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Plexiform malignant peripheral nerve sheath tumor(MPNST) in infancy and childhood--a case report
1Department of Anatomical Pathological, Eulji Hospital, Inha University Hospital, Seoul, Korea.
Abstract:
We present a congenital plexiform cellular tumor with high mitotic activities arising in the right thigh of a 3-days-old infant. This subcutaneous tumor measured 6.5 x 4.5 cm in diameter with multinodular, whitish, elastic cut surface. Microscopically, the tumor was composed of fascicles of closely packed uniform spindle cells with frequent nuclear palisadings. The most remarkable finding was frequent mitoses(4-5/10 HPF). The tumor cells were strongly positive for S-100 protein, myelin basic protein and vimentin, and weakly positive for Leu 7. On electron microscopic examination, the spindle cells were found to be surrounded by continuous basal lamina and had interlocking long cytoplasmic processes. Although the prognosis of this tumor is difficult to predict, aggressive behavior such as recurrences may be suggested, but it is less likely to be metastatic. Further accumulation of similar unusual cases may be helpful in evaluation of its biologic behavior.
Insights
A rare congenital tumor in an infant
Area of Science:
- Pediatric Oncology
- Surgical Pathology
- Developmental Biology
Background:
- Congenital tumors are rare and present unique diagnostic challenges.
- Plexiform cellular tumors are uncommon neoplasms requiring careful histological evaluation.
Observation:
- A 3-day-old infant presented with a large subcutaneous tumor in the right thigh.
- Microscopic examination revealed a plexiform cellular tumor with high mitotic activity (4-5/10 HPF).
- Immunohistochemistry showed strong positivity for S-100 protein, myelin basic protein, and vimentin.
Findings:
- The tumor exhibited features of rapid proliferation and spindle cell morphology.
- Electron microscopy confirmed spindle cells with interlocking cytoplasmic processes and basal lamina.
- The tumor cells expressed markers associated with neural crest differentiation.
Implications:
- The high mitotic rate suggests a potential for aggressive behavior, including recurrence.
- Metastasis is considered less likely, but long-term follow-up is crucial.
- Further case studies are needed to fully understand the biologic behavior and prognosis of this rare tumor.
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