RESUMEN
Paraganglioma of the urinary bladder is a rare pathologic entity with no defi nitive histological, immunohistochemical or molecular features to determine its malignant potential. Malignancy is essentially determined by the presence of deep local invasion, invasion of adjacent structures and lymph node or distant metastases. So far, up to 180 cases of paraganglioma have been reported, with <30 being malignant. A 50-year-old male presented with painless hematuria for 6 months. Cystoscopic biopsy of the bladder mass was given as invasive urothelial carcinoma. Patient underwent radical cystectomy with pelvic lymphadenectomy. The gross morphological brown discoloration of mass on formalin fi xation was suspicious of paraganglioma and was confi rmed on immunohistochemistry. The diagnosis of malignant paraganglioma was made based on regional lymph node metastases. We describe a rare case of a patient with malignant urinary bladder paraganglioma with main differential diagnostic considerations on the histomorphology.
RESUMEN
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.
RESUMEN
Paragangliomas are rare tumors that arise from the extra-adrenal chromaffin cells, and malignancy is defined by the presence of metastases to sites that normally lack chromaffin tissue. The main therapeutic targets are tumor reduction and control of excessive catecholamine secretion. Currently, the adjuvant therapy to surgery is radiotherapy treatment using high-dose MIBG; chemotherapy is currently evolving. We report herein a case of malignant paraganglioma with lung and spine metastasis that occurred 16 years after primary tumor excision and was treated with high dose MIBG radiotherapy and chemotherapy.