(Acta Paediatr Jpn 1992; 34: 483

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488)

Immature Gastric Teratoma in an Infant Nestor A. Mutioz, M.D.’, Hiroo Takehara, M.D.’, Nobuhiko Komi, M.D.’ and Kazuo Hizawa, M.D.2



The First Department of Surgery and ’The First Department of Pathology, School of Medicine, The University of Tokushima, Tokushima, Japan

A 45 day old boy presented with progressive abdominal distension, tarry stools and anemia. A plain roentgenogram of the abdomen showed irregular, coarse calcifications in the left upper quadrant. The ultrasonography and computerized tomography of the abdomen revealed a large heterogeneous tumor with calcified parts in the left hemiabdomen. At operation, a 12 cm x 11cm x 10 cm, multilobular, exogastric and endogastric tumor, including a portion of the anterior wall of the stomach, was extirpated. The pathological examination revealed a gastric teratoma with immature neural elements. Eight years after the total excision of the tumor there has not been any recurrence. This study includes a review of 88 cases (including the present case) of gastric teratoma reported in the English and Japanese literature.

Key Words Gastic teratoma, Teratoma

Introduction Gastric teratomas are rare neoplasms that occur almost exclusively in male infants [l]. These tumors present special characteristics, such as male preponderance and a benign nature, that are not shared with teratomas of other sites [ 11. A recent search of the literature found that 87 cases have been reported (61 in the English literature and 26 in the Japanese literature) and that only five cases were females [2-51. The presence of immature elements have been described in only three cases [6-81. Nevertheless, Received December 25, 199 1 Revised March 6. 1992 Accepted March 13, I992 Correspondence address: Professor Nobuhiko Komi, The First Department of Surgery, School of Medicine, The University of Tokushima, 3-8-1 5 KuramotoCho, Tokushima 770, Japan.

the prognosis for gastric teratomas is regarded as excellent after they have been completely excised [ 1-81. We report a gastric teratoma in a 45 day old boy, in which the histological examination disclosed immature neural tissue. To our knowledge this is the 88th case reported, and the fourth with immature elements.

Case Report A 45 day old boy was referred to our institution due to an abdominal tumor. He was born at 43 weeks of gestation by a normal delivery after an uneventful pregnancy. His birthweight was 3,410 g, and his family history was unremarkable. Since 1 month of age, his parents noticed progressive abdominal distension and he had presented tarry stools several times before admission.

484 ( 100) Muiioz et al.

Physical examination showed a boy with slight pallor weighing 5,010 g. The abdomen was distended by a firm, hard, non-tender tumor of irregular surface occupying the left upper abdominal quadrant, which extended beyond the midline and inferiorly below the level of the umbilicus. Laboratory examination showed hemoglobin (9.3 g/dl). hematocrit (28.8%), reticulocytes (33%),total protein (5.1 g/dl), albumin (3.4 g/dl) and a-fetoprotein ( 1,600 ng/ml). Liver function tests, renal function tests and electrolytes were all within normal limits. A plain roentgenogram of the abdomen showed irregular calcifications in the left upper quadrant of the abdomen, and an upper gastrointestinal series showed an irregular intragastric mass on the anterior wall of the stomach (Fig. 1).

Computerized tomography of the abdomen showed a large solid and cystic tumor with some calcified parts occupying the left upper hemiabdomen (Fig. 2). An abdominal aortography showed a hypervascular mass displacing the left renal artery inferiorly and the superior mesenteric artery medially (Fig. 3). Abdominal ultrasonography showed a heterogeneous cystic mass of the left upper abdomen with internal hypoechoic and hyperechoic areas. A barium enema revealed inferior-medial displacement of the splenic flexure and descending colon. At laparotomy a large multinodular tumor with a smooth white/yellow surface that did not adhere to other organs was found arising from the anterior wall at the body of the stomach, adjacent to the lesser curvature. The tumor was attached to the esophago-cardiac junction, at which it was dissected and then extirpated along

Fig. I : Upper gastrointestinal tract contrast study. The anteroposterior projection shows an irregular intragastric mass. The lateral projection shows the large calcified mass, which occupies and displaces the anterior gastric wall posteriorly (= ).

Fig. 1: Computerized tomography of the abdomen showing a large calcified mass (t) with cystic and solid areas occupying the left hemiabdomen and extending across the midline.

Fig. 3: Abdominal aortography. There is inferior displacement of the Ieft renal artery ( t ) and medial displacement of the superior mesenteric artery (T). Hypervascularity is seen at the left of the aorta, above the right gastroepiploic artery (t).

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Gastric teratoma (101) 485

b

Fig. 4: (a) Intra-operative photograph of the tumor. The tumor is being pulled downward after dissection from the esophago-cardiac junction (+. (b) Schematic diagram of (a) showing the relation of the tumor with the stomach. L, liver; F, fundus; A, antrum; T, tumor.

with a portion of the normal gastric wall (Fig. 4). The gastric defect was closed in two layers and a gastrostomy performed. The resected specimen measured 12 cm x 1 1 cm x 10 cm and weighed 550 g. The tumor was firm, multilobular, covered by a fibrous capsule and presented a portion of endogastric growth (Fig. 5). Histological examination revealed a disorganized mixture of mature tissues from all germ layers. Several sections of the tumor revealed mature bone and cartilage, bone marrow, fat, respiratory and gastrointestinal epithelium, smooth muscle, and skin with appendages such as hair follicles, sebaceous glands and sweat glands. There were some cysts containing keratinized material, and primitive tooth tissue was

Vol. 34 No. 4 August 1992

Fig. 5: Gross appearance of the resected specimen. measuring 12 cm x 11 cm x 10 crn in diameter and 550 g in weight. The arrow points to the endogastric portion of the tumor.

also found. Neural tissue was found in all stages of maturation. Neural canal cells containing melanin pigment and choroid plexus cells were observed. The immature neural cells were small and dark with little cytoplasm (Fig. 6). The postoperative course of the patient became complicated with wound infection, anemia and hypoproteinemia, which responded to antibiotics, transfusions and intravenous alimentation. He has been followed-up for 8 years without any recurrence or symptoms.

Discussion Teratomas may arise from the gonads or from extragonadal sites, like the sacrococcygeal and presacral area, the cervicopharyngeal area, the retroperitoneum, within the cranium, the thoracic cavity or the abdominal cavity [I]. The most frequent extragonadal teratoma in children

486 ( 102) Muiiuz el al.

Fig. 6: Photomicrographs of the gastric teratoma. (a) A submucosal portion of the tumor showing well-defined tumor tissue beneath the lamina muscularis mucosa composed of mature bone, cartilage, connective tissue and blood vessels. Magnification x 40. (b) Immature nervous tissue, with small tumor cells forming neural tube-like structures. Magnification x 100. (c) A higher magnification of immature nervous tissue ( x 200) demonstrates medulloepithelioma-like small neural cells. Several mitoses can be seen.

is the sacrococcygeal area [ 1, 91, whereas gastric teratomas are rare and present the lowest incidence (about 1%) of all teratomas [3, 101. Several authors in the past have reviewed the literature on gastric teratoma [6, 9, 111, and 55

cases were collected from the English literature and 25 cases from the Japanese literature in 1983 [ 2 ] . Until now, 61 cases in the English literature and 26 cases in the Japanese literature have been recorded [I-141.

Acta Paediatr Jpn

Gastric teratoma (1 03) 487 All authors have stressed the striking male preponderance (contrary to sacrococcygeal teratomas and teratomas of other regions), and the essentially benign nature of gastric teratomas [ 1- 141, although there is not yet a clear explanation of either feature. Gastric teratomas have been reported in only five female cases [2-51, of which one was in a 37 year old woman [2]. All gastric teratomas have been reported to be benign, with no malignant elements in any tumor of any size or even in those found in adults [l-141. Only three cases have presented with immature elements [6-81. As immaturity does not always indicate malignancy, and neither metastasis nor recurrence have ever been reported, gastric teratomas are considered to be benign with an excellent prognosis after their total excision [ 1-3,6-141. However, mortality has been reported in 11 cases, of which four cases died without operation due to the moribund condition of the patients, and the others died from postoperative complications [2,9]. Therefore, the complete excision of these tumors is always warranted. The majority of these tumors have been diagnosed during the first 3 months of life, and more than 90% have been diagnosed in chil-dren less than 1 year old [2, lo]. The clinical presentation is usually of an abdominal tumor palpable beyond the midline that causes abdominal distension and may cause respiratory distress [l-141. They have been also repcrted to cause premature labor or dystocia [5, 111. Bleeding of the gastrointestinal tract associated with anemia

may occur if the tumor exhibits endogastric growth [4,6, 7, 10, 121. The differential diagnosis should include neuroblastoma, Wilms’ tumor, pancreatic cyst, hepatoblastoma, intestinal duplication, fetus in fetu and retroperitoneal teratoma. While imaging examinations and contrast studies usually suggest a teratoma, a diagnosis of gastric teratoma has never been made pre-operatively [ 1-14]. Furthermore, a retroperitoneal teratoma may be firmly attached to the gastric wall, and may eventually be misdiagnosed as a gastric teratoma. Among the 77 cases fully described (including the present case), the growth of the tumor has been solely exogastric in 65% of cases, solely endogastric in 9% of cases and endolexogastric in 26% of cases (Table 1) [l-141. On the other hand, the most common site of teratomas in the stomach was the posterior wall in 31% of cases, followed by the anterior wall in 13Y0 of cases (Table 1) [l-141. Thoracic extension of gastric teratomas has been reported in three cases [ 5 , 13, 141. The optimum treatment is complete excision of the tumor, but the surgical procedure depends on the extent of involvement of the stomach [I-141. The presence of immature elements does not seem to imply malignancy in gastric teratoma, but morbidity and mortality may occur due to complications related to the tumor itself, such as severe gastrointestinal bleeding, life-threatening respiratory distress or malnutrition.

Table 1. Location in the stomach and type of growth of gastric teratorna in 88 cases Growth location

Solely endogastric

Solely exogastric

Endogastric and exogastric

Not reported

Posterior wall 14 4 I 2 (1) 2** 1 Anterior wall 9 Minor curvature 5 (2) 2 3 (1) 2 Major curvature 6 1 Posterior/minor 6 2 (1) Posterior/major 1 1 Anterioriminor 4* 2 Anterior/major 1 Fundus I Entire circumference 1 Not reported 3 1 2 6* Number of female cases are within parentheses. *One case associated with intrathoracic extension of the tumor I l l , 13, 14J. **Numbers include the present case.

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488 (104) Mufioz et al. The present case did not present respiratory distress or any other symptoms that may impede the immediate operation. Therefore, sufficient pre-operative nutritional therapy may prevent the postoperative complication.

7. 8. 9.

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Woolley MM. Teratoma. In Welch KJ. Randolph JG, Ravitch MM et al. eds: Pediatric Surgery. 4th ed. Chicago L, 1989, Year Book Medical Publishers, p 265-276. Kawamitsu T. Nagashima K, Ikeda Set al. Gastric teratoma of a newborn. Jpn J Pediatr Surg 1984: 16: 6 17-627 (in Jpn). Senocak ME, Kale G. Buyukpamukcu N et al. Gastric teratoma in children including the third reported female case. J Pediatr Surg 1990: 2 5 : 681-684. Purvis JM, Miller RC, Blumenthal B1. Gastric teratoma: First reported case in a female. J Pediatr Surg 1979: 14: 86-87. Esposito G., Cigliano B, Paludetto R. Abdominothoracic gastric teratoma in a female newborn infant. J Pediatr Surg 1983: 18: 304-305. Moriuchi A. Nakayama 1. Muta H et al. Gastric teratoma of children - A case report with review

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Acta Paediatr Jpn

Immature gastric teratoma in an infant.

A 45 day old boy presented with progressive abdominal distension, tarry stools and anemia. A plain roentgenogram of the abdomen showed irregular, coar...
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