Metanephric dysplastic hematoma of the sacral region explained

Metanephric dysplastic hematoma of the sacral region (MDHSR) has been described by Cozzutto and Lazzaroni-Fossati in 1980,^[1] by Posalaki et al. in 1981 ^[2] and by Cozzutto et al. in 1982.^[3] Three additional cases were seen by Finegold.^[4]

Case studies

The case reported by Cozzutto and Lazzaroni-Fossati involved a premature male newborn with bilateral renal dysplasia and a sacrococcygeal mass featuring a histological picture of renal dysplasia. The case reported by Cozzutto et al. and those studied by Finegold featured changes of renal dysplasia including immature tubules surrounded by a collarette of cellular mesenchyme, glomeruloid figures, tubules and nests of cartilage in a background of adipose tissue and fibrous tissue where muscle fibres, nerve bundles, and calcospherites were also seen.

The two cases reported by Posalaki et al. showed blastema, glomeruli, and tubuli. In several cases the mass was removed during reparative surgery for meningocele or myelomeningocele.

Alston et al. described immature renal tissue in a lumbosacral subcutaneous lipoma with intradural extension in a 6-day-old female.^[5] Horestein et al. reported a lumbosacral nephrogenic rest including blastema (mature tubules and nephrons) in abundant stroma unassociated to spinal dysraphism.^[6] The case reported by Ibrahim et al. in a 2-year-old boy comprised adipose and fibrous tissue with neuroglial elements, striated muscle fibres and clusters of glomeruloid structures and immature tubules.^[7]

Sacrococcygeal teratoma with nephroblastoma was reported by Ward and Dehner and by Trebbi et al.^{[8] [9]} Cases of primary lumbosacral Wilms tumor were described by Abrahams et al. and by Govender et al.^{[10] [11]}

It is therefore of primary importance the distinction of MDHSR from sacrococcygeal teratoma and from primary lumbosacral Wilms tumor.

Notes and References

1. Cozzutto C, Lazzaroni-Fossati F (1980) Renal dysplasia with sacral-perineal location. J Urol 124:901-903
2. Posalaky Z, Drake RM, Mawk JR, Franciosi RA, Spencer BH, Athinarayan PR, Baker TA, Viring N (1982) Ectopic immmature renal tissue over the dorsum of lumbar and sacral area in two infants. Pediatrics 69:336-339
3. Cozzutto C, Stracca-Pansa V, Salano F (1983) Renal dysplasia of the sacral region: Metanephric dysplastic hematoma of the sacral region. Virchows Arch (Pathol Anat) 402:99-106
4. Finegold M (1986) Pathology of neoplasia in children and adolescents. Vol 18 in the Series Major Problems in Pathology. Philadelphia, W.B. Saunders Company, p 300
5. Alston SR, Fuller GN, Boyko OB, Goshin SA, Di Sciafani A (1989) Ectopic immature renal tissue in a lumbosacral lipoma: pathologic and radiologic findings. Pediatr Neuroscie 15:100-103
6. Horestein M, Manci E, Walker A, Dehner LP (2004) Lumbosacral ectopic nephrogenic rest unassociated with spinal dysraphism. Am J Surg Pathol 28:1389-1392
7. Ibrahim AE, Myles L, Lang DA, Ellison DW (1998) Case of the month: June 1998 - 2-year-old boy with lumbosacral mass. Brain Pathol 8:817-818
8. Ward SP, Dehner LP (1974) Sacrococcygeal teratoma with nephroblastoma (Wilms tumor). A variant of extragonadal teratoma in childhood. A histologic and ultrastructural study. Cancer 33:1355-1363
9. Tebbi K, Ragab AH, Ternberg JL, Vietti TJ (1974) An extrarenal Wilms tumor arising from a sacrococcygeal teratoma. Clin Pediatr 13:1009-1023
10. Abrahams JM, Powel BR, Duhaime A-C, Sutton LN, Schut L (1999) Extrarenal nephroblastic proliferation in spinal dysraphism. Pediatr Neurosurg 31:40-44
11. Govender D, Hadley GP, Nadvi SS, Donnellan RB (2000) Primary lumbosacral Wilms tumor with occult dysraphism. Virchows Arch 436:502-505