Langerhans cell sarcoma with BRAF?V600E mutation and hemophagocytosis: An autopsy report

Langerhans cell sarcoma (LCS) is a rare high-grade neoplasm of langerhans cell phenotype having unambiguous malignant cytological features. We report such a rare case in a 20-year-old man who presented with dyspnea and high-grade fever. On evaluation, he had generalized lymphadenopathy, hepatosplenomegaly, and a large anterior mediastinal mass. Fine needle aspiration from the mediastinal mass and bone marrow aspirate showed numerous atypical cells, many of which showed grooved nuclei. In addition, the bone marrow showed prominent hemophagocytosis. The patient had a stormy hospital stay and succumbed to the illness. The autopsy revealed a rare multisystem involvement by LCS involving the lymph nodes, liver, spleen, lungs, and intestine, which harbored a BRAFV600E mutation and was associated with hemophagocytosis

A Patient with Sequential Diseases of Langerhans Cell Sarcoma, Langerhans Cell Histiocytosis, and Acute Lymphoblastic Leukemia / 罕见病研究

Langerhans cell histiocytosis(LCH)and Langerhans cell sarcoma(LCS)are characterized by clone proliferation of Langerhans-type cells, which may occur concurrently or sequentially with T-cell acute lymphoblastic leukemia (T-ALL) and other Lymphoid neoplasms. A 15-year old female patient diagnosed with T-ALL developed LCH involving multiple systems during maintenance chemotherapy of T-AL. After treated with chemotherapy with improved result, the patient showed progression of the illness and refractory to the second-line treatment. We found c.G35A (p.G12D)mutation in the KRAS gene and used the targeted drug Trametinib for treatment. The treatment proved effective, leading to partial remission within a week. Three months after Trametinib treatment, the patient developed new lymphadenopathy. Biopsy revealed the existence of LCS. The disease progressed quickly, and the patient died 7 days after diagnosis of LCS. The case of patients with T-ALL then developing LCH and LCS sequentially is extraordinarily rare. The causes of the case is unclear and may be related to cell transdifferentiation, clonal evolution, and chemotherapy. Targeted drugs can contain this disease for a short time.

Langerhans Cell Sarcoma of the Scalp with Concomitant Multiple Cervical Lymph Node and Adjacent Soft Tissue Involvement: A Case Report / 대한피부과학회지

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.

Langerhans Cell Sarcoma in Two Young Children: Imaging Findings on Initial Presentation and Recurrence

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.

Langerhans Cell Sarcoma Arising in a Lymph Node: A Case Report and Review of the Literature

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.