Choristoma
Choristoma is a benign, heterotopic mass of normal tissue located in an abnormal anatomical site. Unlike neoplasms, which are abnormal growths of tissue that proliferate in an uncontrolled manner, choristomas are composed of normal cells growing in locations where they are not typically found. The term is derived from the Greek words chorio, meaning "place" or "space", and stoma, meaning "mouth" or "opening", reflecting the concept of tissue that has developed in an incorrect location.
Etiology and Pathogenesis
The exact cause of choristoma formation is not well understood, but it is thought to result from developmental anomalies during embryogenesis. During this period, the misplacement or ectopic rest of normal cells may occur, leading to the development of choristoma. Genetic factors, environmental influences, and disruptions in normal developmental signaling pathways may contribute to this aberrant cell placement.
Classification
Choristomas can be classified based on the type of tissue present and their location. Common types include:
- Osseous Choristoma: Composed of bone tissue, often found in the tongue or the periocular region.
- Dermoid Choristoma: Contains skin and skin appendages, frequently located in the ovary, testes, or near the eye.
- Salivary Gland Choristoma: Consists of salivary gland tissue, typically occurring in the middle ear.
- Gastrointestinal Choristoma: Comprises gastrointestinal tissue, which can be found in various locations such as the esophagus, stomach, or Meckel's diverticulum.
Clinical Presentation
The clinical presentation of choristomas varies depending on their location and size. Many choristomas are asymptomatic and are discovered incidentally during imaging studies or surgery for unrelated conditions. When symptoms do occur, they are often related to the mass effect of the choristoma, such as obstruction, compression of adjacent structures, or cosmetic concerns.
Diagnosis
Diagnosis of choristoma typically involves a combination of clinical evaluation, imaging studies, and histopathological examination. Imaging techniques such as ultrasound, computed tomography (CT), or magnetic resonance imaging (MRI) can help delineate the size, location, and characteristics of the mass. However, definitive diagnosis usually requires biopsy or surgical excision and subsequent histological analysis to confirm the presence of normal tissue in an ectopic location.
Treatment
The treatment of choristoma depends on its size, location, and associated symptoms. Asymptomatic choristomas may not require immediate intervention and can be managed with observation. Symptomatic choristomas or those causing functional impairment or cosmetic concerns may necessitate surgical removal. The surgical approach and extent of resection depend on the choristoma's anatomical site and relationship to surrounding structures.
Prognosis
The prognosis for individuals with choristoma is generally excellent, as these lesions are benign and do not undergo malignant transformation. Surgical removal is usually curative, although recurrence may occur if the choristoma is not completely excised.
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Contributors: Prab R. Tumpati, MD