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Pheochromocytomas are rare catecholamine-secreting neuroendocrine tumors that originate from chromaffin cells in the adrenal medulla. They cause severe hypertension and various clinical manifestations. While most arise in the adrenal medulla, some occur in extra-adrenal locations.We present a case report with clinical, laboratory and radiographic data, along with a brief literature review. We report a 33-year-old woman who presented with flushing and sharp lower abdominal pain. Abdominal ultrasound and urinary metanephrines suggested a pheochromocytoma. Subsequent Computed tomography (CT) and Ga-DOTATATE Positron emission tomography/Computed tomography (PET/CT) scans confirmed a pheochromocytoma in the organ of Zuckerkandl with distant bony metastasis. Extra-adrenal pheochromocytomas, or paragangliomas, are rare tumors found in specific anatomical locations. Their diagnostic challenges stem from variable clinical presentations and imaging findings. CT scans and Ga-DOTATATE PET/CT scans are crucial in diagnosis and prognosis. Surgical resection can cure localized cases, while metastatic disease requires palliative options, such as chemotherapy and I131-MIBG, due to a poorer prognosis.

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BACKGROUND: To evaluate the safety and efficacy of US-guided microwave ablation in patients with thyroid nodules at Zuckerkandl tubercle. METHODS: 103 consecutive patients with thyroid nodules at Zuckerkandl tubercle (ZTTN) were enrolled in this study from November 2017 to August 2021. Prior to the surgery or US-guided microwave ablation (MWA), preoperative ultrasound visualization of the recurrent laryngeal nerve (RLN) and ZTTN was performed, the size and the position relationship between them were observed. Patients were followed up at 1, 3, 6, and 12 months after MWA and the volume reduction rates (VRR) of the thyroid nodules were analyzed. RESULTS: All patients successfully had the RLN and ZTTN detected using ultrasound before surgery or ablation with a detection rate of 100%. For the 103 patients, the majority of ZTTN grades were categorized as grade 2, with the distance from the farthest outside of ZTTN to the outer edge of thyroid ranging between 6.0 and 10.0 mm. The position relationship between ZTTN and RLN was predominantly type A in 98 cases, with type D observed in 5 cases. After MWA, the median nodule volume had significantly decreased from 4.61 (2.34, 8.70) ml to 0.42 (0.15, 1.41) ml and the VRR achieved 84.36 ± 13.87% at 12 months. No nodules regrew throughout the 12-month follow-up period. Of the 11 patients experienced hoarseness due to RLN entrapment before ablation, 7 recovered immediately after separation of the RLN and ZTTN during MWA, 2 recovered after one week, and the other 2 recovered after two months. CONCLUSIONS: The RLN is closely related to ZTTN and mainly located at the back of ZTTN. The RLN can be separated from ZTTN by hydrodissection during MWA. US-guided MWA is a safe and effective treatment for ZTTN.

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Objective: To determine the incidence and characteristics of Zuckerkandl's tubercle (ZT), and its relationship with recurrent laryngeal nerve (RLN) and the superior parathyroid gland (SPG) in the setting of total thyroidectomy. Methods: A total of 421 patients (mean (min-max) age: 45.6 (18-78) years, 76.2% were females) who had total thyroidectomy were included in this prospective single-surgeon thyroidectomy series study. Patient demographics and thyroidectomy indications (benign and malignant) were recorded in each patient. The presence, grade and laterality of ZT, and its relationship with RLN and SPG were recorded during surgery. Results: Most of the thyroidectomy indications (69.1%) were related to a malignant disease. The ZT was unrecognizable in 41(9.7%) of 421 patients. In 380 patients with identifiable ZT, the grade 2 (46.3%) ZT was the most common finding. Majority of ZTs (92.9%) were unilaterally located (right-sided: 64.9%; left-sided: 35.1%). In majority of the cases (83.2%), the RLN was found to lie medial to ZT. Overall, SPG was identified in close proximity to ZT in 66.6% of patients (Class 2 [0.5-1 cm from ZT] in 46.6% and Class 3 [<0.5 cm from ZT] in 20.0%). SPG was more likely to be identified in close proximity to ZT when the grade of ZT was higher, which was found to be located 0.5-1 cm from the ZT in 56.9% and 42.7% of grade 2 and grade 3 ZTs, respectively, and <0.5 cm from the ZT in 46.1% of grade 3 ZTs. Conclusion: In conclusion, this prospective single-surgeon thyroidectomy series study indicates the likelihood of localizing the RLN medial to ZT, and the SPG in close proximity to ZT during total thyroidectomy operations. Hence, the ZT can be used as a reliable and constant landmark to localize both the RLN and the SPG during thyroid surgery, which enables minimizing the risk of iatrogenic injury to RLN, while ensuring a parathyroid-sparing thyroidectomy. The thyroid surgeon should have complete knowledge of thyroid gland anatomy and embryogenesis and should follow a careful and meticulous approach particularly for dissections around larger ZTs, given the increased likelihood of SPG and RLN to be in close proximity.

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Zuckerkandl tubercles are posteromedial projections of normal thyroid gland with relations with important neck structures. In our case, we reported "Thyroid ring", that is a ring configuration of the thyroid gland encasing the trachea and esophagus, without compression symptoms. Ring was formed by the elongated zuckerkandl tubercles, passing through the column between the esophagus and vertebral body and abutting each other at left posterolateral border of esophagus. Reporting of these relations and variations is important as it helps the operating surgeon in planning the approach to the procedure. Supplementary Information: The online version contains supplementary material available at 10.1007/s12070-023-04079-4.

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Hypertension in young patients can mask rare conditions like paragangliomas, especially in the absence of conventional symptoms. A comprehensive diagnostic evaluation and multidisciplinary approach are crucial for optimal management and outcomes.

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One of the eponyms most frequently cited in dental morphology texts, together with the Carabelli tubercle of the first permanent maxillary molars, is the Zuckerkandl tubercle of deciduous molars. However, references about Emil Zuckerkandl in the field of dental history and this particular entity are scarce. The reason this dental eponym was pushed "into the shadows" probably lies in the many other anatomical parts (including another tubercle, the pyramidal one of the thyroids), which took their names from this great anatomist.

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Pheochromocytomas (PHEOs) and paragangliomas are generally grouped as rare chromaffin cell tumors. The co-occurrence of PHEOs and paragangliomas of the organ of Zuckerkandl (POZ) is extremely rare. The most common symptom of pheochromocytoma-paraganglioma (PPGL) is hypertension, and open surgery is still recommended for the treatment of large PPGLs. Herein, we report a case of a successful simultaneous laparoscopic resection of a large PHEO accompanied by POZ in a 40-year-old man with normal blood pressure. DNA analysis revealed a mutation in the succinate dehydrogenase subunit B in both the PHEO and the POZ. To the best of our knowledge, this is the first report of tumors occurring simultaneously in these two locations. We believe that the co-occurrence of PHEO and POZ is extremely rare, and the possibility of PPGL cannot be ruled out in patients with normal blood pressure. The decision to perform laparoscopic surgery remains questionable for patients with a large PHEO and POZ. In addition, a genetic examination should be performed to identify the existence of PPGL-related inherited syndromes.

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During neck surgery; Zuckerkandl's tubercle, Berry's ligament, the inferior horn of thyroid cartilages have become crucial anatomical landmarks in order to protect the integrity of the recurrent laryngeal nerve. Forty-two male postmortem human cadavers were used. The proximal part of the recurrent laryngeal nerve, before the inferior thyroid artery arises from its source has been observed in 87% inside the tracheoesophageal groove and in 13% running laterally to the trachea. The recurrent laryngeal nerve was encountered passing behind and through the branches of the inferior thyroid artery in 92% and 8% respectively. At all sides; the nerve was piercing the larynx 0.6 ± 0.1 mm below the inferior horn of thyroid cartilage, passing next to the inner-lower side of Berry's ligament and running under the lower middle part of Zuckerkandl's tubercle. These landmarks and their upper mentioned distances to the laryngeal nerve can be taken into consideration as important surgical guides.

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Thyroid surgeons should be able to identify factors that prevent parathyroid damage. The aim of the study was (i) to compare the effectiveness of using Zuckerkandl's Tubercle (ZT) versus superior thyroid artery (STA) and inferior parathyroid artery (ITA) as markers for identification of superior and inferior parathyroid glands and (ii) to demonstrate a series of detailed, logical and orderly operative steps to identify ZT during thyroidectomy operation. This 1-year prospective observational study was carried out in the Department of Otolaryngology in a tertiary medical institute. Out of 36 cases of thyroidectomy, parathyroid identification in Group A was based on STA and ITA and in Group B was based on ZT. The surgical steps, parathyroid location, preservation and its anatomical relations were noted. The mean age in Group A and Group B was 38.8 years and 44.9 years respectively with 77.4 and 62.5% SPT identified above the intersection of RLN and ITA respectively. On left side 62.5% SPT were  located at 2 o'clock position and 50% at 10 o'clock location in right side. In Group A, 60.7% of IPT glands were related close to ITA while in Group B it was 44.4%. Group B reported a higher rate of successful identification and preservation (93.75%). ZT greatly improved the reliability for localising and preserving the parathyroid glands during thyroidectomy. SPT is usually found to lie cranial to ZT, above the intersection of RLN & ITA and behind RLN whereas IPT is variable and lies below the intersection.

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PURPOSE: This study investigated the incidence of thyroid Zuckerkandl Tubercle (ZT) using a computed tomography (CT) scan of the neck with contrast to identify the typologies in ZT-positive CT scans, investigate the presence of nodules located in that area, and compare the results with the relevant literature. METHODS: We selected and retrospectively examined the CT scan images of patients who presented to our clinic with CT scans of the neck with contrast that clearly showed thyroid tissue and boundaries. Patients were evaluated based on age, gender, and presence of thyroid ZT. Patients with thyroid ZT were further investigated for localization and type of tubercles as well as for presence of nodules at the tubercle level. RESULTS: A total of 1000 patients (mean age: 48.4 ± 19.1) were included in the study. Out of the total 222 (22.2%) patients who had thyroid ZT, 134 (60.4%) patients had unilateral thyroid ZT on the right side and 29 (13.1%) patients had unilateral thyroid ZT on the left side; 59 (26.6%) patients had bilateral thyroid ZT. In addition, nodules at the ZT level were observed in 13 (1.3%) of the patients. A review of all cases with ZTs indicated that 63% were Type 1, 31% were Type 2, and 5% were Type 3. CONCLUSION: During the thyroid operations, ZT is essential for locating the recurrent laryngeal nerve. Reporting the presence of ZTs based on CT scans is crucial because it can prevent unnecessary interventional procedures, misdiagnoses, and likely complications in patients with planned thyroid operations.

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Purpose: (i) To examine the criterion taller-than-wide (TTW) for the sonographic assessment of thyroid nodules in areas of iodine deficiency in terms of frequency, anatomical distribution within the thyroid gland and risk of malignancy. (ii) To develop a model for nodule growth in the thyroid gland. Methods: German multicenter study consisting of two parts. In the prospective part, thyroid nodules were sonographically measured in all three dimensions, location within the thyroid gland and contact to a protrusion-like formation (horn) in the dorsal position of thyroid gland was noted. In addition, further sonographic features such as the composition, echogenity, margins and calcifications were investigated. All nodules from the prospective part were assessed for malignancy as part of clinical routine at the decision of the treating physician adhering to institutionally based algorithms. In the retrospective part, only nodules with fine needle aspiration and/or histology were included. The risk of malignancy in TTW nodules was determined by correlating them with cyotological and histological results. Results: Prospective part: out of 441 consecutively evaluated thyroid nodules, 6 were found to be malignant (1.4%, 95% CI 0.6-2.7%). Among the 74 TTW nodules (17%), 1 was malignant (1%, 95% CI 0-4%). TTW nodules were more often located in the dorsal half of the thyroid than non-TTW nodules (factor 2.3, p = 0.01, 95% CI 2.1-2.5) and more often located in close proximity to a horn than non-TTW nodules (factor 3.0, p = 0.01, 95% CI 2.4-3.8). Retrospective part: out of 1315 histologically and/or cytologically confirmed thyroid nodules, 163 TTW nodules were retrieved and retrospectively analyzed. A TTW nodule was 1.7 times more often benign when it was dorsal (95% CI 1.1-2.5) and 2.5 times more often benign when it was associated with a horn (95% CI 1.2-5.3). The overall probability of malignancy for TTW nodules was 38% (95% CI 30-46%) in this highly preselected patient group. Conclusion: TTW nodules are common in iodine deficient areas. They are often located in the dorsal half of the thyroid gland and are frequently associated with a dorsal protrusion-like formation (horn) of the thyroid. Obviously, the shape of benign nodules follows distinct anatomical preconditions within the thyroid gland. The frequency of TTW nodules and their predominant benignity can be explained by a pole concept of goiter growth. The difference between the low malignancy risk of TTW nodules found on a prospective basis and the high risk found retrospectively may be the result of a positive preselection in the latter.

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The low incidence of injury to the recurrent laryngeal nerve (RLN) and external branch of the superior laryngeal nerve (EBSLN) quoted in the literature is derived from expert series. The exact incidence of nerve injury of a thyroid surgeon will be revealed only if pre-operative and post-operative laryngoscopy is becoming routine practice. It is found that the injury rates are increased with routine post-operative laryngoscopy. Subjective voice change occurred in one third of patients all whom had normal vocal cord motion. Therefore, it is important to take written informed consent for voice change in addition to identification of both nerves and documenting it.

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The primary objective of the study was to assess the Tubercle of zuckerkandl (TZ) during thyroid surgeries and its relationship with RLN and Superior parathyroid (SP). A prospective study was done in, 30 consecutive cases of total thyroidectomy in whom per operatively TZ was identified. The presence of TZ, its laterality, size, relationship with RLN and parathyroid glands were documented. A grading system outlined by Pelizzo was applied in our current study. In majority of the cases the RLN was found to lie medial to TZ (26/30), followed by lateral position (3/30) and one case it was found to be posterior to TZ (1/30). The superior parathyroid was identified in close relation (< 2 cm) to the TZ in 27/30 cases. The distance between the TZ and SP was assessed. We proposed a classification for location of SP based on the distance between SP and TZ and also attempted to relate each class of SP location with TZ grade. There was strong association of Grade of TZ with the class of SP location (p value = 0.00046). TZ is constant surgical landmark with good reliability to identify the RLN and SP. RLN is found medial to TZ in majority of cases with few exceptions. SP is found to be closely associated with TZ in majority of cases and there is a strong relationship of proximity of SP and TZ with respect to TZ grade. Although this required further studies with larger population.

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Embryologic developmental variants of the thyroid and parathyroid glands may cause cervical anomalies that are detectable in ultrasound examinations of the neck. For some of these developmental variants, molecular genetic factors have been identified. Ultrasound, as the first-line imaging procedure, has proven useful in detecting clinically relevant anatomic variants. The aim of this article was to systematically summarize the ultrasound characteristics of developmental variants of the thyroid and parathyroid glands as well as ectopic thymus and neck cysts. Quantitative measures were developed based on our findings and the respective literature. Developmental anomalies frequently manifest as cysts that can be detected by cervical ultrasound examinations. Median neck cysts are the most common congenital cervical cystic lesions, with a reported prevalence of 7% in the general population. Besides cystic malformations, developmental anomalies may appear as ectopic or dystopic tissue. Ectopic thyroid tissue is observed in the midline of the neck in most patients and has a prevalence of 1/100,000 to 1/300,000. Lingual thyroid accounts for 90% of cases of ectopic thyroid tissue. Zuckerkandl tubercles (ZTs) have been detected in 55% of all thyroid lobes. Prominent ZTs are frequently observed in thyroid lobes affected by autoimmune thyroiditis compared with normal lobes or nodular lobes (P = 0.006). The correct interpretation of the ultrasound characteristics of these variants is essential to establish the clinical diagnosis. In the preoperative assessment, the identification of these cervical anomalies via ultrasound examination is indispensable.

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OBJECTIVES: Pheochromocytomas are rare catecholamine-secreting neuroendocrine tumors that arise from chromaffin cells of the adrenal medulla or extra-adrenal paraganglia. The most common location of these tumors is within the adrenal medulla. Extra-adrenal pheochromocytomas (EAPs) may occur in any portion of the paraganglion system. The most common location of EAPs is at the organ of Zuckerkandl, which is a collection of chromaffin cells near the origin of the inferior mesenteric artery. METHODS: We present a case of an EAP of the organ of Zuckerkandl incidentally discovered and resected during urgent open repair of a symptomatic 6.7-cm juxtarenal abdominal aortic aneurysm (AAA). RESULTS: The patient underwent successful open surgical repair of a juxtarenal AAA and resection of the pheochromocytoma. CONCLUSIONS: Concomitant pheochromocytomas and abdominal aortic aneurysms are rare, with a small number described in the literature. We describe the case of a simultaneous EAP of the organ of Zuckerkandl and AAA repair. This case demonstrates that these lesions can be safely resected in the same setting as AAA repair.

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Paragangliomas are uncommon neuroendocrine neoplasms that occur in characteristic locations. While parasympathetic paragangliomas are mainly located at the head and neck, sympathetic paragangliomas are mostly located below the neck. Among parasympathetic paragangliomas, pheochromocytomas are the most common. Ninety percent of cases of pheochromocytomas arise within the adrenal gland. We report a case of a 63-year-old woman with an extra-adrenal pheochromocytoma of the organ of Zuckerkandl detected by CT and MRI and subsequently confirmed by postoperative histology and immunohistochemistry.

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Background: Paraganglioma of the organ of Zuckerkandl (OZ) is a rare surgically challenging tumor because of its critical location and the nature of catecholamine secretion. We describe the technique of laparoscopic excision as well as provide a literature review to confirm its feasibility. Case Presentation: In a 23-year-old male patient, laparoscopic excision of a 5 × 4 cm tumor located at the aortic bifurcation and indenting the vertebral column was performed. Preoperatively, the patient received α- and ß-adrenergic blockers as well as underwent sperm banking. The patient was put in the lateral position, five ports were used: four in the midline and one in the left iliac fossa. The tumor was approached by the reflection of the colon. Ureter, gonadal vein, and sympathetic chain were preserved. Dissection of the tumor from the inferior mesenteric artery was done followed by control of three feeding arteries and two draining veins posteriorly and inferiorly. The procedure was completed laparoscopically with minimal blood loss. Intraoperatively, three episodes of hypertension developed and required stoppage and the administration of vasodilators. The patient recovered on the second day postoperatively and all antihypertensive medications were stopped. At 1 year of follow-up, the patient is tumor-free but developed anejaculation for which he is under current treatment. Conclusion: Laparoscopic excision of paraganglioma located at the OZ is safe and feasible.

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Not required for Clinical Vignette.

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BACKGROUND: Large paraganglioma of the Zuckerkandl organ (POZ) is extremely rare. The patient can occasionally be paucisymptomatic, further obscuring the diagnosis and carrying high mortality. Recommended treatment for large paraganglioma (PGL) is open surgical removal. We report a case of successful laparoscopic resection of a large POZ with normal blood pressure in a 45-year-old man. CASE PRESENTATION: A 45-year-old man was hospitalized because of hyperglycemia. Computed tomography of the abdomen and the serum and urinary catecholamine levels confirmed the diagnosis of large POZ. But his blood pressure was normal and he underwent laparoscopic tumor excision successfully. During 6 months follow-up after laparoscopy, serum and urinary catecholamines were normal but glycaemia remained high level. DNA analysis of the succinate dehydrogenase complex subunits B (SDHB) and SDHD revealed no mutation. CONCLUSIONS: POZ is an unusual mass and preoperative diagnosis can be difficult in clinically silent cases. PGL cannot be excluded in patients with normal blood pressure. Even a large POZ can be excised laparoscopically by following proper techniques.

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El estudio de las venas tiroideas no ha recibido una investigación tan exhaustiva como lo tuvieron las arterias tiroideas y los nervios laríngeos en relación a la cirugía tiroidea.De los tres pedículos venosos de la glándula tiroides, el medio, de lejos es el menos estudiado. La vena tiroidea media es inconstante y es el primer elemento vascular de la glándula tiroides que debe ser seccionado antes de luxar el lóbulo hacia medial para evaluar sus relaciones posteriores. Su lesión puede provocar sangrado intraoperatorio dificultando secundariamente la identificación del nervio laríngeo inferior y las glándulas paratiroides, próximas a la misma. Se realizó un estudio descriptivo de corte transversal evaluando la pesencia, número, simetría y asociación de la vena tiroidea media con variables tales como edad y sexo del paciente, así como la hiperfuncionalidad de la glándula tiroides y la presencia del tubérculo de Zuckerkandl en 100 tiroidectomías totales llevadas a cabo en el Instituto Nacional del Cáncer y en el Servicio de Otorrinolaringología del Hospital Central del Instituto de Previsión Social del Paraguay. La prevalencia global de vena tiroidea media fue del 74 %. En el lóbulo derecho, la vena se presentó en el 60 %, mientras que en el lóbulo izquierdo en el 53 %. En el 38 % se encontró la vena en ambos lóbulos. En 4 pacientes se localizaron venas tiroideas medias dobles, en una de ellas fue bilateral. El mayor porcentaje de las venas tiroideas medias se originó en el tercio medio del lóbulo, el 72 % en el lado derecho y el 70% en el izquierdo. No se encontró asociación entre la presencia de la vena tiroidea media y la edad, sexo, estado de hiperfunción glandular, así como tampoco con la presencia del tubérculo de Zuckerkandl.

The study of the thyroid veins has not received an investigation as extensive as the thyroid arteries and laryngeal nerves did in relation to thyroid surgery. Of the three veins pedicles of the gland the middle is far the least studied. This vein is inconstant and is the first vascular element of the gland that must be sectioned before the medial lobe is dislocated to evaluate ist posterior relationships. His injury can cause intraoperative bleeding, making it difficult to identify the inferior laryngeal nerve and the parathyroid glands, proximal to it. A descriptive crossseccional study was carried aot evaluating the presence, number, symmetricity and association of the middle thyroid vein with variables such as age and sex of the patient, as well as the hyperfunctionality of the gland and the presence of the Zuckerkandl tubercle in 100 total thyroidectomies undergoing at the National Cancer Institute and the ENT Service of the Social Security Institute´s Central Hospital. The overall prevalence of the middle thyroid vein was 74%. In the right lobe the vein appeared in 60% while in the left lobe in 53 %. Double middle thyroid vein was found in 4 patients, in one of them it was bilateral. The highest percentage of the middle thyroid veins originated in the middle third of the lobe, 72 % on the right and 70% on the left side. No association was found between the presence of the vain and age and sex, the state of glandular hyperfunstion, as well as the presence of Zuckerkandl tubercle.

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