ABSTRACT
Currently, the World Health Organization classifies Langerhans cell tumors into Langerhans cell histiocytosis and Langerhans cell sarcoma (LCS). LCS is a neoplastic proliferation of Langerhans cells showing malignant cytological features and aggressive clinical behavior with grave prognosis. Only a few cases have been reported in the available literature; therefore, to date, no definitive treatment has been established. A 64-year-old woman presented with a 1-year history of an asymptomatic, slow-growing erythematous nodule measuring 0.7 cm on her scalp. The patient also reported a 3-month history of a painful swelling on the right side of her neck. Histopathological examination of a scalp biopsy specimen revealed sheets of atypical cells with hyperchromatic nucleoli and clear cytoplasm. Immunohistochemical studies revealed malignant cells positive for CD1a, CD31, CD68, and S-100 expression. Additionally, positron emission tomography–computed tomography and fine-needle aspiration revealed LCS of the cervical lymph nodes and surrounding soft tissue. We recommended surgical excision and adjunctive chemotherapy; however, the patient refused treatment and died of the disease 28 months later.
Subject(s)
Female , Humans , Middle Aged , Biopsy , Biopsy, Fine-Needle , Cytoplasm , Drug Therapy , Electrons , Histiocytosis, Langerhans-Cell , Langerhans Cell Sarcoma , Langerhans Cells , Lymph Nodes , Neck , Parotid Gland , Prognosis , Scalp , World Health OrganizationABSTRACT
Langerhans cell sarcoma is a rare, proliferative tumor of Langerhans cells, which shows cytologic characteristics and clinical features of malignant tumor. Langerhans cell sarcoma primarily occurs in lymph nodes, skin, lung, liver, and spleen. However, very few cases have been reported in the head and neck region. Because of its rarity, an optimal treatment approach is unknown; however, Langerhans cell sarcoma grows aggressively and shows a poor prognosis, such that a more aggressive and multi-modality treatment approach is necessary. Here, we report the case of a 36-year-old male with Langerhans cell sarcoma, who presented with a mass in the submandibular gland area and was treated with wide excision and postoperative radiotherapy.
Subject(s)
Adult , Humans , Male , Head , Langerhans Cell Sarcoma , Langerhans Cells , Liver , Lung , Lymph Nodes , Neck , Prognosis , Radiotherapy , Sarcoma , Skin , Spleen , Submandibular GlandABSTRACT
Langerhans cell sarcoma (LCS) is a neoplastic proliferation of Langerhans cells with malignant cytological features and multi-organ involvement that typically has a poor prognosis. We experienced 2 cases of LCS in children less than 2 years of age and report them based primarily on CT and MR findings. Both children had findings of hepatosplenomegaly with low-attenuation nodular lesions, had multiple lymphadenopathy, and had shown recurrent lesions invading the skull during follow-up after chemotherapy.
Subject(s)
Female , Humans , Infant , Hepatomegaly/diagnosis , Langerhans Cell Sarcoma/diagnosis , Magnetic Resonance Imaging , Mediastinal Neoplasms/diagnosis , Neoplasm Recurrence, Local , Skull Neoplasms/diagnosis , Splenomegaly/diagnosis , Tomography, X-Ray ComputedABSTRACT
<p><b>OBJECTIVE</b>To explorer the clinical features, diagnosis and therapy of Langerhans cell sarcoma (LCS).</p><p><b>METHODS</b>The clinical data of a case of LCS originated from cervical lymph nodes was analyzed. The pathological biopsy was studied by cell morphology, immunohistochemistry and electron microscopy, and the related literature was reviewed.</p><p><b>RESULTS</b>The giant tumor cells were characterized by markedly malignant proliferation, irregular nuclei and obviously chromatin abnormality, the positive S-100, CD1a and Langerin (CD207) tumor cells were revealed by immunohistochemistry, and Birbeck granules could be found by electron microscopy. All of them supported the diagnosis of LCS. The patient's condition progressed rapidly and died of multiple organ failure in a short time.</p><p><b>CONCLUSION</b>LCS is an extremely rare neoplastic proliferation of Langerhans cells with overtly malignant cytologic features and spreads aggressively. The diagnosis of LCS mainly relies on pathological cell morphology, immunohistochemistry and electron microscopy if necessary. The treatment includes chemotherapy, surgery and radiotherapy, etc, but lack of generally accepted optimal treatment regimen currently. In short, LCS has intensive invasiveness and poor prognosis.</p>
Subject(s)
Aged , Female , Humans , Immunohistochemistry , Langerhans Cell Sarcoma , Diagnosis , Therapeutics , Langerhans CellsABSTRACT
We report a case of Langerhans cell sarcoma presented as a solitary mass in the left supraclavicular area in a 31-year-old woman. Computed tomography revealed a relatively well-defined and lightly enhancing mass in the left supraclavicular area, measuring 5.5x4.5x3.2 cm. Excision was subsequently performed. Microscopically, the specimen consisted of an enlarged and partially effaced lymph node. Nests of different size composed of atypical tumor cells were located in the paracortex and the medulla of the lymph node. The tumor cells exhibited abundant eosinophilic or clear cytoplasm and displayed marked nuclear atypia and increased mitotic figures. Infiltration of many eosinophils was identified in the periphery and between the tumor cells. The tumor cells were reactive for CD1a and S100 protein. Ultrastructually, they were found to have Birbeck granules in the cytoplasm.