Gangliocytic Paraganglioma of Ampulla Masquerading as a Neuroendocrine Tumor
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CASE REPORT
Gangliocytic Paraganglioma of Ampulla Masquerading as a Neuroendocrine Tumor Ashutosh Mishra 1
&
Kunal Dhall 1
&
Sandeep Bhoriwal 1 & Rajni Yadav 2
&
Anubhav Narwal 2 & Sunil Kumar 1
# Springer Science+Business Media, LLC, part of Springer Nature 2020
Introduction Gangliocytic paraganglioma (GP) is a rare tumor mostly located in the ampulla or periampullary region of the duodenum. It is primarily composed of triphasic cellular differentiation— epithelioid endocrine cells, spindle cells, and ganglion cells [1]. The most common symptomatology includes gastrointestinal bleeding, abdominal pain, anemia, nausea, and weight loss, but occasionally, they are diagnosed incidentally on endoscopy or radiology [2]. It is usually considered a benign tumor, but cases with lymph node metastasis and distant metastasis have been reported [3]. We report a rare case of a 50year-old female with an ampullary mass which was successfully treated with Whipple’s pancreaticoduodenectomy. Histological and immunohistochemistry (IHC) findings were diagnostic of gangliocytic paraganglioma. Our case had lymph node metastases despite the tumor having a low Ki67 proliferation index, and the metastatic tumor showed all three tumor components. This case highlights the need for
* Sandeep Bhoriwal [email protected] Ashutosh Mishra [email protected] Kunal Dhall [email protected] Rajni Yadav [email protected] Anubhav Narwal [email protected] Sunil Kumar [email protected] 1
Department of Surgical Oncology, All India Institute of Medical Sciences, New Delhi 110029, India
2
Department of Pathology, All India Institute of Medical Sciences, New Delhi, India
awareness of this entity to avoid overtreatment, however, requiring close follow-up of the patient.
Case History A 50-year-old female presented to us with a history of upper abdominal pain since 2 months duration. There was no history of vomiting, hematemesis, jaundice, or fever. She had a history of open cholecystectomy 10 years back for gall stones disease. On physical examination, no lump was palpable in the abdomen. There was no generalized lymphadenopathy, and the rest of the systemic examination was normal. Previous open cholecystectomy subcostal scar was also present in the right hypochondrial region. The blood parameters, including complete blood count, serum bilirubin levels and other liver function tests, and tumor markers (CA19.9, CEA), were within normal limits. Ultrasound abdomen found a grossly dilated common bile duct with a slightly dilated pancreatic duct. MRI abdomen revealed a dilated CBD with abrupt tapering of CBD at periampullary region although no mass was seen. On endoscopic evaluation, side - viewing endoscopy demonstrated no growth or ulcer, but the endoscopic ultrasound found a 16 × 12 mm hypoechoic mass lesion at ampulla leading to blocked and dilated CBD (22 mm) and PD (10 mm). There were no suspicious lymph nodes found on endoscopic ultrasound. Pancreatic protocol CECT of the chest, abdomen, and pelvis showed
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