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Intraoperative finding of retroperitoneal mature cystic teratoma in a female infant treated for segmental multicystic kidney: A case report
Journal of Pediatric Surgery Case Reports 22 (2017) 28e30
Contents lists available at ScienceDirect
Journal of Pediatric Surgery Case Reports journal homepage: www.jpscasereports.com
Intraoperative finding of retroperitoneal mature cystic teratoma in a female infant treated for segmental multicystic kidney: A case report Marianna Iaquinto a, *, Carmelo Perrone b, Roberto De Castro a a b
Department of Urologic and Paediatric Surgery, “Prof. Petrucciani” Clinic, Viale Aldo Moro, 28, 73100, Lecce, Italy Department of Paediatrics, “Veris Delli Ponti” Hospital, Via Giuseppina Delli Ponti, 73020, Scorrano, LE, Italy
a r t i c l e i n f o Article history: Received 1 March 2017 Received in revised form 19 April 2017 Accepted 20 April 2017 Available online 22 April 2017
Post-operative recovery was uneventful. Histological examination of the lesion showed that it was benign mature cystic teratoma and its excision was complete. US and tumor markers controls at 2 and 6 months were normal (unscathed kidneys, no signs of recurrence, no suspicious limphadenopathy). 2. Discussion
1. Case report A 2-years-old asymptomatic girl comes to our observation. Baby delivered normally (weighing at birth 2.9 kg); left hydronephrosis at antenatal ultrasound (US) was suspected. Post-natal abdominal US (Fig. 1) revealed a left segmental multicystic kidney (presence of 3 cysts at upper pole). Dynamic renal scintigraphy, performed at 3 months of life, was normal (Fig. 2). Voiding cystourethrography (VCUG), performed at 6 months of life, was normal. US controls (practiced every 3 months in the first year and every six months in the second year of life) revealed cysts persistence (Fig. 3), whose dimensions were fairly stable over the years (respectively about 16, 18 and 19 mm). Given no spontaneous regression of cysts, we decided to perform a left partial nephrectomy with open, retroperitoneal approach. A retroperitoneal mass (6 6 cm) was found (Fig. 4), mainly cystic with septa and a little solid middle area. It was adherent to the adjacent structures (left renal upper pole, left adrenal gland) but there was no infiltration into any vessels or organs neither was any significant lymphadenopathy noted. Furthermore, there was not any connection with the peritoneal cavity and absence of peritumoral fluid for cytological analysis. The mass was excised in toto, without difficulty (Fig. 5). Intraoperative serum dosage of tumor markers (a-FP, b-HCG, CEA) and dosage urinary catecholamines were normal.
* Corresponding author. E-mail address: [email protected] (M. Iaquinto).
Multicystic dysplasic kidney (MCDK) in infant and children is a common anomaly well described in the literature, but less well described when involving only a portion of a kidney. About segmental MCDK, most of the cases occur in the upper pole of a duplex kidney and often involute spontaneously without significant complication. The clinical presentation and imaging findings depend on the associated anomalies and complications. US is the elective and sufficient exam for a correct diagnosis in most cases, even after birth. Computed tomography (CT) and magnetic resonance imaging (MRI) do not add useful information to the diagnosis and also require the child sedation [1,2]. Segmental MCDK itself rarely requires resection. Instead, in the cases with absence of natural involution of the cysts, the hypothesis of a possible neoplastic degeneration, although rare, supports the indication for removal of the multicystic kidney. Atypical segmental MCDK may present a diagnostic dilemma and be included in the differential diagnosis of multiloculated cystic masses [3,4]. In our case, there were not associated urinary anomalies. After two years of US controls in an asymptomatic infant, the persistence of the cysts, associated with the suspect of a benign multiloculated cystic masse (as the multiloculated cystic nephroma), has supported the decision to perform a left partial nephrectomy. Instead, a retroperitoneal mass, independent from the kidney, was found. Its evolution over the years, its intraoperative appearance (mainly cystic area, no infiltration into any vessels or organs neither any significant lymphadenopathy) and the easy resection supported, according to us, the hypothesis of a benign mass. Intraoperative serum dosage of tumor markers (a-FP, b-HCG, CEA) was normal. Furthermore, the dosage of urinary catecholamines (normal) was performed to exclude the presence of a
http://dx.doi.org/10.1016/j.epsc.2017.04.008 2213-5766/© 2017 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
M. Iaquinto et al. / Journal of Pediatric Surgery Case Reports 22 (2017) 28e30
29
Fig. 1. Post-natal abdominal US.
retroperitoneal neuroblastoma. It was a possible differential diagnosis for the age of the presentation and the position, though the stability over the years is not at all a typical feature of neuroblastoma. Histological examination of the lesion showed that it was benign mature cystic teratoma and its excision was complete. Retroperitoneal teratomas comprise 3.5e4% of all germ cell
tumors in children [5,6]. Occasionally, the tumor is present antenatally and diagnosed at birth [7]. Bilateral lesions and malignancy are uncommon [10]. Patients present with abdominal distension or a palpable mass, in most cases. The majority of the tumors are benign, situated on the left side and pararenal in location. Benign retroperitoneal teratomas are cured by complete removal. Tumor markers like serum a-FP level is good indicator for diagnosis and
Fig. 2. Dynamic renal scintigraphy, performed at 3 months of life.
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M. Iaquinto et al. / Journal of Pediatric Surgery Case Reports 22 (2017) 28e30
Fig. 3. Preoperative US.
assessing the recurrence of tumor [5e9]. In our case, probably, performing another diagnostic investigation like CT scan or MRI, would have shown the independence of the lesion from the kidney, the presence of calcifications and then it would have allowed to better study the extent of the mass before surgery. However, given the history and the evolution of our patient, we are not sure to confirm that CT scan or MRI are really necessary in the case of atypical and doubtful segmental MCDK.
3. Conclusion Atypical segmental MCDK may present a diagnostic dilemma. According to our experience, we recommend to be also focused on the differential diagnosis, like renal and extra-renal multiloculated cystic masses. Fig. 5. The mass excised in toto.
References
Fig. 4. Intraoperative image of the retroperitoneal mass (arrow) and the malrotated kidney.
[1] Lin CC, Tsai JD, Sheu JC, Lu HJ, Chang BP. Segmental multicystic dysplastic kidney in children: clinical presentation, imaging finding, management, and outcome. J Pediatr Surg 2010 Sep;45(9):1856e62. [2] Jeon A, Cramer BC, Walsh E, Pushpanathan C. A spectrum of segmental multicystic renal dysplasia. Pediatr Radiol 1999 May;29(5):309e15. [3] Raja F, Romano W, Dawson W. Segmental renal dysplasia presenting as a solid mass on sonography. J Ultrasound Med 2012;31:505e9. [4] Agrons GA, Wagner BJ, Davidson AJ, ES Suarez. Multilocular cystic renal tumor in children: radiologic - pathologic correlation. Radiographics 1995;15(3): 653e69. [5] Grosfeld JL, Ballantine TV, Lowe D, Bahener RL. Benign and malignant teratomas in children: analysis of 85 Patients. Surgery 1976;80:297e305. [6] Papanicolau N, Yoder IC, Lee MJ. Primary retroperitoneal neoplasms: how close can we come in making the correct diagnosis. Urol Radiol 1992;14: 221e8. [7] Patil PS, Kothari P, Gupta A, Dikshit V, Kamble R, Kekre G, et al. Retroperitoneal mature cystic teratoma in a neonate: a case report. J Neon Surg 2016;5(2):15. [8] Hayasaka K, Yamada T, Saitoh Y, Sakura KCT. Evaluation of primary benign retroperitoneal tumor. Radiat Med 1994;12:115e20. [9] Chaudhary S, Misra A, Wakhlu RK, Tandon AK. Wakhlu. Retroperitoneal teratomas in children. Indian J Pediatr 2006:73. [10] Jones NM, Kiely EM. Retroperitoneal teratomas-potential for surgical misadventure. J Pediatr Surg 2008;43:184e6.
Contents lists available at ScienceDirect
Journal of Pediatric Surgery Case Reports journal homepage: www.jpscasereports.com
Intraoperative finding of retroperitoneal mature cystic teratoma in a female infant treated for segmental multicystic kidney: A case report Marianna Iaquinto a, *, Carmelo Perrone b, Roberto De Castro a a b
Department of Urologic and Paediatric Surgery, “Prof. Petrucciani” Clinic, Viale Aldo Moro, 28, 73100, Lecce, Italy Department of Paediatrics, “Veris Delli Ponti” Hospital, Via Giuseppina Delli Ponti, 73020, Scorrano, LE, Italy
a r t i c l e i n f o Article history: Received 1 March 2017 Received in revised form 19 April 2017 Accepted 20 April 2017 Available online 22 April 2017
Post-operative recovery was uneventful. Histological examination of the lesion showed that it was benign mature cystic teratoma and its excision was complete. US and tumor markers controls at 2 and 6 months were normal (unscathed kidneys, no signs of recurrence, no suspicious limphadenopathy). 2. Discussion
1. Case report A 2-years-old asymptomatic girl comes to our observation. Baby delivered normally (weighing at birth 2.9 kg); left hydronephrosis at antenatal ultrasound (US) was suspected. Post-natal abdominal US (Fig. 1) revealed a left segmental multicystic kidney (presence of 3 cysts at upper pole). Dynamic renal scintigraphy, performed at 3 months of life, was normal (Fig. 2). Voiding cystourethrography (VCUG), performed at 6 months of life, was normal. US controls (practiced every 3 months in the first year and every six months in the second year of life) revealed cysts persistence (Fig. 3), whose dimensions were fairly stable over the years (respectively about 16, 18 and 19 mm). Given no spontaneous regression of cysts, we decided to perform a left partial nephrectomy with open, retroperitoneal approach. A retroperitoneal mass (6 6 cm) was found (Fig. 4), mainly cystic with septa and a little solid middle area. It was adherent to the adjacent structures (left renal upper pole, left adrenal gland) but there was no infiltration into any vessels or organs neither was any significant lymphadenopathy noted. Furthermore, there was not any connection with the peritoneal cavity and absence of peritumoral fluid for cytological analysis. The mass was excised in toto, without difficulty (Fig. 5). Intraoperative serum dosage of tumor markers (a-FP, b-HCG, CEA) and dosage urinary catecholamines were normal.
* Corresponding author. E-mail address: [email protected] (M. Iaquinto).
Multicystic dysplasic kidney (MCDK) in infant and children is a common anomaly well described in the literature, but less well described when involving only a portion of a kidney. About segmental MCDK, most of the cases occur in the upper pole of a duplex kidney and often involute spontaneously without significant complication. The clinical presentation and imaging findings depend on the associated anomalies and complications. US is the elective and sufficient exam for a correct diagnosis in most cases, even after birth. Computed tomography (CT) and magnetic resonance imaging (MRI) do not add useful information to the diagnosis and also require the child sedation [1,2]. Segmental MCDK itself rarely requires resection. Instead, in the cases with absence of natural involution of the cysts, the hypothesis of a possible neoplastic degeneration, although rare, supports the indication for removal of the multicystic kidney. Atypical segmental MCDK may present a diagnostic dilemma and be included in the differential diagnosis of multiloculated cystic masses [3,4]. In our case, there were not associated urinary anomalies. After two years of US controls in an asymptomatic infant, the persistence of the cysts, associated with the suspect of a benign multiloculated cystic masse (as the multiloculated cystic nephroma), has supported the decision to perform a left partial nephrectomy. Instead, a retroperitoneal mass, independent from the kidney, was found. Its evolution over the years, its intraoperative appearance (mainly cystic area, no infiltration into any vessels or organs neither any significant lymphadenopathy) and the easy resection supported, according to us, the hypothesis of a benign mass. Intraoperative serum dosage of tumor markers (a-FP, b-HCG, CEA) was normal. Furthermore, the dosage of urinary catecholamines (normal) was performed to exclude the presence of a
http://dx.doi.org/10.1016/j.epsc.2017.04.008 2213-5766/© 2017 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
M. Iaquinto et al. / Journal of Pediatric Surgery Case Reports 22 (2017) 28e30
29
Fig. 1. Post-natal abdominal US.
retroperitoneal neuroblastoma. It was a possible differential diagnosis for the age of the presentation and the position, though the stability over the years is not at all a typical feature of neuroblastoma. Histological examination of the lesion showed that it was benign mature cystic teratoma and its excision was complete. Retroperitoneal teratomas comprise 3.5e4% of all germ cell
tumors in children [5,6]. Occasionally, the tumor is present antenatally and diagnosed at birth [7]. Bilateral lesions and malignancy are uncommon [10]. Patients present with abdominal distension or a palpable mass, in most cases. The majority of the tumors are benign, situated on the left side and pararenal in location. Benign retroperitoneal teratomas are cured by complete removal. Tumor markers like serum a-FP level is good indicator for diagnosis and
Fig. 2. Dynamic renal scintigraphy, performed at 3 months of life.
30
M. Iaquinto et al. / Journal of Pediatric Surgery Case Reports 22 (2017) 28e30
Fig. 3. Preoperative US.
assessing the recurrence of tumor [5e9]. In our case, probably, performing another diagnostic investigation like CT scan or MRI, would have shown the independence of the lesion from the kidney, the presence of calcifications and then it would have allowed to better study the extent of the mass before surgery. However, given the history and the evolution of our patient, we are not sure to confirm that CT scan or MRI are really necessary in the case of atypical and doubtful segmental MCDK.
3. Conclusion Atypical segmental MCDK may present a diagnostic dilemma. According to our experience, we recommend to be also focused on the differential diagnosis, like renal and extra-renal multiloculated cystic masses. Fig. 5. The mass excised in toto.
References
Fig. 4. Intraoperative image of the retroperitoneal mass (arrow) and the malrotated kidney.
[1] Lin CC, Tsai JD, Sheu JC, Lu HJ, Chang BP. Segmental multicystic dysplastic kidney in children: clinical presentation, imaging finding, management, and outcome. J Pediatr Surg 2010 Sep;45(9):1856e62. [2] Jeon A, Cramer BC, Walsh E, Pushpanathan C. A spectrum of segmental multicystic renal dysplasia. Pediatr Radiol 1999 May;29(5):309e15. [3] Raja F, Romano W, Dawson W. Segmental renal dysplasia presenting as a solid mass on sonography. J Ultrasound Med 2012;31:505e9. [4] Agrons GA, Wagner BJ, Davidson AJ, ES Suarez. Multilocular cystic renal tumor in children: radiologic - pathologic correlation. Radiographics 1995;15(3): 653e69. [5] Grosfeld JL, Ballantine TV, Lowe D, Bahener RL. Benign and malignant teratomas in children: analysis of 85 Patients. Surgery 1976;80:297e305. [6] Papanicolau N, Yoder IC, Lee MJ. Primary retroperitoneal neoplasms: how close can we come in making the correct diagnosis. Urol Radiol 1992;14: 221e8. [7] Patil PS, Kothari P, Gupta A, Dikshit V, Kamble R, Kekre G, et al. Retroperitoneal mature cystic teratoma in a neonate: a case report. J Neon Surg 2016;5(2):15. [8] Hayasaka K, Yamada T, Saitoh Y, Sakura KCT. Evaluation of primary benign retroperitoneal tumor. Radiat Med 1994;12:115e20. [9] Chaudhary S, Misra A, Wakhlu RK, Tandon AK. Wakhlu. Retroperitoneal teratomas in children. Indian J Pediatr 2006:73. [10] Jones NM, Kiely EM. Retroperitoneal teratomas-potential for surgical misadventure. J Pediatr Surg 2008;43:184e6.