Gastric teratoma disclosed by neonatal digestive hemorrhage

A gastric teratoma diagnosed after a gastro-intestinal tract bleeding in a neonate is reported. The endogastric tumor was shown by gastric endoscopy. The tumor was pediculated and a simple tumorectomy was performed, without trouble later. Fifty-three other cases have been found in the literature. Most of them presented with abdominal distension and a palpable mass; diagnosis was always made after surgery and the diagnosis of mature gastric teratoma was confirmed by histological examination. These rare tumors are always of benign nature, but are often revealed by complications. Their frequency is less than 1% of infants teratoma and 85% are found in the first year of life; they are more frequent in males.     

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, 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 × 11 cm × 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.     

Gastroschisis with extracorporeal liver and stomach associated with sacrococcygeal teratoma and limb abnormalities

Gastroschisis is the herniation of abdominal viscera through a paramedian abdominal wall fusion defect without involvement of the umbilical cord. Evisceration usually contains intestinal loops and has no surrounding membrane. Rarely, herniation of other major viscera such as stomach and liver occurs, which makes the prognosis worse. Gastroschisis is usually not associated with sacrococcygeal teratoma. In the present report, a very rare case of gastroschisis associated with sacrococcygeal teratoma is described. The gastroschisis had complete evisceration of the stomach, bowel and extracorporeal liver. A large sacrococcygeal mass was located on the posteroinferior part of the trunk and gluteal region, and was completely external. The fetus also showed a malrotated lower limb and talipes equinovarus.     

Ovary-sparing surgery for teratomas in children

Aim

To share our experience in ovary-sparing surgery for teratomas in children.

Patients and methods

The medical records of nine patients (mean age of 11.2 years, r 6–15 years) who had undergone ovary-sparing surgery for teratoma were analyzed retrospectively. Mean duration for follow-up was 29.5 months (r 15–75 months).

Results

Five patients suffered from chronic abdominal pain; two had acute colicky abdominal pain. In two patients, there was no presenting clinical symptom. Two patients were operated on emergency basis due to symptoms related with acute abdomen. On the other hand, seven were operated electively. Ultrasonography was performed in all patients. Additionally, MRI and tumor markers were performed in all but two. Main radiologic findings consisted of heterogenous cystic and solid ovarian masses predictive of teratoma. The definitive diagnosis in emergency cases were as follows: perforated appendicitis plus teratoma [1]; torsion of the ovarian mass with teratoma [1]. The final diagnosis in electively treated seven patients were: unilateral ovarian teratoma [4], bilateral ovarian teratoma [1], bilateral teratoma plus appendiceal inflammatory mass [1], unilateral teratoma and contralateral corpus hemorrhagicum cyst [1]. The operations were performed by open conventional surgery in six and laparoscopy in three patients. The procedures were ovary-sparing surgery in 12 ovaries, appendectomy in 2 patients and detorsion of ovary in 1 patient. The main indication for ovary-sparing surgery was the “existence of a perfect dissection plane between the tumor margins and healthy ovarian tissue”. The remaining ovarian tissue was evaluated macroscopically for residual lesions. Frozen section was performed in three suspected patients and the ovarian margins were free of any tumor cell. The pathologic diagnosis was: mature cystic teratoma in 10, immature teratoma in 1 and corpus hemorrhagicum cyst in 1. The postoperative outcome and follow-up was uneventful.

Conclusion

Heterogenous ovary mass containing solid and cystic portions with echogenic areas on ultrasound imaging is highly suggestive of ovarian teratomas. Emergent surgical intervention is indicated if there is any suspicion of ovarian torsion. Otherwise, MRI is performed for further radiological evaluation. Based on radiologic findings, ovary-sparing surgery can be safely performed if the preoperative diagnosis is teratoma and there is always a plane of dissection between the normal ovary and cyst wall.     

Malignant teratoma of the greater omentum

A 9-year-old girl with a malignant teratoma of the greater omentum is presented. The main presenting symptoms and signs were mild abdominal pain and a palpable abdominal mass. Exploratory laparotomy revealed a huge tumor occupying the peritoneal cavity and stemming exclusively from the greater omentum. This case represents an uncommon site of development of a malignant teratoma and may be the only one in the literature.     

Retroperitoneal teratomas in infancy and childhood

Retroperitoneal teratoma poses a significant problem in the differential diagnosis of Wilms’ tumor, neuroblastoma, and other intraabdominal tumors. In an attempt to establish the best diagnostic and treatment modality, we reviewed our experience with retroperitoneal teratomas at a single institution in a 5-year period. Between January 1998 and December 2002, retroperitoneal teratomas were identified in 10 patients, seven females and three males, ranging from age 4 days to 12 years, with seven patients under the age of 1 year. The presence of calcifications or bony structure within the tumor was revealed on abdominal ultrasound study or computed tomography in nine of the 10 lesions. Total excision was performed in nine patients; another patient with grade III immature teratoma received postoperative chemotherapy besides excision. One patient with grade III immature teratoma who did not receive postoperative chemotherapy had a local recurrence 6 months later and was treated by repeated surgical excision and postoperative chemotherapy. All of these patients were free of disease at 8 months to 5 years of follow-up. Retroperitoneal teratomas were usually noted in patients under the age of 1 year. Though large, they are mostly benign lesions with no apparent connection to the retroperitoneal organs and are amenable to curative excision, but histologic evidence of grade III immature teratoma or malignancy demands aggressive postoperative chemotherapy to prevent local recurrence.     

Sonographic diagnosis of solid space-occupying abdominal lesions in childhood

Between 1981 and May 1986 31 children with solid abdominal tumor masses were observed in our clinic. The first diagnostic procedure was a sonographic examination, followed by further radiological investigations if necessary. 30 cases were examined histologically; in one case the sonographic findings were confirmed by an angiography. The most frequent abdominal masses were neuroblastomas and Wilms tumors (7 cases each). A mesoblastic nephroma was diagnosed in 3 cases, a lymphoma, a hepatoblastoma and a rhabdomyosarcoma 2 times each. One time we found a pancreas carcinoma, a teratoma, a hemangiomatosis of the liver, a malignant Schwannoma, a Ewing sarcoma, an adenoma of the adrenal gland, a pheochromocytoma and an osteosarcoma. According to our own experience and recent reports in the literature it seems possible in most cases, to predict the correct diagnosis of solid abdominal masses using the informations of sonographic imaging. Sonography is a highly specific non-invasive diagnostic tool for planning treatment (e.g. early surgery, cytostatic therapy and/or radiation) of solid abdominal masses. Nevertheless the histological examination should be performed in every case to confirm the definitive diagnosis.     

胎儿腹腔囊性占位的生后治疗及转归

目的 分析产前诊断胎儿腹腔囊性占位的病因、随访、预后及转归,指导产前会诊咨询工作.方法 收集2006年4月至2009年4月产前发现胎儿腹腔囊性占位的病例32例,分析病因、就诊年龄、占位大小、手术治疗效果及随访情况,总结预后与转归.结果 32例患儿,男女比例为7:25,女性占绝对多数.新生儿期就诊23例,其余生后3个月内就诊.就诊时囊性占位较产前明显增大者7例(21.9%).病因包括卵巢囊肿、畸胎瘤、肠源性囊肿、肾上腺来源性占位及胆总管囊肿.27例经Ⅰ期手术预后良好.2例经Ⅱ期手术恢复良好.2例未行手术预后良好.1例因恶性肿瘤死亡.结论 胎儿腹腔囊性占位以女性多见,以卵巢囊肿最多见.肠重复畸形、囊性畸胎瘤亦是常见病因.多数患儿因产前发现肿块在新生儿早期就诊.巨大腹腔囊性肿块造成消化道梗阻症状是早期就诊的主要原因.腹腔囊性占位的胎儿多数预后良好,转归满意.

Abstract：

Objective To summarize our experience with prenatal diagnosis of the fetus with cystic abdominal mass, and retrospectively analyze their clinical outcome after birth. Methods From April 2006 to April 2009, 32 fetuses were diagnosed with cystic abdominal mass in prenatal tests.Their clinical data including etiology, age at diagnosis, mass size, surgical treatment and follow-up were retrospectively analyzed. Results The 32 cases included 7 males and 25 females. Twenty three (71.9%) patients visited doctors in the neonatal period. The other patients (71.9%) visited doctors in the first 3 months after birth. The chief compliant was intestinal obstruction caused by abdominal mass. Seven patients' (21.9%) cystic abdominal masses enlarged after birth. The origins of the cystic abdominal masses were ovarian cysts, teratorna, enterogenous cysts, adrenal tumors and common bile duct cysts. Among the 32 patients, 27 patients underwent one stage tumor resection, and 2 were performed 2 stage surgery. Two patients didn't receive any surgery. And 1 patient with malignant tumor died. All patients recovered eventually from surgery. Conclusions Cystic abdominal mass in female fetus is more common than that in male fetus. The most common origin of cystic abdominal mass in female fetus is ovarian cysts. Intestinal duplication and cystic teratoma is also the common origins. Most neonates visit doctors for prenatal diagnosis of cystic abdominal mass. The prognosis of the majority of fetus with abdominal cyst is good.     

Split liver transplantation for retroperitoneal immature teratoma masquerading as hepatoblastoma

Extragonadal teratoma originating from the retroperitoneum represents less than 5% of all teratomas and accounts for less than 10% of all pediatric retroperitoneal neoplasms. To date, there has been no report of teratoma managed with LT. This study reports an infant aged 3 months with retroperitoneal immature teratoma involving the hepatic hilum, refractory to chemotherapy and treated with LT. The patient was referred to our hospital for management of a growing abdominal mass. Histopathology of a fine needle biopsy of the lesion suggested the possibility of a hepatoblastoma with teratoid features. Cisplatin‐based chemotherapy was initiated, but rapid growth of the tumor encasing the hepatic artery proper was detected, even after two cycles of chemotherapy. A split LT was carried out, and pathological examination of the explanted liver revealed the involvement of numerous neuroepithelial components, confirming the diagnosis of a Norris grade 3 immature teratoma. The patient recovered well and was discharged on day 19 post‐LT. As on date, on postoperative day 240, he has completed seven cycles of a 12‐cycle vinblastine and doxorubicin‐based adjuvant chemotherapy.     

A Huge Intrapericardial Teratoma in an Infant

A case of huge intrapericardial teratoma diagnosed in a 9-month-old infant is presented. Echocardiography and thoracic computed tomography showed a voluminous multicystic intrapericardial mass with pericardial effusion. The tumor was resected surgically. Histopathological examination confirmed the diagnosis of immature teratoma.     

Prenatal ultrasonographic detection of abdominal and pelvic tumors: impact on management

An abdominal or pelvic tumor in very seldom detected in a prenatal ultrasound examination. The most commonly detected tumors are, in order of decreasing frequency, sacrococcygeal teratomas, neuroblastomas and kidney tumors. According to the present state of the art, diagnosis of a sacrococcygeal teratoma only leads to specific monitoring of the pregnancy, because an early delivery or delivery by cesarean section will be necessary in some cases to improve the prognosis of child and/or mother. For other types of tumors investigations and actions should be delayed until after birth.     

De novo spinal teratoma after treatment of an intracranial germ cell tumor

The authors report an extremely rare case of de novo spinal teratoma after treatment for intracranial germ cell tumor. A 17-year-old male developed pain of bilateral lower extremities and urinary retention 18 months after complete remission of intracranial mixed germ cell tumor. Magnetic resonance imaging revealed a huge spinal tumor associated with spina bifida occulta. Total resection was performed, and histogenetical findings led to the diagnosis of a mature teratoma with normal p16 gene, whereas analysis of intracranial tumor showed p16 deletion. The spinal anomaly and genetic analysis strongly suggest that the spinal teratoma was a de novo tumor rather than a metastasis or dissemination of the original intracranial germ cell tumor.     

Intraosseus teratoma of the iliac bone

We present a 4-year-old child with a large iliac bone mass incidentally discovered in a plain abdominal radiograph. The pathological examination revealed a benign mature teratoma. To the best of our knowledge, the occurrence of intraosseous mature teratoma has not been previously reported. The child had had an immature teratoma of the neck discovered in a fetal ultrasound, and resected on day 6 of life. The neck teratoma recurred twice, at 16 months and at 3.5 years of age. In these two recurrences the lesion appeared progressively more mature. At the time of discovery of the iliac bone teratoma there was no evidence of residual neck disease. The radiological and pathological characteristics, differential diagnosis, and clinical course are discussed. Received: 2 November 1999/Accepted: 9 November 1999     

儿童未成熟畸胎瘤与成熟畸胎瘤中神经上皮组织的生物学特点分析

目的 对儿童未成熟畸胎瘤和成熟畸胎瘤内决定肿瘤分化程度的神经上皮组织进行多项免疫组化指标检测,分析肿瘤细胞的生物学特性.方法 收集儿童畸胎瘤病理组织标本55例,根据病理诊断分为成熟畸胎瘤和未成熟畸胎瘤二组,提取肿瘤内神经上皮组织标本分别进行能反映肿瘤细胞增殖、凋亡、侵袭力和神经胶质细胞成熟度的Bax、Bcl-2、PCNA、Ki-67、CyclinE、p14ARF/p16β、MMP-9和GFAP各指标分别进行免疫组化染色.结果 未成熟畸胎瘤中的神经上皮组织CyclinE呈低表达,成熟畸胎瘤也呈低度表达;p14AW/p16β两组均为较高表达;PCNA在未成熟畸胎瘤呈较高表达,在成熟畸胎瘤中较低表达;Ki-67在未成熟畸胎瘤呈较低表达,在成熟畸胎瘤中表达程度较前者更低;Bcl-2在未成熟畸胎瘤呈中度表达,而在成熟畸胎瘤中为较低表达;Bax在二组均呈中度表达;MMP-9在二组均为较低表达;而GFAP在未成熟畸胎瘤为低表达,而在成熟畸胎瘤中则为高表达.结论 儿童未成熟畸胎瘤中分化程度较低的神经上皮组织具有一定的增殖、分化、侵袭转移能力,但在良恶性指标方面仍接近成熟畸胎瘤的分子生物学特点,偏向良性或交界性肿瘤.GFAP可能是成熟与未成熟畸胎瘤的一种有效的鉴别指标.     

Malignant gastric sarcoma — diagnosis by ultrasound and endoscopy

A 14-year-old boy was presented with a mass in the epigastrium. The tumor developed 13 years after removal of a neuroblastoma of the left adrenal gland. Therapy of the primary tumor had included irradiation and chemotherapy. Diagnosis of the gastric tumor was made by ultrasound and endoscopy. A total gastrectomy was performed showing a malignant undifferentiated sarcoma. Primary malignant tumors of the stomach such as gastric teratoma of the newborn [4], leiomyosarcoma [6], malignant lymphoma and carcinoma [1] are very rare in childhood. As cancer is treated more aggressively with radiation and cytostatics, more therapy-induced-tumors, including gastric sarcoma, must be expected in children.     

儿童未成熟畸胎瘤肿瘤标志物基础研究进展

儿童未成熟畸胎瘤成分复杂,具有潜在恶性。特异性肿瘤标志物、肿瘤生物学行为的进一步研究将为临床诊疗、随访、预后评估提供更多的客观指标。文章就目前国内外比较有争议的肿瘤标志物包括AFP异质体L3、神经元特异性烯醇化酶、波形蛋白、神经细胞特异抗原等,并结合癌基因和抑癌基因、细胞周期相关抗原和端粒酶、染色体核型分析等进行归纳总结,为临床治疗和随访提供病理和生物学上的资料,为临床治疗和预后评估提供参考。     

Intraperitoneal testicular fetus in fetu

A 5-month-old boy presented with a right flank abdominal mass. Plain film of the abdomen showed a vertebral column within the mass. Surgery confirmed the diagnosis of fetus in fetu developed within an ectopic intraperitoneal right testicle. Fetus in fetu differs from teratoma by the presence of axial skeleton and organogenesis. When intratumoral axial skeleton is present, plain films are sufficient for the diagnosis.     

Bilateral testicular teratoma in infancy: report of a rare case treated by testis-sparing surgery.

Testicular tumors are rarely seen in childhood, with germ-cell tumors as the most common type. Teratoma is second only to yolk sac tumor in frequency. Bilateral testicular teratoma is extremely rare. Orchiectomy has been the standard treatment for most tumors of the testes in the past. However, in children, testis-sparing surgery has become the treatment of choice for benign lesions including teratoma. A 10-month-old infant with synchronous bilateral testicular teratomas, one of which was disclosed by ultrasonography, is presented here. While the larger teratoma required unilateral orchiectomy, the smaller tumor in the contralateral testicle was enucleated by testis-sparing surgery. The follow-up at three years was uneventful with normal development of the testis and the boy. Testicular teratoma may reside in both testicles without clinical symptoms. Ultrasound of both testes provides coherent preoperative diagnosis, allowing the surgeon to consider testicular-sparing procedures, thus preventing anorchia in these rare occurrences.