Thoracic Ganglioneuromas Resulting in Nonimmune Hydrops Fetalis

Abstract

Introduction Most often, ganglioneuromas affect older pediatric and adult patients. They are typically slow growing tumors that remain clinically silent until they become large enough to cause symptoms by compression of adjacent structures.

Case We report a case of a 22-year-old Hispanic gravida 2 para 1 female patient who was found to have massive hydrops fetalis at 20 completed gestational weeks. Fetal echocardiography revealed a narrowed distal ductal arch and proximal descending aorta. Cesarean delivery was undertaken at 29 completed gestational weeks for refractory labor and nonreassuring fetal status. The neonate expired at 47 minutes of life despite aggressive resuscitation. At autopsy, multiple thoracic masses were found adjacent to a compressed proximal descending aorta. Histological and immunohistochemical analysis confirmed the diagnosis of a ganglioneuroma, a rare type of neural crest tumor.

Discussion A variety of intrathoracic masses have previously been reported to cause hydrops fetalis including teratomas, fibrosarcomas, and lymphangiomas. To our knowledge, this case is the first description of hydrops fetalis caused by ganglioneuromas. We propose that multiple thoracic ganglioneuromas led to biventricular distal outflow tract obstruction and hydrops fetalis.

Keywords

ganglioneuroma - hydrops fetalis - thoracic - neuroblastoma

• References

• 1 Shetty PKBK, Gnana Prakash S, Shetty PK. Ganglioneuroma always a histopathological diagnosis. Online J Health Allied Scs. 2010; 9 (4) 19
• 2 Gary C, Robertson H, Ruiz B, Zuzukin V, Walvekar RR. Retropharyngeal ganglioneuroma presenting with neck stiffness: report of a case and review of literature. Skull Base 2010; 20 (5) 371-374
• 3 Aktüre E, Salamat MS, Korkmaz H, Baskaya MK. Ganglioneuroma of the sphenoid wing: a case report and literature review. Clin Neuropathol 2011; 30 (6) 313-317
• 4 Kanthan R, Senger JL, Kanthan S. Three uncommon adrenal incidentalomas: a 13-year surgical pathology review. World J Surg Oncol 2012; 10: 64
• 5 Müller A, Förster G, Behrendt W, Kosmehl H. Headache as an unusual presenting symptom of retropharyngeal ganglioneuroma. Acta Otolaryngol 2002; 122 (5) 565-568
• 6 Papso S, Bencat M. Ganglioneuroma in retropharyngeal location. Case report of a sixteen-month-old boy. Helv Paediatr Acta 1989; 43 (4) 303-305
• 7 Preidler KW, Ranner G, Szolar D, Walch C, Anderhuber W. Retropharyngeal ganglioneuroma: ultrasound, CT and MRI findings in a 57-year-old patient. Eur J Radiol 1995; 19 (2) 108-110
• 8 Preidler KW, Ranner G, Szolar D, Walch C, Anderhuber W. Ultrasonography, CT and MRI of retropharyngeal ganglioneuroma: a very rare neoplasm in adults. Neuroradiology 1996; 38 (4) 378-380
• 9 Rabjerg M, Kolodziejczyk A. [A solitary ganglioneuroma occurring in the sigmoid colon]. Ugeskr Laeger 2012; 174 (39) 2305-2306
• 10 Walch C, Anderhuber W, Preidler K, Humer U. Retro- and para-pharyngeal ganglioneuroma. J Laryngol Otol 1996; 110 (1) 87-90
• 11 Jain M, Shubha BS, Sethi S, Banga V, Bagga D. Retroperitoneal ganglioneuroma: report of a case diagnosed by fine-needle aspiration cytology, with review of the literature. Diagn Cytopathol 1999; 21 (3) 194-196
• 12 Lonergan GJ, Schwab CM, Suarez ES, Carlson CL. Neuroblastoma, ganglioneuroblastoma, and ganglioneuroma: radiologic-pathologic correlation. Radiographics 2002; 22 (4) 911-934
• 13 Hayes FA, Green AA, Rao BN. Clinical manifestations of ganglioneuroma. Cancer 1989; 63 (6) 1211-1214
• 14 Gültekin M, Dursun P, Salman C , et al. Ganglioneuroma mimicking ovarian tumor: a report of a case and review of the ganglioneuromas. Arch Gynecol Obstet 2005; 271 (1) 66-68
• 15 Rosai J , ed. Adrenal gland and other paraganglia. In: Ackerman's surgical pathology (8th ed.). St. Louis, MO: Mosby-Year Book; 1996: 1015-1058
• 16 Kleihaus PCW , ed. WHO Classification of Tumors. In: Pathology and Genetics: Tumors of the Nervous system. Lyon, France: IARC press; 2000: 96-98
• 17 Gentile S, Rainero I, Luda E, Pinessi L. Autonomic dysfunction associated with multiple pelvic ganglioneuromas. Acta Neurol Scand 2001; 104 (1) 54-56
• 18 Bjellerup P, Theodorsson E, Kogner P. Somatostatin and vasoactive intestinal peptide (VIP) in neuroblastoma and ganglioneuroma: chromatographic characterisation and release during surgery. Eur J Cancer 1995; 31A (4) 481-485
• 19 Ostertun B, Harder T, Reetz KP. [Ganglioneuroma of the lesser pelvis]. Rofo 1987; 147 (1) 93-94
• 20 Santo S, Mansour S, Thilaganathan B , et al. Prenatal diagnosis of non-immune hydrops fetalis: what do we tell the parents?. Prenat Diagn 2011; 31 (2) 186-195
• 21 Rasanen J, Wood DC, Weiner S, Ludomirski A, Huhta JC. Role of the pulmonary circulation in the distribution of human fetal cardiac output during the second half of pregnancy. Circulation 1996; 94 (5) 1068-1073
• 22 Harlass FE, Duff P, Brady K, Read J. Hydrops fetalis and premature closure of the ductus arteriosus: a review. Obstet Gynecol Surv 1989; 44 (7) 541-543
• 23 Cass DL, Olutoye OO, Cassady CI , et al. Prenatal diagnosis and outcome of fetal lung masses. J Pediatr Surg 2011; 46 (2) 292-298
• 24 Dolkart LA, Reimers FT, Kuonen CA. Intrathoracic congenital fibrosarcoma. A case report. J Reprod Med 1995; 40 (5) 391-393
• 25 Froberg MK, Brown RE, Maylock J, Poling E. In utero development of a mediastinal teratoma: a second-trimester event. Prenat Diagn 1994; 14 (9) 884-887
• 26 Kuller JA, Laifer SA, Martin JG, MacPherson TA, Mitre B, Hill LM. Unusual presentations of fetal teratoma. J Perinatol 1991; 11 (3) 294-296
• 27 Lindinger A, Boos R, Pistorius K, Limbach HG, Jesberger HJ, Hoffmann W. [Mediastinal cystic lymphangioma as a cause of hydrops fetalis]. Klin Padiatr 1992; 204 (2) 118-122
• 28 Tollens T, Casselman F, Devlieger H , et al. Fetal cardiac tamponade due to an intrapericardial teratoma. Ann Thorac Surg 1998; 66 (2) 559-560
• 29 Weinraub Z, Gembruch U, Födisch HJ, Hansmann M. Intrauterine mediastinal teratoma associated with non-immune hydrops fetalis. Prenat Diagn 1989; 9 (5) 369-372
• 30 Fénart D, Deville A, Donzeau M, Bruneton JN. [Retroperitoneal neuroblastoma diagnosed in utero. Apropos of 1 case]. J Radiol 1983; 64 (5) 359-361
• 31 Ganick DJ, Kodroff MB, Marrow HG, Holbrook CT, Pories WJ. Thoracic neuroblastoma presenting as a cystic hygroma. Arch Dis Child 1988; 63 (10) 1270-1271
• 32 Giulian BB, Chang CC, Yoss BS. Prenatal ultrasonographic diagnosis of fetal adrenal neuroblastoma. J Clin Ultrasound 1986; 14 (3) 225-227
• 33 Guin GH, Gilbert EF, Jones B. Incidental neuroblastoma in infants. Am J Clin Pathol 1969; 51 (1) 126-136
• 34 Hendry GM. Cystic neuroblastoma of the adrenal gland—a potential source of error in ultrasonic diagnosis. Pediatr Radiol 1982; 12 (4) 204-206
• 35 Ho PT, Estroff JA, Kozakewich H , et al. Prenatal detection of neuroblastoma: a ten-year experience from the Dana-Farber Cancer Institute and Children's Hospital. Pediatrics 1993; 92 (3) 358-364
• 36 Jennings RW, LaQuaglia MP, Leong K, Hendren WH, Adzick NS. Fetal neuroblastoma: prenatal diagnosis and natural history. J Pediatr Surg 1993; 28 (9) 1168-1174
• 37 de Filippi G, Canestri G, Bosio U, Derchi LE, Coppi M. Thoracic neuroblastoma: antenatal demonstration in a case with unusual post-natal radiographic findings. Br J Radiol 1986; 59 (703) 704-706
• 38 Gorincour G, Dugougeat-Pilleul F, Bouvier R , et al. Prenatal presentation of cervical congenital neuroblastoma. Prenat Diagn 2003; 23 (8) 690-693
• 39 Isaacs Jr H. Fetal and neonatal neuroblastoma: retrospective review of 271 cases. Fetal Pediatr Pathol 2007; 26 (4) 177-184
• 40 Sebire NJ, Jauniaux E. Fetal and placental malignancies: prenatal diagnosis and management. Ultrasound Obstet Gynecol 2009; 33 (2) 235-244
• 41 Granata C, Fagnani AM, Gambini C , et al. Features and outcome of neuroblastoma detected before birth. J Pediatr Surg 2000; 35 (1) 88-91