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42 12. Raweily E, Gibson A, Burt A (1990) Abnormalities of intrahepatic bile ducts in intrahepatic biliary atresia. Histopathol 17:521–527 13. Tan C, Driver M, Howard R, Moscoso G (1994) Extrahepatic biliary atresia: a first trimester event Clues from light microscopy and immunohistochemistry. J Ped Surg 29:808–814 14. Weerasoriya V, White F, Shepherd R (2004) Hepatic fibrosis and survival in biliary atresia. J Pediatr 144:123–125 15. Volpert D, White F, Finegold M, Molleston J, De Baum M, Perlmutter D (2001) Outcome of early hepatic portoenterostomy for biliary atresia. J Pediatr Gastroenterol Nutr 32(3):265–268 16. Yee Low, Vijaylaxmy Vijayan, Carolyn Tan (2001) The prognostic value of ductal plate malformation and other histologic parameters in biliary atresia. An immunohistochemical study. J Pediatr 139:320–322
Annals of Diagnostic Paediatric Pathology 2006, 10(1–2):43–46 © Copyright by Polish Paediatric Pathology Society Annals of Paraganglioma associated with neuroblastoma: rare composite tumor in a 16-year-old girl Przemyslaw Galazka 1 , Malgorzata Chrupek 1 , Roman Kazmirczuk 2 , Jaroslaw Peregud-Pogorzelski 3 , Malgorzata Pacholska 1 , Grzegorz Meder 4 , Przemyslaw Kluge 5 , Zdzislaw Skok 6 , Krzysztof Kusza 2 and Andrzej Igor Prokurat 1 Diagnostic Paediatric Pathology 1 Department of Paediatric Surgery 2 Department of Anaesthesiology and Intensive Care 4 Department of Radiology 6 Department of Pathology Nicolaus Copernicus University, Collegium Medicum in Bydgoszcz, Poland 3 Department of Oncology I Clinic of Pediatric Disorders, Pomeranian Medical University in Szczecin, Poland 5 Department of Pathology Children’s Memorial Health Institute in Warsaw, Poland Abstract Composite pheochromocytomas are very rare and account for only 3% of both adrenal and extraadrenal pheochromocytomas. We report herein a case of 16-year-old girl with tumor of the retroperitoneal space. In her history she had episode of circulatory-respiratory collapse with pulmonary oedema during induction to general anaesthesia. Diagnosis from microscopic examination was unclear: paraganglioma in specimens obtained by core needle biopsy; neuroblastoma in material from open tumor biopsy. Preoperative imaging and scintigraphic studies as well as laboratory tests did not allowed to distinguish between paraganglioma and neuroblastoma. Preoperative studies have not shown any metastasis; the tumor size did not decrease after 4 courses of chemotherapy for neuroblastoma. After preoperative preparation with phenoxybenzamine patient underwent surgical removal of the tumor. Histopathologic study revealed paraganglioma with extensive areas of fibrosis. Patient remains disease free in 2 years follow up. In case of composite pheochromocytomas/paragangliomas an adequate preoperative diagnosis is not always possible using clinical, laboratory and imaging studies. In particular cases, even histopathologic examination of the biopsy specimen did not allow to establish firm diagnosis. Key words: composite paraganglioma, pheochromocytoma-neuroblastoma Introduction Composite pheochromocytomas are very rare and account for only 3% of both adrenal and extraadrenal pheochromocytomas [1]. These tumors contain a ganglioneuroma or less frequently a ganglioneuroblastoma component. Children with composite pheochromocytomas according to the literature tended to be older than patients with classical pheochromocytoma, and the tumors were mainly functional (catecholamine secreting) [5]. Some of these cases were associated with neurofibromatosis and multiple endocrine neoplasia [1, 8]. Extraadrenal paraganglioma is rare in the pediatric po- Address for correspondence Przemyslaw Galazka Phone: +48 052 585 40 15 Department of Pediatric Surgery Fax. +48 052 585 40 95 Ul. Sklodowskiej-Curie 9 E-mail: kikchirdz@cm.umk.pl 85-094 Bydgoszcz, Poland
Page 1 and 2: This issue includes the proceedings
Page 3 and 4: Annals of Diagnostic Paediatric Pat
Page 9 and 10: 9 Table 1 Nongastrointestinal sympt
Page 11 and 12: 11 Screening tests Screening tests
Page 15: 15 Discussion Fig. 3 PAN - Stage A
Page 18 and 19: 18 Introduction Primary malignant l
Page 20 and 21: 20 Table 6 Microscopic radicality a
Page 22 and 23: 22 are in agreement with the result
Page 24 and 25: 24 Introduction Recto-cutaneous or
Page 26 and 27: 26 Table 3 Incidence of different t
Page 28 and 29: 28 value of MARP between manometric
Page 33 and 34: 33 Fig. 2 The level of pro-allergic
Page 35: 16. Salama M, Sandine W, Giovannoni
Page 38 and 39: 38 Patients, material and methods T
Page 40 and 41: 40 A D B C Fig. 1 Demonstration of
Page 44 and 45: 44 pulation and occur mostly in old
Page 46 and 47: 46 composite pheochromocytomas / pa
Page 48 and 49: Szanowni Pañstwo, Mamy zaszczyt za
Page 50 and 51: 50 10 CZERWCA (sobota) 7.30 - 8.30
Page 52 and 53: 52 Leczenie operacyjne guzów chrom
Page 54 and 55: 54 Paediatric differentiated thyroi

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