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Yonsei Medical Journal Sep 2020Histiocytic and dendritic cell neoplasms are rare hematologic tumors. This study aimed to describe the epidemiologic features of the entire spectrum of histiocytic and...
PURPOSE
Histiocytic and dendritic cell neoplasms are rare hematologic tumors. This study aimed to describe the epidemiologic features of the entire spectrum of histiocytic and dendritic cell neoplasms, including clinicopathological variables and patient outcomes.
MATERIALS AND METHODS
We comprehensively reviewed 274 patients who were diagnosed with histiocytic and dendritic neoplasms at Severance Hospital, Seoul, South Korea between 1995 and 2018.
RESULTS
The most common neoplasm was Langerhans cell histiocytosis (LCH), followed by dermal xanthogranuloma. Among non-LCH sarcomas, histiocytic sarcoma (HS) showed a relatively high prevalence, followed by follicular dendritic cell sarcoma (FDCS). Disseminated juvenile xanthogranuloma (DJG), Erdheim-Chester disease (ECD), indeterminate dendritic cell tumor (IDCT), and interdigitating dendritic cell sarcoma (IDCS) rarely occurred. Generally, these tumors presented in childhood, although the non-LCH sarcoma (HS/FDCS/IDCS/IDCT) group of tumors and ECD occurred in late adulthood. Multiorgan involvement and advanced Ann-Arbor stage, as well as recurrence and death of disease, were not uncommon. The non-LCH sarcoma group had the worst overall survival, compared to the DJG, ECD, and LCH groups.
CONCLUSION
Our findings indicate that histiocytic and dendritic cell neoplasms exhibit heterogeneous epidemiologic characteristics and that some patients may have unfavorable outcomes, especially those with non-LCH sarcoma.
Topics: Adult; Child; Dendritic Cell Sarcoma, Follicular; Dendritic Cells; Female; Histiocytes; Histiocytic Disorders, Malignant; Histiocytic Sarcoma; Histiocytosis, Langerhans-Cell; Humans; Male; Neoplasm Recurrence, Local; Prevalence; Republic of Korea; Seoul; Xanthogranuloma, Juvenile
PubMed: 32882761
DOI: 10.3349/ymj.2020.61.9.774 -
Journal of Clinical and Experimental... 2013Histiocytic sarcoma (HS) is an extremely rare malignant neoplasm showing morphologic and immunophenotypic evidence of histiocytic differentiation. The vast majority of... (Review)
Review
Histiocytic sarcoma (HS) is an extremely rare malignant neoplasm showing morphologic and immunophenotypic evidence of histiocytic differentiation. The vast majority of previously reported HSs are now generally recognized to be misdiagnosed examples of non-Hodgkin lymphomas, predominantly diffuse large B-cell lymphoma or anaplastic large cell lymphoma. The recognition of such tumors parallels the development and widespread use of immunohistochemical techniques, along with the development of molecular genetic methods to detect immunoglobulin (IG) or T-cell receptor (TCR) gene rearrangement. The 2001 World Health Organization (WHO) definition of HS requires the absence of clonal B/T-cell receptor gene rearrangements. However, the 2008 WHO classification no longer strictly requires the absence of clonal immunoglobulin heavy chain (IGH) or TCR gene rearrangement for the diagnosis of HS. Recent studies demonstrated that HSs that occur subsequent to or concurrent with B- or T-lymphoblastic lymphoma/leukemia or mature B-cell neoplasms generally show clonal IgH and/or TCR gene rearrangement. These findings suggest the possibility of transdifferentiation of the two otherwise morphologically and immunohistochemically distinctive neoplasms. In addition, a recent study suggested clonal IG gene rearrangements may be detected at a high frequency in sporadic HS, indicating that a large subset of sporadic HSs may inherit the B-lymphocyte genotype. These findings provide new insights into the pathogenesis of HS, although the etiology of HS is still unknown. HS is a diagnosis of exclusion. It is necessary to rule out other diseases that could be misdiagnosed as HS with extensive immunophenotypical analysis before diagnosing HS.
Topics: Histiocytic Sarcoma; Humans; Immunophenotyping; Molecular Biology; World Health Organization
PubMed: 23801128
DOI: 10.3960/jslrt.53.1 -
Hippokratia 2021Diffuse cystic lung diseases are a group of heterogeneous pathophysiological processes and include neoplastic, inflammatory, and infectious etiologies. This manuscript...
BACKGROUND
Diffuse cystic lung diseases are a group of heterogeneous pathophysiological processes and include neoplastic, inflammatory, and infectious etiologies. This manuscript focuses on manifestations of pulmonary Langerhans cell histiocytosis (PLCH) and lymphangioleiomyomatosis (LAM). Description of the cases: Three female patients with LAM and one with PLCH are described. Stress dyspnea was a key symptom. There were similar cyst patterns in more than one lung lobe with a slow, progressive course. Histopathology confirmed the LAM diagnosis resulting from the nodular proliferate and the cyst wall that strongly expressed Human Melanoma Black-45 (HMB-45). A typical constellation for PLCH was demonstrated in high-resolution computed tomography (HRCT). It was found to be disseminated and relatively thick-walled cysts, mainly in the upper and middle parts. An individualized therapy was applied. Three patients with mild symptoms were followed up, including HRCT evaluations. Sirolimus was administered to one patient with a severe manifestation of LAM.
CONCLUSION
LAM and PLCH are rare. High-resolution computed tomography is an essential diagnostic tool. Lung emphysema as misdiagnosis should be avoided. The characteristics of pulmonary cysts, the cyst's wall regularity, and identification of associated pulmonary lesions, should be evaluated. A promising new therapy concept are mTOR inhibitors are, especially in LAM. The most important recommendation in PLCH is the cessation of cigarette smoking. HIPPOKRATIA 2021, 25 (2):83-86.
PubMed: 35937517
DOI: No ID Found -
Frontiers in Immunology 2023Histiocytic sarcoma (HS) is a rare hematological malignancy with limited treatment options, and it is also prone to complications such as hemophagocytic...
Histiocytic sarcoma (HS) is a rare hematological malignancy with limited treatment options, and it is also prone to complications such as hemophagocytic lymphohistiocytosis (HLH) in the later stages of the disease, leading to difficulties in treatment and poor prognosis. It highlights the importance of developing novel therapeutic agents. Herein, we present a case of a 45-year-old male patient who was diagnosed with PD-L1-positive HS with HLH. The patient was admitted to our hospital with recurrent high fever, multiple skin rashes with pruritus throughout the body and enlarged lymph nodes. Subsequently, pathological biopsy of the lymph nodes revealed high expression of CD163, CD68, S100, Lys and CD34 in the tumor cells and no expression of CD1a and CD207, confirming this rare clinical diagnosis. Concerning the low remission rate by conventional treatment in this disease, the patient was administered with sintilimab (an anti-programmed cell death 1 [anti-PD-1] monoclonal antibody) at 200 mg/d combined with a first-line chemotherapy regimen for one cycle. Further exploration of pathological biopsy by using next-generation gene sequencing led to the use of targeted therapy of chidamide. After one cycle of combination therapy (chidamide+sintilimab, abbreviated as CS), the patient achieved a favorable response. The patient showed remarkable improvement in the general symptoms and laboratory examination results (e.g., elevated indicators of inflammation); even the clinical benefits was not persistent, he survived one more month after his cessation of treatment by himself due to economic difficulty. Our case suggests that PD-1 inhibitor coupled with targeted therapy might constitute a potential therapeutic option for primary HS with HLH.
Topics: Male; Humans; Middle Aged; Lymphohistiocytosis, Hemophagocytic; Histiocytic Sarcoma; Programmed Cell Death 1 Receptor; Mutation
PubMed: 36969238
DOI: 10.3389/fimmu.2023.1127599 -
Archives of Pathology & Laboratory... Dec 2022A wide spectrum of mesenchymal tumors harboring ALK gene rearrangements has been identified outside the archetypal example of ALK-positive inflammatory myofibroblastic...
CONTEXT.—
A wide spectrum of mesenchymal tumors harboring ALK gene rearrangements has been identified outside the archetypal example of ALK-positive inflammatory myofibroblastic tumors.
OBJECTIVE.—
To evaluate the molecular pathology of unusual ALK-positive mesenchymal tumors and their response to ALK-targeted treatments.
DESIGN.—
Seven patients with ALK-positive mesenchymal tumors, including inflammatory epithelioid cell sarcoma, undifferentiated sarcoma, histiocytic neoplasm, smooth muscle tumor of uncertain malignant potential (STUMP), and atypical fibrohistiocytic tumor, were included on the basis of aberrant ALK immunoexpression. Patients with inflammatory myofibroblastic tumors were excluded from the study. ALK gene rearrangement was investigated either by fluorescence in situ hybridization or next-generation sequencing.
RESULTS.—
ALK was immunolabeled in all patients, diffusely (≥50%) in 6 patients and partially (10%-50%) in 1 patient. ALK gene rearrangement was discovered in 5 of the 6 available patients. The 3'-partners of ALK fusion were identified in 3 of 4 investigated patients as follows: PRKAR1A-ALK (ALK-positive histiocytic neoplasm), TNS1-ALK (STUMP), and KIF5B-ALK (ALK-positive atypical fibrohistiocytic tumor). We failed to discover ALK translocation in 1 patient with ALK-positive inflammatory epithelioid cell sarcoma. However, transcriptomic investigation showed that this tumor was significantly enriched with ALK-related pathways, which suggested activation of ALK through a nontranslocation pathway, as a constitutive oncogenic mark in this tumor. ALK-targeted inhibitors, which were administered to 3 patients with metastatic diseases, achieved partial remission in 1 patient with ALK-positive inflammatory epithelioid cell sarcoma and stable disease in patients with ALK-positive undifferentiated sarcoma and STUMP.
CONCLUSIONS.—
Molecular investigation of ALK-positive mesenchymal neoplasms could allow for an accurate diagnosis and personalized treatment.
Topics: Humans; In Situ Hybridization, Fluorescence; Anaplastic Lymphoma Kinase; Sarcoma; Soft Tissue Neoplasms; Smooth Muscle Tumor; Protein Kinase Inhibitors; Liver Neoplasms
PubMed: 35438749
DOI: 10.5858/arpa.2021-0330-OA -
World Journal of Clinical Cases Oct 2022Malignant splenic tumors are rare but fatal, presenting a challenge in diagnosis and management involving hematology, oncology, and general surgery. By contrast,...
BACKGROUND
Malignant splenic tumors are rare but fatal, presenting a challenge in diagnosis and management involving hematology, oncology, and general surgery. By contrast, diagnosing and treating other common malignant tumors (such as lung and gastrointestinal cancer) offers multiple strategies for chemotherapy, radiotherapy, targeted therapy, and immunotherapy with the prospect of a cure. With various specialists involved in clinical multiple disciplinary team (MDT) discussion, personal bias can be minimized. It can also ignite important discussion which can benefit not only one patient but many patients.
CASE SUMMARY
Here, we report on the MDT diagnosis and management of the malignant splenic tumors littoral cell angiosarcoma and histiocytic sarcoma. Although only two cases of rare primary splenic malignancy are presented, MDT is a novel means of rare disease treatment.
CONCLUSION
To benefit patients, imaging analysis, safe operation, precise pathology examination, and individualized therapeutic treatment strategies are required. The involvement of various specialists in a clinical MDT discussion minimizes personal bias and can create important ideas to benefit all patients.
PubMed: 36312480
DOI: 10.12998/wjcc.v10.i29.10535 -
Journal of Clinical and Experimental... 2015We report a 16-year-old male with histiocytic sarcoma (HS) originating in the lung. Partial resection of the lung was performed for a 3-cm mass with a clear boundary... (Review)
Review
We report a 16-year-old male with histiocytic sarcoma (HS) originating in the lung. Partial resection of the lung was performed for a 3-cm mass with a clear boundary detected in the right inferior pulmonary lobe on a health checkup. Histologically, the tumor infiltrated into the surrounding tissue, and was comprised of spindle cells, mainly, and foam cells accompanied by mild nuclear atypia. The tumor cells were immunohistochemically positive for CD68 and CD163, indicating histiocytic lineage and the MIB-1-positive rate was low. Spindle cell morphology of HS is quite rare and only 3 cases of pulmonary HS have previously been reported.
Topics: Adolescent; Biomarkers; Histiocytic Sarcoma; Humans; Immunohistochemistry; Lung; Lung Neoplasms; Male; Tomography, X-Ray Computed
PubMed: 26106007
DOI: 10.3960/jslrt.55.45 -
The Journal of Veterinary Medical... May 2016Histiocytic sarcoma is a progressive and fatal malignant neoplasm that mainly occurs in middle- to old-aged dogs. This study describes clinicopathological, histological...
Histiocytic sarcoma is a progressive and fatal malignant neoplasm that mainly occurs in middle- to old-aged dogs. This study describes clinicopathological, histological and immunohistochemical characteristics of intracranial histiocytic sarcomas in 23 dogs. Magnetic resonance imaging and/or computed tomography of the brains revealed that the tumors mainly located in the cerebrum, particularly the frontal lobe. Seizure was a predominant clinical sign in most of the cases. Histologically, the tumor cells were morphologically classified into round/polygonal- and spindle-shaped cell types. There was a significant association between tumor cell types and hemophagocytic activity (P<0.05). However, there was no significant difference in other clinicopathological parameters and mitotic index between the 2 types. Immunohistochemically, tumor cells were strongly positive for HLA-DR, Iba-1 and CD204 in all the 23 cases, for iNOS in 20, for CD163 in 17, for CD208 (DC-LAMP) in 9, for lysozyme in 8 and for S100 in 5 cases. In addition, the Ki67-proliferative index showed range of 0.50-64.33% (Average 26.60 ± 3.81%). These observations suggest that canine primary intracranial histiocytic sarcomas tend to exhibit both dendritic cell and macrophage phenotypes of histiocytic differentiation.
Topics: Animals; Biomarkers, Tumor; Brain Neoplasms; Dog Diseases; Dogs; Female; Histiocytic Sarcoma; Immunohistochemistry; Male
PubMed: 26668164
DOI: 10.1292/jvms.15-0627 -
Cancer Diagnosis & Prognosis 2022Primary mediastinal non-seminomatous germ cell tumors (PMNSGCTs) are occasionally complicated by a hematologic malignancy, as with somatic-type malignant tumors called...
BACKGROUND/AIM
Primary mediastinal non-seminomatous germ cell tumors (PMNSGCTs) are occasionally complicated by a hematologic malignancy, as with somatic-type malignant tumors called germ cell tumors with somatic-type malignancy (GCTSTM) and are known to have a poor prognosis.
PATIENTS AND METHODS
Data obtained between September 1997 and February 2020 for patients with mediastinal germ cell tumor at our institution were retrospectively analyzed. Key outcome measures included survival rates and the clinical features of non-seminoma cases.
RESULTS
Of 16 patients, 9 had pure seminoma, and 7 had non-seminoma. At the median follow-up of 56.2 months, the 5-year survival rate was significantly higher in patients with seminoma (100%) than in those with non-seminoma (37%) (log-rank test, p=0.0153). Regarding PMNSGCT, two patients evolved into GCTSTM and three had concomitant hematological malignancies.
CONCLUSION
Patients with PMNSGCTs, GCTSTM complications, and hematologic malignancies showed poor survival, suggesting the need for the development of treatment strategies.
PubMed: 35530648
DOI: 10.21873/cdp.10116 -
Veterinary Pathology Sep 2017In the past decade, NOD.Cg- Prkdc Il2rg/SzJ (NSG, NOD scid gamma) mice have become a model of choice in several areas of biomedical research; however, comprehensive data...
In the past decade, NOD.Cg- Prkdc Il2rg/SzJ (NSG, NOD scid gamma) mice have become a model of choice in several areas of biomedical research; however, comprehensive data on their spontaneous age-related pathology are not currently available in the literature. The prevalence of spontaneous morbidity affecting aged NSG female breeders enrolled in a parasitology study was documented with classification of neoplastic and non-neoplastic (inflammatory, metabolic, degenerative) lesions. Malignant mammary neoplasms were most commonly diagnosed, often accompanied by pulmonary metastases, while a low frequency of lymphoma and histiocytic sarcoma was documented. The major inflammatory conditions were suppurative pleuropneumonia and bronchopneumonia with abscess formation, from which Pasteurella pneumotropica was commonly isolated, followed by otitis media. Both inflammatory and degenerative lesions of the genital tract were identified, along with neoplasms such as endometrial yolk sac carcinomas and granulosa cell tumors. Novel conditions identified included renal tubular degeneration and necrosis associated with 2 concurrent types of intranuclear inclusions, focal or multifocal hyperostosis of the skull, and neuroendocrine tumors of the mesometrium. The majority of degenerative lesions that affected the genital tract, endocrine, and skeletal systems did not represent the actual underlying cause of death but rather were considered incidental findings. This study indicates that both inflammatory and neoplastic conditions contribute to morbidity and mortality in experimentally manipulated aged female NSG mice.
Topics: Aging; Animals; Disease Models, Animal; Female; Longitudinal Studies; Mice; Mice, Inbred NOD; Mice, SCID
PubMed: 28355107
DOI: 10.1177/0300985817698210