Histopathology Image of Carotid Body Tumour:

Paragangliomas are neuroendocrine tumors derived from the extra-adrenal paraganglia of the autonomic nervous system. Within the head and neck, they are generally defined and named according to their site of origin, and may be found frequently near vascular structures.

These tumors generally exhibit a slow rate of growth, most often presenting asymptomatically as a space occupying mass lesion noted clinically or radiographically. Visit: Extraadrenal Paraganglioma

The most common paraganglioma of the head and neck is the carotid body tumor followed by the jugulo-tympanic and vagal varieties.

Carotid body tumours (CBT) are a rare entity that should be considered in evaluating every lateral neck mass. The tumour is classically located in  the carotid bifurcation, between internal and external carotid arteries.

The carotid body tumour was first described by von Haller in 1743. The first two,unsuccessfully surgically treated carotid body tumours, were done by Reinger in 1880 (his patient died), and by Maydel in 1886 (his patient developed hemiplegia). Scudder made the first successful surgical removal of the carotid body tumour in 1903.

The clinical course of carotid body paragangliomas is characterized by two modes of development: locoregional growth and distant metastatic dissemination. Few cases of malignant carotid body paraganglioma have been reported in the literature.

Malignant forms of carotid paraganglioma are very uncommon and there is no cellular or histological appearance significantly contributing to the diagnosis of malignancy. Clinical findings are the most reliable criteria for malignancy, with the development of distant metastases usually after about 9 years. Long follow-up is essential. Histology in needed to establish the diagnosis of metastasis.

Some patients have von Hippel-Lindau syndrome, an autosomal dominant condition which includes cerebellar hemangioblastoma, renal cell carcinoma and paragangliomas amidst its features.

We present a case of synchronous bilateral carotid body tumor and vagal paraganglioma in a 49-year-old man who presented with 3 months history of tenderness and palpable neck masses bilaterally. An encapsulated mass which was thought to be a carotid body tumor and an incidental 3cmx2cm, 5cmx1cm, 5cm mass which seemed to be originated from vagus nerve were dissected from the left side. Pathology revealed carotid body tumor and vagal paraganglioma. Six months after the first operation, the carotid body tumor on the right side was totally excised. A discussion of this case is followed by a review of the literature surrounding this rare clinic and pathological entity.

Carotid body tumors: review of a 20-year experience.Oral Oncol. 2005 Jan;41(1):56-61.

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 carotid paraganglioma. A case report. Ann Otolaryngol Chir Cervicofac . 2003 Apr;120(2):103-8.

The most common sites of paragangliomas occurring in the head and neck region are the carotid body and vagal glomus. The clinical course of carotid body paragangliomas is characterized by two modes of development: locoregional growth and distant metastatic dissemination. Few cases of malignant carotid body paraganglioma have been reported in the literature. We report a case managed in the ENT Department of Grenoble university hospital in 1993. A 53-year-old woman presented a bilateral functional (dopamine secretion) carotid tumor, stage II on the right side and stage I on the left side according to the Shamblin classification. Two successive excisions with complete resection were performed without complication. Two years after the second surgical excision, bone and liver metastases were diagnosed and visualized with a Ostreoscan scintigraphy. Clinical course was rapidly unfavorable and the patient died a few weeks later. Malignant forms of carotid paraganglioma are very uncommon and there is no cellular or histological appearance significantly contributing to the diagnosis of malignancy. Clinical findings are the most reliable criteria for malignancy, with the development of distant metastases usually after about 9 years. Long follow-up is essential. Histology in needed to establish the diagnosis of metastasis. A histology specimen may not be possible for a functional paraganglioma. In this case scintigraphy can provide the diagnosis. Malignant forms are more frequent in case of functional paraganglioma, more particularly in case of dopamine secretion.

Cytologic findings in carotid body tumors. Acta Cytol. 2002 Nov-Dec;46 (6): 1101-4.

OBJECTIVE: To describe the cytologic findings in 13 cases of carotid body tumors (CBTs) and discuss the differential diagnoses. STUDY DESIGN: Cytologic smears were obtained from 13 cases of carotid body tumors by fine needle aspiration. All tumors were clinically suspected and later confirmed by arteriography or color Doppler ultrasonography. The cytomorphologic findings are described. RESULTS: The smears revealed hypercellularity, a bloody background, isolated and clusters of round to oval cells with indistinct cytoplasm, reticular chromatin, no prominent nucleoli, giant bare nuclei, anisocytosis, acinar structures and plasmacytoid cells. These findings suggested metastatic tumors. CONCLUSION: The cytologic diagnosis of CBTs is very difficult. The aspiration findings can be easily mistaken for those of metastatic tumors. However, when used with clinical and color Doppler findings, fine needle aspiration plays an important role in the preoperative diagnosis and management of CBTs.

Management of carotid body paragangliomas and review of a 30-year experience. Ann Vasc Surg. 2002 May;16(3):331-8.

Carotid body tumors are rare neoplasms and must be considered in the evaluation of all lateral neck masses; early surgical removal has been recommended. In this study, the medical records of 29 patients with 34 carotid body paragangliomas who were treated at our institution between 1971 and 2001 were retrospectively reviewed. An overview is provided of this lesion, including diagnosis, classification, metastatic potential, possible secretory function, operative techniques, and nonsurgical methods of management. Carotid body tumors may be familial and are more often bilateral in these instances; five patients (17%) had bilateral tumors in this series. The criterion for malignancy is demonstrated by metastatic tumor in lymph nodes or distant organs. Three patients (10%) had malignant tumors, one with hepatic metastases. One patient (3%) in our series exhibited abnormal serotonin production. Vascular reconstruction was necessary in eight cases (28%). No stroke occurred, however, two arterial thromboses (7%), five permanent cranial nerve deficits (17%), and one death (3%) from massive pulmonary embolism were seen. Our experience demonstrates that early operative management is warranted to avoid the possibility of eventual metastasis and progressive local invasion to the point of inoperability.