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Hemangiopericytoma in children and infants
C Rodriguez-Galindo1, K Ramsey, J J Jenkins
1Department of Hematology-Oncology, St. Jude Children's Research Hospital, Memphis, Tennessee 38105-2794, USA.
Insights
Childhood hemangiopericytoma (HPC) differs by age. Infantile HPC shows benign behavior and chemoresponsiveness, unlike adult-like HPC in older children, requiring aggressive treatment.
Area of Science:
- Pediatric Oncology
- Soft Tissue Neoplasms
- Clinical Pathology
Background:
- Hemangiopericytoma (HPC) is a rare soft-tissue neoplasm, with 5-10% of cases occurring in children.
- Childhood HPC presents as two distinct clinical entities: infantile HPC with a benign course and HPC in children over 1 year behaving like adult HPC.
- The differing natural histories of infantile and older childhood HPC remain poorly understood.
Purpose of the Study:
- To investigate the clinicopathologic features, treatment, and outcomes of pediatric hemangiopericytoma.
- To elucidate the distinct clinical behaviors of infantile versus older childhood HPC.
- To inform therapeutic strategies based on age-related HPC characteristics.
Main Methods:
- Retrospective review of 12 pediatric HPC cases treated over 35 years.
- Analysis of clinicopathologic features, treatment modalities, and patient outcomes.
- Comparison of outcomes between infantile HPC and HPC in children over 1 year of age.
Main Results:
- Nine patients were over 1 year old; tumors often occurred in lower extremities. Three patients died of disease progression, with two showing responses to chemotherapy.
- Three infants (under 1 year) had unresectable tumors but responded well to neoadjuvant chemotherapy, with one case showing maturation to hemangioma.
- All three infants remain alive and disease-free, highlighting chemoresponsiveness and potential for regression.
Conclusions:
- Hemangiopericytoma in children over 1 year requires aggressive multimodality therapy, similar to adult HPC.
- Infantile hemangiopericytoma exhibits favorable clinical behavior, including chemoresponsiveness and spontaneous regression, necessitating a conservative surgical approach.
- Maturation into hemangioma is observed in some infantile HPC cases, correlating with its benign nature.
Background:
Hemangiopericytoma (HPC) is a soft-tissue neoplasm most commonly seen in adults; only 5-10% of cases occur in children. Childhood HPC comprises two distinct clinical entities. In children older than 1 year, it behaves in a manner similar to adult HPC. Infantile HPC, however, although histologically identical to adult HPC, has a more benign clinical course. The reasons for these differences in the natural history of HPC are not well understood.
Methods:
The authors reviewed the clinicopathologic features of HPC as well as the treatment and outcomes of the 12 children (9 males and 3 females) treated for this disease at St. Jude Children's Research Hospital over a 35-year period.
Results:
At diagnosis, 9 patients were older than 1 year and 3 were younger than 1 year. Among the 9 older patients, tumors were most commonly found in the lower extremities (n = 5). One patient had been treated for acute lymphoblastic leukemia 15 years earlier. One patient had metastatic disease at diagnosis, and three had unresectable tumors. Two patients experienced objective responses to chemotherapy. Three patients died of disease progression. Among the three infants, two had unresectable disease at diagnosis, and both experienced excellent responses to neoadjuvant chemotherapy. In one case, the response of the tumor to chemotherapy correlated with maturation to hemangioma. All three infants are alive without evidence of disease.
Conclusions:
HPC in children older than 1 year does not differ from adult HPC, and aggressive multimodality therapy is required. Infantile HPC, on the other hand, is characterized by better clinical behavior, with documented chemoresponsiveness and spontaneous regression, and requires a more conservative surgical approach. In some cases of infantile HPC, this benign behavior correlates with maturation to hemangioma.