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May 23, 1977

Hereditary Presacral Teratoma

Author Affiliations

St Luke's Hospital Kansas City, Mo

JAMA. 1977;237(21):2286-2287. doi:10.1001/jama.1977.03270480026005

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To the Editor.—  In the article by Drs Anderson and Burke (237:39, 1977) entitled "Anterior Sacral Meningocele," they identified sacral and coccygeal bony defects associated with the presentation of meningoceles in the pelvis communicating with the spinal subarachnoid space. I would like to call attention to a recent article in Radiology (122:187, 1977) in which we report a pattern of hereditary presacral teratoma occurring in 26 of 105 family members in six unrelated families.Of those 26 patients with a palpable presacral mass on rectal examination, 19 ultimately came to surgery, and there was pathologic examination of their tumors. As with the patients described by Anderson and Burke, most of our patients (22 of 26) had constipation. Thirteen of the 26 had anal stenosis, and 25 of 26 showed a typical hook-shaped or large central sacrococcygeal bony defect.One of our patients died of meningitis because the subarachnoid space was