1 case. The evolution was favorable in all our patients. CONCLUSIONS: Carotid body tumor requires early diagnosis and an adequate multidisciplinary team. The diagnosis must be considered in the case of any pulsatile cervical mass. Surgery is the treatment of choice despite its risks especially in large tumors. The therapeutic indication should, ideally, be set in a multidisciplinary consultation.

Multidisciplinary management of locally advanced and widely metastatic paraganglioma in a patient with life-threatening compressive symptoms.

BACKGROUND: Patients presenting with locally advanced, metastatic paraganglioma with life-threatening compressive symptoms of critical anatomic structure pose a significant management challenge. METHODS: We present a case of a 15-year-old patient with enlarging right neck mass causing dysphagia and respiratory compromise from near complete obstruction of the oropharynx. RESULTS: Evaluation of the patient's family history led to the identification of a mutation in the succinate dehydrogenase subunit B (SDSD) gene (G725A). Laboratory and imaging workup revealed an 8.8 × 6.6 × 4.1 cm metabolically and biochemically active right neck mass, a tumor in the left para-aortic region, and multiple bony lesions consistent with widely metastatic disease. Multidisciplinary management included preoperative clinical optimization, coil embolization, and palliative resection of the neck mass. CONCLUSION: Although the currently available treatment options for patients with advanced metastatic paraganglioma render no survival benefit, a multidisciplinary management approach aimed at relief of tumor-related symptoms and catecholamine excess should be undertaken.

Metastasis of carcinoma of the lung to a carotid body paraganglioma.

Tumour-to-tumour metastasis is a rare phenomenon, but has been described in the literature in just over 100 cases. It can be particularly puzzling for the reporting pathologists, when encountered unexpectedly in a tumour showing abrupt transition from the usual morphology to another unusual pattern. The literature reports a variety of combinations with carcinoma-to-carcinoma being the most common; and renal cell carcinomas appear to the most common recipient tumours with common donor tumours being breast, lung and renal cell carcinomas. The authors report a case of poorly-differentiated lung carcinoma metastasising into a carotid body paraganglioma. Our case is unique and in our knowledge the first described case of carotid body paraganglioma with metastasis from a lung primary. To the best of our knowledge this is the first report of this interesting biological phenomenon in this combination.

Malignant carotid body tumor with systemic metastases.

Carotid body tumors (CBTs) are rare neoplasms of the carotid body. Most CBTs are benign; however, some can show malignant behavior. Malignant CBTs have an unpredictable history; often, there is no correlation between the histologic findings and the clinical behavior. They are usually diagnosed by the development of local recurrence or lymph node metastasis following total resection of the primary mass, or by the detection of distant metastasis. There are few reports of histopathologic confirmation of malignant CBTs. We report a rare case of malignant CBT with distant metastases, in which the diagnosis was confirmed by histopathology, and present a review of the literature.

A role for ipsilateral, selective neck dissection in carotid body tumours.

INTRODUCTION: A reliable diagnosis of malignant carotid body tumour can only be made in the presence of metastatic disease, because the histological features of the primary tumour do not correlate with clinical behaviour. CASE REPORT: We report two cases of malignant carotid body tumour in which regional nodal biopsy at the time of excision of the primary tumour revealed unsuspected metastatic disease. DISCUSSION: Reoperation in the neck for recurrent metastatic carotid body tumour is difficult and potentially hazardous. The presence of occult metastatic disease is easily identified if a selective - or sentinel - nodal dissection is performed routinely in cases of carotid body tumour excision. Such an approach adds very little morbidity, effort or time to the primary surgery, and is recommended. This view has been supported by some other authors but is generally overlooked in clinical practice.

Bilateral carotid body tumor: the role of fine-needle aspiration biopsy in the preoperative diagnosis.

Carotid body (CB) is a round to ovoid or flattened structure situated within the adventitia of the common carotid artery bifurcation on both sides of the neck. CB contains two basic types of cells: chief cells (or glomus type 1) and sustentacular cells (glomus type 2). Carotid body tumor (CBT) or paraganglioma arises from the chief cells of the carotid body. The diagnosis of CBT is typically made with radiological studies. Fine needle aspiration biopsy (FNAB) is seldom requested for this purpose due to rare but dreadful reported complications such as hemorrhage and damage to the carotid artery. In this report we discuss the cytological findings of a malignant CBT diagnosed by FNAB in a 22 year-old female.

[Malignant paraganglioma with vertebral and skull metastasis]. / Paragangliome malin avec métastases vertébrales et de la voûte crânienne.

A paraganglioma is a rare tumor composed of chromaffin cells. Malignant paraganglioma is a very rare presentation diagnosed by local recurrence after total resection of the primary mass or findings of distant metastasis. We report a case of a 30-year-old man who had undergone a carotid body tumor resection 10 years before and who presented to us with recurrence of the tumor associated with lymph node, vertebra and skull metastasis. The diagnosis of malignant paraganglioma was based on loco-regional and distant metastasis.

First report of hepatic lobectomy for metastatic carotid body tumor.

Hepatic lobectomy for metastatic colon cancer is well accepted, yielding a 30-35% five-year survival with a low mortality of less than 5%. Less commonly is hepatic resection for selected metastasis from other organs. We report here what we believe is the first hepatic lobectomy for a metastatic carotid body tumor. The patient was a 41-year-old white female who presented with a large incapacitating hepatic metastasis and an incidental lung metastasis from a carotid body tumor resected 12 years earlier. The patient underwent left hemihepatectomy and local lymph node dissection at our university. Twenty-one months after the operation the patient is asymptomatic and has no sign of tumor reoccurrence . We discuss here the clinical features, pathophysiology, treatment and the surgical literature of this rare entity. This is yet another example of the effectiveness of hepatic resection for noncolonic metastasis (26 references).

Experiencia en el tratamiento quirúrgico del tumor del corpúsculo carotídeo / Our experience in surgical treatment of carotid body tumor

Antecedentes: El tumor del corpúsculo carotídeo (TCC) es una lesión infrecuente del área de cabeza y cuello, con características clínicas y quirúrgicas que merecen especial consideración. Objetivos: Describir la metodología diagnóstica, técnica quirúrgica y evolución clínica de una serie de pacientes operados de un TCC. Lugar de aplicación: Servicio de cirugía oncológica. Diseño: Estudio observacional retrospectivo. Población: 9 pacientes consecutivos operados entre 1993 y 1997. Método: Revisión de historias clínicas. Resultados: Promedio de edad de 43 años; 5 fueron mujeres. El diagnóstico se basó en el examen físico y fue confirmado por arteriografía en todos los casos. Cinco pacientes fueron embolizados en el preoperatorio. Los 9 tumores fueron completamente resecados. En 3 casos fue necesario resecar el bulbo carotídeo previa colocación de shunt arterial. No hubo mortalidad, se presentaron complicaciones menores en 2 casos. Todas las lesiones fueron benignas y no se registraron recurrencias durante el seguimiento en la presente serie. Conclusión: A pesar de ser una lesión inusual, el TCC puede ser diagnosticado y satisfactoriamente resecado con una mínima morbilidad mientras se observen determinados detalles técnicos (AU)

Experiencia en el tratamiento quirúrgico del tumor del corpúsculo carotídeo / Our experience in surgical treatment of carotid body tumor

Antecedentes: El tumor del corpúsculo carotídeo (TCC) es una lesión infrecuente del área de cabeza y cuello, con características clínicas y quirúrgicas que merecen especial consideración. Objetivos: Describir la metodología diagnóstica, técnica quirúrgica y evolución clínica de una serie de pacientes operados de un TCC. Lugar de aplicación: Servicio de cirugía oncológica. Diseño: Estudio observacional retrospectivo. Población: 9 pacientes consecutivos operados entre 1993 y 1997. Método: Revisión de historias clínicas. Resultados: Promedio de edad de 43 años; 5 fueron mujeres. El diagnóstico se basó en el examen físico y fue confirmado por arteriografía en todos los casos. Cinco pacientes fueron embolizados en el preoperatorio. Los 9 tumores fueron completamente resecados. En 3 casos fue necesario resecar el bulbo carotídeo previa colocación de shunt arterial. No hubo mortalidad, se presentaron complicaciones menores en 2 casos. Todas las lesiones fueron benignas y no se registraron recurrencias durante el seguimiento en la presente serie. Conclusión: A pesar de ser una lesión inusual, el TCC puede ser diagnosticado y satisfactoriamente resecado con una mínima morbilidad mientras se observen determinados detalles técnicos

Primary intrathyroidal paraganglioma with metachronous carotid body tumor: report of a case and review of the literature.

A case of primary intrathyroidal paraganglioma is reported, and the light microscopic and immunohistochemical findings are described. Primary paragangliomas of the thyroid region are extremely uncommon and are therefore often confused clinically and histopathologically with more common intrathyroidal mass lesions. The diagnostic difficulties are underscored by the present case, which was misdiagnosed twice, firstly as a medullary thyroid carcinoma and secondly as a follicular thyroid carcinoma. Immunohistochemistry may be very helpful in arriving at the correct diagnosis. The case was further complicated by a second neck mass contralateral to the original thyroid nodule, which was interpreted as consistent with metastasis. The second lesions was proved angiographically and histologically to be a carotid body paraganglioma.

Characteristics, surgical management, and outcome in 17 carotid body tumors.

Tumors of the carotid body are relatively rare and may pose a difficult surgical problem because of their vascularity and compression of cranial nerves in the neck. This article reviews the physiology of the carotid body, its surgical history, and retrospectively reviews the management and outcome of 17 carotid body tumors occurring in 14 patients over an 18-year period at the University of Louisville Hospitals. The average age at presentation was 54.4 years. Three patients had bilateral tumors. Two patients (12%) had postoperative cranial nerve paralysis lasting greater than 6 months. One patient had a postoperative stroke after discharge from the hospital and subsequently died 2 months later from a pulmonary embolus. One patient had a malignant carotid body tumor and pulmonary metastasis and died 11 years after her original operation during an attempted embolization of recurrent carotid lesion. Early operation for the tumor is indicated to prevent nerve dysfunction due to compression and stretch injury as the lesion increases in size.

Carotid body tumor.

To better define the management and behavior of carotid body tumors, a 34-year surgical experience with 33 tumors in 30 patients was reviewed. There were 20 women and 10 men with an age range of 20 to 78 years. All but one presented with a neck mass. Arteriography was performed on 21 patients and was diagnostic in every case. There was no surgical mortality. Complications occurred in eight patients (27%), and one long-term neurologic deficit occurred. Three of 33 tumors were malignant, and aggressive resection of all approachable disease with radiotherapy for unresectable metastases led to prolonged survival in each case. Carotid body tumors present with neck mass. Arteriography is diagnostic and surgery is indicated. Even for malignant tumors and metastases, aggressive resection and radiotherapy are indicated.

Carotid body tumours: the University of Alberta Hospital experience.

At the University of Alberta Hospital between 1950 and 1988, 17 patients who had a diagnosis of carotid body tumour were seen; 15 of them were followed up for an average of 8 years (range from 1 to 38 years). In 14 patients the tumour was removed surgically. There were no operative deaths and no strokes occurred. The most frequent complication was cranial nerve deficit. Of the 15 patients followed up, 10 (67%) manifested a deficit of the facial, vagus or hypoglossal nerve. The primary tumour was diagnosed histologically as a benign neoplasm in all 14 patients operated on, but in 3 distant metastases developed or there was invasive local recurrence. Patients with malignant tumour were significantly (p less than or equal to 0.01) younger than those with a benign tumour. Carotid body tumours can be managed safely with respect to stroke complications, but cranial nerve injuries continue to be a problem. Malignant tumours are difficult to distinguish from benign tumours except that they tend to occur in younger patients. Prompt surgery and close follow-up is particularly important in patients with carotid body tumour.