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Immature gastric teratoma in a newborn
Journal of Pediatric Surgery Case Reports 47 (2019) 101222
Contents lists available at ScienceDirect
Journal of Pediatric Surgery Case Reports journal homepage: www.elsevier.com/locate/epsc
Immature gastric teratoma in a newborn a
b
T c
d
a
Sandheeah Ramdeny , Katherine Broad , Prabhu Sekaran , Sara Harrison , Philip Connor , Mallinath Chakrabortyb,e,∗ a
Department of Paediatric Haemato-Oncology, University Hospital of Wales, Cardiff, UK Regional Neonatal Intensive Care Unit, University Hospital of Wales, Cardiff, UK Department of Paediatric Surgery, University Hospital of Wales, Cardiff, UK d Department of Paediatric Radiology, University Hospital of Wales, Cardiff, UK e Centre for Medical Education, Cardiff University, Cardiff, UK b c
A B S T R A C T
We present a full-term male infant with a palpable abdominal mass who presented with severe haematemesis associated with cardiovascular compromise. Although an initial ultrasound imaging identified a left supra-renal calcified mass suggesting a neuroblastoma, further imaging suggested an atypical mass which was separate from the spleen and left kidney. An upper gastro-intestinal contrast study localised the mass to the stomach and a computed tomograph suggested the possibility of a gastric teratoma likely in combination with a raised serum Alpha-fetoprotein. The mass was completely surgically resected, and histology confirmed an immature gastric teratoma.We would like to draw the attention of clinicians of the possibility of a gastric teratoma in an infant who presents with an abdominal mass and haematemesis.
1. Case report A male infant weighing 3.79 kg was born at term in good condition, requiring no resuscitation to non-consanguineous parents. Routine antenatal screening was unremarkable and there was no family history of note. The primiparous mother had no significant past medical history other than a previous early miscarriage. The infant fed well by bottle on formula milk and opened his bowels normally on day 1 of life. A palpable mass in the left hypochondrium was noted on the newborn examination, and an ultrasound scan (USS) was arranged as an outpatient. The infant appeared otherwise healthy. On day 3 of life, he had significant haematemesis resulting in cardiovascular compromise followed by repeated episodes of melaena, and required admission to the neonatal unit (NNU). There was a drop in serum haemoglobin from 21.6 g/dL to 12.8 g/dL. The infant required fluid resuscitation and packed red blood cell (PRBC) transfusion. The haematemesis stopped after intravenous administration of ranitidine and tranexamic acid. 2. Investigations 2.1. Blood and urine Full blood count (FBC) showed normal white blood cell (WBC) and platelet count with a significant drop in haemoglobin between day 2 and 3 of life. Red cell indices were normal. His baseline liver and kidney
∗
function were also normal. Serum Alpha-fetoprotein (AFP) level was 12,067 KU/L on day 3 of life and Beta human chorionic gonadotrophin (β-HCG) level was within the normal range as outlined in Table 1. Urinary excretion of catecholamine was within the normal range. These included Homovanillic acid (HVA): creatinine ratio of 13.5 μmol/mmol (normal range < 35) and 4-hydroxy-3-methoxy-mandelate (HMMA): creatinine ratio of 4.9 μmol/mmol (normal range < 20)]. Thus, apart from confirming an acute bleed, the above results did not point towards any specific diagnosis. 2.2. Radiological diagnostic dilemma A chest X-ray (CXR) and abdominal X-ray (AXR) performed during the episode suggested a large calcified mass in the left hypochondrium. Abdominal ultrasound scan (US) performed on day 4 of life initially suggested the location of the mass to be suprarenal, possibly arising from the left adrenal gland, raising the possibility of a neuroblastoma. At this point, he was referred to a tertiary centre. A magnetic reasonance imaging (MRI) scan was performed on day 6 of life which showed that there was a mass measuring 6 × 6.4 × 4.5 cm which appeared to arise from the adrenal glands. The mass was noted to be separate from the adjacent kidney and spleen. The mass was partially cystic and solid and was not entirely typical of a neuroblastoma. The results of the MRI scan (Fig. 1A) showed that the most likely diagnosis at the time was a neuroblastoma.
Corresponding author. Regional Neonatal Intensive Care Unit, University Hospital of Wales, Heath Park, Cardiff, CF14 4XW, UK. E-mail address: chakrabortym@cardiff.ac.uk (M. Chakraborty).
https://doi.org/10.1016/j.epsc.2019.101222 Received 4 April 2019; Accepted 11 May 2019 Available online 14 May 2019 2213-5766/ © 2019 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/).
Journal of Pediatric Surgery Case Reports 47 (2019) 101222
S. Ramdeny, et al.
There were numerous cysts with squamous, respiratory, gastric, intestinal or fallopian tube type epithelium. The pathologist concluded that the mass was an immature multicomponent teratoma and no other germ cells tumour had been identified.
Table 1 Blood results for tumour markers. Blood Results for Tumour Markers
Results
Unit and Range
AFP on day AFP on day AFP on day AFP on day AFP on day Beta- HCG LDH Uric acid
12,067 6249 288 113 18 1 579 209
KU/L Range (< 1600KU/L) KU/L Range (< 1600KU/L) KU/L Range (< 1600KU/L) KU/L Range (< 1600KU/L) KU/L Range (< 1600KU/L) IU/L Range (< 5) U/L Range (309–1222) umol/L Range (164–751)
3 of life 7 of life 25 of life, 9 days post-op 64 of life 172 of life
2.5. Progress and follow-up The infant recovered uneventfully on the neonatal unit. Feeding was fully established on day 28 of life and he was discharged home one day 30 of life. He continues to tolerate oral feeding and is gaining weight appropriately. His serum AFP level has dropped down to 288 KU/L. He will be regularly reviewed as outpatient with clinical and radiological surveillance with the aim to perform MRI scans every three months.
The results of the urinary catecholamine which was normal came back on the same day as the result of the MRI scan making the diagnosis of neuroblastoma unlikely. However, the serum AFP was 12, 067 KU/L suggestive of an alternative pathology. An abdominal USS was repeated on day 7 of life despite the fact that the abdominal USS scan on day 4 of life showed that the mass could be arising from the left adrenal gland. When the abdominal USS was performed, the left adrenal gland was identified in the expected position. There was a cystic structure that was noted which correlated with the posterior cystic structure lying in the suprarenal space on the MRI scan. The stomach appeared to be draped across the mass. A contrast study was then performed using a nasogastric tube (NG) which was placed within the stomach. Contrast which was instilled, initially appeared to flow around a filling defect. With subsequent passage of contrast, a lobulated filling defect was identified within a massively distended stomach. The stomach was not obstructed, contrast left the stomach to outline normal duodenum and proximal small bowel with no evidence of malrotation, as shown in Fig. 1B. It was then concluded that the mass was unusual in appearance and arose from within the stomach. A Computed Tomography (CT) scan of the abdomen with contrast which was subsequently performed showed that the mixed density mass contained foci of calcification and appeared likely to originate from the stomach but could possibly have arisen within the spleen and invaded the stomach as demonstrated in Fig. 1C. At this stage, the diagnosis of gastric teratoma was considered. After discussions amongst the paediatric radiologists, surgical and oncology teams, debating the risks and benefits of a biopsy versus a complete excision, a decision was taken to carry out primary resection of the tumour.
3. Discussion A teratoma is a germ cell tumour which consist of tissues that originate from the ectoterm, endoderm and mesoderm [1]. Teratoma, which is derived from the precursor totipotential stem cell accounts for the commonest germ cell tumour in paediatrics and can be classified as either arising from the gonadal tissue which present in younger children or the extragonadal tissue found in older children [2]. The location of extragonadal teratoma are mainly in the sacrococcygeal (60–65%) area, mediastinal (5–10%) area, sacral (5%) area and seldom within the intracranial, retroperitoneal and alimentary area [3]. Gastric teratoma is not a common presentation in the paediatric population and account for less than 1% amongst teratoma in paediatrics [4]. Furthermore, immature gastric teratoma is even rarer and there have been only 30 such cases which have been described in the literature [5]. It is associated with a good prognosis, once the tumour has been completely surgically excised. Adjuvant treatment with chemotherapy or radiotherapy is not clinically indicated. However, surveillance is required by measuring the level of AFP. Chemotherapy may be needed in the event of recurrence. The differential diagnosis of the possible aetiology of the upper gastrointestinal (GI) bleed in a neonate is wide and includes swallowed maternal blood or local causes such as oesophagitis, gastritis or vascular malformation, haemorrhage secondary to necrotising enterocolitis or haemorrhagic disease of the newborn. Bleeding can also result from congenital coagulation defects and secondary coagulopathy resulting from infection. Other causes for lower GI bleeding can also lead to upper GI bleeding [4]. Haematological disorders such as Haemophilia A or B and Von Willebrand disease can also result in GI bleeding [6], although it was unlikely in our patient as the coagulation profile was normal. The presence of a palpable mass in the left upper quadrant initially raised the possibility of a splenomegaly. Conditions such as biliary atresia and portal vein thrombosis can lead to portal hypertension causing the development of oesophageal varices leading to upper GI bleed [7]. Normal bilirubin, liver enzymes and USS scan made the possibility of any liver pathology unlikely. The fact that in this case, the infant had the episode of melaena and haematemesis requiring fluid bolus and blood transfusion to maintain haemodynamic stability was an indication that an endoscopy may have been required [8]. However, this was not possible in our case. There are no studies in the published literature outlining the investigation required to determine the cause of an upper gastrointestinal bleed in a newborn infant. The methodology for the diagnostic pathway for establishing the cause of upper GI bleed has mainly been extrapolated from adult studies [4]. There have been other case reports of gastric teratoma published in the literature by Borelia et al. [9] and Yoon et al. [10] and Al-Salem [11] with a newborn presenting with respiratory distress and abdominal distention. But in those cases, the accurate diagnosis was made post-operatively after an exploratory laparotomy was performed which
2.3. Surgical management The infant underwent primary resection of the mass on day 17 of life. The paediatric surgeon performed a rooftop incision and noted that 95% of the stomach was intact. The tumour was peeled off from the posterior wall of the stomach, the pancreas and lesser sac. A complete resection of the 6 × 6.4 × 4.5 cm mass was achieved with primary closure of the stomach. No other organ was noted to be involved. The macroscopic appearance of the tumour is demonstrated in Fig. 1D. The procedure was uncomplicated. The serum AFP was monitored and it was noted to fall within the normal range post-operatively as noted on Table 1. 2.4. Histopathological findings Histology of the mass confirmed an immature gastric teratoma which was completely resected. The macroscopic appearance of the mass showed a cauliflower appearance. Microscopic examination of the mass as outlined in Fig. 1E demonstrated that the lesion was a complex of different tissues and histological structures including glands, fat, fibrous tissue, bone, cartilage, tooth, neural tissue and lymphoid tissue. 2
Journal of Pediatric Surgery Case Reports 47 (2019) 101222
S. Ramdeny, et al.
Fig. 1. (A) Magnetic resonance image demonstrating the heterogenous atypical mass in the abdomen. (B) Representative image from upper gastro-intestinal contrast study, demonstrating contrast outlining the gastric mass in a hugely distended stomach. Contrast flowed freely out of the stomach and no other upper GI abnormalities were detected. (C) Computed tomography showing heterogenous mass with calcified areas in the body of the distended stomach. (D) Macroscopic appearance of the mass after resection in theatre. (E) Histology of teratoma with mature and immature neuroectodermal component (thick arrows), cartilage (thin arrow), cystic structures (asterisks), and fibrous tissue.
performing an initial biopsy through endoscopic, percutaneous or open techniques. A study conducted by Kasrean et al. showed that performing an open surgical biopsy allowed the most accurate diagnosis in terms of the exact grade and subtype of tumour to be acquired in 100% of the cases. However, performing a fine needle aspiration (FNA) and core biopsies allowed the correct diagnosis to be obtained in only 33.3% and 45.6% of the cases respectively. Open biopsy of unknown soft tissue masses provided the most reliable way of allowing the precise diagnosis of defining the nature of the tumour to be established. Consequently, this will guide future treatment and management plan to be made accordingly [12].
was riskier. The other case report of immature gastric teratoma with the presentation of GI bleed and abdominal distention have not been in newborn but in older infants such as the case report by Junhasavasdikul et al. [5] who presents the case of an 8 months infant with Down syndrome who presented with haematemesis. This is first case report to the best of our knowledge of a newborn with normal antenatal scans who presented with GI bleeding and abdominal distention who had an accurate diagnosis of a gastric teratoma made pre-operatively to have undergone complete surgical resection of the tumour without the need for an explorative laparotomy. In our patient, we elected to openly resect the tumour instead of 3
Journal of Pediatric Surgery Case Reports 47 (2019) 101222
S. Ramdeny, et al.
Authorship.
In this particular case, a balanced decision was made to delay any surgical procedure as the life threatening bleed in the presence of a left upper quadrant mass on day 3 of life had been controlled with the use of tranexamic acid and ranitidine. The rationale was to confirm the diagnosis pre-operatively before performing a definitive surgical procedure in a stable neonate. We would like to stress the importance of performing different radiological investigation as well as repeating radiological investigation when the diagnosis is uncertain before performing a surgical procedure to resect a tumour. For instance, in this particular case a repeat abdominal ultrasound after an MRI scan guided the radiologist to perform a contrast study which led to the diagnosis of a gastric teratoma to be confirmed hence allowing the surgical intervention to be carried out appropriately in a safe way in a neonate. We would like to draw the attention of clinicians to consider the diagnosis of gastric teratoma as a differential diagnosis when dealing with an infant with abdominal mass associated with gastrointestinal bleed. A prompt diagnosis to allow the timely medical and surgical management remains the cornerstone of the management of gastric teratoma [5].
Conflict of interest The following authors have no financial disclosures: (PSR, KB, PS, SH, PC, MC). Consent Signed consent was obtained from legal guardians to publish anonymised information. Appendix A. Supplementary data Supplementary data to this article can be found online at https:// doi.org/10.1016/j.epsc.2019.101222. References [1] Sharma A, Arora R, Gupta R, Dinda AK. Immature gastric teratoma in an infant: report of a case and review of the literature. Indian J Pathol Microbiol 2010;53:868–70. [2] Eusterman GB, Sentry EG. Benign tumors of the stomach: report of twenty‐seven cases. Surg Gynecol Obstet 1922;34:372–8. [3] Mohta A, Sengar M, Neogi S, Khurana N. Gastric teratoma with predominant nephroblastic elements. Pediatr Surg Int 2010;26:923–5. [4] Owensby S DO, Taylor K, et al. Diagnosis and management of upper gastrointestinal bleeding in children. Fam Med January-February 2015;28(1):134–45. https://doi. org/10.3122/jabfm.2015.01.140153J Am Board. 5 Mohta A, Sengar M, Neogi S, Khurana N. Gastric teratoma with predominant nephroblastic elements. Pediatr Surg Int 2010;26:923–5. [6] Dolatkhah R, Khoshbaten M, Asvadi Kermani I, et al. Upper gastrointestinal bleedings in patients with hereditary coagulation disorders in northwest of Iran: prevalence of Helicobacter pylori infection. Eur J Gastroenterol Hepatol 2011;23:1172–7. [7] Goncalves ME, Cardoso SR, Maksoud JG. Prophylactic sclerotherapy in children with esophageal varices: long-term results of a controlled prospective randomized trial. J Pediatr Surg 2000;35:401–5. [8] Colle I, Wilmer A, Le Moine O, et alUpper gastrointestinal tract bleeding management: Belgian guidelines for adults and children. Acta Gastroenterol Belg2011;74:45–66. [9] Barolia DK, Mathur V, Gupta PK, Garg D, Yadav RK, Dogra N, et al. Immature gastric teratoma arising from lesser curvature. World J Surg Surgical Res 2018;1:1013. [10] Yoon S, Park G, Suh W, et al. Immature gastric terataom in a newborn : a case report. Neonatal Med 2017 May;24(2):97–100https://doi.org/10.5385/nm.2017. 24.2.97. [12] Kasraeian, et al. A comparison of fine-needle aspiration, core biopsy, and surgical biopsy in the diagnosis of extremity of soft tissue masses. Clin Orthop Relat Res 2010;468:2992–3002. https://doi.org/10.1007/s11999-010-1401.
4. Conclusion More studies are required to establish the best diagnostic approach to investigate the cause of the upper GI bleed in the paediatric population to allow for the early diagnosis so as to enable the most appropriate surgical management to be offered to the patient. The diagnostic approach should involve a multi-disciplinary team including a neonatologist, paediatric radiologist, paediatric haematologist, pathologist, paediatric oncologist and a paediatric surgeon. Patient consent Although this report does not contain any personal information that could lead to the identification of the patient, parents did consent for the details of the case to be reported. Funding No funding or grant support. Authorship All authors attest that they meet the current ICMJE criteria for
4
Contents lists available at ScienceDirect
Journal of Pediatric Surgery Case Reports journal homepage: www.elsevier.com/locate/epsc
Immature gastric teratoma in a newborn a
b
T c
d
a
Sandheeah Ramdeny , Katherine Broad , Prabhu Sekaran , Sara Harrison , Philip Connor , Mallinath Chakrabortyb,e,∗ a
Department of Paediatric Haemato-Oncology, University Hospital of Wales, Cardiff, UK Regional Neonatal Intensive Care Unit, University Hospital of Wales, Cardiff, UK Department of Paediatric Surgery, University Hospital of Wales, Cardiff, UK d Department of Paediatric Radiology, University Hospital of Wales, Cardiff, UK e Centre for Medical Education, Cardiff University, Cardiff, UK b c
A B S T R A C T
We present a full-term male infant with a palpable abdominal mass who presented with severe haematemesis associated with cardiovascular compromise. Although an initial ultrasound imaging identified a left supra-renal calcified mass suggesting a neuroblastoma, further imaging suggested an atypical mass which was separate from the spleen and left kidney. An upper gastro-intestinal contrast study localised the mass to the stomach and a computed tomograph suggested the possibility of a gastric teratoma likely in combination with a raised serum Alpha-fetoprotein. The mass was completely surgically resected, and histology confirmed an immature gastric teratoma.We would like to draw the attention of clinicians of the possibility of a gastric teratoma in an infant who presents with an abdominal mass and haematemesis.
1. Case report A male infant weighing 3.79 kg was born at term in good condition, requiring no resuscitation to non-consanguineous parents. Routine antenatal screening was unremarkable and there was no family history of note. The primiparous mother had no significant past medical history other than a previous early miscarriage. The infant fed well by bottle on formula milk and opened his bowels normally on day 1 of life. A palpable mass in the left hypochondrium was noted on the newborn examination, and an ultrasound scan (USS) was arranged as an outpatient. The infant appeared otherwise healthy. On day 3 of life, he had significant haematemesis resulting in cardiovascular compromise followed by repeated episodes of melaena, and required admission to the neonatal unit (NNU). There was a drop in serum haemoglobin from 21.6 g/dL to 12.8 g/dL. The infant required fluid resuscitation and packed red blood cell (PRBC) transfusion. The haematemesis stopped after intravenous administration of ranitidine and tranexamic acid. 2. Investigations 2.1. Blood and urine Full blood count (FBC) showed normal white blood cell (WBC) and platelet count with a significant drop in haemoglobin between day 2 and 3 of life. Red cell indices were normal. His baseline liver and kidney
∗
function were also normal. Serum Alpha-fetoprotein (AFP) level was 12,067 KU/L on day 3 of life and Beta human chorionic gonadotrophin (β-HCG) level was within the normal range as outlined in Table 1. Urinary excretion of catecholamine was within the normal range. These included Homovanillic acid (HVA): creatinine ratio of 13.5 μmol/mmol (normal range < 35) and 4-hydroxy-3-methoxy-mandelate (HMMA): creatinine ratio of 4.9 μmol/mmol (normal range < 20)]. Thus, apart from confirming an acute bleed, the above results did not point towards any specific diagnosis. 2.2. Radiological diagnostic dilemma A chest X-ray (CXR) and abdominal X-ray (AXR) performed during the episode suggested a large calcified mass in the left hypochondrium. Abdominal ultrasound scan (US) performed on day 4 of life initially suggested the location of the mass to be suprarenal, possibly arising from the left adrenal gland, raising the possibility of a neuroblastoma. At this point, he was referred to a tertiary centre. A magnetic reasonance imaging (MRI) scan was performed on day 6 of life which showed that there was a mass measuring 6 × 6.4 × 4.5 cm which appeared to arise from the adrenal glands. The mass was noted to be separate from the adjacent kidney and spleen. The mass was partially cystic and solid and was not entirely typical of a neuroblastoma. The results of the MRI scan (Fig. 1A) showed that the most likely diagnosis at the time was a neuroblastoma.
Corresponding author. Regional Neonatal Intensive Care Unit, University Hospital of Wales, Heath Park, Cardiff, CF14 4XW, UK. E-mail address: chakrabortym@cardiff.ac.uk (M. Chakraborty).
https://doi.org/10.1016/j.epsc.2019.101222 Received 4 April 2019; Accepted 11 May 2019 Available online 14 May 2019 2213-5766/ © 2019 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/).
Journal of Pediatric Surgery Case Reports 47 (2019) 101222
S. Ramdeny, et al.
There were numerous cysts with squamous, respiratory, gastric, intestinal or fallopian tube type epithelium. The pathologist concluded that the mass was an immature multicomponent teratoma and no other germ cells tumour had been identified.
Table 1 Blood results for tumour markers. Blood Results for Tumour Markers
Results
Unit and Range
AFP on day AFP on day AFP on day AFP on day AFP on day Beta- HCG LDH Uric acid
12,067 6249 288 113 18 1 579 209
KU/L Range (< 1600KU/L) KU/L Range (< 1600KU/L) KU/L Range (< 1600KU/L) KU/L Range (< 1600KU/L) KU/L Range (< 1600KU/L) IU/L Range (< 5) U/L Range (309–1222) umol/L Range (164–751)
3 of life 7 of life 25 of life, 9 days post-op 64 of life 172 of life
2.5. Progress and follow-up The infant recovered uneventfully on the neonatal unit. Feeding was fully established on day 28 of life and he was discharged home one day 30 of life. He continues to tolerate oral feeding and is gaining weight appropriately. His serum AFP level has dropped down to 288 KU/L. He will be regularly reviewed as outpatient with clinical and radiological surveillance with the aim to perform MRI scans every three months.
The results of the urinary catecholamine which was normal came back on the same day as the result of the MRI scan making the diagnosis of neuroblastoma unlikely. However, the serum AFP was 12, 067 KU/L suggestive of an alternative pathology. An abdominal USS was repeated on day 7 of life despite the fact that the abdominal USS scan on day 4 of life showed that the mass could be arising from the left adrenal gland. When the abdominal USS was performed, the left adrenal gland was identified in the expected position. There was a cystic structure that was noted which correlated with the posterior cystic structure lying in the suprarenal space on the MRI scan. The stomach appeared to be draped across the mass. A contrast study was then performed using a nasogastric tube (NG) which was placed within the stomach. Contrast which was instilled, initially appeared to flow around a filling defect. With subsequent passage of contrast, a lobulated filling defect was identified within a massively distended stomach. The stomach was not obstructed, contrast left the stomach to outline normal duodenum and proximal small bowel with no evidence of malrotation, as shown in Fig. 1B. It was then concluded that the mass was unusual in appearance and arose from within the stomach. A Computed Tomography (CT) scan of the abdomen with contrast which was subsequently performed showed that the mixed density mass contained foci of calcification and appeared likely to originate from the stomach but could possibly have arisen within the spleen and invaded the stomach as demonstrated in Fig. 1C. At this stage, the diagnosis of gastric teratoma was considered. After discussions amongst the paediatric radiologists, surgical and oncology teams, debating the risks and benefits of a biopsy versus a complete excision, a decision was taken to carry out primary resection of the tumour.
3. Discussion A teratoma is a germ cell tumour which consist of tissues that originate from the ectoterm, endoderm and mesoderm [1]. Teratoma, which is derived from the precursor totipotential stem cell accounts for the commonest germ cell tumour in paediatrics and can be classified as either arising from the gonadal tissue which present in younger children or the extragonadal tissue found in older children [2]. The location of extragonadal teratoma are mainly in the sacrococcygeal (60–65%) area, mediastinal (5–10%) area, sacral (5%) area and seldom within the intracranial, retroperitoneal and alimentary area [3]. Gastric teratoma is not a common presentation in the paediatric population and account for less than 1% amongst teratoma in paediatrics [4]. Furthermore, immature gastric teratoma is even rarer and there have been only 30 such cases which have been described in the literature [5]. It is associated with a good prognosis, once the tumour has been completely surgically excised. Adjuvant treatment with chemotherapy or radiotherapy is not clinically indicated. However, surveillance is required by measuring the level of AFP. Chemotherapy may be needed in the event of recurrence. The differential diagnosis of the possible aetiology of the upper gastrointestinal (GI) bleed in a neonate is wide and includes swallowed maternal blood or local causes such as oesophagitis, gastritis or vascular malformation, haemorrhage secondary to necrotising enterocolitis or haemorrhagic disease of the newborn. Bleeding can also result from congenital coagulation defects and secondary coagulopathy resulting from infection. Other causes for lower GI bleeding can also lead to upper GI bleeding [4]. Haematological disorders such as Haemophilia A or B and Von Willebrand disease can also result in GI bleeding [6], although it was unlikely in our patient as the coagulation profile was normal. The presence of a palpable mass in the left upper quadrant initially raised the possibility of a splenomegaly. Conditions such as biliary atresia and portal vein thrombosis can lead to portal hypertension causing the development of oesophageal varices leading to upper GI bleed [7]. Normal bilirubin, liver enzymes and USS scan made the possibility of any liver pathology unlikely. The fact that in this case, the infant had the episode of melaena and haematemesis requiring fluid bolus and blood transfusion to maintain haemodynamic stability was an indication that an endoscopy may have been required [8]. However, this was not possible in our case. There are no studies in the published literature outlining the investigation required to determine the cause of an upper gastrointestinal bleed in a newborn infant. The methodology for the diagnostic pathway for establishing the cause of upper GI bleed has mainly been extrapolated from adult studies [4]. There have been other case reports of gastric teratoma published in the literature by Borelia et al. [9] and Yoon et al. [10] and Al-Salem [11] with a newborn presenting with respiratory distress and abdominal distention. But in those cases, the accurate diagnosis was made post-operatively after an exploratory laparotomy was performed which
2.3. Surgical management The infant underwent primary resection of the mass on day 17 of life. The paediatric surgeon performed a rooftop incision and noted that 95% of the stomach was intact. The tumour was peeled off from the posterior wall of the stomach, the pancreas and lesser sac. A complete resection of the 6 × 6.4 × 4.5 cm mass was achieved with primary closure of the stomach. No other organ was noted to be involved. The macroscopic appearance of the tumour is demonstrated in Fig. 1D. The procedure was uncomplicated. The serum AFP was monitored and it was noted to fall within the normal range post-operatively as noted on Table 1. 2.4. Histopathological findings Histology of the mass confirmed an immature gastric teratoma which was completely resected. The macroscopic appearance of the mass showed a cauliflower appearance. Microscopic examination of the mass as outlined in Fig. 1E demonstrated that the lesion was a complex of different tissues and histological structures including glands, fat, fibrous tissue, bone, cartilage, tooth, neural tissue and lymphoid tissue. 2
Journal of Pediatric Surgery Case Reports 47 (2019) 101222
S. Ramdeny, et al.
Fig. 1. (A) Magnetic resonance image demonstrating the heterogenous atypical mass in the abdomen. (B) Representative image from upper gastro-intestinal contrast study, demonstrating contrast outlining the gastric mass in a hugely distended stomach. Contrast flowed freely out of the stomach and no other upper GI abnormalities were detected. (C) Computed tomography showing heterogenous mass with calcified areas in the body of the distended stomach. (D) Macroscopic appearance of the mass after resection in theatre. (E) Histology of teratoma with mature and immature neuroectodermal component (thick arrows), cartilage (thin arrow), cystic structures (asterisks), and fibrous tissue.
performing an initial biopsy through endoscopic, percutaneous or open techniques. A study conducted by Kasrean et al. showed that performing an open surgical biopsy allowed the most accurate diagnosis in terms of the exact grade and subtype of tumour to be acquired in 100% of the cases. However, performing a fine needle aspiration (FNA) and core biopsies allowed the correct diagnosis to be obtained in only 33.3% and 45.6% of the cases respectively. Open biopsy of unknown soft tissue masses provided the most reliable way of allowing the precise diagnosis of defining the nature of the tumour to be established. Consequently, this will guide future treatment and management plan to be made accordingly [12].
was riskier. The other case report of immature gastric teratoma with the presentation of GI bleed and abdominal distention have not been in newborn but in older infants such as the case report by Junhasavasdikul et al. [5] who presents the case of an 8 months infant with Down syndrome who presented with haematemesis. This is first case report to the best of our knowledge of a newborn with normal antenatal scans who presented with GI bleeding and abdominal distention who had an accurate diagnosis of a gastric teratoma made pre-operatively to have undergone complete surgical resection of the tumour without the need for an explorative laparotomy. In our patient, we elected to openly resect the tumour instead of 3
Journal of Pediatric Surgery Case Reports 47 (2019) 101222
S. Ramdeny, et al.
Authorship.
In this particular case, a balanced decision was made to delay any surgical procedure as the life threatening bleed in the presence of a left upper quadrant mass on day 3 of life had been controlled with the use of tranexamic acid and ranitidine. The rationale was to confirm the diagnosis pre-operatively before performing a definitive surgical procedure in a stable neonate. We would like to stress the importance of performing different radiological investigation as well as repeating radiological investigation when the diagnosis is uncertain before performing a surgical procedure to resect a tumour. For instance, in this particular case a repeat abdominal ultrasound after an MRI scan guided the radiologist to perform a contrast study which led to the diagnosis of a gastric teratoma to be confirmed hence allowing the surgical intervention to be carried out appropriately in a safe way in a neonate. We would like to draw the attention of clinicians to consider the diagnosis of gastric teratoma as a differential diagnosis when dealing with an infant with abdominal mass associated with gastrointestinal bleed. A prompt diagnosis to allow the timely medical and surgical management remains the cornerstone of the management of gastric teratoma [5].
Conflict of interest The following authors have no financial disclosures: (PSR, KB, PS, SH, PC, MC). Consent Signed consent was obtained from legal guardians to publish anonymised information. Appendix A. Supplementary data Supplementary data to this article can be found online at https:// doi.org/10.1016/j.epsc.2019.101222. References [1] Sharma A, Arora R, Gupta R, Dinda AK. Immature gastric teratoma in an infant: report of a case and review of the literature. Indian J Pathol Microbiol 2010;53:868–70. [2] Eusterman GB, Sentry EG. Benign tumors of the stomach: report of twenty‐seven cases. Surg Gynecol Obstet 1922;34:372–8. [3] Mohta A, Sengar M, Neogi S, Khurana N. Gastric teratoma with predominant nephroblastic elements. Pediatr Surg Int 2010;26:923–5. [4] Owensby S DO, Taylor K, et al. Diagnosis and management of upper gastrointestinal bleeding in children. Fam Med January-February 2015;28(1):134–45. https://doi. org/10.3122/jabfm.2015.01.140153J Am Board. 5 Mohta A, Sengar M, Neogi S, Khurana N. Gastric teratoma with predominant nephroblastic elements. Pediatr Surg Int 2010;26:923–5. [6] Dolatkhah R, Khoshbaten M, Asvadi Kermani I, et al. Upper gastrointestinal bleedings in patients with hereditary coagulation disorders in northwest of Iran: prevalence of Helicobacter pylori infection. Eur J Gastroenterol Hepatol 2011;23:1172–7. [7] Goncalves ME, Cardoso SR, Maksoud JG. Prophylactic sclerotherapy in children with esophageal varices: long-term results of a controlled prospective randomized trial. J Pediatr Surg 2000;35:401–5. [8] Colle I, Wilmer A, Le Moine O, et alUpper gastrointestinal tract bleeding management: Belgian guidelines for adults and children. Acta Gastroenterol Belg2011;74:45–66. [9] Barolia DK, Mathur V, Gupta PK, Garg D, Yadav RK, Dogra N, et al. Immature gastric teratoma arising from lesser curvature. World J Surg Surgical Res 2018;1:1013. [10] Yoon S, Park G, Suh W, et al. Immature gastric terataom in a newborn : a case report. Neonatal Med 2017 May;24(2):97–100https://doi.org/10.5385/nm.2017. 24.2.97. [12] Kasraeian, et al. A comparison of fine-needle aspiration, core biopsy, and surgical biopsy in the diagnosis of extremity of soft tissue masses. Clin Orthop Relat Res 2010;468:2992–3002. https://doi.org/10.1007/s11999-010-1401.
4. Conclusion More studies are required to establish the best diagnostic approach to investigate the cause of the upper GI bleed in the paediatric population to allow for the early diagnosis so as to enable the most appropriate surgical management to be offered to the patient. The diagnostic approach should involve a multi-disciplinary team including a neonatologist, paediatric radiologist, paediatric haematologist, pathologist, paediatric oncologist and a paediatric surgeon. Patient consent Although this report does not contain any personal information that could lead to the identification of the patient, parents did consent for the details of the case to be reported. Funding No funding or grant support. Authorship All authors attest that they meet the current ICMJE criteria for
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