This report describes a case of adult temporal bone Langerhans cell histiocytosis（LCH） that presented as posterior canal dehiscence syndrome（PCDS）. The patient initially presented with vertigo, tinnitus, and hearing loss. Computed tomography（CT） revealed erosive changes in the mastoid and posterior semicircular canal. After the operation, the patient's hearing and vestibular symptoms disappeared and postoperative recovery was favorable. Adult LCH is relatively rare, and this case serves as an example to discuss the clinical features and treatment options of this disease, followed by a literature review.
Humans ; Histiocytosis, Langerhans-Cell/complications* ; Semicircular Canal Dehiscence/etiology* ; Temporal Bone/pathology*

Objective:To analyze the clinical characteristics of malignant lymphoma of the head and neck in children, and to improve the understanding and diagnosis and treatment of this type of disease by otolaryngologists. Methods:Clinical data of 49 children with malignant lymphoma of the head and neck hospitalized in the Department of Otorhinolaryngology, Head and Neck Surgery of Kunming Children's Hospital from 2013-2021 were retrospectively analyzed and statistically analyzed according to gender, age distribution, duration of the disease, site of onset, type of pathology and survival status. Results:A total of 49 cases of malignant lymphoma of the head and neck in children were collected, of which, 39 were male and 10 female. The minimum age was 3 years, the maximum was 14 years and 4 months, the median age of onset was 7 years, and the largest percentage （51.02%） of children was in the school age（6-12 years）. The duration of the disease ranged from 5 days to 2 years, with a median of 1 month, and the site of the lesion was located in the neck in the majority of cases, 41（83.67%）. The pathologic types of hodgkin lymphoma（HL） were 25 cases（51.02%） and non-hodgkin lymphoma（NHL） were 24 cases（48.98%）, and among hodgkin lymphomas, mixed-cell classical hodgkin lymphoma was the most common, with 9 cases（18.37%）; among non-hodgkin lymphomas,originated from B-cells in 16 cases （32.65%） and from T-cells in 7 cases （14.29%）, with Burkitt's lymphoma being the most numerous of B-cell origin in 13 cases （26.53%）, and T-cell lymphoblastoid lymphoma being the most common of T-cell origin in 4 cases （8.16%）. The follow-up period was from 22 days to 6 years and 10 months, with 3 cases losing, 43 cases surviving, 3 cases dying, with a survival rate of 93 5%, and 3 cases relapsing, with a relapse rate of 6 5%. Conclusion:Children's head and neck malignant lymphoma has its own characteristics in terms of age of onset, gender distribution, site of onset, pathological type and treatment prognosis, which should be comprehensively assessed, diagnosed as early as possible, and multidisciplinary joint treatment actively carried out to propose personalized treatment plans.
Humans ; Male ; Female ; Child ; Head and Neck Neoplasms/therapy* ; Child, Preschool ; Retrospective Studies ; Adolescent ; Lymphoma/therapy* ; Survival Rate ; Hodgkin Disease ; Prognosis ; Infant ; Lymphoma, Non-Hodgkin

(+)-Strebloside, a significant bioactive compound isolated from the roots of Streblus asper Lour., demonstrates inhibitory effects against multiple malignancies. However, its specific function and underlying mechanistic pathways in Non-Hodgkin lymphoma (NHL) remain unexplored. This investigation sought to elucidate the role and potential mechanisms of (+)-strebloside-induced NHL cell death. The results demonstrated that (+)-strebloside significantly induced apoptosis and ferroptosis in NHL cells, including those from Raji cell-derived xenograft models. Mechanistic analyses revealed that (+)-strebloside enhanced six-transmembrane epithelial antigen of prostate 3 (STEAP3)-induced ferroptosis in NHL, and STEAP3 inhibition reduced the proliferation-inhibitory effects of (+)-strebloside. Furthermore, (+)-strebloside suppressed NHL proliferation through the mitogen-activated protein kinase (MAPK) pathway, and extracellular signal-regulated kinase (ERK) inhibition diminished the proliferation-inhibitory activity induced by (+)-strebloside. These findings indicate that (+)-strebloside presents promising therapeutic potential for NHL treatment.
Humans ; Ferroptosis/drug effects* ; Lymphoma, Non-Hodgkin/physiopathology* ; Cell Line, Tumor ; MAP Kinase Signaling System/drug effects* ; Animals ; Cell Proliferation/drug effects* ; Mice ; Apoptosis/drug effects* ; Membrane Proteins/genetics* ; Xenograft Model Antitumor Assays ; Male ; Mice, Nude

Primary female genital system lymphoma(PFGSL)is a rare subtype of extranodal lymphoma and patients commonly present in the department of gynecology.At present,there is a lack of uniform standards for the treatment of PFGSL.Although the classification of lymphoid neoplasmas was updated by the World Health Organization classification of haematolymphoid tumors in 2016,PFGSL was still not elaborated in sufficient detail.Most cases of PFGSL are non-Hodgkin lymphoma,involving the ovary and cervix.In some cases,involvement of uterine corpus,vagina,and vulva is reported.In this article,we report two cases of non-Hodgkin lymphoma in the female genital system,one from the uterus and the other from the ovary.By presenting the diagnosis and treatment of the two cases and reviewing the literature,we aim to provide a reference for clinicians in recognizing and treating rare cases.
Female ; Humans ; Genital Neoplasms, Female/diagnosis* ; Lymphoma, Non-Hodgkin/diagnosis* ; Ovarian Neoplasms

Langerhans cell histiocytosis of bone is a rare tumor disease characterized by the large accumulation of CD1a⁺ and CD207⁺ dendritic cells in tissues of unknown cause.It mainly occurs in children aged 1-4 years old,with incidences of 4-6 per million in children and 1-2 per million in adults.Due to its low incidence,diverse clinical manifestations,and no obvious specificity of imaging manifestations,the definitive diagnosis and early treatment of this type of tumor are challenging.In this paper,we report 8 cases of Langerhans cell histiocytosis of bone and review the relevant literature published in the past five years to summarize the clinical characteristics,pathological features,diagnosis,treatment,and prognosis of this disease.
Humans ; Bone Diseases/therapy* ; Histiocytosis, Langerhans-Cell/therapy*

Occult Breast Carcinomas (OBCs) are rare. History and physical examination alone may lead to misdiagnosis hence inappropriate investigative and treatment modalities. Diagnosis is difficult without tissue biopsy and extensive immunohistochemical staining. Presented here is a 74-year-old Filipino male with a 2-month history of axillary mass with erythematous skin, initially assessed as hidradenitis suppurativa failing to resolve with antibiotics. An excision biopsy revealed adenocarcinoma within the lymph nodes. Immunohistochemical stains confirmed a breast primary. Radiologic imaging showed no breast lesions and no distant metastasis. Axillary node dissection done showed metastasis to 5 in 14 nodes harvested, classifying him as OBC Stage IIIA (cT0pN2M0). He completed whole breast radiotherapy and chemotherapy. No tumor recurrence was documented thereafter. Although misdiagnosis is common, OBC is a condition to consider in male patients presenting with axillary lymphadenopathy.

BACKGROUND: T-cell lymphoblastic lymphoma/acute lymphoblastic leukemia (T-LBL/ALL) is an aggressive form of hematological malignancy associated with poor prognosis in adult patients. Histone deacetylases (HDACs) are aberrantly expressed in T-LBL/ALL and are considered potential therapeutic targets. Here, we investigated the antitumor effect of a novel HDAC inhibitor, chidamide, on T-LBL/ALL. METHODS: HDAC1, HDAC2 and HDAC3 levels in T-LBL/ALL cell lines and patient samples were compared with those in normal controls. Flow cytometry, transmission electron microscopy, and lactate dehydrogenase release assays were conducted in Jurkat and MOLT-4 cells to assess apoptosis and pyroptosis. A specific forkhead box O1 (FOXO1) inhibitor was used to rescue pyroptosis and upregulated gasdermin E (GSDME) expression caused by chidamide treatment. The role of the FOXO1 transcription factor was evaluated by dual-luciferase reporter and chromatin immunoprecipitation assays. The efficacy of chidamide in vivo was evaluated in a xenograft mouse. RESULTS: The expression of HDAC1, HDAC2 and HDAC3 was significantly upregulated in T-LBL/ALL. Cell viability was obviously inhibited after chidamide treatment. Pyroptosis, characterized by cell swelling, pore formation on the plasma membrane and lactate dehydrogenase leakage, was identified as a new mechanism of chidamide treatment. Chidamide triggered pyroptosis through caspase 3 activation and GSDME transcriptional upregulation. Chromatin immunoprecipitation assays confirmed that chidamide led to the increased transcription of GSDME through a more relaxed chromatin structure at the promoter and the upregulation of FOXO1 expression. Moreover, we identified the therapeutic effect of chidamide in vivo . CONCLUSIONS This study suggested that chidamide exerts an antitumor effect on T-LBL/ALL and promotes a more inflammatory form of cell death via the FOXO1/GSDME axis, which provides a novel choice of targeted therapy for patients with T-LBL/ALL.
Humans ; Pyroptosis/drug effects* ; Forkhead Box Protein O1/genetics* ; Aminopyridines/pharmacology* ; Animals ; Mice ; Benzamides/pharmacology* ; Cell Line, Tumor ; Precursor T-Cell Lymphoblastic Leukemia-Lymphoma/drug therapy* ; Phosphate-Binding Proteins/metabolism* ; Histone Deacetylase Inhibitors/pharmacology* ; Jurkat Cells ; Histone Deacetylases/metabolism* ; Apoptosis/drug effects* ; Gasdermins

A 4-year-old boy was admitted to the hospital with a 3-day history of rash and intermittent abdominal pain, during which abnormal results from routine blood tests were discovered. Initially, he presented with acute jaundice hepatitis and pancytopenia. The patient's condition progressed rapidly, with recurrent fever, worsening jaundice of the skin and sclera, and progressively worsening hepatosplenomegaly. Liver function impairment and bone marrow failure continued to deteriorate, while cytokine levels continued to rise. After excluding infections, autoimmune diseases, tumors, genetic metabolic disorders, and toxicities, the patient was diagnosed with hepatitis-associated aplastic anemia (HAAA) complicated by hemophagocytic lymphohistiocytosis (HLH). Following treatment with corticosteroids, plasma exchange, intravenous immunoglobulin, and liver protection therapy, the patient's symptoms partially alleviated. Aplastic anemia complicated by HLH is relatively uncommon, and HAAA complicated by HLH is even rarer, often presenting insidiously and severely. This paper presents a case of HAAA complicated by HLH and summarizes previously reported cases in the literature, providing references for the early diagnosis and treatment of this condition.
Humans ; Lymphohistiocytosis, Hemophagocytic/therapy* ; Male ; Anemia, Aplastic/complications* ; Child, Preschool ; Hepatitis/complications*

OBJECTIVES: To study the clinical characteristics, efficacy, and prognosis of pediatric Langerhans cell histiocytosis (LCH). METHODS: A retrospective analysis was conducted on 72 children with newly diagnosed LCH. RESULTS: The median age of the 72 children was 5 years (range: 0-14 years), with skull involvement being the most common (56 cases, 77.8%). The BRAF-V600E mutation was not associated with clinical characteristics, efficacy, or prognosis (P>0.05). The 5-year overall survival rate was 91.6%±4.2%, and the 5-year event-free survival (EFS) rate was 67.5%±5.8%. The 6-week chemotherapy response rate and 5-year EFS rate were lower in the risk organ involvement group compared to the no risk organ involvement group (P<0.05). The five-year overall survival rates for the group with multi-system involvement and the group with platelet count ≥450×10⁹/L were respectively lower than those for the single-system involvement group and the group with platelet count <450×10⁹/L (P<0.05). Risk organ involvement is an independent risk factor for 5-year EFS (P<0.05). CONCLUSIONS Skull is the most commonly affected site in pediatric LCH. The BRAF-V600E mutation is not related to clinical characteristics, efficacy, or prognosis. Elevated platelet count, risk organ involvement, and multisystem involvement are associated with poor prognosis, with risk organ involvement being an independent risk factor for 5-year EFS.
Humans ; Histiocytosis, Langerhans-Cell/therapy* ; Child, Preschool ; Child ; Male ; Infant ; Female ; Adolescent ; Retrospective Studies ; Proto-Oncogene Proteins B-raf/genetics* ; Prognosis ; Infant, Newborn ; Mutation

The patient was a girl, aged 10 years, who was admitted due to fever for 5 days and pancytopenia in peripheral blood for 2 days. Bone marrow examination showed the presence of phagocytic activity, and peripheral blood tests showed pancytopenia, an increase in ferritin, a reduction in fibrinogen, increases in triglyceride and sCD25, and a reduction in natural killer cell activity, which led to the diagnosis of hemophagocytic lymphohistiocytosis (HLH). On the day of admission, the child developed convulsions and rapidly progressed to refractory status epilepticus, which was consistent with the manifestations of febrile infection-related epilepsy syndrome. HLH was controlled after active immunotherapy, with the sequela of refractory epilepsy, and her cognitive function was essentially within normal limits. This article reports the condition of febrile infection-related epilepsy syndrome caused by HLH for the first time in China, in order to improve the awareness of this disease among clinicians.
Humans ; Lymphohistiocytosis, Hemophagocytic/complications* ; Female ; Child ; Epilepsy/etiology* ; Fever/etiology* ; Epileptic Syndromes/etiology*