Orbital medulloepitheliomas—With extensive local invasion and metastasis

Orbital masses in children are uncommon but extremely challenging problems for clinicians and pathologists due to their critical location and availability of limited diagnostic material. We analyzed 47 specimens comprising biopsies, excision specimens, and FNAC of extraconal pediatric orbital masses (excluding retinoblastoma) accessioned in the pathology department over 5 years in a tertiary referral cancer center. Immunohistochemistry (IHC-74%) and molecular methods (one case) were done where necessary. The chief presenting symptom was proptosis in 55.3% patients and radiologically 53.8% malignant tumors showed extraorbital extension. A diagnostic algorithm was formulated to assess which cases needed pathology evaluation. Malignant round cell tumors (76.6%), chiefly embryonal rhabdomyosarcoma (51%), benign spindle cell neoplasms, and infectious lesions (tuberculosis, fungal infections), were seen. Of the malignant tumors, those confined to the orbit achieved good treatment response and had an event-free follow-up while those with extraorbital spread had poor outcome. Pediatric orbital masses range from completely treatable infectious lesions, surgically resectable benign neoplasms to aggressive malignancies requiring chemotherapy and radiotherapy. Pathologists play a key role in distinguishing these on small biopsy material and expediating accurate treatment thus saving the vision or life of a patient.

show abstract

“…One case had two local recurrences over a period of 2 years. This has already been reported by our group [23].…”

Section: Discussionsupporting

confidence: 87%

“…Medulloepitheliomas are rare intraconal tumors; however, they are known for their tendency to mimic carcinomas in their growth and spread [22,23]. If these are very large lesions they present as extraconal tumors and enter into differential diagnosis for these lesions.…”

Section: Discussionmentioning

confidence: 99%

Extraconal orbital tumors in children—a spectrum

et al. 2009

Self Cite

View full text Add to dashboard Cite

show abstract

“…47 This rare PNET, which was first described by Bailey and Cushing in 1926, may arise anywhere in the neuraxis, and even peripherally in the orbit 48 and pelvis. 47 This rare PNET, which was first described by Bailey and Cushing in 1926, may arise anywhere in the neuraxis, and even peripherally in the orbit 48 and pelvis.…”

Section: Medulloepithelioma Clinical Presentationmentioning

confidence: 97%

“…47 This rare PNET, which was first described by Bailey and Cushing in 1926, may arise anywhere in the neuraxis, and even peripherally in the orbit 48 and pelvis. 48 They tend to be well circumscribed and only slightly heterogeneous. 48 They tend to be well circumscribed and only slightly heterogeneous.…”

Section: Medulloepithelioma Clinical Presentationmentioning

confidence: 97%

Uncommon Pediatric Brain Tumors

Castellino

Schniederjan

MacDonald

2012

Textbook of Uncommon Cancer

View full text Add to dashboard Cite

“…Three documented cases of medulloepithelioma in adults have metastasized, which is a relatively high percentage when compared to the overall chance of metastasis for this tumor (table 1) [8,16]. True malignant behavior with metastasis has never been documented in tumors without extrascleral extension of the primary tumor or after invasive procedures [7,8,17,18]. A simple grading scheme of grade I tumors (current WHO benign designation), grade II tumors (tumor progression as evidenced with pleomorphism, increased mitotic activity and local invasion) and grade III tumors (tumor transformation to metastatic potential with extrascleral extension or metastasis) would be more reflective of the unifying hypothesis of a single classifying tumor entity liable to progression and transformation into a potentially metastasizing entity (fig.…”

Section: Tablementioning

confidence: 99%