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State of the Art Cranial Ultrasound Imaging in Neonates
Published on: February 2, 2015
Melanotic progonoma of the skull in infancy
Mehmet Kantar1, Murat Sezak, Tuncer Turhan
1Pediatric Oncology Unit, Department of Pediatrics, Ege University School of Medicine, 35100, Izmir, Turkey. mehmet.kantar@ege.edu.tr
Insights
Melanotic progonoma, a rare infant tumor, presents as a skull mass. Complete surgical removal offers a good prognosis with no long-term recurrence.
Area of Science:
- Pediatric Oncology
- Neurosurgery
- Dermatology
Background:
- Melanotic progonoma, also known as melanotic neuroectodermal tumor, is a rare infantile neoplasm.
- It presents as a potential differential diagnosis for both benign and malignant calvarial lesions in infants.
Observation:
- A case report details a 4-month-old infant with a left retroauricular subcutaneous mass fixed to the skull.
- CT and MRI revealed an expansile left occipitotemporal mass.
- Surgical excision resulted in a brownish-black tumor diagnosed as melanotic progonoma.
Findings:
- Histopathology and immunostaining are crucial for the definitive diagnosis of melanotic progonoma.
- Imaging modalities like CT and MRI aid in differentiating benign from malignant lesions but not in exact diagnosis.
- Complete surgical excision of cranial vault progonomas leads to favorable outcomes.
Implications:
- Early consideration of melanotic progonoma in the differential diagnosis of infantile cranial masses is essential.
- Prompt surgical intervention for cranial vault progonomas ensures a positive long-term prognosis.
- This case highlights the importance of multimodal diagnostic approaches and surgical management for rare pediatric tumors.
Introduction:
Melanotic progonoma or melanotic neuroectodermal tumor is a rare tumor in infancy. This lesion has to be considered in the differential diagnosis of benign or malignant lesions of calvarium.
Case Report:
The authors present a case of a 4-month-old infant with left retroauricular mass. The patient had a subcutaneous mass that is fixed to the underlying skull. CT and MRI scans showed left occipitotemporal expansile mass. The tumor was removed by surgery. A tumor, brownish-black in color, was diagnosed as melanotic progonoma. The patient remained symptom-free for the last 2 years after complete surgery.
Discussion:
Extracranial subcutaneous masses involving the skull are uncommon in infants. Benign or malignant lesions may occur as lumps on calvarium. Physical examination and some laboratory findings are helpful in the assessment of patient. Benign or malignant lesions can be differentiated by craniography, CT, or MRI scans, but exact diagnosis of melanotic progonoma is made by histopathology and immunostaining, as was in the presented case. Cranial vault progonomas have a better outcome by complete surgery. The tumors usually do not recur in long-term period.
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