Lumbar teratoma with intraspinal extension

Teratomas are childhood tumours especially notable for their diversity of anatomic locations and biological behaviour. We present an extremely rare occurrence of a lumbar teratoma with intraspinal extension. The importance of thorough preoperative evaluation and magnetic resonance imaging for tumours in this location is also emphasised.     

Neurocytoma arising in an adrenal gland mature teratoma

Mature teratoma of adrenal gland is a rare entity. Neurocytoma is a low grade neuronal tumor even rarely seen among central nervous system tumors. Nervous system tumors arising in teratomas are frequently originated from glial or primitive neuroectodermal cells. Here we report a neurocytoma arising in mature cystic teratoma in the right adrenal gland of an 8 years old boy. Histological examination revealed a tumor composed of solid sheets in fibrillar basis with small uniform cells in the neuroglial tissue in teratoma. This is only the second case demonstrating a neurocytoma arising in a mature cystic teratoma in the literature.     

Mediastinal teratoma in a newborn infant

A newborn with mediastinal teratoma developed tachypnea and cyanosis within the first hours of life. Chest x-ray revealed a subtotal opacification of the left hemithorax, which at first was suspected to be caused by a cardiomegaly. Ultrasound examination demonstrated a large mass in the left hemithorax with multiple echo-free areas. This finding was suspicious for a mediastinal teratoma. The tumor which was originating from the mediastinum was apple-sized and could be resected totally by thoracotomy. Histology revealed a cystic teratoma covered with normal thymus tissue.     

Extensive epidural teratoma in early infancy treated by multi-stage surgery

We report a rare case of extensive extradural teratoma successfully treated by multi-stage laminotomy and thoracotomy. A 34-day-old, dyspneic infant had a large posterior mediastinal mass identified on a chest X-ray radiograph. Imaging studies disclosed that the mass originated from the extradural space at the level of the lower thoracic spine, extending cephalad to C4 and caudad to L4 and severely compressing the spinal cord anteriorly, causing paraplegia. The tumor expanded bilaterally through the intraspinal foramina, coalescing to form a huge mediastinal mass. The upper half of the teratoma was removed utilizing a laminotomy from T3 through T9; 2 months later the lower half was excised via a laminotomy from T11 to L3. An additional procedure was required to resect recurrent tumor through a laminotomy from T8 to T12. The reconstructed vertebral arches were well-preserved in shape, with an almost normal spinal canal. Accepted: 27 January 1998     

婴幼儿椎管内肿瘤(附63例报告)

目的：分析和探讨婴幼儿椎管内肿瘤的临床特点,早期诊断和手术治疗,方法：对63例婴幼儿椎管内肿瘤的临床资料进行回顾性研究,结果：本组病例皮样囊肿和表皮样囊肿占41．3％,脂肪瘤12．7％,畸胎瘤7．9％,肠源性囊肿5．0％,神经母细胞瘤占4．8％,其他恶性肿瘤占4．8％,手术全切除率为76．7％,大部分切除率为18．3％,部分切除率为5．0％,肢体运动障碍者,术后随诊时78．9％,好转,感觉障碍64％改善,括约肌功能障碍63．3％,改善,全组无死亡。结论：婴幼儿椎管内肿瘤以先天性肿瘤为主,对其应早期诊断和早期手术治疗,大多能做到肿瘤全切除,术后症状缓解,能明显提高患儿生活质量。     

Intracardiac Teratoma in a Child Simulating an Atrioventricular Nodal Tumor

Intracardiac teratoma is an extremely rare pediatric neoplasm. We studied the case of a 6-year-old girl with a right intraventricular cardiac mass. The tumor consisted of clusters of monotonous round epithelial cells scattered in a dense fibrotic stroma and was thought to represent an atrioventricular nodal tumor. Three years later the tumor recurred, with multiple mature elements derived from all three germ layers, and was diagnosed as mature cystic teratoma. Still present, however, were multiple areas that were histologically similar to the earlier lesion. Immunostaining revealed strong positivity for insulin, glucagon, somatostatin, and chromogranin consistent with overgrown pancreatic islets of Langerhans within a mature teratoma.     

Teratoma Associated with Endodermal Sinus Tumor

A 3-1/2-year-old girl was diagnosed as having a mediastinal endodermal sinus tumor with pulmonary, bony, and hilar lymph node metastases. Following 7 months of treatment with chemotherapy, thoracic CT (computerized tomogram) scan showed the presence of a residual mass. Upon further surgical exploration the residual mass, which was found to be intrapericardial and arising from the ascending aorta, was resected. This second lesion was shown to be a benign cystic teratoma.

The combination of an intrapericardial teratoma and a mediastinal endodermal sinus tumor in a female child is probably unique. The possible etiology for the combination of these two pathologies is discussed.     

Superior mediastinal teratoma containing well-differentiated bowel

A previously fit and well 14-month-old-girl presented with a 2-month history of worsening cough and wheeze. Chest radiograph revealed a widened mediastinum and thoracic CT showed a large mixed density mass in the superior mediastinum, consistent with a mediastinal teratoma. Her tumor markers were within the normal range. The mass was resected and found to be a mature cystic teratoma. Surprisingly, well-formed bowel-like structures were present, containing all bowel wall layers and having a ganglionated myenteric plexus. The identification of complete sections of bowel in this context is a rare finding and to the best of our knowledge has not been published previously for the mediastinal teratoma. The possibility of secondary neoplasia developing in these areas is a complicating factor.     

Neonatal teratoma presenting as hygroma colli

We describe a neonate with a large tumour involving cranial, cervical and upper mediastinal regions, which presented clinically as hygroma colli. Radiological and pathological investigations showed characteristics of a mature teratoma and prominent cystic components within the tumour. These findings suggest that during early fetal development primary lymphatic sacs were obstructed by a teratoma leading to hygromatous dilatations of lymphatic vessels or that the abnormal proliferation of lymphatic vessels (hygroma) was part of the teratoma, developing from mesoderm as one of the three germinal layers from which teratomas originate. A third possibility is that the cystic part of the tumour originated from plexus chorioideus tissue, containing CSF. The last possibility is most probable in this patient.     

Mature cystic teratoma of the pancreas in a child

A cystic pancreatic tumour is rare in a child and a mature cystic teratoma of the pancreas is even rarer. This is the first demonstration of the CT appearance of such a tumour in a child. We present a 2-year-old boy who presented with a palpable abdominal mass. Abdominal CT revealed a huge cystic mass in the upper abdomen. Pathology disclosed a mature cystic teratoma originating from the pancreas.     

Teratoma in split cord malformation: an unusual association: a report of two cases with a review of the literature

Split cord malformations (SCM) may be discovered as isolated occult spinal dysraphism or in association with various other overt developmental spinal abnormalities or lesions. However, the occurrence of SCM with intraspinal teratoma is rare. We report 2 cases of teratoma associated with SCM, of which one is intramedullary and the other extradural within meningocele, with a brief review of the literature.     

Life-threatening mediastinal teratoma in a neonate

 This report describes a newborn with a large mediastinal teratoma (MT) presenting with severe respiratory distress (RD) at birth. At operation, there was no space for dissection because the huge cystic and solid tumor completely occupied the left hemithorax. After evacuation of the cystic component, the tumor was removed successfully. To our knowledge, only 16 newborn infants with MT presenting with RD have been reported. Operative morbidities occurred in one-half of the cases. We have reviewed the literature to discuss the potential risks of this entity. Accepted: 17 February 2000     

Metastatic endodermal sinus tumor: CT appearances

A one-week-old boy had undergone resection of a sacrococcygeal benign cystic teratoma. At the age of 12 months, he had a serum alpha-fetoprotein level of 139,000 IU/ml and a recurrent pelvic mass which was removed, and the microscopic examination revealed endodermal sinus tumor. Postoperatively, massively enlarged inguinal lymph nodes and abdominal distention developed. Computerized tomography displayed enlarged inguinal lymph nodes, metastatic lesions in the liver, and a pelvic recurrent mass. He received BEP (bleomycin, etoposide, cisplatin) chemotherapy regimen, and a complete remission was achieved with a normal serum alpha-fetoprotein. Close follow-up and serum alpha-fetoprotein monitoring are mandatory after the resection of a sacrococcygeal teratoma.     

Sacrococcygeal heart: a very rare differentiation in teratoma.

A mature teratoma was identified in a two-month-old girl who was operated for a sacrococcygeal mass. The cystic components of the mass were accidentally opened during surgery, and a solid, rudimentary organ resembling a heart emerged. It had a vascular pedicle and a pulsation like cardiac activity different from the infant's heart rate. The mass was totally excised together with the coccyx, and in histological examinations, it was diagnosed as a mature teratoma and a rudimentary heart. To the best of our knowledge, the case presented in this report is only the second case of a cardiac development in a teratoma in the literature. In the light of data obtained about this case and related literature, we consider that fetus-in-fetu and teratoma may not be irrelevant entities, and that they possibly have the same developmental malformation. We also suggest that such an intermediate case is a combination of fetus-in-fetu and teratoma.     

Fetal surgery: a brief review

Fetal therapy is a logical extension of fetal diagnosis. Fetal surgery has been performed in humans for the past 10 years. During this time, technical obstacles have been overcome and the natural history of many fetal disorders has been defined. A select group of disorders amenable to potential improvement by fetal treatment has been identified including fetal urinary tract obstruction, fetal diaphragmatic hernia, fetal congenital cystic adenomatoid malformation and fetal sacrococcygeal teratoma. The fetal surgical experience with each of these lesions is reviewed and the maternal risk of fetal surgery is discussed. Received: 14 August 1997 Accepted: 18 September 1997     

Cystic teratoma of the mesentery

Cystic teratoma is an unusual cause of mesenteric/omental cysts. A case of cystic teratoma of the mesentery in a 5-year-old girl is reported.     

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.     

Posterior mediastinal cystic teratoma surrounded by fat in a 13 month old boy

A 13 month old boy had a large left sided posterior cystic mass initially thought to be in lung. MRI showed the cystic mass to be surrounded by fat. A benign mediastinal teratoma was removed     

OLIGODENDROGLIOMA ARISING IN A MATURE CYSTIC OVARIAN TERATOMA IN A CHILD

In childhood mature cystic teratomas are the most common type of ovarian germ cell tumors. Tumors of neuroepithelial origin are extremely rare in mature teratomas. To the authors’ knowledge, this is the first case report oligodendroglioma arising in a mature cystic ovarian teratoma in children. A 13-year-old girl presented with a history of pelvic pain for 2 months. An ultrasound examination showed a monolateral multicystic, calcific ovarian mass, measuring approximately 6 × 5 cm, arising in the left over and adnexia, suggesting a teratoma. An exploratorative laparotomy and ooferectomy were performed. On histopatological examination, various samples from cystic areas had mature tissues from all 3 germ cell layers, including skin, bone, bronchial structures, and cerebellum. Sections from solid areas showed uniform population of monotonous, rounded cells with indistinct cytoplasm and having perinuclear halo (fried-egg appearance) on a fibrillary, neuropilic background. Microcalcifications as calcospherites were noted in the stroma. Based on the typical morphological features, the cased was diagnosed as mature cystic teratoma with low-grade oligodendroglioma. To the authors’ knowledge, there are a few reports in the literature of an oligodendroglioma arising in an ovarian teratoma in adults and this presented patient is the first case in childhood.     

Cervical teratoma: prenatal detection and management in the neonate

Routine prenatal ultrasonography in a 34-year-old woman at 28 weeks gestation, revealed a large right cervical mass in the fetus. Subsequent sonographies showed no increase in size of the mass, but polyhydramnios developed. Cesarean section was performed at 38 weeks. At birth a very right cervical mass was present which on palpation was partly cystic and partly solid. CT scan revealed heterogeneous tissue with numerous irregular calcifications. Because of increasing difficulty with breathing and swallowing the tumor was removed surgically. The microscopic findings were compatible with teratoma. The postoperative course was uneventful. Although malignancy has been described, mainly in adults, histologically cervical teratoma is a benign tumor. The importance of its recognition in utero is stressed, as this allows the planning of obstetrical and surgical strategy.