ASOPRSFall Scientific SymposiumSyllabus
157
T4
Malignant RhabdoidTumor of theOrbit
AlisonCallahan, Frederick Jakobiec, Grace Lee, Arthur Grove, Suzanne Freitag. Ophthalmology, HarvardMedical School, Boston,MA,
UnitedStates
Introduction:
Extrarenal rhabdoid tumors are rare neoplastic entities that have only twice previously been described in the
adult orbit.
Methods:
We describe a rhabdoid tumor of the adult orbit,which arosewithin an initially benign, but locally aggressivemyxoid tumor.
Results:
An 80 year oldwoman has now been followed for over a decade for a previously benign, but locally aggressivemyxoid
tumor of the left orbit that has required serial debulking procedures at 1-2 year intervals. In 2012, the tumor began behaving in a
more locally aggressivemanner, growingwith increasing rapidity (Figure 1A-1D). In addition to progressive limitation of extraocular
movement and severe (>35mm proptosis), new afferent visual dysfunction began at that time and rapidly progressed to no light
perception in the left eye. Despite an aggressive surgical debulking, the tumor re-amassedwithinmonths necessitating permanent
tarsorrhaphy.After lengthy consideration and discussions, the patient decided to proceedwith exenteration and planned prosthetic
reconstruction.Microscopically, the bulk of the exenterated specimen demonstrated an infiltrative, hypocellular,myxoid character
consistent with earlier specimens (Figure 1E). However, therewas additionally a focus of mitotically active large round tumor cells
with prominent central nucleoli and intensely eosinophilic cytoplasmwith round pseudoinclusions (Figure 1F).This area stained
positively for calponin andweakly positive for smoothmuscle actin, but wasmyogenin,myosin,myoglobin,muscle specific actin,
desmin, INI1, GFAP, S100 and Keratin 14 negative.The inclusionswere vimentin positive. Ki67 proliferation index increased in the
final three specimens from 7% to 17% to 20-25%.The new focus of malignant cellswere interpreted as rhabdoid. Subsequent
imaging revealedmetastases to the lymph nodes and lungs.
Conclusions:
Since their initial description in the kidneys in 1978
1
, rhabdoid tumors occurring in extrarenal locations have been
reported in the literature. Uncommon orbital occurrences aremore frequently reported in the pediatric population,
2,3
while its
occurrence in the adult orbit has only been described twice: in the lacrimal gland
4
and intraconal space.
5
We report a third incidence
of a rhabdoid tumor in the adult orbit which evolved over the course of greater than a decade.The escalating clinical behavior of this
locally aggressive orbital tumor was paralleled by increasing proliferative indices until the previously benignmyxoid tumor assumed
the phenotype and charactersitics of an orbital rhabdoid tumor.
References:
1. Beckwith JB, Palmer NF. Histopathology and
prognosis ofWilms tumor: results of the First NationalWilms’
Tumor Study. Cancer 1978;41:1937-48.
2. Rootman J, Damji KF, Dimmick JE.Malignant rhabdoid tumor
of the orbit. Ophthalmology. 1989Nov;96(11):1650-4.
3. Gündüz K, Shields JA, Eagle RC Jr, Shields CL, De Potter P,
Klombers L.Malignant rhabdoid tumor of the orbit.Arch
Ophthalmol. 1998 Feb;116(2):243-6. Review.
4. Niffenegger JH, Jakobiec FA, Shore JW,Albert DM.Adult
extrarenal rhabdoid tumor of the lacrimal gland.
Ophthalmology. 1992Apr;99(4):567-74.
5. Johnson LN, Sexton FM, Goldberg SH. Poorly differentiated
primary orbital sarcoma (presumedmalignant rhabdoid tumor).
Radiologic and histopathologic correlation.ArchOphthalmol.
1991Sep;109(9):1275-8.
DetailedProgram
—Thursday,October 16, 2014
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