Langerhans Cell Sarcoma Arising from Langerhans Cell Histiocytosis: A Case Report

Langerhans cell sarcoma (LCS) is a neoplastic proliferation of Langerhans cells that have overtly malignant cytologic features. It is a very rare disease and theoretically, it can present de novo or progress from an antecedent Langerhans cell histiocytosis (LCH). However, to our knowledge, LCS arising from an antecedent LCH has not been reported on. We present here a case of LCS arising from a pulmonary LCH. A 34 yr-old man who was a smoker, had a fever and a chronic cough. Computed tomographic (CT) scan revealed multiple tiny nodules in both lungs. The thoracoscopic lung biopsy revealed LCH. The patient quit smoking, but he received no other specific treatment. One year later, the follow up chest CT scan showed a 4 cm-sized mass in the left lower lobe of the lung. A lobectomy was then performed. Microscopic examination of the mass revealed an infiltrative proliferation of large cells that had malignant cytologic features. Immunohistochemical stains showed a strong reactivity for S-100 and CD68, and a focal reactivity for CD1a. We think this is the first case of LCS arising from LCH.

Primary Intraosseous Malignant Fibrous Histiocytoma of the Skull: A Case Report

Malignant fibrous histiocytoma(MFH) is a rare primary neoplasm that constitutes less than 1% of the malignant tumors of bone, and involvement of the skull is very rare. We present a case of malignant fibrous histiocytoma of the skull, presenting an intraosseous lesion in a 43-yr-old woman. She had a rapidly growing, tender mass in the right parietal region. A plain radiograph showed an osteolytic lesion of the right parietal bone. Magnetic resonance imaging revealed that the lesion showed heterogeneous low signal intensity on T1-weighted images and slightly high signal intensity on T2-weighted images. No evidence of an extraosseous extension to the adjacent dura and soft tissue was found, and a wide excision of the parietal bone was performed. Histologically, the tumor was a typical MFH displaying pleomorphic spindle cells in a storiform pattern. The results of immunohistochemical stainings revealed that the tumor cells were positive for vimentin, alpha-1-antitryp-sin, and p53, and negative for smooth muscle actin, S100 protein, desmin, and MyoD1. Three months later, a mainly cystic, recurrent mass was developed at the previously operated site. Before the resection, we first performed the percutaneous aspiration cytology, revealing diagnostic multinucleated pleomorphic cells. There-after, she had to receive repetitive resections of recurrent or residual lesions, and she died of postoperative meningoencephalitis two years after the first operation.