Case Report On Paraganglioma Duodenale

Transcription

1 Article ID: WMC Case Report On Paraganglioma Duodenale Corresponding Author: Dr. Abhishek Shah, Resident, General Surgery KMC Mangalore INDIA, Room No 204S KMC Falnir Men's Hostel, India Submitting Author: Dr. Abhishek Shah, Resident, KMC Mangalore Manipal University, Dept of General Surgery, India Article ID: WMC Article Type: Case Report Submitted on:08-jan-2011, 11:03:59 AM GMT Article URL: Subject Categories:SURGERY Published on: 10-Jan-2011, 01:17:12 PM GMT Keywords:GIST, Extra Adrenal Pheochromocytoma, Hypertension and α and β blockers How to cite the article:bhat S, Shah A. Case Report On Paraganglioma Duodenale. WebmedCentral SURGERY 2011;2(1):WMC Source(s) of Funding: self and patients Webmedcentral > Case Report Page 1 of 13

2 Case Report On Paraganglioma Duodenale Author(s): Bhat S, Shah A Abstract Paragangliomas are rare tumors that arise from extraadrenal chromaffincells. During embryogenesis, neural crest cells migrate to diverse locations in the body and differentiate into chief and sustentacular cells, which are designated as paraganglia. Presenting a case of paraganglioma who came to our hospital. Preoperatively was diagnosed as GIST but intraop was absolutely different. Introduction Extraluminal perigastric masses are frequently considered to be gastrointestinal stromal tumors (GISTs) or other soft tissue sarcomas, when histological confirmation is not possible. Paragangliomas may have a similar radiographic appearance. In the absence of histological diagnosis and symptoms of catecholamine excess, paragangliomas may be mistaken for GISTs. Perigastric abdominal mass presumed to be a GIST after nondiagnostic biopsy, is presented [1,2]. On abdominal CT, there are no unique imaging characteristics specific for paragangliomas [3]. Consequently, these tumors may be mistaken for other primary epithelial or mesenchymal abdominal tumors [3]. For instance, GIST, the most common sarcoma of the gastrointestinal tract, may have a similar radiographic appearance [3,4]. They are often large (84% N 5 cm) tumors arising from the stomach with heterogeneous enhancement (84%) and central necrosis (37%) [5]. While size and location help to differentiate GISTs from paragangliomas, correlation with clinical symptoms is needed. A presumed GIST considered to be resectable does not necessarily warrant preoperative biopsy, and thus further diagnostic workup is not pursued [8]. Case Report(s) A 48 yr normotensive male came to us with the complaints of pain in the upper abdomen from the last 2 months which was dull aching epigastric non-radiating pricking type of pain no aggravating and reliving factor not associated any other GI complaints. Previous history of gastro jejunostomy 12 yrs back. His general examination and systemic examination was normal except old healed upper midline scar. His blood investigation was within normal limits. Sonographic study suggestive of well defined heterogenous mass lesion 5X4cm in supraumbilical region -? GIST. His CECT was done and reported to be same. Fig 1:- CT picture showing tumor Patient was planned for laparotomy for excision of tumor. Intraoperative tumor was found in retroduodenal retro pancreatic region in between aorta and duodenum around 5 4 cms. It was seen that with handling of tumor there was sudden rise of blood pressure to 240/120 and an episode of ventricular tachycardia. Following this the vein draining it was ligated first and then the tumor was excised carefully without damaging major vessel. Fig2:- intra op picture showing tumor Fig3:- intra op picture showing tumor Fig5:- intra op picture showing tumor after clamping the vein draining tumor Fig6:- resected specimen cut section The specimen was sent for histopathological and diagnosed as paraganglioma. Fig7:- microscopic picture Fig8:- microscopic picture Post operatively patient developed hypotension and was started on vasopressor which was stopped after his BP started rising finally he recovered of his crisis after 3 days and was discharged on 10th post op day. Fig9:- post op picture after removal of sutures Discussion Paragangliomas often present with signs of catecholamine excess. The most common catecholamine secreted is norepinephrine, and a classic triad of catecholamine excess (headache, sweating, palpitations) is described [6,7]. However, this triad may be absent, and patients can be asymptomatic or symptoms can be vague (psychiatric disorders, anxiety, facial pallor, weight loss, polyuria/ polydipsia, hyperglycemia, secondary erythrocytosis, stroke, and cardiomyopathy) [6]. Paraganglioma was not suspected and preoperative catecholamine levels were not measured. The location of the tumor adjacent to the stomach, the large tumor size, and the absence of classic signs of catecholamine secretion led to the presumed diagnosis of GIST or other sarcoma. A Webmedcentral > Case Report Page 2 of 13

3 presumed GIST considered to be resectable does not necessarily warrant preoperative biopsy, and thus further diagnostic workup was not pursued [8]. Sites of paraganglioma Fig10:- showing sites for extra adrenergic pheochromocytoma Paragangliomas synthesize and store catecholamines, which include norepinephrine (noradrenaline), epinephrine (adrenaline), and dopamine. Elevated plasma and urinary levels of catecholamines and the methylated metabolites, metanephrines, are the cornerstone for the diagnosis. The hormonal activity of tumors fluctuates, resulting in considerable variation in serial catecholamine measurements. Thus, there is some value in obtaining tests during or soon after a symptomatic crisis. On the other hand, most tumors continuously leak O-methylated metabolites, which are detected by metanephrine measurements[9]. Adrenergic blockers (phenoxybenzamine) should be initiated at relatively low doses (e.g., 5 10 mg orally three times per day) and increased as tolerated every few days. Because patients are volume constricted, liberal salt intake and hydration are necessary to avoid orthostasis. Adequate alpha blockade generally requires days, with a typical final dose of mg phenoxybenzamine three times per day. Oral prazosin or intravenous phentolamine can be used to manage paroxysms while awaiting adequate alpha blockade. Before surgery, the blood pressure should be consistently below 160/90 mmhg, with moderate orthostasis. Beta blockers (e.g., 10 mg propranolol three to four times per day) can be added after starting alpha blockers, and increased as needed, if tachycardia persists. Because beta blockers can induce a paradoxical increase in blood pressure in the absence of alpha blockade, they should be administered only after effective alpha blockade. Other antihypertensives, such as calcium-channel blockers or angiotensin-converting enzyme inhibitors, have also been used when blood pressure is difficult to control with phenoxybenzamine alone[9]. Finally main stray of treatment is surgical removal or the tumor. References catecholamines in stress-induced cardiomyopathy or Takotsubo cardiomyopathy. Int J Cardiol 2007;114:e15-7. [3] Hayes W, Davidson A, Grimley P, Hartman D. Extraadrenal retroperitoneal paraganglioma: clinical, pathologic, and CT findings. AJR Am J Roentgenol 1990;155: [4] Miettinen M, Lasota J. Gastrointestinal stromal tumors definition, clinical, histological, immunohistochemical and molecular genetic features and differential diagnosis. Virchows Arch 2001;438:1-12. [5] Sandrasegaran K, Rajesh A, Rushing D, Rydberg J, Akisik F, Henley J. Gastrointestinal stromal tumors: CT and MRI findings. Eur J Radiol 2005;15: [6] Yeo H, Roman S. Pheochromocytoma and functional paraganglioma. Curr Opin Oncol 2005;17:13-8. [7] Prys-Roberts C. Phaeochromocytoma: recent progress in its management. Br J Anaesth 2000;85: [8] Demetri GD, Benjamin RS, Blanke CD, et al. NCCN Task Force. NCCN Task Force report: optimal management of patients with gastrointestinal stromal tumor (GIST) update of the NCCN clinical practice guidelines. J Nal Compr Canc Netw 2007;5(Suppl 2):S1-S29. [9] Harrison's Internal Medicine 17th edition Chapter 337 Pheochromocytoma [1] Sanchez-Recalde A, Costero O, Oliver J, Iborra C, Ruiz E, Sobrino J. Pheochromocytoma-related cardiomyopathy: inverted Takotsubo contractile pattern. Circulation 2006;113:e [2] Takizawa M, Kobayakawa N, Uozumi H, et al. A case of transient left ventricular ballooning with pheochromocytoma supporting pathogenetic role of Webmedcentral > Case Report Page 3 of 13

4 Illustrations Illustration 1 pictures and figures Fig 1:- CT picture showing tumor Fig2:- intra op picture showing tumor Webmedcentral > Case Report Page 4 of 13

5 Fig6:- resected specimen cut section Fig7:- microscopic picture Webmedcentral > Case Report Page 5 of 13

6 Fig9:- post op picture after removal of sutures Fig10:- showing sites for extra adrenergic pheochromocytoma Webmedcentral > Case Report Page 6 of 13

7 Disclaimer This article has been downloaded from WebmedCentral. With our unique author driven post publication peer review, contents posted on this web portal do not undergo any prepublication peer or editorial review. It is completely the responsibility of the authors to ensure not only scientific and ethical standards of the manuscript but also its grammatical accuracy. Authors must ensure that they obtain all the necessary permissions before submitting any information that requires obtaining a consent or approval from a third party. Authors should also ensure not to submit any information which they do not have the copyright of or of which they have transferred the copyrights to a third party. Contents on WebmedCentral are purely for biomedical researchers and scientists. They are not meant to cater to the needs of an individual patient. The web portal or any content(s) therein is neither designed to support, nor replace, the relationship that exists between a patient/site visitor and his/her physician. Your use of the WebmedCentral site and its contents is entirely at your own risk. We do not take any responsibility for any harm that you may suffer or inflict on a third person by following the contents of this website. Webmedcentral > Case Report Page 7 of 13

8 Reviews Review 1 Review Title: Case Report On Paraganglioma Duodenale Posted by Dr. naresh kumar on 26 Feb :17:59 PM GMT 1 Is the subject of the article within the scope of the subject category? Yes 2 Are the interpretations / conclusions sound and justified by the data? Yes 3 Is this a new and original contribution? No 4 Does this paper exemplify an awareness of other research on the topic? No 5 Are structure and length satisfactory? Yes 6 Can you suggest brief additions or amendments or an introductory statement that will increase the value of this paper for an international audience? 7 Can you suggest any reductions in the paper, or deletions of parts? No 8 Is the quality of the diction satisfactory? Yes 9 Are the illustrations and tables necessary and acceptable? Yes 10 Are the references adequate and are they all necessary? Yes 11 Are the keywords and abstract or summary informative? Yes Rating: 6 Comment: good work Invited by the author to make a review on this article? : Yes Experience and credentials in the specific area of science: 3yrs resideny Publications in the same or a related area of science: No How to cite: kumar n.case Report On Paraganglioma Duodenale [Review of the article 'Case Report On Paraganglioma Duodenale ' by ].WebmedCentral 1970;2(2):REVIEW_REF_NUM510 No Webmedcentral > Case Report Page 8 of 13

9 Review 2 Review Title: Case Report On Paraganglioma Duodenale Posted by Dr. naresh kumar on 26 Feb :16:31 PM GMT 1 Is the subject of the article within the scope of the subject category? Yes 2 Are the interpretations / conclusions sound and justified by the data? Yes 3 Is this a new and original contribution? No 4 Does this paper exemplify an awareness of other research on the topic? No 5 Are structure and length satisfactory? Yes 6 Can you suggest brief additions or amendments or an introductory statement that will increase the value of this paper for an international audience? 7 Can you suggest any reductions in the paper, or deletions of parts? No 8 Is the quality of the diction satisfactory? Yes 9 Are the illustrations and tables necessary and acceptable? Yes 10 Are the references adequate and are they all necessary? Yes 11 Are the keywords and abstract or summary informative? Yes Rating: 6 Comment: good work Invited by the author to make a review on this article? : Yes Experience and credentials in the specific area of science: 3yrs resideny Publications in the same or a related area of science: No How to cite: kumar n.case Report On Paraganglioma Duodenale [Review of the article 'Case Report On Paraganglioma Duodenale ' by ].WebmedCentral 1970;2(2):REVIEW_REF_NUM509 No Webmedcentral > Case Report Page 9 of 13

10 Review 3 Review Title: Case Report On Paraganglioma Duodenale Posted by Dr. dipen nath on 26 Feb :05:33 PM GMT 1 Is the subject of the article within the scope of the subject category? Yes 2 Are the interpretations / conclusions sound and justified by the data? Yes 3 Is this a new and original contribution? No 4 Does this paper exemplify an awareness of other research on the topic? No 5 Are structure and length satisfactory? No 6 Can you suggest brief additions or amendments or an introductory statement that will increase the value of this paper for an international audience? 7 Can you suggest any reductions in the paper, or deletions of parts? No 8 Is the quality of the diction satisfactory? Yes 9 Are the illustrations and tables necessary and acceptable? Yes 10 Are the references adequate and are they all necessary? Yes 11 Are the keywords and abstract or summary informative? Yes Rating: 6 Comment: good management of the case its really difficult to diagnose these cases preoperatively... Invited by the author to make a review on this article? : Yes Experience and credentials in the specific area of science: 3 yrs residency in gen surgery pgimer Publications in the same or a related area of science: No How to cite: nath d.case Report On Paraganglioma Duodenale [Review of the article 'Case Report On Paraganglioma Duodenale ' by ].WebmedCentral 1970;2(2):REVIEW_REF_NUM506 No Webmedcentral > Case Report Page 10 of 13

11 Review 4 Review Title: Case Report On Paraganglioma Duodenale Posted by Dr. Luigi Mearini on 16 Jan :49:22 AM GMT 1 Is the subject of the article within the scope of the subject category? Yes 2 Are the interpretations / conclusions sound and justified by the data? Yes 3 Is this a new and original contribution? Yes 4 Does this paper exemplify an awareness of other research on the topic? Yes 5 Are structure and length satisfactory? Yes 6 Can you suggest brief additions or amendments or an introductory statement that will increase the value of this paper for an international audience? 7 Can you suggest any reductions in the paper, or deletions of parts? No 8 Is the quality of the diction satisfactory? Yes 9 Are the illustrations and tables necessary and acceptable? Yes 10 Are the references adequate and are they all necessary? Yes 11 Are the keywords and abstract or summary informative? Yes Rating: 7 Comment: a well-done article Competing interests: none Invited by the author to make a review on this article? : No Experience and credentials in the specific area of science: good Publications in the same or a related area of science: No How to cite: Mearini L. Case Report On Paraganglioma Duodenale [Review of the article 'Case Report On Paraganglioma Duodenale ' by ].WebmedCentral 1970;2(1):REVIEW_REF_NUM375 No Webmedcentral > Case Report Page 11 of 13

12 Review 5 Review Title: Case Report On Paraganglioma Duodenale Posted by Dr. Muhammad Shamim on 10 Jan :41:17 PM GMT 1 Is the subject of the article within the scope of the subject category? Yes 2 Are the interpretations / conclusions sound and justified by the data? Yes 3 Is this a new and original contribution? Yes 4 Does this paper exemplify an awareness of other research on the topic? Yes 5 Are structure and length satisfactory? Yes 6 Can you suggest brief additions or amendments or an introductory statement that will increase the value of this paper for an international audience? 7 Can you suggest any reductions in the paper, or deletions of parts? No 8 Is the quality of the diction satisfactory? No 9 Are the illustrations and tables necessary and acceptable? Yes 10 Are the references adequate and are they all necessary? Yes 11 Are the keywords and abstract or summary informative? Yes Rating: 5 Comment: The authors have done good job & presented the case in clear & concise way. No However, the case report section needs language & grammatical corrections. Discussion section needs correlation of international literature with the current case report. Adding simple phrases like as in this case as compared to literature, at appropriate places will suffice. Competing interests: no Invited by the author to make a review on this article? : No Experience and credentials in the specific area of science: 14 years. Publications in the same or a related area of science: No How to cite: Shamim M.Case Report On Paraganglioma Duodenale[Review of the article 'Case Report On Paraganglioma Duodenale ' by ].WebmedCentral 1970;2(1):REVIEW_REF_NUM355 Webmedcentral > Case Report Page 12 of 13

13 Disclaimer This article has been downloaded from WebmedCentral. With our unique author driven post publication peer review, contents posted on this web portal do not undergo any prepublication peer or editorial review. It is completely the responsibility of the authors to ensure not only scientific and ethical standards of the manuscript but also its grammatical accuracy. Authors must ensure that they obtain all the necessary permissions before submitting any information that requires obtaining a consent or approval from a third party. Authors should also ensure not to submit any information which they do not have the copyright of or of which they have transferred the copyrights to a third party. Contents on WebmedCentral are purely for biomedical researchers and scientists. They are not meant to cater to the needs of an individual patient. The web portal or any content(s) therein is neither designed to support, nor replace, the relationship that exists between a patient/site visitor and his/her physician. Your use of the WebmedCentral site and its contents is entirely at your own risk. We do not take any responsibility for any harm that you may suffer or inflict on a third person by following the contents of this website. Webmedcentral > Case Report Page 13 of 13