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Choristoma

Author: Dr David Veitch, Dermatology Registrar, Royal Infirmary of Edinburgh, Scotland. Chief Editor: Dr Amanda Oakley, Dermatologist, Hamilton, New Zealand, November 2015.


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What is choristoma?

Choristoma is a rare benign tumour consisting of microscopically normal tissue derived from germ cell layers foreign to that body site. Choristomas are classified according to their type of tissue. They sometimes arise within the skin or oral cavity, and can also occur in internal organs.

  • Dermoid cyst is made up of the components of epidermis and dermis, including hair.
  • Choristoma of the oral cavity may contain epidermal components (skin, hair follicles, sebaceous glands), osseous (bony), cartilaginous, lingual thyroid, gastric, respiratory, salivary or glial tissue.
  • Phakomatous choristoma of the eyelid or orbit contains embryological precursor cells of the lens (lenticular anlage).
  • Epibulbar or corneal choristoma arise at the limbus of the eye, and may include dermoid tissue, lipodermoid or other tissues.
  • Choristoma within the ear canal usually contains cartilage.
  • Salivary gland choristoma has been reported on the anterior chest wall and breast.

Who gets choristoma?

Choristoma may be diagnosed at any age from birth up until old age. Epidermal choristomas have all occurred in males. Most cases of lingual osseous choristoma have been reported in females in the third and fourth decade. Phakomatous choristoma and salivary gland choristoma tend to arise in newborns.

What causes choristoma?

Choristoma is a developmental abnormality. For example, a dermoid cyst arises from epithelium trapped along lines of embryonic fusion. The reason they present late in life in some cases is unknown. One theory is that it may be due to chronic irritation.

What are the clinical features of choristoma?

Epidermal choristoma on the dorsum of the tongue most often presents as a brown to black pigmented macule of variable size (3–11 mm). The differential diagnosis of pigmented macular choristoma on the tongue includes congenital melanotic macule and melanocytic naevus.

Osseous choristoma presents as a pedunculated, painless hard lump on the dorsum of the tongue, posterior to the foramen caecum. Oral choristoma can also occur on the gingiva or buccal mucosa.

Phakomatous choristoma has similar appearance but arises around the orbit or eyelid region. Clinical concern may be of a rhabdomyosarcoma.

A choristoma in the auditory canal presents as a mass causing hearing loss and may predispose to infection.

How is choristoma diagnosed?

Choristoma is diagnosed on biopsy or excision, by finding histological features confirming microsopically normal cells in an abnormal location.

What is the treatment of choristoma?

Choristoma is usually excised.

What is the outcome for patients with choristoma?

The lesions are benign with little risk of malignant transformation. Recurrence rates after excision are extremely low.

 

References

  • Lee FP. Cartilaginous choristoma of the bony external auditory canal: a study of 36 cases. Otolaryngol Head Neck Surg. 2005 Nov;133(5):786–90. PubMed
  • Kennedy CTC. The External Ear. In: Rook’s Textbook of Dermatology(Burns T, Breathnach S, Cox N, Griffiths C), 3rd edn. Wiley-Blackwell, 2010; 68.1-68.35
  • Scully C, Hegarty A. The Oral Cavity and Lips. In: Rook’s Textbook of Dermatology(Burns T, Breathnach S, Cox N, Griffiths C), 3rd edn. Wiley-Blackwell, 2010; 69.1-69.129
  • Moss C, Shahidullah H. Naevi and other Developmental Defects. In: Rook’s Textbook of Dermatology(Burns T, Breathnach S, Cox N, Griffiths C), 3rd edn. Wiley-Blackwell, 2010; 18.1-18.107
  • Curto-Barredo L, Vicente A, Rovira C García-Diez E, Pujol RM, González-Enseñat MA. Epidermal Choristoma of the Tongue Mimicking a Congenital Melanotic Macule. Pediatr Dermatol. 2015 Jul-Aug;32(4):536–8. PubMed
  • Batra R. The pathogenesis of oral choristomas. J Oral Maxillofac Surg Med Pathol 2012.;24:110–4.
  • Romano RC, McDonough P, Salomao DR, Fritchie KJ. Phakomatous choristoma in a 10-week-old boy: a case report and review of the literature. Pediatr Dermatol. 2015;32(3):405-9. PubMed
  • Aby JL, Patel M, Sundram U, Benjamin LT. Salivary gland choristoma (heterotopic salivary gland tissue) on the anterior chest wall of a newborn. Pediatr Dermatol. 2014 Jan-Feb;31(1):e36-7. doi: 10.1111/pde.12159. Epub 2013 May 16. PubMed PMID: 23679208.

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