Duodenal gangliocytic paraganglioma presenting as an ampullary tumor

Summary Conclusion We report a case of a duodenal gangliocytic paraganglioma that had an unusual clinical presentation and atypical immunohistochemical features. Background Duodenal gangliocytic paragangliomas are rare tumors of the gastrointestinal tract with approx 70 cases reported in the literature. The most common presentation is with gastrointestinal bleeding, whereas obstructive jaundice is very uncommon. Immunohistochemistry typically shows positive epithelial cell staining for somatostatin, pancreatic polypeptide, and other neuroendocrine markers. Methods A 56-yr-old man presented with intermittent episodes of epigastric pain, vomiting, and obstructive jaundice. Upper gastrointestinal endoscopy revealed a pedunculated ampullary tumor that was subsequently confirmed by computed tomography. At operation, there was marked secondary obstructive chronic pancreatitis in the remaining pancreas necessitating a pylorus-preserving total pancreatectomy. Results Histology confirmed typical features of a duodenal gangliocytic paraganglioma, but immunohistochemistry demonstrated negative staining for somatostatin and other polypeptides, which is in contrast to most other reported cases. Two years after surgery, the patient remains alive and well on medical treatment with no evidence of recurrent tumor.     

Malignant gangliocytic paraganglioma of the duodenum with distant metastases and a lethal course

Gangliocytic paraganglioma (GP) is rare and has been regarded as benign in general with a good prognosis. We present a patient with duodenal GP showing a malignant and lethal clinical course. A 47-year-old male patient was found to have a duodenal tumor and enlarged regional lymph nodes. The patient initially underwent a pancreaticoduodenectomy to resect the tumor and involved lymph nodes completely. Histological and immunohistochemical analyses showed findings typical of GP. However, the distant metastatic lesions in the liver and pelvic cavity were rapidly observed after surgery. The patient underwent chemotherapy and radiotherapy, as well as a second surgery to partly remove the metastatic mass in the pelvic cavity. The histological examination revealed no significant difference in histological features between the primary duodenal tumor and the metastatic pelvic mass. However, the patient finally died of the tumor due to the recurrence of the residual pelvic lesion and increased liver mass. To our knowledge, this is the first report of lethal GP with multifocal metastases. Our case confirms that GP should be regarded as a malignant potential tumor with behavior code of “1”, rather than a benign tumor of “0”.     

Emergency catheter probe endoscopic sonography (CP-EUS): An effective procedure in the assessment of a bleeding gangliocytic paraganglioma

Catheter probe endoscopic sonography is a relatively rapid and safe procedure, carried out during standard endoscopy, capable of distinguishing solid from cystic lesions and vascular from avascular masses. Herein we discuss the role of catheter probe endoscopic sonography in the emergency assessment of a patient with recent and severe bleeding from an ulcerated polyp, arising from the papilla. During the endoscopy, catheter probe endoscopic sonography showed the solid and submucosal nature of the lesion, suggesting its localised and benign nature and, most importantly, demonstrating the high risk of rebleeding from vascular structures communicating with the ulcer. Because of this finding three metallic endoclips were positioned. The lesion was not removed endoscopically because of its difficult position and the high risk of haemorrhage. The mass, removed through a duodenotomy, was shown to be a gangliocytic paraganglioma, an uncommon tumour, frequently resulting in surgery because of abdominal pain and gastrointestinal bleeding.     

First reported case of esophageal paraganglioma. A review of the literature of gastrointestinal tract paraganglioma including gangliocytic paraganglioma

Paraganglioma and the variant gangliocytic paraganglioma are rare gastrointestinal tumors. We present the first reported case of an esophageal paraganglioma and a review of the literature. From this review it seems that these tumors can occur at any age and usually present with acute or chronic bleeding with or without abdominal pain. The majority of reported cases originated in the foregut, most commonly the second part of the duodenum. Macroscopically the tumor may be pedunculated, sessile or ulcerated and have been described up to 10 cm in size. There are no reported cases of gut paragangliomas shown to be producing clinically significant amounts of catecholamines. The majority of reported tumors have been benign, only 7% malignant at presentation and all with lymph node metastases. One case developed bone metastases 3 years after excision and another recurred locally. There has been no benefit seen from radiotherapy or chemotherapy to date and it is recommended that all of these tumors are widely excised together with a lymph node resection if possible.     

Duodenal gangliocytic paraganglioma treated with endoscopic hemostasis and resection

Gangliocytic paragangliomas are exceedingly rare tumors that arise in close proximity to the papilla of Vater. There are few reports of the endoscopic resection of duodenal gangliocytic paraganglioma. A 61-year-old woman was admitted with a complaint of melena. Endoscopic examination revealed a pedunculated submucosal tumor with erosion in the third portion of the duodenum. Hemostasis, using a gold probe, was performed. Nine days later, we successfully resected the tumor, using endoscopic polypectomy. To determine the depth of tumor invasion, endoscopic ultrasonography was used. The size of the tumor was 3.0 × 2.5 × 1.0cm. A total of 25 cases of duodenal gangliocytic paraganglioma have been reported in Japan. Generally, this tumor is considered benign. However, resection was performed in many patients because preoperative diagnosis was impossible. In Japan, no previous studies have reported using endoscopic hemostasis, to our knowledge. Our patient is the fourth in Japan to be treated by endoscopic resection. We report on our patient, with a review of the literature.     

Ampullary gangliocytic paraganglioma with lymph node metastasis: A case report with literature review

Rationale:Gangliocytic paraganglioma (GP) is a rare tumor that mostly develops in the duodenum and is composed of the following 3 cell types: epithelioid endocrine, spindle-like, and ganglion-like cells. It manifests as symptoms such as abdominal pain, gastrointestinal bleeding, and weight loss; however, occasionally, it is incidentally detected on endoscopic or radiologic examinations. Although GP is usually benign, it can metastasize to the lymph nodes, and distant metastases have been reported in some cases.Patient concerns:A 46-year-old woman presented with anemia on health surveillance examination. She had no other specific symptoms, and her physical examination did not reveal any abnormal finding.Diagnosis:Endoscopic ultrasound-guided fine-needle aspiration biopsy was performed, and the endoscopist obtained samples from the inner side of the ampullary mass. Pathological examination suggested GP or a neuroendocrine tumor.Interventions:Initially, we planned transduodenal ampullectomy with lymph node excision. However, there was severe fibrosis around the duodenum, and an examination of a frozen biopsy sample from the periduodenal lymph node showed atypical cells in the lymph node. Therefore, we performed pylorus-preserving pancreaticoduodenectomy with lymph node dissection.Outcomes:The final pathological diagnosis was GP located in the ampulla of Vater. The GP showed lymphovascular and perineural invasion and invaded the duodenal wall. Furthermore, 4 out of 18 harvested lymph nodes showed metastasis.Lessons:We described a case of GP confined to the ampulla with regional lymph node metastasis and reviewed published literature on ampullary GP with lymph node metastasis.     

A case of gastrointestinal stromal tumour of the ampulla of Vater

Gastrointestinal stromal tumour rarely develops in the duodenal ampulla region. We report here a case of gastrointestinal stromal tumour of the ampulla of Vater found in a 44-year-old Japanese man presenting with biliary obstruction. He died of hepatic failure with diffuse liver metastasis. The postmortem examination showed a large Borrman type III-like tumour in the duodenal ampullary region with direct invasion of the pancreas and extrahepatic bile duct as well as metastases to the liver and regional lymph nodes. The duct orifice was located at the centre of the tumour. Microscopically, the tumour consisted of anaplastic spindle cells with high mitotic activity (90 mitoses per 50 high-power fields). Immunohistochemically, the spindle cells were positive for KIT and CD34. The final diagnosis was high-grade malignant gastrointestinal stromal tumour of the ampulla of Vater. Considering the recent advances in the diagnosis and treatment of gastrointestinal stromal tumour, this neoplasm should be included in the differential diagnosis of the tumours appearing in the duodenal ampulla region.     

Non-Familial Double Malignancy of the Colon and Ampulla of Vater: A Case Report and Review of Literature

Apart from their association in familial and hereditary cancer syndromes, sporadic double malignancies of the gastrointestinal tract involving the ampulla of Vater and colon are extremely rare. Although synchronous resection of the two by adding a colectomy to a pancreaticoduodenctomy can be accomplished with minimal increase in the surgical morbidity, a few patients, however, are best managed by a staged resection. We report a case of sporadic double malignancy of the ampulla of Vater and right colon who despite the best attempts continued to bleed and remained malnourished and was successfully managed by staged right hemicolectomy followed by a pancreaticoduodenectomy.     

Hereditary paraganglioma

Head and neck paraganglioma is a rare tumour, especially in its familial form. We report a case of a multifocal head and neck paraganglioma in a young man with a family history of cervical tumours. At the age of 24, exploration of a left cervical swelling disclosed jugulotympanic and carotid body paragangliomas. Surgical removal of both tumours was performed. Two years later, a right carotid body as well as vagal paragangliomas were discovered. Follow-up at age 30 demonstrated relapse of the bilateral cervical paragangliomas, but also aortopulmonary and mesogastric paragangliomas. Cervical paragangliomas were also detected in the patient's sister and daughter, but not in his father. Furthermore, the proband's paternal grandmother and a maternal great-uncle had a history of 'neck scar'. This family history is suggestive of an autosomal dominant pattern of inheritance with maternal genomic imprinting. Genetic analysis of paraganglioma kindreds showed linkage with two different loci: 11q13.1 and 11q22.3-q23. Further knowledge of the genes involved could provide early diagnosis and accurate genetic counselling in affected families. Thorough familial investigation is consequently mandatory in all head and neck paragangliomas, especially in younger patients with multiple localizations, as surgical removal is safer at an early stage.     

Vater壶腹部产黏液乳头状腺癌1例

病例:患者男,65岁,因"上腹部胀痛伴消瘦3月余"于2011年7月30日入院。患者3个月前开始发生中上腹疼痛不适,呈阵发性隐痛,进食后加重,空腹时缓解,无明显饥饿痛,伴喛气,无反酸,无恶心、呕吐,无呕血、黑便,无解陶土样大便。既往有高血压病史8年余,无消化性溃疡、慢性胰腺炎等病史。入院查体:体温37.1℃,脉博85次/min,呼吸18次/min,血压145/85 mm Hg(1 mm Hg=0.133 kPa)。神志清晰,轻度贫血貌,巩膜轻度黄染,全身浅表淋巴结未触及肿大,腹部平软,上腹部剑突下轻压痛,无反跳痛,Murphy征     

壶腹部混合性神经内分泌-非神经内分泌肿瘤1例报告

1病例资料患者男性,57岁,以“皮肤、巩膜黄染20 d”为主诉,于2016年12月6日来本院肝胆胰脾外科治疗,伴尿色加深,近期体质量减轻约5 kg。吸烟史50余年,20支/d;饮酒史50余年,350 g/d。入院时查体:皮肤、巩膜轻度黄染,无明显腹部压痛。血常规:白细胞4.29×10⁹/L,红细胞3.96×10¹²/L,血红蛋白119 g/L。     

Surgical and non-surgical management of thoracic and cervical paraganglioma

Thoracic and cervical paragangliomas (PGLs) are rare neuroendocrine tumors arising from chromaffin cells of the neural crest progenitors located outside the adrenal gland. We describe our current protocol as a multidisciplinary team for the management of cervical and thoracic PGLs. Surgery is generally considered the treatment of choice as it offers the best chance for cure. For resection of thoracic PGLs, video-assisted thoracoscopic surgery (VATS) is the main surgical approach, while open thoracotomy is preferred in case of tumors > 6 cm, lacking confirmation of a plane of separation with adjacent structures, or with technical difficulties during VATS. In cervical PGLs, the surgical approach should be individualized according to location, mainly based on the Glasscock-Jackson and the Fisch-Mattox classifications. Surgery is the treatment of choice for most cervical and thoracic PGLs, but radiotherapy or observation could be more suitable options in unresectable cervical and thoracic PGLs or when resection has been incomplete.     

Endoscopic Resection of a Duodenal Gangliocytic Paraganglioma

A gangliocytic paraganglioma (GP) is an extremely rare neurogenic tumor nearly located in the second portion of the duodenum and has been regarded as benign. We report a case of duodenal GP in a 53-year-old man. The GP arose in the second portion of the duodenum and was shown by histological examination to consist of epithelioid cells, spindle cells, and ganglion-like cells. An endoscopic ultrasonography showed a round, well-demarcated, inhomogeneous, submucosal tumor in the 3rd-4th layer. We resected it by endoscopic mucosal resection.     

Signet-ring cell carcinoma of ampulla of Vater： Contrast-enhanced ultrasound findings

Signet-ring cell carcinoma （SRCC） of ampulla of Vater is extremely uncommon, and less than 15 cases have been reported so far in literature. It mainly occurs in elderly people （median age 57 years）. We report a rare case of SRCC of the ampulla of Vater in a 38-year-old woman who presented with a small tumor at the Vater, discovered by the contrast-enhanced ultrasound （CEUS）. Histopathological examination showed prominent signet-ring features. We also describe the imaging features of SRCC of ampulla of Vater in CEUS.     

Allelotype of ampulla of Vater cancer: highly frequent involvement of chromosome 11

Purpose To determine the genetic differences/similarities in ampulla of Vater cancers (AVC) with respect to other pancreatic tumor types.Methods We analyzed eight cases of primary AVC by genome-wide allelotyping on DNA obtained from frozen tissue. A total of 372 microsatellite loci were used for each case, for a total of 2,976 microsatellites analyzed.Results Of the 2,159 informative markers, 400 were allelic losses and 1,759 markers were retained for an average fractional allelic loss of 0.19. Seven cases showed LOH on at least two markers on chromosomal arm 11p, while six cases showed allelic losses on 11q. The high frequency of LOH on chromosome 11 was also confirmed by analysis of an additional 17 paraffin-embedded AVC. Frequent LOH (50% or greater) was also found on chromosome arms 5q, 6q, 9p, 13, 16p, 17p, and 18p.Conclusions It can be inferred that the targets of inactivation on chromosomes 5q, 9p, and 17p appear to be APC, p16, and p53, respectively, while the critical target(s) of inactivation at the other frequently lost loci remain to be characterized. The resulting allelotype reveals that distinctive chromosomal alterations are present in these neoplasms, indicating that it is a tumor entity distinct from pancreatic adenocarcinoma.     

经内镜十二指肠乳头切除术治疗乳头部肿瘤的临床价值

目的 探讨经内镜十二指肠乳头切除术(EP)治疗十二指肠乳头部肿瘤的临床价值.方法 前瞻性观察研究15例经EP治疗的十二指肠乳头部肿瘤患者的临床疗效、并发症及随访观察结果,评价其临床价值.结果 所有15例患者均顺利完成EP治疗,其中4例术前病检为慢性炎症者术后病理诊断为腺瘤;11例术前病检为腺瘤者中术后病理诊断为高分化腺癌1例、低分化腺癌2例、腺瘤恶变1例.EP术后8例合并胰胆管扩张者同时行胆管及胰管塑料支架引流术,1例确诊为低分化腺癌者、1例确诊为腺瘤恶变者及1例腺瘤部分残留者术后追加外科手术治疗,另1例低分化腺癌者因年龄较大拒绝追加手术治疗.EP术后除2例患者出现黑便,2例患者出现一过性血淀粉酶升高外,无急性胰腺炎、胆管炎及穿孔等并发症发生,无患者死亡.EP术后平均随访观察23.4个月,11例(11/15,73.3％)病变完整切除者中,10例腺瘤者均未见复发,另1例乳头部高分化腺癌者术后已随访5个月,多次复查病检均未见复发.结论 EP术不仅可提高十二指肠乳头部肿瘤诊断的准确率;对于十二指肠乳头部腺瘤,EP术可将腺瘤组织完整切除达到根治的目的,是一项安全、有效的微创治疗措施.     

Endoscopic ultrasound findings in duodenal gangliocytic paraganglioma.

Gangliocytic paraganglioma is an uncommon benign neurogenic tumour of the digestive tract that is usually located in the descending duodenum. Due to the unusual location, such lesions are frequently overlooked on routine radiological examinations and initial endoscopy. With the recent development of endoscopic ultrasound, lesions in this location can be more easily detected and better viewed. A case of a duodenal gangliocytic paraglioma detected by endoscopic ultrasound is presented; this is possibly only the second such case to be reported in the literature.     

Coronary paraganglioma

The clinical, angiographic, and anatomical features of an uncommon primary cardiac tumor, a paraganglioma, are presented. The angiographic appearance appears unique. These tumors are slow growing and troublesome primarily because of their firm adherence to underlying cardiac structures. Treatment is complete surgical excision.