BACKGROUD Follicular dendritic cell(FDC)sarcomas are rare neoplasms that occur predominantly in the lymph nodes.They can also occur extranodally.Extranodal FDC sarcomas most commonly present as solitary masses.Inflamm...BACKGROUD Follicular dendritic cell(FDC)sarcomas are rare neoplasms that occur predominantly in the lymph nodes.They can also occur extranodally.Extranodal FDC sarcomas most commonly present as solitary masses.Inflammatory pseudotumor(IPT)-like FDC sarcomas,a subcategory of FDC sarcomas,are rarer than other sarcoma subtypes.They are composed of spindle or ovoid neoplastic cells and exhibit an admixture of plasma cells and prominent lymphoplasmacytic infiltration.Paraneoplastic pemphigus(PNP),also known as paraneoplastic autoimmune multiorgan syndrome,is a rare autoimmune bullous disease that is associated with underlying neoplasms.PNP has a high mortality,and its early diagnosis is usually difficult.CASE SUMMARY We describe a 27-year-old woman who presented with stomatitis,conjunctivitis,and skin blisters and erosions as her first symptoms of PNP with an intraabdominal IPT-like FDC sarcoma.The patient underwent surgical tumor resection and received tapering oral corticosteroid treatment.She showed no recurrence at the 1-year follow-up.CONCLUSION IPT-like FDC sarcomas are rare underlying neoplasms that have an uncommon association with PNP.PNP-associated FDC sarcomas predominantly occur in intra-abdominal sites and suggest a poor prognosis.Surgical resection is an essential and effective treatment for PNP and primary and recurrent FDC sarcomas.展开更多
BACKGROUND Follicular dendritic cell(FDC)sarcoma/tumor is a rare malignant tumor of follicular dendritic cells,which is considered a low-grade sarcoma that can involve lymph nodes or extranodal sites.Conventional FDC ...BACKGROUND Follicular dendritic cell(FDC)sarcoma/tumor is a rare malignant tumor of follicular dendritic cells,which is considered a low-grade sarcoma that can involve lymph nodes or extranodal sites.Conventional FDC sarcomas are negative for Epstein-Barr virus(EBV),whereas the inflammatory pseudotumorlike variant consistently shows EBV in the neoplastic cells.CASE SUMMARY We report two cases of inflammatory pseudotumor-like FDC sarcoma in the liver that received 3D laparoscopic right hepatectomy and open right hepatectomy separately.CONCLUSION EBV probe-based in situ hybridization and detection of immunohistochemical markers of FDC play an important role in the diagnosis and differential diagnosis of inflammatory pseudotumor-like FDC sarcoma.Complete surgical excision combined with regional lymphadenectomy may be effective in reducing the postoperative recurrence and metastasis and improving long-term survival rates.展开更多
Inflammatory pseudotumor(IPT)-like follicular dendritic cell(FDC)sarcoma is rare.The 2017 World Health Organization classification of tumors of hematopoietic and lymphoid tissues noted that data on its clinical outcom...Inflammatory pseudotumor(IPT)-like follicular dendritic cell(FDC)sarcoma is rare.The 2017 World Health Organization classification of tumors of hematopoietic and lymphoid tissues noted that data on its clinical outcome are limited,but that the tumor appears to be indolent.The aim of this study was to summarize the clinical characteristics,treatment outcomes,and prognostic factors for IPT-like FDC sarcoma.A literature review was conducted on retrospective analyses of clinical data and prognostic information on IPT-like FDC sarcoma reported between 2001 and 2020.A total of 67 cases of IPT-like FDC sarcoma were retrieved from the literature,documenting that it occurs predominantly in middle-aged adults,with a marked female predilection.Six patients had a separate malignancy and five had an autoimmune disease.Typically involving the spleen and/or liver,it may also selectively involve the abdomen,gastrointestinal tract,pancreas,retroperitoneum,and mesentery.Necrosis,hemorrhage,noncaseating epithelioid granulomas,and fibrinoid deposits in blood vessel walls are often present.The neoplastic cells are predominantly positive for follicular dendritic cell markers such as cluster of differentiation 21(CD21),CD23,CD35 and CNA.42 and are consistently Epstein-Barr virus(EBV)-positive.Mitoses were very rare in most cases.Most patients were treated by surgery alone.Disease status at the time of last follow-up was known for 57 patients with follow-up time ranging from 2 to 144 mo.Local and/or distant recurrence after initial treatment was seen in 15.8%of the patients.The 1-and 5-year progression-free survival for the entire group was 91.5%and 56.1%,respectively.Kaplan-Meier and multivariate analyses showed that age,sex,tumor size,and pathological features were not risk factors for disease progression.IPT-like FDC sarcoma appears to be mildly aggressive and requires annual surveillance.Surgery is the most effective treatment modality,and the role of adjuvant chemotherapy for postoperative management is unclear.EBV is likely to play an important role in the etiology of IPT-like FDC sarcoma.展开更多
BACKGROUND Inflammatory pseudotumor-like follicular dendritic cell sarcoma(IPT-like FDCS)is rare with a low malignant potential.Hepatic IPT-like FDCS has similar clinical features to hepatocellular carcinoma(HCC),maki...BACKGROUND Inflammatory pseudotumor-like follicular dendritic cell sarcoma(IPT-like FDCS)is rare with a low malignant potential.Hepatic IPT-like FDCS has similar clinical features to hepatocellular carcinoma(HCC),making it extremely difficult to distinguish between them in clinical practice.We describe the case of a young female patient diagnosed with HCC before surgery,which was pathologically diagnosed as IPT-like FDCS after the left half of the liver was resected.During 6 mo of follow-up,the patient recovered well with no signs of recurrence or metastasis.CASE SUMMARY A 23-year-old female patient with a 2-year history of hepatitis B presented to the Affiliated Hospital of Guizhou Medical University.She was asymptomatic at presentation,and the findings from routine laboratory examinations were normal except for slightly elevated alpha-fetoprotein levels.However,ultrasonography revealed a 3-cm diameter mass in the left hepatic lobe,and abdominal contrastenhanced computed tomography revealed that the tumor had asymmetrical enhancement during the arterial phase,which declined during the portal venous phase,and had a pseudo-capsule appearance.Based on the findings from clinical assessments and imaging,the patient was diagnosed with HCC,for which she was hospitalized and had undergone laparoscopic left hepatectomy.However,the tumor specimens submitted for pathological analyses revealed IPT-like FDCS.After surgical removal of the tumor,the patient recovered.In addition,the patient continued to recover well during 6 mo of follow-up.CONCLUSION Hepatic IPT-like FDCS is difficult to distinguish from HCC.Hepatectomy may provide beneficial outcomes in non-metastatic hepatic IPT-like FDCS.展开更多
BACKGROUND Inflammatory pseudotumor-like follicular dendritic cell(IPT-like FDC)tumors of the liver is an uncommon tumor with extremely low incidence.To date,the radiologic findings of this tumor in multiphase compute...BACKGROUND Inflammatory pseudotumor-like follicular dendritic cell(IPT-like FDC)tumors of the liver is an uncommon tumor with extremely low incidence.To date,the radiologic findings of this tumor in multiphase computed tomography(CT)and magnetic resonance imaging(MRI)imaging have not been described.CASE SUMMARY Patient 1 is a 31-year-old Chinese female,whose complaining incidentally coincided with the finding of multiple liver masses.In the local hospital,an abdominal enhanced CT found two hypo-dense solid lesions,with heterogeneous sustained hypoenhancement,in the upper segment of the liver’s right posterior lobe.In our hospital,enhanced magnetic resonance imaging(MRI)with hepatocyte-specific contrast agents showed a similar enhanced pattern of lesions with patchy hyperintensity in the hepatobiliary phase(HBP).The patient underwent surgery and recovered well.The final pathology confirmed an IPTlike FDC tumor.No recurrence was found on the regular re-examination.Patient 2 is a 48-year-old Chinese male admitted to our hospital for a huge unexpected hepatic lesion.A dynamic enhanced abdominal CT revealed a huge heterogeneous enhanced solid tumor in the right lobe of the liver with a size of 100 mm×80 mm,which showed a heterogeneous sustained hypoenhancement.In addition,enlarged lymph nodes were found in the hilum of the liver.This patient underwent a hepatic lobectomy and lymph node dissection.The final pathology confirmed an IPT-like FDC tumor.No recurrence was found upon regular re-examination.CONCLUSION When a hepatic tumor shows heterogeneous sustained hypoenhancement with a patchy enhancement during HBP,an IPT-like FDC tumor should be considered in the differential diagnosis.展开更多
AIM: To establish a model for prognosis assessment of extranodal follicular dendritic cell (FDC) sarcoma.METHODS: Nine lesions were examined by routine and molecular approaches.Clinicopathological factors from the new...AIM: To establish a model for prognosis assessment of extranodal follicular dendritic cell (FDC) sarcoma.METHODS: Nine lesions were examined by routine and molecular approaches.Clinicopathological factors from the new cases and 97 reported cases were analyzed for their prognostic values.RESULTS: The current lesions were found in f ive male and four female patients,located mainly in the head and neck area and averaging 7.2 cm in size.Six patients had recurrence or metastasis and three remained free of disease.The 106 patients (male/female ratio,1.1:1) were aged from 9 to 82 years (median,44 years).The tumor sizes ranged from 1.5 to 21 cm (mean,7.4 cm).Abdominal/pelvic region was affected most frequently (43%).Surgical resection was performed in 100 patients,followed by radiation and/or chemotherapy in 35 of them.Follow-up data were available in 91 cases,covering a period of 3-324 mo (mean,27 mo;median,19 mo).Of the informative cases,38 (42%) had recurrence or metastasis,and 12 (13%) died of the disease.These tumors were classif ied histologically into lowand high-grade lesions.A size ≥ 5 cm (P = 0.003),highgrade histology (P = 0.046) and a mitotic count ≥ 5/10 HPF (P = 0.013) were associated with tumor recurrence.The lesions were def ined as low-,intermediateand high-risk tumors,and their recurrence rates were 16%,46% and 73%,and their mortality rates 0%,4% and 45%,respectively.CONCLUSION: Extranodal FDC tumors behave like soft tissue sarcomas.Their clinical outcomes are variable and can be evaluated according to their sizes and grades.展开更多
BACKGROUND:Hepatic follicular dendritic cell (FDC) sarcoma is an extremely rare neoplasm.Most commonly,FDC sarcoma presents as a solitary mass in lymph nodes,however,several extra-nodal locations have been identified....BACKGROUND:Hepatic follicular dendritic cell (FDC) sarcoma is an extremely rare neoplasm.Most commonly,FDC sarcoma presents as a solitary mass in lymph nodes,however,several extra-nodal locations have been identified.METHODS:We report a case of a 53-year-old female who presented with symptoms of abdominal pain,fever,anemia,and jaundice.After an extensive review of the literature,we have found only 12 cases of hepatic FDC sarcoma.RESULTS:The tumor was 11.5 cm in diameter and composed of spindle and epithelioid cells with ovoid nuclei and associated with mixed inflammatory infiltrate.Immunohistochemical stains were positive for CD35 and CD21.The patient underwent a left hepatic lobectomy.CONCLUSIONS:Liver follicular dendritic cell sarcoma is a very rare tumor.Most cases present with abdominal pain and weight loss,and most of them can be managed by hepatic resection with excellent short-term outcomes.展开更多
BACKGROUND Follicular dendritic cell sarcoma(FDCS) is an uncommon type of tumor with low incidence. To date,no standard treatment for the disease has been established.Surgery remains the main treatment. Adjuvant chemo...BACKGROUND Follicular dendritic cell sarcoma(FDCS) is an uncommon type of tumor with low incidence. To date,no standard treatment for the disease has been established.Surgery remains the main treatment. Adjuvant chemotherapy and radiotherapy are optional approaches. Metastatic cases require multidisciplinary collaborative treatments. However,the choice of chemotherapeutic drugs is controversial.CASE SUMMARY A 66-year-old Chinese woman presented to our hospital complaining of intermittent pain of right upper quadrant. An enhanced computed tomography(CT) scan of the abdomen revealed hepatocellular carcinoma. Subsequently,the patient underwent a radical partial hepatectomy. Primary FDCS of the liver was diagnosed pathologically. Except for regular follow-up examinations,the patient did not receive adjuvant chemotherapy or radiotherapy. However,fluorine-18-fluorodeoxyglucose positron emission tomography/CT(PET/CT) confirmed lymph node metastases in the space of ligamentum hepatogastricum and pancreatic head,as well as the portacaval space. The patient was given systemic chemotherapy with gemcitabine and docetaxel for she was unsuitable for surgery. Satisfactorily,the metastatic lymph nodes were significantly reduced to clinical complete remission after eight cycles of chemotherapy. Then,strengthened radiotherapy was followed when the patient rejected the opportunity of surgery. Eventually,the carcinoma got better control and the patient was free of progression.CONCLUSION This case highlights the importance of making suitable chemotherapy regimens for the rare tumor. The combination of gemcitabine,docetaxel,and consolidated radiotherapy may offer a new promising option for the treatment of metastatic hepatic FDCS in the future.展开更多
The most common organ where follicular dendritic cell sarcoma(FDCS) occurs is in cervical lymph nodes, while few cases are found in extranodal organs such as liver, spleen, and soft tissue. This is a case report that ...The most common organ where follicular dendritic cell sarcoma(FDCS) occurs is in cervical lymph nodes, while few cases are found in extranodal organs such as liver, spleen, and soft tissue. This is a case report that FDCS occurs in the hepatogastric ligament. To our knowledge, there is no such case that has been reported previously. A 47-year-old male patient was found to have an intraabdominal mass during an annual physical examination. Computed tomography showed a 4.2 cm × 4.1 cm mass located at the lesser curvature of the stomach, above the pancreas. During operation, a tumor mass was found in the hepatogastric ligament and a radical resection was performed. The tumor was diagnosed as FDCS by pathology and immunohistochemical testing. The patient had a favorable recovery, and no obvious abnormality was found 3 months postoperation.展开更多
Follicular dendritic cell (FDC) is an essential component of the nonlymphoid, nonphagocytic immunoaccessory reticulum cells of the peripheral lymphoid tissue(1). FDCs are confined largely to the primary and secondary ...Follicular dendritic cell (FDC) is an essential component of the nonlymphoid, nonphagocytic immunoaccessory reticulum cells of the peripheral lymphoid tissue(1). FDCs are confined largely to the primary and secondary B-cell follicles, where they form a tight interlacing meshwork. They play a role in the capture and presentation of antigens, generation and regulation of immune complexes. FDCs can be recognized morphologically by their indistinct cellular borders, pale eosinophilic cytoplasm, round-to-ovoid nuclei with delicate nuclear membranes and clear-to-vesicular chromatin with inconspicuous or small nucleoli. FDCs are best identified through immunostaining using CD21, CD35, R4/23, KiM4, KiM4p and Ki-FDC1p. A proliferation of FDCs may be seen in a variety of lesions, including reactive follicular. hyperplasia, Castleman's disease, follicular lymphoma, mantle cell lymphoma, angioimmunoblastic T-cell lymphoma and nodular lymphocyte predominant Hodgkin's lymphoma. Previous studies(2-5) showed that follicular dendritic cell sarcoma (FDCS) affected predominantly the lymph nodes, with occasional extranodal involvement. As the tumor consists mainly ovoid-to-spindle cells, it is not uncommon to misdiagnose the lesion as a wide variety of other spindle cell sarcomas. This study focused on characterizing the histologic features of extranodal FDCS to promote the recognition of this rare entity for both clinicians and pathologists.展开更多
Follicular dendritic cell sarcoma (FDCS) is a rare spindle cell neoplasm usually identified as lymph node-based neoplasm that occasionally occurs in extranodal regions. It was first characterized by Monda et al in 1...Follicular dendritic cell sarcoma (FDCS) is a rare spindle cell neoplasm usually identified as lymph node-based neoplasm that occasionally occurs in extranodal regions. It was first characterized by Monda et al in 1986. In the present report, we describe the first case of FDCS arising from the appendix.展开更多
文摘BACKGROUD Follicular dendritic cell(FDC)sarcomas are rare neoplasms that occur predominantly in the lymph nodes.They can also occur extranodally.Extranodal FDC sarcomas most commonly present as solitary masses.Inflammatory pseudotumor(IPT)-like FDC sarcomas,a subcategory of FDC sarcomas,are rarer than other sarcoma subtypes.They are composed of spindle or ovoid neoplastic cells and exhibit an admixture of plasma cells and prominent lymphoplasmacytic infiltration.Paraneoplastic pemphigus(PNP),also known as paraneoplastic autoimmune multiorgan syndrome,is a rare autoimmune bullous disease that is associated with underlying neoplasms.PNP has a high mortality,and its early diagnosis is usually difficult.CASE SUMMARY We describe a 27-year-old woman who presented with stomatitis,conjunctivitis,and skin blisters and erosions as her first symptoms of PNP with an intraabdominal IPT-like FDC sarcoma.The patient underwent surgical tumor resection and received tapering oral corticosteroid treatment.She showed no recurrence at the 1-year follow-up.CONCLUSION IPT-like FDC sarcomas are rare underlying neoplasms that have an uncommon association with PNP.PNP-associated FDC sarcomas predominantly occur in intra-abdominal sites and suggest a poor prognosis.Surgical resection is an essential and effective treatment for PNP and primary and recurrent FDC sarcomas.
文摘BACKGROUND Follicular dendritic cell(FDC)sarcoma/tumor is a rare malignant tumor of follicular dendritic cells,which is considered a low-grade sarcoma that can involve lymph nodes or extranodal sites.Conventional FDC sarcomas are negative for Epstein-Barr virus(EBV),whereas the inflammatory pseudotumorlike variant consistently shows EBV in the neoplastic cells.CASE SUMMARY We report two cases of inflammatory pseudotumor-like FDC sarcoma in the liver that received 3D laparoscopic right hepatectomy and open right hepatectomy separately.CONCLUSION EBV probe-based in situ hybridization and detection of immunohistochemical markers of FDC play an important role in the diagnosis and differential diagnosis of inflammatory pseudotumor-like FDC sarcoma.Complete surgical excision combined with regional lymphadenectomy may be effective in reducing the postoperative recurrence and metastasis and improving long-term survival rates.
基金Supported by National Natural Science Foundation of China,No.81570509.
文摘Inflammatory pseudotumor(IPT)-like follicular dendritic cell(FDC)sarcoma is rare.The 2017 World Health Organization classification of tumors of hematopoietic and lymphoid tissues noted that data on its clinical outcome are limited,but that the tumor appears to be indolent.The aim of this study was to summarize the clinical characteristics,treatment outcomes,and prognostic factors for IPT-like FDC sarcoma.A literature review was conducted on retrospective analyses of clinical data and prognostic information on IPT-like FDC sarcoma reported between 2001 and 2020.A total of 67 cases of IPT-like FDC sarcoma were retrieved from the literature,documenting that it occurs predominantly in middle-aged adults,with a marked female predilection.Six patients had a separate malignancy and five had an autoimmune disease.Typically involving the spleen and/or liver,it may also selectively involve the abdomen,gastrointestinal tract,pancreas,retroperitoneum,and mesentery.Necrosis,hemorrhage,noncaseating epithelioid granulomas,and fibrinoid deposits in blood vessel walls are often present.The neoplastic cells are predominantly positive for follicular dendritic cell markers such as cluster of differentiation 21(CD21),CD23,CD35 and CNA.42 and are consistently Epstein-Barr virus(EBV)-positive.Mitoses were very rare in most cases.Most patients were treated by surgery alone.Disease status at the time of last follow-up was known for 57 patients with follow-up time ranging from 2 to 144 mo.Local and/or distant recurrence after initial treatment was seen in 15.8%of the patients.The 1-and 5-year progression-free survival for the entire group was 91.5%and 56.1%,respectively.Kaplan-Meier and multivariate analyses showed that age,sex,tumor size,and pathological features were not risk factors for disease progression.IPT-like FDC sarcoma appears to be mildly aggressive and requires annual surveillance.Surgery is the most effective treatment modality,and the role of adjuvant chemotherapy for postoperative management is unclear.EBV is likely to play an important role in the etiology of IPT-like FDC sarcoma.
基金Supported by Guizhou Medical UniversityAffiliated Hospital of Guizhou Medical University
文摘BACKGROUND Inflammatory pseudotumor-like follicular dendritic cell sarcoma(IPT-like FDCS)is rare with a low malignant potential.Hepatic IPT-like FDCS has similar clinical features to hepatocellular carcinoma(HCC),making it extremely difficult to distinguish between them in clinical practice.We describe the case of a young female patient diagnosed with HCC before surgery,which was pathologically diagnosed as IPT-like FDCS after the left half of the liver was resected.During 6 mo of follow-up,the patient recovered well with no signs of recurrence or metastasis.CASE SUMMARY A 23-year-old female patient with a 2-year history of hepatitis B presented to the Affiliated Hospital of Guizhou Medical University.She was asymptomatic at presentation,and the findings from routine laboratory examinations were normal except for slightly elevated alpha-fetoprotein levels.However,ultrasonography revealed a 3-cm diameter mass in the left hepatic lobe,and abdominal contrastenhanced computed tomography revealed that the tumor had asymmetrical enhancement during the arterial phase,which declined during the portal venous phase,and had a pseudo-capsule appearance.Based on the findings from clinical assessments and imaging,the patient was diagnosed with HCC,for which she was hospitalized and had undergone laparoscopic left hepatectomy.However,the tumor specimens submitted for pathological analyses revealed IPT-like FDCS.After surgical removal of the tumor,the patient recovered.In addition,the patient continued to recover well during 6 mo of follow-up.CONCLUSION Hepatic IPT-like FDCS is difficult to distinguish from HCC.Hepatectomy may provide beneficial outcomes in non-metastatic hepatic IPT-like FDCS.
文摘BACKGROUND Inflammatory pseudotumor-like follicular dendritic cell(IPT-like FDC)tumors of the liver is an uncommon tumor with extremely low incidence.To date,the radiologic findings of this tumor in multiphase computed tomography(CT)and magnetic resonance imaging(MRI)imaging have not been described.CASE SUMMARY Patient 1 is a 31-year-old Chinese female,whose complaining incidentally coincided with the finding of multiple liver masses.In the local hospital,an abdominal enhanced CT found two hypo-dense solid lesions,with heterogeneous sustained hypoenhancement,in the upper segment of the liver’s right posterior lobe.In our hospital,enhanced magnetic resonance imaging(MRI)with hepatocyte-specific contrast agents showed a similar enhanced pattern of lesions with patchy hyperintensity in the hepatobiliary phase(HBP).The patient underwent surgery and recovered well.The final pathology confirmed an IPTlike FDC tumor.No recurrence was found on the regular re-examination.Patient 2 is a 48-year-old Chinese male admitted to our hospital for a huge unexpected hepatic lesion.A dynamic enhanced abdominal CT revealed a huge heterogeneous enhanced solid tumor in the right lobe of the liver with a size of 100 mm×80 mm,which showed a heterogeneous sustained hypoenhancement.In addition,enlarged lymph nodes were found in the hilum of the liver.This patient underwent a hepatic lobectomy and lymph node dissection.The final pathology confirmed an IPT-like FDC tumor.No recurrence was found upon regular re-examination.CONCLUSION When a hepatic tumor shows heterogeneous sustained hypoenhancement with a patchy enhancement during HBP,an IPT-like FDC tumor should be considered in the differential diagnosis.
基金Supported by Grants from National Natural Science Foundation of China,No.30171052,30572125 and 30772508
文摘AIM: To establish a model for prognosis assessment of extranodal follicular dendritic cell (FDC) sarcoma.METHODS: Nine lesions were examined by routine and molecular approaches.Clinicopathological factors from the new cases and 97 reported cases were analyzed for their prognostic values.RESULTS: The current lesions were found in f ive male and four female patients,located mainly in the head and neck area and averaging 7.2 cm in size.Six patients had recurrence or metastasis and three remained free of disease.The 106 patients (male/female ratio,1.1:1) were aged from 9 to 82 years (median,44 years).The tumor sizes ranged from 1.5 to 21 cm (mean,7.4 cm).Abdominal/pelvic region was affected most frequently (43%).Surgical resection was performed in 100 patients,followed by radiation and/or chemotherapy in 35 of them.Follow-up data were available in 91 cases,covering a period of 3-324 mo (mean,27 mo;median,19 mo).Of the informative cases,38 (42%) had recurrence or metastasis,and 12 (13%) died of the disease.These tumors were classif ied histologically into lowand high-grade lesions.A size ≥ 5 cm (P = 0.003),highgrade histology (P = 0.046) and a mitotic count ≥ 5/10 HPF (P = 0.013) were associated with tumor recurrence.The lesions were def ined as low-,intermediateand high-risk tumors,and their recurrence rates were 16%,46% and 73%,and their mortality rates 0%,4% and 45%,respectively.CONCLUSION: Extranodal FDC tumors behave like soft tissue sarcomas.Their clinical outcomes are variable and can be evaluated according to their sizes and grades.
文摘BACKGROUND:Hepatic follicular dendritic cell (FDC) sarcoma is an extremely rare neoplasm.Most commonly,FDC sarcoma presents as a solitary mass in lymph nodes,however,several extra-nodal locations have been identified.METHODS:We report a case of a 53-year-old female who presented with symptoms of abdominal pain,fever,anemia,and jaundice.After an extensive review of the literature,we have found only 12 cases of hepatic FDC sarcoma.RESULTS:The tumor was 11.5 cm in diameter and composed of spindle and epithelioid cells with ovoid nuclei and associated with mixed inflammatory infiltrate.Immunohistochemical stains were positive for CD35 and CD21.The patient underwent a left hepatic lobectomy.CONCLUSIONS:Liver follicular dendritic cell sarcoma is a very rare tumor.Most cases present with abdominal pain and weight loss,and most of them can be managed by hepatic resection with excellent short-term outcomes.
基金Supported by National Natural Science Foundation of China,No.81572850
文摘BACKGROUND Follicular dendritic cell sarcoma(FDCS) is an uncommon type of tumor with low incidence. To date,no standard treatment for the disease has been established.Surgery remains the main treatment. Adjuvant chemotherapy and radiotherapy are optional approaches. Metastatic cases require multidisciplinary collaborative treatments. However,the choice of chemotherapeutic drugs is controversial.CASE SUMMARY A 66-year-old Chinese woman presented to our hospital complaining of intermittent pain of right upper quadrant. An enhanced computed tomography(CT) scan of the abdomen revealed hepatocellular carcinoma. Subsequently,the patient underwent a radical partial hepatectomy. Primary FDCS of the liver was diagnosed pathologically. Except for regular follow-up examinations,the patient did not receive adjuvant chemotherapy or radiotherapy. However,fluorine-18-fluorodeoxyglucose positron emission tomography/CT(PET/CT) confirmed lymph node metastases in the space of ligamentum hepatogastricum and pancreatic head,as well as the portacaval space. The patient was given systemic chemotherapy with gemcitabine and docetaxel for she was unsuitable for surgery. Satisfactorily,the metastatic lymph nodes were significantly reduced to clinical complete remission after eight cycles of chemotherapy. Then,strengthened radiotherapy was followed when the patient rejected the opportunity of surgery. Eventually,the carcinoma got better control and the patient was free of progression.CONCLUSION This case highlights the importance of making suitable chemotherapy regimens for the rare tumor. The combination of gemcitabine,docetaxel,and consolidated radiotherapy may offer a new promising option for the treatment of metastatic hepatic FDCS in the future.
文摘The most common organ where follicular dendritic cell sarcoma(FDCS) occurs is in cervical lymph nodes, while few cases are found in extranodal organs such as liver, spleen, and soft tissue. This is a case report that FDCS occurs in the hepatogastric ligament. To our knowledge, there is no such case that has been reported previously. A 47-year-old male patient was found to have an intraabdominal mass during an annual physical examination. Computed tomography showed a 4.2 cm × 4.1 cm mass located at the lesser curvature of the stomach, above the pancreas. During operation, a tumor mass was found in the hepatogastric ligament and a radical resection was performed. The tumor was diagnosed as FDCS by pathology and immunohistochemical testing. The patient had a favorable recovery, and no obvious abnormality was found 3 months postoperation.
文摘Follicular dendritic cell (FDC) is an essential component of the nonlymphoid, nonphagocytic immunoaccessory reticulum cells of the peripheral lymphoid tissue(1). FDCs are confined largely to the primary and secondary B-cell follicles, where they form a tight interlacing meshwork. They play a role in the capture and presentation of antigens, generation and regulation of immune complexes. FDCs can be recognized morphologically by their indistinct cellular borders, pale eosinophilic cytoplasm, round-to-ovoid nuclei with delicate nuclear membranes and clear-to-vesicular chromatin with inconspicuous or small nucleoli. FDCs are best identified through immunostaining using CD21, CD35, R4/23, KiM4, KiM4p and Ki-FDC1p. A proliferation of FDCs may be seen in a variety of lesions, including reactive follicular. hyperplasia, Castleman's disease, follicular lymphoma, mantle cell lymphoma, angioimmunoblastic T-cell lymphoma and nodular lymphocyte predominant Hodgkin's lymphoma. Previous studies(2-5) showed that follicular dendritic cell sarcoma (FDCS) affected predominantly the lymph nodes, with occasional extranodal involvement. As the tumor consists mainly ovoid-to-spindle cells, it is not uncommon to misdiagnose the lesion as a wide variety of other spindle cell sarcomas. This study focused on characterizing the histologic features of extranodal FDCS to promote the recognition of this rare entity for both clinicians and pathologists.
文摘Follicular dendritic cell sarcoma (FDCS) is a rare spindle cell neoplasm usually identified as lymph node-based neoplasm that occasionally occurs in extranodal regions. It was first characterized by Monda et al in 1986. In the present report, we describe the first case of FDCS arising from the appendix.