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Presented by William Westra, M.D. and prepared by Natasha Rekhtman, M.D., Ph.D.
Case 5: Newborn girl with a neck mass.
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Week 206: Case 5
Newborn girl with a neck massimages/12 6 04 case 5 1.JPG
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images/12 6 04 case 5 5.JPGCorrect
Answer: Benign teratoma
Histology: The histologic composition includes a haphazard and variable combination of cartilage, fat, glial tissue, choroid plexus, gastrointestinal epithelium, and other assorted tissue types. The tissues may appear mature (i.e. adult-like). The presence of immature elements is not appreciated.
Discussion: Teratoma is a neoplasm comprised of an admixture of tissue types reflecting contributions from all three germ cell layers. It presumably arises from pleuripotential cells that become displaced during embryogenesis. Less than 5% of teratomas involve head and neck sites. Of these head and neck teratomas, the cervical soft tissues and nasopharynx are the most commonly targeted sites. Most of these are present at birth, and most are detected in the prenatal period.
The histologic composition of teratomas is highly variable and includes somatic tissue derivatives from all three germ cell layers (i.e. ectodermal, endodermal and mesodermal). These tissues may appear mature (i.e. adult-like) or immature (i.e. embryonal-like). Importantly, for head and neck teratomas that involve the pediatric population, the presence of immature elements does not signify malignant transformation. Developmental errors resulting in an oral mass is not restricted to teratomas. Lingual thyroid, dermoid cysts, hairy polyps, and heterotopic glial tissue are also characterized by abnormally placed somatic tissues within the oral cavity and oropharynx. The microscopic picture is generally dominated by one or two tissue types, and the absence of derivatives from all three germ cell layers is critical in distinguishing these developmental inclusions from teratomas.
Teratomas in the pediatric population are uniformly benign, even those tumors that harbor immature elements. Although they have no malignant potential, morbidity may be high depending on the size and location of the mass. Death is usually related to airway obstruction. Prenatal diagnosis has vastly improved perinatal outcome by permitting a coordinated delivery with emphasis on airway management. Surgical resection is curative, and tumors do not recur when removal is complete.
On very rare occasions, a teratoma may arise in the head and neck region of an adult. Unlike teratomas of newborns and infants, these are biologically aggressive tumors that are usually malignant.
Incorrect
Answer: Benign teratoma
Histology: The histologic composition includes a haphazard and variable combination of cartilage, fat, glial tissue, choroid plexus, gastrointestinal epithelium, and other assorted tissue types. The tissues may appear mature (i.e. adult-like). The presence of immature elements is not appreciated.
Discussion: Teratoma is a neoplasm comprised of an admixture of tissue types reflecting contributions from all three germ cell layers. It presumably arises from pleuripotential cells that become displaced during embryogenesis. Less than 5% of teratomas involve head and neck sites. Of these head and neck teratomas, the cervical soft tissues and nasopharynx are the most commonly targeted sites. Most of these are present at birth, and most are detected in the prenatal period.
The histologic composition of teratomas is highly variable and includes somatic tissue derivatives from all three germ cell layers (i.e. ectodermal, endodermal and mesodermal). These tissues may appear mature (i.e. adult-like) or immature (i.e. embryonal-like). Importantly, for head and neck teratomas that involve the pediatric population, the presence of immature elements does not signify malignant transformation. Developmental errors resulting in an oral mass is not restricted to teratomas. Lingual thyroid, dermoid cysts, hairy polyps, and heterotopic glial tissue are also characterized by abnormally placed somatic tissues within the oral cavity and oropharynx. The microscopic picture is generally dominated by one or two tissue types, and the absence of derivatives from all three germ cell layers is critical in distinguishing these developmental inclusions from teratomas.
Teratomas in the pediatric population are uniformly benign, even those tumors that harbor immature elements. Although they have no malignant potential, morbidity may be high depending on the size and location of the mass. Death is usually related to airway obstruction. Prenatal diagnosis has vastly improved perinatal outcome by permitting a coordinated delivery with emphasis on airway management. Surgical resection is curative, and tumors do not recur when removal is complete.
On very rare occasions, a teratoma may arise in the head and neck region of an adult. Unlike teratomas of newborns and infants, these are biologically aggressive tumors that are usually malignant.
