Obstructive jaundice due to a rare periampullary tumor

Gangliocytic paraganglioma is a rare neuroendocrine tumor predominantly arising in the second part of the duodenum with rare local recurrence or metastasis to regional lymph nodes.A 92-year-old female presented with obstructive jaundice.On exam she had pale conjunctiva and icteric sclera.Abdominal examination revealed tenderness in the upper abdomen.Laboratory data was consistent with obstructive jaundice.Computed tomography of the abdomen revealed a dilated gall bladder and a common bile duct(CBD)with no evidence of liver lesions or pancreatic head mass.Endoscopic ultrasonography revealed a 1 cm isoechoic submucosal nodule at the periampullary area,dilated CBD(9 mm),a prominent pancreatic duct(4.1 mm)and a hydropic gall bladder with no stones.Endoscopic retrograde cholangiopancreaticography was performed to relieve obstruction and showed a 1 cm periampullary mass which underwent an en-bloc snare resection.Histopathology analyses with immunohistochemical stains were positive for cytokeratin,synaptophysin,S-100 protein,neuron specific enolase and negative for actin and desmin consistent with periampullary gangliocytic paraganglioma.Periampullary gangliocytic paraganglioma is a rare benign tumor of the small bowel.Common presentation includes abdominal pain and obstructive jaundice which should be included in differential diagnosis of obstructive jaundice.Endoscopic resection is a curative therapy in the absence of local invasion or distant metastasis.     

Pheochromocytomes et paragangliomes pédiatriques: revue de la littérature

Pheochromocytomas and paragangliomas (PHEO/PGL) are neuroendocrine tumors that arise from sympathetic and parasympathetic paraganglia. Although well describedin the adult population, diagnosis and treatment of these exceptionally rare neoplasms remains poorly characterized in children. This article reviews recent advances in clinical presentation, genetics, biochemistry, imaging and treatment of children with benign or malignant PHEO/PGL. Compared to adults, pediatric PHEO/PGL are more frequently familial, bilateral, multifocal and malignant. Approximately 50% of pediatric PHEO/PGLare associated with a mutationof one of the 12 known susceptibility genes. Von Hippel-Lindau disease, type 1 neurofibromatosis, type 2 multiple endocrine neoplasia and familial PGL syndrome are hereditary tumor syndromes associated with an increased risk of developing such diseases. Clinical presentation includes symptoms related to catecholamine hypersecretion and/or tumor mass effect. Plasma and/or urine metanephrine dosages are recommended as first-line diagnostic biochemical tests. Magnetic resonance imaging is useful asinitial radiological approach. Most pediatric PHEO/PGLs are benign. Surgical resection, with appropriate perioperative management of catecholamine-related symptoms, remains the treatment of choice. In case of metastatic disease, surgical removal of metastases when possible and I-131-MIBG radiotherapy provide limited results whereas chemotherapy is reserved for more advanced stages.     

Treatment of malignant pheochromocytoma/paraganglioma with cyclophosphamide, vincristine, and dacarbazine: recommendation from a 22-year follow-up of 18 patients

BACKGROUND

A long‐term follow‐up was conducted of 18 patients with a diagnosis of pheochromocytoma/paraganglioma treated with a combination of cyclophosphamide, vincristine, and dacarbazine (CVD).

METHODS

The study design was a nonrandomized, single‐arm trial conducted at a government medical referral center. Eighteen patients with metastatic malignant pheochromocytoma/paraganglioma were studied. After controlling symptoms of catecholamine excess, patients were treated with cyclophosphamide at 750 mg/m², vincristine at 1.4 mg/m², and dacarbazine at 600 mg/m² on Day 1 and dacarbazine at 600 mg/m² on Day 2, every 21 to 28 days.

RESULTS

Combination chemotherapy with CVD produced a complete response rate of 11% and a partial response rate of 44%. Median survival from a landmark was 3.8 years for patients whose tumors responded to therapy and 1.8 years for patients whose tumors did not respond (P = .65). All patients with tumors scored as responding reported improvement in their symptoms related to excessive catecholamine release and had objective improvements in blood pressure. CVD was well tolerated with only grade I/II toxicities.

CONCLUSIONS

Combination chemotherapy with CVD produced objective tumor responses in patients with advanced malignant pheochromocytoma/paraganglioma. In this 22‐year follow‐up there was no difference in overall survival between patients whose tumors objectively shrank and those with stable or progressive disease. However, patients reported improvement in symptoms, had objective improvements in blood pressure, and had tumor shrinkage that made surgical resection possible. The authors conclude that CVD therapy is not indicated in every patient with metastatic pheochromocytoma/paraganglioma, but should be considered in the management of patients with symptoms and where tumor shrinkage might be beneficial. Cancer 2008. © 2008 American Cancer Society.     

伽玛刀治疗颈静脉球瘤24例临床分析

目的 探讨伽玛刀治疗颈静脉球瘤的效果。方法 回顾性分析1998年10月至2017年10月应用伽玛刀治疗的24例术后残留或复发的颈静脉球瘤的临床资料。肿瘤体积3.8~19.8 cm3,平均8.26 cm3。照射剂量:肿瘤周边剂量12~16 Gy,平均14.7 Gy;覆盖肿瘤的等剂量曲线为45%~50%,平均48.7%。结果 伽玛刀治疗后随访24~252个月,平均96个月。伽玛刀后5、10年的实际总生存率分别为100%、90.9%。影像随访显示伽玛刀后肿瘤基本消失2例（8.3%）,肿瘤体积缩小13例（54.2%）,肿瘤稳定8例（33.3%）,体积增大1例（4.2%）;肿瘤总控制率为95.8%（23/24）。治疗后症状明显好转或消失7例（29.2%）,无变化15例（54.2%）,加重2例（8.3%）。结论 伽玛刀治疗术后残留或复发的颈静脉球瘤安全有效,可长期控制肿瘤生长