OBJECTIVE AND IMPORTANCE: Giant-eels tumors (GCTs) are primary bone tumors
that involve long bones in 75 to 90% of patients. They seldom develop in th
e cranium and are very rare in patients older than 60 years of age. A GCT r
arely occurs with Paget's disease; when it does, however, it is most common
ly associated with the polyostotic Corm and tends to involve the craniofaci
al bones. Pagetic GCTs are less aggressive than GCTs that are not associate
d with Paget's disease.
CLINICAL PRESENTATION: We report the case of an 81-year-old woman with a pa
inless left parietal mass and asymptomatic monostotic parietal Paget's dise
ase.
INTERVENTION: Surgical resection was performed, and histological examinatio
n of the lesion demonstrated Paget's disease with a malignant CCT. An incid
ental, low-grade, small-cell lymphocytic lymphoma also was noted. The patie
nt experienced local recurrence of the malignant GCT and eventually died af
ter developing pulmonary metastases of the malignant GCT.
CONCLUSION: This case is the first reported example of a patient with a mal
ignant GCT of the cranium associated with monostotic Paget's disease. It pr
ovides evidence that not all pagetic GCTs in the cranium are benign, as has
been reported.