RESUMEN
Follicular dendritic cell sarcoma of the parotid gland is an extremely rare tumor, with only six cases reported in the literature. A 51-year-old female had a 3.0 cm tumor resected from the right parotid gland. The tumor exhibited solid sheets, whorls, fascicular pattern, and syncytium appearance with an indistinct cell border. The lymphocytic infiltrate was sprinkled throughout the neoplasm, with focal prominent perivascular cuffing. Immunohistochemically, it was positive for follicular dendritic cell markers CD21, CD23, and CD35. We aim to enhance the understanding of this neoplasm and alert pathologists to this rare entity in this region to avoid misdiagnosis.
Asunto(s)
Sarcoma de Células Dendríticas Foliculares , Neoplasias de la Parótida , Humanos , Sarcoma de Células Dendríticas Foliculares/patología , Femenino , Neoplasias de la Parótida/patología , Persona de Mediana Edad , Biomarcadores de Tumor/análisisRESUMEN
BACKGROUND: Spleen Epstein-Barr Virus (EBV)-positive inflammatory follicular dendritic cell sarcoma (FDCS) is rare, and the imaging signs are unclear. The COVID-19 has been confirmed to be the cause of pneumonia and can cause a variety of diseases including myocarditis. However, it has not been reported to be the cause of the exacerbation or activation of EBV-positive inflammatory FDCS. OBJECTIVE: The objective is to extract the imaging features of EBV-positive inflammatory FDCS in the spleen and analyze the reasons for the special features of this case. METHODS: By analyzing the patient's treatment process and imaging examinations (A 77-year-old female was admitted to the hospital due to generalized discomfort and pain symptoms. When she was admitted to the hospital a year earlier with COVID-19 pneumonia, a chest CT scan showed that she had a splenic tumor. During this admission, CT scans showed two irregularly shaped and unevenly dense soft tissue density masses within the spleen, with uneven enhancement on contrast-enhanced im-aging within the solid components and along the edges. PET/CT scans revealed elevated glucose metabolism in the masses. Postoperative pathological diagnosis confirmed splenic EBV-positive inflammatory FDCS.), reading the literature, sorting out the disease cognitive process, epidemiology, and pathological data of EBV-positive inflammatory FDCS, we discussed the imaging manifestations and possible differential diagnosis of the disease. RESULTS: The patient was finally diagnosed with splenic EBV-positive inflammatory FDCS. CONCLUSIONS: Imaging features of EBV-positive inflammatory FDCS in the spleen include a high incidence of hemorrhage and necrosis, persistent moderate enhancement of the solid portion, a "capsular-like enhancement" structure at the tumor edge, and possibly active glucose metabolism with high Standardized Uptake Values (SUVs). COVID-19 infection and long-term COVID-19 sequelae may exacerbate and activate EBV-positive inflammatory FDCS in the spleen, and the mechanism remains to be further studied.
Asunto(s)
Sarcoma de Células Dendríticas Foliculares , Infecciones por Virus de Epstein-Barr , Tomografía Computarizada por Tomografía de Emisión de Positrones , Humanos , Femenino , Anciano , Sarcoma de Células Dendríticas Foliculares/patología , Sarcoma de Células Dendríticas Foliculares/diagnóstico , Sarcoma de Células Dendríticas Foliculares/virología , Infecciones por Virus de Epstein-Barr/diagnóstico , Infecciones por Virus de Epstein-Barr/complicaciones , Tomografía Computarizada por Tomografía de Emisión de Positrones/métodos , COVID-19/complicaciones , Neoplasias del Bazo/patología , Neoplasias del Bazo/diagnóstico por imagen , Neoplasias del Bazo/diagnóstico , Neoplasias del Bazo/virología , Bazo/patología , Bazo/diagnóstico por imagen , Herpesvirus Humano 4/aislamiento & purificación , Tomografía Computarizada por Rayos X , SARS-CoV-2RESUMEN
OBJECTIVE: The present study aims to explore the clinicopathological characteristics of Epstein-Barr virus (EBV)-positive inflammatory follicular dendritic cell sarcoma (IFDCS; EBV+ IFDCS). CASE REPORT: The case involved a 32-year-old woman who underwent surgical resection of a splenic nodule. Histological examination and immunohistochemistry were performed using cluster of differentiation (CD) markers, and in-situ hybridization was conducted to detect EBV-encoded RNA (EBER). RESULTS: A microscopic analysis revealed neoplastic cells with various morphologies, including round, ovoid, or spindled shapes, dispersed within a prominent lymphoplasmacytic infiltrate. The tumor cells exhibited nuclear atypia, with some resembling Reed-Sternberg cells. The immunohistochemistry demonstrated focal positivity for follicular dendritic cell markers, such as CD21, CD23 and CD35, and focal negativity for other markers, including CD3, CD34, CD20, CD79a, myeloperoxidase and HMB45. Additionally, the EBER staining showed strongly positive results. The patient showed no local recurrence or metastasis during the 13-month follow-up. CONCLUSION: A comprehensive understanding of EBV+IFDCS, including its clinicopathological features and immunohistochemical characteristics, is crucial for accurate diagnosis and differential diagnosis of this rare tumor.
Asunto(s)
Sarcoma de Células Dendríticas Foliculares , Infecciones por Virus de Epstein-Barr , Herpesvirus Humano 4 , Humanos , Femenino , Sarcoma de Células Dendríticas Foliculares/patología , Sarcoma de Células Dendríticas Foliculares/virología , Sarcoma de Células Dendríticas Foliculares/diagnóstico , Adulto , Herpesvirus Humano 4/aislamiento & purificación , Infecciones por Virus de Epstein-Barr/complicaciones , Infecciones por Virus de Epstein-Barr/patología , Infecciones por Virus de Epstein-Barr/virología , Infecciones por Virus de Epstein-Barr/diagnóstico , Neoplasias del Bazo/patología , Neoplasias del Bazo/virología , Neoplasias del Bazo/diagnóstico , Inmunohistoquímica , Inflamación/patología , Inflamación/virologíaRESUMEN
RATIONALE: Inflammatory pseudotumor-like follicular dendritic cell sarcoma (IPT-like FDCS) of the liver is rare. It was previously believed that Epstein-Barr virus (EBV) positivity was a necessary criterion for pathological diagnosis. However, we found that there were also cases of EBV negativity. Therefore, clinicians and pathologists are reminded that EBV positivity is not a necessary condition for diagnosis. PATIENT CONCERNS: A 70-year-old female underwent computed tomography (CT) examination for upper abdominal discomfort, which revealed the presence of a liver tumor. Follow-up revealed that the tumor had progressively increased in size. DIAGNOSIS: The final diagnosis was an IPT-like follicular cell sarcoma, based on CT, MRI, HE staining, and immunohistochemical staining. INTERVENTIONS: The patient underwent a laparoscopic left hemihepatectomy. OUTCOMES: The patient has not undergone any special treatment, such as radiotherapy and chemotherapy, and has been followed up for over 3 years without experiencing any recurrence. LESSONS: IPT-like FDCS is a rare tumor that lacks definitive criteria, and its diagnosis mainly relies on pathological findings. Previously, it was believed that being EBV-positive was an important condition for diagnosis. Primary IPT-like FDCS in the liver is even rarer, and the patient in this case tested negative for EBV. It may be necessary for pathologists to consider the role of EBV in the diagnosis of IPT-like FDCS.
Asunto(s)
Sarcoma de Células Dendríticas Foliculares , Infecciones por Virus de Epstein-Barr , Granuloma de Células Plasmáticas , Femenino , Humanos , Anciano , Sarcoma de Células Dendríticas Foliculares/diagnóstico , Sarcoma de Células Dendríticas Foliculares/cirugía , Sarcoma de Células Dendríticas Foliculares/patología , Infecciones por Virus de Epstein-Barr/complicaciones , Infecciones por Virus de Epstein-Barr/diagnóstico , Granuloma de Células Plasmáticas/diagnóstico , Granuloma de Células Plasmáticas/cirugía , Granuloma de Células Plasmáticas/patología , Herpesvirus Humano 4 , Hígado/diagnóstico por imagen , Hígado/patologíaRESUMEN
A 72-year-old man was referred to our hospital for the examination of a pancreatic head mass. Abdominal computed tomography revealed a contrasted 8-cm-diameter tumor extending from the dorsal pancreatic head to the porta hepatis. The preoperative diagnosis was challenging due to the absence of specific imaging findings and the inability to perform a biopsy. Positron emission tomography/computed tomography and diffusion-weighted imaging suggested a malignant tumor originating from the organs surrounding the pancreatic head. Subtotal stomach-preserving pancreaticoduodenectomy with regional lymph node dissection was performed, as dissection from the pancreatic head proved unfeasible. Pathological examination identified the tumor as an enlarged lymph node consisting of pleomorphic large cells forming clusters, positive for follicular dendritic cell markers cluster of differentiation (CD) 21 and CD23. No evidence of tumor capsule infiltration, other organ infiltration, or metastasis to other lymph nodes was observed. The final diagnosis was nodal follicular dendritic cell sarcoma (FDCS) originating from the pancreatic head lymph nodes. No recurrence occurred at 3 years postoperatively with no postoperative treatment. Intraperitoneal nodal FDCS is extremely rare, and occasionally, it can lead to postoperative recurrence and progression. It is crucial to differentiate neoplastic lymph node enlargement around the pancreatic head from nodal FDCS.
Asunto(s)
Sarcoma de Células Dendríticas Foliculares , Neoplasias Pancreáticas , Pancreaticoduodenectomía , Humanos , Masculino , Anciano , Neoplasias Pancreáticas/patología , Neoplasias Pancreáticas/cirugía , Neoplasias Pancreáticas/diagnóstico por imagen , Sarcoma de Células Dendríticas Foliculares/patología , Sarcoma de Células Dendríticas Foliculares/cirugía , Sarcoma de Células Dendríticas Foliculares/diagnóstico por imagen , Ganglios Linfáticos/patología , Tomografía Computarizada por Tomografía de Emisión de Positrones , Páncreas/patología , Escisión del Ganglio Linfático , Imagen de Difusión por Resonancia Magnética , Metástasis LinfáticaRESUMEN
Folliclular dendritic cell sarcoma (FDCS) is an extremely rare neoplasm originating from folliclular dendritic cells, both nodally and extranodally. Its primary presentation as a large colonic mass is rare and can be misdiagnosed as epithelial tumor/soft tissue tumor both clinically and through histomorphology. Due to its rarity and limited consensus guidelines about its management, it presents as a diagnostic and therapeutic challenge for pathologists and oncologists. However, accurate diagnosis is imperative due to its distinct prognostic and therapeutic implications. Herein we report, two cases of extranodal FDCS of colon with the aim of contributing to the management of this uncommon entity.
Asunto(s)
Carcinoma , Sarcoma de Células Dendríticas Foliculares , Humanos , Sarcoma de Células Dendríticas Foliculares/diagnóstico , Sarcoma de Células Dendríticas Foliculares/terapia , Sarcoma de Células Dendríticas Foliculares/patología , PronósticoRESUMEN
ABSTRACT: Follicular dendritic cell sarcoma is a rare intermediate-grade malignancy characterized by a proliferation of ovoid to spindle-shaped cells with morphologic and immunophenotypic features similar to normal follicular dendritic cells. It may develop in lymph nodes or extranodal sites. Its presentation in extranodal tissues is a diagnostic challenge. It requires a high index of suspicion because follicular dendritic cell markers are not included in the routine immunohistochemical panels used for differential diagnosis. In an extensive review of the English literature, we found 3 cases of follicular dendritic cell sarcoma developing on the skin. We report a case of a primary cutaneous follicular dendritic cell sarcoma in a 28-year-old man, which presented as a 6-mm skin-colored nodule on the right forearm. We describe the morphologic and immunohistochemical features and a review of the literature.
Asunto(s)
Sarcoma de Células Dendríticas Foliculares , Masculino , Humanos , Adulto , Sarcoma de Células Dendríticas Foliculares/diagnóstico , Sarcoma de Células Dendríticas Foliculares/patología , Piel/patología , Diagnóstico Diferencial , Ganglios Linfáticos/patología , InmunofenotipificaciónRESUMEN
INTRODUCTION: Follicular Dendritic Cell Sarcomas (FDCS)was first found in 1986; the specificity of the disease is its rarity, with an incidence of only 0.4%, numerous doctors for its lack of understanding, the accuracy of imaging diagnosis is not great, which is easy to delay the treatment. This article summarizes several characteristic imaging manifestations of FDCS to provide imaging physicians with an understanding of the imaging properties of this rare disease. When faced with complex cases, the radiologist can consider this disease and include it in the differential diagnosis. FDCS occurs mainly in lymph nodes, mainly in the head and neck. The main symptoms are fatigue, local pain, or painless mass. The treatment method is not uniform, but scholars agree that we should strive for the opportunity of surgery as much as possible. CASE PRESENTATION: This paper reported a case of FDCS with pelvic recurrence 3 years after surgery. The patient was suspected to have lymphoma by postoperative pathology in the local hospital, and it is recommended that the patient be reexamined regularly. A soft tissue mass was recently found again in the left pelvic cavity. After an enhanced CT examination, the radiologist was skeptical of the previous diagnosis of lymphoma. Subsequently, a needle biopsy was performed at Peking University Shougang Hospital. The pathological results rejected the prior diagnosis of lymphoma after consultation with additional hospitals, and the patient was diagnosed with FDCS. CONCLUSIONS: The imaging manifestations of FDCS lack absolute specificity, but it also has imaging characteristics, such as large areas of necrosis in the huge mass, rough mass calcification in the mass, enhanced scan showed "fast in and slow out" mode, and there were blood vessels in the tumor. FDCS mainly occurs in lymph nodes and is easily misdiagnosed as GIST, inflammatory myoblastoma, lymphoma, etc. Radiologists should continue to collect cases of this disease and include suspected cases in the differential diagnosis in clinical work.
Asunto(s)
Sarcoma de Células Dendríticas Foliculares , Linfoma , Humanos , Sarcoma de Células Dendríticas Foliculares/diagnóstico por imagen , Sarcoma de Células Dendríticas Foliculares/patología , Ganglios Linfáticos/patología , Diagnóstico Diferencial , Tomografía Computarizada por Rayos X , Linfoma/diagnóstico , Linfoma/patologíaRESUMEN
Fibroblastic reticular cell tumours (FRCT) originate from the fibroblastic reticular cells (FBRC) which are histiocytic cells, belonging to the dendritic cell family. These tumours are extremely rare, with only a few cases reported in literature. Histomorphologically, they resemble follicular dendritic cell sarcoma (FDCS); however, they differ immunophenotypically. Extranodal presentations are rare. We report a case of malignant FBRC tumour of the left eyelid, in a 23-year-old woman, who had presented with a recurrent swelling over left lower eyelid. Microscopy revealed an ill circumbscribed tumour composed of oval to spindle cells in storiform pattern, sprinkled with lymphocytes. Immunohistochemistry was performed and diagnosis of FRCT was offered. To the best of our knowledge, this is the first report of malignant FBRC tumour arising in the eyelid region. Here we present this extremely rare case with review of the available literature.
Asunto(s)
Sarcoma de Células Dendríticas Foliculares , Neoplasias , Femenino , Humanos , Adulto Joven , Adulto , Sarcoma de Células Dendríticas Foliculares/patología , Inmunohistoquímica , MicroscopíaRESUMEN
Follicular dendritic cell sarcoma (FDCS) is a rare tumor, which mainly originates from follicular dendritic cells (FDCs) in the lymph nodes. Sometimes FDCS can arise from outside the lymph nodes. FDCS is an extremely rare malignant tumor in intraperitoneal or retroperitoneal tissue. We gathered the detailed clinical data of three patients diagnosed with FDCS in the abdomen. The clinical observations and histopathologic and immunohistochemical features of FDCS were analyzed. The patients included two men and one woman aged 55 ~ 61 years old. The mesentery of the small intestine and colon was involved in case 1, spleen in case 2, and retroperitoneal tissues in case 3. Two patients presented with abdominal masses, and one presented with no obvious symptoms. Histology showed ovoid to spindle neoplastic cells arranged in fascicles and storiforms with inflammatory infiltrate as well as whorled patterns in some areas. Immunohistochemical staining was positive for CD21, CD23, CD35, and SSTR2. FDCS exhibits no characteristic clinical manifestations. Morphologically, FDCS can have overlapping features with many other entities, leading to misdiagnosis. The use of histopathology supplemented with FDC markers, such as CD21, CD23, and CD35, is useful for diagnosis and differential diagnosis.
Asunto(s)
Sarcoma de Células Dendríticas Foliculares , Masculino , Femenino , Humanos , Persona de Mediana Edad , Sarcoma de Células Dendríticas Foliculares/diagnóstico , Sarcoma de Células Dendríticas Foliculares/patología , Células Dendríticas Foliculares/patología , Ganglios Linfáticos/patología , Intestino Delgado/patología , Diagnóstico DiferencialRESUMEN
BACKGROUND: Epstein-Barr virus-positive inflammatory follicular dendritic cell sarcoma (EBV+IFDCS) is a rare disease characterized by mild clinical symptoms and non-specific imaging findings. The diagnosis of the disease depends on pathological diagnosis. However, EBV+IFDCS has a very broad spectrum of histological morphology and immune phenotypes, and its histopathological features have not been fully described by pathologists. CASE PRESENTATION: A 59-year-old female, with no significant discomfort, was found to have a splenic mass during a routine physical examination. Microscopic examination at low magnification revealed numerous epithelioid granulomas, amidst which a substantial inflammatory response was observed. Interspersed among the dense inflammatory cells were spindle or oval-shaped cells, distributed sporadically with indistinct boundaries. Under high magnification, these spindle cells had subtle features: smooth and clear nuclear membranes, inconspicuous small nucleoli, and infrequent mitotic figures. Immunophenotypically, the spindle cells expressed CD21 and CD23, and Epstein-Barr encoding region (EBER) in situ hybridization yielded positive results. The inflammatory milieu predominantly consisted of T cells, with a minority of plasma cells expressing IgG4. The confluence of morphological and immunohistochemical findings led to the final pathological diagnosis of EBV+IFDCS in this case. CONCLUSIONS: The presentation of EBV+IFDCS with pronounced granulomatous changes is rare. This morphological variant poses a high risk of misdiagnosis, frequently leading to confusion with other granulomatous diseases. Accurate diagnosis necessitates a comprehensive analysis, integrating immunohistochemistry and in situ hybridization. The case presented here is instrumental in raising awareness and understanding of EBV+IFDCS, with the goal of reducing misdiagnoses and unrecognized cases.
Asunto(s)
Sarcoma de Células Dendríticas Foliculares , Infecciones por Virus de Epstein-Barr , Granuloma de Células Plasmáticas , Neoplasias de los Tejidos Blandos , Femenino , Humanos , Persona de Mediana Edad , Sarcoma de Células Dendríticas Foliculares/diagnóstico , Sarcoma de Células Dendríticas Foliculares/patología , Herpesvirus Humano 4/genética , Infecciones por Virus de Epstein-Barr/complicaciones , Infecciones por Virus de Epstein-Barr/diagnóstico , Infecciones por Virus de Epstein-Barr/patología , Granuloma de Células Plasmáticas/diagnóstico , Granuloma/diagnósticoRESUMEN
The 5th edition of the World Health Organization classification of hematolymphoid tumors (WHO Blue Book) is soon to be published. Significant revisions have been made in the chapters on histiocytic/dendritic cell neoplasms and stroma-derived neoplasms of lymphoid tissues, leading to the reclassification and renaming of specific diseases. This article provides a concise interpretation and summary of these updates, highlighting the differences from the fourth edition. Pertinent changes from clinical pathological diagnosis to treatment and prognosis are explored, with an emphasis on recent advancements in molecular genetics. Newly introduced disease classifications are discussed, and the section on follicular dendritic cell sarcoma contributed by the author is detailed to assist readers in quickly understanding and assimilating the new classification standards.
Asunto(s)
Sarcoma de Células Dendríticas Foliculares , Neoplasias de los Tejidos Blandos , Humanos , Tejido Linfoide/patología , Neoplasias de los Tejidos Blandos/patología , Sarcoma de Células Dendríticas Foliculares/genética , Sarcoma de Células Dendríticas Foliculares/patología , Células Dendríticas/patología , Organización Mundial de la SaludRESUMEN
Follicular dentritic cell sarcomatous neoplasms originate from dendritic cells contained within the lymph nodes. In extranodal location, these neoplasms, are rare clinical entities, and even more so, their location in the head-neck region is extremely rare. Only 17 cases of primary dendritic cell sarcoma of the tonsil are reported in the literature at present. Being such a rare entity, histopathological diagnosis can be complex and requires great expertise and proper immunohistochemical analysis [1]. We present a case of a 48-year-old young man diagnosed with follicular dendritic cell sarcoma of the tonsil who underwent, probably for the first time in the literature, transoral robotic surgical resection.
Asunto(s)
Sarcoma de Células Dendríticas Foliculares , Sarcoma , Neoplasias Tonsilares , Masculino , Humanos , Persona de Mediana Edad , Sarcoma de Células Dendríticas Foliculares/diagnóstico , Sarcoma de Células Dendríticas Foliculares/cirugía , Sarcoma de Células Dendríticas Foliculares/patología , Tonsila Palatina/cirugía , Tonsila Palatina/patología , Neoplasias Tonsilares/cirugía , Neoplasias Tonsilares/patología , Sarcoma/patología , InmunohistoquímicaRESUMEN
The 5th edition of the World Health Organization classification of hematolymphoid tumors (WHO Blue Book) is soon to be published. Significant revisions have been made in the chapters on histiocytic/dendritic cell neoplasms and stroma-derived neoplasms of lymphoid tissues, leading to the reclassification and renaming of specific diseases. This article provides a concise interpretation and summary of these updates, highlighting the differences from the fourth edition. Pertinent changes from clinical pathological diagnosis to treatment and prognosis are explored, with an emphasis on recent advancements in molecular genetics. Newly introduced disease classifications are discussed, and the section on follicular dendritic cell sarcoma contributed by the author is detailed to assist readers in quickly understanding and assimilating the new classification standards.
Asunto(s)
Humanos , Tejido Linfoide/patología , Neoplasias de los Tejidos Blandos/patología , Sarcoma de Células Dendríticas Foliculares/patología , Células Dendríticas/patología , Organización Mundial de la SaludRESUMEN
ABSTRACT: Follicular dendritic cell sarcoma of the gastrointestinal tract is a rare malignancy. We describe contrast-enhanced CT and FDG PET/CT findings in a case of jejunal follicular dendritic cell sarcoma with hepatic metastasis. The primary jejunal tumor showed inhomogeneous enhancement on contrast-enhanced CT and intense FDG uptake on PET/CT mimicking adenocarcinoma or gastrointestinal stromal tumor. The hepatic metastatic tumor showed hypovascularity on contrast-enhanced CT and mild FDG uptake on PET/CT. This case indicates that follicular dendritic cell sarcoma should be included in the differential diagnosis of the hypermetabolic intestinal lesions.
Asunto(s)
Sarcoma de Células Dendríticas Foliculares , Neoplasias del Yeyuno , Neoplasias Hepáticas , Sarcoma de Células Dendríticas Foliculares/diagnóstico por imagen , Sarcoma de Células Dendríticas Foliculares/patología , Neoplasias del Yeyuno/diagnóstico por imagen , Neoplasias del Yeyuno/patología , Neoplasias Hepáticas/secundario , Tomografía Computarizada por Tomografía de Emisión de Positrones , Humanos , Masculino , Persona de Mediana EdadRESUMEN
RATIONALE: Follicular dendritic cell sarcoma (FDCS) is a rare mesenchymal tumor that typically develops in lymph nodes; it is clinically uncommon and has only occasionally been documented in cases of soft tissue, liver and spleen, and retroperitoneum; it is also extremely uncommon to develop in the stomach. PATIENT CONCERNS: A 64-year-old woman who discovered a lump in her left upper abdomen 6 months prior and was taken to the hospital due to excruciating abdominal pain. DIAGNOSIS: An abdominal computed tomography scan showed a soft tissue mass around the cardia. The immunohistochemical and postoperative histopathology results were compatible with FDCS. INTERVENTIONS: The patient underwent "radical total gastrectomy and esophagojejunostomy" (Roux-Y anastomosis). OUTCOMES: The patient recovered well 2 months after surgery. LESSONS: We report a case of FDCS occurring in the stomach and abdominal cavity, which was unique in terms of clinical location, clinical presentation, and imaging signs. This case report aims to enhance clinicians' understanding and diagnosis of FDCS in the stomach and abdominal cavity and reduce the rate of clinical misdiagnosis.
Asunto(s)
Cavidad Abdominal , Sarcoma de Células Dendríticas Foliculares , Humanos , Femenino , Persona de Mediana Edad , Sarcoma de Células Dendríticas Foliculares/diagnóstico , Sarcoma de Células Dendríticas Foliculares/cirugía , Sarcoma de Células Dendríticas Foliculares/patología , Estómago/patología , Abdomen/patología , Cavidad Abdominal/patología , Gastrectomía/métodosRESUMEN
Background: Follicular dendritic cell (FDC) sarcoma is an uncommon mesenchymal origin neoplasm derived from the abnormal proliferation and differentiation of FDCs. EpsteinâBarr virus-positive inflammatory follicular dendritic cell sarcoma (EBV+ iFDCS), which used to be known as the inflammatory pseudotumour (IPT)-like variant, occurs exclusively in the liver and spleen and has rarely been reported in the gastrointestinal tract. Case study: Here, we report a case of a 52-year-old woman with a special family history undergoing a routine physical examination. The colonoscope revealed an approximately 18 mm transverse colonic polyp, and the endoscopic polypectomy was performed. Microscopically, the excised polypoid mass was composed predominantly of inflammatory cells scattered with atypical ovoid to spindle tumor cells. Interestingly, there was a remarkable infiltration of IgG4+ cells. Immunohistochemistry showed that the tumor cells were positive for CD21, CD23 and CD35. EBV-encoded mRNA (EBER) in situ hybridization also gave positive signals. These histopathology features supported the diagnosis of EBV+ iFDCS. The patient was free of disease over 1-year follow-up. Conclusion: Identification of the potential pathogenesis sites of EBV+ iFDCS in extra-hepatosplenic regions is necessary for correct and timely diagnosis, and we consider it very meaningful to share our experience of diagnosing this tumor type. Furthermore, we summarize the clinicopathological features of EBV+ iFDCS presenting as a colon polyp after a thorough review of the literature.
Asunto(s)
Pólipos del Colon , Sarcoma de Células Dendríticas Foliculares , Infecciones por Virus de Epstein-Barr , Femenino , Humanos , Persona de Mediana Edad , Sarcoma de Células Dendríticas Foliculares/diagnóstico , Sarcoma de Células Dendríticas Foliculares/metabolismo , Sarcoma de Células Dendríticas Foliculares/patología , Herpesvirus Humano 4/genética , Infecciones por Virus de Epstein-Barr/complicaciones , Infecciones por Virus de Epstein-Barr/diagnóstico , Pólipos del Colon/diagnóstico , Hígado/patologíaRESUMEN
ABSTRACT: Follicular dendritic cell sarcoma (FDCS) is a low-grade sarcoma of mesenchymal dendritic cell origin, and it constitutes <0.4% of soft tissue sarcomas. We report a rare case of FDCS in a 32-year-old man. 18 F-FDG PET/CT demonstrated the involvement of cervical, axillary, mediastinal, abdominal, and pelvic groups of lymph nodes and spleen. A cervical lymph node biopsy suggested FDCS. 18 F-FDG PET/CT scan done after 3 cycles of chemotherapy (CHOP regime) revealed a complete metabolic response. This case presents the rarity of extensive presentation and complete response to the CHOP regime.
Asunto(s)
Sarcoma de Células Dendríticas Foliculares , Tomografía Computarizada por Tomografía de Emisión de Positrones , Masculino , Humanos , Adulto , Fluorodesoxiglucosa F18 , Sarcoma de Células Dendríticas Foliculares/diagnóstico por imagen , Sarcoma de Células Dendríticas Foliculares/tratamiento farmacológico , Sarcoma de Células Dendríticas Foliculares/patología , Tomografía de Emisión de Positrones , Ganglios Linfáticos/diagnóstico por imagen , Ganglios Linfáticos/patologíaRESUMEN
Follicular dendritic cell sarcoma (FDCS) is a rare malignant neoplasm, postulated to arise from follicular dendritic cells, with approximately 343 reported cases. Less than 100 cases of FDCS were in the gastrointestinal tract, with only four cases described in the stomach, none of them diagnosed on fine needle aspiration (FNA) cytology. We report here the first case of FDCS of the stomach diagnosed on FNA. Our patient is a 31-year-old male who presented with several years history of intermittent abdominal pain prompting occasional emergency-room visits. Imaging showed a 10.6 cm mass arising from the stomach, concerning for gastrointestinal stromal tumor. FNA cytology was performed using five passes with a 22-gauge needle. The smears were moderately cellular consisting of sheets and large, loosely cohesive clusters of ovoid to spindle cells with indistinct cytoplasmic borders and abundant cytoplasm, peppered with numerous small mature lymphocytes. The nuclei of the tumor cells were oval with finely granular chromatin with frequent nuclear grooves, pseudoinclusions, and easily recognizable mitotic figures. The tumor cells were positive for FDCS markers (CD21, CD23, and CD35).
Asunto(s)
Sarcoma de Células Dendríticas Foliculares , Tumores del Estroma Gastrointestinal , Masculino , Humanos , Adulto , Sarcoma de Células Dendríticas Foliculares/diagnóstico , Sarcoma de Células Dendríticas Foliculares/patología , Biopsia con Aguja Fina , Tumores del Estroma Gastrointestinal/diagnóstico , Tumores del Estroma Gastrointestinal/patología , Células Dendríticas Foliculares/patología , Estómago/patologíaRESUMEN
BACKGROUND: Follicular dendritic cell sarcoma is a malignant neoplasm derived from germinal center follicular dendritic cells, which both share a characteristic immunophenotype (namely CD21, CD23, and CD35). Cytomorphologic descriptions are few, consisting of only 26 prior cases from 24 publications. Identification by cytologic means appears challenging as the majority of previous reports disclose an erroneous or indeterminate initial cytologic diagnosis. Herein, we present the largest cytology series to date with the aim of expanding upon this small body of literature and discuss possible factors resulting in misinterpretation. MATERIALS AND METHODS: A retrospective search was conducted from 2 academic medical centers to identify histologically confirmed cases of follicular dendritic cell sarcoma with an associated cytologic component. Clinicopathologic data were tabulated and a comparative analysis of cytomorphologic and immunohistochemical features was performed. RESULTS: Seven separate cases were identified. All cases showed cohesive tumor cells with a characteristic voluminous, ill-defined cytoplasm with interconnecting fibrillary processes and intimately admixed mature lymphocytes. Features were maintained across various cytologic preparations, including conventional smear, liquid-based cytology, and touch imprint. Unusual immunohistochemical profiles were noted in a subset of cases. CONCLUSIONS: Cytomorphology is highly conserved across cases and preparations; however, a propensity for aberrant immunoexpression may contribute to diagnostic errors. Cytomorphologic features, supported by immunohistochemistry, suggest fine-needle aspiration as a reasonable diagnostic modality. Tumors with these features should include CD21, CD23, and/or CD35 in the workup.