To date, only 30 cases of Langerhans cell sarcoma, including the present case, have been reported. We report a new case of Langerhans cell sarcoma that presented with multifocal cutaneous involvement. [ncbi.nlm.nih.gov]

Neoplastic cells can have moderate or abundant cytoplasm, ± foamy or vacuolated Focal necrosis often present Eosinophils are few or absent in background Lymph nodes LCS may show variety of patterns; often a mixture present Diffuse effacement Vaguely [basicmedicalkey.com]

• Lymphadenopathy

  FDG PET/CT demonstrated heterogeneous high-grade activity in the pulmonary mass located in the right upper lung (standardized uptake value of 20), with central necrosis, bilateral upper mediastinal lymphadenopathy, right supraclavicular lymphadenopathy [ncbi.nlm.nih.gov]

  LCH was diagnosed on FNA smears in seven cases confined to the head and neck region, which included three cases of lymphadenopathy, three cases of scalp swelling, and one case of orbital swelling. [jcytol.org]

• Inflammation

  This proliferation is accompanied by inflammation and granuloma formation. Electron microscopy may reveal characteristic Birbeck granules. [radiopaedia.org]

  These cells are produced by the bone marrow and has a role in the inflammation response of the body. They are named after the scientist Paul Langerhans who first discovered them in 1968. [cancerwall.com]

  In cases of liver involvement of LCH, periportal low density can be seen in CT images due to periportal histiocyte infiltration, inflammation, and edema. [kjronline.org]

• Asymptomatic

  Intracranial xanthogranulomas are asymptomatic in the majority of cases and discovered only at autopsy (5). Most of them arise in the choroid plexus of the ventricular systems (14). [path.upmc.edu]

  Largest series consisted of 4 cases o Mostly elderly adults with no sex predilection · Sites of Involvement o Wide presentation o Solitary LN (most common) o Skin, intestinal, soft tissue, and hepatosplenomegaly been reported · Clinical features o Usually asymptomatic [hemepathreview.com]

  Treatment dilemmas in asymptomatic children with primary hemophagocytic lymphohistiocytosis. Blood. 2018; 132(19): 2088-2096. DOI:10.1182/blood-2018-01-872485. [biocrucesbizkaia.org]

• Hepatosplenomegaly

  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. [ncbi.nlm.nih.gov]

  Contrast-enhanced abdominal CT showed hepatosplenomegaly, periportal low density and multiple small, hypodense-lesions in the liver. [kjronline.org]

  Systemic symptoms (fever and weight loss), even in patients with solitary mass o Skin manifestations vary widely § Benign-appearing rash § Solitary lesions § Innumerable tumors on trunk and extremities o GI lesions § Possible intestinal obstruction o Hepatosplenomegaly [hemepathreview.com]

  […] sarcoma usually arises de novo Rare cases of LCS progress from antecedent Langerhans cell histiocytosis (LCH) Rare case reports Most patients present with extranodal mass without other symptoms ˜ 40% of patients have stage III or IV disease ˜ 20% have hepatosplenomegaly [basicmedicalkey.com]

• Fracture

  Although it rarely happens, these lesions may even lead to pathological fractures [1, 2, 4]. Proliferation of the Langerhans’ cells on the skin will manifest as skin rashes. These rashes may be petechial, papular, macular or nodular in nature. [cancerwall.com]

  Sometimes, children experience spontaneous fractures as a result of these bone lesions. [hopkinsmedicine.org]

  Record details Read Online Read More Add to Saved list × Academic Journal Frontal sinus fractures in children. Authors : Epure, Veronica 1 ; Gheorghe, D. [masader.om]

• Back Pain

  We present here a case of Langerhans cell sarcoma involving the eighth thoracic vertebra in a 31-yr-old man who presented with a two-month history of back pain. [medicineandhealthukm.com]

• Sweating

  […] present with extranodal mass without other symptoms ˜ 40% of patients have stage III or IV disease ˜ 20% have hepatosplenomegaly ˜ 10% have pancytopenia; often associated with bone marrow involvement Systemic symptoms in subset of patients Fatigue, night sweats [basicmedicalkey.com]

  Other symptoms that LCH can cause are enlargement of lymph nodes, hepatomegaly, splenomegaly, anorexia, night sweats, fever and weight loss [1, 2, 4]. [cancerwall.com]

  […] pelvis Yellow skin Vomiting Diarrhea Blood in stool Liver/spleen Enlarged liver or belly Abnormal blood tests Hormones (endocrine system) Increased thirst or dehydration Increased need to urinate Fatigue Rapid weight change (either up or down) Fever or sweats [stjude.org]

  […] sex predilection · Sites of Involvement o Wide presentation o Solitary LN (most common) o Skin, intestinal, soft tissue, and hepatosplenomegaly been reported · Clinical features o Usually asymptomatic mass o Systemic symptoms: fatigue, fever, night sweats [hemepathreview.com]

• Urticaria

  Eosinophils, a common finding in LCH can also be seen in a variable number of conditions such as drug eruptions, urticaria and wells syndrome. Furthermore, a group of conditions named as non-Langerhans cells histiocytosis (N-LCH) can mimic LCH. [intechopen.com]

• Headache

  […] system) Increased thirst or dehydration Increased need to urinate Fatigue Rapid weight change (either up or down) Fever or sweats Change in menstrual cycle Lungs Chest pain Problems breathing or shortness of breath Cough Brain or Central Nervous System Headaches [stjude.org]

  Other potential signs and symptoms include: weight loss, jaundice, vomiting, limping, short stature, delayed puberty, mental deterioration, headache, dizziness, seizures, protruding eyeballs, and/or a generalized rash. [hopkinsmedicine.org]

  There was no associated B symptoms, cough, headache, change in voice, dysphagia or any bony pains. On clinical examination, ECOG performance status was 1and vitals were stable. [omicsonline.org]

• Neck Stiffness

  Neurologic findings including irritability, bulging fontanel, neck stiffness, hypotonia, hypertonia, convulsions, cranial nerve palsies, ataxia, hemiplegia, blindness, and unconsciousness; 3. [hopkinsmedicine.org]

Even though these results may be inflated due to a strong referral bias, the study highlights the possible progression of unifocal bone disease and the importance of a comprehensive workup and follow-up. [28] ↑ Hester CC, Silkiss RZ. [eyewiki.aao.org]

Blood workup showed a WBC count of 12.6 × 10 9 /L with 1% of lymphocytes. One lymph node from her right groin was biopsied. [najms.org]

• Leukocytosis

  The second case presented with leukocytosis, thrombocytopenia and splenomegaly, which resolved with chemotherapy. [pagepress.org]

We suggested palliative treatment with chemotherapy and radiation. The patient refused any treatment and died 2 weeks later. [ncbi.nlm.nih.gov]

Chemotherapy alone was not considered an effective treatment. [pagepress.org]

Treatment depends on the severity of the cancer. Doctors may suggest patients undergo surgery to remove the abnormal cell growth. [diseaseinfosearch.org]

Uchida et al. ( 5 ) reported the successful treatment by mesna, doxorubicin, ifosfamide, and dacarbazine regimen, which is one of the treatment protocols for soft tissue sarcoma. [kjronline.org]

KEYWORDS: Head and neck; Langerhans cell sarcoma; Management; Prognosis [ncbi.nlm.nih.gov]

Treatment and prognosis The prognosis can be extremely variable with eosinophilic granuloma carrying the best and Letterer-Siwe disease-carrying the worst prognosis. [radiopaedia.org]

Etiology FDC express CD21 which is EBV receptor, however the correlation is not well documented Hyaline-vascular Castleman disease may be a predisposing factor to FDC p53 pathway may play a role Epidermal growth factor receptor(EGFR) expression has [slideshare.net]

Cervical LNs most often affected o Also common LNs: maxillary, mediastinal, mesenteric and retroperitoneum o Extranodal sites: tonsil, spleen, oral cavity, GI tract, liver, soft tissue, skin, and breast o Common sties of metastasis: LNs, lung, and liver · Etiology [hemepathreview.com]

Although the etiology of FDCS is unknown, the condition has been associated with Epstein-Barr virus ( 7 ). [e-crt.org]

[…] the characteristic granuloma-like lesions of LCH consists of Langerhans cells, macrophages, eosinophils, T lymphocytes and plasma cells, it is thought that clonal expansion of defective immature Langerhans cells is the key pathogenetic element. [3] Etiology [eyewiki.aao.org]

The etiology of LCH/LCS is unknown in most cases, and there is not clear understanding of the pathogenesis. [intechopen.com]

Epidemiology 80 cases was documented until 2011 in English literature Pancreat Dis Int，Vol 10，No 4 • August 15，2011 Hepatobiliary Onset age is various (9-82), mean age is about 50 Female: male: 1.2:1 Presentation: Painless, slow-growing mass Lab: [slideshare.net]

National Cancer Institutes, Surveillance, Epidemiology, and End Results Program. Available online at http://seer.cancer.gov/statfacts/html/nhl.html. Accessed July 21, 2015. SEER Training Modules, WHO Classification. [labtestsonline.it]

Abbreviations Langerhans cell sarcoma (LCS) Synonyms Dendritic/histiocytic sarcoma, Langerhans cell type Malignant histiocytosis X Malignant Langerhans cell tumor Definitions Neoplastic proliferation of overtly malignant Langerhans cells CLINICAL ISSUES Epidemiology [basicmedicalkey.com]

Histiocytic disorders of children and adult, introduction to the problem, overview, historical perspective and epidemiology. [path.upmc.edu]

Epidemiology, antibiotic therapy and clinical outcomes of healthcare-associated pneumonia in critically ill patients: a Spanish cohort study. Intensive Care Med. 2014; 40(4): 572-581. DOI:10.1007/s00134-014-3239-2. [biocrucesbizkaia.org]

Unlike in LCH, the pathophysiology and clinical course of LCS are unclear due to its rarity. Here, we report the case of a 73-year-old male patient who was diagnosed with cutaneous LCH and pulmonary LCS at the same time. [ncbi.nlm.nih.gov]

These image findings may be seen in cases of LCS due to the similar pathophysiology. However, unlike LCH, in which punctate, calcification in the thymus has been described, thymic calcification could not be seen in our 2 patients ( 10 ). [kjronline.org]

The lesions of LCH may involve the bones, skin, hypothalamus-pituitary region, as well as other organs. [2] Pathophysiology While the characteristic granuloma-like lesions of LCH consists of Langerhans cells, macrophages, eosinophils, T lymphocytes and [eyewiki.aao.org]

Spontaneous regression has been reported, perhaps suggesting an incomplete understanding of the pathophysiology 26. A number of factors could be responsible for the worse prognosis in intraabdominal, extranodal fdcs. [current-oncology.com]

Influenza Vaccine Effectiveness in Preventing Outpatient, Inpatient, and Severe Cases of Laboratory-Confirmed Influenza. Clin Infect Dis. 2013;57:167-75. [biocrucesbizkaia.org]