[Orbital tumor--hemangiopericytoma--in a neonate]. / Guz oczodolu--haemangiopericytoma--u noworodka.

PURPOSE: To present a case of Haemangiopericytoma (HPC), a rare neopalsm which originates from the vascular pericytes. HPC occurs most commonly in adults. Only 5-10% of cases occur in children. Congenital orbital HPC is generally unknown in the field of ophthalmology. MATERIAL AND METHODS: A case of congenital, large exophthalmus is reported in a 1 day old male neonate. Imaging studies demonstrated a vascular, orbital mass involving skull base and cranial fossa. RESULTS: The diagnosis of HPC was established after histological exmination. Lesion did not qualify to surgical resection. The child was treated with chemotherapy for 10 months and a great regression of tumor was noted. There was no tumor recurrence during 4 years of a follow up. CONCLUSIONS: Chemotherapy may have a significant role in the treatment of infants with nonoperative malignant hemangiopericytoma.

Proton beam therapy for loco-regional control of a recurrent mixed malignant germ cell tumor of the skull in a 22-month-old girl.

BACKGROUND: Germ cell tumors (GCT) situated in the head and neck region are very rare and occur predominantely in newborns or young infants. Recurrent CTs are often resectable only by mutilating surgery and the need for alternative treatment strategies is obvious. In this situation radiation therapy is the most important treatment option for loco-regional tumor control, but bear in this area the risk of possible impairment of brain function and face deformation as long term effects. CASE REPORT: In a girl with a connatal expansive growing teratoma of the skull the tumor recurred in spite of repeated surgery as mixed malignant GCT at the age of 15 months. Tumor control could not be achieved with chemotherapy and additional surgery seemed not promising. Therefore high dose proton beam therapy (PT) (54 Gy) has been administered to the child at the age of 22 months and led to local tumor control with only mild side effects. CONCLUSION: PT treatment may be an option for specific clinical conditions in germ cell tumors where local tumor control cannot be achieved by chemotherapy and/or surgery and long lasting side effects of conventional radiotherapy due to tumor localization and age have to be considered. However, PT should be implemented in treatment protocols for specific situations to guarantee supervised application, central documentation and follow-up.

A congenital teratoma with a cleft palate: report of a case.

Teratomas are benign tumors containing cells from ectodermal, mesodermal and endodermal layers. They occur in about 1 in every 4000 births and most commonly in the sacrococcygeal region, followed by the ovaries. Congenital epignathus teratomas are rare embryological neoplasms localised in the region of head and neck. An epignathus is found in approximately 1:35,000 to 1:200,000 live births. This accounts for 2-9% of all teratomas. Size and location of the neoplasm in the oronasopharynx is variable. Teratomas are partly undiagnosed at the time of birth. They may exist with an intracranial extension or as small polyps. Large epignathi can lead to difficult management during and after birth. The case of a newborn girl with a combination of an epignathus and a cleft palate is described. The epignathus presented as a huge mass extending out of the mouth of the infant girl. On the day of birth debulking of the extraoral portion of the tumor, followed by intraoral exstirpation, was performed. The results of the histologic examination indicated a congenital epignathus. Six months later a recurrence was found.

Basicranial diplomyelia: an extension of the split cord malformation theory. Case report.

Basicranial diastematomyelia is an extremely rare congenital disorder. A review of the literature indicates only one reported case of basicranial diastematomyelia in which an osseous peg divided the brainstem in two. The authors present the first reported case of basicranial diplomyelia split by a fibrous band and correlate its pathogenesis with that of split cord malformation (SCM). The patient described in the present report had a fibrous stalk dividing the brainstem, and therefore the condition was categorized as a diplomyelia, or SCM Type II. Because the occipital dermatomes behave similarly to the spinal dermatomes early in development, they may be subject to the same embryonic error that results in SCM. The authors propose that the mechanism leading to SCM is the same as that found in basicranial split malformations and that the theory explaining it be modified to include the posterior fossa.

Teratomas of the head and neck region.

BACKGROUND: Teratomas are exceptionally rare malformations in the head and neck region. They are mostly benign but as a direct result of their rarity, most clinician's experience of these tumours is very limited, and consequently most of the associated literature consists of single case reports. In this paper, however, all the cases managed by a major Craniofacial Unit (the Australian Craniofacial Unit) were reviewed to attempt to identify common problems encountered in their management. MATERIAL AND METHODS: All cases managed by the Australian Craniofacial Unit over the last 25 years were reviewed. In total a series of nine cases was identified, but two were seen and operated on in overseas centres and the data in these cases were incomplete, and have been excluded from the study. Case note, radiology and pathology review was undertaken to collect data. RESULTS: In total a series of seven cases was identified as suitable for inclusion in this study. Six of these have had a minimum of 9 years follow-up, three having completed growth. CONCLUSION: The initial and subsequent management demonstrates that these tumours when benign can be successfully removed, but depending on the affected site may require continued multidisciplinary management until growth has finished.

[Congenital teratoma of the skull base: case report of an interdisciplinary treatment]. / Teratom der Schädelbasis bei einem Neugeborenen: Verlaufsbeschreibung einer interdisziplinären Behandlung.

Teratomas of the oropharynx are quite rare congenital tumors. Even more rare than oropharyngeal teratomas are those with additional intracranial extension. Reviewing the literature the prognosis of these cases has been poor. In the majority stillbirth or immediate postpartum death following respiratory obstruction is reported. We present a case of a congenital teratoma of the oral cavity with intracranial extension in a female neonate. After several intraoral tumor resections to establish secure airway in the postpartum period the total tumor resection had to be performed at the age of 6 month via a lateral transmandibular approach to the skull base. By using extraoral distaction devices the operation related microsomia of the mandible could be corrected at the age of 5 years. At 7 years follow-up the girl presented free of recurrence and without any neurological or functional deficits.

Role of radical surgery for intracranial melanotic neuroectodermal tumor of infancy: case report.

OBJECTIVE AND IMPORTANCE: Melanotic neuroectodermal tumor of infancy (MNTI) is a rare, locally aggressive tumor that arises most commonly from the maxilla or mandible. Infrequently, it originates from the cranial vault, and recent reports have described a favorable outcome after radical surgery. Some lesions are particularly problematic, such as those located along the cranial midline or cranial base and those with significant intracranial extension. Currently, there is no effective adjuvant therapy for MNTI; radiation is precluded by the patients' young age, and chemotherapy trials have not demonstrated long-term efficacy. CLINICAL PRESENTATION: A 2-month-old infant boy presented with a firm, immobile subcutaneous mass behind the right ear. The mass had been present at birth and enlarged with time. INTERVENTION: Initial resective surgery down to the dura resulted in massive tumor recurrence within weeks. Successful management required repeat surgery including excision of the dura and dural venous sinuses. CONCLUSION: This patient's large MNTI of the cranial base was successfully managed by radical surgery. Although MNTI is a rapidly growing tumor that is locally highly invasive, radical surgery may be associated with a favorable outcome and offers the potential for long-term cure.

An unusual congenital nasopharyngeal teratoma.

A case of congenital nasopharyngeal teratoma with invasion of the skull base and right temporal region is reported.

Intracranial nasal dermoid sinus cyst associated with colloid cyst of the third ventricle. Case report and new concepts.

A case of a 16-year-old male with both a nasal dermoid sinus cyst (NDSC) and a third ventricle colloid cyst is presented. The NDSC was excised via a single-stage combined intracranial-extracranial approach and the third ventricle colloid cyst was resected endoscopically. The pathogenetic theories of NDSC and third ventricle colloid cyst are discussed, and an embryological explanation for the simultaneous development of the two lesions in this patient is explored. This case is best classified among congenital developmental malformations in a category we propose to call 'anterior neuropore corridor defects.'