We report a rare case of intrathoracic lymphangiomatosis associated with chylothorax in a 3-year-old boy. The patient had been healthy until he had chickenpox followed by continued dyspnea. The biopsy specimen showed proliferating lymphatic channels & spindle cells in the lung tissue. The boy died of respiratory failure despite conservative treatment and surgical treatment with pleurodesis. An autopsy was performed. Pleura and pericardium showed severe adhesion to the right lung parenchyma. The cut surface of lung showed thickened interlobular septum with honeycomb-appearance. Histologically, extensive intercommunicating and anastomosing endothelial-lined lymphatic channels were noted along the visceral and parietal pleura, pericardium, around the great vessels, and in the anterior mediastinum over the thymus. These lymphatic channels infiltrated into the pulmonary parenchyme along the bronchovascular bundles. There were scattered areas of spindle cell proliferation with extravasation of RBCs mimicking Kaposi's sarcoma. Histologic diagnosis and differential diagnosis on biopsy materials can be difficult to establish but awareness of the intrathoracic lymphangiomatosis and its various clinical presentation may be helpful for diagnosis.
Autopsy* ; Biopsy ; Cell Proliferation ; Chickenpox ; Child, Preschool ; Chylothorax ; Diagnosis ; Diagnosis, Differential ; Dyspnea ; Humans ; Lung ; Male ; Mediastinum ; Pericardium ; Pleura ; Pleurodesis ; Respiratory Insufficiency ; Sarcoma, Kaposi ; Thymus Gland

Cavernous angiomas are being increasingly well recognized throughout the central nervous system due to the widespread use of magnetic resonance imaging(MRI). However, these malformations are uncommon in the spinal column and rarely occur in the spinal cord. Here, we report a case of a cervical cord intramedullary cavernous angioma in a 49-year-old man. The patient had complained of left upper extremity paresthesia and weakness in the left hand for 5 days prior to admission. A neurological examination showed a left C-6 dermatome paresthesia and a weakness in the left hand grasping power. A MRI demonstrated a mixed signal intensity core at the C-5 level and a surrounding edema on the T-2 weighted image. Conservatively, a laminectomy was performed and slightly hard and well demarcated intramedullary mass was removed. A histological examination confirmed the diagnosis of a cavernous angioma.
Central Nervous System ; Diagnosis ; Edema ; Hand ; Hand Strength ; Hemangioma, Cavernous* ; Humans ; Laminectomy ; Magnetic Resonance Imaging ; Middle Aged ; Neurologic Examination ; Paresthesia ; Spinal Cord* ; Spine ; Upper Extremity

Neurofibromatosis type 1 is characterized by cutaneous neurofibromas and pigmented lesions of the skin called cafe au lait spots. Although neurofibromatosis type 1 represents a major risk factor for the development of malignancy, especially of nervous system tumors, malignant lymphoma rarely occurs in a patients with neurofibromatosis type 1. Recently, a 77-year-old woman with neurofibromatosis type 1 was diagnosed as non-Hodgkin's Lymphoma (diffuse large B cell). She had multiple cafe au lait spots, neurofibromas and right axillary lymph node enlargement. An abdominal CT scan demonstrated a left pelvic mass and para-aortic lymphadenopathy. Because non-Hodgkin's Lymphoma in a neurofibromatosis patient has never been reported in Korea, herein, we describe this case and include a review of the literature.
Aged ; Cafe-au-Lait Spots/complications/pathology ; Female ; Human ; Lung Neoplasms/*complications/pathology/radiography ; Lymphoma, Non-Hodgkin/*complications/pathology/radiography ; Neurofibromatosis 1/*complications/pathology/radiography ; Tomography, X-Ray Computed

The borderline tumor is not benign but has low malignant potential, which accounts for 10-15% of all ovarian tumors. The mucinous borderline tumors make up approximately 40% of all borderline tumors. About 5% of mature cystic teratomas of ovary include some with mucinous cystadenoma. The malignant transformation is occurred in about 1-3% of patients who have a mature cystic teratoma. There has been reported frequently about the malignant transformation of a mature cystic teratoma. After the first description of a patient with mucinous borderline tumor associated with a mature cystic teratoma in 1988, the borderline tumorous change of a mature cystic teratoma has been reported rarely. This report presents a patient with atypical proliferating mucinous tumor arising in a mature cystic teratoma.
Cystadenoma, Mucinous ; Female ; Humans ; Mucins ; Ovary ; Teratoma*