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Laparoscopic adrenalectomy for pheochromocytoma
Endocrine Laparoscopic Adrenalectomy for Pheochromocytoma Guest Reviewers: Jamie C. Mitchell, MD, and Sareh Parangi, MD, Department of Surgery, Harvard University, Beth Israel Deaconess Medical Center, Boston, Massachusetts LAPAROSCOPIC ADRENALECTOMY FOR PHEOCHROMOCYTOMA. Cheah KW, Clark OH, Horn JK, Siperstein AE, Duh QY. World J Surg. 2002;26:1048-1051. Objective: To evaluate the safety and effectiveness of laparo-
scopic adrenalectomy for patients with pheochromocytomas. Design: Retrospective case review. Setting: University of California-San Fransisco (UCSF). Participants: A total of 39 consecutive patients who underwent laparoscopic adrenalectomy for pheochromocytoma from 1993 to 2000 at UCSF. Results: The study group consisted of 39 patients (22M, 17F) with a mean age of 48 years (range, 20 to 84). Overall, 35 patients had unilateral tumors and 4 had bilateral tumors. A total of 43 adrenalectomies were performed. Three groups were identified based on presentation, 17 patients with classic symptoms (group A), 17 patients whose tumors were discovered incidentally during imaging (computed tomography or ultrasound) for other symptoms (group B), and 5 patients who presented with hypertensive crises and multiorgan failure requiring prolonged intensive care unit stay (group C). All patients screened for pheochromocytoma had increased urinary metanephrine and catecholamine levels. Preoperative imaging with either computed tomography (CT) or magnetic resonance imaging (MRI), or both, was performed on all patients with 100% accuracy. A total of 34 patients (87%) had sporadic lesions, 5 patients had familial tumors (2 Multiple Endocrine Neoplasia
II, 1 each neurofibromatosis type I, von-Hippel- Lindau, and Osler-Weber-Renu). Patients were treated preoperatively with phenoxybenzamine and aggressive hydration. Laparoscopic adrenalectomy was performed via a lateral transabdominal approach in 31 patients and via a posterior retroperitoneal approach in 4 patients. One patient with a large tumor greater than 12 cm had a hand-assisted resection. No cases required conversion to an open approach. Operative time averaged 217 minutes for unilateral tumors and 341 minutes for bilateral tumors. Blood loss averaged 160 cc. Postoperative hospital stay averaged 1.5 days for elective and 3.0 days for patients presenting with acute hypertensive crises. Complications occurred in 3 patients (8%), 2 patients required blood transfusions, and 1 patient developed postoperative heart failure requiring pressor support. There were no operative mortalities. Conclusions: Patients with pheochromocytomas can present in several different ways, ranging from those with minimal or no symptoms to those with life-threatening hypertensive crises and multiorgan system failure. Because of increased use of imaging studies in medicine today, many of these tumors now present as incidentalomas, which should be evaluated with appropriate screening urinary tests. Percutaneous fine-needle aspiration biopsy should not be performed, as it is unnecessary and potentially hazardous. Laparoscopic adrenalectomy can be performed safely and effectively for pheochromocytomas with adequate preoperative preparation with alpha blockade and hydration. The laparoscopic approach results in less blood loss, fewer complications, and shorter postoperative stays compared with open surgery and should be the standard of care for benign tumors.
REVIEWER COMMENTS
This article is one of several in the literature of a surgical center reporting their experience with the use of laparoscopic adrenalectomy (LA) for the resection of pheochromocytomas. Although these series tend to be fairly small because of the relative rarity of these tumors, this retrospective study of 39 patients represents one of the largest individual series of patients reported. The authors have shown, similar to other reported series, that laparoscopic adrenalectomy for pheochromocytomas can be performed safely and effectively in experienced hands, with less blood loss, less postoperative pain, shorter hospital stay, and less overall complications than with open adrenalectomy. This series documents, as do the majority of series in the
literature, that operative times for LA are significantly longer than for open adrenalectomy. Some of this is because of the nature of laparoscopic surgery, but surgeon experience and the “learning curve” also play a role. As surgeons gain more experience with this procedure, this time difference is likely going to become less significant. One critique of this study is the relatively small number of patients with familial pheochromocytoma syndromes; however, the patient population presented herein is likely to be representative of the patients seen by most general surgeons, including those with fairly large endocrine surgery experiences. The authors identified three groups of patients according to
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their presentation, and they stress the fact that because of the increased use of advanced imaging studies in general medical practice, more patients are presenting with incidentally discovered adrenal masses. These patients, after a thorough history and physical, should all undergo routing biochemical screening to rule out a functional adrenal tumor. The authors also note that nearly half of the patients referred for an incidentaloma had undergone inappropriate FNA biopsy before any biochemical screening, which should be avoided because needle aspiration of a pheochromocytoma can result in hypertensive crisis and death.1 While not specifically addressed in this paper, the much debated question of the upper limit of tumor size when contemplating safe laparoscopic adrenalectomy is indirectly ad-
dressed since the authors point out that resection of larger tumors was more difficult, with tumors greater than 8 cm taking 2.5 times longer than smaller tumors. Malignancy is difficult to establish in these tumors preoperatively. The risk for malignancy is thought to increase with tumor size greater than 6 cm, and is very common in tumors larger than 10 cm 2 However, no size limit has been established in the literature for safe use of a laparoscopic approach. Although size alone is not a contraindication for a safe laparoscopic approach, surgeons must use caution in planning a laparoscopic approach when tumor size is between 6 and 10 cm. Imaging studies should be carefully reviewed, and laparoscopic surgery should be avoided if other parameters such as lymphadenopathy, local, or vascular invasion are present.
ADRENALECTOMY FOR FAMILIAL PHEOCHROMOCYTOMA IN THE LAPAROSCOPIC ERA.
Two patients underwent bilateral procedures, and five unilateral procedures were in patients who had undergone prior open adrenalectomy. Surgical approach was laparoscopic in 18 of the 21 patients, laparoscopic converted to open in 2 patients (one for bleeding and one for disruption of the adrenal capsule near the tumor), and open adrenalectomy in 1 patient with previous adrenalectomy and presence of megacolon. Fifteen patients had intraoperative hypertension easily controlled pharmacologically. Mean operative time was 216 ⫾ 57 minutes with an average blood loss of 168 ml. Mean hospital stay was 3.1 ⫾ 1.3 days. Mean tumor size was 3.1 ⫾ 1.0cm. Three patients (14.3%) had minor complications, including fever, atelectasis, and self-limited bleeding. Two patients (9.5%) had major complications, including urethral injury from urinary catheter placement and port site hernia. During a mean follow-up of 57 months, 4 of the patients with MEN2A and 2B who underwent unilateral adrenalectomy with a remaining gland in situ developed contralateral pheochromocytomas (33%). None of these patients were hypertensive.
Brunt ML, Larmoire TC, Doherty GM, Quaserbarth MA, DeBenedetti M, Moley JF. Ann Surgery. 2002;235:713-721. Objective: To report the results of treatment of patients with familial pheochromocytomas in the laparoscopic era. Design: Retrospective case review. Setting: Washington University Medical Center, St. Louis,
MO. Participants: Twenty-one patients with familial pheochromocytoma undergoing adrenalectomy between 1993 and July 2001. Results: Twenty-one patients with familial pheochromocytoma underwent adrenalectomy between December 1993 and July 2001. A total of 15 patients had multiple endocrine neoplasia (MEN)2A, 4 had MEN 2B, and 1 patient each had von Hippel-Lindau (VHL) and neurofibromatosis type 1(NF-1). Mean age at diagnosis was 37 ⫾ 4 years. Twenty of 21 patients had increased urinary catecholamines, and all patients had radiographic evidence of one or more adrenal tumors. Five patients (24%) had undergone contralateral adrenalectomy an average of 13.2 ⫾ 9.1 years previously via an open transabdominal approach with manual exploration of the unaffected side. In all, 10 patients (48%) had undergone previous abdominal surgery. Eleven patients (51%) had pheochromocytoma-related symptoms. A total of 24 adrenalectomies were performed.
Conclusions: A laparoscopic approach to unilateral or bilateral adrenalectomy is appropriate for most patients with familial pheochromocytoma. Patients whose conditions are diagnosed by regular clinical and biochemical screening frequently have minimal or no symptoms, and they often do not have marked intraoperative hemodynamic changes. For patients who have unilateral adrenalectomy, the risk of developing a contralateral tumor is 33% during the next 5 years, and the risk for hypertension related complications is low. Laparoscopic adrenalectomy has many advantages for this patient population and should be the preferred surgical approach.
REVIEWER COMMENTS
This article, like the first paper reviewed, demonstrates the safety and efficacy of a laparoscopic approach to adrenalectomy in pheochromocytoma patients. This paper, however, focuses on patients with hereditary pheochromocytomas, which 562
present unique challenges to the surgeon because of the associated endocrinopathies and potential for bilateral involvement seen in these familial syndromes. Most hereditary pheochromocytomas occur in the setting of MEN2 or VHL, with only 5%
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occurring in patients with NF-1. These tumors occur in 30% to 50% of patients with MEN2A and approximately 50% in those with MEN2B. von Hippel-Lindau is associated with a 15% to 20% incidence of pheochromocytoma. Identification of genetic defects associated with these diseases has been a major advance in the evaluation of patients with familial syndromes, which allows family members to be screened for specific germ line defects, for example, the ret proto-oncogene associated with MEN2.3 The authors identify several points about laparoscopic resection of pheochromocytomas specific to this patient population. The first is the tendency for these patients to develop multiple or bilateral tumors. Historically, there has been some controversy as to the best management pheochromocytomas in patients with MEN2. Some groups have advocated bilateral adrenalectomies because of the relatively high likelihood of development of additional tumors in the future. This is somewhat undesirable, however, as it commits the patient to lifelong steroid therapy with its own high risks. The authors have shown that it is safe to perform unilateral adrenalectomies in these patients followed by yearly clinical and biochemical evaluation for recurrences. In their series, 5 patients, or 33%, did develop contralateral tumors, but they were detected early by screening, and the patients did not suffer any hypertensive complications.
Although one-third of patients ultimately did require interval bilateral adrenalectomy, in two-thirds of patients, bilateral adrenalectomy could be avoided or delayed, thus avoiding the morbidity of chronic steroid replacement therapy. Another important issue addressed in this study is the safety of reoperation using laparoscopic techniques in those patients in whom a previous contralateral open adrenalectomy had been performed. The authors encountered five such patients in this series. They were able to perform adhesiolysis and adrenalectomy using a laparoscopic approach for the contralateral adrenal safely in all cases. An additional point made by the authors is the importance of inspecting the liver for lesions during LA, which may not have been seen on preoperative imaging. Patients with MEN2 can have medullary thyroid carcinoma (MTC) as part of their disease syndrome. Four patients in this series underwent liver biopsy, and two of these were positive for metastatic MTC. Finally the authors point out that their operative times are longer compared with LA performed for adrenal adenomas. This can be explained by the fact that pheochromocytomas tend to be larger and more vascular than other adrenal tumors. Additionally, specific to this patient population is the relatively frequent need for additional surgery, such as liver biopsy and adhesiolysis.
IATROGENIC PHEOCHROMOCYTOMATOSIS: A PREVIOUSLY UNREPORTED RESULT OF LAPAROSCOPIC ADRENALECTOMY.
developed symptoms of pheochromocytoma, increased urinary catecholamines, and had CT or MIBG imaging consistent with recurrence. At reoperation, performed through a bilateral subcostal or thoracodorsal incision, multiple tumor nodules were identified at the previous resection bed in two cases, and one patient had diffuse disease requiring en-bloc resection of the spleen, left kidney, part of the left hemidiaphragm, and the tail of the pancreas. Review of original operative notes revealed a possible method of local tumor seeding, with one case noting tumor fragmentation and spillage, one case documenting a friable tumor, and a third case documenting excessive tumor manipulation during resection. Despite this, each surgeon felt at the time of operation that the tumor had been removed in its entirety.
Li ML, Fitzgerald PA, Price DC, Norton JA. Surgery. 2001; 130:1072-1077. Objective: To describe laparoscopic seeding of tumor as ori-
gin of recurrence after LA for pheochromocytoma. Design: Retrospective case review. Setting: University of California-San Francisco (UCSF). Participants: Three patients with biochemical and radiolabeled metaiodobenzylguanidine (MIBG) evidence of recurrent pheochromocytoma after prior presumed curative laparoscopic resection. Results: Three patients were referred to UCSF from January 1 to December 31, 2000, with evidence of recurrent pheochromocytoma after prior LA. The original tumor size ranged from 5.5 to 6.5 cm in diameter, and it was not believed to be malignant at the time of initial resection based on clinical and histopathological data. At 3 to 4 years follow-up, each patient
Conclusions: Pheochromocytomas resected laparoscopically can recur locally despite lack of evidence of malignancy at the time of operation. Tumor fragmentation, spillage, friable tumor, or need for excessive manipulation during laparoscopic resection should result in immediate conversion to an open operation to try and avoid future recurrences in the tumor bed. More long-term data is needed in laparoscopic resections for pheochromocytomas.
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REVIEWER COMMENTS
Although this paper includes a very small series of only three patients, it is an important article in that it is one of the first papers to report a possible negative aspect to LA for pheochromocytoma based on long-term follow-up data. All of the initial retrospective data, as discussed with the first two articles reviewed, showed that laparoscopic adrenalectomy is safe, better than open surgery, and should be the preferred surgical approach. This article does not challenge that contention, but it does point out a possible problem with the laparoscopic approach to these tumors. None of the patients in this series had any evidence of malignancy at initial operation, clinically or histopathologically. However, they all developed local recurrence. As previously mentioned, suspicion of malignancy based on large size, vascular or local invasion, or lymphadenopathy should be considered an absolute contraindication to a laparoscopic approach. However, this paper points to the fact that all three patients with recurrence had tumors less than or equal to 6.5 cm, which emphasizes the difficulty of establishing malig-
nancy based on size alone. Historically, 10% of all pheochromocytomas are malignant; thus, surgeons should be always alert to this possibility. Not only should laparoscopic surgery not be performed in patients with tumors larger than 6 to 8 cm or those with radiologic signs of malignancy, but if difficult manipulation, tumor friability, or rupture is noted during a laparoscopic approach, prompt conversion to open surgery should be performed in an attempt to avoid seeding. Thus, although only three cases are reported here, the authors may have identified additional situations encountered during the operation that should prompt conversion to an open procedure. Certainly, more data are needed, but as these data emerge through long-term follow-up, it may be that the authors’ suspicions are confirmed, and more concrete practice guidelines for the indications and contraindications for LA for pheochromocytoma will evolve. This article stresses the importance of continued vigilance in gathering long-term follow-up data in these patients.
REVIEWER SUMMARY
Since the time a pheochromocytoma was first successfully resected in 1926 until the early 1950s, the operative mortality associated with this procedure was approximately 25%. Since that time, significant change has taken place in the surgical treatment of this disease. As surgeons gained a better understanding of the physiologic and hemodynamic changes encountered during resection of these tumors, the advent of preoperative alpha-blockade and volume expansion described by Priestly et al in 1956 led to a significant reduction in perioperative mortality.4 Despite improved mortality, surgical resection of these tumors continued to be associated with a significant morbidity, mostly because of the extensive exploration routinely performed to rule out additional extra-adrenal tumors. With the introduction of CT, magnetic resonance imaging, and radiolabeled MIBG scanning, the preoperative localization of these tumors improved significantly. This allowed surgeons to perform more focused resections without the large, morbid incisions and extensive dissection previously required. In 1987, the landscape of abdominal surgery was changed forever when Dubois and associates first described laparoscopic cholecystectomy. 5 In 1992, Gagner et al reported the first laparoscopic adrenalectomy in a patient with Cushing’s syndrome from an adrenal adenoma.6 Since then, many centers have reported their clinical experience with this technique for resecting various adrenal tumors. The results of two such studies, one by Gagner et al at Mount Sinai School of Medicine and another by Thompson et al at the Mayo Clinic presented compelling data favoring a laparoscopic approach to adrenalectomy, including lower blood loss, shorter 564
hospital stay, and lower complication rates despite longer operating times.1, 7 Enthusiasm for LA continued to grow, becoming the standard of care for benign functioning and nonfunctioning adrenal tumors. Indications for this procedure were essentially the same as for open adrenalectomy, with two exceptions. Surgeons continued to perform open adrenalectomies for tumors with a high suspicion for malignancy and for pheochromocytomas, because of the worries centered on serious hemodynamic fluctuations encountered during the resection of these tumors. Centers began reporting their experiences in laparoscopic resection of adrenal pheochromocytomas. As in the first two papers reviewed here, the results of several retrospective studies led the authors to conclude that the laparoscopic approach to resecting pheochromocytomas was as safe as open surgery. One worry was the effects pneumoperitoneum would have on patient hemodynamics. Experience has been variable, with some authors noting significant hypertension associated with CO2 insufflation and others reporting no significant hemodynamic changes. However, even those authors who experienced hypertension reported that this was easily controlled pharmacologically. Once pneumoperitoneum had been established, hypertension and hypotension occurred at the same frequency as in open surgery and were easily controlled.8-10 Several papers show that LA is as safe as open surgery with a significantly reduced morbidity. The first paper reviewed here was chosen based on the relatively large number of patients in the study. Collectively, these papers have shown that, with the exception of obviously malignant tumors (based on invasion of adjacent organs or vasculature) and possibly very large tumors greater than 8 cm,
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in experienced hands, LA should be the standard of care for most adrenal tumors, including pheochromocytomas. The second paper reviewed addresses the issue of how best to manage familial pheochromocytomas as seen in MEN2, VHL, and NF-1. Although much less common than sporadic tumors, these hereditary tumors present somewhat differently. Given aggressive screening strategies, patients with familial diseases tend to present younger, have smaller tumors at diagnosis, and have a lower incidence of hypertension. This makes these patients ideal for a minimally invasive approach. Because familial tumors also have a higher incidence of bilaterality, these patients historically underwent bilateral adrenalectomies because of the fear of hypertensive complications caused by a contralateral tumor. Bilateral adrenalectomy, however, sentenced the patient to the morbidity of lifelong steroid treatment. This study, however, reports their experience treating these patients with unilateral adrenalectomy followed by yearly clinical and biochemical monitoring. Only patients with documented bilateral tumors by preoperative imaging underwent bilateral adrenalectomy. Twelve patients in this series with MEN2A and 2B underwent unilateral adrenalectomy and had the contralateral gland remaining in situ. Four of these patients went on to develop contralateral tumors at a mean interval of 57 ⫾ 8.5 months. These patients presented with increased urinary catecholamines and metabolites, but none had hypertension and were subsequently treated laparoscopically without difficulty. Therefore, although there is a real risk of developing a contralateral tumor in these patients, additional tumors can be picked up early with yearly measurements of urinary catecholamines without serious risk for hypertensive complications while preserving adrenal function for as long as possible. In terms of the technical aspects of reoperating on these patients, five patients in their series had undergone previous contralateral open adrenalectomy with manual exploration of the unaffected side. The authors were able to safely perform adhesiolysis and exposure of the remaining diseased adrenal gland in all attempted cases. This provides additional support that managing familial pheochromocytoma with unilateral adrenalectomy can be safely done with close postoperative monitoring. Although the short-term data overwhelmingly support laparoscopic resection of adrenal pheochromocytomas as the standard of care for all but very large and obviously malignant tumors, there is not much data concerning long-term outcomes in these patients. The third reviewed paper reports three cases of recurrent pheochromocytomatosis after LA postulated to be caused by laparoscopic seeding of tumor during resection. This is the first paper to present data on negative outcomes related to a laparoscopic approach. Despite the fact that this paper only reports on three cases, the points made by the authors concerning the description from the initial operative notes of friability of the tumor mass, tumor spillage, and prolonged tumor manipulation certainly provide food for thought. This final article stresses the importance of continued close long-term follow-up of patients with both sporadic and familial pheochromocyto-
mas. Further data will help guide patient selection and technical safety during laparoscopic adrenalectomy to help generate the best long-term outcomes in this uniquely challenging disease. doi: 10.1016/j.cursur.2003.06.001
REFERENCES 1. Jossart G, Burpee S, Gagner M. Surgery of the Adrenal
Glands. Endosc Met Clin North Am. 2000;29:57-68. 2. Kebebew E, et al. Results of laparoscopic adrenalectomy
for suspected and unsuspected malignant adrenal neoplasms. Arch Surg. 2000;137:948-953. 3. Donis-Keller H, Dou S, Chi D, et al, Mutations in the
RET proto-oncogene are associated with MEN 2A and FMTC. Hum Mol Genet. 1993;7:851-856. 4. Kuate W, Roth G, Manger W, Priestley J. Pheochromo-
cytoma. Circulation. 1956;146:22-30. 5. Soper N, Brunt C, Kerbl K. Laparoscopoic general sur-
gery. N Eng J Med. 1994;330:409-419. 6. Gagner M, Lacroix A, Bolte E. Laparoscopoic adrenalec-
tomy in Cushing’s syndrome and pheochromocytoma. N Eng J Med. 1992;327:1033. 7. Thompson G, Grant C, van Herdeen J, et al. Laparo-
scopic versus open posterior adrenalectomy: a case control study of 100 patients. Surgery. 1997;122:1132-1136. 8. Gagner M, Breton G, Pharand D, et al. Is laparoscopic
adrenalectomy indicated for pheochromocytomas? Surgery. 1996;120:1076-1080. 9. Inabnet W, Pitre J, Bernard D, et al. Comparison of the
hemodynamic parameters of open and laparoscopic adrenalectomy for pheochromocytoma. World J Surg. 2000; 24:574-578. 10. Fernandez-Cruz L, Taura P, Saenz A, et al. Laparoscopic
approach to pheochromocytoma: hemodynamic changes and catecholamine secretion. World J Surg. 1996;20:762768.
QUESTIONS AND ANSWERS Questions 1. At what size tumor does laparoscopic resection of pheochromocytomas become contraindicated? 2. What syndromes are associated with familial pheochromocytoma? 3. T or F: Patients found to have familial pheochromocytomas
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should always undergo bilateral adrenalectomy because of the high risk of recurrence. Answers 1. No specific size criteria have been established as contraindications for resecting pheochromocytomas laparoscopically. Data have shown that risk for malignancy increases in tumors greater than 6c m in size, and it is very common in tumors greater than 10 cm. As malignancy is difficult to establish preoperatively, surgeons must use these general size criteria, as well as carefully review preoperative imaging studies when deciding how to safely resect these tumors.
2. MEN2A and 2B, von Hippel-Lindau, and neurofibromatosis type 1. Most familial pheochromocytomas are seen in MEN and VHL, with only 5% occurring in patients with NF-1. Overall, 30% to 50% of patients with MEN2A and 50% of patients with MEN2B develop these tumors. There is a 15% to 20% incidence of pheochromocytoma with VHL. Because of the increased incidence of bilateral tumors in these patients, preoperative localization is particularly important. 3. F. Although some surgeons feel this is the correct management of familial tumors, it has been shown that unilateral adrenalectomy with surveillance of the contralateral adrenal gland can spare up to 66% of patients the morbidity associated with bilateral adrenalectomy with resultant steroid therapy.
Endocrine Location, Location, Location: Finding the Elusive Parathyroid Guest Reviewers: Scott M. Wilhelm, MD, and Jerry M. Shuck, MD, Department of Surgery, Case Western Reserve University, University Hospitals of Cleveland, Cleveland, Ohio PROSPECTIVE EVALUATION OF DELAYED TECHNECTIUM-99M SESTAMIBI SPECT SCINTIGRAPHY FOR PREOPERATIVE LOCALIZATION OF PRIMARY HYPERPARATHYROIDISM.
hours) to identify and localize abnormal parathyroid glands. Location and number of abnormal glands was recorded at operation and correlated with sestamibi results to calculate sensitivity, specificity, and accuracy of sestamibi-SPECT.
Civelek AC, Ozalp E, Donovan P, Udelsman, R. Surgery.
Results: (1) Surgical results: 338 consecutive patients with PHPT underwent parathyroidectomy with the excision of 400 abnormal glands (299 patients had a single adenoma, 23 had double adenoma, 14 had multiglandular hyperplasia, and 2 patients had no abnormal glands found in the neck). Curative resection as indicated by a normalization of serum calcium was achieved in 98.5% of patients with a 0.7% failure rate in initial operations and 5.9% failure in reoperative surgery. (2) Sestamibi-SPECT results: For solitary adenomas, sestamibi correctly lateralized the abnormal gland (sensitivity) in 96% of patients. In the 23 patients with double adenoma, 15 patients had both adenomas localized and the remaining 8 patients had at least 1 adenoma localized, 83% sensitivity. The sensitivity in patients with multigland disease was poor at only 45%. For patients undergoing initial operation, sensitivity of sestamibi was 87% compared to 92% in reoperative cases.
2002;13:149-157. Objective: To evaluate the ability of preoperative Techne-
tium-99m sestamibi with single photon emission computerized tomography (SPECT) to detect and localize abnormal parathyroid glands in patients with primary hyperparathyroidism (PHPT). Design: Prospective study from 1994 to 2000 of 338 patients (287 patients—first operation, 51 patients—reexploration) who underwent preoperative sestamibi SPECT and parathyroidectomy. Setting: The Department of Surgery, Yale University School of Medicine, and the Russell H. Morgan Department of Radiology and Radiological Science, Divisions of Nuclear Medicine and Radiation Health Sciences, New Haven, Connecticut. Tertiary Care Referral Center. Participants: A total of 338 consecutive patients, from 1994 to 2000 with biochemically proven PHPT, including 287 patients—first operation, and 51 patients—reoperative surgery for persistent or recurrent PHPT. Each patient underwent preoperative sestamibi-SPECT with initial and delayed scans (2.5
566
Conclusions: Technetium-99m sestamibi-SPECT is highly sensitive in detecting abnormal parathyroid glands in patients with single adenomas and works well in both initial and reoperative cases. However, the sensitivity for multigland disease is limited. This imaging modality is a very useful guide for “directed- minimal invasive” parathyroidectomy, but it requires a solid knowledge of parathyroid disease.
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scopic adrenalectomy for patients with pheochromocytomas. Design: Retrospective case review. Setting: University of California-San Fransisco (UCSF). Participants: A total of 39 consecutive patients who underwent laparoscopic adrenalectomy for pheochromocytoma from 1993 to 2000 at UCSF. Results: The study group consisted of 39 patients (22M, 17F) with a mean age of 48 years (range, 20 to 84). Overall, 35 patients had unilateral tumors and 4 had bilateral tumors. A total of 43 adrenalectomies were performed. Three groups were identified based on presentation, 17 patients with classic symptoms (group A), 17 patients whose tumors were discovered incidentally during imaging (computed tomography or ultrasound) for other symptoms (group B), and 5 patients who presented with hypertensive crises and multiorgan failure requiring prolonged intensive care unit stay (group C). All patients screened for pheochromocytoma had increased urinary metanephrine and catecholamine levels. Preoperative imaging with either computed tomography (CT) or magnetic resonance imaging (MRI), or both, was performed on all patients with 100% accuracy. A total of 34 patients (87%) had sporadic lesions, 5 patients had familial tumors (2 Multiple Endocrine Neoplasia
II, 1 each neurofibromatosis type I, von-Hippel- Lindau, and Osler-Weber-Renu). Patients were treated preoperatively with phenoxybenzamine and aggressive hydration. Laparoscopic adrenalectomy was performed via a lateral transabdominal approach in 31 patients and via a posterior retroperitoneal approach in 4 patients. One patient with a large tumor greater than 12 cm had a hand-assisted resection. No cases required conversion to an open approach. Operative time averaged 217 minutes for unilateral tumors and 341 minutes for bilateral tumors. Blood loss averaged 160 cc. Postoperative hospital stay averaged 1.5 days for elective and 3.0 days for patients presenting with acute hypertensive crises. Complications occurred in 3 patients (8%), 2 patients required blood transfusions, and 1 patient developed postoperative heart failure requiring pressor support. There were no operative mortalities. Conclusions: Patients with pheochromocytomas can present in several different ways, ranging from those with minimal or no symptoms to those with life-threatening hypertensive crises and multiorgan system failure. Because of increased use of imaging studies in medicine today, many of these tumors now present as incidentalomas, which should be evaluated with appropriate screening urinary tests. Percutaneous fine-needle aspiration biopsy should not be performed, as it is unnecessary and potentially hazardous. Laparoscopic adrenalectomy can be performed safely and effectively for pheochromocytomas with adequate preoperative preparation with alpha blockade and hydration. The laparoscopic approach results in less blood loss, fewer complications, and shorter postoperative stays compared with open surgery and should be the standard of care for benign tumors.
REVIEWER COMMENTS
This article is one of several in the literature of a surgical center reporting their experience with the use of laparoscopic adrenalectomy (LA) for the resection of pheochromocytomas. Although these series tend to be fairly small because of the relative rarity of these tumors, this retrospective study of 39 patients represents one of the largest individual series of patients reported. The authors have shown, similar to other reported series, that laparoscopic adrenalectomy for pheochromocytomas can be performed safely and effectively in experienced hands, with less blood loss, less postoperative pain, shorter hospital stay, and less overall complications than with open adrenalectomy. This series documents, as do the majority of series in the
literature, that operative times for LA are significantly longer than for open adrenalectomy. Some of this is because of the nature of laparoscopic surgery, but surgeon experience and the “learning curve” also play a role. As surgeons gain more experience with this procedure, this time difference is likely going to become less significant. One critique of this study is the relatively small number of patients with familial pheochromocytoma syndromes; however, the patient population presented herein is likely to be representative of the patients seen by most general surgeons, including those with fairly large endocrine surgery experiences. The authors identified three groups of patients according to
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their presentation, and they stress the fact that because of the increased use of advanced imaging studies in general medical practice, more patients are presenting with incidentally discovered adrenal masses. These patients, after a thorough history and physical, should all undergo routing biochemical screening to rule out a functional adrenal tumor. The authors also note that nearly half of the patients referred for an incidentaloma had undergone inappropriate FNA biopsy before any biochemical screening, which should be avoided because needle aspiration of a pheochromocytoma can result in hypertensive crisis and death.1 While not specifically addressed in this paper, the much debated question of the upper limit of tumor size when contemplating safe laparoscopic adrenalectomy is indirectly ad-
dressed since the authors point out that resection of larger tumors was more difficult, with tumors greater than 8 cm taking 2.5 times longer than smaller tumors. Malignancy is difficult to establish in these tumors preoperatively. The risk for malignancy is thought to increase with tumor size greater than 6 cm, and is very common in tumors larger than 10 cm 2 However, no size limit has been established in the literature for safe use of a laparoscopic approach. Although size alone is not a contraindication for a safe laparoscopic approach, surgeons must use caution in planning a laparoscopic approach when tumor size is between 6 and 10 cm. Imaging studies should be carefully reviewed, and laparoscopic surgery should be avoided if other parameters such as lymphadenopathy, local, or vascular invasion are present.
ADRENALECTOMY FOR FAMILIAL PHEOCHROMOCYTOMA IN THE LAPAROSCOPIC ERA.
Two patients underwent bilateral procedures, and five unilateral procedures were in patients who had undergone prior open adrenalectomy. Surgical approach was laparoscopic in 18 of the 21 patients, laparoscopic converted to open in 2 patients (one for bleeding and one for disruption of the adrenal capsule near the tumor), and open adrenalectomy in 1 patient with previous adrenalectomy and presence of megacolon. Fifteen patients had intraoperative hypertension easily controlled pharmacologically. Mean operative time was 216 ⫾ 57 minutes with an average blood loss of 168 ml. Mean hospital stay was 3.1 ⫾ 1.3 days. Mean tumor size was 3.1 ⫾ 1.0cm. Three patients (14.3%) had minor complications, including fever, atelectasis, and self-limited bleeding. Two patients (9.5%) had major complications, including urethral injury from urinary catheter placement and port site hernia. During a mean follow-up of 57 months, 4 of the patients with MEN2A and 2B who underwent unilateral adrenalectomy with a remaining gland in situ developed contralateral pheochromocytomas (33%). None of these patients were hypertensive.
Brunt ML, Larmoire TC, Doherty GM, Quaserbarth MA, DeBenedetti M, Moley JF. Ann Surgery. 2002;235:713-721. Objective: To report the results of treatment of patients with familial pheochromocytomas in the laparoscopic era. Design: Retrospective case review. Setting: Washington University Medical Center, St. Louis,
MO. Participants: Twenty-one patients with familial pheochromocytoma undergoing adrenalectomy between 1993 and July 2001. Results: Twenty-one patients with familial pheochromocytoma underwent adrenalectomy between December 1993 and July 2001. A total of 15 patients had multiple endocrine neoplasia (MEN)2A, 4 had MEN 2B, and 1 patient each had von Hippel-Lindau (VHL) and neurofibromatosis type 1(NF-1). Mean age at diagnosis was 37 ⫾ 4 years. Twenty of 21 patients had increased urinary catecholamines, and all patients had radiographic evidence of one or more adrenal tumors. Five patients (24%) had undergone contralateral adrenalectomy an average of 13.2 ⫾ 9.1 years previously via an open transabdominal approach with manual exploration of the unaffected side. In all, 10 patients (48%) had undergone previous abdominal surgery. Eleven patients (51%) had pheochromocytoma-related symptoms. A total of 24 adrenalectomies were performed.
Conclusions: A laparoscopic approach to unilateral or bilateral adrenalectomy is appropriate for most patients with familial pheochromocytoma. Patients whose conditions are diagnosed by regular clinical and biochemical screening frequently have minimal or no symptoms, and they often do not have marked intraoperative hemodynamic changes. For patients who have unilateral adrenalectomy, the risk of developing a contralateral tumor is 33% during the next 5 years, and the risk for hypertension related complications is low. Laparoscopic adrenalectomy has many advantages for this patient population and should be the preferred surgical approach.
REVIEWER COMMENTS
This article, like the first paper reviewed, demonstrates the safety and efficacy of a laparoscopic approach to adrenalectomy in pheochromocytoma patients. This paper, however, focuses on patients with hereditary pheochromocytomas, which 562
present unique challenges to the surgeon because of the associated endocrinopathies and potential for bilateral involvement seen in these familial syndromes. Most hereditary pheochromocytomas occur in the setting of MEN2 or VHL, with only 5%
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occurring in patients with NF-1. These tumors occur in 30% to 50% of patients with MEN2A and approximately 50% in those with MEN2B. von Hippel-Lindau is associated with a 15% to 20% incidence of pheochromocytoma. Identification of genetic defects associated with these diseases has been a major advance in the evaluation of patients with familial syndromes, which allows family members to be screened for specific germ line defects, for example, the ret proto-oncogene associated with MEN2.3 The authors identify several points about laparoscopic resection of pheochromocytomas specific to this patient population. The first is the tendency for these patients to develop multiple or bilateral tumors. Historically, there has been some controversy as to the best management pheochromocytomas in patients with MEN2. Some groups have advocated bilateral adrenalectomies because of the relatively high likelihood of development of additional tumors in the future. This is somewhat undesirable, however, as it commits the patient to lifelong steroid therapy with its own high risks. The authors have shown that it is safe to perform unilateral adrenalectomies in these patients followed by yearly clinical and biochemical evaluation for recurrences. In their series, 5 patients, or 33%, did develop contralateral tumors, but they were detected early by screening, and the patients did not suffer any hypertensive complications.
Although one-third of patients ultimately did require interval bilateral adrenalectomy, in two-thirds of patients, bilateral adrenalectomy could be avoided or delayed, thus avoiding the morbidity of chronic steroid replacement therapy. Another important issue addressed in this study is the safety of reoperation using laparoscopic techniques in those patients in whom a previous contralateral open adrenalectomy had been performed. The authors encountered five such patients in this series. They were able to perform adhesiolysis and adrenalectomy using a laparoscopic approach for the contralateral adrenal safely in all cases. An additional point made by the authors is the importance of inspecting the liver for lesions during LA, which may not have been seen on preoperative imaging. Patients with MEN2 can have medullary thyroid carcinoma (MTC) as part of their disease syndrome. Four patients in this series underwent liver biopsy, and two of these were positive for metastatic MTC. Finally the authors point out that their operative times are longer compared with LA performed for adrenal adenomas. This can be explained by the fact that pheochromocytomas tend to be larger and more vascular than other adrenal tumors. Additionally, specific to this patient population is the relatively frequent need for additional surgery, such as liver biopsy and adhesiolysis.
IATROGENIC PHEOCHROMOCYTOMATOSIS: A PREVIOUSLY UNREPORTED RESULT OF LAPAROSCOPIC ADRENALECTOMY.
developed symptoms of pheochromocytoma, increased urinary catecholamines, and had CT or MIBG imaging consistent with recurrence. At reoperation, performed through a bilateral subcostal or thoracodorsal incision, multiple tumor nodules were identified at the previous resection bed in two cases, and one patient had diffuse disease requiring en-bloc resection of the spleen, left kidney, part of the left hemidiaphragm, and the tail of the pancreas. Review of original operative notes revealed a possible method of local tumor seeding, with one case noting tumor fragmentation and spillage, one case documenting a friable tumor, and a third case documenting excessive tumor manipulation during resection. Despite this, each surgeon felt at the time of operation that the tumor had been removed in its entirety.
Li ML, Fitzgerald PA, Price DC, Norton JA. Surgery. 2001; 130:1072-1077. Objective: To describe laparoscopic seeding of tumor as ori-
gin of recurrence after LA for pheochromocytoma. Design: Retrospective case review. Setting: University of California-San Francisco (UCSF). Participants: Three patients with biochemical and radiolabeled metaiodobenzylguanidine (MIBG) evidence of recurrent pheochromocytoma after prior presumed curative laparoscopic resection. Results: Three patients were referred to UCSF from January 1 to December 31, 2000, with evidence of recurrent pheochromocytoma after prior LA. The original tumor size ranged from 5.5 to 6.5 cm in diameter, and it was not believed to be malignant at the time of initial resection based on clinical and histopathological data. At 3 to 4 years follow-up, each patient
Conclusions: Pheochromocytomas resected laparoscopically can recur locally despite lack of evidence of malignancy at the time of operation. Tumor fragmentation, spillage, friable tumor, or need for excessive manipulation during laparoscopic resection should result in immediate conversion to an open operation to try and avoid future recurrences in the tumor bed. More long-term data is needed in laparoscopic resections for pheochromocytomas.
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REVIEWER COMMENTS
Although this paper includes a very small series of only three patients, it is an important article in that it is one of the first papers to report a possible negative aspect to LA for pheochromocytoma based on long-term follow-up data. All of the initial retrospective data, as discussed with the first two articles reviewed, showed that laparoscopic adrenalectomy is safe, better than open surgery, and should be the preferred surgical approach. This article does not challenge that contention, but it does point out a possible problem with the laparoscopic approach to these tumors. None of the patients in this series had any evidence of malignancy at initial operation, clinically or histopathologically. However, they all developed local recurrence. As previously mentioned, suspicion of malignancy based on large size, vascular or local invasion, or lymphadenopathy should be considered an absolute contraindication to a laparoscopic approach. However, this paper points to the fact that all three patients with recurrence had tumors less than or equal to 6.5 cm, which emphasizes the difficulty of establishing malig-
nancy based on size alone. Historically, 10% of all pheochromocytomas are malignant; thus, surgeons should be always alert to this possibility. Not only should laparoscopic surgery not be performed in patients with tumors larger than 6 to 8 cm or those with radiologic signs of malignancy, but if difficult manipulation, tumor friability, or rupture is noted during a laparoscopic approach, prompt conversion to open surgery should be performed in an attempt to avoid seeding. Thus, although only three cases are reported here, the authors may have identified additional situations encountered during the operation that should prompt conversion to an open procedure. Certainly, more data are needed, but as these data emerge through long-term follow-up, it may be that the authors’ suspicions are confirmed, and more concrete practice guidelines for the indications and contraindications for LA for pheochromocytoma will evolve. This article stresses the importance of continued vigilance in gathering long-term follow-up data in these patients.
REVIEWER SUMMARY
Since the time a pheochromocytoma was first successfully resected in 1926 until the early 1950s, the operative mortality associated with this procedure was approximately 25%. Since that time, significant change has taken place in the surgical treatment of this disease. As surgeons gained a better understanding of the physiologic and hemodynamic changes encountered during resection of these tumors, the advent of preoperative alpha-blockade and volume expansion described by Priestly et al in 1956 led to a significant reduction in perioperative mortality.4 Despite improved mortality, surgical resection of these tumors continued to be associated with a significant morbidity, mostly because of the extensive exploration routinely performed to rule out additional extra-adrenal tumors. With the introduction of CT, magnetic resonance imaging, and radiolabeled MIBG scanning, the preoperative localization of these tumors improved significantly. This allowed surgeons to perform more focused resections without the large, morbid incisions and extensive dissection previously required. In 1987, the landscape of abdominal surgery was changed forever when Dubois and associates first described laparoscopic cholecystectomy. 5 In 1992, Gagner et al reported the first laparoscopic adrenalectomy in a patient with Cushing’s syndrome from an adrenal adenoma.6 Since then, many centers have reported their clinical experience with this technique for resecting various adrenal tumors. The results of two such studies, one by Gagner et al at Mount Sinai School of Medicine and another by Thompson et al at the Mayo Clinic presented compelling data favoring a laparoscopic approach to adrenalectomy, including lower blood loss, shorter 564
hospital stay, and lower complication rates despite longer operating times.1, 7 Enthusiasm for LA continued to grow, becoming the standard of care for benign functioning and nonfunctioning adrenal tumors. Indications for this procedure were essentially the same as for open adrenalectomy, with two exceptions. Surgeons continued to perform open adrenalectomies for tumors with a high suspicion for malignancy and for pheochromocytomas, because of the worries centered on serious hemodynamic fluctuations encountered during the resection of these tumors. Centers began reporting their experiences in laparoscopic resection of adrenal pheochromocytomas. As in the first two papers reviewed here, the results of several retrospective studies led the authors to conclude that the laparoscopic approach to resecting pheochromocytomas was as safe as open surgery. One worry was the effects pneumoperitoneum would have on patient hemodynamics. Experience has been variable, with some authors noting significant hypertension associated with CO2 insufflation and others reporting no significant hemodynamic changes. However, even those authors who experienced hypertension reported that this was easily controlled pharmacologically. Once pneumoperitoneum had been established, hypertension and hypotension occurred at the same frequency as in open surgery and were easily controlled.8-10 Several papers show that LA is as safe as open surgery with a significantly reduced morbidity. The first paper reviewed here was chosen based on the relatively large number of patients in the study. Collectively, these papers have shown that, with the exception of obviously malignant tumors (based on invasion of adjacent organs or vasculature) and possibly very large tumors greater than 8 cm,
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in experienced hands, LA should be the standard of care for most adrenal tumors, including pheochromocytomas. The second paper reviewed addresses the issue of how best to manage familial pheochromocytomas as seen in MEN2, VHL, and NF-1. Although much less common than sporadic tumors, these hereditary tumors present somewhat differently. Given aggressive screening strategies, patients with familial diseases tend to present younger, have smaller tumors at diagnosis, and have a lower incidence of hypertension. This makes these patients ideal for a minimally invasive approach. Because familial tumors also have a higher incidence of bilaterality, these patients historically underwent bilateral adrenalectomies because of the fear of hypertensive complications caused by a contralateral tumor. Bilateral adrenalectomy, however, sentenced the patient to the morbidity of lifelong steroid treatment. This study, however, reports their experience treating these patients with unilateral adrenalectomy followed by yearly clinical and biochemical monitoring. Only patients with documented bilateral tumors by preoperative imaging underwent bilateral adrenalectomy. Twelve patients in this series with MEN2A and 2B underwent unilateral adrenalectomy and had the contralateral gland remaining in situ. Four of these patients went on to develop contralateral tumors at a mean interval of 57 ⫾ 8.5 months. These patients presented with increased urinary catecholamines and metabolites, but none had hypertension and were subsequently treated laparoscopically without difficulty. Therefore, although there is a real risk of developing a contralateral tumor in these patients, additional tumors can be picked up early with yearly measurements of urinary catecholamines without serious risk for hypertensive complications while preserving adrenal function for as long as possible. In terms of the technical aspects of reoperating on these patients, five patients in their series had undergone previous contralateral open adrenalectomy with manual exploration of the unaffected side. The authors were able to safely perform adhesiolysis and exposure of the remaining diseased adrenal gland in all attempted cases. This provides additional support that managing familial pheochromocytoma with unilateral adrenalectomy can be safely done with close postoperative monitoring. Although the short-term data overwhelmingly support laparoscopic resection of adrenal pheochromocytomas as the standard of care for all but very large and obviously malignant tumors, there is not much data concerning long-term outcomes in these patients. The third reviewed paper reports three cases of recurrent pheochromocytomatosis after LA postulated to be caused by laparoscopic seeding of tumor during resection. This is the first paper to present data on negative outcomes related to a laparoscopic approach. Despite the fact that this paper only reports on three cases, the points made by the authors concerning the description from the initial operative notes of friability of the tumor mass, tumor spillage, and prolonged tumor manipulation certainly provide food for thought. This final article stresses the importance of continued close long-term follow-up of patients with both sporadic and familial pheochromocyto-
mas. Further data will help guide patient selection and technical safety during laparoscopic adrenalectomy to help generate the best long-term outcomes in this uniquely challenging disease. doi: 10.1016/j.cursur.2003.06.001
REFERENCES 1. Jossart G, Burpee S, Gagner M. Surgery of the Adrenal
Glands. Endosc Met Clin North Am. 2000;29:57-68. 2. Kebebew E, et al. Results of laparoscopic adrenalectomy
for suspected and unsuspected malignant adrenal neoplasms. Arch Surg. 2000;137:948-953. 3. Donis-Keller H, Dou S, Chi D, et al, Mutations in the
RET proto-oncogene are associated with MEN 2A and FMTC. Hum Mol Genet. 1993;7:851-856. 4. Kuate W, Roth G, Manger W, Priestley J. Pheochromo-
cytoma. Circulation. 1956;146:22-30. 5. Soper N, Brunt C, Kerbl K. Laparoscopoic general sur-
gery. N Eng J Med. 1994;330:409-419. 6. Gagner M, Lacroix A, Bolte E. Laparoscopoic adrenalec-
tomy in Cushing’s syndrome and pheochromocytoma. N Eng J Med. 1992;327:1033. 7. Thompson G, Grant C, van Herdeen J, et al. Laparo-
scopic versus open posterior adrenalectomy: a case control study of 100 patients. Surgery. 1997;122:1132-1136. 8. Gagner M, Breton G, Pharand D, et al. Is laparoscopic
adrenalectomy indicated for pheochromocytomas? Surgery. 1996;120:1076-1080. 9. Inabnet W, Pitre J, Bernard D, et al. Comparison of the
hemodynamic parameters of open and laparoscopic adrenalectomy for pheochromocytoma. World J Surg. 2000; 24:574-578. 10. Fernandez-Cruz L, Taura P, Saenz A, et al. Laparoscopic
approach to pheochromocytoma: hemodynamic changes and catecholamine secretion. World J Surg. 1996;20:762768.
QUESTIONS AND ANSWERS Questions 1. At what size tumor does laparoscopic resection of pheochromocytomas become contraindicated? 2. What syndromes are associated with familial pheochromocytoma? 3. T or F: Patients found to have familial pheochromocytomas
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should always undergo bilateral adrenalectomy because of the high risk of recurrence. Answers 1. No specific size criteria have been established as contraindications for resecting pheochromocytomas laparoscopically. Data have shown that risk for malignancy increases in tumors greater than 6c m in size, and it is very common in tumors greater than 10 cm. As malignancy is difficult to establish preoperatively, surgeons must use these general size criteria, as well as carefully review preoperative imaging studies when deciding how to safely resect these tumors.
2. MEN2A and 2B, von Hippel-Lindau, and neurofibromatosis type 1. Most familial pheochromocytomas are seen in MEN and VHL, with only 5% occurring in patients with NF-1. Overall, 30% to 50% of patients with MEN2A and 50% of patients with MEN2B develop these tumors. There is a 15% to 20% incidence of pheochromocytoma with VHL. Because of the increased incidence of bilateral tumors in these patients, preoperative localization is particularly important. 3. F. Although some surgeons feel this is the correct management of familial tumors, it has been shown that unilateral adrenalectomy with surveillance of the contralateral adrenal gland can spare up to 66% of patients the morbidity associated with bilateral adrenalectomy with resultant steroid therapy.
Endocrine Location, Location, Location: Finding the Elusive Parathyroid Guest Reviewers: Scott M. Wilhelm, MD, and Jerry M. Shuck, MD, Department of Surgery, Case Western Reserve University, University Hospitals of Cleveland, Cleveland, Ohio PROSPECTIVE EVALUATION OF DELAYED TECHNECTIUM-99M SESTAMIBI SPECT SCINTIGRAPHY FOR PREOPERATIVE LOCALIZATION OF PRIMARY HYPERPARATHYROIDISM.
hours) to identify and localize abnormal parathyroid glands. Location and number of abnormal glands was recorded at operation and correlated with sestamibi results to calculate sensitivity, specificity, and accuracy of sestamibi-SPECT.
Civelek AC, Ozalp E, Donovan P, Udelsman, R. Surgery.
Results: (1) Surgical results: 338 consecutive patients with PHPT underwent parathyroidectomy with the excision of 400 abnormal glands (299 patients had a single adenoma, 23 had double adenoma, 14 had multiglandular hyperplasia, and 2 patients had no abnormal glands found in the neck). Curative resection as indicated by a normalization of serum calcium was achieved in 98.5% of patients with a 0.7% failure rate in initial operations and 5.9% failure in reoperative surgery. (2) Sestamibi-SPECT results: For solitary adenomas, sestamibi correctly lateralized the abnormal gland (sensitivity) in 96% of patients. In the 23 patients with double adenoma, 15 patients had both adenomas localized and the remaining 8 patients had at least 1 adenoma localized, 83% sensitivity. The sensitivity in patients with multigland disease was poor at only 45%. For patients undergoing initial operation, sensitivity of sestamibi was 87% compared to 92% in reoperative cases.
2002;13:149-157. Objective: To evaluate the ability of preoperative Techne-
tium-99m sestamibi with single photon emission computerized tomography (SPECT) to detect and localize abnormal parathyroid glands in patients with primary hyperparathyroidism (PHPT). Design: Prospective study from 1994 to 2000 of 338 patients (287 patients—first operation, 51 patients—reexploration) who underwent preoperative sestamibi SPECT and parathyroidectomy. Setting: The Department of Surgery, Yale University School of Medicine, and the Russell H. Morgan Department of Radiology and Radiological Science, Divisions of Nuclear Medicine and Radiation Health Sciences, New Haven, Connecticut. Tertiary Care Referral Center. Participants: A total of 338 consecutive patients, from 1994 to 2000 with biochemically proven PHPT, including 287 patients—first operation, and 51 patients—reoperative surgery for persistent or recurrent PHPT. Each patient underwent preoperative sestamibi-SPECT with initial and delayed scans (2.5
566
Conclusions: Technetium-99m sestamibi-SPECT is highly sensitive in detecting abnormal parathyroid glands in patients with single adenomas and works well in both initial and reoperative cases. However, the sensitivity for multigland disease is limited. This imaging modality is a very useful guide for “directed- minimal invasive” parathyroidectomy, but it requires a solid knowledge of parathyroid disease.
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