Feocromocitoma maligno. A propósito de tres casos / Malignant Pheochromocytoma. As Regards of Three Cases

Introducción: Los feocromocitomas son tumores que se desarrollan a partir de las células cromafines localizadas en la médula suprarrenal, ganglios simpáticos y parasimpáticos. La distinción entre tumores benignos y malignos es compleja, pues no existen marcadores que puedan discriminar esta diferencia con alta sensibilidad y especificidad. Objetivo: Presentar tres casos clínicos de feocromocitoma maligno, diagnosticados y/o tratados en la sala de hospitalización de adultos del INEN. Método: Se revisaron las historias clínicas y se resumieron los datos. Presentación de los casos: Los 3 pacientes eran del sexo masculino, con edades entre 41 y 51 años al diagnóstico del tumor primario. De ellos, dos tenían una hipertensión arterial controlada y el tercero se comenzó a estudiar por crisis paroxísticas de hipertensión; dos tenían antecedentes de diabetes mellitus y no se demostraron evidencias en ningún caso de lesiones en órganos diana por la hipertensión. En los tres pacientes el tumor se localizó en la suprarrenal derecha. Solo en uno de los casos, la etiología maligna fue informada en el estudio anatomopatológico. En los otros dos pacientes, la malignidad se diagnosticó a punto de partida de lesiones metastásicas, años después de la adrenalectomía, en un caso de cadera y en el otro, de pulmón. Conclusiones: El feocromocitoma maligno es una enfermedad infrecuente de la edad mediana y el diagnóstico de su naturaleza es complejo. Se debe sospechar la aparición de enfermedad metastásica ante la reaparición de los síntomas que motivaron el estudio inicial en el paciente, incluso, años después de la adrenalectomía(AU)

Introduction: Pheochromocytomas are tumors developed from chromaffin cells located in the suprarenal medulla, as well as in sympathetic and parasympathetic ganglia. The distinction between benign and malignant tumors is complex, since there are no markers to discriminate this difference with high sensitivity and specificity. Objective: To present three clinical cases of malignant pheochromocytoma, diagnosed and/or treated in the adult hospitalization ward at the National Institute of Endocrinology. Method: The medical records were reviewed and the data was summarized. Case presentation: The three patients were male and aged between 41 and 51 years at the diagnosis of the primary tumor. Of them, two had controlled arterial hypertension and the third began to be studied for paroxysmal hypertension crises; two had a history of diabetes mellitus and no evidence of target-organ lesions due to hypertension was shown in any case. In the three patients, the tumor was located in the right suprarenal gland. Only in one of the cases, malignant etiology was reported in the pathological study. In the other two patients, the malignancy was diagnosed at the starting point of metastatic lesions, years after adrenalectomy: two respective hip and lung cases. Conclusions: Malignant pheochromocytoma is a rare disease that appears at middle age and whose diagnosis is complex, due to its etiological nature. The appearance of metastatic disease should be suspected, given the reappearance of the symptoms that motivated the initial study in the patient, even years after adrenalectomy(AU)

An Experience of Embolization of Multiple Recurrence Pheochromocytoma Nodules

@#Recurrence of pheochromocytoma is quite common. We present a case report of a malignant pheochromocytoma with recurrent nodules in right paranephric and posterior-lateral abdominal wall, which underwent transarterial embolization of the nodules.

Clinical efficacy of 131I-MIBG therapy in malignant pheochromocytoma/malignant paraganglioma / 中华泌尿外科杂志

Objective To evaluate the efficacy and safety of 131I-metaiodobenzylguanidine (131 I-MIBG) in treatment of malignant pheochromocytoma/malignant paraganglioma (MPHEO/MPGL).Methods The clinical data of 96 cases of MPHEO/MPGL (60/36) treated with 131I-MIBG between December 1998 and April 2014 were retrospectively reviewed.Among them,the malignant pheochromocytoma was found in 60 cases and malignant paraganglioma was found in 36 cases.Seventy-eight patients (81.2％)presented initially with hypertension,whereas 18 patients (18.8％) presented adrenal incidentaloma.Before 131I-MIBG treatment,24 h urinary norepinephrine was (409.5± 127.2) nmol,24 h urinary dopamine was (99.3±41.1) nmol,24 h urine adrenaline was (1 409.9±336.0) nmol.Before treatment,the compound iodine solution was given to each one.Then,all patients were given an initial course of 131I-MIBG therapy (5.55,7.40 GBq).Subsequent 131I-MIBG treatment (5.55,7.40 GBq) was undertaken every three to six months.The patients got symptomatic,hormonal or radiological response underwent sbsequent 131I-MIBG therapy (3.70,5.55 GBq) every year.All patients underwent clinical symptoms (headache,palpitate,sweating,hypertension),biochemical (24 h urine catecholamin) and radiological evaluation (CT/MRI) within 6 months to evaluate the efficacy and safety of 131I-MIBG treatment.Results After one to eleven sessions of 131 I-MIBG treatment,in total,266 doses of 131 I-MIBG were administered,average dose was 6.49 GBq.22.9％ of patients demonstrated radiological partial response (≥ 50％ reduction in tumor size) after first or repeated 131 I-MIBG treatment.Eleven cases (11.5％) achieved clinical complete response,41 cases (42.7％) achieved clinical partial response and 23 cases (24.0％) maintained the stable clinic symptoms.After treatment,24 h urinary norepinephrine (164.3±71.6) nmol and dopamine (49.7±24.7) nmol showed significantly decline,compared with those before treatment (P＜ 0.05).While,24 h urinary epinephrine (1 354.7±433.4) nmol had no obvious change (P＞0.05).No life-threatening adverse events were reported,but 2 MPGL patients developed transient leucopenia or thrombocytopenia after four and five times 131 I-MIBG treatment,respectively.Conclusions Treatment with repeated low dose 131I-MIBG is well tolerated and effective in controlling the progression and alleviating the clinical symptoms.The 131I-MIBG therapy is an effective and safe treatment modality for MPHEO/MPGL.

Feocromocitoma maligno: comunicación de 2 casos tratados con cirugía de mínima invasión / Malignant pheochromocytoma treated with minimally invasive surgery

Introducción: El Feocromocitoma es un tumor de células cromafines productor de catecolaminas. Su variedad maligna es muy poco frecuente. Objetivo: Describir 2 casos de Feocromocitoma maligno tratados con cirugía mínimamente invasiva. Casos clínicos: Presentamos 2 pacientes de sexo femenino, de 31 y 51 años respectivamente. La primera con historia de hipertensión arterial de larga data y uso de múltiples fármacos. La segunda paciente debuta con dolor abdominal. En ambos casos los estudios radiológicos y metabólicos confirmaron el diagnóstico de Feocromocitoma. A la primera paciente se le realizó un procedimiento laparoscópico puro, mientras que a la segunda paciente se le realizó un procedimiento laparoscópico asistido por robot. En ambos casos fue necesario realizar adrenalectomía en bloque con el riñón, por compromiso del hilio renal. No hubo complicaciones severas intra ni post-operatorias, como tampoco conversión a cirugía abierta. La histopatología confirmó el diagnóstico de Feocromocitoma maligno con metástasis ganglionares regionales y márgenes quirúrgicos negativos. Ambas se mantienen en control, sin evidencia de recurrencia. Conclusión: El Feocromocitoma maligno puede ser manejado de forma mínimamente invasiva, con morbilidad y resultados oncológicos comparables a cirugía abierta.

Introduction: Pheochromocytoma is a tumor derived from chromaffin cells that produces cathecolamines. Malignant pheochromocytomas are extremely rare. Aim: To describe 2 cases of malignant Pheochromocytoma treated with pure laparoscopic and robot assisted surgery. Clinical cases: Two female patients, 31 and 51 years of age, presented with a history of high blood pressure and abdominal pain. Radiologic and metabolic studies confirmed the diagnosis of Pheochromocytoma in both patients. A pure laparoscopic approach was performed in the first patient while a robotic assisted laparoscopy was done in the second patient. In both cases, adrenalectomy with in bloc nephrectomy was performed due to compromise of the renal sinus. There were no complications or conversion to open surgery. Histopathology confirmed the diagnosis of malignant Pheochromocytoma with metastasis to regional nodes and negative surgical margins. At follow-up, there is no evidence of recurrence. Conclusion: Malignant Pheochromocytoma can be treated with minimally invasive surgery, with morbidity and oncological outcomes similar to open approach.

Incidentally Detected Inoperable Malignant Pheochromocytoma with Hepatic Metastasis Treated by Transcatheter Arterial Chemoembolization

Malignant pheochromocytoma (PCC) is a rare condition. Although the liver is the second most frequent site of metastasis in malignant PCC, no definite treatments have been established. Herein, we report a case of liver metastasis of PCC that was successfully treated by transcatheter arterial chemoembolization (TACE). A 69-year-old man was admitted to the Department of Gastroenterology for evaluation of an incidental hepatic mass in August 2013. He had undergone right adrenalectomy in May 2005 and PCC had been confirmed on the basis of histopathological findings. Liver biopsy was performed, and metastatic PCC was diagnosed. The lesion appeared inoperable because of invasion of the portal vein and metastases in the lymph nodes along the hepatoduodenal ligament. Thus, TACE was performed instead. After TACE, symptoms including dizziness and cold sweating improved, and the patient's serum catecholamine levels decreased. On the basis of this case, we believe that TACE may be a useful treatment for liver metastasis in malignant PCC.

Multi-disciplinary diagnosis and treatment of a malignant pheochromocytoma with multiple metastases / 中国肿瘤临床

Malignant pheochromocytomas are rare tumors that arise from chromaffin tissue, and the diagnostic criterion of malig-nancy is based on the development of metastases. In the case a patient suffers the tumor with liver, lung and bone metastases. However, the test results of tumor markers, gastroscopy, chest and abdominal CT, and PET-CT examination are hard to make a definite diagnosis. The patient was finally diagnosed with malignant pheochromocytoma with liver, lung and bone metastases following the needle biopsy of liver and underwent the excision of a right adrenal pheochromocytoma. Therapeutic standard for the malignant pheochromocytomas is not available so far. It is reported that chemotherapeutic CVD regimen (cyclophosphamide, vincristine, and daecarbazine) and suni-tinib may be effectual in the alike cases. The patient received two cycles of CVD and one cycle of sunitinib, nevertheless, slow progres-sion of the disease remained after the treatment. The results of multi-disciplinary treatment have suggested that 131I-MIBG may just be a choice for this patient.

Clinical Analysis of Pheochromocytoma and Abdominal Paragangliomas / 대한내분비외과학회지

PURPOSE: We compared clinical characteristics between pheochromocytoma and abdominal paragangliomas and identified predictive factors of malignancy. METHODS: Between November, 1995 and January, 2011, we retrospectively reviewed the medical records of 145 patients with pheochromocytoma and abdominal paraganglioma at Samsung Medical Center. We compared two tumors (pheochromocytoma vs abdominal paraganglioma) about a potential of hypersecretion of cathecholamines and identified predictive factors of malignancy by analysis of clinical characteristics, biochemical markers, tumor features. Their postoperative results were also evaluated. RESULTS: This study included 103 (71%) pheochromocytomas and 42 (29%) abdominal paragangliomas. Eighty-six percent were benign and 14% were malignant. Patients with paraganglioma were more predominantly men and exhibited a higher malignancy rate (P<0.01) than pheochromocytoma patients. Most (95%) pheochromocytoma was hyperfunctional, but abdominal paraganglioma were hyperfunctional in 74%. There were no significant differences in biochemical markers between the pheochromocytoma and paraganglioma groupd. When compared with benign tumor, malignant tumors were significantly related with higher mean PASS (P<0.01) and higher 24-hour urinary VMA (P=0.02), but not with larger tumor size. CONCLUSION: It is not easy to distinguish malignant from benign tumors by clinical characteristics and pathologic features in the management of pheochromocytoma and paraganglioma. We should keep in mind that abdominal paraganglioma can be also hyperfunctional in many pheochromocytoma patients and has a higher risk of malignancy.

A Discrepancy between (131)I-Metaiodobenzylguanidine ((131)I-MIBG) Scintigraphy and (18)F-FDG PET/CT after (131)I-MIBG Therapy in a Patient with Recurred Malignant Pheochromocytoma / 핵의학분자영상

A 59-year-old woman who was diagnosed with malignant pheochromocytoma underwent (18)F-fluorodeoxyglucose positron emission tomography/computed tomography ((18)F-FDG PET/CT). She had undergone left adrenalectomy for pheochromocytoma 4 years previously. Recent multiple metastatic pulmonary nodules were noted on the chest X-ray. After treatment with (131)I-metaiodobenzylguanidine ((131)I-MIBG) with 7.4 GBq, post-therapy (131)I-MIBG scintigraphy depicted multiple distant metastases including lung, liver, abdominal para-aortic and mesenteric lymph nodes. (18)F-FDG PET/CT also depicted multiple metastases in lung, liver, and abdominal para-aortic lymph nodes, but some lesions were not shown. In this case, (131)I-MIBG scintigraphy found additional lesions in metastatic malignant pheochromocytoma.

Nonfunctioning Huge Malignant Pheochromocytoma with Liver Metastasis

A 27-year-old woman with a 4-month history of right upper quadrant dull pain and normal blood pressure was admitted in May 2001. Abdominal ultrasonography and abdominal computed tomography revealed a huge retroperitoneal mass with hepatic nodules. Histologic diagnosis was paraganglioma based on gun biopsy. Hormonal study for pheochromocytoma was negative. I-metaiodobenzylguanidine (MIBG) scintigraphy and angiography were performed for operation. The tumor was removed en bloc with part of the pancreas, spleen and hepatic mass. Pathologic examination with immunohistochemical staining revealed a malignant pheochromocytoma growing exophytically from the left adrenal and metastasizing to the liver. The postoperative course has been uneventful and no recurrence has been noted over a 2-months follow-up period.

Clinical Analysis of Malignant Pheochromocytoma / 대한암학회지

PURPOSE: There are no specific clinical and histopathologic characteristics of malignant pheochromocytoma and the optimal treatment modality has not been established yet. We analyzed the clinical and histopathologic features of malignant pheochromocytoma and treatment results. MATERIALS AND METHODS: We reviewed the clinical records of 10 patients with malignant pheochromocytoma diagnosed at Seoul National University Hospital from March 1987 to June 1998. RESULTS: Nine of 10 (90%) patients had functional tumors. The biochemical laboratory findings showed elevated 24-hour urine VMA level in nine patients available. The median size of the tumors was 11x11 cm. Six of 10 (60%) patients were initially diagnosed as malignant tumors because of direct invasions to adjacent tissues or distant metastases. On the other hand, remaining 4 patients were initially diagnosed as benign, but the distant metastases developed metachronously after resection of the primary lesion. The median duration between the initial operation and the detection of metastases was 57 months (range: 47~72 months) in these patients. The liver was the most common site of metastases (60%). With regards to the histopathological features, most of the tumors (87.5%) showed capsulation, necrosis and hemorrhage. The findings of lymphatic invasion, angio-invasion, and mitosis were found in 62.5% of the cases. All but 2 patients were initially treated with radical operation for the primary lesions. The disease recurrences or metastases occurred in 7 out of 10 patients. Of these, 4 patients were treated with chemotherapy or interferon- a after recurrences. Overall, the median survival for all patients was 82 months (range: 37~143 months). Two patients is alive and only one patient is alive without recurrence. CONCLUSION: The careful follow-up for at least 5 years and the aggressive multi-disciplinary therapy may be needed for the diagnosis and the management of malignant pheochromocytoma.

Radiotherapy and malignant pheochromocytoma.

This is a report of a case of a 19-year old female patient who suffered from malignant pheochromocytoma with metastasis to abdominal paraaortic lymph nodes, liver and spleen. She was treated with partial resection of the tumor mass and received 2,000 rads radiation twice within 5 years on the metastasized lesions combined with antihypertensive deugs: prazosin (blocker) and propranolol (blocker). Reduction in size of the metastatic lesions and controlled blood pressure were achieved. Now, the patient in still alive, enjoying life and happy after 12 years of treatment.