Congenital thymoma.

Thymomas are a heterogeneous group of tumors arising from the epithelium of the thymus. They are categorized by the proportion of neoplastic epithelia to lymphocytes and by the degree of cytologic atypia. Thymomas constitute 0.2-1.5% of all malignancies and nearly all occur in patients over 20 years. We reviewed the available literature and found less than 50 cases of thymoma reported in children (<18 years of age), the youngest being 4 years old, and no cases in newborns. They represent less than 1% of all mediastinal tumors in children. Due to the limited number of cases in the pediatric population, the diagnosis and treatment in this population is extremely challenging. Thymomas in all age groups may be associated with paraneoplastic syndromes, being myasthenia gravis the most common, which is associated with a worse prognosis in the pediatric population. We present the first case of a newborn infant with congenital thymoma. This case demonstrates a rare tumor in an unusual age group and emphasizes the importance of multidisciplinary teamwork in the decision-making and management of this condition.

Congenital thymoma

Thymomas are a heterogeneous group of tumors arising from the epithelium of the thymus. They are categorized by the proportion of neoplastic epithelia to lymphocytes and by the degree of cytologic atypia. Thymomas constitute 0.2-1.5% of all malignancies and nearly all occur in patients over 20 years. We reviewed the available literature and found less than 50 cases of thymoma reported in children (<18 years of age), the youngest being 4 years old, and no cases in newborns. They represent less than 1% of all mediastinal tumors in children. Due to the limited number of cases in the pediatric population, the diagnosis and treatment in this population is extremely challenging. Thymomas in all age groups may be associated with paraneoplastic syndromes, being myasthenia gravis the most common, which is associated with a worse prognosis in the pediatric population. We present the first case of a newborn infant with congenital thymoma. This case demonstrates a rare tumor in an unusual age group and emphasizes the importance of multidisciplinary teamwork in the decision-making and management of this condition.

Hyalinizing clear cell carcinoma of the soft palate: a review of literature review.

Hyalinizing clear cell carcinoma (HCCC), also known as clear cell carcinoma, not otherwise specified [CCC, (NOS)], is a rare minor salivary gland tumor characterized by proliferation of clear cells, organized in trabecular cords, or solid nests within loose to densely hyalinized stroma. It is considered a diagnosis of exclusion by the World Health Organization (WHO) because other salivary tumors may also have a clear cell component. Hence, there is a wide differential diagnosis. EWSR1-ATF1 gene rearrangements are fairly specific for this tumor, however, one of the recent studies have described its presence in clear cell odontogenic carcinoma (CCOC) one of its histologic mimickers. EWSR1 and CREM fusions have recently been described in these tumors but its importance is still not well described. Here we present a case of a 33-year-old woman who presented with a recurrent lesion of the soft palate. Her initial lesion was resected and diagnosed as low-grade myoepithelial tumor. Surgical margins at the time of initial resection were positive and the re-excision was recommended but the patient did not undergo surgery. Two years later, local recurrence at the same site was found and an excision was performed yielding negative margins. Histopathologic examination revealed features consistent with hyalinizing clear cell carcinoma. The patient remains disease free 1 year after the re-excision. The pathology, clinical characteristics, differential diagnosis and treatment of hyalinizing clear cell carcinoma are reviewed.

Hyalinizing clear cell carcinoma of the soft palate: a review of literature review

Hyalinizing clear cell carcinoma (HCCC), also known as clear cell carcinoma, not otherwise specified [CCC, (NOS)], is a rare minor salivary gland tumor characterized by proliferation of clear cells, organized in trabecular cords, or solid nests within loose to densely hyalinized stroma. It is considered a diagnosis of exclusion by the World Health Organization (WHO) because other salivary tumors may also have a clear cell component. Hence, there is a wide differential diagnosis. EWSR1-ATF1 gene rearrangements are fairly specific for this tumor, however, one of the recent studies have described its presence in clear cell odontogenic carcinoma (CCOC) one of its histologic mimickers. EWSR1 and CREM fusions have recently been described in these tumors but its importance is still not well described. Here we present a case of a 33-year-old woman who presented with a recurrent lesion of the soft palate. Her initial lesion was resected and diagnosed as low-grade myoepithelial tumor. Surgical margins at the time of initial resection were positive and the re-excision was recommended but the patient did not undergo surgery. Two years later, local recurrence at the same site was found and an excision was performed yielding negative margins. Histopathologic examination revealed features consistent with hyalinizing clear cell carcinoma. The patient remains disease free 1 year after the re-excision. The pathology, clinical characteristics, differential diagnosis and treatment of hyalinizing clear cell carcinoma are reviewed.