tumor in the light of the revised SIOP-01 classification

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74 16. Meier-Ruge WA, Brönnimann PB, Gambazzi F, Schmid PC, Schmidt CP, Stoss F (1995) Histopathological criteria for intestinal neuronal dysplasia of the submucosal plexus (type B). Virchows Arch 426: 549–556 17. Meier-Ruge WA, Gambazzi F, Käufeler RE, Schmid P, Schmidt ChP (1994) The Neuropathological Diagnosis of Neuronal Intestinal Dysplasia (NID B). Eur J Pediatr. Surg 4: 267–273 18. Meier-Ruge WA, Schärli AF, Stoss F (1995) How to improve histopathological results in the biopsy diagnosis of gut dysganglionosis. A methodological review. Pediatr Surg Int 10: 454–458 19. Meier-Ruge WA, Schmidt PC, Stoss F (1995) Intestinal neuronal dysplasia and its morphometric evidences. Pediatr Surg Int 10: 447–453 20. Miyazaki E, Ohshiro K, Puri P (1998) NADPH-diaphorase histochemical staining of suction rectal biopsies in the diagnosis of Hirschsprung’s disease and allied disorders. Pediatr Surg Int 13: 464–467 21. Monfore-Munoz H, Gonzales-Gomez I, Rowland JM, Landing BH (1998) Increased submucosal nerve trunk caliber in aganglionosis. A “positive” and objective finding in suction biopsies and segmental resections in Hirschsprung’s disease. Arch Pathol Lab Med 122: 721–725 22. Munakata K, Okabe I, Morita K, (1985) Clinical and histological studies of neuronal intestinal dysplasia. J Pediatr Surg 20: 231–235 23. Puri P (1997) Variants of Hirschsprung’s disease. J Pediatr Surg 32: 149–157 24. Puri P (1998) Preface of Hirschsprung’s disease and related disorders – recent progress. Semin Pediatr Surg 7: 137–139 25. Puri P, Wester T (1998) Intestinal neuronal dysplasia. Semin Pediatr Surg 7: 181–186 26. Qualman SJ, Pysher T, Schauer G (1997) Hirschsprung’s disease: differential diagnosis and seguelae. In: Dahms BB, Qualman SJ (eds) Gastrointestinal disease (Perspectives in Pediatric Pathology, Vol 20). S. Karger AG, Basel (Switzerland) pp: 111–126 27. Schärli AF (1992) Neuronal intestinal dysplasia. Pediatr Surg Int 2: 2–7 28. Schärli AF, Meier-Ruge W (1981) Localized and disseminate forms of neuronal intestinal dysplasia mimicking Hirschsprung’s disease. J Pediatr Surg 16: 164–170 29. Schofield DE, Yunis EY (1991) Intestinal neuronal dyspasia. J Pediatr Gastr Nutr 12 (2): 182–189
Annals of Diagnostic Paediatric Pathology 2006, 10 (3–4): 75–79 © Copyright by Polish Paediatric Pathology Society Annals of Treatment of malignant pheochromocytoma in children Jolanta Krajewska, Kornelia Hasse-Lazar, Barbara Jarz¹b Nuclear Medicine and Endocrine Oncology Department M. Sklodowska−Curie Memorial Cancer Center and Institute of Oncology, Gliwice Branch Gliwice, Poland Introduction Abstract Pheochromocytoma and paraganglioma are unusual neoplasms in children. Both tumors develop from chromaffin cells and produce catecholamines including norepinephrine, epinephrine and dopamine. Tumors that derive from the adrenal medulla are defined as pheochromocytomas, whereas extraadrenal tumors are named paragangliomas [4, 22, 23]. Pheochromocytoma is diagnosed in approximately 1% of pediatric hypertensive patients. About 20% of these tumors occur in children, mostly in preadolescent boys and teenage girls with an incidence of 2 per million [5]. The most common symptoms in children are: hypertension, headaches, palpitations, sweating and nausea [2, 22]. Blood pressure elevation is rather permanent in contrast to adults who develop paroxysmal hypertension in 30% of cases [2]. It is important to differentiate between paragangliomas and neuroblastomas, the last more common in pediatric population, because of their similarity in location, histologic features and ability to secrete hormones [5, 22]. Neuroblastomas usually are found in retroperitoneum, origin from neural crest cells and produce vanillylmandelic acid (VMA) and Address for correspondence Pheochromocytoma is a rare neoplasm in children with the great difficulties in distinction between benign and malignant tumors on the basis of histopathological examination. The only unequivocal evidence of malignancy are distant metastases in sites where chromaffin tissue is absent. Although the treatment of choice is radical surgical resection of primary tumor and metastases the adjuvant therapy is necessary. 131 I-MIBG therapy is the first-line systemic treatment. Somatostatin analogues labeled with 90 Y make an alternative therapeutic option for patients with no 131 I-MIBG uptake. Chemotherapy, based on cyclophosphamide, vincristine and dacarbazine is reserved for patients with recurrent and progressive disease or without satisfactory response to previous treatment. Key words: 131 I MIBG therapy, malignant pheochromocytoma, treatment Diagnostic Paediatric Pathology homovanillic acid (HVA). Catecholamines are produced rarely. Approximately 25% of pheochromocytomas are associated with hereditary syndromes [21]. Genetic predisposition is found as von Hippel-Lindau disease due to VHL gene mutations, multiple endocrine neoplasia type 2A and 2B (MEN) due to RET protooncogene mutations, in familiar paraganglioma/pheochromocytoma syndromes associated with mutations of succinate dehydrogenase family genes (SDHB, SDHC and SDHD) and neurofibromatosis type 1 (NF1) due to NF gene mutations [12, 21]. RET and VHL-dependent pheochromocytomas are almost exclusively benign. The risk of malignancy is the highest in SDHB mutation carriers [12]. The risk of malignancy reported in the literature on adult population is about 10% but it ranges between of 5–26% depending on malignancy definition and population investigated [6, 10, 12, 28]. The 5-year survival rate of patients with malignant pheochromocytoma varies between 34–60% [2, 4, 18, 19, 24, 28]. However, more than 20-year survival was also observed [2, 4, 28]. Children more often have bilateral, multiple or extraadrenal tumors [2, 5, 16, 23]. Similarly, malignant tumors, although very rare, occur more Jolanta Krajewska MD Tel: +48 32 2789301, fax +48 32 278 93 25 Nuclear Medicine and Endocrine Oncology Department E-mail: jkrajewska@io.gliwice.pl M. Sklodowska-Curie Memorial Cancer Center and Institute of Oncology Wybrze¿e AK 15 44-101 Gliwice
Page 1 and 2: ISSN 1427−4426 THE CHILDREN’S M
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Fig. 4 Fistulogram of urachal sinus
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Fig. 2 2 MRI lesions of patient 1 A
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