Nasir Ud Din1, Khurram Minhas2, Muhammad Shahzad Shamim3, Naureen Mushtaq4, Zehra Fadoo5. 1. Department of Pathology and Laboratory Medicine, Aga Khan University Hospital Karachi, Karachi, Pakistan. nasir.uddin@aku.edu. 2. Department of Pathology and Laboratory Medicine, Aga Khan University Hospital Karachi, Karachi, Pakistan. khurram.minhas@aku.edu. 3. Department of Neurosurgery, Aga Khan University Hospital Karachi, Karachi, Pakistan. shahzad.shamim@aku.edu. 4. Department of Oncology, Aga Khan University Hospitali Karachi, Karachi, Pakistan. naureen.mushtaq@aku.edu. 5. Department of Oncology, Aga Khan University Hospitali Karachi, Karachi, Pakistan. zehra.fadoo@aku.edu.
Abstract
INTRODUCTION: Congenital infantile fibrosarcoma (CIFS) is a soft tissue sarcoma of infants mainly involving lower extremities usually presenting during the first year of life. A subset of cases occur in the head and neck, but scalp involvement is exceptionally rare. PATIENTS AND METHODS: We report clinicopathological features of three cases of CIFS involving the scalp diagnosed between 2011 and 2012. RESULTS: The ages of the three patients at the time of diagnosis ranged from 12 to 90 days (mean 48 days). All were males and presented with scalp swelling at birth which grew rapidly in size. The tumor was located in the left temporal region in two cases and the right temporoparietal region in one case. On imaging, underlying bone involvement was noted in two cases. The mean size of the resected tumors was 8 cm. All cases exhibited a cellular tumor arranged in sheets of uniform oval to spindle cells, increased mitosis, and hemangiopericytoma-like vessels. All patients are alive after a mean follow-up of 39.6 months. Recurrence was seen in one case due to incomplete excision. No metastasis was seen in any of the cases. CONCLUSION: CIFS of the scalp is rare and carries a good prognosis. Underlying bone erosion is rare but was noted seen in two of our cases. A male predominance was seen in our cases.
INTRODUCTION:Congenital infantile fibrosarcoma (CIFS) is a soft tissue sarcoma of infants mainly involving lower extremities usually presenting during the first year of life. A subset of cases occur in the head and neck, but scalp involvement is exceptionally rare. PATIENTS AND METHODS: We report clinicopathological features of three cases of CIFS involving the scalp diagnosed between 2011 and 2012. RESULTS: The ages of the three patients at the time of diagnosis ranged from 12 to 90 days (mean 48 days). All were males and presented with scalp swelling at birth which grew rapidly in size. The tumor was located in the left temporal region in two cases and the right temporoparietal region in one case. On imaging, underlying bone involvement was noted in two cases. The mean size of the resected tumors was 8 cm. All cases exhibited a cellular tumor arranged in sheets of uniform oval to spindle cells, increased mitosis, and hemangiopericytoma-like vessels. All patients are alive after a mean follow-up of 39.6 months. Recurrence was seen in one case due to incomplete excision. No metastasis was seen in any of the cases. CONCLUSION: CIFS of the scalp is rare and carries a good prognosis. Underlying bone erosion is rare but was noted seen in two of our cases. A male predominance was seen in our cases.
Authors: Adrián Mariño-Enríquez; Peining Li; Joan Samuelson; Michael R Rossi; Miguel Reyes-Múgica Journal: Hum Pathol Date: 2008-07-25 Impact factor: 3.466