Hostname: page-component-745bb68f8f-cphqk Total loading time: 0 Render date: 2025-01-24T06:34:10.508Z Has data issue: false hasContentIssue false
  • English
  • Français

Lipomatous Lumbar Mass with an Attached Digit and Associated Split Cord Malformation

Published online by Cambridge University Press:  02 December 2014

CMH Snelling ,
PM Ellis ,
RML Smith  and
JP Rossiter
CMH Snelling
Affiliation:
Department of Pathology and Molecular Medicine, Queen’s University and Kingston General Hospital, Kingston, Ontario, Canada
PM Ellis
Affiliation:
Department of Surgery, Division of Neurosurgery, Queen’s University and Kingston General Hospital, Kingston, Ontario, Canada
RML Smith
Affiliation:
Department of Radiology, Queen’s University and Kingston General Hospital, Kingston, Ontario, Canada
JP Rossiter
Affiliation:
Department of Pathology and Molecular Medicine, Queen’s University and Kingston General Hospital, Kingston, Ontario, Canada
Rights & Permissions [Opens in a new window]

Abstract

Core share and HTML view are not available for this content. However, as you have access to this content, a full PDF is available via the ‘Save PDF’ action button.
Background:

A male infant was born with a digit attached to a skin-covered lumbar lipomatous mass and an underlying split cord malformation.

Methods:

Surgical removal of the mass was performed at four months-of-age. By this time the digit had grown a nail and imaging and histology showed ongoing development of articulated phalanges.

Results:

The lipomatous mass contained a long bone, a clavicle-and scapula-like structure and a variety of other mature germ layer derivatives. These features raised a number of diagnostic considerations, including: mature teratoma, hamartoma, rudimentary parasitic twin, lipomyelomeningocele and dorsal accessory limb.

Conclusions:

Based on review of the literature, the authors hypothesize that there is a pathogenetically related spectrum of skin-covered dorsal mass lesions, often associated with spinal dysraphism. These consist of a major lipomatous component and a variety of mature germ layer derivatives that can vary widely in their degree of anatomical organization from case to case.

résumé:

<span class='bold'>RÉSUMÉ:</span> <span class='bold'> <span class='italic'>Contexte:</span></span>

À la naissance, on a constaté chez un bébé de sexe masculin la présence d’un doigt fixé à une masse lipomateuse recouverte de peau à la région lombaire et d’une malformation sous–jacente de la moelle épinière.

<span class='bold'> <span class='italic'>Méthodes:</span></span>

On a procédé à une exérèse chirurgicale de la masse lorsque le bébé était àgé de quatre mois. Un ongle avait poussé sur le doigt et des phalanges articulées en développement étaient visibles à l’imagerie ainsi qu’à l’histologie.

<span class='bold'> <span class='italic'>Résultats:</span></span>

La masse lipomateuse contenait un os long, des structures ressemblant à une clavicule et à une omoplate ainsi que d’autres structures matures, dérivées des feuillets embryonnaires. Plusieurs diagnostics ont été considérés : un tératome mature, un hamartome, un jumeau parasite rudimentaire, un lipomyéloméningocèle et un membre dorsal accessoire.

<span class='bold'> <span class='italic'>Conclusion:</span></span>

Après révision de la littérature, les auteurs ont émis l’hypothèse qu’il existe une gamme de masses dorsales recouvertes de peau qui sont reliées au point de vue pathogénique et souvent associées à une myélodysraphie. Elles sont constituées d’une composante lipomateuse importante et de structures matures variées, dérivées des feuillets embryonnaires, dont l’organisation anatomique varie considérablement d’un cas à l’autre.

Type
Case Report
Information
Canadian Journal of Neurological Sciences , Volume 35 , Issue 2 , May 2008 , pp. 250 - 254
Copyright
Copyright © The Canadian Journal of Neurological 2008

References

1. Walsh, JW, Markesbery, WR. Histological features of congenital lipomas of the lower spinal canal. J Neurosurg. 1980; 52(4): 564–9.CrossRefGoogle ScholarPubMed
2. Pierre-Kahn, A, Zerah, M, Renier, D, Cinalli, G, Sainte-Rose, C, Lellouch-Tubiana, A, et al. Congenital lumbosacral lipomas. Childs Nerv Syst. 1997; 13(6): 298–334.Google Scholar
3. Sutton, LN. Lipomyelomeningocele. Neurosurg Clin N Am. 1995; 6(2): 325–38.Google Scholar
4. Tibbs, PA, James, HE, Rorke, LB, Schut, L, Bruce, DA. Midline hamartomas masquerading as meningomyeloceles or teratomas in the newborn infant. J Pediatr. 1976; 89(6): 928–33.CrossRefGoogle ScholarPubMed
5. Morris, GF, Murphy, K, Rorke, LB, James, HE. Spinal hamartomas: a distinct clinical entity. J Neurosurg. 1998; 88(6): 954–7.CrossRefGoogle ScholarPubMed
6. Parizek, J, Nemecek, S, Pospisilova, B, Prochazkova, O, Nemeckova, J, Cernoch, Z, et al. Mature sacrococcygeal teratoma containing the lower half of a human body. Childs Nerv Syst. 1992; 8(2): 108–10.Google Scholar
7. Koen, JL, McLendon, RE, George, TM. Intradural spinal teratoma: evidence for a dysembryogenic origin. Report of four cases. J Neurosurg. 1998; 89(5): 844–51.Google Scholar
8. Hamada, H, Kurimoto, M, Hayashi, N, Hirashima, Y, Matsumura, N, Endo, S. Intramedullary spinal teratoma with spina bifida. Childs Nerv Syst. 2001; 17(1-2): 109–11.CrossRefGoogle ScholarPubMed
9. Vaishya, S, Pandey, P. Unusual spinal teratoma with an accessory penis on the back. Childs Nerv Syst. 2006; 22(4): 440–3.Google Scholar
10. Fulmer, BB, Wilkins, RH, Oakes, WJ, Filston, HC. Midline dorsal appendages. Pediatr Neurosurg. 1997; 27(5): 242–5.CrossRefGoogle ScholarPubMed
11. Parkinson, D. Accessory limbs and spinal dysraphism. J Neurosurg. 1991; 75(3): 498–9.Google Scholar
12. Humphreys, RP, Manwaring, KH, Carroll, NC. Accessory arm - dysraphism or disparity? Case report. J Neurosurg. 1991; 74(3): 297–300.Google Scholar
13. Krishna, A, Lal, P. Accessory limbs associated with spina bifida - a second look. Pediatr Surg Int. 1999; 15(3-4): 248–250.CrossRefGoogle ScholarPubMed
14. Drut, R, Garcia, C, Drut, RM. Poorly organized parasitic conjoined twins: report of four cases. Pediatr Pathol. 1992; 12(5): 691–700.Google Scholar
15. Rossiter, JP, Kydd, DW, Ellis, PM, Smith, R, Sauerbrei, E. Digit-bearing lumbar mass with spinal dysraphism. J Neuropathol ExperNeurol. 1998; 57(5): 484, Abstract 62.CrossRefGoogle Scholar
16. Pang, D, Dias, MS, Ahab-Barmada, M. Split cord malformation: part I: a unified theory of embryogenesis for double spinal cord malformations. Neurosurgery. 1992; 31(3): 451–80.Google Scholar
17. Bucy, PC, Haymond, HE. Lumbosacral teratoma associated with spina bifida occulta. Am J Pathol. 1932; 8: 339–46.Google ScholarPubMed
18. Muthukumar, N. Split cord malformation and cystic teratoma masquerading as lipomeningomyelocele. Childs Nerv Syst. 2003; 19(1): 46–9.CrossRefGoogle ScholarPubMed
19. Spencer, R. Parasitic conjoined twins: external, internal (fetuses in fetu and teratomas), and detached (acardiacs). Clin Anat. 2001; 14(6): 428–44.CrossRefGoogle ScholarPubMed
20. Ilkehan, H, Coban, YK, Guven, MA, Ceylaner, S. Three new cases of disorganizationlike syndrome: one with accessory extrophia vesicalis. J Pediatr Surg. 2004; 39(9): e6–e8.Google Scholar
21. Chapman, P, Stieg, PE, Magge, S, Barnes, P, Feany, M. Spinal lipoma controversy. Neurosurgery. 1999; 44(1): 186–92.Google Scholar
22. Lellouch-Tubiana, A, Zerah, M, Catala, M, Brousse, N, Kahn, AP. Congenital intraspinal lipomas: histological analysis of 234 cases and review of the literature. Pediatr Dev Pathol. 1999; 2(4): 346–52.CrossRefGoogle ScholarPubMed
23. Lemire, RJ, Beckwith, JB. Pathogenesis of congenital tumors and malformations of the sacrococcygeal region. Teratology. 1982; 25(2): 201–13.CrossRefGoogle ScholarPubMed
24. Catala, M Embryogenesis. Why do we need a new explanation for the emergence of spina bifida with lipoma? Childs Nerv Syst. 1997; 13(6): 336–40.CrossRefGoogle Scholar
25. Ersahin, Y, Mutluer, S, Kocaman, S, Demirtas, E. Split spinal cord malformations in children. J Neurosurg. 1998; 88(1): 57–65.CrossRefGoogle ScholarPubMed
26. Gamanagatti, S, Garg, R, Srivastava, DN, Gupta, A, Aggarwal, S. Spinal dysraphism with accessory thigh. Pediatr Radiol. 2003; 33(11): 809–10.CrossRefGoogle ScholarPubMed
27. Gardner, WJ. Hypothesis; overdistention of the neural tube may cause anomalies of non-neural organs. Teratology. 1980; 22(2): 229–38.CrossRefGoogle ScholarPubMed
28. Egar, MW. Accessory limb production by nerve-induced cell proliferation. Anat Rec. 1988; 221(1): 550–64.Google Scholar
29. Nye, HL, Cameron, JA, Chernoff, EA, Stocum, DL. Regeneration of the urodele limb: a review. Dev Dyn. 2003; 226(2): 280–94.CrossRefGoogle ScholarPubMed
30. Solanki, GA, Evans, J, Copp, A, Thompson, DN. Multiple coexistent dysraphic pathologies. Childs Nerv Syst. 2003; 19(5-6): 376–9.CrossRefGoogle ScholarPubMed
31. Sarnat, HB. Molecular genetic classification of central nervous system malformations. J Child Neurol. 2000; 15(10): 675–87.CrossRefGoogle ScholarPubMed
32. Vickaryous, MK, Hall, BK. Human cell type diversity, evolution, development, and classification with special reference to cells derived from the neural crest. Biol Rev Camb Philos Soc. 2006; 81(3): 425–55.CrossRefGoogle ScholarPubMed
33. Martinez-Frias, ML, Frias, JL, Opitz, JM. Errors of morphogenesis and developmental field theory. Am J Med Genet. 1998; 76(4): 291–6.Google Scholar