1.Rare metastatic primary intra-abdominal follicular dendritic cell sarcoma poorly responsive to chemotherapy: A case report
Leah Anne E. Legaspi ; Mary Ondinee-Igot ; Gracieux Fernando
Philippine Journal of Internal Medicine 2024;62(1):308-313
Background:
Follicular dendritic cell sarcoma (FDCS) accounts for about 0.4% of soft tissue sarcomas. Approximately onethird of cases occur in extranodal sites and about 28% of extranodal FDCS may metastasize. Intra-abdominal occurrence is
rare and there is limited published data to guide oncologists on how to best treat this malignancy.
Case Presentation:
This is a case of a 33-year-old female who came in due to incidental finding of a left supraclavicular
mass with 2-year history of early satiety. Neck node biopsy revealed a poorly differentiated malignant tumor with positive
staining for CD21, CD23, vimentin and S100 consistent with FDCS. PET-CT revealed an intensely FDG-avid large mass in
the left upper abdomen with signs of necrosis and mass effect. The patient was given three different chemotherapy
regimens that included (1) gemcitabine/docetaxel, (2) single agent doxorubicin and (3) ifosfamide/etoposide, but she
progressed on all these. Off-label use of bendamustine was then offered and after just the first cycle, the patient reportedly
regained strength and was able to get up from wheelchair with noted interval decrease in size of the cervical mass.
Unfortunately, the patient deteriorated and succumbed to infection and multiple pulmonary embolisms.
Conclusion
Intra-abdominal FDCS is a rare malignancy with heterogenous outcomes with no uniform treatment strategy
at present. Molecular tumor board discussion and multi-disciplinary approach in extranodal FDCS is important in the
diagnosis and management. Patients with multiple poor prognostic factors are at risk for tumor recurrence, metastasis, and
death.
Dendritic Cell Sarcoma, Follicular
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Abdominal Neoplasms
;
Drug Therapy
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Bendamustine Hydrochloride
;
Prognosis
2.MOR106 alleviates inflammation in mice with atopic dermatitis by blocking the JAK2/STAT3 signaling pathway and inhibiting IL-17C-mediated Tfh cell differentiation.
Limin TIAN ; Xiaohui HUYAN ; Sen YANG ; Mengjie WANG ; Yuenan YANG
Chinese Journal of Cellular and Molecular Immunology 2024;40(1):26-32
Objective To explore the significance of interleukin-17C(IL-17C)-mediated follicular helper T cell (Tfh) differentiation in atopic dermatitis (AD) model. Methods BALB/c mice were divided into control group, AD model group, low-dose MOR106 (anti-IL-17C huIgG1)(MDR106-L)treatment group and high-dose MOR106 (MOR106-H) treatment group, 8 mice in each group. Except for the control group, all the other groups were treated with 2, 4- dinitrochlorobenzene (DNCB) to establish AD models. The low-dose and high-dose MOR106 groups were treated with 5 mg/kg or 10 mg/kg MOR106 respectively. The differentiation of Tfh cell subsets in peripheral blood of mice was analyzed by flow cytometry, and the expression of Janus kinase 2/signal transducer and activator of transcription 3(JAK2/STAT3) signal pathway protein in skin tissue was detected by Western blot analysis. Results Compared with the control group, the dermatitis severity score, mass difference between two ears, spleen mass and spleen index of DNCB group increased significantly, while those of MOR106-L group and MOR106-H group decreased significantly. Compared with the control group, the Tfh subgroup of AD mice showed deregulated differentiation, resulting in a significant increase in the percentage of CD4+CXCR5+IFN-γ+Tfh1 cells, CD4+CXCR5+IL-17A+Tfh17 and CD4+CXCR5+IL-21+Tfh21 cells, and a significant decrease in the percentage of CD4+CXCR5+IL-10+Tfh10 cells and CD4+CXCR5+FOXP3+Tfr cells in peripheral blood. The protein levels of phosphorylated JAK2(p-JAK2) and p-STAT3 were significantly increased. MOR106 effectively reversed these changes of Tfh1, Tfh10, Tfh17, Tfh21 and Tfr cells in peripheral blood of AD mice. Compared with AD group, the levels of p-JAK2 and p-STAT3 protein in low-dose and high-dose MOR106 treatment groups decreased significantly. Conclusion MOR106 can reduce the inflammatory response of AD mice by blocking JAK2/STAT3 signaling pathway and inhibiting the differentiation of Tfh cells mediated by IL-17C.
Animals
;
Mice
;
Dermatitis, Atopic/drug therapy*
;
Interleukin-17
;
T Follicular Helper Cells
;
Janus Kinase 2
;
Dinitrochlorobenzene
;
Inflammation
;
Cell Differentiation
;
Signal Transduction
3.Synchronous primary head and neck tumors: Follicular thyroid carcinoma and squamous cell carcinoma of the tonsil
Princess B. Maristela ; Emmanuel Tadeus S. Cruz
Philippine Journal of Otolaryngology Head and Neck Surgery 2024;39(1):49-52
Simultaneously occurring malignancies may be detected in different organs or tissues at any given time. Patients diagnosed with a tumor may be found to have another tumor or second primary cancer. Second primary cancers (SPCs) may be further classified as synchronous or metachronous. Synchronous SPCs are lesions detected simultaneously or within 6 months after the diagnosis of the primary tumor while metachronous SPCs are tumors diagnosed 6 months after primary tumor diagnosis.1There is an increased risk of having second primary cancer in Head and Neck Squamous Cell Carcinoma (HNSCC) patients.1 In a study by Strojan et al. in 2013, among 2,106 head and neck cancer patients, 2.4% developed synchronous second primary cancers.2 A systematic review by Coco-Pelaz et al. in 2020, showed that second primary tumors most frequently occur in the head and neck area followed by the lungs and esophagus.3We present a case of follicular thyroid carcinoma with an incidental finding of cervical lymph-node metastatic squamous cell carcinoma from the tonsil and discuss the clinical presentation, ancillary procedures and management.
Adenocarcinoma, Follicular
;
Positron-Emission Tomography
4.Aggressive synchronous papillary and likely follicular thyroid carcinomas in a patient with Graves’ disease
Gerald Sng Gui Ren ; Sarah Tan Ying Tse ; Edwin Chew Jun Chen ; Sangeeta Mantoo ; Chng Chiaw Ling
Journal of the ASEAN Federation of Endocrine Societies 2024;39(2):119-123
We report a case of an uncommonly aggressive presentation of the rare entity of synchronous papillary (PTC) and follicular thyroid carcinomas (FTC) in a 67-year-old woman initially presenting with thyrotoxicosis from Graves’ disease. She was found to have two thyroid nodules with extensive intra-cardiac tumour thrombus, symptomatic left pelvis bony metastasis with pathological fracture, pulmonary metastases and mediastinal lymph node metastases. Further investigations suggested a diagnosis of synchronous papillary and metastatic follicular thyroid cancer. Treatment with radical surgery followed by adjuvant therapeutic radioiodine ablation was proposed, but the patient declined all forms of cancer-specific therapy and was elected solely for a palliative approach to treatment. We discuss the diagnostic considerations in arriving at the diagnosis of synchronous thyroid malignancy – in this case the clear features of PTC and the strong probability of FTC due to invasiveness and metastatic follicular lesions. This case underscores potential limitations of the ACR TI-RADS system, notably with certain ultrasonographic features suggesting malignancy that might not be adequately captured. Notably, the aggressive presentation of DTC in this case may be contributed by the concurrent presence of Graves’ Disease, suggesting heightened vigilance when assessing potential thyroid malignancies in such patients.
Papillary Thyroid Carcinoma
;
Thyroid Cancer, Papillary
;
Follicular Thyroid Carcinoma
;
Adenocarcinoma, Follicular
;
Graves Disease
5.Mechanism of programmed cell death in follicular atresia.
Xin WANG ; Yi-Long JIANG ; Ze-Long WANG ; Bo KANG
Acta Physiologica Sinica 2023;75(1):82-90
Apoptosis and autophagy of follicular granulosa cells play an important regulatory role in the process of ovarian follicular atresia in animals. Recent studies have shown that ferroptosis and pyroptosis are also involved in the process of ovarian follicular atresia. Ferroptosis is a form of cell death caused by iron-dependent lipid peroxidation and reactive oxygen species (ROS) accumulation. Studies have confirmed that autophagy- and apoptosis-mediated follicular atresia also have typical characteristics of ferroptosis. Pyroptosis is a pro-inflammatory cell death dependent on Gasdermin protein, which can regulate ovarian reproductive performance by regulating follicular granulosa cells. This article reviews the roles and mechanisms of several types of programmed cell death independently or interactively regulating follicular atresia, in order to expand the theoretical research on follicular atresia mechanism and provide the theoretical reference for the mechanism of programmed cell death-induced follicular atresia.
Female
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Animals
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Follicular Atresia
;
Apoptosis
;
Cell Death
;
Ferroptosis
;
Pyroptosis
7.Clinical study of mature B-cell lymphoma in 11 children with chromosome 11 long-arm abnormalities.
Nan ZHANG ; Yan Long DUAN ; Chun Ju ZHOU ; Ling JIN ; Jing YANG ; Shuang HUANG ; Meng ZHANG ; Nan LI
Chinese Journal of Hematology 2023;44(11):924-929
Objective: To explore the clinical, pathological, diagnostic, treatment, and prognostic features of children with mature B-cell lymphoma (MBCL) . Methods: This retrospective study included pediatric patients with MBCL with chromosome 11 long-arm abnormalities who were diagnosed and treated at our hospital from December 2018 to February 2023. Results: Among the 11 pediatric patients with MBCL, nine were male and two were female, with a median age of 9 (2-13) years and a median disease course of 1.8 (0.5-24) months. The clinical manifestations were cervical lymph node enlargement in four patients, nasal congestion and snoring in four patients, abdominal pain in two patients, and difficulty breathing in one patient. There were seven cases of Burkitt's lymphoma, two of follicular lymphoma, and two of advanced B-cell lymphoma according to the pathological morphology examination. No patients had central nervous system or bone marrow involvement, and no extensive metastasis was observed on B-ultrasound or positron emission tomography-computed tomography (PET/CT). One patient had a huge tumor lesion. The Revised International Pediatric Non-Hodgkin Lymphoma Staging System classified four patients as stage Ⅱ, five as stage Ⅲ, and two as stage Ⅳ. 11q probe detection showed five cases of 11q gain, three of 11q loss, and three of both gain and loss. FISH showed positive MYC expression in three patients, including eight with advanced B-cell lymphoma with 11q abnormalities and three with Burkitt's lymphoma with 11q abnormalities. According to the 2019 edition of the National Health Commission's diagnostic and treatment guidelines for invasive MBCL in children, one patient was classified as Group A, two as Group B, and eight as Group C. Early evaluation of the efficacy showed complete remission. After mid-term evaluation, the intensity of chemotherapy was reduced in Group B and Group C. Among two cases of chemotherapy, the remaining nine cases had a median follow-up of 32 (6-45) months, and none had event-related survival. Conclusion: The incidence of MBCL with 11q abnormalities in children is low, clinical symptoms are mild, and progression is slow. The absence of MYC, BCL2, BCL6 rearrangements, C-MYC negative and 11q abnormalities on FISH is an important diagnostic indicator, and reducing the intensity of chemotherapy can improve prognosis.
Humans
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Female
;
Male
;
Child
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Adolescent
;
Burkitt Lymphoma/genetics*
;
Chromosomes, Human, Pair 11
;
Positron Emission Tomography Computed Tomography
;
Retrospective Studies
;
Lymphoma, Follicular
;
Chromosome Aberrations
8.Analysis of 9 cases of pediatric-type follicular lymphoma.
Gang Ping LI ; Di ZHANG ; Yong Qi WANG ; Zi Ye LI ; Fang Fang YUAN ; Ming Hui LI ; Lu WANG ; Jian Wei DU ; Li Na ZHANG ; Yu Fu LI ; Xu Dong WEI ; Yue Wen FU
Chinese Journal of Pediatrics 2023;61(12):1129-1132
Objective: To summarize the pathological diagnosis, clinical features, treatment methods and outcomes of pediatric-type follicular lymphoma (PTFL). Methods: Clinical data including the pathology, clinical features, treatment methods, and follow-up results of 9 PTFL patients admitted to Henan Cancer Hospital from February 2017 to February 2023 were analyzed retrospectively. Results: The age of onset in 9 children was 6 to 18 years, all the patients were males. The clinical manifestation was local painless lymph node enlargement in the head and neck, with a stage of Ⅰ-Ⅱ. The histomorphological characteristics of PTFL were similar to those of classic follicular lymphoma (FL). The germinal center of most follicles were enlarged, the mantle zone disappeared, centroblasts were easily visible, and the histological grade were mostly grade Ⅲ, which may be accompanied by the "starry sky" phenomenon. Monoclonal peaks can be seen in B cell clonal rearrangements (BCR). Immunohistochemistry (IHC) showed CD20 positive, CD10 positive, Bcl-6 positive, Bcl-2 negative, C-myc negative, and Ki-67 was 70%-95%. Fluorescence in situ hybridization (FISH) test was negative for t (14, 18), Bcl-2 translocation, and C-myc translocation. Six cases underwent surgical resection, and 3 cases underwent surgical resection combined with chemotherapy. Up to February 2023, with a follow-up time of 45 to 72 months, all children survived without any recurrence and were in a complete remission state. Conclusions: PTFL is mainly characterized by adolescent male onset, with early clinical manifestations and pathological manifestations of high-level histological status, high proliferation index, and lack of t (14; 18)/Bcl-2 translocation and Bcl-2 expression. It is mainly treated by localized surgical excision and has a good prognosis.
Child
;
Adolescent
;
Humans
;
Male
;
Female
;
Lymphoma, Follicular/pathology*
;
Lymphoma, B-Cell/pathology*
;
In Situ Hybridization, Fluorescence
;
Retrospective Studies
;
Proto-Oncogene Proteins c-bcl-2/genetics*
9.Diagnostic Value of American Thyroid Association Guidelines,American College of Radiology Thyroid Imaging Reporting and Data System,and Chinese Thyroid Imaging Reporting and Data System Alone and Combined With BRAFV600E Mutation in Atypia of Undetermined Significance/Follicular Lesion of Undetermined Significance.
Yu LIN ; Yu-Kun LUO ; Jie LI ; Xiu-Yun REN ; Hong-Wei WANG
Acta Academiae Medicinae Sinicae 2023;45(6):921-928
Objective To explore the diagnostic efficacy of American Thyroid Association(ATA)guidelines,American College of Radiology Thyroid Imaging Report and Data System(ACR-TIRADS),and Chinese Thyroid Imaging Reporting and Data System(C-TIRADS)alone and combined with BRAFV600E mutation in atypia of undetermined significance/follicular lesion of undetermined significance(AUS/FLUS).Methods A total of 138 patients who underwent ultrasound-guided fine needle aspiration(FNA)in the Chinese PLA General Hospital from January 2020 to May 2023 were selected.The clinicopathological and ultrasound characteristics were retrospectively analyzed for each nodule.Each nodule underwent preoperative BRAFV600E mutation testing and was diagnosed according to the ATA guidelines,ACR-TIRADS,and C-TIRADS.The diagnostic efficacy of ATA guidelines,ACR-TIRADS,and C-TIRADS alone and combined with BRAFV600E mutation was assessed based on the results of histopathological diagnosis.Results The 138 AUS/FLUS thyroid nodules included 45(32.6%)benign ones and 93(67.4%)malignant ones.The patient age(t=1.444,P=0.151),gender(χ2=0.259,P=0.611),and location of nodules(χ2=2.055,P=0.358)had no statistical significance for the differentiation between benign and malignant nodules,while nodule size(Z=2.500,P=0.012),echo(χ2=14.693,P<0.001),composition(χ2=17.075,P<0.001),aspect ratio ≥1(χ2=9.477,P=0.002),and microcalcification(χ2=6.892,P=0.009)were of significance for the differentiation.When applied alone,BRAFV600E mutation showed high specificity(95.56%)and positive predictive value(95.65%).Among the three ultrasound grading systems,ACR-TIRADS had the highest sensitivity(χ2=37.923,P<0.001;χ2=40.462,P<0.001)and accuracy(χ2=81.595,P<0.001;χ2=76.912,P<0.001),while C-TIRADS had the highest specificity(χ2=11.746,P<0.001;χ2=21.235,P<0.001).However,the three systems showed no statistically significant difference in the diagnostic efficiency when applied alone(Z=1.177,P=0.239;Z=0.213,P=0.831;Z=1.016,P=0.310).The combination of BRAFV600E mutation with ACR-TIRADS or C-TIRADS improved the diagnostic efficacy of BRAFV600E mutation in distinguishing the benign and malignant AUS/FLUS nodules(Z=2.107,P=0.035;Z=2.752,P=0.006).The combination of ATA guidelines with BRAFV600E mutation increased the diagnostic accuracy of BRAFV600E mutation(χ2=20.679,P<0.001),while it had no statistically significant difference in distinguishing the benign and malignant AUS/FLUS nodules(Z=1.321,P=0.186).The combination of ATA guidelines,ACR-TIRADS,or C-TIRADS with BRAFV600E mutation improved the diagnostic efficacy of ultrasound grading systems for AUS/FLUS nodules(Z=2.770,P=0.006;Z=2.770,P=0.006;Z=2.890,P=0.004).Specifically,ACR-TIRADS combined with BRAFV600E mutation showed the highest sensitivity(χ2=4.712,P=0.030;χ2=4.712,P=0.030),while C-TIRADS combined with BRAFV600E mutation showed the highest accuracy(χ2=77.627,P<0.001;χ2=85.827,P<0.001).However,there were no statistically significant differences in diagnostic performance between the combinations(Z=1.276,P=0.202;Z=0.808,P=0.419;Z=1.615,P=0.106).Conclusion ATA guidelines,ACR-TIRADS,and C-TIRADS combined with BRAFV600E mutation can improve the diagnostic efficacy of BRAFV600E mutation or ultrasound grading system alone in AUS/FLUS nodules,which can facilitate the further management and treatment of such patients.
Humans
;
United States
;
Infant
;
Thyroid Neoplasms/genetics*
;
Proto-Oncogene Proteins B-raf/genetics*
;
Adenocarcinoma, Follicular/pathology*
;
Retrospective Studies
;
Data Systems
;
Thyroid Nodule/genetics*
;
Ultrasonography/methods*
;
Mutation
;
China
;
Radiology
10.Clinical Research Advances of Duodenal-Type Follicular Lymphoma--Review.
Hong-Yi LI ; Jun-Dong ZHANG ; Xue-Chun LU
Journal of Experimental Hematology 2023;31(2):581-584
Duodenal-type follicular lymphoma (DFL) is a unique subtype of follicular lymphoma (FL), which often involves the second portion of duodenum (descending part of duodenum). Due to its specific pathological features, such as lack of follicular dendritic cells meshwork and disappearance of activation-induced cytidine deaminase expression, DFL presents an inert clinical course and is often confined to the intestinal tract. Inflammation-related biomarkers suggest that the microenvironment may play a likely role in the pathogenesis and favorable prognosis of DFL. Since patients generally have no obvious clinical symptoms and low progression rate, the treatment regimen for DFL is mainly observation and waiting (W&W) strategy. This study will review the latest research progress of epidemiology, diagnosis, treatment and prognosis of DFL in recent years.
Humans
;
Lymphoma, Follicular/drug therapy*
;
Duodenal Neoplasms/pathology*
;
Prognosis
;
Tumor Microenvironment


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