Small Pheochromocytomas Research Articles

8769 A Case of Metastatic and Catecholamine Producing Bilateral Pheochromocytoma in a Patient with RET Gene Mutation

Abstract Disclosure: R.A. Mayers: None. A. Szafran-Swietlik: None. RET mutation, observed in multiple endocrine neoplasia type 2 syndromes, is implicated in the development of pheochromocytomas of the kinase signaling cluster. Such tumors have lower metastatic risk and adrenergic production compared to the ones from the other clusters. We present a case of a metastatic, bilateral, small and biochemically active pheochromocytoma .This is the case of a 27-year-old man diagnosed with MEN 2A. He underwent total thyroidectomy due to medullary thyroid carcinoma at 2 years old. He was deemed cured and started on levothyroxine replacement. Since then, his plasma metanephrines by liquid chromatography-mass spectrometry and urine metanephrines done in a yearly basis as screening, were always within range of normality. Over the years, he developed substance abuse disorder with use of Kratom and methamphetamines and was admitted in a rehabilitation center multiple times in the last three years. During this time, the patient was reported to be slightly tachycardiac which was initially attributed to the use of substances. On the last year, an echocardiogram done as evaluation of his tachycardia showed an ejection fraction of 35%. Later, his annual metanephrine levels were markedly elevated (free metanephrine: 137 pg/mL and free normetanephrine : 157 pg/mL, normal ranges reported as less than 57 pg/mL and less than 148 pg/mL respectively). Abdominal tomography showed a right adrenal nodule of 1.2 cm and a left adrenal nodule of 1.6 cm, both with more than 10 Hounsfield Units. A Metaiodobenzylguanidine scan revealed a left adrenal nodule of 1.8 cm with diffuse radiotracer uptake, a right adrenal nodule of 1.2 cm with radiotracer activity and 3 subtle areas of focally increased radiotracer activity in the liver, two in left hepatic lobe and other in posterior right upper lobe. He underwent bilateral adrenalectomy with cortical sparring. Pathology results evidenced a left adrenal with a unifocal mass of 1.8 cm and a right adrenal with a mass of 1.2 cm , both without lymphovascular, capsular, local invasion or necrosis, a mitotic rate of 1-2 without atypical mitosis, not encapsulated with clean margins and positive for synaptophysin , chromogranin and S100 stains. An additional mass of 0.7 cm on the right adrenal gland was described with similar characteristics to the described before except for the involvement of the surgical margins. Currently on the literature there are different opinions regarding screening approach for pheochromocytomas in the presence of RET mutations. There are not strong recommendations regarding the use of imaging as a screening tool in this group of patients. It is important to consider that there are cases, as the one described in this report, that might present metastatic disease even with the presence of small pheochromocytomas that belong to the cluster of kinase signaling. Presentation: 6/3/2024

Comparison of the effectiveness of various methods for determining the level of metanephrines in the diagnosis of pheochromocytomas

Pheochromocytoma (PHEO) is a tumor from the chromaffin tissue of the adrenal medulla, capable of hyperproduction of catecholamines. The increased production of hormones by the tumor leads to catecholamine crises, which have a pathological effect on all organs and systems. In the primary diagnosis of pheochromocytomas, it is important to determine the level of the metabolite of catecholamines - metanephrines. Currently, in clinical practice, various methods are used to determine the level of this metabolite: in blood plasma or in urine, total or only free form, fractionated analysis or unfractionated. Comparison of the effectiveness of various methods for determining the level of metanephrines for the diagnosis of pheochromocytomas. A retrospective single-center cohort study was conducted on a sample of patients who were initially operated on for adrenal neoplasm at the Pirogov St. Petersburg State University High Medical Technology Clinic from November 2007 to December 2022 and who passed analysis to determine the level of blood or urine metanephrins before surgical treatment. The results of tests for metanephrine and tumor size were evaluated. 1088 patients with adrenal neoplasms who underwent surgical treatment were examined, of which 348 had histologically confirmed the presence of pheochromocytoma. Four types of metanephrine assays were compared: free fractionated plasma metanephrines (232 patients), unfractionated daily urine metanephrines (431 patients), fractionated total daily urine metanephrines (427 patients) and fractionated free daily urine metanephrines (178 patients). The greatest sensitivity was demonstrated by the analysis of free fractionated plasma methanephrines (95.4%). Unlike others, the sensitivity of this analysis did not decrease in the group of patients with small pheochromocytomas (3 cm or less). The greatest specificity was demonstrated by the analysis of unfractionated metanephrines in daily urine (97.8%), with the lowest sensitivity among all tests (67.6%). The study of fractionated total daily urine metanephrins showed good results of sensitivity and specificity, only slightly inferior to the best indicators, and the analysis of free daily urine metanephrins demonstrated unexpectedly low efficiency. There is a positive correlation between the level of metanephrine in the blood and the size of the tumor. Based on the data obtained, the preferred assays for the primary diagnosis of pheochromocytoma can be considered the determination of fractionated free plasma metanephrines and fractionated total daily urine metanephrines, which is consistent with relevant clinical recommendations. It was found that the size of the tumor correlates with the severity of an increase in the level of metanephrins determined by any of the described methods.

False iodine-131 MIBG scintigraphy findings in adrenal tumors: correlation with MR imaging

In this study, we report our experience regarding the occurrence of false radionuclide findings in adrenal iodine-131 MIBG scintigraphy. We present a total of five patients in which nuclear images were false negative or positive in three and two cases, respectively, according to the standard radionuclide established criteria. In particular, the three cases of false-negative MIBG images consisted of two patients with necrotic or cystic pheochromocytomas (Cases 1 and 3) and a patient with a small pheochromocytoma (Case 2); the two cases of false-positive MIBG imaging consisted of a patient with an adenoma showing intense tracer uptake and of a large primary necrotic carcinoma with heterogeneous tracer concentration.

Ectopic Cushing\u2019s syndrome associated with a pheochromocytoma in a dog: a case report

BackgroundEctopic Cushing’s syndrome (ECS) associated with malignant tumors, such as small cell lung carcinoma, bronchial carcinoids, and pheochromocytoma, has been reported in human medicine. However, ECS related to pheochromocytoma has not been reported in dogs.Case presentationAn 11-year-old castrated, male Scottish terrier was diagnosed with a left adrenal mass. Cushing’s syndrome was suspected based on clinical signs, including pot belly, polyuria, polydipsia, bilateral alopecia, recurrent pyoderma, and calcinosis cutis. Cushing’s syndrome was diagnosed on the basis of consistent clinical signs and repeated adrenocorticotropic hormone (ACTH) stimulation tests. In addition, tests for fractionated plasma metanephrine/normetanephrine suggested a pheochromocytoma. Unilateral adrenalectomy was performed after medical management with trilostane and phenoxybenzamine. Histopathology confirmed a diagnosis of pheochromocytoma without cortical lesions. After surgery, fractionated metanephrine/normetanephrine and the findings of low-dose dexamethasone suppression and ACTH stimulation tests were within the normal ranges without any medication. There were no clinical signs or evidence of recurrence and metastasis on thoracic and abdominal X-rays and ultrasonography up to 8 months after surgery.ConclusionsPheochromocytoma should be considered a differential diagnosis for dogs with Cushing’s syndrome with an adrenal tumor. A good prognosis can be expected with prompt diagnosis and surgical intervention.

Hemodynamic stability during adrenalectomy for pheochromocytoma: A case control study of posterior retroperitoneal vs lateral transperitoneal approaches.

Hemodynamic stability is one of the most critical aspects of adrenal surgery for pheochromocytoma. Few articles have evaluated the hemodynamic status of patients undergoing posterior retroperitoneal adrenalectomy (PRA) for pheochromocytoma. The aim of this study is to compare the intraoperative hemodynamic parameters between lateral transperitoneal adrenalectomy (TPA) and PRA in this groups of patients.This report describes a retrospective study of 53 pheochromocytoma patients who underwent endoscopic adrenalectomy via transperitoneal (22 patients) or posterior retroperitoneal (31 patients) approaches from January 2008 to March 2015. Data from these patients were compared to investigate the differences in hemodynamic parameters between the 2 approaches.Clinical parameters at presentation were similar between the 2 groups, except for tumor size, which was larger in the TPA group. The PRA group is associated with reduced operative time, blood loss, and length of hospital stay compared to TPA even after adjusting for the tumor size. There was greater BP fluctuations and higher maximum systolic and diastolic blood pressure (BP) within the TPA group compared to PRA during univariate analysis. This was however not significant after adjusting for tumor size. There was no difference in the intraoperative inotropic support requirement between the 2 groups.PRA is associated with stable intraoperative hemodynamic status, as well as favorable perioperative outcomes compared to TPA in patients with small pheochromocytomas.

A side-by-side evaluation of [18F]FDOPA enantiomers for non-invasive detection of neuroendocrine tumors by positron emission tomography

Neuroendocrine tumors (NETs) are an extremely heterogenous group of malignancies with variable clinical behavior. Molecular imaging of patients with NETs allows for effective patient stratification and treatment guidance and is crucial in selection of targeted therapies. Positron emission tomography (PET) with the radiotracer L-[18F]FDOPA is progressively being utilized for non-invasive in vivo visualization of NETs and pancreatic β-cell hyperplasia. While L-[18F]FDOPA-PET is a valuable tool for disease detection and management, it also exhibits significant diagnostic limitations owing to its inherent physiological uptake in off-target tissues. We hypothesized that the D-amino acid structural isomer of that clinical tracer, D-[18F]FDOPA, may exhibit superior clearance capabilities owing to a reduced in vivo enzymatic recognition and enzyme-mediated metabolism. Here, we report a side-by-side evaluation of D-[18F]FDOPA with its counterpart clinical tracer, L-[18F]FDOPA, for the non-invasive in vivo detection of NETs. In vitro evaluation in five NET cell lines, including invasive small intestinal neuroendocrine carcinomas (STC-1), insulinomas (TGP52 and TGP61), colorectal adenocarcinomas (COLO-320) and pheochromocytomas (PC12), generally indicated higher overall uptake levels of L-[18F]FDOPA, compared to D-[18F]FDOPA. While in vivo PET imaging and ex vivo biodistribution studies in PC12, STC-1 and COLO-320 mouse xenografts further supported our in vitro data, they also illustrated lower off-target retention and enhanced clearance of D-[18F]FDOPA from healthy tissues. Cumulatively our results indicate the potential diagnostic applications of D-[18F]FDOPA for malignancies where the utility of L-[18F]FDOPA-PET is limited by the physiological uptake of L-[18F]FDOPA, and suggest D-[18F]FDOPA as a viable PET imaging tracer for NETs.

Small Pheochromocytomas: Clinical, Diagnostic and Perioperative Issues of Disease

Small Pheochromocytomas: Clinical, Diagnostic and Perioperative Issues of Disease

Pheochromocytomatosis associated with a novel TMEM127 mutation.

Pheochromocytomatosis, a very rare form of pheochromocytoma recurrence, refers to new, multiple, and often small pheochromocytomas growing in and around the surgical resection bed of a previous adrenalectomy for a solitary pheochromocytoma. We here report a case of pheochromocytomatosis in a 70-year-old female. At age 64 years, she was diagnosed with a 6-cm right pheochromocytoma. She underwent laparoscopic right adrenalectomy, during which the tumor capsule was ruptured. At age 67 years, CT of abdomen did not detect recurrence. At age 69 years, she began experiencing episodes of headache and diaphoresis. At age 70 years, biochemical markers of pheochromocytoma became elevated with normal calcitonin level. CT revealed multiple nodules of various sizes in the right adrenal fossa, some of which were positive on metaiodobenzylguanidine (MIBG) scan. She underwent open resection of pheochromocytomatosis. Histological examination confirmed numerous pheochromocytomas ranging 0.1–1.2 cm in size. Next-generation sequencing of a panel of genes found a novel heterozygous germline c.570delC mutation in TMEM127, one of the genes that, if mutated, confers susceptibility to syndromic pheochromocytoma. Molecular analysis showed that the c.570delC mutation is likely pathogenic. Our case highlights the typical presentation of pheochromocytomatosis, a rare complication of adrenalectomy for pheochromocytoma. Previous cases and ours collectively demonstrate that tumor capsule rupture during adrenalectomy is a risk factor for pheochromocytomatosis. We also report a novel TMEM127 mutation in this case.Learning points:Pheochromocytomatosis is a very rare form of pheochromocytoma recurrence.Pheochromocytomatosis refers to new, multiple and often small pheochromocytomas growing in and around the surgical resection bed of a previous adrenalectomy for a solitary pheochromocytoma.Tumor capsule rupture during adrenalectomy predisposes a patient to develop pheochromocytomatosis.Surgical resection of the multiple tumors of pheochromocytomatosis is recommended.Pheochromocytoma recurrence should prompt genetic testing for syndromic pheochromocytoma.

Assessment of clinical and radiologic differences between small and large adrenal pheochromocytomas

ObjectiveTo evaluate differences in clinical and radiologic features of small and large pheochromocytomas. Materials and methodsThis study included 39 patients with adrenal pheochromocytomas. Several clinical and radiologic features were statistically analyzed and compared between small and large pheochromocytomas. ResultsNo significant differences were found in clinical features between them. Small pheochromocytomas had more relatively homogeneous attenuation although large pheochromocytomas had more cystic or necrotic changes. ConclusionsPheochromocytomas tend to have different CT imaging features mimicking other tumors according to the size of the tumors. However, clinical features, CT imaging characteristics, and radioisotope activity are not different between small and large pheochromocytomas.

Case of a patient with a small pheochromocytoma

Case of a patient with a small pheochromocytoma

Small pheochromocytomas: clinical, diagnostic and perioperative issues of disease

Background. Pheochromocytoma (P) is considered as relatively rare adrenal tumor with clinicalmanifestation after reaching the size of 4-6 cm. Meanwhile, it’s supposing that small P can be not rare found within different diagnostic imagination. Some of these, being clinically silent, can cause serious cardiovascular risk undergoing unrelated medical procedures.Aim. To compare clinical course, peculiarities of diagnostics and treatment of small P (less than 3 cm) and bigger P on the basement of own clinical experience among patients underwent adrenalectomy on proven or suspected P.Results and discussion. Group of small P was comprised by 14 patients aged from 21 to 62 years with mean size of tumor 23 mm (range: 4-29 mm). Control group consisted of 35 patients corresponded on age (23-75 years) with mean diameter of P 56 mm (range: 30-127 mm). Small P comprised 8% of all 175 patients with P operated on during the last 19 years in endocrine surgery hospital from the whole group of 967 adrenalectomies. Prevalence of women and right side lesions characterized both groups. Principal differences between groups were less prominent clinical activity (any symptoms were detected in half of small P) with less level of catecholamines and metanefrines in lab examination of serum and urea of small P patients. Computed tomography was often the first but not targeted diagnostic step. Silent clinical course was the main reason that small P have been discovered predominantly incidentally with no blockage of alpha-adrenergic receptors before surgery in 6 of 14 patients. Most of them had a critical elevation of blood pressure during operation (43% against 9% in control group). Despite small size and mild catecholamine excess in group of small P, 4 from 14 patients (29%) experienced some episodes of potentially harmful hypertensive crisis during unrelated medical procedures in the past. All small P were treated with successful laparoscopic adrenalectomy. Most of patients became free from arterial hypertension.Conclusion. Small P may carry actual risk of unexpected hypertensive crisis during any unrelated medical procedures. The clinical course of small P is hidden in half of patients and laboratory investigations are interfered with mild elevation of biochemical markers. Laparoscopic adrenalectomy is reliable and safe mode of treatment of small P in case of adequate preparation with alpha-blockers.

43 HIGH PREOPERATIVE PLASMA EPINEPHRINE LEVEL IS A RISK FACTOR FOR INTRA OPERATIVE HYPERTENSION DURING LAPAROSCOPIC ADRENALECTOMY FOR PHEOCHROMOCYTOMA

You have accessJournal of UrologyAdrenal1 Apr 201343 HIGH PREOPERATIVE PLASMA EPINEPHRINE LEVEL IS A RISK FACTOR FOR INTRA OPERATIVE HYPERTENSION DURING LAPAROSCOPIC ADRENALECTOMY FOR PHEOCHROMOCYTOMA Junichi Inokuchi, Katsunori Tatsugami, Kentaro Kuroiwa, Akira Yokomizo, and Seiji Naito Junichi InokuchiJunichi Inokuchi Fukuoka, Japan More articles by this author , Katsunori TatsugamiKatsunori Tatsugami Fukuoka, Japan More articles by this author , Kentaro KuroiwaKentaro Kuroiwa Fukuoka, Japan More articles by this author , Akira YokomizoAkira Yokomizo Fukuoka, Japan More articles by this author , and Seiji NaitoSeiji Naito Fukuoka, Japan More articles by this author View All Author Informationhttps://doi.org/10.1016/j.juro.2013.02.1419AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookTwitterLinked InEmail INTRODUCTION AND OBJECTIVES Laparoscopic adrenalectomy (LA) is a safe and feasible approach to adrenal pathology, providing the patients with the benefits of minimally invasive surgery. In contrast to other adrenal tumors, pheochromocytoma was once considered as a contraindication for LA. Recently improved peri-operative management and advances in laparoscopic surgery have resulted in LAs becoming the standard of care for small pheochromocytomas. However, there are still some debates for the safety and feasibility of LA for large pheochromocytoma because of the difficult techniques and the possibility of uncontrollable hypertensive crises, hemorrhage, and malignancy. The aim of this study was to evaluate the validity of LA for pheochromocytoma and to investigate the risk factors that affect the intra-operative hypertension. METHODS Clinical data were retrospectively reviewed. From April 1997 to March 2012, 253 LAs were performed in our hospital. 56 cases were diagnosed as pheochromocytoma. The operative data and characteristics such as gender, age, laterality, tumor size, operating time, blood loss and preoperative catecholamine level in plasma and urine were compared to the intra-operative systolic blood pressure (SBP) variations. RESULTS In a comparison of operative data during LAs for pheochromocytoma or other adrenal tumors, we detected significant differences in operative time and blood loss. However, operative time and blood loss were improved significantly in recent cases compared with initial cases of LAs for pheochromocytoma. Therefore, no significant difference was detected in comparison of these operative data during LAs performed only recently. Although the intra-operative SBP rose to 180 mmHg or more in 30 cases (53.6%) and 200 mmHg or more in 16 cases (28.6%) in LAs for pheochromocytoma, no patient experienced a hypertensive crisis with uncontrollable high blood pressure. In a comparison of SBP (<200 vs. >200 mmHg) showed that the tumor size was significant risk factor for intra-operative hypertension (P=0.045). Furthermore patients with high preoperative plasma epinephrine level greater than 3 fold of upper normal limit were associated with higher incidence of intra-operative hypertension (P=0.003). CONCLUSIONS Laparoscopic adrenalectomy is not contraindicated for pheochromocytoma and can be performed safely. The tumor size and high pre-operative plasma epinephrine level might be risk factors for the intra-operative high SBP. © 2013 by American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetails Volume 189Issue 4SApril 2013Page: e17 Advertisement Copyright & Permissions© 2013 by American Urological Association Education and Research, Inc.MetricsAuthor Information Junichi Inokuchi Fukuoka, Japan More articles by this author Katsunori Tatsugami Fukuoka, Japan More articles by this author Kentaro Kuroiwa Fukuoka, Japan More articles by this author Akira Yokomizo Fukuoka, Japan More articles by this author Seiji Naito Fukuoka, Japan More articles by this author Expand All Advertisement Advertisement PDF downloadLoading ...

Unexpected Small Urinary Bladder Pheochromocytoma: A Nonspecific Presentation

Objectives. Pheochromocytoma of the urinary bladder is an extremely rare tumor that typically presents with a hypertensive crisis during micturition. Preoperatively, it may be misdiagnosed due to nonspecific symptomatology, physical, and radiologic findings. Method. We report a case of unsuspected small pheochromocytoma which was incidentally found by CT scan and confirmed by the histological aspects after transurethral resection in a 63-year-old woman. Here, we have described the clinical presentation, physical findings, laboratory investigations, and treatment provided in our case. We have also included radiological images and histopathology slides with input from both radiologists and pathologists. Surgical management and postoperative follow-up are discussed, as are details of previous published data. Results. After undergoing surgical treatment (transurethral resection), our patient is asymptomatic, with complete resolution of her pathology. Conclusion. Diagnosis is difficult before histopathological examination and should be considered in patients with no risk factors for usual bladder tumor. Our purpose is to raise clinician's awareness for this condition so that they will be more likely to diagnose it. This will facilitate prompt diagnosis and treatment and especially prevent complications due to pheochromocytoma which may be severe.

Safety and Feasibility of Laparoscopic Resection for Large (≥ 6 CM) Pheochromocytomas Without Suspected Malignancy

To determine whether laparoscopic adrenalectomy in patients without radiologic evidence of cancer compromises the perioperative and long-term outcomes in patients with large (≥ 6 cm) pheochromocytomas. We analyzed a prospective adrenal database of consecutive patients who underwent adrenalectomy at our institution between September 2000 and September 2010. Patients with diagnosed pheochromocytoma who underwent laparoscopic adrenalectomy were included. Patients with tumors smaller than 6 cm were compared with those presenting with tumors 6 cm or larger. One hundred fifty-seven patients underwent adrenalectomy, and there were 32 catecholamine-secreting tumors. Of the 33, 7 were excluded from the study because of open surgery. Thus, 25 patients presented with 26 pheochromocytomas and underwent laparoscopic adrenalectomy. Thirteen of the 25 patients (52%) were women. Mean age (± standard error of the mean) was 53 ± 3 years. Mean tumor size was 5.2 ± 0.5 cm, and 11 pheochromocytomas (42%) were 6 cm or larger. Tumor size was significantly different between the large pheochromocytoma and the small pheochromocytoma groups (7.6 ± 0.4 vs 3.6 ± 0.4 cm, P<.001), but there was no significant difference in intraoperative complications, estimated blood loss, cancer diagnosis, or recurrence. The length of stay was comparable between the 2 cohorts, and there were no incidents of capsular invasion or adverse cardiovascular events. Laparoscopic adrenalectomy of pheochromocytomas larger than 6 cm is feasible and safe with comparable results to those achieved with laparoscopic adrenalectomy in patients with smaller pheochromocytomas.

Pheochromocytoma in Multiple Endocrine Neoplasia Type 2A

An 123I metaiodobenzylguanidine (MIBG) planar image showed abnormal uptake in the left adrenal gland and thyroid in a 24-year-old woman with multiple endocrine neoplasia type 2A. 131I MIBG showed abnormal uptake in the thyroid but not in the adrenal gland. Abdominal CT with 2-mm thin slices demonstrated a normal adrenal gland. Postoperative pathologic findings were consistent with medullary carcinoma in both thyroid lobes and a small 1-mm pheochromocytoma in the left adrenal gland. 123I MIBG could clearly show the small pheochromocytoma, which was negative in thin-slice CT and equivocal in 131I MIBG.

Small Pheochromocytomas: Significance, Diagnosis, and Outcome

To address the unique challenges in the diagnosis and management of small pheochromocytomas, the authors performed a retrospective study of 24 patients with small pheochromocytomas (≤ 3 cm) treated between 1995 and 2011, using 51 patients with larger pheochromocytomas (> 3 cm) as controls. Patient demographics were similar between the two groups. Small pheochromocytomas presented mainly as adrenal mass and hypertension and their major complication was hypertensive crisis during unrelated procedures in 4 patients (17%). Hypertension was improved in a quarter of the patients after pheochromocytoma resection. The biochemical marker levels in patients with small pheochromocytomas were generally lower than those with larger tumors and half of the patients exhibited modestly elevated or normal levels. The authors conclude that small pheochromocytomas are frequent and may not contribute to baseline hypertension, but can cause hypertensive crisis during unrelated medical procedures. Small pheochromocytomas should be removed to prevent hypertensive crisis and future complications of pheochromocytoma.

Postoperative Outcomes of Familial Adrenal Pheochromocytoma1

ABSTRACT There are no current guidelines for the management of familial pheochromocytoma (FP). We tried to determine the optimal management of patients with FP. Among 191 patients with pheochromocytoma who underwent surgical resection between 1979 and 2010, there were 18 FP (13 different kindreds; 11 females/7 males; mean age at initial operation: 38.7 years). The 18 FP cases comprised 10 with MEN2A, 2 with MEN2B, 4 with von Hippel-Lindau disease, and 2 with FP only, and all pheochromocytomas were of adrenal origin. The number of probands and family members was 9 and 9 respectively. Mean tumor size was 6.4 cm in diameter. Simultaneous bilateral adrenalectomy was performed in 6 patients, and unilateral adrenalectomy was performed as the initial surgery in 12 patients. A metachronous contralateral adrenalectomy was performed in 3 patients, 90, 236 and 312 months after the primary operation, respectively. None of the patients received partial adrenalectomy. Among another 9 patients with unilateral adrenalectomy, contralateral pheochromocytomas were suspected in 4 cases at the initial operation. However, none of these contralateral lesions developed severe symptoms or tumor enlargement during a median follow-up of 116 months. In the remaining 5 patients, pheochromocytoma did not develop in the contralateral adrenals over a median follow-up of 80.5 months. Bilateral lesions of adrenal pheochromocytoma in familial cases occurred in 78% of cases (14/18); 9 patients (including 4 with contralateral pheochromocytoma) did not require a contralateral adrenalectomy during a median follow-up of 119 months. No patients have suffered from Addisonian crisis. The ipsilateral adrenalectomy and follow-up of contralateral small pheochromocytoma is one of the management options to preserve adrenocortical function in FP patients.

Laparoscopic adrenalectomy for large pheochromocytoma

To determine the feasibility and safety of laparoscopic adrenalectomy for large adrenal pheochromocytomas as although the safety and efficacy for small pheochromocytomas is relatively well documented its use for large pheochromocytomas is controversial because of a perceived increased risk of malignancy. All pheochromocytomas (>8 cm) managed prospectively using a laparoscopic approach between January 2002 and April 2006 were included. Blood loss, operative duration, complications, and hospital stay were assessed. In all, 11 consecutive patients underwent laparoscopic adrenalectomy for large pheochromocytomas. The adrenal sizes were 8-15 cm. The mean blood loss was <100 mL, the mean operative duration was 145 min and the mean postoperative stay was 3.6 days. Only one patient had an intraoperative hypertensive crisis, and recovery was uneventful in all. Laparoscopic transperitoneal, lateral adrenalectomy is safe and efficient means of resecting large (>8 cm) adrenal pheochromocytomas. Although intraoperative catecholamine surges are a cause of major concern, early clipping and dividing of the adrenal vein helps to avoid a catecholamine-induced hypertensive crisis.

Analysis of large versus small pheochromocytomas: Operative approaches and patient outcomes

Analysis of large versus small pheochromocytomas: Operative approaches and patient outcomes

Analysis of large versus small pheochromocytomas: Operative approaches and patient outcomes

Laparoscopic adrenalectomy for small pheochromocytomas, although challenging, is widely accepted. However, its application to pheochromocytomas larger than 6 cm is questioned due to concerns of malignancy and case complexity. Our aim was to examine the impact of pheochromocytoma tumor size (>/=6 cm vs <6 cm) on operative approach and postoperative patient outcomes. A retrospective review of adrenalectomies performed at 3 university hospitals over 1 decade was analyzed. All pheochromocytomas were identified and then divided based on size into large (>/=6 cm) and small (<6 cm) groups. We examined patient and tumor demographics, pathologic diagnosis, operative approach (laparoscopic vs open), postoperative complications, and biochemical cure rates. Data were analyzed using the Student t test and Fisher exact test with a P value <.05 considered significant. From 1995 to 2005, 65 pheochromocytomas were resected. Of the total, 38% (n = 25) tumors were >/=6 cm and 62% (n = 40) were <6 cm. For the large tumors, 1 out of 25 (4%) was malignant, whereas no small tumors were malignant. There was no statistically significant increased risk of malignancy in tumors >/=6 cm in size (P = .31). Initial operative approach was based on surgeon preference. Of the adrenalectomies performed, 88% were laparoscopic, with 3 of 25 (12%) large tumors requiring conversion from laparoscopic to open for intraoperative bleeding. None of the small tumors required conversion. No major postoperative complications (eg, stroke or myocardial infarction) occurred in either group. Minor complications (eg, wound infections and hematomas) were noted in 16% of large tumors and 12.5% of small tumors (P = .45). A total of 96% (24 of 25) patients with large tumors and 100% with small tumors showed postoperative biochemical cure. Tumor recurrence was noted in 1 patient with a tumor <6 cm. Pheochromocytomas >/=6 cm pose a challenge for laparoscopic resection, and concerns have been raised about the validity of this operative approach. This study demonstrates that there is no significant difference in the rate of malignancy for pheochromocytomas >/=6 cm versus <6 cm. There also were no significant differences identified in complication rates, postoperative biochemical cures, or tumor recurrence rates between these groups. Laparoscopic resection of pheochromocytomas can be safely accomplished regardless of size in centers with surgeons experienced in these procedures.