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Bilateral adrenal teratomas
Journal of Pediatric Surgery Case Reports 51 (2019) 101319
Contents lists available at ScienceDirect
Journal of Pediatric Surgery Case Reports journal homepage: http://www.elsevier.com/locate/epsc
Bilateral adrenal teratomas Yaying Eileen Xu a, *, Brendan Jones b, Roy Kimble c a
Department of Paediatric Surgery, Urology, Burns and Trauma, Queensland Children’s Hospital, Australia, 4101 Queensland Children’s Hospital, Australia c Department of Paediatric Surgery, Urology, Burns and Trauma, Queensland Children’s Hospital, Australia b
A R T I C L E I N F O
A B S T R A C T
Keywords: Bilateral Mature teratoma Paediatric Adrenal
Teratoma is a rare type of germ cell neoplasm (GCN) which has an incidence of 0.9/100,000. These tumours occur more frequently in girls than in boys, and they usually are diagnosed before four years of age, with a mean age of 18 months. About 75% of these lesions are benign teratomas. Teratomas usually occur in the ovaries and testes, and less commonly in anterior mediastinum, retroperitoneum, sacrococcygeal region, and intestine. Primary adrenal teratomas are extremely rare, with only 18 cases of primary adrenal teratoma previously re ported and only three paediatric cases (youngest age 1 day old). The management of adrenal teratoma is surgical resection. Surgery for mature adrenal teratomas is thought to be curative, but follow-up to monitor reoccurrence is warranted as long-term data are very limited. Bilateral adrenal teratoma has never been reported in the literature. We report a case on a two-year-old boy with bilateral mature adrenal teratoma who underwent successful surgical resections and summarise the related literature.
1. Introduction Teratoma is a rare type of germ cell neoplasm (GCN) which has an incidence of 0.9/100000 [1]. GCNs are believed to originate from the primordial germ cells, which become visible during the fourth week of gestation as they form in the yolk sac near the origin of the allantois [2, 3]. Teratomas occur more commonly in girls than in boys, and they usually are diagnosed before four years of age (mean age 18 months), and most are benign [1]. Teratomas usually occur in the ovaries and testes, however, they can arise in a variety of locations, including anterior mediastinum, retroperitoneum, sacrococcygeal region, stom ach, and intestine [1,4]. Primary adrenal teratomas are extremely rare, and there are no reports of bilateral mature adrenal teratomas. We report a two-year boy with bilateral mature adrenal teratomas who underwent successful staged surgical resections. 2. Case report The patient was referred to the Paediatric Surgery service, via the Oncology service with new adrenal masses suspicious for neuroblas toma. He presented with three months of intermittent abdominal pain, bloody diarrhoea, and weight loss of 1 kg over two weeks. Ultrasound was initially performed to assess for colitis, but instead had an incidental
finding of a left upper quadrant mass. The patient had no significant post-natal history. He was born at 34/40 following an IVF pregnancy with donor sperm. There is no known family history of malignancy. There were no abnormal physical findings on examination. He subse quently had CT chest/abdomen/pelvis to further evaluate the left upper quadrant mass, which showed bilateral adrenal masses, with a larger right (85 mm � 80 mm x 60 mm) and a smaller left (17 mm � 14 mm x 13 mm) (Fig. 1), both containing components of variable density, consistent with fat, soft tissue, and bone. Radiological opinion raised the possibility of bilateral retroperitoneal or adrenal teratomas Following functional studies and tumour markers, including alpha fetoprotein/ Adrenocorticotropic hormone (ACTH)/cortisol/beta human chorionic gonadotropin//luteinising hormone/plasma catecholamines which were normal, we then proceeded with staged resection of the adrenal masses (right then left). He underwent open right adrenalectomy in February 2015. At lap arotomy, his right adrenal was completely replaced by teratoma and no normal adrenal gland was seen. There was no invasion of surrounding tissues, consistent with benign behaviour. There were no post-operative complications and he was discharged home on day 5 post operatively. Histology of the first specimen demonstrated a mature teratoma with no malignant features (Figs. 2–6). The mass included organized intestinal tissue complete with a muscular layer, nerves and ganglion cells;
* Corresponding author. E-mail addresses: [email protected] (Y.E. Xu), [email protected] (B. Jones), [email protected] (R. Kimble). https://doi.org/10.1016/j.epsc.2019.101319 Received 20 September 2019; Received in revised form 7 October 2019; Accepted 9 October 2019 Available online 10 October 2019 2213-5766/Crown Copyright © 2019 Published by Elsevier Inc. This is an open (http://creativecommons.org/licenses/by-nc-nd/4.0/).
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Y.E. Xu et al.
Journal of Pediatric Surgery Case Reports 51 (2019) 101319
respiratory mucosa, mature adipocytes, cartilage, bone, marrow, lymphoid tissue, skin and hair shafts. Ten weeks later, the patient underwent second laparotomy with left subcostal incision. A 1.7 � 1.5 cm tumour was found in anterolateral portion of left adrenal gland which was easily separated from the normal adrenal tissue, and >90% of adrenal gland was preserved, again tumour was not invading any surrounding structures. Histological examination of the second mass showed mature adipose tissue, and intestinal tissue with mature nerve fibres and ganglion cells (Fig. 7). As both teratomas were not secretory based on pre-operative catecholamines, cortisol and ACTH levels, stress doses of steroids were not given in both operations. He recovered without complication and was discharge home on day 5 post operatively. Both ACTH and cortisol levels were normal three
Fig. 3. Right mature teratoma x 200 ciliated respiratory type mucosa.
Fig. 4. Right mature teratoma, x 100 cartilage, bone and bone marrow.
Fig. 1. Coronal view of a large volume right adrenal and a smaller volume left adrenal masses (red arrows), both containing variable density macroscopic components indicating fat, soft tissue/fluid, calcium/bone. (For interpretation of the references to colour in this figure legend, the reader is referred to the Web version of this article.)
Fig. 5. Right mature teratoma x 40 skin including adnexae.
months after resections of bilateral teratomas. The patient has been reviewed yearly in outpatients with annual abdominal ultrasound, which has shown no sonographic signs of recurrence to last follow-up at 2 years post-operatively. 3. Discussion Teratomas are neoplasms derived from more than one germ cell layers - ectoderm, mesoderm, and endoderm. Most are mature cystic teratomas (MCT) with predominantly ectodermal derivatives such as hair and teeth [4]. Retroperitoneal teratomas are extremely rare and more common in childhood than in adults [5]. Teratomas are classified
Fig. 2. Right mature teratoma, x 100 intestinal type mucosa. 2
Y.E. Xu et al.
Journal of Pediatric Surgery Case Reports 51 (2019) 101319
laparoscopic resection are both considered valid treatment options, the choice depending on patient and tumour characteristics (especially the tumour volume), as well as surgeon’s experience. Laparoscopic surgery has been performed safely in tumours as large as 11 cm, as long as no local invasion is seen on preoperative imaging or found intraoperatively [6,7]. The goal of treatment is complete resection with preservation of surrounding structures, especially in the case of gonadal tumours and in the patient in this report. Due to their rarity, there are limited data on recurrence or malignant transformation of mature adrenal teratomas. The rate of malignant transformation of teratomas in general has been reported to be approximately 1%. However, the rate of malignancy in retroperitoneal teratomas however has been reported as high as 25% [7]. Therefore, we recommend complete resection at the earliest convenience after appropriate staging and exclusion of differential diagnoses. Fig. 6. Right mature teratoma x 40 Adrenal and cystic squamous epithelium.
4. Conclusion In conclusion, mature adrenal teratoma is extremely rare, and bilateral adrenal teratomas have never been described in the literature. The gold standard of managing adrenal teratoma is surgical resection and the prognosis after complete resection is excellent [2]. The optimal follow-up duration is unknown, however it was recommended to have close follow-ups with symptom review, clinical examination, and abdominal ultrasound to allow for early detection and appropriate treatment of recurrence [2,4]. Patient consent Consent to publish the case report was not obtained. This report does not contain any personal information that could lead to the identifica tion of the patient. Funding
Fig. 7. Left mature teratoma - x 40 intestinal type mucosa.
No funding or grant support.
as one of four types [1]: mature, when they contain differentiated tissue [2]; immature, when they comprise predominantly embryonic or un differentiated tissue [3]; teratoma with malignant transformation; and [4] monodermal, when there is a predominance of tissue arising from one germ cell layer [3]. Most mature teratomas are benign but up to 26% are malignant. These benign neoplasm also has malignant trans formation potential into non germ cell malignancies, such as sarcoma and carcinoma [1,6]. Reported cases of unilateral adrenal teratoma show the most com mon presentation to be incidental discovery on investigation of other symptoms or screening (for example, on investigation for secondary causes of hypertension). However, up to half of patients can present with abdominal pain, distention, or bowel obstruction from tumour compression [1]. Tumour rupture has been reported and may cause sudden onset of abdominal pain [4]. The diagnosis is largely based on imaging. The first imaging modality is usually ultrasound, which can identify the cystic, solid or complex components of the tumour. CT and MRI are used for further definition of the components of the teratoma, to clarify any local invasion, and to exclude differential diagnoses [1]. Adrenal teratomas have been mistaken for hamartomas, myelolipomas, and even retroperitoneal abscesses [4,5]. Between 1952 and 2019, only 18 cases of primary adrenal teratoma were reported, and only three paediatric cases (youngest age 1 day old) (Kuo et al.). Half of the patients presented with abdominal pain and rest were diagnosed incidentally on imaging. Most teratomas were found in the left adrenal gland (61.1%), and 17/18 were benign [7]. None of the cases identified were bilateral mature teratomas. The management of teratoma is surgical excision due to the possi bility of malignant transformation and symptoms caused by continued growth and compression of surrounding structures. Open surgery and
Authorship All authors attest that they meet the current ICMJE criteria for Authorship. Declaration of competing interest The following authors have no conflict of interest: YX, BJ, RK. Appendix A. Supplementary data Supplementary data to this article can be found online at https://doi. org/10.1016/j.epsc.2019.101319. References [1] Ciftci I, Cihan T, Koksal Y, Ugras S, Erol C. Giant mature adrenal cystic teratoma in an infant. Acta Inf Med 2013;21(2):140–1. [2] Ramakant P, Rana C, Singh KR, Mishra A. Primary adrenal teratoma: an unusual tumor - challenges in diagnosis and surgical management. J Postgrad Med 2018;64: 112–4. [3] Okulu E, Ener K, Aldemir M, Isik E, Irkkan C, Kayigil O. Primary mature cystic teratoma mimicking an adrenal mass in an adult male patient. Korean J Urol 2014; 55(2):148–51. [4] Li Y, Zhong Z, Zhao X. Primary mature teratoma presenting as an adrenal tumor in a child. Urology 2011;78(3):689–91. [5] Bhatti A, Al-Hindi H, Azzam A, Amin T, Abu-Zaid A. Mature (benign) cystic retroperitoneal teratoma involving the left adrenal gland in a 22-year-old male: a case report and literature review. Case Rep Oncol Med 2013:1–5. [6] Laparoscopic resection of a huge mature cystic teratoma of the right adrenal gland through retroperi. [7] Kuo EJ, Sisk AE, Yang Z, Huang J, Yeh MW, Livhits MJ. Adrenal teratoma: a case series and review of the literature, vol. 28. Endocrine Pathology; 2017. p. 152–8.
3
Contents lists available at ScienceDirect
Journal of Pediatric Surgery Case Reports journal homepage: http://www.elsevier.com/locate/epsc
Bilateral adrenal teratomas Yaying Eileen Xu a, *, Brendan Jones b, Roy Kimble c a
Department of Paediatric Surgery, Urology, Burns and Trauma, Queensland Children’s Hospital, Australia, 4101 Queensland Children’s Hospital, Australia c Department of Paediatric Surgery, Urology, Burns and Trauma, Queensland Children’s Hospital, Australia b
A R T I C L E I N F O
A B S T R A C T
Keywords: Bilateral Mature teratoma Paediatric Adrenal
Teratoma is a rare type of germ cell neoplasm (GCN) which has an incidence of 0.9/100,000. These tumours occur more frequently in girls than in boys, and they usually are diagnosed before four years of age, with a mean age of 18 months. About 75% of these lesions are benign teratomas. Teratomas usually occur in the ovaries and testes, and less commonly in anterior mediastinum, retroperitoneum, sacrococcygeal region, and intestine. Primary adrenal teratomas are extremely rare, with only 18 cases of primary adrenal teratoma previously re ported and only three paediatric cases (youngest age 1 day old). The management of adrenal teratoma is surgical resection. Surgery for mature adrenal teratomas is thought to be curative, but follow-up to monitor reoccurrence is warranted as long-term data are very limited. Bilateral adrenal teratoma has never been reported in the literature. We report a case on a two-year-old boy with bilateral mature adrenal teratoma who underwent successful surgical resections and summarise the related literature.
1. Introduction Teratoma is a rare type of germ cell neoplasm (GCN) which has an incidence of 0.9/100000 [1]. GCNs are believed to originate from the primordial germ cells, which become visible during the fourth week of gestation as they form in the yolk sac near the origin of the allantois [2, 3]. Teratomas occur more commonly in girls than in boys, and they usually are diagnosed before four years of age (mean age 18 months), and most are benign [1]. Teratomas usually occur in the ovaries and testes, however, they can arise in a variety of locations, including anterior mediastinum, retroperitoneum, sacrococcygeal region, stom ach, and intestine [1,4]. Primary adrenal teratomas are extremely rare, and there are no reports of bilateral mature adrenal teratomas. We report a two-year boy with bilateral mature adrenal teratomas who underwent successful staged surgical resections. 2. Case report The patient was referred to the Paediatric Surgery service, via the Oncology service with new adrenal masses suspicious for neuroblas toma. He presented with three months of intermittent abdominal pain, bloody diarrhoea, and weight loss of 1 kg over two weeks. Ultrasound was initially performed to assess for colitis, but instead had an incidental
finding of a left upper quadrant mass. The patient had no significant post-natal history. He was born at 34/40 following an IVF pregnancy with donor sperm. There is no known family history of malignancy. There were no abnormal physical findings on examination. He subse quently had CT chest/abdomen/pelvis to further evaluate the left upper quadrant mass, which showed bilateral adrenal masses, with a larger right (85 mm � 80 mm x 60 mm) and a smaller left (17 mm � 14 mm x 13 mm) (Fig. 1), both containing components of variable density, consistent with fat, soft tissue, and bone. Radiological opinion raised the possibility of bilateral retroperitoneal or adrenal teratomas Following functional studies and tumour markers, including alpha fetoprotein/ Adrenocorticotropic hormone (ACTH)/cortisol/beta human chorionic gonadotropin//luteinising hormone/plasma catecholamines which were normal, we then proceeded with staged resection of the adrenal masses (right then left). He underwent open right adrenalectomy in February 2015. At lap arotomy, his right adrenal was completely replaced by teratoma and no normal adrenal gland was seen. There was no invasion of surrounding tissues, consistent with benign behaviour. There were no post-operative complications and he was discharged home on day 5 post operatively. Histology of the first specimen demonstrated a mature teratoma with no malignant features (Figs. 2–6). The mass included organized intestinal tissue complete with a muscular layer, nerves and ganglion cells;
* Corresponding author. E-mail addresses: [email protected] (Y.E. Xu), [email protected] (B. Jones), [email protected] (R. Kimble). https://doi.org/10.1016/j.epsc.2019.101319 Received 20 September 2019; Received in revised form 7 October 2019; Accepted 9 October 2019 Available online 10 October 2019 2213-5766/Crown Copyright © 2019 Published by Elsevier Inc. This is an open (http://creativecommons.org/licenses/by-nc-nd/4.0/).
access
article
under
the
CC
BY-NC-ND
license
Y.E. Xu et al.
Journal of Pediatric Surgery Case Reports 51 (2019) 101319
respiratory mucosa, mature adipocytes, cartilage, bone, marrow, lymphoid tissue, skin and hair shafts. Ten weeks later, the patient underwent second laparotomy with left subcostal incision. A 1.7 � 1.5 cm tumour was found in anterolateral portion of left adrenal gland which was easily separated from the normal adrenal tissue, and >90% of adrenal gland was preserved, again tumour was not invading any surrounding structures. Histological examination of the second mass showed mature adipose tissue, and intestinal tissue with mature nerve fibres and ganglion cells (Fig. 7). As both teratomas were not secretory based on pre-operative catecholamines, cortisol and ACTH levels, stress doses of steroids were not given in both operations. He recovered without complication and was discharge home on day 5 post operatively. Both ACTH and cortisol levels were normal three
Fig. 3. Right mature teratoma x 200 ciliated respiratory type mucosa.
Fig. 4. Right mature teratoma, x 100 cartilage, bone and bone marrow.
Fig. 1. Coronal view of a large volume right adrenal and a smaller volume left adrenal masses (red arrows), both containing variable density macroscopic components indicating fat, soft tissue/fluid, calcium/bone. (For interpretation of the references to colour in this figure legend, the reader is referred to the Web version of this article.)
Fig. 5. Right mature teratoma x 40 skin including adnexae.
months after resections of bilateral teratomas. The patient has been reviewed yearly in outpatients with annual abdominal ultrasound, which has shown no sonographic signs of recurrence to last follow-up at 2 years post-operatively. 3. Discussion Teratomas are neoplasms derived from more than one germ cell layers - ectoderm, mesoderm, and endoderm. Most are mature cystic teratomas (MCT) with predominantly ectodermal derivatives such as hair and teeth [4]. Retroperitoneal teratomas are extremely rare and more common in childhood than in adults [5]. Teratomas are classified
Fig. 2. Right mature teratoma, x 100 intestinal type mucosa. 2
Y.E. Xu et al.
Journal of Pediatric Surgery Case Reports 51 (2019) 101319
laparoscopic resection are both considered valid treatment options, the choice depending on patient and tumour characteristics (especially the tumour volume), as well as surgeon’s experience. Laparoscopic surgery has been performed safely in tumours as large as 11 cm, as long as no local invasion is seen on preoperative imaging or found intraoperatively [6,7]. The goal of treatment is complete resection with preservation of surrounding structures, especially in the case of gonadal tumours and in the patient in this report. Due to their rarity, there are limited data on recurrence or malignant transformation of mature adrenal teratomas. The rate of malignant transformation of teratomas in general has been reported to be approximately 1%. However, the rate of malignancy in retroperitoneal teratomas however has been reported as high as 25% [7]. Therefore, we recommend complete resection at the earliest convenience after appropriate staging and exclusion of differential diagnoses. Fig. 6. Right mature teratoma x 40 Adrenal and cystic squamous epithelium.
4. Conclusion In conclusion, mature adrenal teratoma is extremely rare, and bilateral adrenal teratomas have never been described in the literature. The gold standard of managing adrenal teratoma is surgical resection and the prognosis after complete resection is excellent [2]. The optimal follow-up duration is unknown, however it was recommended to have close follow-ups with symptom review, clinical examination, and abdominal ultrasound to allow for early detection and appropriate treatment of recurrence [2,4]. Patient consent Consent to publish the case report was not obtained. This report does not contain any personal information that could lead to the identifica tion of the patient. Funding
Fig. 7. Left mature teratoma - x 40 intestinal type mucosa.
No funding or grant support.
as one of four types [1]: mature, when they contain differentiated tissue [2]; immature, when they comprise predominantly embryonic or un differentiated tissue [3]; teratoma with malignant transformation; and [4] monodermal, when there is a predominance of tissue arising from one germ cell layer [3]. Most mature teratomas are benign but up to 26% are malignant. These benign neoplasm also has malignant trans formation potential into non germ cell malignancies, such as sarcoma and carcinoma [1,6]. Reported cases of unilateral adrenal teratoma show the most com mon presentation to be incidental discovery on investigation of other symptoms or screening (for example, on investigation for secondary causes of hypertension). However, up to half of patients can present with abdominal pain, distention, or bowel obstruction from tumour compression [1]. Tumour rupture has been reported and may cause sudden onset of abdominal pain [4]. The diagnosis is largely based on imaging. The first imaging modality is usually ultrasound, which can identify the cystic, solid or complex components of the tumour. CT and MRI are used for further definition of the components of the teratoma, to clarify any local invasion, and to exclude differential diagnoses [1]. Adrenal teratomas have been mistaken for hamartomas, myelolipomas, and even retroperitoneal abscesses [4,5]. Between 1952 and 2019, only 18 cases of primary adrenal teratoma were reported, and only three paediatric cases (youngest age 1 day old) (Kuo et al.). Half of the patients presented with abdominal pain and rest were diagnosed incidentally on imaging. Most teratomas were found in the left adrenal gland (61.1%), and 17/18 were benign [7]. None of the cases identified were bilateral mature teratomas. The management of teratoma is surgical excision due to the possi bility of malignant transformation and symptoms caused by continued growth and compression of surrounding structures. Open surgery and
Authorship All authors attest that they meet the current ICMJE criteria for Authorship. Declaration of competing interest The following authors have no conflict of interest: YX, BJ, RK. Appendix A. Supplementary data Supplementary data to this article can be found online at https://doi. org/10.1016/j.epsc.2019.101319. References [1] Ciftci I, Cihan T, Koksal Y, Ugras S, Erol C. Giant mature adrenal cystic teratoma in an infant. Acta Inf Med 2013;21(2):140–1. [2] Ramakant P, Rana C, Singh KR, Mishra A. Primary adrenal teratoma: an unusual tumor - challenges in diagnosis and surgical management. J Postgrad Med 2018;64: 112–4. [3] Okulu E, Ener K, Aldemir M, Isik E, Irkkan C, Kayigil O. Primary mature cystic teratoma mimicking an adrenal mass in an adult male patient. Korean J Urol 2014; 55(2):148–51. [4] Li Y, Zhong Z, Zhao X. Primary mature teratoma presenting as an adrenal tumor in a child. Urology 2011;78(3):689–91. [5] Bhatti A, Al-Hindi H, Azzam A, Amin T, Abu-Zaid A. Mature (benign) cystic retroperitoneal teratoma involving the left adrenal gland in a 22-year-old male: a case report and literature review. Case Rep Oncol Med 2013:1–5. [6] Laparoscopic resection of a huge mature cystic teratoma of the right adrenal gland through retroperi. [7] Kuo EJ, Sisk AE, Yang Z, Huang J, Yeh MW, Livhits MJ. Adrenal teratoma: a case series and review of the literature, vol. 28. Endocrine Pathology; 2017. p. 152–8.
3