Successful therapy in a child with a congenital peripheral medulloepithelioma and disruption of hindquarter development.
OBJECTIVE
Medulloepithelioma is an embryonal multipotential neuroepithelial tumor with a striking potential for divergent differentiation. It is usually intraocular or intracerebral and associated with a good prognosis only if completely surgically excised. Data regarding therapy in children with incompletely resected tumors are limited.


METHODS
A girl was born with a large, peripheral, congenital medulloepithelioma associated with complete absence of the left hindquarter and anus. Plain film, ultrasonography, and magnetic resonance imaging demonstrated complete absence of the left kidney and hemipelvis. A subtotal resection of the mass and reconstruction of the tumor-related anatomical defects were performed.


RESULTS
Pathologic examination showed neuroglia and pseudostratified neuroectoderm diagnostic of medulloepithelioma. She was treated with multiagent chemotherapy including vincristine, cisplatin, cyclophosphamide, carboplatin, and etoposide. She is now 50 months of age and developing normally without recurrent disease.


CONCLUSIONS
A child with an incompletely resected congenital peripheral medulloepithelioma who has experienced long-term disease-free survival after treatment with chemotherapy is described. This report supports a role for adjuvant chemotherapy in the treatment of children with peripheral medulloepithelioma.
Bruggers
C S
CS
Pediatrics Division of Hematology-Oncology, University of Utah School of Medicine, The Center for Children of the Huntsman Cancer Institute and Primary Children's Medical Center, Salt Lake City 84108, USA.
Welsh
C T
CT
Boyer
R S
RS
Byrne
J L
JL
Pysher
T J
TJ
D000008:Abdominal Neoplasms / Q000151:congenital* / Q000188:drug therapy / Q000601:surgery; D000015:Abnormalities, Multiple; D001003:Anal Canal / Q000002:abnormalities*; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D016190:Carboplatin / Q000008:administration & dosage; D017024:Chemotherapy, Adjuvant; D002945:Cisplatin / Q000008:administration & dosage; D003131:Combined Modality Therapy; D003520:Cyclophosphamide / Q000008:administration & dosage; D003964:Diaphragm / Q000002:abnormalities*; D005047:Etoposide / Q000008:administration & dosage; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D007668:Kidney / Q000002:abnormalities*; D007866:Leg / Q000002:abnormalities*; D008591:Meningomyelocele; D018302:Neoplasms, Neuroepithelial / Q000151:congenital* / Q000188:drug therapy / Q000601:surgery; D010386:Pelvic Neoplasms / Q000151:congenital* / Q000188:drug therapy / Q000601:surgery; D010388:Pelvis / Q000002:abnormalities*; D014750:Vincristine / Q000008:administration & dosage
D014750:Vincristine; D005047:Etoposide; D003520:Cyclophosphamide; D016190:Carboplatin; D002945:Cisplatin