Extraskeletal myxoid chondrosarcoma (EMC) is aggressive in children. The condition in children differs to that in adults and to skeletal myxoid chondrosarcoma. We report on a 9-year-old girl with EMC in her left thigh. She underwent above-knee amputation. Five months later, a small mass was noted at the right lower lobe of the lung. The patient underwent one course of ifosfamide, carboplatin, and etoposide chemotherapy, followed by resection of the mass and 8 more courses of chemotherapy. At the 2-year follow-up, she was in remission radiologically.
Acral melanoma has been reported to have distinctive clinical presentation and ethnic distribution compared to other histological types of malignant melanoma. Acral melanoma also exhibits distinctive focused gene amplifications, including cyclin D1 overexpression. We reviewed archived histological material of malignant melanoma in the Sarawak General Hospital from year 2004 to 2010. 43 tumours, comprising 28 acral melanoma and 15 non-acral melanoma, had sufficient material to be included in the study. The majority (36%) of acral melanoma tumours occurred in the heel. The tumours were analyzed for cyclin D1 expression by immunohistochemistry. 68% of acral melanoma were cyclin D1 positive compared to a positivity of 33% in non-acral tumours. This difference was statistically significant (p < 0.05). This finding may improve the histological diagnosis of acral melanoma and detection of positive resection margins.
Lipoblastoma is extremely rare and mainly occurs in children younger than 3 years old. It is predominantly found in the extremities and trunk. Head and neck region occurrences are rare; only 4 such cases involving patients who presented with stridor have been previously reported. We report the fifth case of lipoblastoma of the neck with stridor in a 9-year-old girl, which had gradually worsened over the previous year. Imaging showed a retrotracheal mass extending superiorly to the thyroid level and inferiorly to below the carina of the trachea. Total resection of the tumor was performed, and the histopathologic findings were consistent with lipoblastoma. Postoperatively, the patient was well with no complications.
Extracranial schwannomas in the head and neck region are rare neoplasms. The tumours often present as asymptomatic, slowly enlarging lateral neck masses and determination of the nerve origin is not often made until the time of surgery. Preoperative diagnosis maybe aided by imaging studies such as magnetic resonance imaging or computed tomography, while open biopsy is no longer recommended. The accepted treatment for these tumors is surgical resection with preservation of the neural pathway. We report four cases of cervical schwannomas that we encountered at our center during four years of period. The clinical features, diagnosis and origin, management and pathological findings of these benign tumors are discussed.