Kikuchi-Fujimoto disease,a rare form of necrotizing lymphadenitis,is an uncommon,benign,self-limiting disorder of obscure etiology.It affects mostly young adults of both genders.Clinically,it presents with fever and l...Kikuchi-Fujimoto disease,a rare form of necrotizing lymphadenitis,is an uncommon,benign,self-limiting disorder of obscure etiology.It affects mostly young adults of both genders.Clinically,it presents with fever and lymphadenopathy of a firm to rubbery consistency frequently involving cervical lymph nodes while weight loss,splenomegaly,leucopenia,and elevated erythrocyte sedimentation rate feature in severely affected patients.Cutaneous involvement occurs in about 30%-40%of cases as facial erythema and nonspecific erythematous papules,plaques,acneiform or morbilliform lesions of great histologic heterogeneity.Both Kikuchi-Fujimoto disease and systemic lupus erythematosus share an obscure and complex relationship as systemic lupus erythematosus may occasionally precede,develop subsequently,or sometimes be associated concurrently with Kikuchi-Fujimoto disease.It is often mistaken for non-Hodgkin lymphoma while lupus lymphadenitis,cat-scratch disease,Sweet’s syndrome,Still’s disease,drug eruptions,infectious mononucleosis,and viral or tubercular lymphadenitis are other common differentials.Fine needle aspiration cytology mostly has features of nonspecific reactive lymphadenitis and immunohistochemistry studies usually show variable features of uncertain diagnostic value.Since its diagnosis is exclusively from histopathology,it needs to be evaluated more carefully;an early lymph node biopsy will obviate the need for unnecessary investigations and therapeutic trials.Its treatment with systemic corticosteroids,hydroxychloroquine,or antimicrobial agents mostly remains empirical.The article reviews clinicoepidemiological,diagnostic,and management aspects of KFD from the perspective of practicing clinicians.展开更多
Primary histiocytic sarcoma of the spleen is a rare but potentially lethal condition. It can remain asymptomatic or only mildly symptomatic for a long time. An 81-year-old woman presented with an extremely enlarged sp...Primary histiocytic sarcoma of the spleen is a rare but potentially lethal condition. It can remain asymptomatic or only mildly symptomatic for a long time. An 81-year-old woman presented with an extremely enlarged spleen. She suffered from progressive anemia and required a red blood cell transfusion once a month. Although computed tomography, ultrasonography, and magnetic resonance imaging were performed for diagnosis, a confirmed diagnosis was not obtained. Her enlarged spleen compressed her stomach, and she suffered from gastritis and a sense of gastric fullness just after meals. She underwent laparoscopic splenectomy for therapeutic and diagnostic purposes. Her postoperative course was uneventful. After surgery, her red blood cell and platelet counts increased markedly. The tumor was diagnosed as splenic histiocytic sarcoma. Post-surgical chemotherapy was not performed, and the patient died of liver failure due to liver metastasis 5 mo after surgery. Laparoscopic splenectomy is minimally invasive and useful for the relief of symptoms related to hematological disorders. However, in cases of an enlarged spleen, optimal views and working space are limited. In such cases, splenic artery ligation can markedly reduce the size of the spleen, thus facilitating the procedure. The case reported herein suggests that laparoscopic splenectomy may be useful for the treatment of splenic malignancy.展开更多
Kikuchi-Fujimoto disease(KFD), also known as histiocytic necrotizing lymphadenitis, is an uncommon condition, typically characterized by lymphadenopathy and fevers. It usually has a benign course; however, it may prog...Kikuchi-Fujimoto disease(KFD), also known as histiocytic necrotizing lymphadenitis, is an uncommon condition, typically characterized by lymphadenopathy and fevers. It usually has a benign course; however, it may progress to fatality in extremely rare occasions. The diagnosis is made via lymph node biopsy and histopathology. Our patient was a young female who presented with shortness of breath, fever, and malaise. Physical examination revealed significant cervical and axillary lymphadenopathy. Chest X-ray displayed multilobar pneumonia. She required intubation and mechanical ventilation for progressive respiratory distress. Histopathology of lymph nodes demonstrated variable involvement of patchy areas of necrosis within the paracortex composed of karyorrhectic debris with abundant histiocytes consistent with KFD. After initial stabilization, the patient's condition quickly deteriorated with acute anemia, thrombocytopenia and elevated prothrombin time, partial prothrombin time, and D-dimer levels. Disseminated intravascular coagulopathy(DIC) ensued resulting in the patient's fatality. DIC in KFD is not well understood, but it is an important cause of mortality in patients with aggressive disease.展开更多
BACKGROUND Granuloma annulare(GA)has diverse clinical manifestations including papules,plaques,and nodules on the extremities that are skin-colored,pink,or purple.Approximately 15%of all GA cases are considered genera...BACKGROUND Granuloma annulare(GA)has diverse clinical manifestations including papules,plaques,and nodules on the extremities that are skin-colored,pink,or purple.Approximately 15%of all GA cases are considered generalized GA.CASE SUMMARY Herein,we describe the case of a pediatric patient who initially presented with papules and later developed generalized atrophic macules.Upon examination,two different morphologic lesions were histopathologically confirmed:Epithelioid nodular GA and scattered histiocytic infiltrative GA.This patient exhibited rare clinical manifestations that differed throughout the course of the disease.The varying histopathological types and clinical manifestations of GA may be linked to the different stages of the disease.CONCLUSION This rare case demonstrates the different histopathological features of different stages and clinical manifestations of granuloma annulare in an infant.展开更多
Objective: As a rare cause of fever and lymphadenopathy (especially in the neck) in children, the purpose of this study is to explore the clinical characteristics of histiocytic necrotizing lymphadenitis (HNL) in chil...Objective: As a rare cause of fever and lymphadenopathy (especially in the neck) in children, the purpose of this study is to explore the clinical characteristics of histiocytic necrotizing lymphadenitis (HNL) in children and improve the diagnosis and treatment of this disease. Methods: We retrospectively collected the data of 6 pediatric patients with HNL diagnosed by lymph node biopsy from 2012 to 2021 in department of pediatric, the affiliated Changsha Central Hospital, Hengyang Medical School, University of South China, including the clinical characteristics, laboratory examinations, imaging findings and histopathological examinations, treatment and follow-up of the patients and reviewed relevant literature. Results: All the patients were Han girls, with no kinship with each other, and four cases were from rural areas. The age of onset was from 7 to 14 years old, with durations ranged from 1 week to 3 months. All children had cervical lymphadenopathy with tenderness, five cases of fever, two cases of cough, one case of weight loss, four cases of leukopenia, one case of liver impairment, one case of combined bacterial infection of leukocytosis. All children underwent lymph node biopsy and were diagnosed with HNL, four children were treated with anti-infection before diagnosis, two children were treated with steroid therapy after diagnosis, and 1 case of weakened immune function was used immunoglobulin for modulating immunity. All children improved and discharged, and no recurrence was observed until October 2021. Conclusion: In our study, the children with swollen lymph nodes in the neck were accompanied by tenderness and fever as the main clinical manifestations, without specificity. Histopathology and immunohistochemical examination by removing the affected lymph nodes could confirm HNL. Most children had a good prognosis and long-term follow-up were required.展开更多
Histiocytic sarcoma(HS)is a rare malignant neoplasm that originates from a histiocytic hematopoietic lineage characterized by histiocytic differentiation and its corresponding immunophenotypic features.We herein repor...Histiocytic sarcoma(HS)is a rare malignant neoplasm that originates from a histiocytic hematopoietic lineage characterized by histiocytic differentiation and its corresponding immunophenotypic features.We herein reported a case of primary HS of the stomach which was confirmed through histopathologic examination and immunohistochemical staining.A 52-year-old woman presented with progressive difficulty in feeding and dull pain in the epigastric region.Gastroscopy,endo-scopic ultrasonography,double contrast examination, and computed tomography revealed a mass located on the posterior wall of fundus and lesser curvature of the stomach.Microscopically,the cytoplasm of the tumor cells was abundant and eosinophilic.Immunohisto-chemical staining revealed that the tumor cells were positive for CD45RO and CD68.It is difficult to differentiate HS of stomach from other gastric malignancies by radiological evaluation alone.However,HS may be considered when a protruding and ulcerated mass in stomach shows heterogeneous hypervascular features.To the best of our knowledge,this is the first report in English language literature that emphasizes the imaging findings of human gastric HS.展开更多
BACKGROUND Histiocytic sarcoma(HS)of the spleen is reported to be a rare and lethal disease.The clinicopathological features of splenic HS have not been well described.The objective of this paper is to describe the di...BACKGROUND Histiocytic sarcoma(HS)of the spleen is reported to be a rare and lethal disease.The clinicopathological features of splenic HS have not been well described.The objective of this paper is to describe the diagnosis and treatment of a case of this rare disease and provide a review of the literature.CASE SUMMARY In this article,we discuss the case of a 40-year-old Hispanic female who presented with progressive thrombocytopenia and multiple hypoechoic lesions in the spleen without splenomegaly.Positron emission tomography-computed tomography showed increased activity in cervical lymph nodes,as well as multiple bone and splenic lesions with positive uptake.Two bone marrow biopsies and fine-needle aspiration of the cervical lymph node were inconclusive.Laparoscopic splenectomy was performed,and gross examination showed a 110.1 g spleen with multiple rubbery,nodular lesions within the subcapsular sinus and splenic parenchyma.The microscopic findings showed multinodular histiocyte proliferation with atypia and multilobulated nuclei,which were positive for CD163,CD4,and CD68 by immunohistochemical analysis.The final pathologic diagnosis was difficult and was found to be low-grade HS of the spleen,after consultations with two renowned hematopathology institutions.At the patient’s five-month follow-up visit,her bone marrow metastasis had progressed.She is waiting to be enrolled in a clinical trial.CONCLUSION Pathologic diagnosis of splenic HS can be challenging.Low-grade differentiation may be associated with a slow progressive disease.展开更多
Histiocytic sarcoma is an extremely rare and very aggressive malignancy, with poor prognosis. The cases described in the literature are few and the treatment is not currently considered consensual. The clinical presen...Histiocytic sarcoma is an extremely rare and very aggressive malignancy, with poor prognosis. The cases described in the literature are few and the treatment is not currently considered consensual. The clinical presentation is very variable. Its characterization is made based primarily on the histology. The authors present a case of an 82-year-old woman, with multiple adenopatic retroperitoneal and left iliac conglomerates, with left leg associated edema (extrinsic compression by conglomerates). After intensive study and approach ganglion biopsy, the histologic diagnosis revealed a histiocytic sarcoma.展开更多
BACKGROUND Germ cell tumors(GCTs)account for 2%of human malignancies but are the most common malignant tumors among males aged 15-35.Since 1983,an association between mediastinal GCT(MGCT)and hematologic malignancies ...BACKGROUND Germ cell tumors(GCTs)account for 2%of human malignancies but are the most common malignant tumors among males aged 15-35.Since 1983,an association between mediastinal GCT(MGCT)and hematologic malignancies has been recognized.CASE SUMMARY We report a case in which malignant histiocytosis was associated with mediastinal GCTs.The clinical data of a male patient with MGCT admitted to Beijing Children's Hospital were collected retrospectively.The patient was first diagnosed according to imaging and pathological features as having MGCT,and was treated with surgery and chemotherapy.One year after stopping chemotherapy,imaging showed metastases in the right supraclavicular,mediastinum,hilar region and retroperitoneal lymph node,right pleura,right lung,and right para-cardiac margin.Pathological diagnosis of the liver nodular and hilar lymph nodes included systemic juvenile xanthogranuloma and Rosai-Dorfman lesions with malignant transformation(i.e.,morphological characteristics and immunophenotype of histiocytic sarcoma).Following diagnosis,the patient accepted chemotherapy with vindesine,cytarabine and dexamethasone.Positron emission tomography–computed tomography showed partial remission.The patient was followed-up for 10 mo after the diagnosis of malignant histiocytosis,and no sign of progression or relapse was observed.CONCLUSION Physicians should recognize the possibility of hematologic malignancies being associated with MGCT.Suitable sites should be selected for pathological examination.展开更多
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 tu...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.展开更多
Histiocytic sarcoma is an uncommon non-Langerhans histiocyte disorder of mature tissue histiocytes.The authors presented an example of this rare tumor in a 14-year-old girl who presented with left upper quadrant pain,...Histiocytic sarcoma is an uncommon non-Langerhans histiocyte disorder of mature tissue histiocytes.The authors presented an example of this rare tumor in a 14-year-old girl who presented with left upper quadrant pain,loss of appetite,and weight loss.A large 18 cm×10 cm heterogeneous solid and cystic enhancing mass was found in the left lobe of the liver.Based on the histomorphology and positivity for histiocyte-specific markers in a needle biopsy,a diagnosis of histiocytic sarcoma was made.Chemotherapy was initiated,but the tumor did not respond well,and she died about 7 weeks following initial diagnosis with multi-organ failure.At autopsy,the tumor showed extensive necrosis,with no evidence of metastatic spread.In conclusion,the diagnosis of histiocytic sarcoma is challenging,and requires a high index of suspicion,with an appropriate panel of confirmatory immunohistochemical stains.Recognition of this rare tumor is important because of its poor response to chemotherapy and high mortality.展开更多
In this article,a case of histiocytic sarcoma(HS) arising from tonsil in a 60 year-old male patient was reported.Consideried a local disease,a regime consisting of 4 cycles of(CHOP) associated with radiotherapy was us...In this article,a case of histiocytic sarcoma(HS) arising from tonsil in a 60 year-old male patient was reported.Consideried a local disease,a regime consisting of 4 cycles of(CHOP) associated with radiotherapy was used.A positive result was gained after treatment.But HS is a rare hematopoietic neoplasm derived from hematopoietic monoblasts with aggressive characteristic and poor outcome.Stage of disease and tumor size are possibly associated with the prognosis.展开更多
A 54-year-old man was admitted for the evaluation of fever and abodominal pain. Radiological and endo- scopic examination revealed a lung nodule and multiple small intestine uclers. Clinical diagnosis such as tubercul...A 54-year-old man was admitted for the evaluation of fever and abodominal pain. Radiological and endo- scopic examination revealed a lung nodule and multiple small intestine uclers. Clinical diagnosis such as tuberculosis and Crohn＇s disease had been proposed. He developed intestine perforation after small bowel endoscopic procedure. During emergent surgery the involved intestinal segments were resected and a pathological diagnosis of fibroblastic histiocytic sarcoma （FBRC） was made. The patient died in the sixth month after the operation. The management of this cases highlig^t~ the ~rawback of pattern recogn^tio~ as the most commonly used clinical reasoning method, and the importance of histological investigation.展开更多
Cytophagic histiocytic panniculitis （CHP） was first described by Winkelmann and Bowie in 1980.vj It is a rare group of diverse illnesses involving benign and malignant proliferation of macrophages in various organs ...Cytophagic histiocytic panniculitis （CHP） was first described by Winkelmann and Bowie in 1980.vj It is a rare group of diverse illnesses involving benign and malignant proliferation of macrophages in various organs and tissues. It presents with subcutaneous panniculitis with or without a hemophagocytic syndrome （HPS）. It occurs predominantly in women （male： female ratio 1：1.3） between the years of 5-61 （average, 33.5）. The major clinical features are recurrent fever, multiple panniculitic lesions, anemia, leukopenia and coagulation abnormalities. In the later phase, liver dysfuction, serosal effusion, mucosal ulceration and hemorrhage may occur. Histological findings show activated histiocyte infiltration of the fat tissue. Cytologically the benign-looking histiocytes containing cell fragments （bean-bag cells） are very typical. CHP has a broad spectrum from mild to severe. Benign CHP is selflimiting and sensitive to treatment, but up to now there is no effective therapy for malignant CHP. We report here a case of progressive and fatal cytophagic histiocytic panniculitis in a young woman who had encephaloclastic changes immediately prior to her death.展开更多
文摘Kikuchi-Fujimoto disease,a rare form of necrotizing lymphadenitis,is an uncommon,benign,self-limiting disorder of obscure etiology.It affects mostly young adults of both genders.Clinically,it presents with fever and lymphadenopathy of a firm to rubbery consistency frequently involving cervical lymph nodes while weight loss,splenomegaly,leucopenia,and elevated erythrocyte sedimentation rate feature in severely affected patients.Cutaneous involvement occurs in about 30%-40%of cases as facial erythema and nonspecific erythematous papules,plaques,acneiform or morbilliform lesions of great histologic heterogeneity.Both Kikuchi-Fujimoto disease and systemic lupus erythematosus share an obscure and complex relationship as systemic lupus erythematosus may occasionally precede,develop subsequently,or sometimes be associated concurrently with Kikuchi-Fujimoto disease.It is often mistaken for non-Hodgkin lymphoma while lupus lymphadenitis,cat-scratch disease,Sweet’s syndrome,Still’s disease,drug eruptions,infectious mononucleosis,and viral or tubercular lymphadenitis are other common differentials.Fine needle aspiration cytology mostly has features of nonspecific reactive lymphadenitis and immunohistochemistry studies usually show variable features of uncertain diagnostic value.Since its diagnosis is exclusively from histopathology,it needs to be evaluated more carefully;an early lymph node biopsy will obviate the need for unnecessary investigations and therapeutic trials.Its treatment with systemic corticosteroids,hydroxychloroquine,or antimicrobial agents mostly remains empirical.The article reviews clinicoepidemiological,diagnostic,and management aspects of KFD from the perspective of practicing clinicians.
文摘Primary histiocytic sarcoma of the spleen is a rare but potentially lethal condition. It can remain asymptomatic or only mildly symptomatic for a long time. An 81-year-old woman presented with an extremely enlarged spleen. She suffered from progressive anemia and required a red blood cell transfusion once a month. Although computed tomography, ultrasonography, and magnetic resonance imaging were performed for diagnosis, a confirmed diagnosis was not obtained. Her enlarged spleen compressed her stomach, and she suffered from gastritis and a sense of gastric fullness just after meals. She underwent laparoscopic splenectomy for therapeutic and diagnostic purposes. Her postoperative course was uneventful. After surgery, her red blood cell and platelet counts increased markedly. The tumor was diagnosed as splenic histiocytic sarcoma. Post-surgical chemotherapy was not performed, and the patient died of liver failure due to liver metastasis 5 mo after surgery. Laparoscopic splenectomy is minimally invasive and useful for the relief of symptoms related to hematological disorders. However, in cases of an enlarged spleen, optimal views and working space are limited. In such cases, splenic artery ligation can markedly reduce the size of the spleen, thus facilitating the procedure. The case reported herein suggests that laparoscopic splenectomy may be useful for the treatment of splenic malignancy.
文摘Kikuchi-Fujimoto disease(KFD), also known as histiocytic necrotizing lymphadenitis, is an uncommon condition, typically characterized by lymphadenopathy and fevers. It usually has a benign course; however, it may progress to fatality in extremely rare occasions. The diagnosis is made via lymph node biopsy and histopathology. Our patient was a young female who presented with shortness of breath, fever, and malaise. Physical examination revealed significant cervical and axillary lymphadenopathy. Chest X-ray displayed multilobar pneumonia. She required intubation and mechanical ventilation for progressive respiratory distress. Histopathology of lymph nodes demonstrated variable involvement of patchy areas of necrosis within the paracortex composed of karyorrhectic debris with abundant histiocytes consistent with KFD. After initial stabilization, the patient's condition quickly deteriorated with acute anemia, thrombocytopenia and elevated prothrombin time, partial prothrombin time, and D-dimer levels. Disseminated intravascular coagulopathy(DIC) ensued resulting in the patient's fatality. DIC in KFD is not well understood, but it is an important cause of mortality in patients with aggressive disease.
文摘BACKGROUND Granuloma annulare(GA)has diverse clinical manifestations including papules,plaques,and nodules on the extremities that are skin-colored,pink,or purple.Approximately 15%of all GA cases are considered generalized GA.CASE SUMMARY Herein,we describe the case of a pediatric patient who initially presented with papules and later developed generalized atrophic macules.Upon examination,two different morphologic lesions were histopathologically confirmed:Epithelioid nodular GA and scattered histiocytic infiltrative GA.This patient exhibited rare clinical manifestations that differed throughout the course of the disease.The varying histopathological types and clinical manifestations of GA may be linked to the different stages of the disease.CONCLUSION This rare case demonstrates the different histopathological features of different stages and clinical manifestations of granuloma annulare in an infant.
文摘Objective: As a rare cause of fever and lymphadenopathy (especially in the neck) in children, the purpose of this study is to explore the clinical characteristics of histiocytic necrotizing lymphadenitis (HNL) in children and improve the diagnosis and treatment of this disease. Methods: We retrospectively collected the data of 6 pediatric patients with HNL diagnosed by lymph node biopsy from 2012 to 2021 in department of pediatric, the affiliated Changsha Central Hospital, Hengyang Medical School, University of South China, including the clinical characteristics, laboratory examinations, imaging findings and histopathological examinations, treatment and follow-up of the patients and reviewed relevant literature. Results: All the patients were Han girls, with no kinship with each other, and four cases were from rural areas. The age of onset was from 7 to 14 years old, with durations ranged from 1 week to 3 months. All children had cervical lymphadenopathy with tenderness, five cases of fever, two cases of cough, one case of weight loss, four cases of leukopenia, one case of liver impairment, one case of combined bacterial infection of leukocytosis. All children underwent lymph node biopsy and were diagnosed with HNL, four children were treated with anti-infection before diagnosis, two children were treated with steroid therapy after diagnosis, and 1 case of weakened immune function was used immunoglobulin for modulating immunity. All children improved and discharged, and no recurrence was observed until October 2021. Conclusion: In our study, the children with swollen lymph nodes in the neck were accompanied by tenderness and fever as the main clinical manifestations, without specificity. Histopathology and immunohistochemical examination by removing the affected lymph nodes could confirm HNL. Most children had a good prognosis and long-term follow-up were required.
文摘Histiocytic sarcoma(HS)is a rare malignant neoplasm that originates from a histiocytic hematopoietic lineage characterized by histiocytic differentiation and its corresponding immunophenotypic features.We herein reported a case of primary HS of the stomach which was confirmed through histopathologic examination and immunohistochemical staining.A 52-year-old woman presented with progressive difficulty in feeding and dull pain in the epigastric region.Gastroscopy,endo-scopic ultrasonography,double contrast examination, and computed tomography revealed a mass located on the posterior wall of fundus and lesser curvature of the stomach.Microscopically,the cytoplasm of the tumor cells was abundant and eosinophilic.Immunohisto-chemical staining revealed that the tumor cells were positive for CD45RO and CD68.It is difficult to differentiate HS of stomach from other gastric malignancies by radiological evaluation alone.However,HS may be considered when a protruding and ulcerated mass in stomach shows heterogeneous hypervascular features.To the best of our knowledge,this is the first report in English language literature that emphasizes the imaging findings of human gastric HS.
基金supported (in whole or part) by HCA Healthcare and/or an HCA Healthcare affiliated entity
文摘BACKGROUND Histiocytic sarcoma(HS)of the spleen is reported to be a rare and lethal disease.The clinicopathological features of splenic HS have not been well described.The objective of this paper is to describe the diagnosis and treatment of a case of this rare disease and provide a review of the literature.CASE SUMMARY In this article,we discuss the case of a 40-year-old Hispanic female who presented with progressive thrombocytopenia and multiple hypoechoic lesions in the spleen without splenomegaly.Positron emission tomography-computed tomography showed increased activity in cervical lymph nodes,as well as multiple bone and splenic lesions with positive uptake.Two bone marrow biopsies and fine-needle aspiration of the cervical lymph node were inconclusive.Laparoscopic splenectomy was performed,and gross examination showed a 110.1 g spleen with multiple rubbery,nodular lesions within the subcapsular sinus and splenic parenchyma.The microscopic findings showed multinodular histiocyte proliferation with atypia and multilobulated nuclei,which were positive for CD163,CD4,and CD68 by immunohistochemical analysis.The final pathologic diagnosis was difficult and was found to be low-grade HS of the spleen,after consultations with two renowned hematopathology institutions.At the patient’s five-month follow-up visit,her bone marrow metastasis had progressed.She is waiting to be enrolled in a clinical trial.CONCLUSION Pathologic diagnosis of splenic HS can be challenging.Low-grade differentiation may be associated with a slow progressive disease.
文摘Histiocytic sarcoma is an extremely rare and very aggressive malignancy, with poor prognosis. The cases described in the literature are few and the treatment is not currently considered consensual. The clinical presentation is very variable. Its characterization is made based primarily on the histology. The authors present a case of an 82-year-old woman, with multiple adenopatic retroperitoneal and left iliac conglomerates, with left leg associated edema (extrinsic compression by conglomerates). After intensive study and approach ganglion biopsy, the histologic diagnosis revealed a histiocytic sarcoma.
文摘BACKGROUND Germ cell tumors(GCTs)account for 2%of human malignancies but are the most common malignant tumors among males aged 15-35.Since 1983,an association between mediastinal GCT(MGCT)and hematologic malignancies has been recognized.CASE SUMMARY We report a case in which malignant histiocytosis was associated with mediastinal GCTs.The clinical data of a male patient with MGCT admitted to Beijing Children's Hospital were collected retrospectively.The patient was first diagnosed according to imaging and pathological features as having MGCT,and was treated with surgery and chemotherapy.One year after stopping chemotherapy,imaging showed metastases in the right supraclavicular,mediastinum,hilar region and retroperitoneal lymph node,right pleura,right lung,and right para-cardiac margin.Pathological diagnosis of the liver nodular and hilar lymph nodes included systemic juvenile xanthogranuloma and Rosai-Dorfman lesions with malignant transformation(i.e.,morphological characteristics and immunophenotype of histiocytic sarcoma).Following diagnosis,the patient accepted chemotherapy with vindesine,cytarabine and dexamethasone.Positron emission tomography–computed tomography showed partial remission.The patient was followed-up for 10 mo after the diagnosis of malignant histiocytosis,and no sign of progression or relapse was observed.CONCLUSION Physicians should recognize the possibility of hematologic malignancies being associated with MGCT.Suitable sites should be selected for pathological examination.
基金Supported by the NSFC,No.81502696Technology Plan Program of Sichuan Province,No.2018JY0185+1 种基金Program of General Hospital of Western Theater,No.2021-XZYG-C3Science&Technology Department of Sichuan Province International Cooperation Program,No.2019YFH0056 and 2020YFSY0022。
文摘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.
文摘Histiocytic sarcoma is an uncommon non-Langerhans histiocyte disorder of mature tissue histiocytes.The authors presented an example of this rare tumor in a 14-year-old girl who presented with left upper quadrant pain,loss of appetite,and weight loss.A large 18 cm×10 cm heterogeneous solid and cystic enhancing mass was found in the left lobe of the liver.Based on the histomorphology and positivity for histiocyte-specific markers in a needle biopsy,a diagnosis of histiocytic sarcoma was made.Chemotherapy was initiated,but the tumor did not respond well,and she died about 7 weeks following initial diagnosis with multi-organ failure.At autopsy,the tumor showed extensive necrosis,with no evidence of metastatic spread.In conclusion,the diagnosis of histiocytic sarcoma is challenging,and requires a high index of suspicion,with an appropriate panel of confirmatory immunohistochemical stains.Recognition of this rare tumor is important because of its poor response to chemotherapy and high mortality.
文摘In this article,a case of histiocytic sarcoma(HS) arising from tonsil in a 60 year-old male patient was reported.Consideried a local disease,a regime consisting of 4 cycles of(CHOP) associated with radiotherapy was used.A positive result was gained after treatment.But HS is a rare hematopoietic neoplasm derived from hematopoietic monoblasts with aggressive characteristic and poor outcome.Stage of disease and tumor size are possibly associated with the prognosis.
文摘A 54-year-old man was admitted for the evaluation of fever and abodominal pain. Radiological and endo- scopic examination revealed a lung nodule and multiple small intestine uclers. Clinical diagnosis such as tuberculosis and Crohn＇s disease had been proposed. He developed intestine perforation after small bowel endoscopic procedure. During emergent surgery the involved intestinal segments were resected and a pathological diagnosis of fibroblastic histiocytic sarcoma （FBRC） was made. The patient died in the sixth month after the operation. The management of this cases highlig^t~ the ~rawback of pattern recogn^tio~ as the most commonly used clinical reasoning method, and the importance of histological investigation.
文摘Cytophagic histiocytic panniculitis （CHP） was first described by Winkelmann and Bowie in 1980.vj It is a rare group of diverse illnesses involving benign and malignant proliferation of macrophages in various organs and tissues. It presents with subcutaneous panniculitis with or without a hemophagocytic syndrome （HPS）. It occurs predominantly in women （male： female ratio 1：1.3） between the years of 5-61 （average, 33.5）. The major clinical features are recurrent fever, multiple panniculitic lesions, anemia, leukopenia and coagulation abnormalities. In the later phase, liver dysfuction, serosal effusion, mucosal ulceration and hemorrhage may occur. Histological findings show activated histiocyte infiltration of the fat tissue. Cytologically the benign-looking histiocytes containing cell fragments （bean-bag cells） are very typical. CHP has a broad spectrum from mild to severe. Benign CHP is selflimiting and sensitive to treatment, but up to now there is no effective therapy for malignant CHP. We report here a case of progressive and fatal cytophagic histiocytic panniculitis in a young woman who had encephaloclastic changes immediately prior to her death.
