Paraganglioma in pregnancy is an extremely rare condition and its diagnosis is often delayed because the clinical symptoms can mimic those of preeclampsia or gestational hypertension. Here, we report the case of a 32-year-old, gravida 2, para 1 woman who presented with severe headache, palpitation, and sweating at 37 weeks' gestation. Although emergent cesarean section was performed on the assumption of severe preeclampsia, blood pressure fluctuated and heart rate remained tachycardiac. We suspected that she might have thromboembolic lesion in the chest or pheochromocytoma. Chest and abdominal computed tomography revealed a 4 cm mass in the left para-aortic space. Serum and urinary catecholamine levels were found to be significantly increased. She underwent laparoscopic mass removal and the pathology confirmed paraganglioma. When typical paroxysmal hypertension is accompanied by headache, palpitation, and sweating during pregnancy, adrenal tumors should be considered.
Adult ; Blood Pressure ; Cesarean Section ; Diagnosis ; Female ; Headache ; Heart Rate ; Humans ; Hypertension ; Hypertension, Pregnancy-Induced ; Paraganglioma ; Paraganglioma, Extra-Adrenal* ; Pathology ; Pheochromocytoma ; Pre-Eclampsia* ; Pregnancy ; Sweat ; Sweating ; Thorax

Despite all technical progress in modern diagnostic methods and treatment modalities of pheochromocytoma/paraganglioma, early consideration of the presence of these tumors remains the pivotal link towards the best possible outcome for patients. A timely diagnosis and proper treatment can prevent the wide variety of potentially catastrophic cardiovascular complications. Modern biochemical testing should include tests that offer the best available diagnostic performance, measurements of metanephrines and 3-methoxytyramine in plasma or urine. To minimize false-positive test results particular attention should be paid to pre-analytical sampling conditions. In addition to anatomical imaging by computed tomography (CT) or magnetic resonance imaging, new promising functional imaging modalities of photon emission tomography/CT using with somatostatin analogues such as ⁶⁸Ga-DOTATATE (⁶⁸Ga-labeled DOTA(0)-Tyr(3)-octreotide) will probably replace ¹²³I-MIBG (iodine-123-metaiodobenzylguanidine) in the near future. As nearly half of all pheochromocytoma patients harbor a mutation in one of the 14 tumor susceptibility genes, genetic testing and counseling should at least be considered in all patients with a proven tumor. Post-surgical annual follow-up of patients by measurements of plasma or urinary metanephrines should last for at least 10 years for timely detection of recurrent or metastatic disease. Patients with a high risk for recurrence or metastatic disease (paraganglioma, young age, multiple or large tumors, genetic background) should be followed up lifelong.
Catecholamines ; Counseling ; Diagnosis ; Follow-Up Studies ; Genetic Testing ; Humans ; Magnetic Resonance Imaging ; Metanephrine ; Paraganglioma* ; Pheochromocytoma* ; Plasma ; Recurrence ; Somatostatin

Approximately 10–15% of pheochromocytomas are malignant. There are insufficient histologic criteria for the diagnosis of malignant pheochromocytoma. Thus, the term malignant pheochromocytoma is restricted to tumors with local invasion or distant metastases. We experienced a case of malignant pheochromocytoma recurred with spinal metastasis 4 years after the surgery for huge benign pheochromocytoma. A 68-year-old female was admitted for trunk and back pain. The patient had a history of surgery 4 years ago for a 10.0×9.5×7.5 cm sized benign pheochromocytoma at the left adrenal gland. A thoracolumbar magnetic resonance imaging showed a tumor in the 7th thoracic vertebral body and a 24-hour urinary norepinephrine increased, suggesting metastatic recurrence of malignant pheochromocytoma. After metastasectomy in the 7th thoracic vertebral body, urine catecholamine was normalized and pain also disappeared. However, a metastatic lesion was found in the paraaortic area on a follow-up abdominal computed tomography scan and an additional metastasectomy was performed. The pathology confirmed the diagnosis of metastatic pheochromocytoma in the paraaortic lymph nodes. She is supposed to be treated with adjuvant iodine 131-meta-iodobenzylguanidine therapy. In our experience, a close follow-up should be considered in patients who had a huge benign pheochromocytoma due to the possibility of malignant metastases.
Adrenal Gland Neoplasms ; Adrenal Glands ; Aged ; Back Pain ; Catecholamines ; Diagnosis ; Female ; Follow-Up Studies ; Humans ; Iodine ; Lymph Nodes* ; Magnetic Resonance Imaging ; Metastasectomy ; Neoplasm Metastasis* ; Norepinephrine ; Pathology ; Pheochromocytoma* ; Recurrence ; Spine*

Approximately 10–15% of pheochromocytomas are malignant. There are insufficient histologic criteria for the diagnosis of malignant pheochromocytoma. Thus, the term malignant pheochromocytoma is restricted to tumors with local invasion or distant metastases. We experienced a case of malignant pheochromocytoma recurred with spinal metastasis 4 years after the surgery for huge benign pheochromocytoma. A 68-year-old female was admitted for trunk and back pain. The patient had a history of surgery 4 years ago for a 10.0×9.5×7.5 cm sized benign pheochromocytoma at the left adrenal gland. A thoracolumbar magnetic resonance imaging showed a tumor in the 7th thoracic vertebral body and a 24-hour urinary norepinephrine increased, suggesting metastatic recurrence of malignant pheochromocytoma. After metastasectomy in the 7th thoracic vertebral body, urine catecholamine was normalized and pain also disappeared. However, a metastatic lesion was found in the paraaortic area on a follow-up abdominal computed tomography scan and an additional metastasectomy was performed. The pathology confirmed the diagnosis of metastatic pheochromocytoma in the paraaortic lymph nodes. She is supposed to be treated with adjuvant iodine 131-meta-iodobenzylguanidine therapy. In our experience, a close follow-up should be considered in patients who had a huge benign pheochromocytoma due to the possibility of malignant metastases.
Adrenal Gland Neoplasms ; Adrenal Glands ; Aged ; Back Pain ; Catecholamines ; Diagnosis ; Female ; Follow-Up Studies ; Humans ; Iodine ; Lymph Nodes ; Magnetic Resonance Imaging ; Metastasectomy ; Neoplasm Metastasis ; Norepinephrine ; Pathology ; Pheochromocytoma ; Recurrence ; Spine

A 9-year-old boy presented with increased sweating and abdominal pain. His mother and uncle had been diagnosed with bilateral pheochromocytoma and medullary thyroid carcinoma. Magnetic resonance imaging of the boy's abdomen revealed a 7.5 cm×7.0 cm×6.0 cm mass with a thick peripheral enhancing wall and fluid-fluid level at the right suprarenal region. His ¹²³I-meta-iodobenzylguanidine (MIBG) scan showed a large mass with increased MIBG uptake in the right adrenal gland. The levels of serum norepinephrine, urine epinephrine/norepinephrine, metanephrine, and vanillylmandelic acid were elevated. He, his mother, and two sisters tested positive for the known mutation of multiple endocrine neoplasia type 2A, Cys634Tyr in RET proto-oncogene. Laparoscopic tumor excision and right adrenalectomy were performed. Final diagnosis was pheochromocytoma with malignant behavior, based on adrenal gland scoring scale. However, there was no overt metastasis. After surgery, his symptoms resolved and abnormal laboratory tests were normalized.
3-Iodobenzylguanidine ; Abdomen ; Abdominal Pain ; Adrenal Glands ; Adrenalectomy ; Child ; Diagnosis ; Humans ; Magnetic Resonance Imaging ; Male ; Metanephrine ; Mothers ; Multiple Endocrine Neoplasia Type 2a ; Multiple Endocrine Neoplasia ; Neoplasm Metastasis ; Norepinephrine ; Pheochromocytoma ; Proto-Oncogenes ; Siblings ; Sweat ; Sweating ; Thyroid Neoplasms ; Vanilmandelic Acid

Rarely, a paraganglioma can lead to disastrous cardiac complications such as heart failure, cardiomyopathy, or myocardial infarction and inflammatory complications such as sepsis. We describe a 41-year-old man who developed severe dilated cardiomyopathy with cardiogenic shock and sepsis who had a paraganglioma in the retroperitoneum. Echocardiography showed extensive global hypokinesia with severe left ventricular systolic dysfunction. In addition, leukocytosis with elevated inflammatory markers and positive blood cultures indicated sepsis. Abdominal enhanced computed tomography and magnetic resonance imaging showed a large heterogeneous mass with cystic changes in the retroperitoneum. Serum and urine samples indicated elevated levels of catecholamine and its metabolites, and a 131I-meta-iodobenzylguanidine (MIBG) scan indicated uptake of MIBG. After intensive medical treatment and surgical removal of the paraganglioma, the dilated cardiomyopathy and sepsis resolved. The diagnosis of paraganglioma should be considered in patients presenting with acute heart failure or sepsis of nonspecific origin.
3-Iodobenzylguanidine ; Adult ; Cardiomyopathies ; Cardiomyopathy, Dilated* ; Diagnosis ; Echocardiography ; Heart Failure ; Humans ; Hypokinesia ; Leukocytosis ; Magnetic Resonance Imaging ; Myocardial Infarction ; Paraganglioma* ; Pheochromocytoma ; Sepsis* ; Shock, Cardiogenic*

PURPOSE: To compare outcomes of robotic adrenalectomy with conventional laparoscopic adrenalectomy. MATERIALS AND METHODS: This retrospective study included 63 patients who underwent robotic or laparoscopic adrenalectomy between March 2005 and April 2016, with all operations performed using a transperitoneal approach. Outcomes were compared in the 29 patients who underwent robotic adrenalectomy and the 34 who underwent conventional laparoscopic adrenalectomy. RESULTS: Mean age (53.1±12.2 years vs. 51.4±15.1 years, p=0.631) and body mass index (25.9±3.8 kg/m2 vs. 25.2±3.5 kg/m2, p=0.461) were similar in the robotic and laparoscopic groups. A significant percentage of patients in the robotic group had undergone prior abdominal surgery (38% vs. 12%, p=0.015). Mean tumor sizes (3.0±1.5 cm vs. 3.7±2.7 cm, p=0.134) and the percentage of incidentalomas (75.9% vs. 73.5%, p=0.354) were similar in the robotic and laparoscopic groups. There were no statistical significance between-group differences in tumor size, operative time, estimated blood loss, perioperative hemoglobin change, length of hospital stay, and complication rates. Pathologic diagnosis showed that pheochromocytoma (21% vs. 3%) and metastatic tumor (24% vs. 6%) were more frequent in the robotic than in the laparoscopic group (p=0.019). Subgroup analysis of patients with low tumor volume (≤6.8 cm3) showed that operation time was significantly shorter in the robotic than in the laparoscopic group (p=0.045). CONCLUSIONS: Robotic adrenalectomy is feasible, with outcomes comparable to those of laparoscopic adrenalectomy.
Adrenal Glands ; Adrenalectomy* ; Body Mass Index ; Diagnosis ; Humans ; Length of Stay ; Operative Time ; Pheochromocytoma ; Retrospective Studies ; Tumor Burden

Adrenalectomy ; Female ; Humans ; Pheochromocytoma ; diagnosis ; surgery ; Tachycardia, Ventricular ; diagnosis ; Tomography, X-Ray Computed

Multiple endocrine neoplasia type 2B (MEN 2B) is an autosomal dominant disorder characterized by medullary thyroid cancer, pheochromocytoma, neuroma and Marfanoid feature. Medullary thyroid cancer occurs in more than 95% patients of MEN 2B and increases mortality. So, the early diagnosis of multiple endocrine neoplasia is very important, because in the early diagnosed and treated medullary thyroid cancer, the prognosis is excellent. This is a case of multiple endocrine neoplasia type 2B that diagnosed early by conjunctival neuroma. A 15-year-old female patient was presented with both conjunctival masses that occurred 6 months ago. The excisional biopsy revealed conjunctival neuroma. The multiple endocrine tumor was suspected, further evaluation was performed. Medullary thyroid cancer was confirmed by thyroid ultrasound and fine needle aspiration. Finally, MEN type 2B was confirmed by a RET mutation genetic testing.
Adolescent ; Biopsy ; Biopsy, Fine-Needle ; Early Diagnosis ; Female ; Genetic Testing ; Humans ; Male ; Mortality ; Multiple Endocrine Neoplasia Type 2b* ; Multiple Endocrine Neoplasia* ; Neuroma* ; Pheochromocytoma ; Prognosis ; Thyroid Gland ; Thyroid Neoplasms ; Ultrasonography

Pheochromocytoma is an extremely rare tumor in pregnant women with potentially fatal consequences. We report a case of pregnant woman at 22 weeks of gestation with pheochromocytoma. A correct diagnosis on the basis of differential clues from severe preeclampsia was obtained and laparoscopic adrenalectomy was performed.
Adrenalectomy* ; Diagnosis ; Female ; Humans ; Laparoscopy ; Pheochromocytoma* ; Pre-Eclampsia ; Pregnancy* ; Pregnant Women