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- Hemangiopericytoma is a malignant vascular tumor arising from mesenchymal cells with pericytic differentiation. Hemangiopericytoma is most commonly seen in adults and only 5–10% of cases occur in children. The tumor is extremely rare in head and neck region (16%). Cytogenic abnormalities have been present in some hemangiopericytoma cases. Surgical resection remains the mainstay treatment. Adjuvant chemotherapy and radiotherapy is appropriate for in case of incomplete resections, life-threatening tumors particularly in children. Late relapses may occur and require long-term follow-up.
- A 4-year-old child patient with hemangiopericytoma of the maxilla, who presented with firm, recurrent but painless jaw mass. Radiographic investigations revealed a poorly circumscribed radiolucency. Biopsy of the lesion showed well circumscribed multiple lobules of tumor mass consisting of spindle shaped cells. The chemotherapy and radiotherapy of the lesion was done.
- The role of pediatric dental team is extensive in children with hemangiopericytoma for regular dental review. The patient’s oncologist should be immediately contacted if there is any suspicion of recurrence.
Hemangiopericytoma is a very rare slow growing vascular tumour with a variable malignant potential, constituting less than 1% of all neoplasm. The tumor is extremely rare in head and neck region.
The first description of this tumor goes back to stout and murray1 in 1942. The tumor takes its origin from pericapillary pericytic cells. On histopathological examination the tumor is a characterized by a proliferation of oval and spindle shaped pericytic cells. On first diagnosis it is generally difficult to distinguish between benign and malignant forms. This frequently becomes evident only during the further course of the disease. The features of malignant transformation are increased mitotic activity, a higher cell density, an appearance of undifferentiated cells as well as necrotic and hemorrhagic zones in the tumor tissue. Cytogenic abnormalities have been found in hemangiopericytoma. Hallen et al2 concluded that most HPC are near diploid, and break points in 12q13, 12 q 24 and 19q13 seem to be common, with recurrent t (12; 19), (q 13; q 13) translocation. Children with this lesion are likely to present to the pediatric dental department, as illustrated in the following case. The aim of this report is to describe the presentation, investigation, diagnosis and treatment of hemangiopericytoma in maxilla of a pediatric patient.
A 4 year old male child presented with a firm, diffuse swelling of the maxilla on left side that had been present for 3 months. The swelling was painless. This had not improved with antibiotics prescribed by a general dental practitioner.
Clinically, the boy was pale with non-palpable non-tender lymph nodes in the submandibular region. Extraoral examination showed a single diffuse swelling of 3 × 4 cm size on left side of the face involving the alveolar part of the maxilla. The margins of swelling were ill defined. There was obliteration of the nasolabial fold and left ala of nose was deviated. The philtrum was slightly deviated to the right side. The skin over swelling was normal and there was no surface pulsation.
Intraorally growth extends both labially and palatally in the region of upper left primary teeth. On the labial side, superiorly it extended upto labial sulcus and inferiorly 3 mm below to the occlusal line. There was slight displacement of upper left primary canine. The palatal aspect showed indentations of mandibular teeth on the swelling. (Fig 1). The lesion showed bleeding on provocation, consistency was firm and growth was sessile.
The provisional diagnosis of the neoplasm of anterior maxilla are following tumors: Fibrous Sarcoma, Hemangiopericytoma, Chondrosarcoma, Burkitts lymphoma, Malignant peripheral nerve sheath tumor, Thymomas, Osteosarcoma.
A provisional diagnosis of hemangiopericytoma was made and relevant hematological, radiological, biochemical investigations were undertaken confirm the above.
Occlusal radiograph revealed a poorly circumscribed radiolucency involving upper left primary and permanent teeth. Computed Tomography scan scan showed osteolytic lesion involving left maxillary alveolar ridge and soft tissue attentuation measuring upto to 1.2 × 1 cms. (Fig 2)
Under general anesthesia incisional biopsy of the lesion was performed using monopolar electrocautery. This technique has advantage in obtaining good haemotasis and avoidance of need of sutures.
Lesion showed well circumscribed multiple lobules of tumor mass consisting of tightly packed spindle shaped cells around ramifying walled endothelium lined vascular channels showing a stag horn configuration. (Fig 3).
Actin was showed positive expression with a diffuse pattern by the tumor cells. (Fig 4) Vimentin was also positive.
The various investigations showed that the final diagnosis of the lesion is hemangiopericytoma of the anterior maxilla.
The chemotherapy and radiotherapy of lesion was done. No perforation of the cortical plate was noted at the time of treatment. Currently the patient remains well and is under regular review.
Initially described by stout and murray1, hemangiopericytoma is a soft tissue tumor derived from mesenchymal cells with pericytic differentiation. It can either be benign or malignant.
Two subtypes have been described infantile hemangiopericytoma, infants under one year and adult hemangiopericytoma in children over one year and adults. It is a rare tumor of adult life (fifth decade) and pediatric cases account for approximately 3% of all soft tissue sarcomas in this age group. Most infantile hemangiopericytoma are considered congenital, they represent about one third of all pediatric hemangiopericytoma.3
The tumour may occur any where in the body, the most common anatomic locations being the lower extremities, the pelvis and the head and neck region4. Hemangiopericytoma are usually deep seated and are found in the muscle tissue, dermal and subcutaneous hemangiopericytoma are much less common. Clinical presentation of the hemangiopericytoma non-specific pain is a late symptom associated with enlarging mass.5
Hemangiopericytoma in pediatric ages is very rare and so only few publication reports or data about its clinical management are available. Surgical resection is the main treatment.6 The hemangiopericytoma of children is characterized by high response to chemotherapy when required in case of unrespectable, life threatening tumors7. Responses to Chemotherapy have been reported with vincristine, cyclophosphamide, deoxorubicin, dactinomycin, methotrexate, mitoxantrone and other alkylating agents. Considering the high chemoresponsiveness of the tumor as well as early age of patients less toxic chemotherapientic rejimens that limit use of authracylines and/or alkylating agent needed to investigated8. Radiotherapy is effective in some patients but indications are limited because of the age of the child patient.
Prognosis of hemangiopericytoma is usually favourable and depends on mitotic activity in tumour. The number of mitotic figures are variable and of prognostic significance. Espat et al 9 reported 93% and 86%, 2 and 5 year overall survival rates respectively. They also reported that incidence of metastases varies from approximately 10% to 60%, depending on the diagnostic criteria and the therapy. Local and distant relapses after prolonged disease-free interval have been reported by Sptiz et al10 suggesting a mandatory long-term follow up.
In hemangiopericytoma, child patient prognosis and long term survival can vary greatly from individual to individual. Prompt medical attention and aggressive therapy are important for the best prognosis. Continuous follow up care by pediatric dental team is essential for a child diagnosed with hemangiopericytoma. Side effects of radiation and chemotherapy as well as recurrence of the tumour, can occur in the survivors of hemangiopericytoma.
The authors wish to thank the staff and the patient whose unfailing help and support made the study possible.