ABSTRACT
BACKGROUND: Follicular dendritic cell sarcoma (FDCS) is a rare malignancy that arises from follicular dendritic cells and typically presents as a slow-growing painless mass without specific symptoms. Here we report an unusual case of a 55-year-old female with retroperitoneal FDCS who presented with progressive abdominal pain onset and acute exacerbation. METHODS: On CTA, a middle-upper abdominal mass (58*40 mm) was shown with multiple enlarged lymph nodes. After en-bloc resection of the tumor, the patient recovered completely from her symptoms and was discharged without complication. One month later, the patient returned for follow-up and the relevant tests were completed. RESULTS: In this case, CA724 elevated significantly and seemed to be associated with tumor progression. The results of positron emission tomography/computed tomography (PET/CT) and radiological examinations, including magnetic resonance imaging (MRI) and computed tomography angiography (CTA), were discussed to improve our understanding of diagnostic tools on FDCS. Targeted genomic sequencing analysis revealed three novel gene mutations, EPHA3 (nonsense mutation), DDR2 (SNV), and BIRC3 (InDel). CONCLUSION: We reported an unusual case of retroperitoneal FDCS with acute exacerbated abdominal pain. The interpretations of CA724, PET/CT, as well as imaging results deserve further investigation in FDCS. Genomic sequencing revealed three novel gene mutations in FDCS, including EPHA3 (nonsense mutation), DDR2 (SNV), and BIRC3 (InDel).
Subject(s)
Dendritic Cell Sarcoma, Follicular , Humans , Female , Middle Aged , Dendritic Cell Sarcoma, Follicular/complications , Dendritic Cell Sarcoma, Follicular/diagnosis , Dendritic Cell Sarcoma, Follicular/genetics , Positron Emission Tomography Computed Tomography , Codon, Nonsense , Abdominal Pain/diagnosis , Abdominal Pain/etiology , GenomicsABSTRACT
OBJECTIVE: To test the hypothesis that both indolent and aggressive chronic lymphocytic leukemia (CLL) can be differentiated from diffuse large B cell lymphoma (DLBCL) of Richter syndrome (RS) by CT texture analysis (CTTA) of involved lymph nodes. MATERIAL AND METHODS: We retrospectively included 52 patients with indolent CLL (26/52), aggressive CLL (8/52), and DLBCL of RS (18/52), who underwent standardized contrast-enhanced CT. In main lymphoma tissue, VOIs were generated from which CTTA features including first-, second-, and higher-order textural features were extracted. CTTA features were compared between the entire CLL group, the indolent CLL subtype, the aggressive CLL subtype, and DLBCL using a Kruskal-Wallis test. All p values were adjusted after the Bonferroni correction. ROC analyses for significant CTTA features were performed to determine cut-off values for differentiation between the groups. RESULTS: Compared with DLBCL of RS, CTTA of the entire CLL group showed significant differences of entropy heterogeneity (p < 0.001), mean intensity (p < 0.001), mean average (p = 0.02), and number non-uniformity gray-level dependence matrix (NGLDM) (p = 0.03). Indolent CLL significantly differed for entropy (p < 0.001), uniformity of heterogeneity (p = 0.02), mean intensity (p < 0.001), and mean average (p = 0.01). Aggressive CLL showed significant differences in mean intensity (p = 0.04). For differentiation between CLL and DLBCL of RS, cut-off values for mean intensity and entropy of heterogeneity were defined (e.g., 6.63 for entropy heterogeneity [aggressive CLL vs. DLBCL]; sensitivity 0.78; specificity 0.63). CONCLUSIONS: CTTA features of ultrastructure and vascularization significantly differ in CLL compared with that in DLBCL of Richter syndrome, allowing complementary to visual features for noninvasive differentiation by contrast-enhanced CT. KEY POINTS: ⢠Richter transformation of CLL into DLBCL results in structural changes in lymph node architecture and vascularization that can be detected by CTTA. ⢠First-order CT textural features including intensity and heterogeneity significantly differ between both indolent CLL and aggressive CLL and DLBCL of Richter syndrome. ⢠CT texture analysis allows for noninvasive detection of Richter syndrome which is of prognostic value.
Subject(s)
Dendritic Cell Sarcoma, Follicular/pathology , Leukemia, Lymphocytic, Chronic, B-Cell/pathology , Lymphoma, Large B-Cell, Diffuse/pathology , Aged , Cell Differentiation , Dendritic Cell Sarcoma, Follicular/complications , Dendritic Cell Sarcoma, Follicular/diagnostic imaging , Female , Humans , Leukemia, Lymphocytic, Chronic, B-Cell/complications , Leukemia, Lymphocytic, Chronic, B-Cell/diagnostic imaging , Lymph Nodes/pathology , Lymphoma, Large B-Cell, Diffuse/complications , Lymphoma, Large B-Cell, Diffuse/diagnostic imaging , Male , Middle Aged , Prognosis , Retrospective Studies , Sensitivity and Specificity , Syndrome , Tomography, X-Ray Computed/methodsABSTRACT
BACKGROUND: Follicular dendritic cell sarcoma (FDCS) is a rare neoplasm that originates from follicular dendritic cells in lymphoid tissue while paraneoplastic pemphigus (PNP) is an autoimmune blistering disease associated with neoplasms. Pancreatic FDCS associated with PNP and myasthenia gravis (MG) is even rarer and highly malignant. We present the clinical data, pathological materials and computed tomography (CT) features of a rare case of this disease. CASE PRESENTATION: A 49-year-old woman presented with repeated ptosis of both eyelids, oral ulcers and erosions. Her laboratory results showed a slight elevation of CA125 and positivity of some autoimmune antibodies. CT revealed a round solid mass with central necrosis in the pancreatic tail. The solid component of the mass showed slight enhancement and serpentine feeding arteries in the arterial phase, moderate enhancement with a draining vein around the tumor in the portal venous phase and persistent enhancement in the delayed phase. Surgical resection was performed, and the pathological diagnosis was FDCS. However, the patient died of inability to excrete sputum and occlusion of the respiratory tract. CONCLUSIONS: Pancreatic FDCS manifested as PNP and MG is very rare. Its CT features are not specific, and the disease should be differentiated from neuroendocrine tumors, solid pseudopapillary neoplasms and acinar cell carcinoma.
Subject(s)
Dendritic Cell Sarcoma, Follicular/diagnostic imaging , Myasthenia Gravis/diagnostic imaging , Pancreatic Neoplasms/diagnostic imaging , Paraneoplastic Syndromes/diagnostic imaging , Pemphigus/diagnostic imaging , Tomography, X-Ray Computed , Dendritic Cell Sarcoma, Follicular/complications , Female , Humans , Middle Aged , Myasthenia Gravis/etiology , Pancreas/diagnostic imaging , Pancreatic Neoplasms/complications , Paraneoplastic Syndromes/complications , Pemphigus/etiologyABSTRACT
Paraneoplastic pemphigus is a severe autoimmune blistering disease presenting in the setting of underlying malignancy. Paraneoplastic pemphigus is associated with diffuse painful stomatitis throughout the oral cavity with extension to the lips. The cutaneous findings are varied and have been described as lichenoid, pemphigoid, and targetoid lesions. Herein, we report a patient with paraneoplastic pemphigus whose routine testing led to a diagnosis of pemphigus vulgaris. However, further testing was pursued revealing an antibody profile consistent with paraneoplastic pemphigus. Subsequent neoplastic workup revealed an intra-abdominal mass. Our case represents a subtle, non-classic presentation of paraneoplastic pemphigus and suggests the importance of a comprehensive investigative work-up in atypical cases of pemphigus.
Subject(s)
Abdominal Neoplasms/diagnosis , Dendritic Cell Sarcoma, Follicular/diagnosis , Desmoglein 1/immunology , Desmoglein 2/immunology , Paraneoplastic Syndromes/immunology , Pemphigus/immunology , Abdominal Neoplasms/complications , Dendritic Cell Sarcoma, Follicular/complications , Diagnosis, Differential , Female , Humans , Middle Aged , Paraneoplastic Syndromes/diagnosis , Pemphigus/diagnosis , Pemphigus/etiology , Positron-Emission TomographySubject(s)
Bronchiolitis Obliterans/etiology , Dendritic Cell Sarcoma, Follicular/complications , Dendritic Cell Sarcoma, Follicular/pathology , Paraneoplastic Syndromes/etiology , Paraneoplastic Syndromes/pathology , Pemphigus/etiology , Adult , Bronchiolitis Obliterans/pathology , Female , Humans , Pemphigus/pathologySubject(s)
Abdominal Pain/etiology , Dendritic Cell Sarcoma, Follicular/complications , Epstein-Barr Virus Infections/complications , Granuloma, Plasma Cell/complications , Aged , Dendritic Cell Sarcoma, Follicular/diagnostic imaging , Dendritic Cell Sarcoma, Follicular/pathology , Epstein-Barr Virus Infections/diagnosis , Female , Granuloma, Plasma Cell/diagnostic imaging , Granuloma, Plasma Cell/pathology , Humans , Tomography, X-Ray ComputedABSTRACT
BACKGROUND: Inflammatory pseudotumor-like follicular dendritic cell sarcoma (IPT-like FDCS) is often associated with Epstein-Barr (EB) virus infection. The tumor is commonly found in the spleen and liver, and it has been reported in the literature that it can be associated with paraneoplastic pemphigus, myasthenia gravis, and other diseases. A case of IPT-like FDCS with clinical features of thrombocytopenia has not been reported. PATIENT CONCERNS: A 59-year-old male patient visited our hospital in September 2020 due to bleeding gums and epistaxis. DIAGNOSIS: Splenic lymphoma with marked thrombocytopenia was initially diagnosed. The patient underwent pathological examination after splenectomy. Microscopic examination showed spindle-shaped or oval cells arranged in loose bundles, a large number of lymphocytes and plasma cells infiltrating the interstitium, and fibrin-like changes in the blood vessel wall. Immunohistochemical detection of tumor cells was positive for CD21, CD35, and Epstein-Barr virus in situ hybridization, and the patient was diagnosed with IPT-like FDCS. INTERVENTIONS: The patient underwent a splenectomy. The patient received platelet-raising therapy postoperatively. OUTCOMES: No tumor recurrence or metastasis was found during the 17-month follow-up period, and the platelet count returned to normal. CONCLUSION: IPT-like FDCS is an uncommon tumor, and its first presentation with marked thrombocytopenia is even rarer. The tumor was clinically and radiographically nonspecific. Definitive diagnosis relies on histopathological and immunohistochemical staining. IPT-like FDCS is biologically indolent and has a favorable prognosis.
Subject(s)
Dendritic Cell Sarcoma, Follicular , Epstein-Barr Virus Infections , Granuloma, Plasma Cell , Thrombocytopenia , Male , Humans , Middle Aged , Dendritic Cell Sarcoma, Follicular/complications , Dendritic Cell Sarcoma, Follicular/diagnosis , Dendritic Cell Sarcoma, Follicular/surgery , Epstein-Barr Virus Infections/complications , Herpesvirus 4, Human , Granuloma, Plasma Cell/complications , Granuloma, Plasma Cell/diagnosis , Neoplasm Recurrence, Local/complications , Thrombocytopenia/complicationsABSTRACT
PURPOSE: To describe a case of paraneoplastic pemphigus (PNP) presenting as spontaneous bilateral corneal perforations in a patient with follicular dendritic cell sarcoma. METHODS: Retrospective chart reviewResults: A 73-year-old Greek woman with a history of follicular dendritic cell sarcoma (FDCS) presented with bilateral corneal perforations and a cicatrizing conjunctivitis. Her diagnosis was consistent with PNP with corneal and conjunctival involvement after a change in her chemotherapy regimen from intravenous cyclophosphamide to gemcitabine. She was treated with a multilayered amniotic membrane in the right eye and cyanoacrylate glue in the left eye. Systemic intravenous cyclophosphamide and oral prednisone were re-started. Both perforations healed but the patient passed away soon after precluding further follow-up. CONCLUSIONS: Ocular manifestations of PNP can rarely present with spontaneous corneal perforations. This is the first case of FDCS-associated PNP with corneal involvement. Such cases should be diagnosed expediently and managed with aggressive systemic immunosuppressive therapy.
Subject(s)
Corneal Perforation , Dendritic Cell Sarcoma, Follicular , Paraneoplastic Syndromes , Pemphigus , Aged , Corneal Perforation/diagnosis , Corneal Perforation/etiology , Cyclophosphamide/therapeutic use , Dendritic Cell Sarcoma, Follicular/complications , Dendritic Cell Sarcoma, Follicular/diagnosis , Female , Humans , Paraneoplastic Syndromes/diagnosis , Paraneoplastic Syndromes/etiology , Pemphigus/complications , Pemphigus/diagnosis , Pemphigus/drug therapy , Retrospective StudiesSubject(s)
Airway Obstruction/etiology , Dendritic Cell Sarcoma, Follicular/complications , Goiter, Nodular/surgery , Lymphatic Diseases/complications , Mediastinal Neoplasms/complications , Respiratory Insufficiency/etiology , Tracheal Neoplasms/diagnosis , Acute Disease , Airway Obstruction/surgery , Bronchoscopy , Dendritic Cell Sarcoma, Follicular/diagnostic imaging , Dendritic Cell Sarcoma, Follicular/surgery , Electrosurgery , Female , Goiter, Nodular/complications , Goiter, Nodular/pathology , Humans , Lymphatic Diseases/pathology , Lymphatic Diseases/surgery , Mediastinal Neoplasms/diagnosis , Mediastinal Neoplasms/diagnostic imaging , Middle Aged , Neoplasm Recurrence, Local , Positron-Emission Tomography , Respiratory Insufficiency/surgery , Thyroidectomy , Tracheal Neoplasms/secondary , Tracheal Neoplasms/surgerySubject(s)
Dendritic Cell Sarcoma, Follicular/complications , Paraneoplastic Syndromes , Pemphigus/etiology , Skin/pathology , Biopsy , Combined Modality Therapy , Dendritic Cell Sarcoma, Follicular/diagnosis , Dendritic Cell Sarcoma, Follicular/therapy , Humans , Male , Pemphigus/diagnosis , Pemphigus/therapy , Young AdultSubject(s)
Dendritic Cell Sarcoma, Follicular/pathology , Immunologic Factors/therapeutic use , Lymph Nodes/pathology , Myasthenia Gravis/drug therapy , Rituximab/therapeutic use , Autoantibodies/immunology , Axilla , Dendritic Cell Sarcoma, Follicular/complications , Female , Humans , Middle Aged , Myasthenia Gravis/etiology , Myasthenia Gravis/immunology , Neck , Receptor Protein-Tyrosine Kinases/immunology , Receptors, Cholinergic/immunology , Treatment OutcomeABSTRACT
Currently, of less than 50 cases of head and neck follicular dendritic cell (FDC) sarcoma reported in the literature, 5 have been found to occur in the background of Castleman disease. We report another case of head and neck FDC sarcoma with emphasise on its associated lesions and review the outcome of treatment from the existing cases in the literature.
Subject(s)
Castleman Disease/pathology , Dendritic Cell Sarcoma, Follicular/pathology , Head and Neck Neoplasms/pathology , Aged , Castleman Disease/complications , Dendritic Cell Sarcoma, Follicular/complications , Dendritic Cell Sarcoma, Follicular/therapy , Head and Neck Neoplasms/complications , Head and Neck Neoplasms/therapy , Humans , MaleABSTRACT
OBJECTIVE: To study the clinicopathologic features of follicular dendritic cell sarcoma (FDCS) and its differential diagnosis. METHODS: Ten cases of FDCS were studied by light microscopy, immunohistochemistry and in-situ hybridization. The clinical features and follow-up information were analyzed. RESULTS: Amongst the 10 cases of FDCS studied, the male-to-female ratio was 1:1. The mean age of the patients was 42 years. Six of them were located in cervical and peritoneal lymph nodes and four in extranodal sites (including tonsil, pelvic cavity, tail of pancreas and spleen). Histologically, the tumor cells had whorled, storiform or diffuse growth patterns. They were spindle in shape and contained syncytial eosinophilic cytoplasm, with round or oval nuclei, vesicular chromatin, distinct nucleoli and a variable number of mitotic figures. Multinucleated tumor giant cells and intranuclear pseudoinclusions were occasionally seen. There was a sprinkling of small lymphocytes and neutrophils within the tumor as well as in the perivascular region. Immunohistochemical study showed that the tumor cells were diffusely or focally positive for CD21, CD23, CD35 and D2-40, but negative for LCA, CD20, CD3, CD1a, HMB45 and CK. Some of them showed EMA, CD68 and S-100 reactivity. In-situ hybridization for Epstein-Barr virus-encoded RNA (EBER) showed positive signals in only one case (which was diagnosed as inflammatory pseudotumor-like FDCS). Of the 7 patients with follow-up information available (duration: 2 months to 39 months; mean: 14 months), 2 cases with paraneoplastic pemphigus died of pulmonary infection at 5 and 7 months respectively. The remaining 5 patients were alive and disease-free after surgical excision (+/- chemotherapy and radiotherapy). CONCLUSIONS: FDCS is a rare low to intermediate-grade malignant tumor. Appropriate application of FDC markers, such as CD21, CD35 and D2-40, would be helpful for arriving at a correct diagnosis. Most cases are associated with good prognosis after surgical treatment, with or without chemotherapy and radiotherapy. Patients with paraneoplastic pemphigus carry a less favorable prognosis.
Subject(s)
Dendritic Cell Sarcoma, Follicular/pathology , Lymph Nodes/pathology , Tonsillar Neoplasms/pathology , Adult , Antibodies, Monoclonal, Murine-Derived/metabolism , Dendritic Cell Sarcoma, Follicular/complications , Dendritic Cell Sarcoma, Follicular/metabolism , Dendritic Cell Sarcoma, Follicular/surgery , Dendritic Cell Sarcoma, Interdigitating/pathology , Diagnosis, Differential , Female , Follow-Up Studies , Humans , Lymph Node Excision , Lymph Nodes/surgery , Male , Meningioma/pathology , Middle Aged , Nasopharyngeal Neoplasms/pathology , Paraneoplastic Syndromes/complications , Pemphigus/complications , Receptors, Complement 3b/metabolism , Receptors, Complement 3d/metabolism , Receptors, IgE/metabolism , Tonsillar Neoplasms/metabolism , Tonsillar Neoplasms/surgery , Young AdultABSTRACT
RATIONALE: Follicular dendritic cell sarcoma (FDCS) is a rare malignant tumor derived from follicular dendritic cells, and is often associated with Castleman disease. Here we present a rare case of paraneoplastic pemphigus (PNP) with FDCS which required multidisciplinary approach for the diagnosis and treatment. PATIENT CONCERNS: A 28-year-old Chinese female had FDCS recurrence, and primary clinical manifestation was PNP. DIAGNOSES: PNP with FDCS. INTERVENTIONS: The patient received gamma globulin infusion, took anlotinib, and underwent plasma exchange therapy. OUTCOMES: The skin lesions recovered and there was no evidence of tumor recurrence. LESSONS: The diagnosis and management of PNP with FDCS require close cooperation among surgeons, dermatologists, hematologists, otolaryngologists, oncologists, radiologists, pathologists, and respiratory doctors. The interesting clinical manifestations of this patient provide a multifaceted approach to the investigation of the interactions among FDCS, Castleman disease, and PNP.
Subject(s)
Abdomen/pathology , Dendritic Cell Sarcoma, Follicular/complications , Paraneoplastic Syndromes/complications , Pemphigus/complications , Adult , Dendritic Cell Sarcoma, Follicular/diagnosis , Dendritic Cell Sarcoma, Follicular/therapy , Female , Humans , Paraneoplastic Syndromes/diagnosis , Paraneoplastic Syndromes/therapy , Pemphigus/diagnosis , Pemphigus/therapyABSTRACT
A 48-year-old woman presented with refractory oral ulcers and skin rashes on the palms and trunk, diagnosed as paraneoplastic pemphigus. The chest x-ray revealed a mass in the right lower chest, and the F-FDG PET/CT scan showed the lesion in the right anterior-inferior mediastinum with intense F-FDG uptake, accompanied by right parasternal adenopathy and pleural effusion. The surgical pathology proved a follicular dendritic cell sarcoma, with right parasternal lymph node metastasis.