Pathologic quiz case 2. Melanotic neuroectodermal tumor of infancy (MNTI).
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Diagnosis, Differential↗
Biomedical subjects
M A Bergevin
Publications and source records attributed to M A Bergevin.
The internal carotid artery siphon in children and adolescents.
We observed significant lesions of the carotid artery siphon in two young subjects with fatal stroke. Because stroke in children and adolescents is uncommon and poorly understood, we examined the internal carotid artery in the 'siphon' of the skull in 24 unselected, but nearly consecutive autopsies. The age range was 10 days to 38 years, with 11 males and 13 females, six blacks, and 18 whites. In no case was stroke the cause of death. Intimal lesions of two types were found in the carotid siphon of all cases. (1) The first was focal splitting and/or duplication of the internal elastic lamina with variable proliferation of smooth muscle. The resulting 'fibrous' plaques or cushions, when severe, were usually found at natural bends in the artery. The number and severity of this type of lesion increased with age, but there were no differences in severity or distribution when compared by sex, race, or mode of death. (2) The second was internal elastic calcification which was found in all cases older than 9. This was increasingly severe with age. Although the frequency of the vascular lesions was surprisingly high, the relationship of either type to dissecting aneurysm or other stroke lesion remains unclear.
Adolescent↗
Congenital midline cervical cleft.
We present a case of congenital midline cervical cleft (CMCC), a sporadic lesion with a predilection for white females. It characteristically occurs in the midline ventral neck and consists of a cephalad skin tag, a mucosal surface, and a caudal sinus. There is an associated submucosal fibrous cord and interwoven bundles of skeletal muscle. This is occasionally tethered to the mandible and/or sternum causing neck contractures. CMCC is usually isolated, but rare cases have been associated with midline clefts of the tongue, lower lip, mandible, and sternum. The embryogenesis is unclear but arguments are given here that CMCC represents ectopic first branchial arch derivatives, including the lower lip and/or tongue.
Branchial Region↗
Myeloma and generalized mesenchymal amyloidosis causing malnutrition and cardiac failure.
A 39-year-old Negro woman had the mesenchymal type of generalized amyloidosis and myeloma of bone. Malnutrition and heart failure dominated the clinical course and led to her death. The autopsy study is summarized in the following diagnoses.
Adult↗
In situ fixation of the neonatal brain and spinal cord.
A delay in the autopsy can result in significant tissue autolysis, especially in the central nervous system. We have developed a rapid technique of in situ fixation that preserves central nervous system tissues until the formal autopsy can be performed. Through the lateral margin of the anterior fontanelle, Zamboni's solution is injected percutaneously into the lateral ventricles and allowed to exit via an intrathecal spinal needle. The choice of fixative allows a wide array of postmortem studies to be done.
Autopsy↗