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Ambiguous Genitalia and Differences of Sexual Development (DSD)
Published in S Paige Hertweck, Maggie L Dwiggins, Clinical Protocols in Pediatric and Adolescent Gynecology, 2022
Congenital causes/tumorsGonadal dysgenesis46,XX disorder of sexual development46,XY disorder of sexual developmentAdrenal enzyme deficiency (CAH)NeurofibromaChoristoma (aberrant or heterotopic tissue)
Congenital Tumors
Published in Asim Kurjak, CRC Handbook of Ultrasound in Obstetrics and Gynecology, 2019
The term “fetal tumor” should be used when it is evident that the tumor arises during the prenatal period.1 As in adults, fetal tumors may arise from any tissue and are consequently characterized by a variety of pathophysiological findings. One of the numerous classifications divided fetal tumors into choristoma, hamartoma, embryoma, teratoma and malignant neoplasm. The first four masses may primarily be caused by faulty histogenesis and organogenesis, i.e., dysontogenesis or dysembryoplasia. Thus, their pathogenesis may, at least in part, be similar to that of congenital malformations. Malignant tumors may arise from faulty cytogenesis of normally developed organs. Obviously, fetal tumors represent a rare and heterogeneous group of abnormalities (Table 1).
The Twentieth Century
Published in Arturo Castiglioni, A History of Medicine, 2019
In the field of cancer, D. hansemann’s (1872–1920) concept (1902) of “anaplasia” — that tumour cells may become more embryonic in appearance and grow more rapidly — was another important addition to the understanding of the nature of cancer. Many different varieties of tumours were described by investigators too numerous to mention here, all of which knowledge was assembled and evaluated in the masterly work of James ewing (1866–1943) on Neoplastic Diseases. The difficult borderline between anomalies and new growths was illuminated by E. albrecht’s concept of hamartoma and choristoma, structures having the microscopic appearance of tumours, yet differing from them in that they are congenital and of limited size rather than possessing powers of unlimited growth.
Orbital and periorbital dermoid cysts: a retrospective analysis of 270 lesions
Published in Orbit, 2022
Diana H. Kim, Daphna Landau Prat, Samuel Tadros, William R. Katowitz
The dermoid cyst is the most frequent cystic orbital lesion seen in the pediatric population,1 accounting for up to 46% of all childhood orbital tumors.2 It is a benign congenital malformation (choristoma) arising from embryonic ectoderm left between fetal lines of closure, most often in the frontozygomatic suture line. It can also present in the frontoethmoidal and frontomaxillary sutures. The dermoid cyst exhibits a slow growing behavior and becomes noticeable in the first few years of life. In the majority of patients, the lesion is superficial but a small portion may present with a “tip of the iceberg” effect, in which a deeper lesion with possible trans-osseous extension is present3; this is often referred to as a dumbbell cyst. Growth often involves CNS, orbital, or nasal extension. Provisional diagnosis is made clinically with or without imaging and confirmed by histopathology.4,5
Hepatic and Adrenocortical Choristomas in the Placenta
Published in Fetal and Pediatric Pathology, 2022
Different designations were used in previous reports, such as “ectopic liver” or “heterotopic liver” even though no bile ducts were present in those proliferations. Similarly, “adenoma” or “monodermal teratoma” were used despite the fact that there was no evidence of a mutation-based proliferation in terms of a neoplasia. In the umbilical cord, liver tissue with bile ducts hardly ever manifests as ectopic/heterotopic liver, independent of its association with omphalocele malformation [23–29]. Intra-placental yolk sac tumors can show hepatic differentiation but are characterized by proliferation of primitive glands and are uncommon in the placenta [30]. We therefore suggest the term “choristoma” for this type of non-placental tissue, rather than implying an organ formation or a neoplasia. Even nodules with large diameters are no evidence for neoplasia. Likewise, the designation of nodular capillary proliferations within the placenta as “hemangioma” or “chorangioma” implies a benign tumor, despite the lack of evidence for mutations in these vascular proliferations [31]. These vascular nodules most likely represent hamartomas derived from placental endothelial cells.
A giant dermoid cyst of the orbit
Published in Orbit, 2019
Bipasha Mukherjee, Akruti Desai
Orbital teratomas are unilateral germ cell tumors, which commonly present at birth with moderate to massive proptosis.1 There exists some differences in the terminology of developmental lesions. Dermoid cyst was initially classified as a mature cystic teratoma. But, it has now been termed as a choristoma, which arises from sequestered embryonic ectoderm trapped within bony suture lines during fetal closure. Dermoid cysts make up 3–9% of all orbital masses.2 Incidence varies from as low as 1.6% to as high as 46% in different studies.3 Clinical features depend upon the location and extent within the orbit. The purpose of this report is to describe a unique case of a giant orbital dermoid which presented in adulthood with marked proptosis and loss of vision. So far, there have been only six cases of giant orbital dermoids reported in the literature.