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Rivera D, Wang WJ, Chan KH, Ali H, Wang W, Medeiros LJ, Hu Z. From the archives of MD Anderson Cancer Center Castleman disease involving the thymus gland: Case report and literature review. Ann Diagn Pathol 2023; 65:152136. [PMID: 37060884 DOI: 10.1016/j.anndiagpath.2023.152136] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/07/2023] [Revised: 04/05/2023] [Accepted: 04/06/2023] [Indexed: 04/17/2023]
Abstract
Castleman disease is a nodal based disease and very rarely involves the thymus gland. We report a 52-year-old man who was found incidentally to have a single thymic mass by computerized tomography scan. Thymectomy was performed, and the gross specimen showed a well-circumscribed, multi-loculated cystic mass. Histologic examination showed thymus involved by Castleman disease, hyaline-vascular variant. The lesion was characterized by lymphoid follicles with wide mantle zones, variably lymphocyte-depleted germinal centers with sclerotic radial blood vessels, and prominent interfollicular/stromal changes including numerous endothelial venules with sclerotic walls and hyaline sclerosis, scattered and frequent dysplastic follicular dendritic cells and foci of dystrophic calcification. Immunohistochemical analysis showed that the follicle mantle zones were composed of numerous B-cells positive for CD20, PAX5, and IgD. Antibodies specific for CD21 and CD23 highlighted prominent follicular dendritic cell networks within follicles. There was no evidence of human herpes virus 8. We searched the literature and could identify only 10 additional cases of thymic CD. Previously reported cases included 8 unicentric and 2 multicentric, classified pathologically as plasma cell variant (n = 4), hyaline vascular variant (n = 3), and mixed (n = 3). Thymectomy, as was done in the currently reported case, most often leads to the diagnosis of Castleman disease and was a mainstay of treatment in other reported cases.
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Affiliation(s)
- Daniel Rivera
- Department of Pathology and Laboratory Medicine, The University of Texas Health Science Center at Houston, TX, United States of America
| | - Wei J Wang
- Department of Pathology and Laboratory Medicine, The University of Texas Health Science Center at Houston, TX, United States of America
| | - Kok Hoe Chan
- Department of Medicine, The University of Texas Health Science Center at Houston, TX, United States of America
| | - Haval Ali
- Department of Pathology and Laboratory Medicine, The University of Texas Health Science Center at Houston, TX, United States of America
| | - Wei Wang
- Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, United States of America
| | - L Jeffrey Medeiros
- Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, United States of America
| | - Zhihong Hu
- Department of Laboratory Medicine, The University of Texas MD Anderson Cancer Center, Houston, TX, United States of America.
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Facchetti F, Simbeni M, Lorenzi L. Follicular dendritic cell sarcoma. Pathologica 2021; 113:316-329. [PMID: 34837090 PMCID: PMC8720404 DOI: 10.32074/1591-951x-331] [Citation(s) in RCA: 33] [Impact Index Per Article: 8.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/21/2021] [Accepted: 06/21/2021] [Indexed: 12/17/2022] Open
Abstract
Follicular dendritic cells (FDC) are mesenchymal-derived dendritic cells located in B-follicles where they play a pivotal role in triggering and maintaining B-cell adaptive immune response. In 1986 Dr. Juan Rosai first reported a series of neoplasms showing features of FDC and defined it as Follicular Dendritic Cell Tumor, subsequently renamed as "sarcoma" (FDCS). In its seminal and subsequent articles Rosai and colleagues highlighted the heterogeneous microscopic appearance of FDCS and its immunohistochemical and ultrastructural features. FDCS mostly occurs in extranodal sites (79.4% of cases) and lymph nodes (15.1%); in about 7%-10% of cases it is associated with hyaline-vascular Castleman disease. Given its significant growth pattern and cytological variability, FDCS can be confused with various neoplasms and even inflammatory processes. The diagnosis requires the use of a broad spectrum of FDC markers (e.g. CD21, CD23, CD35, clusterin, CXCL13, podoplanin), particularly considering that tumor antigen-loss is frequent. The inflammatory-pseudotumor-like (IPT-like) variant of FDCS, in addition to its peculiar histopathological and clinical features, is characterized by positivity of tumor cells for Epstein-Barr virus, representing a diagnostic requisite. No distinctive genetic and molecular anomalies have been identified in FDCS. It often carries an aberrant clonal karyotype and chromosomal structural alterations, frequently involving onco-suppressor genes. Direct or next generation sequencing showed alterations on genes belonging to the NF-κB regulatory pathway and cell-cycle regulators. In contrast to hematopoietic-derived histiocytic and dendritic cells tumors, FDCS typically lacks mutations in genes related to the MAPK pathway. FDCS recurs locally in 28% and metastasizes in 27% of cases. Extent of the disease, surgical resectability and histopathological features are significantly associated with the outcome. IPT-like FDCS behaves as an indolent tumor, even if it often recurs locally over years. Complete surgical excision is the gold standard of treatment. Data on targeted therapies (e.g.: tyrosine kinase inhibitors) or immune checkpoint inhibitors are very limited and responses are variable. A better understanding of the molecular drivers of this tumor may lead to potential new therapeutic strategies.
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Affiliation(s)
- Fabio Facchetti
- Department of Molecular and Translational Medicine, Section of Pathology, University of Brescia, Spedali Civili, Brescia (Italy)
| | - Matteo Simbeni
- Department of Molecular and Translational Medicine, Section of Pathology, University of Brescia, Spedali Civili, Brescia (Italy)
| | - Luisa Lorenzi
- Department of Molecular and Translational Medicine, Section of Pathology, University of Brescia, Spedali Civili, Brescia (Italy)
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Abstract
Myopia, also known as short-sightedness or near-sightedness, is a very common condition that typically starts in childhood. Severe forms of myopia (pathologic myopia) are associated with a risk of other associated ophthalmic problems. This disorder affects all populations and is reaching epidemic proportions in East Asia, although there are differences in prevalence between countries. Myopia is caused by both environmental and genetic risk factors. A range of myopia management and control strategies are available that can treat this condition, but it is clear that understanding the factors involved in delaying myopia onset and slowing its progression will be key to reducing the rapid rise in its global prevalence. To achieve this goal, improved data collection using wearable technology, in combination with collection and assessment of data on demographic, genetic and environmental risk factors and with artificial intelligence are needed. Improved public health strategies focusing on early detection or prevention combined with additional effective therapeutic interventions to limit myopia progression are also needed.
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Nakamura T, Yoshihara M, Tamauchi S, Kajiyama H, Kikkawa F. Follicular dendritic cell sarcoma of the uterine cervix: a case report. BMC WOMENS HEALTH 2020; 20:182. [PMID: 32807169 PMCID: PMC7433043 DOI: 10.1186/s12905-020-01045-y] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 04/26/2020] [Accepted: 08/04/2020] [Indexed: 11/19/2022]
Abstract
Background Follicular dendritic cell sarcoma (FDCS) is a rare mesenchymal tumor that mostly occurs in systemic lymph nodes. FDCS in the uterine cervix has not yet been reported. Case presentation A 49-year-old woman was referred to our department with a cervical tumor, which was histologically suspected to be undifferentiated carcinoma. She underwent hysterectomy, salpingo-oophorectomy, and pelvic lymphadenectomy after neoadjuvant chemotherapy with paclitaxel and carboplatin. The resected specimen contained high numbers of spindle cells and was immunohistochemically confirmed to be FDCS. The tumor was completely resected and recurrence was not detected at a 16-month follow-up. Conclusion FDCS is an extremely rare malignant tumor in the uterine cervix, and an accurate diagnosis and complete resection are essential for a good prognosis.
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Affiliation(s)
- Takuto Nakamura
- Department of Obstetrics and Gynecology, Anjo Kosei Hospital, Anjo, Aichi, Japan
| | - Masato Yoshihara
- Department of Obstetrics and Gynecology, Nagoya University Graduate School of Medicine, 65, Tsuruma-cho, Showa-ku, Nagoya, Aichi, Japan.
| | - Satoshi Tamauchi
- Department of Obstetrics and Gynecology, Nagoya University Graduate School of Medicine, 65, Tsuruma-cho, Showa-ku, Nagoya, Aichi, Japan
| | - Hiroaki Kajiyama
- Department of Obstetrics and Gynecology, Nagoya University Graduate School of Medicine, 65, Tsuruma-cho, Showa-ku, Nagoya, Aichi, Japan
| | - Fumitaka Kikkawa
- Department of Obstetrics and Gynecology, Nagoya University Graduate School of Medicine, 65, Tsuruma-cho, Showa-ku, Nagoya, Aichi, Japan
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Li P, Liu H, Li H, Li A, Yu G, Yin W. Hyaline vascular variant of unicentric Castleman disease of the tonsil: a case report. Diagn Pathol 2019; 14:70. [PMID: 31253137 PMCID: PMC6599372 DOI: 10.1186/s13000-019-0836-y] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/16/2019] [Accepted: 06/06/2019] [Indexed: 11/10/2022] Open
Abstract
Background Castleman disease (CD) is a lymphoproliferative disorder with an unknown etiology. The disease may be unicentric (UCD) or multicentric (MCD), and three histopathologic variants have been described: hyaline vascular (HV), plasma cell (PC), and mixed type. Extranodal CD is rare. Herein, we report a case of CD presenting as a tonsillar mass, which has not been documented in the literature. Case presentation The patient was a 32-year-old man. Laryngoscopy revealed tonsillar hypertrophy, and the patient underwent a low-temperature plasma tonsillectomy. Microscopic examination of permanent sections showed lymphoid follicular hyperplasia, a portion of which appeared to be a fusion of nodular hyperplasia (composed of lymphoid follicles of variable size and shape). These distinctive follicles with atrophic hyalinized germinal centers and a broad mantle zone of small lymphocytes formed concentric rings (so-called onion-skin arrangement). Medium-sized vessels and a plethora of capillaries were present in the center of the lymphatic follicles, mantle zones, and interfollicular areas. A characteristic lollipop appearance was also observed due to the onion-skin arrangement of the expanded mantle zone lymphocytes with a vessel penetrating the germinal center. No aberrant lymphoid population was present based on CD3, CD5, CD20, CD79α, CD21, CD23, bcl-2, cyclin D1, and ki-67 immunostaining. Tests for human herpesvirus (HHV)-8 and Epstein Barr virus (EBV)-encoded small RNA (EBER) were negative. Therefore, a diagnosis of an HV variant UCD was rendered. The patient was treated by local excision without any other therapy based on the diagnosis. At the 7-month follow up, the patient had no recurrent symptoms or masses. Conclusion We present an unusual case of a tonsil presenting hyaline vascular Castleman disease (HVCD). This study aims to highlight CD as a differential diagnosis that should be considered by otolaryngologists and pathologists for lymphoproliferative disorders of the tonsil.
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Affiliation(s)
- Ping Li
- Department of Pathology, Shenzhen Hospital of Peking University, 1120 Lianhua road, Shenzhen, 518036, China
| | - Huaipu Liu
- Department of Cardiothoracic Surgery, Shenzhen Children's Hospital, 7019 Yitian road, Shenzhen, 518038, China
| | - Hao Li
- Department of Pathology, Shenzhen Hospital of Peking University, 1120 Lianhua road, Shenzhen, 518036, China
| | - Ang Li
- Department of Pathology, Shenzhen Hospital of Peking University, 1120 Lianhua road, Shenzhen, 518036, China
| | - Guangyin Yu
- Department of Pathology, Shenzhen Hospital of Peking University, 1120 Lianhua road, Shenzhen, 518036, China
| | - Weihua Yin
- Department of Pathology, Shenzhen Hospital of Peking University, 1120 Lianhua road, Shenzhen, 518036, China.
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Abstract
Castleman disease (CD) is divided clinically into unicentric or multicentric type. Pathologically, CD is divided into hyaline-vascular and plasma cell variants. Unicentric CD is most common, about 75% of these cases are hyaline-vascular variant, and surgical excision is often curative. In contrast, there are a number of types of multicentric CD including HHV8-associated, idiopathic, and a subset of cases that arise in association with POEMS syndrome. Therapy is required for most patients with multicentric CD, but there is no consensus approach currently. As is evidence, the designation Castleman disease encompasses a heterogeneous group of diseases of varied pathogenesis and which require different therapies.
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Affiliation(s)
- Wei Wang
- Department of Hematopathology, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX 77030, USA
| | - L Jeffrey Medeiros
- Department of Hematopathology, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX 77030, USA.
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Yan WX, Yu YX, Zhang P, Liu XK, Li Y. Follicular dendritic cell sarcoma detected in hepatogastric ligament: A case report and review of the literature. World J Clin Cases 2019; 7:116-121. [PMID: 30637261 PMCID: PMC6327130 DOI: 10.12998/wjcc.v7.i1.116] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/13/2018] [Revised: 10/16/2018] [Accepted: 10/22/2018] [Indexed: 02/05/2023] Open
Abstract
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 post-operation.
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Affiliation(s)
- Wen-Xin Yan
- Department of Hepatobiliary and Pancreatic Surgery, the First Hospital of Jilin University, Changchun 130021, Jilin Province, China
| | - You-Xi Yu
- Department of Hepatobiliary and Pancreatic Surgery, the First Hospital of Jilin University, Changchun 130021, Jilin Province, China
| | - Ping Zhang
- Department of Hepatobiliary and Pancreatic Surgery, the First Hospital of Jilin University, Changchun 130021, Jilin Province, China
| | - Xing-Kai Liu
- Department of Hepatobiliary and Pancreatic Surgery, the First Hospital of Jilin University, Changchun 130021, Jilin Province, China
| | - Yan Li
- Department of Surgery, School of Medicine, University of Louisville, Louisville, KY 40202, United States
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Wala SJ, Fallon EM, Forlenza CJ, Shukla N, LaQuaglia MP. Unicentric Castleman disease in the mediastinum. JOURNAL OF PEDIATRIC SURGERY CASE REPORTS 2018. [DOI: 10.1016/j.epsc.2018.05.005] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/16/2022] Open
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Piris MA, Aguirregoicoa E, Montes-Moreno S, Celeiro-Muñoz C. Castleman Disease and Rosai-Dorfman Disease. Semin Diagn Pathol 2018; 35:44-53. [DOI: 10.1053/j.semdp.2017.11.014] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/17/2022]
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Medina EA, Fuehrer NE, Miller FR, Kinney MC, Higgins RA. Dysplastic follicular dendritic cells in hyaline-vascular Castleman disease: a rare occurrence creating diagnostic difficulty. Pathol Int 2016; 66:535-9. [DOI: 10.1111/pin.12446] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/07/2016] [Revised: 06/28/2016] [Accepted: 07/20/2016] [Indexed: 11/28/2022]
Affiliation(s)
- Edward A. Medina
- Department of Pathology; University of Texas Health Science Center; San Antonio TX USA
| | - Neil E. Fuehrer
- Department of Pathology; University of Texas Health Science Center; San Antonio TX USA
| | - Frank R. Miller
- Department Otolaryngology-Head & Neck Surgery; University of Texas Health Science Center; San Antonio TX USA
| | - Marsha C. Kinney
- Department of Pathology; University of Texas Health Science Center; San Antonio TX USA
| | - Russell A. Higgins
- Department of Pathology; University of Texas Health Science Center; San Antonio TX USA
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12
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13
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Abstract
We report two cases of follicular dendritic cell tumor associated with Castleman's disease, one of the hyaline vascular type and one a mixed hyaline-vascular and plasma cell type. The reactive lymphoid follicles in both cases contained a proliferation of abnormal follicular dendritic cells characterized by enlarged, irregular contoured nuclei and prominent nucleoli (in-situ component). Both tumors had similar histology. They were vaguely fascicular and composed by eosinophilic, ovoid to spindled cells with vesicular nuclei and prominent nucleoli admixed with small lymphocytes. Some of these cells seen in the reactive follicles were similar in appearance to the ones seen in the adjacent invasive tumors. The association of follicular dendritic cell tumor with Castleman's disease and the presence of in-situ follicular dendritic cell tumor within reactive lymphoid follicles suggests that there may be a progression from dysplastic follicular dendritic cells to follicular dendritic cell tumor in Castleman's disease. The sequence of events and the likelihood that patients with Castleman's disease showing follicles with dysplastic changes will develop follicular dendritic cell tumors is not known at this time owing to the limited number of cases reported.
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Affiliation(s)
| | - Olivia Chan
- The Lillian and Henry M. Stratton-Hans Popper Department of Pathology, New York, NY
| | - James A. Strauchen
- Department of Pathology, Mount Sinai School of Medicine, One Gustave L. Levy Place, New York, NY 10029; Division of Neoplastic Diseases, Department of Medicine, Mount Sinai School of Medicine, New York, NY
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Viola P, Vroobel KM, Devaraj A, Jordan S, Ladas G, Dusmet M, Montero A, Rice A, Wotherspoon AC, Nicholson AG. Follicular dendritic cell tumour/sarcoma: a commonly misdiagnosed tumour in the thorax. Histopathology 2016; 69:752-761. [DOI: 10.1111/his.12998] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/28/2016] [Accepted: 05/18/2016] [Indexed: 12/17/2022]
Affiliation(s)
- Patrizia Viola
- Department of Histopathology; Royal Brompton and Harefield NHS Foundation Trust; London UK
| | | | - Anand Devaraj
- Department of Imaging; Royal Brompton and Harefield NHS Foundation Trust; London UK
| | - Simon Jordan
- Department of Thoracic Surgery; Royal Brompton and Harefield NHS Foundation Trust; London UK
| | - George Ladas
- Department of Thoracic Surgery; Royal Brompton and Harefield NHS Foundation Trust; London UK
| | - Michael Dusmet
- Department of Thoracic Surgery; Royal Brompton and Harefield NHS Foundation Trust; London UK
| | - Angeles Montero
- Department of Histopathology; Royal Brompton and Harefield NHS Foundation Trust; London UK
- National Heart and Lung Institute; Imperial College; London UK
| | - Alexandra Rice
- Department of Histopathology; Royal Brompton and Harefield NHS Foundation Trust; London UK
- National Heart and Lung Institute; Imperial College; London UK
| | | | - Andrew G Nicholson
- Department of Histopathology; Royal Brompton and Harefield NHS Foundation Trust; London UK
- National Heart and Lung Institute; Imperial College; London UK
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Vassos N, Raptis D, Lell M, Klein P, Perrakis A, Köhler J, Croner RS, Hohenberger W, Agaimy A. Intra-abdominal localized hyaline-vascular Castleman disease: imaging characteristics and management of a rare condition. Arch Med Sci 2016; 12:227-32. [PMID: 26925142 PMCID: PMC4754385 DOI: 10.5114/aoms.2016.57600] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/26/2014] [Accepted: 06/25/2014] [Indexed: 11/21/2022] Open
Affiliation(s)
- Nikolaos Vassos
- Department of Surgery, University Hospital Erlangen, Erlangen, Germany
| | - Dimitrios Raptis
- Department of Surgery, University Hospital Erlangen, Erlangen, Germany
| | - Michael Lell
- Institute of Diagnostic and Interventional Radiology, University Hospital Erlangen, Erlangen, Germany
| | - Peter Klein
- Department of Surgery, University Hospital Erlangen, Erlangen, Germany
| | | | - Jens Köhler
- Department of Surgery, Nuremberg Medical Center, Nuremberg, Germany
| | - Roland S. Croner
- Department of Surgery, University Hospital Erlangen, Erlangen, Germany
| | | | - Abbas Agaimy
- Institute of Pathology, University Hospital Erlangen, Erlangen, Germany
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Extranodal Castleman disease of the extremities: a case report and review of the literature. Skeletal Radiol 2014; 43:1627-31. [PMID: 24970669 DOI: 10.1007/s00256-014-1945-z] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/24/2014] [Revised: 05/02/2014] [Accepted: 06/08/2014] [Indexed: 02/02/2023]
Abstract
Castleman disease is a rare lymphoproliferative disorder of unknown etiology that most commonly presents as a mediastinal nodal mass or, in the extranodal form of the disease, a mass located in the mediastinum or retroperitoneum. It is exceptionally uncommon for Castleman disease to present in the extremities. We report a rare case of extranodal Castleman disease presenting as a muscular forearm mass. We compare our case with the seven other reported cases in which Castleman disease presented as an isolated soft tissue mass in the extremities.
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Hwang SO, Lee TH, Bae SH, Cho HD, Choi KH, Park SH, Kim CH, Kim SJ. Transformation of Castleman's disease into follicular dendritic cell sarcoma, presenting as an asymptomatic intra-abdominal mass. THE KOREAN JOURNAL OF GASTROENTEROLOGY 2014; 62:131-4. [PMID: 23981949 DOI: 10.4166/kjg.2013.62.2.131] [Citation(s) in RCA: 16] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 12/19/2022]
Abstract
Follicular dendritic cell (FDC) sarcoma is an extremely rare malignant neoplasm arising from FDCs. The exact origin of FDCs remains unclear; both a hematopoietic lineage origin and a stromal cell derivation have been proposed. Proliferation of FDCs can lead to benign reactive lesions or generate neoplastic conditions. The lesions are most commonly found in lymph nodes and usually involve the head and neck area. Castleman's disease is a rare non-neoplasitic lymphoproliferative disorder. Rare cases of hyaline-vascular Castleman's disease have been associated with FDC sarcoma, but a clonal relationship has not been convincingly demonstrated. A pathway toward tumor evolution, beginning with hyperplasia and dysplasia of FDCs, has been proposed. Despite this known association between Castleman's disease and FDC sarcoma, there have only been few reported cases of sarcoma arising as a complication of pre-existing Castleman's disease, especially in abdominal lesions. We describe here a 51-year-old female with an FDC sarcoma arising from unicentric, hyaline-vascular type Castleman's disease in an intra-abdominal mass. Pathologically, the lesion showed a series of changes during the process of transformation from Castleman's disease to FDC sarcoma. (Korean J Gastroenterol 2013;62:131-134).
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Affiliation(s)
- Soon Oh Hwang
- Department of Internal Medicine, Digestive Disease Center, Soonchunhyang University Hospital Cheonan, Soonchunhyang University College of Medicine, 31 Suncheonhyang 6-gil, Dongnam-gu, Cheonan 330-903, Korea
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Chang KC, Wang YC, Hung LY, Huang WT, Tsou JH, M Jones D, Song HL, Yeh YM, Kao LY, Medeiros LJ. Monoclonality and cytogenetic abnormalities in hyaline vascular Castleman disease. Mod Pathol 2014; 27:823-31. [PMID: 24201121 DOI: 10.1038/modpathol.2013.202] [Citation(s) in RCA: 68] [Impact Index Per Article: 6.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/04/2013] [Accepted: 07/31/2013] [Indexed: 02/07/2023]
Abstract
Hyaline vascular Castleman disease is traditionally regarded as a reactive hyperplastic process. Occasional cases, however, have been reported with cytogenetic anomalies bringing this concept into question. In this study, we used conventional and methylation-specific polymerase chain reaction methods to assess the human androgen receptor α (HUMARA) gene in 29 female patients with hyaline vascular Castleman disease and compared the results with three cases of plasma cell Castleman disease and 20 cases of age-matched lymphoid hyperplasia. We also assessed for immunoglobulin gene and T-cell receptor gene rearrangements, and conventional cytogenetic analysis was performed in three cases of hyaline vascular Castleman disease. In cases with informative results, conventional and methylation-specific human androgen receptor α gene analyses yielded a monoclonal pattern in 10 of 19 (53%) and 17 of 23 (74%) cases of hyaline vascular Castleman disease, respectively. A monoclonal pattern was also detected in three cases of plasma cell Castleman disease but not in cases of lymphoid hyperplasia. The frequency of monoclonality was higher for lesions >5 cm in size (100%) and for the stromal-rich variant (91%). Cytogenetic abnormalities in stromal cells were revealed in two cases of hyaline vascular Castleman disease and no cases showed monoclonal immunoglobulin or T-cell receptor gene rearrangements. Follow-up data showed persistent disease in 4 of 23 (17%) patients. We conclude that hyaline vascular Castleman disease is often a monoclonal proliferation, most likely of lymph node stromal cells.
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Affiliation(s)
- Kung-Chao Chang
- Department of Pathology, College of Medicine, National Cheng Kung University and Hospital, Tainan, Taiwan
| | - Yu-Chu Wang
- Institute of Bioinformatics and Biosignal Transduction, College of Bioscience and Biotechnology, National Cheng Kung University, Tainan, Taiwan
| | - Liang-Yi Hung
- Institute of Bioinformatics and Biosignal Transduction, College of Bioscience and Biotechnology, National Cheng Kung University, Tainan, Taiwan
| | - Wan-Ting Huang
- Department of Pathology, Chang Gung Memorial Hospital-Kaohsiung Medical Center, Chang Gung University College of Medicine, Kaohsiung, Taiwan
| | - Jen-Hui Tsou
- Institute of Bioinformatics and Biosignal Transduction, College of Bioscience and Biotechnology, National Cheng Kung University, Tainan, Taiwan
| | - Dan M Jones
- School of Medicine, The University of Texas Anderson Cancer Center, Houston, TX, USA
| | - Hsiang-Lin Song
- Department of Pathology, College of Medicine, National Cheng Kung University and Hospital, Tainan, Taiwan
| | - Yu-Min Yeh
- Department of Internal Medicine, College of Medicine, National Cheng Kung University and Hospital, Tainan, Taiwan
| | - Lin-Yuan Kao
- Department of Pathology, College of Medicine, National Cheng Kung University and Hospital, Tainan, Taiwan
| | - L Jeffrey Medeiros
- Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA
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T-lymphoblastic proliferation and florid multifocal follicular dendritic cell proliferation occurring in hyaline-vascular Castleman disease in a patient with a possible familial predisposition. J Hematop 2013. [DOI: 10.1007/s12308-013-0184-x] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/26/2022] Open
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20
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Lorenzi L, Lonardi S, Petrilli G, Tanda F, Bella M, Laurino L, Rossi G, Facchetti F. Folliculocentric B-cell–rich follicular dendritic cells sarcoma: a hitherto unreported morphological variant mimicking lymphoproliferative disorders. Hum Pathol 2012; 43:209-15. [DOI: 10.1016/j.humpath.2011.02.029] [Citation(s) in RCA: 12] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/28/2011] [Revised: 02/18/2011] [Accepted: 02/24/2011] [Indexed: 10/17/2022]
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Pizzi M, Ludwig K, Palazzolo G, Busatto G, Rettore C, Altavilla G. Cervical Follicular Dendritic Cell Sarcoma: A Case Report and Review of the Literature. Int J Immunopathol Pharmacol 2011; 24:539-44. [DOI: 10.1177/039463201102400231] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/03/2023] Open
Abstract
Follicular dendritic cell (FDC) sarcoma is a rare tumour with a low-to-intermediate grade of malignancy. It frequently occurs in cervical, mediastinal and axillary lymph nodes. In approximately 30% of cases an extranodal localization has been reported (tonsils, oral cavity, mediastinum, liver, and spleen). Very little is known about possible treatment options and overall prognosis. This case reports a 66 year-old patient, who underwent surgical removal of a persistently enlarged right cervical lymph node. The histopathological examination revealed a spindle cell tumour with lymphocyte and plasma cell infiltrates. Neoplastic cells stained positive for CD21, CD23 and CD35, thus confirming the diagnosis of FDC sarcoma. The neoplasm recurred two years later and partial regression was achieved by IGEV rescue therapy. We briefly discuss clinical history, histopathological differential diagnosis and treatment options of FDC sarcoma.
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Affiliation(s)
- M. Pizzi
- Department of Diagnostic Medical Sciences and Special Therapies University of Padova
| | - K. Ludwig
- Department of Diagnostic Medical Sciences and Special Therapies University of Padova
| | - G. Palazzolo
- Division of Medical Oncology, U.L.S.S. 15 “Alta Padovana”, Cittadella
| | - G. Busatto
- Department of Anatomic Pathology, U.L.S.S. 15 “Alta Padovana”, Cittadella
| | - C. Rettore
- Division of Radiology, U.L.S.S. 15 “Alta Padovana”, Cittadella, Padova, Italy
| | - G. Altavilla
- Department of Diagnostic Medical Sciences and Special Therapies University of Padova
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22
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Hyaline-vascular castleman disease: a rare cause of solitary subcutaneous soft tissue mass. Am J Dermatopathol 2010; 32:293-297. [PMID: 20098298 DOI: 10.1097/dad.0b013e3181b7269a] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022]
Abstract
Castleman disease (CD) is a rare lymphoproliferative disorder that primarily affects mediastinal, retroperitoneal, and cervical lymph nodes. Clinically, these lesions occur as a localized (unicentric) or less frequently as a systemic (multicentric) disease. Two main distinct histologic variants are recognized, the more common hyaline-vascular (HV) type and the plasma cell (PC) type. Extranodal Castleman disease, HV type (HVCD) is even less common. We describe a case of subcutaneous HVCD in a 57-year-old woman with a palpable chest mass and without systemic symptoms. Although the histologic findings are similar to those of HVCD in lymph nodes and other sites, a plethora of differential diagnosis is raised particularly with the more commonly occurring lymphoproliferative lesions in this location. This is one of the few bona fide cases of HVCD in subcutaneous location published to date. A review of the literature with an emphasis on pathogenesis of the disease subtypes is presented.
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Seo HY, Kim EB, Kim JW, Shin BK, Kim SJ, Kim BS. Complete remission in a patient with human herpes virus-8 negative multicentric Castleman disease using CHOP chemotherapy. Cancer Res Treat 2009; 41:104-7. [PMID: 19707509 DOI: 10.4143/crt.2009.41.2.104] [Citation(s) in RCA: 13] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/02/2009] [Accepted: 03/11/2009] [Indexed: 01/08/2023] Open
Abstract
Multicentric Castleman disease (MCD) is a rare lymphoproliferative disorder. Although MCD pathogenesis is unclear, studies have suggested that human herpesvirus 8 (HHV-8) may be associated with the disorder. Recent reports have identified MCD cases without viral infection. A 43-year-old woman presented to our hospital for fever and myalgia of 6 months' duration. The complete blood count revealed an elevated leukocyte count (15.1x10(3)/microL) and a decreased hemoglobin level of 10.0 g/dL. The C-reactive protein level was elevated at 276.5 mg/L. Thoracic computed tomography (CT) scans revealed bilateral axillary lymphadenopathy. There was no evidence of HHV-8, human immunodeficiency virus (HIV), or Mycobacterium infection. Histologic evaluation of a lymph node biopsy from the left axilla yielded a diagnosis of MCD. Cyclophosphamide, adriamycin, vincristine, and prednisone (CHOP) were administered for a total of 4 cycles. The patient's fever and lymphadenopathy resolved after the course of chemotherapy. She has been in complete remission for 24 months at this writing. As previously reported, this case report suggests that MCD can develop without viral infection. CHOP chemotherapy may be an effective treatment option for newly diagnosed MCD patients.
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Affiliation(s)
- Hee Yeon Seo
- Division of Oncology and Hematology, Department of Internal Medicine, Korea University College of Medicine, Seoul, Korea
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24
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Ono Y, Terashima K, Liu A, Yokoyama M, Yokoshima K, Mizukami M, Watanabe K, Mochimaru Y, Furusaka T, Shimizu N, Yamamoto N, Ishiwata T, Sugisaki Y, Yagi T, Naito Z. Follicular dendritic cell sarcoma with microtubuloreticular structure and virus-like particle productionin vitro. Pathol Int 2009; 59:332-44. [DOI: 10.1111/j.1440-1827.2009.02375.x] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/30/2022]
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25
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Youens KE, Waugh MS. Extranodal follicular dendritic cell sarcoma. Arch Pathol Lab Med 2008; 132:1683-7. [PMID: 18834231 DOI: 10.5858/2008-132-1683-efdcs] [Citation(s) in RCA: 40] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 03/06/2008] [Indexed: 11/06/2022]
Abstract
Extranodal follicular dendritic cell sarcoma is a rare tumor of follicular dendritic cells that can occur in a wide variety of sites. Although fairly well characterized histologically, with a distinct immunophenotype, it remains under-recognized, with as many as one third of cases initially misdiagnosed. This is often due to a failure to consider the entity. Patients with this tumor may have a worse prognosis than originally described. Prompted by a recent case at our institution, we briefly review the clinical features, etiology, histologic, and cytologic appearance of the tumor, as well as the ancillary studies useful in resolving diagnostic issues, primarily in an attempt to increase recognition of this rare neoplasm.
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Affiliation(s)
- Kenneth E Youens
- Department of Pathology, Duke University Medical Center, Durham, NC 27710, USA.
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26
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Vasudev Rao T, Alkindi S, Pathare AV. Follicular dendritic cell hyperplasia in plasma cell variant of Castleman's disease with interfollicular Hodgkin's disease. Pathol Res Pract 2007; 203:479-84. [PMID: 17418501 DOI: 10.1016/j.prp.2006.12.006] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/01/2006] [Revised: 12/05/2006] [Accepted: 12/06/2006] [Indexed: 10/23/2022]
Abstract
We report a case of a multicentric plasma cell (PC) variant of Castleman's disease (CD) in association with interfollicular type of classic Hodgkin's disease (HD), both diseases identified in the same lymph node. The histologic features of CD were the classic ones, with hyperplastic and atrophic follicles, some with prominent mantle zones, hyalinzed vessels, and a very rich polyclonal proliferation of PCs in the interfollicular region. The presence of LCA-negative, but CD30- and CD15-positive typical and atypical Reed-Sternberg (RS) cells in the interfollicular region confirmed the presence of HD. In addition, many of the RS cells stained positive for EBV. CD35- and CD21-positive follicular dendritic cell (FDC) hyperplasia was a striking feature, a finding that has not been well documented in the PC variant of CD.
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Affiliation(s)
- T Vasudev Rao
- Department of Pathology, College of Medicine and Health Sciences, Sultan Qaboos University, Alkhoud, Muscat, Oman
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27
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Choi JW, Lee JH, Kim A, Kim CH, Chae YS, Kim I. Follicular dendritic cell sarcoma arising in the dura mater of the spine. Arch Pathol Lab Med 2006; 130:1718-21. [PMID: 17076538 DOI: 10.5858/2006-130-1718-fdcsai] [Citation(s) in RCA: 11] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 04/20/2006] [Indexed: 11/06/2022]
Abstract
There have been some individual case reports and a few large series of reports describing the clinicopathologic features of follicular dendritic cell sarcoma. This tumor originates from the dendritic cells of lymphoid follicles and is extremely rare in the extranodal location. We report the case of a 68-year-old man who presented with low back pain. A mass was found in the lumbar dura mater and extended to the right epidural space. The tumor was composed of nodules, sheets, and interlacing fascicles of oval-to-spindle cells intermingled with the dense infiltrates of small lymphocytes. Immunohistochemical staining revealed that the tumor cells were positive for CD21, CD23, CD35, and clusterin, and focally positive for CD68, CD20, and CD79a. To our knowledge, this is the first case of follicular dendritic cell sarcoma occurring in the dura mater of the spine.
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Affiliation(s)
- Jung Woo Choi
- Department of Pathology, College of Medicine, Korea University, Seoul, Korea
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28
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Shia J, Chen W, Tang LH, Carlson DL, Qin J, Guillem JG, Nobrega J, Wong WD, Klimstra DS. Extranodal follicular dendritic cell sarcoma: clinical, pathologic, and histogenetic characteristics of an underrecognized disease entity. Virchows Arch 2006; 449:148-58. [PMID: 16758173 DOI: 10.1007/s00428-006-0231-4] [Citation(s) in RCA: 71] [Impact Index Per Article: 3.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/24/2006] [Accepted: 05/05/2006] [Indexed: 12/19/2022]
Abstract
It has been more than 10 years since follicular dendritic cell (FDC) sarcoma was first reported to occur in extranodal sites, yet extranodal FDC sarcoma still appears underrecognized, and its clinical and pathological characteristics remain to be defined. This study analyzed the clinical and pathological findings of three such cases that the authors encountered recently and 43 previously reported cases identified in the literature. Assessment of all 46 cases showed a slight female predominance (1.2:1) with a median age of 41.5 years. One-third of the cases were misdiagnosed at initial evaluation mainly because the possibility of FDC sarcoma was not considered. When considered, this disease had distinct pathological characteristics that allowed an accurate diagnosis. Staining for FDC markers, CD21, CD35, and clusterin was particularly helpful. The pathogenesis of the disease appeared heterogeneous, and associated factors included Epstein-Barr virus infection (in hepatic cases) and inflammatory pseudotumor-like conditions. Treatment modality varied widely although surgical resection was often included. With a median follow-up of 18 months, 43% of the cases recurred and 7% died of disease. The 5-year recurrence-free survival was 27.4%. From data available at the current time, we were not able to identify prognostically significant pathologic factors.
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Affiliation(s)
- Jinru Shia
- Department of Pathology, Memorial Sloan-Kettering Cancer Center, 1275 York Avenue, New York, NY 10021, USA
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29
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Agaimy A, Wünsch PH. Follicular dendritic cell tumor of the gastrointestinal tract: Report of a rare neoplasm and literature review. Pathol Res Pract 2006; 202:541-8. [PMID: 16564140 DOI: 10.1016/j.prp.2006.01.013] [Citation(s) in RCA: 20] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/15/2005] [Accepted: 01/30/2006] [Indexed: 01/09/2023]
Abstract
Follicular dendritic cell (FDC) tumor is a rare neoplasm of the accessory immune system showing FDC differentiation. Histologically, a conventional type and an inflammatory pseudotumor (IPT-like) type are recognized. The etiology of FDC tumor is unknown. While rare FDC tumors were associated with hyaline-vascular Castleman's disease (HVCD), hepatosplenic IPT-like FDC tumors consistently harbor EBV infection. FDC tumors of the gastrointestinal (GI) tract and mesentery/omentum are exceedingly rare, with only 17 cases reported so far. We report an additional case of an IPT-like FDC tumor of the ileum and mesentery in a 52-year-old schizophrenic man. The tumor consisted of highly atypical multinucleated giant cells in a background of intense lymphoid infiltrate with prominent eosinophilia reminiscent of Hodgkin's lymphoma. Tumor cells were immunoreactive for vimentin, CD21, CD35, fascin, smooth muscle actin and CD68, but were negative for all lineage-specific lymphoreticular, myeloid, mesenchymal and epithelial markers. Immunostaining for HHV-8 and in situ hybridization for EBV-encoded RNA (EBER) were negative. Some mesenteric lymph nodes showed HVCD-like changes. The differential diagnostic considerations of this unusual and rare neoplasm, mainly lymphocyte-rich GI stromal tumor (GIST), malignant lymphoma and inflammatory neoplasia of diverse histogenetic types, will be discussed together with a literature review on gastrointestinal FDC tumors.
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Affiliation(s)
- Abbas Agaimy
- Institut für Pathologie, Klinikum Nürnberg, Prof.-Ernst-Nathan-Strasse 1, 90419 Nürnberg, Germany.
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30
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Diacovo TG, Blasius AL, Mak TW, Cella M, Colonna M. Adhesive mechanisms governing interferon-producing cell recruitment into lymph nodes. ACTA ACUST UNITED AC 2006; 202:687-96. [PMID: 16147979 PMCID: PMC2212867 DOI: 10.1084/jem.20051035] [Citation(s) in RCA: 92] [Impact Index Per Article: 4.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/17/2022]
Abstract
Natural interferon-producing cells (IPCs) are found in peripheral lymph nodes (PLNs), where they support NK cell, T cell, and B cell responses to pathogens. However, their route of entry and the adhesive mechanisms used to gain access to PLNs remain poorly defined. We report that IPCs can enter PLNs via a hematogenous route, which involves a multistep adhesive process, and that transmigration is enhanced by inflammation. Results indicate that L-selectin on IPCs is required for efficient attachment and rolling on high endothelial venules in vivo in both nonstimulated and inflamed PLNs. IPCs, however, also possess functional ligands for E-selectin that contribute to this process only in the latter case. In conjunction with selectin-mediated adhesion, both β1- and β2-integrins participate in IPC attachment to the inflamed vessel wall, whereas chemotaxis relies in part on the chemokine receptor CCR5. Identification of the adhesive machinery required for IPC trafficking into PLNs may provide opportunities to regulate immune responses reliant on the activity of these cells.
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Affiliation(s)
- Thomas G Diacovo
- Department of Pathology and Immunology, Washington University School of Medicine, St. Louis, MO 63110, USA.
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31
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Kasajima-Akatsuka N, Maeda K. Development, maturation and subsequent activation of follicular dendritic cells (FDC): immunohistochemical observation of human fetal and adult lymph nodes. Histochem Cell Biol 2006; 126:261-73. [PMID: 16470387 DOI: 10.1007/s00418-006-0157-6] [Citation(s) in RCA: 20] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 01/17/2006] [Indexed: 10/25/2022]
Abstract
To elucidate the processes involved in development and activation of human follicular dendritic cells (FDC), immunohistochemistry was performed on paraffin sections of fetal lymph nodes (FLN) obtained from archived autopsy material, and of adult reactive lymph nodes (ARLNs) excised for diagnostic purpose, using a panel of antibodies. Our study showed that tiny clusters of CNA.42(+ )KiM4p(+) cells, surrounded by some B-lymphocytes, initially arose in the cortical area of underdeveloped FLN around the 20th gestational week. No co-expression of CD21 and CD35 was found. In the relatively developed FLN of the same gestational age, small eddies of immature FDC, which expressed CD21, CD35, and nerve growth factor receptor (NGFR), as well as CNA.42 and KiM4p, were observed within ill-defined aggregations of B-lymphocytes. As gestation progressed, more B-lymphocytes assembled in a compact manner and formed primary lymphoid follicles containing an extending web of mature FDC, which expressed CNA.42, KiM4p, CD21, CD35, NGFR, and sometimes CD23 and X-11. In well-developed secondary follicles of ARLNs, activated FDC expressed additional molecules such as CD55, CD106, and S100alpha. Our observations identified the processes of phenotypic alteration of human FDC and established practical indicators determining their developmental stage and functional phase.
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Affiliation(s)
- Naoko Kasajima-Akatsuka
- Department of Pathology, Yamagata University School of Medicine, 2-2-2 Iida-Nishi, Yamagata 990-9585, Japan
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32
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Torres U, Hawkins WG, Antonescu CR, DeMatteo RP. Hepatic follicular dendritic cell sarcoma without Epstein-Barr virus expression. Arch Pathol Lab Med 2006; 129:1480-3. [PMID: 16253031 DOI: 10.5858/2005-129-1480-hfdcsw] [Citation(s) in RCA: 22] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/06/2022]
Abstract
Follicular dendritic cell sarcoma of the liver is an uncommon pathologic entity, and only 5 cases have been reported previously. Herein, we report the first case, to our knowledge, of hepatic follicular dendritic cell sarcoma without evidence of Epstein-Barr virus infection. The patient is an elderly man who was found to have an incidental liver mass and then developed weight loss and fever. The diagnosis was based on the typical morphologic appearance of spindle cell proliferation associated with a brisk lymphoplasmacytic infiltrate and strong immunoreactivity to CD21 and CD35. Based on our experience and a review of the published reports, we summarize the clinical and pathologic features of hepatic follicular dendritic cell sarcoma and its surgical management.
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Affiliation(s)
- Ulises Torres
- Department of Surgery, Memorial Sloan-Kettering Cancer Center, New York, NY 10021, USA
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33
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Nonaka D, Rodriguez J, Rollo JL, Rosai J. Undifferentiated Large Cell Carcinoma of the Thymus Associated With Castleman Disease-Like Reaction. Am J Surg Pathol 2005; 29:490-5. [PMID: 15767803 DOI: 10.1097/01.pas.0000155148.45423.b5] [Citation(s) in RCA: 18] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/04/2023]
Abstract
Five cases of a distinctive type of undifferentiated large cell thymic carcinoma accompanied by an inflammatory reaction having morphologic features closely resembling those of Castleman disease (CD) of the hyaline vascular type (HVCD) are reported. The tumors occurred in 3 men and 2 women with a median age of 53 years. Three patients were asymptomatic and the tumors were found incidentally; 1 patient presented with fatigue, weight loss, dyspnea, and chest pain, and another with acanthosis nigricans. The tumors were characterized by the admixture of two components: a neoplasm of cytokeratin (+)/CD5(-) undifferentiated large tumor cells and an inflammatory reaction resembling the late stage of HVCD. Some of the cases were associated with a remarkably indolent clinical course, especially when considering their high-grade morphology. Three patients were alive without disease at 1, 10, and 22 years. One patient was alive with persistent disease at 1 year. One patient's clinical course was unique in that the primary thymic tumor was found 16 years after a metastasis had been detected in two distant lymph nodes. The two possible explanations for the coexistence here described are as follows: 1) a CD-like reaction to the tumor as the morphologic manifestation of a host immune response; and 2) a malignancy engrafted upon preexisting HVCD of the thymus, in a manner analogous to that operating in the reported cases of tumors of dendritic/reticulum cells complicating HVCD at other sites. The first hypothesis is favored, with the added suggestion that the CD-like reaction may bear a relationship to the peculiarly indolent behavior that these tumors exhibit.
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Affiliation(s)
- Daisuke Nonaka
- Department of Pathology, National Cancer Institute, Milan, Italy.
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34
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Grogg KL, Lae ME, Kurtin PJ, Macon WR. Clusterin expression distinguishes follicular dendritic cell tumors from other dendritic cell neoplasms: report of a novel follicular dendritic cell marker and clinicopathologic data on 12 additional follicular dendritic cell tumors and 6 additional interdigitating dendritic cell tumors. Am J Surg Pathol 2004; 28:988-98. [PMID: 15252304 DOI: 10.1097/01.pas.0000112536.76973.7f] [Citation(s) in RCA: 87] [Impact Index Per Article: 4.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/06/2023]
Abstract
While tumors of dendritic cell lineage may have overlapping histomorphologic features, most but not all cases can be classified using an immunohistochemical panel, including CD21, CD23, CD35, CD1a, and S-100. Based on observations that clusterin is expressed in benign follicular dendritic cells, clusterin expression in 32 dendritic cell tumors was evaluated. Diffuse strong staining for clusterin was seen in 12 of 12 follicular dendritic cell tumors. Two of these cases were negative for traditional markers (CD21, CD23, CD35); they were classified based on characteristic ultrastructural features. Three of 6 interdigitating dendritic cell tumors were negative for clusterin and 3 showed focal weak positivity. Clusterin staining in Langerhans cell histiocytosis ranged from negative (6 of 14) to weak/moderate (8 of 14). Follicular dendritic cell tumors behaved as benign tumors or low-grade sarcomas. Interdigitating dendritic cell tumors demonstrated a widely variable behavior, ranging from benign to rapidly fatal disease. Based on this initial study, strong clusterin staining supports a diagnosis of follicular dendritic cell tumor. Thus, staining for clusterin is useful in classification of dendritic cell tumors, particularly when the more common markers of follicular dendritic cells are not expressed.
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Affiliation(s)
- Karen L Grogg
- Division of Anatomic Pathology, Department of Laboratory Medicine and Pathology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA
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35
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Pileri SA, Grogan TM, Harris NL, Banks P, Campo E, Chan JKC, Favera RD, Delsol G, De Wolf-Peeters C, Falini B, Gascoyne RD, Gaulard P, Gatter KC, Isaacson PG, Jaffe ES, Kluin P, Knowles DM, Mason DY, Mori S, Müller-Hermelink HK, Piris MA, Ralfkiaer E, Stein H, Su IJ, Warnke RA, Weiss LM. Tumours of histiocytes and accessory dendritic cells: an immunohistochemical approach to classification from the International Lymphoma Study Group based on 61 cases. Histopathology 2002; 41:1-29. [PMID: 12121233 DOI: 10.1046/j.1365-2559.2002.01418.x] [Citation(s) in RCA: 445] [Impact Index Per Article: 19.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/06/2023]
Abstract
Neoplasms of histiocytes and dendritic cells are rare, and their phenotypic and biological definition is incomplete. Seeking to identify antigens detectable in paraffin-embedded sections that might allow a more complete, rational immunophenotypic classification of histiocytic/dendritic cell neoplasms, the International Lymphoma Study Group (ILSG) stained 61 tumours of suspected histiocytic/dendritic cell type with a panel of 15 antibodies including those reactive with histiocytes (CD68, lysozyme (LYS)), Langerhans cells (CD1a), follicular dendritic cells (FDC: CD21, CD35) and S100 protein. This analysis revealed that 57 cases (93%) fit into four major immunophenotypic groups (one histiocytic and three dendritic cell types) utilizing six markers: CD68, LYS, CD1a, S100, CD21, and CD35. The four (7%) unclassified cases were further classifiable into the above four groups using additional morphological and ultrastructural features. The four groups then included: (i) histiocytic sarcoma (n=18) with the following phenotype: CD68 (100%), LYS (94%), CD1a (0%), S100 (33%), CD21/35 (0%). The median age was 46 years. Presentation was predominantly extranodal (72%) with high mortality (58% dead of disease (DOD)). Three had systemic involvement consistent with 'malignant histiocytosis'; (ii) Langerhans cell tumour (LCT) (n=26) which expressed: CD68 (96%), LYS (42%), CD1a (100%), S100 (100%), CD21/35 (0%). There were two morphological variants: cytologically typical (n=17) designated LCT; and cytologically malignant (n=9) designated Langerhans cell sarcoma (LCS). The LCS were often not easily recognized morphologically as LC-derived, but were diagnosed based on CD1a staining. LCT and LCS differed in median age (33 versus 41 years), male:female ratio (3.7:1 versus 1:2), and death rate (31% versus 50% DOD). Four LCT patients had systemic involvement typical of Letterer-Siwe disease; (iii) follicular dendritic cell tumour/sarcoma (FDCT) (n=13) which expressed: CD68 (54%), LYS (8%), CD1a (0%), S100 (16%), FDC markers CD21/35 (100%), EMA (40%). These patients were adults (median age 65 years) with predominantly localized nodal disease (75%) and low mortality (9% DOD); (iv) interdigitating dendritic cell tumour/sarcoma (IDCT) (n=4) which expressed: CD68 (50%), LYS (25%), CD1a (0%), S100 (100%), CD21/35 (0%). The patients were adults (median 71 years) with localized nodal disease (75%) without mortality (0% DOD). In conclusion, definitive immunophenotypic classification of histiocytic and accessory cell neoplasms into four categories was possible in 93% of the cases using six antigens detected in paraffin-embedded sections. Exceptional cases (7%) were resolvable when added morphological and ultrastructural features were considered. We propose a classification combining immunophenotype and morphology with five categories, including Langerhans cell sarcoma. This simplified scheme is practical for everyday diagnostic use and should provide a framework for additional investigation of these unusual neoplasms.
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Affiliation(s)
- S A Pileri
- Service of Pathologic Anatomy and Hematopathology, Institute of Haematology and Clinical Oncology L.e A. Seràgnoli, Bologna University, Italy.
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36
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Cokelaere K, Debiec-Rychter M, De Wolf-Peeters C, Hagemeijer A, Sciot R. Hyaline vascular Castleman's disease with HMGIC rearrangement in follicular dendritic cells: molecular evidence of mesenchymal tumorigenesis. Am J Surg Pathol 2002; 26:662-9. [PMID: 11979097 DOI: 10.1097/00000478-200205000-00013] [Citation(s) in RCA: 69] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022]
Abstract
Chromosomal aberrations involving chromosome segment 12q13-15 are a common finding in a variety of benign mesenchymal tumors. The target gene encodes for HMGIC, a member of the high mobility group protein family. These proteins act as architectural transcription factors. HMGIC plays a role as a common genetic denominator in benign mesenchymal tumorigenesis. We report a case of hyaline vascular Castleman's disease with intragenic HMGIC rearrangement, due to a clonal cytogenetic aberration involving the long arm of chromosome 12 [46,XX, add(1)(q21),der(6)t(6;12) (q23;q15),add(7)(p22), -9,inv(9)(p11q13),del(12)(q15),+mar] obtained after short-term primary cultures. A combined immunocytologic-cytogenetic approach enabled us to demonstrate the exclusive presence of HMGIC rearrangement in anti-CD21 reactive follicular dendric cells. This finding confirms that a clonal proliferation of follicular dendritic cells occurs in the hyaline vascular variant of Castleman's disease. It also provides a possible molecular pathway explaining stromal overgrowths and stromal neoplasms developing from this disorder.
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Affiliation(s)
- K Cokelaere
- Department of Pathology, University Hospital St Raphael, Katholieke Universiteit Leuven, Leuven, Belgium
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37
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Shah RN, Ozden O, Yeldandi A, Peterson L, Rao S, Laskin WB. Follicular dendritic cell tumor presenting in the lung: a case report. Hum Pathol 2001; 32:745-9. [PMID: 11486174 DOI: 10.1053/hupa.2001.25595] [Citation(s) in RCA: 28] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/11/2022]
Abstract
An example of extranodal follicular dendritic cell sarcoma (FDCS) presenting in the lung, a heretofore unreported site, is described. Macroscopically, a 9.5-cm, tan-white, dominant mass and multiple smaller parenchymal and pleural nodules were identified. Microscopically, the tumor was composed of spindled cells with uniform cytologic features arranged in short, intersecting fascicles and intermixed small lymphocytes and plasma cells. One of 4 peribronchial and hilar lymph nodes evaluated microscopically was focally involved by the process. Immunohistochemically, the neoplastic spindled cells expressed complement receptors CD21 and CD35 and low-affinity nerve growth factor receptor but did not express keratin (AE1/AE3 and CAM5.2), CD45 (leukocyte common antigen), CD20 (L26), S-100 protein, muscle-specific actin, or gp100 protein (HMB45). Ultrastructurally, the tumor cells have complex interdigitating cell surface processes and desmosomes. Epstein-Barr virus (EBV) was not detected in the tumor cells by in situ hybridization for EBV-encoded RNA or by polymerase chain reaction for viral DNA. FDCS should be considered in the differential diagnosis of any spindled-cell tumor with interspersed chronic inflammatory cells occurring in the lung. An immunohistochemical panel, including anti-CD21 and -CD35, can assist in its diagnosis, especially with small bronchial biopsy specimens. 2001 by W.B. Saunders Company.
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Affiliation(s)
- R N Shah
- Department of Pathology, Northwestern University Medical School, Chicago, IL, USA
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38
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Chan AC, Chan KW, Chan JK, Au WY, Ho WK, Ng WM. Development of follicular dendritic cell sarcoma in hyaline-vascular Castleman's disease of the nasopharynx: tracing its evolution by sequential biopsies. Histopathology 2001; 38:510-8. [PMID: 11422494 DOI: 10.1046/j.1365-2559.2001.01134.x] [Citation(s) in RCA: 124] [Impact Index Per Article: 5.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/20/2022]
Abstract
AIMS Hyaline-vascular Castleman's disease (HVCD) and follicular dendritic cell (FDC) sarcoma occurring in the nasopharynx are both extremely rare. We report the first case of transformation of the former into the latter as documented by sequential biopsies. The steps involved in the transformation were described in detail and the possible role of p53 studied. METHODS AND RESULTS The patient presented at the age of 23 years with nasopharyngeal HVCD. Hyaline- vascular Castleman's disease with FDC overgrowth was diagnosed in a recurrence 8 years later, and a frank FDC sarcoma developed at the same site 11 years after initial presentation. The patient remained disease-free 3 years after excision and adjuvant chemotherapy. The FDC sarcoma comprised swirling fascicles of spindly cells with indistinct cell borders. The tumour cells expressed the FDC markers CD21, CD35 and CNA.42 and in-situ hybridization for Epstein-Barr virus-encoded RNAs was negative. Over-expression of p53 protein was observed in the FDC sarcoma and an increased number of weakly p53-positive spindly cells could also be demonstrated in the HVCD specimen. This finding suggested a possible role of p53 in the evolution from HVCD to FDC sarcoma. Critical analysis of the literature shows that, among the 13 reported cases of FDC sarcoma associated with Castleman's disease, possible progression from the latter to the former is documented in only two cases. CONCLUSIONS The sequential changes observed in the current case provide further evidence to strengthen the role of HVCD as a possible precursor of FDC sarcoma. There is a possible role of p53 in the transformation process but confirmation by future studies is needed.
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Affiliation(s)
- A C Chan
- Department of Pathology, Queen Elizabeth Hospital, Kowloon, Hong Kong.
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39
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Chang KC, Jin YT, Chen FF, Su IJ. Follicular dendritic cell sarcoma of the colon mimicking stromal tumour. Histopathology 2001; 38:25-9. [PMID: 11135043 DOI: 10.1046/j.1365-2559.2001.01035.x] [Citation(s) in RCA: 31] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/20/2022]
Abstract
AIMS Follicular dendritic cell tumours are very rare neoplasms that often occur in lymph nodes. We report here a case in the colon, a hitherto unreported site, in a 37-year-old female. The differentiation from gastrointestinal stromal tumour is emphasized. METHODS AND RESULTS The tumour was tan, elastic and solid with surface ulceration. Microscopically, it was composed of oval to spindle tumour cells with syncytial cytoplasm arranged in fascicular and whorled patterns. There were many infiltrating lymphocytes. The histological appearance resembled gastrointestinal stromal tumour, thymoma or meningioma. Distinct from the stromal tumour, the lymph node was also involved by the tumour. Immunohistochemically, the tumour cells were positive for CD21, CD35 and CD68, but negative for cytokeratin, CD34, smooth muscle actin, desmin, S100 protein, epithelial membrane antigen, leukocyte common antigen, HMB-45 and c-kit. In-situ hybridization study was negative for Epstein-Barr virus RNA sequences. Ultrastructurally, the tumour cells possessed cytoplasmic processes joined by desmosomes. CONCLUSIONS This entity should be considered in the list of differential diagnoses for gastrointestinal stromal tumour. The lymph node metastasis and immunohistochemical features are of value for identification of this rare neoplasm.
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Affiliation(s)
- K C Chang
- Department of Pathology, National Cheng Kung University Medical Center, Tainan, Taiwan
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40
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Pauwels P, Dal Cin P, Vlasveld LT, Aleva RM, van Erp WF, Jones D. A chromosomal abnormality in hyaline vascular Castleman's disease: evidence for clonal proliferation of dysplastic stromal cells. Am J Surg Pathol 2000; 24:882-8. [PMID: 10843293 DOI: 10.1097/00000478-200006000-00016] [Citation(s) in RCA: 66] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022]
Abstract
The pathogenesis of the hyaline vascular variant of Castleman's disease is currently unknown; however, vascular and dendritic cell proliferations are common in this disorder. We report a clonal karyotypic abnormality (46,XX,t(1;16) (p11;p11), del(7)(q21q22),del(8)(q12q22)) in 15 of 20 cells obtained after short-term stromal cultures of a typical case of hyaline vascular Castleman's disease (HVCD). There was no histologic, immunohistochemical, or genotypic evidence of a clonal lymphoid or plasma cell proliferation supporting origin of this aberration from the stromal component, possibly dendritic cells. We re-examined 15 previous cases of HVCD and identified a spectrum of dysplastic changes in the follicular dendritic cells (FDC) of atrophic lymphoid follicles, with some cases showing expansions of FDC networks by CD21 immunostaining. We propose that localized clonal proliferations of stromal elements, particularly follicular dendritic cells, occur in typical HVCD and likely explain the increased incidence of FDC sarcomas in these patients.
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Affiliation(s)
- P Pauwels
- Stichting PAMM, Catharina Ziekenhuis, Eindhoven, The Netherlands
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41
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Taylor GB, Smeeton IW. Cytologic demonstration of "dysplastic" follicular dendritic cells in a case of hyaline-vascular Castleman's disease. Diagn Cytopathol 2000; 22:230-4. [PMID: 10787143 DOI: 10.1002/(sici)1097-0339(200004)22:4<230::aid-dc6>3.0.co;2-5] [Citation(s) in RCA: 23] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/06/2022]
Abstract
Large atypical follicular dendritic cells, considered dysplastic by some authors, were first described in association with Castleman's disease in 1991, but until now there has been no cytologic account regarding these cells. We report the cytologic and histologic findings of a case of hyaline-vascular Castleman's disease associated with "dysplastic "follicular dendritic cells which presented as a mediastinal mass in a young man. The presence of giant cells within the preoperative fine-needle aspirate specimen caused initial diagnostic uncertainty and their true nature was only confirmed retrospectively following application of an immunostain for CD21 to direct smears. Awareness of "dysplastic" follicular dendritic cells within aspirates of Castleman's disease will result in less cytological confusion in the future and may help to avoid the possibility of misdiagnosing Hodgkin's lymphoma which has certain cytologic similarities.
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Affiliation(s)
- G B Taylor
- Department of Cytopathology, Greenlane Hospital, Auckland, New Zealand.
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42
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Nakamura S, Nagahama M, Kagami Y, Yatabe Y, Takeuchi T, Kojima M, Motoori T, Suzuki R, Taji H, Ogura M, Mizoguchi Y, Okamoto M, Suzuki H, Oyama A, Seto M, Morishima Y, Koshikawa T, Takahashi T, Kurita S, Suchi T. Hodgkin's disease expressing follicular dendritic cell marker CD21 without any other B-cell marker: a clinicopathologic study of nine cases. Am J Surg Pathol 1999; 23:363-76. [PMID: 10199466 DOI: 10.1097/00000478-199904000-00001] [Citation(s) in RCA: 32] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022]
Abstract
Reed-Sternberg (RS) and Hodgkin's (H) cells are considered to be the neoplastic cells in Hodgkin's disease (HD). Although most data suggest their lymphoid origin, the nature of these cells still remains a subject of controversy. Recently, a number of RS cells have been found to express an antigen that is also present on follicular dendritic cells (FDCs), asserting FDCs as the possible progenitor cells of H-RS cells. This prompted us to investigate whether these CD21-positive cases had distinct clinicopathologic characteristics. In a series of 94 examined cases of HD, we identified 9 CD21-positive ones (4 of 37 cases of nodular sclerosis, 1 of 41 mixed cellularity, and 4 of 12 lymphocyte depletion HD) without any other B-cell marker on paraffin sections. The patients varied in age from 16 to 82 years (median, 50 years) and included six men and three women. They had superficial or mesenteric lymphadenopathy without hepatosplenomegaly. Peripheral blood leukocytosis was seen in three patients. The clinical course was indolent, and all patients but one achieved an initial complete response with HD-based treatment regimens, although three of them relapsed. Morphologically, two subgroups could be delineated. Six of the cases were characterized, besides by the classic RS cells, by a varying number of the cells with the distinctive walnutlike or cerebrumlike nuclei and cytologically with cytoplasmic processes. Their fine structural examination also revealed villous processes, but no desmosomes. The other three cases had multinucleated RS cells often with triangular nuclei, but not cytoplasmic processes. The percentage of CD21-positive tumor cells ranged from less than 10% to 60% among the H-RS cells. These RS cells were positive for CD30 (9 of 9), CD15 (7 of 9), CD68 (1 of 8), fascin (8 of 8), S-100 protein (1 of 7), and epithelial membrane antigen (2 of 8) on paraffin sections. Notably, of eight cases examined on frozen sections, two showed immunostaining for DRC1, CD35, R4/23, and Ki-M4p. Only CD35 was also detected in the other two cases. Genotypic investigation showed germline configuration of the T-cell receptor beta and gamma chain genes and the immunoglobulin heavy chain gene in all eight cases examined. In situ hybridization showed Epstein-Barr virus sequences in four cases, three of which were examined by the terminal region analysis and showed the Epstein-Barr virus to be monoclonal. We concluded that in a small proportion (9.6%) of HD, H-RS cells might be derived from FDCs and that they appear to represent a distinct pathologic variant based on morphologic and phenotypic traits within the framework of HD.
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Affiliation(s)
- S Nakamura
- Department of Pathology and Clinical Laboratories, Aichi Cancer Center Hospital, Japan
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43
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Andriko JW, Kaldjian EP, Tsokos M, Abbondanzo SL, Jaffe ES. Reticulum cell neoplasms of lymph nodes: a clinicopathologic study of 11 cases with recognition of a new subtype derived from fibroblastic reticular cells. Am J Surg Pathol 1998; 22:1048-58. [PMID: 9737236 DOI: 10.1097/00000478-199809000-00002] [Citation(s) in RCA: 88] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
Abstract
Lymph nodes contain nonlymphoid accessory cells including follicular dendritic cells (FDCs), interdigitating dendritic cells (IDCs) and fibroblastic reticular cells (FBRCs). Neoplasms derived from FDCs are uncommon, and those of IDC origin are even more rare. We report the clinicopathologic features of 11 reticulum cell neoplasms, including 2 of FBRC origin. There were seven male patients and four female patients ranging in age from 13 to 73 years. All cases involved lymph nodes (cervical or supraclavicular-6 cases), (abdominal--2 cases), epitrochlear (1 case); two had more than one site of involvement (cervical lymph node and mediastinum--1 case, cervical and abdominal lymph nodes--1 case). One case of FDC tumor had concomitant Castleman's disease, plasma cell variant. Each neoplasm showed similar histology with oval-to-spindle-shaped cells in a storiform or fascicular pattern. Based on immunophenotypic findings, the neoplasms were classified as FDC (five cases), IDC (two cases), FBRC (three cases), and reticulum cell neoplasm, not otherwise specified (one case). The FDC tumors showed immunoreactivity for CD21 or CD35, vimentin, and CD68. The IDC tumors showed strong positivity for S-100 protein and variable positivity for CD68 and CD1a. The cases derived from FBRCs were positive for vimentin, desmin, and smooth-muscle actin. The neoplasm classified as reticulum cell neoplasm, not otherwise specified had similar morphologic features but showed only equivocal positivity for CD68 and vimentin. Follow-up was available for 9 of 11 (82%) cases with a mean of 3.5 years. Four of five patients with FDC tumors were alive with disease when last seen; the fifth is alive and well with no evidence of disease at 4-year follow-up. One patient with IDC tumor had a recurrence in a different nodal site. Two patients with FBRC tumor were disease free at follow-up of 2 years and 8 years, respectively. The patient with reticulum cell neoplasm, not otherwise specified, was alive and disease free 8 years after diagnosis.
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Affiliation(s)
- J W Andriko
- Department of Hematatologic and Lymphatic Pathology, Armed Forces Institute of Pathology, Washington, DC 20306-6000, USA
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44
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Smith RM, Dubinett SM, Grudko K, Rosenow EC, Budman DR, Schnader J. Clinical conference on management dilemmas: a young woman with a 10-cm chest mass. Chest 1998; 114:295-306. [PMID: 9674482 DOI: 10.1378/chest.114.1.295] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/01/2022] Open
Affiliation(s)
- R M Smith
- Department of Medicine, University of California, San Diego, VA Healthcare System, USA
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45
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Lin O, Frizzera G. Angiomyoid and follicular dendritic cell proliferative lesions in Castleman's disease of hyaline-vascular type: a study of 10 cases. Am J Surg Pathol 1997; 21:1295-306. [PMID: 9351567 DOI: 10.1097/00000478-199711000-00004] [Citation(s) in RCA: 76] [Impact Index Per Article: 2.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/05/2023]
Abstract
Castleman's disease of hyaline-vascular type (HV CD) may rarely be associated with a confusing variety of stromal cell overgrowths and neoplasms. We report here on the pathologic and clinical findings of 10 such cases. In addition to the usual complex histoimmunophenotype of the stroma of HV CD and some unusual features that mimicked neoplasms, we observed focal proliferations of angiomyoid (five cases) and follicular dendritic cell type (five cases). The former were nonneoplastic growths featuring compact tangles of spindle cells, exhibiting immunoreactivity for smooth muscle actin and interpreted as vessel-related pericytes and myoid cells. The latter were neoplastic growths of oval to spindle cells intermixed with lymphocytes; the tumor cells grew in long, intersecting bundles, featured various degree of atypia, and expressed the markers of follicular dendritic cells (CD21, CD35, KiM4p). The two types were clinically distinct. Four of five patients with angiomyoid proliferations were young women, who presented with an abdominal mass and were cured by surgery; that is, they had a clinical profile similar to that of patients with the stroma-rich variant of HV CD. The follicular dendritic cell proliferations were in older patients of either gender presenting with masses at various sites, recapitulating the profile of follicular dendritic cell tumors arising independently from HV CD; in three patients with long-term follow-up, recurrences or metastases developed at various intervals from the initial diagnosis (1 1/6, 3 1/2, and 11 years), and one patient died as a result. This study confirms the potential for, and the variety of, stromal cell proliferations in HV CD. Because their biologic behavior differs, correct identification of these various proliferative lesions is clinically important.
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Affiliation(s)
- O Lin
- Department of Pathology, New York University Medical Center, New York 10016, USA
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46
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47
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Perez-Ordonez B, Erlandson RA, Rosai J. Follicular dendritic cell tumor: report of 13 additional cases of a distinctive entity. Am J Surg Pathol 1996; 20:944-55. [PMID: 8712294 DOI: 10.1097/00000478-199608000-00003] [Citation(s) in RCA: 203] [Impact Index Per Article: 7.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/01/2023]
Abstract
Follicular dendritic cell (FDC) tumor is an extremely rare malignant neoplasm with approximately 17 well-documented cases in the literature. We report 13 additional cases of this distinctive neoplasm. There were seven men and six women, with a mean age of 46.5 years (range, 27-62 years). There was involvement of cervical lymph nodes (six cases), mediastinum (three cases), axilla, tonsil, spleen, and peripancreatic soft tissues (one case each). The neoplasms were grey to tan, ranging in size from 1 to 13 cm. They were formed by oval to spindle cells with eosinophilic cytoplasm growing in sheets and fascicles, with a focal storiform pattern and whorls reminiscent of those seen in meningioma. The nuclei were oval or elongated with thin nuclear membranes, inconspicuous or small eosinophilic nucleoli, and clear or dispersed chromatin. Typically, the tumor cells were intimately admixed with small lymphocytes, with a prominent perivascular cuffing. Multinucleated tumor cells were present in seven cases. Necrosis, marked cellular atypia, high mitotic rate, and/or abnormal mitoses were present in seven cases. The tumor cells were positive for CD21 (10 of 11), CD35 (10 of 11), Ki-M4p (seven of eight) Ki-FDRC1p (six of seven), vimentin (five of nine), and S100 protein (five of nine). One case stained with actin. In situ hybridization, done in six cases, did not show Epstein-Barr virus RNA sequences. Ultrastructural examination of eight cases showed long, complex, occasionally interdigitating cytoplasmic processes joined by desmosomes. The behavior of these tumors is more akin to that of a low-grade soft tissue sarcoma than a malignant lymphoma and is characterized by local recurrences and occasional metastases. Two patients died of tumor, two were alive with recurrent or metastatic disease, eight were alive with no disease, and one was lost to follow-up.
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Affiliation(s)
- B Perez-Ordonez
- Department of Pathology, Memorial Sloan-Kettering Cancer Center, New York 10021, USA
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48
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Shek TW, Ho FC, Ng IO, Chan AC, Ma L, Srivastava G. Follicular dendritic cell tumor of the liver. Evidence for an Epstein-Barr virus-related clonal proliferation of follicular dendritic cells. Am J Surg Pathol 1996; 20:313-24. [PMID: 8772785 DOI: 10.1097/00000478-199603000-00008] [Citation(s) in RCA: 144] [Impact Index Per Article: 5.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/02/2023]
Abstract
Tumors of the follicular dendritic cell are uncommon, and most occur as primary lymph node tumors. We report a case of primary follicular dendritic cell tumor of the liver that was initially reported as an inflammatory pseudotumor. The neoplasm recurred as two separate tumor masses 30 months after complete resection of the "hepatic inflammatory pseudotumor." It showed a wide spectrum of morphologic features ranging from areas with fascicles of very bland spindle cells amidst a background population of lymphocytes, reminiscent of inflammatory pseudotumor, to areas of dispersed sheets of highly pleomorphic tumor cells with a relative paucity of reactive inflammatory cells. The diagnosis was confirmed by positive immunohistochemical staining with CD21, CD35, R4/23, and Ki-M4 and by ultrastructural demonstration of convoluted interdigitating cell processes joined by desmosomes. The background lymphocytes were oligoclonal, CD8-positive T cells. In situ hybridization for Epstein-Barr virus (EBV)-encoded RNA was positive in the tumor cells in the original and recurrent tumors. More importantly, the cells showed identical episomal clonal EBV on Southern blot analysis, implying that the initial and recurrent tumors are due to clonal proliferation of EBV-positive neoplastic follicular dendritic cells. The tumor cells expressed latent membrane protein but not EBV-encoded nuclear antigen 2 (EBNA2) or ZEBRA. Such gene expression is very similar to that of Hodgkin's disease and nasopharyngeal carcinoma. The strong expression of latent membrane protein restricted to the tumor cells and the clonality of the EBV suggest that the virus may be involved in the pathogenesis of this tumor and not present merely as a "bystander."
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Affiliation(s)
- T W Shek
- Department of Pathology, University of Hong Kong, Queen Mary Hospital, Hong Kong
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49
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Winter SS, Howard TA, Ritchey AK, Keller FG, Ware RE. Elevated levels of tumor necrosis factor-beta, gamma-interferon, and IL-6 mRNA in Castleman's disease. MEDICAL AND PEDIATRIC ONCOLOGY 1996; 26:48-53. [PMID: 7494511 DOI: 10.1002/(sici)1096-911x(199601)26:1<48::aid-mpo6>3.0.co;2-v] [Citation(s) in RCA: 19] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/25/2023]
Abstract
Castleman's disease (CD) is a lymphoproliferative disorder characterized by enlarged hyperplastic lymph nodes. CD may be localized or multifocal, and is often associated with signs and symptoms of generalized inflammation. The systemic manifestations of CD have been previously attributed to an overproduction of interleukin-6 (IL-6) by the tumor, although there is evidence that IL-6 is not responsible for all of the symptoms. We describe a 9-year-old boy who developed Castleman's disease with systemic findings of hypochromic microcytic anemia, growth arrest, inflammation, and hyperimmunoglobulinemia. Following surgical resection, all of the symptoms and laboratory abnormalities resolved. Using reverse transcriptase polymerase chain reaction (RT-PCR) analysis of the tumor, we found elevated levels of IL-6 mRNA as expected, but also elevated levels of tumor necrosis factor beta (TNF-beta) and gamma interferon (gamma-IFN) mRNA. Because these cytokines are mediators of immune regulation and inflammation, we propose that TNF-beta and gamma-IFN also play an important role in the pathophysiology of Castleman's disease.
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Affiliation(s)
- S S Winter
- Department of Pediatrics, Duke University Medical Center, Durham, North Carolina 27710, USA
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50
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Nakao A, Hasegawa Y, Tsuchiya Y, Shimokata K. Expression of cell adhesion molecules in the lungs of patients with idiopathic pulmonary fibrosis. Chest 1995; 108:233-9. [PMID: 7541743 DOI: 10.1378/chest.108.1.233] [Citation(s) in RCA: 34] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/25/2023] Open
Abstract
Idiopathic pulmonary fibrosis (IPF) is a chronic inflammatory disorder restricted to the lungs. Leukocyte entry into the area of inflammation is regulated, at least partly, by endothelial expression of leukocyte-selective cell adhesion molecules (CAMs). To investigate the relevance of these CAMs to the accumulation of leukocytes in IPF, we examined the expression of E-selectin (endothelial leukocyte adhesion molecule-1; ELAM-1), intercellular adhesion molecule-1 (ICAM-1), and vascular cell adhesion molecule-1 (VCAM-1) by immunohistochemistry in lung tissue from nine patients with IPF and five nonsmoking normal subjects. The results demonstrated that in normal lungs, ICAM-1 was weakly expressed on endothelial cells, but neither E-selectin nor VCAM-1 was detected. In the lungs of patients with IPF, E-selectin expression on endothelial cells was restricted to honeycombing regions. Endothelial expression of ICAM-1 was increased throughout the tissue, but VCAM-1 was not detected in IPF. The distribution of leukocytes in lungs with IPF consisted of mostly lymphocyte accumulation in the interstitium and neutrophil accumulation within the airspaces of honeycomb regions. These results suggest that E-selectin may play a role in the recruitment of neutrophils in regions of honeycombing and that ICAM-1 may play a role in lymphocyte recruitment into the interstitium in addition to contributing to neutrophil recruitment in regions of honeycombing in patients with IPF.
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Affiliation(s)
- A Nakao
- First Department of Internal Medicine, Nagoya University School of Medicine, Japan
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