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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
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Abstract

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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

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