DOI: 10.30928/2527-2039e-20212755 _______________________________________________________________________________________Relato de caso CAROTID BODY TUMOR ASSOCIATED WITH PRIMARY HYPERPARATHYROIDISM TUMOR DO CORPO CAROTÍDEO ASSOCIADO COM HIPERPARATIREOIDISMO PRIMÁRIO Duilio Antonio Palacios1; Ledo Massoni1; Climério Pereira do Nascimento1; Marilia D'Elboux Brescia, TCBC-SP1; Sérgio Samir Arap, TCBC-SP1; Fabio Luiz de Menezes Montenegro, TCBC-SP1. ABSTRACT Introduction: Carotid body tumors (CBT) are an uncommon tumor of head and neck. The associa- tion between this entity with primary hyperparathyroidism (PHPT) is even rarer and few cases have been reported. Case Report: We described two cases of association between CBT and PHPT. The first case was a 55-year-old male patient with Shambling type III malignant paraganglioma and PHPT sin- gle adenoma. The second one was a 56-year-old male patient with Shambling type III paraganglioma and double parathyroid adenoma. Conclusion: The adequate preoperative evaluation allowed to iden- tify and treat simultaneously both neoplasms in these patients without compromising the appropriate treatment. Treatment of the two neoplasms when identified could be performed satisfactorily at the same surgical time. Keywords: Carotid Body Tumor. Hyperparathyroidism. Hypercalcemia. Treatment Outcome. RESUMO Introdução: O paraganglioma de corpo carotídeo (PCC) é um dos tumores menos frequente da cabeça e do pescoço. A associação entre essa entidade e o hiperparatireoidismo primário (HPT) é ainda mais rara e poucos casos foram relatados. Relato do Caso: Relatam-se dois novos casos de PCC e HPT. O primeiro é um paciente de 55 anos com um paraganglioma maligno que envolvia as artérias carótidas interna e externa (Shambling III) e um adenoma de paratireoide. O segundo trata-se de paciente mas- culino de 56 anos, também com tumor Shambling III, mas com duplo adenoma de paratireoide. Con- clusão: A avaliação pré-operatória adequada permitiu o tratamento simultâneo na mesma operação. O tratamento das duas neoplasias, quando identificadas, pôde ser realizado de forma satisfatória no mesmo tempo cirúrgico. Palavras-chave: Tumor do Corpo Carotídeo. Hiperparatireoidismo. Hipercalcemia. Resultado do Tra- tamento. INTRODUCTION and both neoplasms were successfully trea- Carotid body tumors (CBTs) are rare ted with the same surgical approach. neoplasms that derive from specialized The Institutional Review Board ap- neural crest cells. They are located at the proved the report in the Ethics Committee carotid artery bifurcation and, generally, under the number 26119519.0.0000.0068. they are slowly growing tumors1. On the other hand, primary hyperparathyroidism CASE REPORT (PHPT) is a common endocrine disorder of CASE 1 calcium metabolism characterized by hy- A 55-year-old male with a previous percalcemia and elevated or inappropriately history of acute myocardial infarction from normal concentrations of parathyroid hor- the year before presented a right cervical mone (PTH)2. The association between these mass that had slowly grown for 6 years. He two entities is very uncommon, and to the complained of recent local pain and dys- best of our knowledge, only five cases have phagia. Computerized tomography (CT been reported so far. In these case reports, scan) showed a carotid paraganglioma in- we describe the presence of this association volving completely both internal and exter- in two patients in whom hyperparathyroi- nal carotid arteries (Shambling type III). dism was diagnosed in preoperative studies During preoperative workup, there was hy- _____________________________________ 1Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo, Head and Neck Surgery, Department of Surgery, Parathyroid Unit - São Paulo - SP - Brasil Relatos Casos Cir. 2021;7(1):e2755 Palacios et al. Carotid body tumor associated with primary hyperparathyroidism _______________________________________________________________________________________Relato de caso percalcemia (total calcium 10.2 mg/dL: ease-free after four years of regular follow– normal range 8.6 to 10.2 mg/dL) that up. The carotid body was nonfunctional, proved to be PTH-dependent (PTH 69 and there has not been evidence of medul- pg/mL: normal range 15 to 65 pg/mL). lary thyroid cancer until now. MIBI parathyroid scintigraphy showed dis- crete anomalous retention of the tracer in the projection of the left upper parathyroid, in agreement with a paraesophageal image observed by a CT scan (Figure 1). Figure 2. (a) The arrowhead indicates the en- larged left upper parathyroid gland. (b) Carotid body paraganglioma resected, (c) reconstruction of the right internal carotid artery. Figure 3. (a) Parathyroid adenoma: homogene- ous cellular proliferation, well-delimited , com- posed of 90% of oxyphylic cells; (b) Paragangli- oma: groups of large and eosinophilic cytoplasm cells, with nuclear atypia among antique hem- orrhage and fibroesclerosis compatible with chemical embolization changes; (c) Lymph node metastasis of paraganglioma. Staining: Hema- toxylin-Eosin. Figure 1. Axial (a) and coronal (b) images in CT CASE 2 scan. The arrowheads indicate the paragangli- 56-year-old male presented a pulsa- oma wrapping around the internal and external tile mass of 3×4 cm in the carotid triangle carotid arteries. (c) The arrowhead indicates the of his left neck. It had grown slowly over the enlarged upper parathyroid gland. previous 5 years, with the diagnosis of a nonfunctional carotid body paraganglioma. These preoperative findings suggest- The CBT was considered as Shambling type ed that parathyroid resection would not III, as an internal carotid artery was com- add significantly and costly time before CBT pletely covered by the mass (Figure 4). Hy- resection in spite of an asymptomatic percalcemia (Ca total 11.6 mg/dL: normal PHPT. The patient underwent preoperative 8.6 to 10.2 mg/dL) was detected during embolization. The operation started with hospitalization for preoperative emboliza- the excision of a left upper parathyroid ad- tion of the CBT. A CT scan review allowed enoma, measuring 14×10×7 mm. While the us to suspect a concomitant increase size intraoperative PTH was running, the CBT in the right superior parathyroid gland. We resection was started. A successful decay had no time to do routine PTH sampling. occurred (from the pre-excision value of However, conducted sampling intraopera- 164 to 42 pg/mL 10 minutes after removal tive, and the result was 346 pg/mL. After of the parathyroid). Paraganglioma excision an uneventful resection of the paragangli- required resection of the carotid bifurcation oma, we performed a bilateral exploration of and reconstruction of the right internal ca- the parathyroid glands, finding an enlarged rotid artery with a saphenous graft (Figure right upper parathyroid (19×11×0.5 mm) 2). The pathological examination revealed and left upper parathyroid (12×08×03 mm) paraganglioma metastases in two lymph (Figure 5). The other glands were normal. nodes (Figure 3). The patient received no Ten minutes after resection of these two complementary treatment, and he is dis- parathyroid glands, PTH levels dropped to Relatos Casos Cir. 2021;7(1):e2755 Palacios et al. Carotid body tumor associated with primary hyperparathyroidism _______________________________________________________________________________________Relato de caso 28 pg/ml, with subsequent normocalcemia. our perspective, this speculation is not Pathological examination confirmed the proper because the parathyroid glands are diagnosis of a benign paraganglioma and derived from the endoderm. Our present double parathyroid adenoma. Retrospec- cases showed no evidence of multiple endo- tively, a more careful evaluation showed crine neoplasms. Given that primary hy- that the patient had complained of repeti- perparathyroidism is common in the popu- tive episodes of nephrolithiasis in the past. lation, a fortuitous association may have After one year of irregular follow-up, the occurred in the present report. In our case, patient stopped attending the outpatient however, we were able to identify the pres- clinic. Furthermore, he refused bone densi- ence of hyperparathyroidism in both pa- tometry. When he last followed up, he had tients who were initially consulted for neck a normal level 9.3 mg/dL without a calcium masses that were compatible with carotid supplement and his PTH was 54 pg/ml. paragangliomas. Preoperative calcium sam- pling led us to suspect of hyperparathyroid- ism. This aspect can be emphasized in Case 2, wherein hypercalcemia was detect- ed during admission for embolization. This prompted us to review the CT scan. It was not possible to perform MIBI scintigraphy before the operation. Intraoperative PTH dosage was very helpful to detect double Figure 4. (a) Axial and (b) sagittal images in CT parathyroid adenoma. scan suggestive of a Shambling III carotid body In Case 1, as hypercalcemia was de- paraganglioma (arrowhead); (c) axial view sug- tected at the office, a MIBI scan was added gestive of an enlarged right-side parathyroid (c) preoperatively. Both patients benefited from (arrowhead). surgical treatment in a single surgical ap- proach. Parathyroid resection did not sig- nificantly increase the operative time, and the Case 2 patient was clearly symptomat- ic. Notably, Case 1 demonstrates cardiovas- cular effects of primary hyperparathyroid- ism which is an area of research interest. In Case 1, as the hypercalcemia was detected at the office, a MIBI scan was