Case report and review of the literature: rare fetus-in-fetu presenting as oropharyngeal epignathus

An epignathus is caused by a continuous spectrum of masses of the oral cavity or oropharynx ranging in its entity from mature teratoma to the exceedingly rare fetus-in-fetu. Due to its location, regardless of the entity, the occurrence of an epignathus is frequently associated with life threatening airway obstruction. Here we demonstrate a case of a fetus-in-fetu presenting as an epignatus. We describe its successful management and review the available literature. Early diagnosis and knowledge of the preoperative workup are essential to enable a multidisciplinary management. Once the airway is secured, surgical excision is the treatment of choice often resulting in a good clinical outcome and prognosis

Oropharyngeal congenital teratoma: a case report

Oropharyngeal teratoma is the most rare type of teratoma, with only 2% of fetal teratomas. The diagnosis must be established as early as possible, preferably during the prenatal period. The prognosis will depend on the size and location of the lesion, growth rate of the lesion, degree of intracranial spread, its resectability, and immediate care at birth by a multisciplinary team. We report aparticular case of congenital oropharyngeal teratoma (epignathus). The diagnosis was made during the prenatal period by ultrasound, and the fetus evolved to intrauterine death at the 29th week. The anatomopathological examination revealed a female fetus, compatible with 27-28 weeks, oropharyngeal teratoma and congenital malformations.O teratoma congênito de orofaringe é o tipo mais raro de teratoma, compreendendo apenas 2% desses tumores fetais. O diagnóstico deve ser realizado o mais precocemente possível, preferencialmente durante o pré-natal. O prognóstico irá depender do tamanho e localização da lesão, da velocidade de crescimento desta, do envolvimento de estruturas intracranianas e da ressecção adequada do tumor com equipe multidisciplinar. Relatamos o caso de uma paciente que teve diagnosticado durante a gestação feto com teratoma congênito de orofaringe (epignathus) por meio de ultra-sonografia. O feto evoluiu para óbito intra-uterino na 29ª semana de gestação, sendo então induzido o parto por via vaginal. O exame anatomopatológico revelou feto do sexo feminino, compatível com 27-28 semanas, teratoma orofaríngeo e outras malformações congênitas.Universidade Federal de São Paulo (UNIFESP) Departamento de GinecologiaUNIFESP, Depto. de GinecologiaSciEL

Epignato: teratoma orofaríngeo: comunicación de un caso

El epignathus es un teratoma orofaríngeo, que tiene una incidencia de 1:35000 a 1:200000 nacidos vivos. Los teratomas orofaríngeos surgen de la base del cráneo o de la pared posterior de la faringe y se extienden inferiormente causando obstrucción parcial o completa del tracto aéreo y digestivo por lo que es importante el diagnóstico prenatal para establecer un plan de manejo adecuado e inmediato y mejorar la supervivencia de los recién nacidos. Se reporta un caso de Epignathus en una paciente primigesta que cursaba un embarazo de 32 semanas y a la cual se le realizó el diagnóstico prenatalmente por ultrasonido anatómico y corroborado al nacimiento

Hamartomas, teratomas and teratocarcinosarcomas of the head and neck: Report of 3 new cases with clinico-pathologic correlation, cytogenetic analysis, and review of the literature

<p>Abstract</p> <p>Background</p> <p>Germ-cell tumors (GCT) are a histologically and biologically diverse group of neoplasms which primarily occur in the gonads but also develop at different extragonadal sites in the midline of the body. The head and neck region including the upper respiratory tract is a very rare location for such tumors in both children and adults, which can cause diagnostic and therapeutic difficulties.</p> <p>Methods</p> <p>We describe here two new cases of multilineage tumors including sinonasal teratocarcinosarcoma [SNTCS], and congenital oronasopharyngeal teratoma (epignathus) and compare their features with those of a new case of a rare salivary gland anlage tumor [SGAT], an entity for which the pathogenesis is unclear (i.e. hamartoma versus neoplasm). We correlate their presenting clinico-pathological features and compare histologic and cytogenetic features in an attempt to elucidate their pathogenesis and biologic potentials.</p> <p>Results and discussion</p> <p>Cytogenetic analysis revealed chromosomal abnormalities only in the case of SNTCS that showed trisomy 12 and 1p deletion. Both cytogenetic abnormalities are characteristically present in malignant germ cell tumors providing for the first time evidence that this rare tumor type indeed might represent a variant of a germ cell neoplasm. The SGAT and epignathus carried no such cytogenetic abnormalities, in keeping with their limited and benign biologic potential.</p> <p>Conclusion</p> <p>The comparison of these three cases should serve to emphasize the diversity of multilineage tumors (hamartomas and GCT) of the upper respiratory tract in regards to their biology, age of presentation and clinical outcomes. Malignant tumors of germ cell origins are more likely to affect adults with insidious symptom development, while benign tumors can nevertheless cause dramatic clinical symptoms which, under certain circumstances, can be fatal.</p

Prenatal ultrasound detection of congenital gingival granular cell tumor.

Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/135345/1/jum1991103185.pd

Antenatal Sonographic Diagnosis of Pharyngeal Teratoma: Our Experience of a Rare Case with Review of the Literature

Background. Teratomas are the most common tumors. They are usually localized in the sacrococcygeal area, while the pharyngeal localization is very rare. The number of cases of stomatopharyngeal teratomas detected prenatally via sonography is very small. Case Report. We present the case of a 24-year-old primipara at 18 weeks' gestation, that at the routine ultrasound scan, the fetus was found with an echogenic mass, filling the stomatopharyngeal cavity and protruding from the mouth. Other abnormalities were not found. Termination of pregnancy was achieved using misoprostol. A female stillborn fetus with a weight of 250 g and length of 25.5 cm was delivered. The postmortem and pathologic examination confirmed the diagnosis. Conclusion. Pharyngeal teratomas can be diagnosed with the use of ultrasounds in utero facilitating parents' counseling in early time

Malignant epignathus teratoma

A baby boy who had a left facial mass detected on antenatal ultrasound was delivered by Caesarian section after foetal distress was detected. Imaging investigations by plain radiographs and MRI showed a large mass with calcifications, soft tissue, fat and fluid components. A total surgical excision was perfomed and histology examination showed teratoma with no malignant features. Two weeks postoperatively, there was rapid recurrence of the tumour with intracranial involvement and obstructive hydrocephalus shown on MRI. The tumour was inoperable at surgery and the baby subsequently died at 5 weeks of life. This case describes the clinical course and imaging features of a neonatal epignathus teratoma with malignant and aggressive features

A Case of an Epignathus with Intracranial Extension Appearing as a Persistently Open Mouth at 16 Weeks and Subsequently Diagnosed at 20 Weeks of Gestation

ABSTRACT: We report a rare case of oral mass (epignathus) with intracranial extension originally suspected antenatally at 16 weeks&apos; gestation because of a persistent open mouth. Postmortem MRI and pathologic examination of the fetus confirmed an oral teratoma with bilateral ventricular dilatation, corpus callosum agenesis, and a neuroepithelial intracranial cyst. The relevant literature regarding this anomaly is reviewed

Human monstrosities: their origin and treatment

1. Human monstrosities are of great interest to the physician, surgeon, and obstretician. || 2. Until the 19th century, malformations were attributed to the influences of wrathful gods, devils, witches, sex perversions, the moon, eclipses, comets, and "maternal impressions". || 3. Scientific teratology only commenced in the 17th century. || 4. Connections between animals of different species have been proved conclusively to be sterile. || 5. Germinal life of embryo lasts about one week. || 6. Embryonic life lasts about five weeks. || 7. Foetal life lasts for the remainder of the ante-natal period. || 8. Normal development is a form of twinning - "symmetrical division". || 9. Uniovular twins originate from one ovum, and is not a normal physiological process. || 10. Double monsters also originate from one ovum, and are due to partial fission of embryonic axis. || 11. Binovular twinning is not true twinning. || 12. The armadillo has supplied valuable data regarding early mammalian development. || 13. The corpus luteum is a guide as to the number of ova involved in a pregnancy. || 14. There is a period of quiescence of about three weeks in early development of the armadillo. This may explain its polyembryony, and may also explain uniovular twinning in man. || 15. Twinning is due to a period of quiescence as a result of an upset in the growth regulating mechanism of the ovum. || 16. In epignathus we have complete fission with partial inclusion || 17. In foetus in foetu we have complete fission with total inclusion. || 18. Malformations can be produced experimentally in some animals by: (1) violent agitation, (2) variations in physiochemical conditions, (3) variations in temperature, and (4) disturbance of normal respiratory interchange. || 19. The principal causes of human malformations are: (1 )amniotic bands or adhesions, (2) faulty implantation of ovum due to endometritis, (3) developmental arrest at critical stases of gastrulation, and (4) heredity. || 20. There is a growth regulating mechanism in the ovum. || 21. This mechanism is controlled by two ovular secretions: "inhibitin" which inhibits growth, and "stimulin" which stimulates growth. || 22. Normal tissues contain a similar mechanism with similar secretions. || 23. In ante-natal life, inco-ordination between these two factors leads to mal- developments and. monsters, || 24. In post -natal life such into -ordination results in simple or malignant growths. || 25. "Inhibitin" may prove a powerful "de- cancerising" agent || 26. "Stimulin" may prove equally useful in most clinical conditions of a non -malignant nature requiring the speeding-up of tissue repair. || 27. Surgery has a distinct field of usefulness in certain cases of double monsters namely, omphalopagus parasiticus, xiphopagus, thoracopagus and craniopagus. || 28. Heredity is a potent factor in physical and mental peculiarities in the offspring. || 29. There is a constant tendency towards recovery and return to a healthy stock. || 30. Eugenics is " the study of the agencies under social control that may improve or impair the racial qualities of future generations, either physically or mentally". || 31. Mendel's laws of heredity have been found also applicable in man as regards mental and physical characters. || 32. Several methods for checking the supply of human defectives have been proposed e.g. segregation, sterilisation, restrictive marriage, laws, etc. || 33. Eugenics is one of the world's most pressing problems