CHORDOMATA: A REVIEW OF THE LITERATURE, WITH REPORT OF A SACROCOCCYGEAL CASE

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Abstract
Chordomata are tumors derived from misplaced notochordal tissue. Histologically they resemble notochordal tissue with a mucinous ground substance surrounded by, and containing, polygonal cells in lobulated chords, with many cells containing large amounts of mucin and occasional vacuolated nuclei. Malignancy increases with cellularity and relative decreased mucin. 48% of reported cases are located in the sacrococcygeal region, 36% in the cranial region, mainly around the sphenoid, the remainder around the vertebral column, with few exceptions. The symptoms are mainly those of a space-occupying lesion. Growth usually takes several yrs., depending on the degree of malignancy, with infrequent metastases, generally to adjacent lymph nodes and viscera. Treatment is practically confined to surgical excision. A case is reported with a 9 mo. history of cough and loss of wt., and for 3 mos. dull lumbar pain. Examination showed a mass in the right lower quarter with displacement of the ureter and colon to the left, and irregular densities in the lung. In spite of deep roentgen therapy, death occurred in 1 yr. Autopsy revealed a large firm lobulated retroperitoneal tumor attached to the sacrum, multiple metastases to the lung and one in the adrenal. The histology varied from compact masses of large pink cells with varying amts. of intra- and extracellular mucus, occasionally with intranuclear vacuoles and rare hemorrhages, to areas with an acellular matrix with cyst-like, spaces lined with vacuolated tumor cells.

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