Without treatment, the 5-year survival is generally less than 50%.
The course, however, can be highly variable with occasional patients living more than 20 years after diagnosis.
10% normal blood pressure
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Introduction
The most frequent site of metastases is the skeleton
Additional sites are liver, retroperitoneum with lymph nodes, CNS, pleura, and kidney
(Mundschenk et al. 1998)
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Problem
When signs of regional involvement or distant disease are absent, there is currently no reliable preoperative diagnostic test that can differentiate between malignant and benign pheochromocytohere is currently no effective cure for malignant pheochromocytoma, most treatment are palliative, but in some cases may reduce tumor burden and prolong survival.
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In 1997, Loh et al. published a review of the worldwide experience involving 116 patients treated with 131I-MIBG for malignant pheochromocytoma.
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Once malignancy is diagnosed, therapy is generally directed at controlling blood pressure, but may also include tumor debulking.
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impossible Other treatment modalities have to be
considered
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Alternative of Current Therapy
Surgery Radiopharmaceuticals Combined Chemotherapy Arterial Embolization
Only five CRs to 131I-MIBG were reported.
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Limitations
恶性嗜铬细胞瘤的治疗 ppt课件
Introduction
rule of 10s for pheochromocytoma (PCC)
10% bilateral
10% extra-adrenal
10% extra-abdomen
10% malignant
10% familial
10% children
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Laparoscopic adrenalectomy for pheochromocytoma should be converted to open adrenalectomy for difficult dissection, invasion, adhesions, or surgeon inexperience
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Surgical approach
Transabdominal approach is necessary minimally invasive procedures retroperitoneal approaches should be
abandoned to definitely preserve the tumor capsule and
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131 I-MIBG is of certain therapeutic effectiveness of symptomatic improvement
Complete tumor mass disappearance has only been found in small tumors
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Primary surgical resection is the treatment of choice whenever possible
Limited disease: curative intention
Extended disease: still to be considered in the first place for debulking and as palliative treatment
In group 1, the mean absorption dose per gram of tumor was above 1 000 cGy. After treatment ,tumors disappeared or shrinked in all patients
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Malignant PCCs presenting with only local disease cannot be discriminated from benign PCCs by size alone.
When PCCs do not have evidence of invasion or distant metastases and the surgeon acquires an appropriate level of experience, the majority of these tumors can be safely resected laparoscopically.
Comparison of tumor size for benign pheochromocytomas and malignant pheochromocytomas with local disease only
Size does not reliably predict malignancy in pheochromocytomas with local disease only
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Due to the rarity of the tumor, clinical studies about pheochromocytoma suffer from a fragmented nature and usually involve too small a number of cases to reach conclusive results.
perform total lymphadecectomy
(Orchard et al. 1993)
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Secondary Tumors
No experience with adjuvant pre and postoperative radiation exists
Generally are multiple Radical surgical resection is often
(ZHU Ruisen et al. 1999)
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Patients were classified into 3 groups according to their tumor size
< 8 cm3 (11 cases) , 8～20cm3 (21 cases) , > 20 cm3 (26 cases)
Treatment with 131 I-MIBG should be instituted immediately after surgical resection to eradicate the residual tumor cells and to prevent recurrences
Bone marrow suppression is temporary and not dosage related
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Tumor size (mean ± SD) <2 cm 2.0-3.9 cm 4.0-5.9 cm 6.0-7.9 cm 8.0-9.9 cm ≥10 cm
Malignant (n = 29)
6.1 ± 3.1 cm 0 9 6 5 5 4
Benign (n = 55)
5.3 ± 2.3 cm 1 10 25 13 3 3
In group 2 , the absorption dose was similar to that of group 1, but the mean absorption dose per gram was 717.6 cGy , and tumor mass regression was 36 % ;76 % reduced urinary catecholamine
In group 3 , the absorption dose per gram tumor tissue was 277 cGy , and 30 % tumor enlargement , 20 % died ; the remaining 50 % symptomatic improvement without any change in tumor size