1 , Carlos MARQUES 1 At surgery a 10 cm encapsulated and vascularized The tumor was vascularized and independent report the patient was offered an exploratory laparotomy. report the patient was offered solid and cystic mass was found beginning just right renal artery below and extending to the level of the broad The normal. morphologically were ovaries Both ligament. tumor was completely excised (Fig. 3). of primary infertility of 2 years followed at our Infertility Clinic was submitted to routine pelvic ultrasound study which revealed a uterus with and 3 solid of mass abdominopelvic large a and ovary right small fibroids, normal cystic nature, measuring 64.4 x 52.6 x 57.1 mm (Fig.s 1 and 2). from the right ovary; there were no signs hydronephrosis. of ascitis Magnetic or Resonance Imaging revealed a multicystic, (MRI) vascularized adnexal mass with 60 x 35 x 104 mm located close to the iliac had vessels patient and the Although origin. ovarian right a questioning a normal body mass index her first physical examination did not reveal any abdomino pelvic mass. Evaluation of blood pressure was also normal. Blood normal values, tests including showed tumor markers levels and such 125, antigen as carbohydrate antigen, carcinoembryonic α-feto-protein. Based on magnetic resonance imaging 114 - - - - - , Carlos BARROS 1 Copyright © Ordem dos Médicos 2015 jugulotympanic. , Pedro SERENO 1 globus They are chromaffin cell tumors comprise 10–18% of all chromaf all of 10–18% comprise R e v i s ta C i e n tífi c a d a Or d e m d o s M é d i c o s w w w.a c ta m e d i c a p o r tu g u e s a .c o m 1

, Raquel ROBALO Neoplasias Abdominais; Neoplasias Pélvicas; Paraganglioma. Neoplasias 1 Abdominal Neoplasms; Paraganglioma; Pelvic Neoplasms.

Parasympathetic ganglia-derived tumors are 2

Paragangliomas are rare tumors, with a reported inci A 32-year-old healthy nulliparous woman with a history a with woman nulliparous healthy 32-year-old A The authors report a case of a large inferior para-aortic inferior large a of case a report authors The procedemos a uma laparotomia exploradora e encontrámos uma massa encapsulada, muito vascularizada que se estendia desde o com consistente foi histologia a e removida totalmente foi massa A direito. largo ligamento ao até direita renal artéria da inferior bordo um Paraganglioma. Palavras-chave: Os paragangliomas são tumores raros com uma incidência descrita de 2-8 por milhão. São tumores de células cromafins do sistema nulípara anos, 32 de jovem uma de caso um Apresentamos parassimpáticos. ou simpáticos gânglios dos provêm que neuroendócrino referenciada à nossa Unidade de Infertilidade. No decurso da investigação, na ecografia abdomino-pélvica identificámos a presença de uma massa que se estendia da pélvis ao abdómen, desde ovário direito extra-ovárico (foram até identificados osjunto doisdos ovários comvasos características morfológicasArenais, normais). ressonância magnéticacujo revelouponto uma de partida era massa anexial multiquística e muito vascularizada junto aos vasos ilíacos, tendo colocado em hipótese origem ovárica. Desta forma, Paragangliomas are rare tumors, with a reported incidence of 2–8 per million. They are chromaffin cell tumors that develop from the neural crest cells and may be divided in tumors derived from the parasympathetic or sympathetic 32-year-old ganglia. nulliparous We woman, report referred a to case our a Infertility of Clinic. Abdomino-pelvic a ultrasound identified a large abdominopelvic tumor, without ovarian origin (both ovaries were identified and had normal morphology).adnexal multicystic, Magnetic vascularized Resonancemass close Imagingto suggestedligament. iliac broad the vessels aof level and the to right extending questioning and artery an renal right below ovarian just beginning origin. found At was mass exploratory vascularized and encapsulated laparotomy, a 10 cm was consistent with Paraganglioma. This mass was totally excised and histopathology Keywords: Um Caso Raro de Tumor Abdominopélvico: Paraganglioma Abdominopélvico: Paraganglioma de Tumor Um Caso Raro Paraganglioma A Rare Abdomino-Pelvic Tumor: Tumor: Abdomino-Pelvic Rare A ABSTRACT Recebido: 04 de Junho de 2014 - Aceite: 14 de Outubro de 2014 | Recebido: 04 de Junho de 2014 - CASE REPORT Lisbon. Portugal. Alfredo da Costa Maternity. 1. Department of Obstetrics and Gynecology. paraganglioma in a 32-year-old woman. 10% are in the thorax and 75% are in the abdomen, with (cen Zuckerkandl of organ the in located latter the of most tered around the root of the inferior mesenteric artery). found anywhere along the sympathetic chain base of the from skull and the neck (5% of cases) to the bladder and prostate gland (10%). Of those found along the aorta, known as pheochromocytoma. In contrast, sympathetic paragangliomas, also known as extraadrenal pheochro mocytomas, arise outside the adrenal gland and can be found almost exclusively in the neck and skull base and arise within the carotid body and The best known and most common sympathetic ganglia- derived tumors grow within the adrenal medulla and are in tumors derived from the parasympathetic or sympathet or parasympathetic the from derived tumors in tumors These ganglia. ic fin tumor. dence of 2–8 per million. divided be may and cells crest neural the from develop that INTRODUCTION RESUMO Célia PEDROSO Acta Med Port 2015 Jan-Feb;28(1):114-116

CASO CLÍNICO the pelvis 1 Figure with ultrasoundimages) 3 Figure catecholamines produce and the peripheral system, nervous sympathetic with associated chromaffin, are latter The nerves and are believed to have a chemoreceptor function. diaphragmatic branches of the vagus and glossopharyngeal supra- the along mainly found are nonchromaffin, usually former,The system. sympathetic the with connected those categories: those related to the parasympathetic system and extra-adrenal as known are pheochromocytomas/paragangliomas. gland adrenal located the those outside while pheochromocytomas as known are adrenal medulla, the most common site of paragangliomas, the of Paragangliomas location. of regardless paraganglia DISCUSSION consistent withaParaganglioma(Fig.s4and5). 5.5 cm, nests of cells with a trabecular and alveolar pattern xr-dea prgnla r dvdd no two into divided are paraganglia Extra-adrenal of tumors to applied term generic the is Paraganglioma x 6.5 x 11.5 of tumor solid a revealed Histopathology - Paraganglioma: longitudinal section (note the similarity the (note section longitudinal Paraganglioma: - – Pelvic Ultrasound: image of a solid and cystic mass in mass cystic and solid a of image Ultrasound: Pelvic – Pedroso C,etal. A rareabdomino-pelvictumor:paraganglioma, ica da Ordem dos Médicos www m o c . a s e u g u t r o p a c i d e m a t c a w. w w s o c i d é M s o d m e d r O a d a c fi í t n e i C a t s i v e R 3,4 3 115

retroperitoneum. and pelvis, predominantly in the posterior mediastinum and abdomen the to ganglion cervical superior the near the neck, in high from region, paravertebral thoracolumbar the in response to sympathetic neural stimulation. They lie along tic massintheabdomen Figure 2 dniiain n caatrzto ofparagangliomas characterization the in and radiotracer-labeled essential identification with are (MIBG) performed metaiodobenzylguanidine MRI Abdominal scintigraphy condition. are this and there for however, features diagnosis; specific for no useful is scan (CT) up ofpatientswithchromaffin-celltumorsmandatory. follow- life-long make disease, rare this of course variable the with coupled malignancy, of predictors firm of lack the more sensitive and specific diagnostic means. widely accepted and there is a need for the development of features can be suggestive; however, these features are not follow-up. lymph nodes, lung and/or bone, either liver,at diagnosis the or during to mainly metastases distant of presence the by inadequate to predict malignancy account fornearly10%ofallpheochromocytomas. paragangliomas are benign; malignant pheochromocytomas vomiting, diarrhea,abdominaldistensionandweightloss. (incidentalomas). the in done usually context of diagnostic imaging is procedures for other purposes diagnosis the and are asymptomatic patients of 15% About patient. with our associated as masses, when large even asymptomatic be may they mass abdominal pain and/orpalpable abdominal life. of decade cavity. the to extending organ, Zuckerkandl of area the in located was patient our in paraganglioma The ovary. and prostate kidney,mesentery,gallbladder, the as such sites, otatehne admnl optd tomography computed abdominal Contrast-enhanced are features radiological and biochemical Clinical, and pheocromocytomas abdominal of majority The fifth to third the in diagnosed are paragangliomas Most Rarely, paragangliomas have been described in unusual Acta MedPort2015Jan-Feb;28(1):114-116 – Abdominal Ultrasound: image of the same solid and cys 11 oa ivso ad aiu histopathological various and invasion Local 2 Extra-adrenal paragangliomas present with present paragangliomas Extra-adrenal 5,6 1 te smtm icue nausea, include symptoms Other 8-10 which can be established 12,13 7 This said, however,

pelvic

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CASO CLÍNICO - Histopathology: Paraganglioma (alveolar pattern) - Histopathology: Paraganglioma Acta Med Port 2015 Jan-Feb;28(1):114-116 Port 2015 Acta Med The authors wish to thank Duarte Rosa, for his The authors declare that there are no conflicts of No subsidies or grants contributed to this work. These cases highlight how paraganglioma may be Eisenhofer G, Bornstein S, Brouwers Cheung F, Dahia NV, PL, Krijger Eisenhofer G, Lenders J, and diagnosis pheochromocytoma: Extra-adrenal MA. Palese GI, Disick Pacak K. Phaeochromocytoma, Kaltsas GA, Papadogias D, Grossman AB. The clinical presentation future with field the of State phaeochromocytoma. Malignant H. Ahlman Oates WW, Neblett WM, Morgan GW, Holcomb JA. O’Neill RE, Goldstein Ann Surg. 1999;229:755–66. RR, et al. Malignant phaeochromocytoma: current status and initiatives 2004;11:423-36. for future progress. Endocr Relat Cancer. pathophysiology and 2004;31:76–106. clinical management. Front management. Curr Urol Rep. 2007;8:83-8. Horm Res. prospects. Endocr Rev. 2003;24:539–53. prospects. Endocr Rev. (symptoms and signs) of sporadic and familial chromaffin cell tumours (phaeochromocytomas and 2004;31:61–75. paragangliomas). Front Horm Acad Sc. 2006;1073:449–64. Res. Y Ann N projections. JA, et al. Clinical experience over 48 years with phaeochromocytoma. MRI is important in differential diagnosis of adnexal masses masses adnexal of diagnosis differential in important is MRI and has the highest sensivity in detection of extra-adrenal paragangliomas and pheochromocytomas. ACKNOWLEDGMENTS cooperation in taking the photographs that histopathology of images the thank also authors The paper. illustrate this service. CONFLITS OF INTEREST interest. FUNDING SOURCES Figure 5 hospital such as ours. mistaken for gynecological masses. A during careful sonography, approach depending on the some operator, cases better may exclude the in ovarian origin of Although it is masses important than not MRI. to forget adnexal that 12. 13. 14. 9. 10. 11. 116 7 14 R e v i s ta C i e n tífi c a d a Or d e m d o s M é d i c o s w w w.a c ta m e d i c a p o r tu g u e s a .c o m Pedroso C, et al. A rare abdomino-pelvic tumor: paraganglioma, paraganglioma, tumor: abdomino-pelvic rare A C, et al. Pedroso . Sporadic paraganglioma. World J Surg. 2008;32:683– Surg. J World paraganglioma. Sporadic . - Histopathology: Paraganglioma (trabecular pattern) - Histopathology: Paraganglioma It may appear as an incidental finding on gynecological Paraganglioma of the abdomino-pelvic cavity is an The primary treatment for paragangliomas is complete Bravo EL, Tagle R. Phaeochromocytoma: state of the art and future Huan L, Jetly R, Kandil E. Paraganglioma of the organ of Zuckerkandl. J J Zuckerkandl. of organ the of Paraganglioma E. Kandil R, Jetly L, Huan Young W. Paragangliomas clinical overview. Ann N Lee Y K, Acad Whan Oh Y, Sci. Jun Noh H, Jin Lee Y, Yong H, Kang E, et Toutouzas al. P, Drimousis C, Dikoglou H, Markogiannakis F, Archontovasilis Petri BJ, van Eijck C, Herder Q-Y Duh JA, WW, Lee Wagner A, Krijger RR. Chetty R. Familial paraganglioma syndromes. J Clin Pathol. K, Theodorou D, et al. Paraganglioma of the greater J Surg Oncol. 2007;5:1-6. omentum: report and review of the literature. World case 2010;63:488e-91. La State Med Soc. 2012;164:26-30. 2006;1073:21-9. Extraadrenal paragangliomas of the body: Roentgenol. 2006;187:492–504. imaging features. Am J Pheochromocytomas and sympathetic paragangliomas. 2009;96:1381–92. Br J Surg. 7. ultrasound examination, especially in a tertiary women’s with extra-adrenal, intra-abdominal paragangliomas variable. Many of are them may be asymptomatic even when the lesion is quite large. CONCLUSION extremely rare Clinical tumor. and imaging data of patients for metastatic disease, appearance of multiple primary tumors. tumor recurrence or delayed vanillylmandelic acid, urinary total urinary metanephrines, fractionated and metanephrines), MRI CT and MIBG scans scans and/or are essential in the assessment surgical resection. Conventional open treatment exploration consists and of biochemical resection of testing the (plasma free mass. catecholamines, Annual metanephrines, plasma- urinary catecholamines, urinary has the highest sensitivity in paragangliomas and pheochromocytomas. detection of extra-adrenal (distinguishing functional from non-functional tumors). MRI 8. 6. 7. 4. 5. 2. 3. REFERÊNCIAS 1. Figure 4

CASO CLÍNICO Célia PEDROSO, Raquel ROBALO, Pedro SERENO, Carlos BARROS, Carlos MARQUES A Rare Abdomino-Pelvic Tumor: Paraganglioma Acta Med Port 2015:28:114-116

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