tailieunhanh - báo cáo khoa học: "Kaposiform hemangioendothelioma in tonsil of a child associated with cervical lymphangioma: a rare case report"

Tuyển tập báo cáo các nghiên cứu khoa học quốc tế ngành y học dành cho các bạn tham khảo đề tài: Kaposiform hemangioendothelioma in tonsil of a child associated with cervical lymphangioma: a rare case report | Rekhi et al. World Journal of Surgical Oncology 2011 9 57 http content 9 1 57 7 2 WORLD JOURNAL OF SURGICAL ONCOLOGY CASE REPORT Open Access Kaposiform hemangioendothelioma in tonsil of a child associated with cervical lymphangioma a rare case report 1 1 2 1 Bharat Rekhi Shweta Sethi Suyash S Kulkarni and Nirmala A Jambhekar Abstract Kaposiform hemangioendothelioma KHE is an uncommon vascular tumor of intermediate malignant potential usually occurs in the extremities and retroperitoneum of infants and is characterized by its association with lymphangiomatosis and Kasabach-Merritt phenomenenon KMP in certain cases. It has rarely been observed in the head and neck region and at times can present without KMP. Herein we present an extremely uncommon case of KHE occurring in tonsil of a child associated with a neck swelling but unassociated with KMP. A 2-year-old male child referred to us with history of sore throat dyspnoea and right-sided neck swelling off and on since birth was clinicoradiologically diagnosed with recurrent tonsillitis including right sided peritonsillar abscess for which he underwent right-sided tonsillectomy elsewhere. Histopathological sections from the excised tonsillar mass were reviewed and showed a tumor composed of irregular infiltrating lobules of spindle cells arranged in kaposiform architecture with slit-like crescentic vessels. The cells displayed focal lumen formation containing red blood cells RBCs along with platelet thrombi and eosinophilic hyaline bodies. In addition there were discrete foci of several dilated lymphatic vessels containing lymph and lymphocytes. On immunohistochemistry IHC spindle cells were diffusely positive for CD34 focally for CD31 and smooth muscle actin SMA the latter marker was mostly expressed around the blood vessels. Immunostaining for HHV8 was negative and Ki-67 proliferation marker displayed focal positivity. Diagnosis of KHE was made. Platelet count was towards lower side of range. .