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Prasad P, Kumar A, Yadav N, Sharma VK. Hiding in plain sight: Microfilaria in a case of splenic littoral cell angioma. INDIAN J PATHOL MICR 2024; 67:720-722. [PMID: 39078983 DOI: 10.4103/ijpm.ijpm_818_22] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/12/2022] [Accepted: 01/22/2023] [Indexed: 01/30/2025] Open
Affiliation(s)
- Pallavi Prasad
- Department of Pathology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
| | - Ashok Kumar
- Department of Pathology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
| | - Neha Yadav
- Department of Pathology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
| | - Vipin K Sharma
- Department of Surgical Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
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Gulati S, Jeon H, Vijay A. Littoral cell angiomas: Benign lesion with a penchant for visceral malignancies. Ann Hepatobiliary Pancreat Surg 2023; 27:1-5. [PMID: 36380480 PMCID: PMC9947364 DOI: 10.14701/ahbps.22-062] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/05/2022] [Revised: 08/31/2022] [Accepted: 09/07/2022] [Indexed: 11/17/2022] Open
Abstract
Littoral cell angiomas are rare vascular tumors of the spleen. Because of their rarity, unclear etiopathogenesis, and association with other malignancies, these tumors can pose diagnostic and therapeutic challenges. Due to paucity of published literature on this entity often limited to case reports, relevant data on this topic were procured and synthesized with the aid of a comprehensive Medline search in addition to oncologic, pathologic, radiologic, and surgical literature review on littoral cell angiomas. This article provides an in-depth review into postulated etiopathogenesis, pathology, clinical manifestations, associated malignancies, and prognostic features of littoral cell angiomas.
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Affiliation(s)
- Snigdha Gulati
- Department of Cardio-Thoracic and Neurosurgery, All India Institute of Medical Sciences (AIIMS), New Delhi, India
| | - Hoonbae Jeon
- Division of Hepato-Pancreato-Biliary & Abdominal Transplant Surgery, Tulane University School of Medicine, New Orleans, LA, United States
| | - Adarsh Vijay
- Division of Hepato-Pancreato-Biliary & Abdominal Transplant Surgery, Tulane University School of Medicine, New Orleans, LA, United States,Corresponding author: Adarsh Vijay, MD Division of Hepato-Pancreato-Biliary & Abdominal Transplant Surgery, Tulane Abdominal Transplant Institute, Tulane University School of Medicine, 1415 Tulane Ave., #HC-05, New Orleans, LA 70112-2632, United States Tel: +1-504-988-0794, Fax: +1-504-988-7510, E-mail: ORCID: https://orcid.org/0000-0002-6108-5218
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Arcuri PP, Taglianetti S, Vavalà B, Battaglia C, Laganà D, Manti F. Incidental littoral cell angioma of the spleen: cross-sectional imaging findings and review of the literature. Radiol Case Rep 2022; 17:3545-3550. [PMID: 35923330 PMCID: PMC9340147 DOI: 10.1016/j.radcr.2022.06.063] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/18/2022] [Revised: 06/13/2022] [Accepted: 06/21/2022] [Indexed: 11/24/2022] Open
Abstract
Littoral cell angioma (LCA) is a primary splenic hemangioma found mostly in normal red sinus shore cells of the reticuloendothelial cell system of the spleen. In most cases is benign, but sometimes malignancies have been reported. This tumor displayed epithelial and histiocytic properties based on its cell of origin, splenic littoral cells. In this case report, we will describe a case of a 21-year-male presenting with an incidentally discovered LCA illustrated by cross-sectional imaging techniques, highlighting how the diffusional sequence and the positron emission tomography study, thanks to their greater specificity, have contributed to reaching a correct diagnostic orientation more than dynamic studies with contrast agent in both computed tomography and magnetic resonance.
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Sangiorgio VFI, Arber DA. Vascular neoplasms and non-neoplastic vascular lesions of the spleen. Semin Diagn Pathol 2020; 38:154-158. [PMID: 32674844 DOI: 10.1053/j.semdp.2020.07.001] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/14/2020] [Revised: 06/26/2020] [Accepted: 07/02/2020] [Indexed: 02/02/2023]
Abstract
Vascular neoplasms are among the most common conditions affecting the spleen. The majority of these are idiopathic, benign in nature and asymptomatic and therefore treated with a conservative management. Only rare cases cause splenomegaly and/or chronic consumption coagulopathies, thus requiring splenectomy. Among these, the most common is splenic hemangioma, followed by littoral cell angioma and lymphangioma. Peliosis is a peculiar tumor-like non-neoplastic vascular lesion that diffusely affects the spleen and frequently presents with concomitant hepatic involvement. As a distinctive feature, peliosis can occur as a secondary manifestation of infections, malignancies and in individuals using certain drugs. On the opposite spectrum of clinical behavior lies splenic angiosarcoma, a vascular endothelial malignancy with aggressive presentation and poor prognosis. In some cases the endothelial nature of this neoplasm may not be evident on routine histologic examination and immunohistochemistry is used to disclose such phenotype. The term hemangioendothelioma is rarely used to describe borderline vascular neoplasms which appear more aggressive than conventional hemangiomas, but that do not entirely fulfill the diagnostic criteria for angiosarcoma. Some of these neoplasms coexpress endothelial and histiocytic markers and therefore have been proposed as the borderline counterpart of littoral cell angioma. The existence of hemangioendothelioma as a diagnostic entity per se is debated and this diagnosis should be rendered with caution. The current review aims at highlighting the main histologic features of vascular neoplasms and non-neoplastic vascular lesions of the spleen.
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Affiliation(s)
| | - Daniel A Arber
- Department of Pathology, University of Chicago, Chicago, IL, USA.
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5
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McHugh A, O’Gorman C, Cooley S, McEntee G, Burke N. Littoral cell angioma with splenic rupture in pregnancy. CASE REPORTS IN PERINATAL MEDICINE 2018. [DOI: 10.1515/crpm-2017-0064] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/15/2022]
Abstract
Abstract
Splenic rupture in pregnancy is a rare event. It is generally due to trauma or an underlying splenic pathology. Timely diagnosis and intervention of splenic rupture is essential given the high rates of associated maternal and fetal morbidity and mortality. This case illustrates a 38 year old lady in the third trimester of pregnancy who presented with maternal collapse and a non-reassuring foetal heart rate tracing following a fall at home one week previously. A massive hemoperitoneum was identified at caesarean section and a splenectomy was performed. Histological examination revealed a littoral cell angioma of the spleen. This vascular tumour arising in the splenic red pulp sinuses is a rare entity and specific immunophenotypic features help distinguish it from other vascular tumours of the spleen. Both trauma and an underlying splenic pathology led to splenic rupture in this unique case.
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Affiliation(s)
- Ann McHugh
- Rotunda Hospital, Department of Obstetrics and Gynaecology , Dublin , Ireland
| | - Catherine O’Gorman
- Rotunda Hospital, Department of Obstetrics and Gynaecology , Dublin , Ireland
| | - Sharon Cooley
- Rotunda Hospital, Department of Obstetrics and Gynaecology , Dublin , Ireland
| | - Gerry McEntee
- Mater Misericordiae University Hospital , Dublin , Ireland
| | - Naomi Burke
- Rotunda Hospital, Department of Obstetrics and Gynaecology , Dublin , Ireland
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6
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Peckova K, Michal M, Hadravsky L, Suster S, Damjanov I, Miesbauerova M, Kazakov DV, Vernerova Z, Michal M. Littoral cell angioma of the spleen: a study of 25 cases with confirmation of frequent association with visceral malignancies. Histopathology 2016; 69:762-774. [PMID: 27374010 DOI: 10.1111/his.13026] [Citation(s) in RCA: 31] [Impact Index Per Article: 3.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/01/2016] [Revised: 06/28/2016] [Accepted: 06/30/2016] [Indexed: 12/20/2022]
Abstract
AIMS Littoral cell angioma (LCA) is a rare primary splenic tumour that is frequently associated with internal malignancies. Immunohistochemistry can demonstrate a distinct hybrid endothelial-histiocytic phenotype of littoral cells, and is a helpful adjunct for making the correct diagnosis. The aims of this study were to present a series of 25 LCAs, with an emphasis on the frequent association of the neoplasm with visceral malignancies, and to provide a detailed immunohistochemical analysis by employing new markers. METHODS AND RESULTS All 25 cases with available tissue blocks were immunohistochemically stained for endothelial and histiocytic markers. Clinical and follow-up data were retrieved from the respective institutions. The tumours were obtained from 16 males and nine females, whose age ranged from 32 to 86 years (mean 56.2 years). Clinical information was available for 24 of 25 patients, and follow-up for 11 of 25 patients (range 2-19 years; mean 11.6 years). Immunohistochemically, all cases were positive for LYVE-1, factor VIII, FLI-1, vascular endothelial growth factor receptor (VEGFR)-2, VEGFR-3, claudin-5, ERG, LMO2, CD31, CD163, lysozyme, and CD4, but negative for D2-40, CD8, and factor XIIIa. Fifteen of 25 cases were associated with various malignancies, including epithelial, mesenchymal and haematological tumours. CONCLUSIONS The cohort of 25 patients is the largest series of LCAs published to date. By using antibodies against recently introduced endothelial markers, we have expanded the immunoprofile of LCA. We have further highlighted the clinical significance of LCA, as more than half of the patients in this study also harboured a coexisting visceral malignancy. Therefore, we conclude that the finding of splenic LCA mandates a thorough clinical evaluation for a concomitant malignancy.
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Affiliation(s)
- Kvetoslava Peckova
- Department of Pathology, Charles University, Medical Faculty and Charles University Hospital Plzen, Pilsen, Czech Republic.
| | - Michael Michal
- Department of Pathology, Charles University, Medical Faculty and Charles University Hospital Plzen, Pilsen, Czech Republic.,Biomedical Centre, Faculty of Medicine in Plzen and Charles University Hospital Plzen, Pilsen, Czech Republic
| | - Ladislav Hadravsky
- Department of Pathology, Charles University, Medical Faculty and Charles University Hospital Plzen, Pilsen, Czech Republic
| | - Saul Suster
- Department of Pathology and MCW Cancer Center, Medical College of Wisconsin, Milwaukee, WI, USA
| | - Ivan Damjanov
- Department of Pathology and Laboratory Medicine, The University of Kansas School of Medicine, Kansas City, KS, USA
| | - Marketa Miesbauerova
- Department of Pathology, Charles University, Medical Faculty and Charles University Hospital Plzen, Pilsen, Czech Republic
| | - Dmitry V Kazakov
- Department of Pathology, Charles University, Medical Faculty and Charles University Hospital Plzen, Pilsen, Czech Republic
| | - Zdenka Vernerova
- Department of Pathology, Charles University, Third Medical Faculty and Charles University Hospital Kralovske Vinohrady, Prague, Czech Republic
| | - Michal Michal
- Department of Pathology, Charles University, Medical Faculty and Charles University Hospital Plzen, Pilsen, Czech Republic
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Bailey A, Vos J, Cardinal J. Littoral cell angioma: A case report. World J Clin Cases 2015; 3:894-899. [PMID: 26488026 PMCID: PMC4607808 DOI: 10.12998/wjcc.v3.i10.894] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/14/2015] [Revised: 06/21/2015] [Accepted: 08/07/2015] [Indexed: 02/05/2023] Open
Abstract
Primary splenic lesions are rare entities among which littoral cell angioma (LCA) is a recently described, uncommon vascular lesion that is unique to the spleen. It has heretofore been described primarily in pathologic series and has been found mostly to behave as a benign entity. A few reports of malignant variants have been reported. We present a case report of a solitary LCA discovered after splenectomy for an incidentally discovered splenic lesion, along with a literature review.
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Namdaroglu OB, Sevim Y, Yildirim O, Ozhamam E, Kocer B. Littoral cell angioma of the spleen: case report and literature review. Eur Surg 2015. [DOI: 10.1007/s10353-015-0332-x] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/30/2022]
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Cai YQ, Wang X, Ran X, Liu XB, Peng B. Laparoscopic splenectomy for splenic littoral cell angioma. World J Gastroenterol 2015; 21:6660-6664. [PMID: 26074704 PMCID: PMC4458776 DOI: 10.3748/wjg.v21.i21.6660] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/26/2014] [Revised: 07/17/2014] [Accepted: 09/05/2014] [Indexed: 02/06/2023] Open
Abstract
AIM: To establish the safety and feasibility of laparoscopic splenectomy (LS) for littoral cell angioma (LCA).
METHODS: From September 2003 to November 2013, 27 patients were diagnosed with LCA in our institution. These patients were divided into two groups based on operative procedure: LS (13 cases, Group 1) and open splenectomy (14 cases, Group 2). Data were collected retrospectively by chart review. Comparisons were performed between the two groups in terms of demographic characteristics (sex and age); operative outcomes (operative time, estimated blood loss, transfusion, and conversion); postoperative details (length of postoperative stay and complications); and follow-up outcome.
RESULTS: LS was successfully carried out in all patients except one in Group 1, who required conversion to hand-assisted LS because of perisplenic adhesions. The average operative time for patients in Group 1 was significantly shorter than that in Group 2 (127 ± 34 min vs 177 ± 25 min, P = 0.001). The average estimated blood loss in Group 1 was significantly lower than in Group 2 (62 ± 48 mL vs 138 ± 64 mL, P < 0.01). No patient in Group 1 required a blood transfusion, whereas one in Group 2 required a transfusion. Two patients in Group 1 and four in Group 2 suffered from postoperative complications. All the complications were cured by conservative therapy. There were no deaths in our series. All patients were followed up and no recurrence or abdominal metastasis were found.
CONCLUSION: LS for patients with LCA is safe and feasible, with preferable operative outcomes and long-term tumor-free survival.
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Zhang YH, Liu LM, Wang WP, Ding H, Wang XN, Xia HS. Littoral cell angioma of the spleen: sonographic-pathologic comparison. JOURNAL OF ULTRASOUND IN MEDICINE : OFFICIAL JOURNAL OF THE AMERICAN INSTITUTE OF ULTRASOUND IN MEDICINE 2013; 32:691-697. [PMID: 23525396 DOI: 10.7863/jum.2013.32.4.691] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 06/02/2023]
Abstract
OBJECTIVES The purpose of this study was to evaluate the sonographic and pathologic features of littoral cell angioma of the spleen in 7 patients. METHODS The sonographic appearance in 7 cases of littoral cell angioma confirmed by surgical pathologic examination was retrospectively reviewed. All underwent color Doppler imaging. Two underwent contrast-enhanced sonography. The sonographic appearance was compared with pathologic findings. RESULTS Splenic lesions were solitary in 5 cases and multiple in 2 cases. The masses ranged from 10 to 64 mm in maximum diameter. Five hypoechoic and 2 hyperechoic lesions on grayscale sonography corresponded to few and multiple blood-filled spaces on pathologic examination, respectively. Four hypovascular lesions, 1 hypervascular lesion, and the other 2 hypervascular lesions full of color flow signals on color Doppler imaging corresponded to few, several, and multiple arteries on pathologic examination. On contrast-enhanced sonography, 1 hypervascular lesion full of color flow signals showed homogeneous hyperenhancement for 8 minutes during the arterial and parenchymal phases. One hypovascular lesion showed inhomogeneous isoenhancement transiently during the arterial phase and became hypoechoic later. CONCLUSIONS Littoral cell angioma is a primary vascular splenic neoplasm with variable features on grayscale sonography and color Doppler imaging as well as contrast-enhanced sonography. The sonographic appearance of littoral cell angioma mainly depends on the type and number of tumor vessels.
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Affiliation(s)
- Yun-Hua Zhang
- Department of Ultrasound, Zhongshan Hospital, Fudan University, Shanghai, China
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11
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Cordesmeyer S, Pützler M, Titze U, Paulus H, Hoffmann MW. Littoral cell angioma of the spleen in a patient with previous pulmonary sarcoidosis: a TNF-α related pathogenesis? World J Surg Oncol 2011; 9:106. [PMID: 21929754 PMCID: PMC3187736 DOI: 10.1186/1477-7819-9-106] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/14/2011] [Accepted: 09/19/2011] [Indexed: 12/14/2022] Open
Abstract
BACKGROUND Littoral cell angioma (LCA) is a rare vascular tumor of the spleen. Generally thought to be benign, additional cases of LCA with malignant features have been described. Thus, its malignant potential seems to vary and must be considered uncertain. The etiology remains unclear, but an immune dysregulation for the apparent association with malignancies of visceral organs or immune-mediated diseases has been proposed. CASE PRESENTATION We report a case of LCA in a 43-year old male patient who presented with a loss of appetite and intermittent upper abdominal pain. Computed tomography showed multiple hypoattenuating splenic lesions which were hyperechogenic on abdominal ultrasound. Lymphoma was presumed and splenectomy was performed. Pathological evaluation revealed LCA. CONCLUSIONS LCA is a rare, primary vascular neoplasm of the spleen that might etiologically be associated with immune dysregulation. In addition, it shows a striking association with synchronous or prior malignancies. With about one-third of the reported cases to date being co-existent with malignancies of visceral organs or immune-mediated diseases, this advocates for close follow-ups in all patients diagnosed with LCA. To our knowledge, this report is the first one of LCA associated with previous pulmonary sarcoidosis and hypothesizes a TNF-α related pathogenesis of this splenic tumor.
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Bhavsar T, Wang C, Huang Y, Karachristos A, Inniss S. Littoral cell angiomas of the spleen associated with solid pseudopapillary tumor of the pancreas. World J Gastrointest Pathophysiol 2011; 2:53-6. [PMID: 21860837 PMCID: PMC3158892 DOI: 10.4291/wjgp.v2.i3.53] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/21/2010] [Revised: 03/31/2011] [Accepted: 04/07/2011] [Indexed: 02/06/2023] Open
Abstract
Littoral cell angiomas (LCA) of the spleen are vascular tumors of unknown etiology arising from the littoral cells of the splenic red pulp sinuses. Usually a benign and incidental finding, LCA have been repeatedly reported in association with a variety of visceral malignancies and hold the potential for dissemination per se. We encountered a case of a 30 year old female who was diagnosed with solid pseudopapillary tumor of the head and distal pancreas by fine needle aspiration cytology. A distal pancreatectomy with splenectomy was performed in addition to a pylorus-preserving Whipple’s procedure and cholecystectomy. Histopathological examination confirmed solid pseudopapillary tumor of the pancreas and showed multiple well-circumscribed anastomosing vascular channels in the spleen. The diagnosis of LCA of the spleen was confirmed by immunohistochemistry that revealed co-expression of endothelial cell marker, CD31 and CD34, along with histiocytic marker, CD68 by the vascular lining cells. LCA has been previously reported in association with colorectal and pancreatic adenocarcinoma, malignant lymphoma, myelodysplasia and autoimmune disorders. We report the first case of LCA associated with solid pseudopapillary tumor of the pancreas.
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Yamate J, Izawa T, Kuwamura M, Mitsunaga F, Nakamura S. Vasoformative disorder, resembling littoral cell angioma, of the spleen in a geriatric Japanese macaque (Macaca fuscata). Vet Pathol 2009; 46:520-5. [PMID: 19176500 DOI: 10.1354/vp.08-vp-0228-y-cr] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/02/2023]
Abstract
A 30-year-old female Japanese macaque showed marked splenomegaly. The enlarged spleen consisted of neoplastic proliferation of anastomosing vascular channels resembling morphologic structures of red pulp sinuses; occasionally, papillary fronds were seen in dilated channels. Immunohistochemically, the lining cells reacted to both endothelial cell (von Willebrand factor) and macrophage (macrophage scavenger receptor class A) markers, indicating features of littoral cells of the spleen. Based on the pathologic characteristics, particularly the presence of neoplastic cells with macrophage/histiocyte-like attributes, this tumor was regarded as littoral cell angioma; this is a rare benign splenic vascular tumor.
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Affiliation(s)
- J Yamate
- Laboratory of Veterinary Pathology, Life and Environmental Sciences, Osaka Prefecture University, Gakuencho 1-1, Nakaku, sakai, Osaka 599-8531, Japan.
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Tee M, Vos P, Zetler P, Wiseman SM. Incidental littoral cell angioma of the spleen. World J Surg Oncol 2008; 6:87. [PMID: 18713469 PMCID: PMC2527567 DOI: 10.1186/1477-7819-6-87] [Citation(s) in RCA: 20] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/14/2008] [Accepted: 08/19/2008] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Littoral cell angioma (LCA) is a recently described primary vascular neoplasm of the spleen that may be associated with other malignancies and may itself also have malignant potential. CASE PRESENTATION We present a case of LCA that was discovered incidentally in a 52-year-old woman who presented with biliary colic at the time of consultation for cholecystectomy. This vascular neoplasm was evaluated by ultrasound, CT, MRI, Tc-99m labelled red blood cell scintigraphy, and core biopsy. A splenectomy revealed LCA by pathological evaluation. Post-operative outcome was favourable with no evidence of complication or recurrent disease. Following this case presentation, clinical, radiographic, and pathological features of LCA will be reviewed as well as recent advances in our understanding of this uncommon splenic lesion. CONCLUSION LCA is a rare, generally benign, primary vascular tumour of the spleen that typically is discovered incidentally. Individuals diagnosed with this tumour must be carefully evaluated to exclude primary, secondary, and synchronous malignancies.
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Affiliation(s)
- May Tee
- Faculty of Medicine, University of British Columbia, Vancouver, British Columbia, Canada.
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15
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Li R, Morris SW. Development of anaplastic lymphoma kinase (ALK) small-molecule inhibitors for cancer therapy. Med Res Rev 2008; 28:372-412. [PMID: 17694547 DOI: 10.1002/med.20109] [Citation(s) in RCA: 107] [Impact Index Per Article: 6.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/26/2023]
Abstract
Anaplastic lymphoma kinase (ALK) is a receptor tyrosine kinase (RTK) involved in the genesis of several human cancers; indeed, ALK was initially identified in constitutively activated and oncogenic fusion forms--the most common being nucleophosmin (NPM)-ALK--in a non-Hodgkin's lymphoma (NHL) known as anaplastic large-cell lymphoma (ALCL) and subsequent studies identified ALK fusions in the human sarcomas called inflammatory myofibroblastic tumors (IMTs). In addition, two recent reports have suggested that the ALK fusion, TPM4-ALK, may be involved in the genesis of a subset of esophageal squamous cell carcinomas. While the cause-effect relationship between ALK fusions and malignancies such as ALCL and IMT is very well established, more circumstantial links implicate the involvement of the full-length, normal ALK receptor in the genesis of additional malignancies including glioblastoma, neuroblastoma, breast cancer, and others; in these instances, ALK is believed to foster tumorigenesis following activation by autocrine and/or paracrine growth loops involving the reported ALK ligands, pleiotrophin (PTN) and midkine (MK). There are no currently available ALK small-molecule inhibitors approved for clinical cancer therapy; however, recognition of the variety of malignancies in which ALK may play a causative role has recently begun to prompt developmental efforts in this area. This review provides a succinct summary of normal ALK biology, the confirmed and putative roles of ALK fusions and the full-length ALK receptor in the development of human cancers, and efforts to target ALK using small-molecule kinase inhibitors.
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Affiliation(s)
- Rongshi Li
- High-Throughput Medicinal Chemistry, ChemBridge Research Laboratories, 16981 Via Tazon, Suites K, San Diego, California 92127, USA.
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16
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Qu ZB, Liu LX, Wu LF, Zhao S, Jiang HC. Multiple littoral cell angioma of the spleen: a case report and review of the literature. Oncol Res Treat 2007; 30:256-8. [PMID: 17460421 DOI: 10.1159/000101010] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/30/2023]
Abstract
BACKGROUND Littoral cell angioma (LCA) is a rare benign vascular tumor of the spleen with characteristic histomorphologic features. It is a unique splenic tumor which may present with abdominal pain or as an incidental finding. CASE REPORT In this case report, we present the case of a 34-year-old woman with multiple splenic LCA. Initially, the patient presented with abdominal pain, weakness and fatigue. After a diagnostic abdominal ultrasound, multiple hemangiomas were observed in the spleen. Computed tomography scans confirmed splenomegaly with multiple round and hyperdense lesions. The patient subsequently underwent splenectomy. Postoperative histological and immunohistochemical profiles confirmed the diagnosis of LCA. CONCLUSION LCA is a unique splenic tumor diagnosed in patients with abdominal pain or as an incidental finding. Only a few case reports of this tumor have been published, and those accounts are inconsistent. In the present paper, we report on a case and conduct a literature review.
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Affiliation(s)
- Zhi-Bo Qu
- Department of General Surgery, First Clinical College of Harbin Medical University, P.R. China
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17
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Ramdall RB, Alasio TM, Cai G, Yang GCH. Primary vascular neoplasms unique to the spleen: littoral cell angioma and splenic hamartoma diagnosis by fine-needle aspiration biopsy. Diagn Cytopathol 2007; 35:137-42. [PMID: 17304535 DOI: 10.1002/dc.20568] [Citation(s) in RCA: 18] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/19/2022]
Abstract
We report the fine-needle aspiration (FNA) biopsy diagnosis of two rare cases of primary vascular neoplasms unique to the spleen: a littoral cell angioma from a 31-yr-old Caucasian woman and a splenic hamartoma from a 46-yr-old black man. The cytologic features of splenic hamartoma and of littoral cell angioma of the spleen were described three times in cytologic literature: two were bench-top aspirates and one was FNA biopsy thought to be metastatic carcinoma. To the best of our knowledge, the current two cases were the first diagnosed by FNA biopsy. Our approach to the FNA biopsy diagnosis of these rare vascular neoplasms via compact cell block and immunohistochemistry is described. The differential diagnosis with other primary vascular splenic neoplasms is also discussed.
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Affiliation(s)
- Risha B Ramdall
- Department of Pathology, New York University School of Medicine, New York, NY 10016, USA
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18
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Harmon RL, Cerruto CA, Scheckner A. Littoral cell angioma: a case report and review. ACTA ACUST UNITED AC 2006; 63:345-50. [PMID: 16971207 DOI: 10.1016/j.cursur.2006.06.011] [Citation(s) in RCA: 29] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/27/2006] [Revised: 05/08/2006] [Accepted: 06/12/2006] [Indexed: 02/07/2023]
Affiliation(s)
- Rhonda L Harmon
- GI/Endocrine Surgery, Department of Surgery, University of Texas Southwestern, Dallas, Texas 75390-9156, USA.
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Erçin C, Gürbüz Y, Hacihanefioğlu A, Turgut Karakaya A. Multiple littoral cell angioma of the spleen in a case of myelodysplastic syndrome. Hematology 2005; 10:141-4. [PMID: 16019460 DOI: 10.1080/10245330400026121] [Citation(s) in RCA: 16] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/17/2023] Open
Abstract
Littoral cell angioma (LCA) of the spleen is a relatively recently described rare entity, which is associated with haemophagocytosis. In this case report, we will present a case of myelodysplastic syndrome with multiple LCA of the spleen. The patient presented with pancytopenia and multiple haemangiomas were observed in spleen during diagnostic abdominal ultrasound. She required a transfusion of one to two units of packed blood every 4-6 weeks and she eventually underwent a splenectomy in order to decrease the transfusion requirement. Although the patient's pancytopenia improved after surgery, the patient did not achieve a normal haemoglobin level in the two years following the splenectomy. Following further studies, bone marrow aspiration was undertaken and demonstrated minimal dysplasia in erythroid series including nuclear abnormalities, irregular cytoplasmic borders and irregular staining of the cytoplasm. The patient was diagnosed as myelodysplastic syndrome. As LCA of the spleen and myelodysplastic syndrome may both cause cytopenia their co-existence may complicate the clinical presentation leading to an incomplete or inappropriate diagnosis. In cases of haematological disorders with an atypical course, the radiological examination of the spleen should be considered and the possibility of angiomas must be included to the differential diagnosis.
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Affiliation(s)
- Cengiz Erçin
- Department of Pathology, University of Kocaeli Medical School, Derince, Sopali, Kocaeli, 41900, Turkey
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Kim HG, Park IS, Lee JI, Jeong S, Lee JW, Kwon KS, Lee DH, Kim PS, Kim HG, Shin YW, Kim YS, Ahn IS, Lee KY. Littoral cell angioma (LCA) associated with liver cirrhosis. Yonsei Med J 2005; 46:184-8. [PMID: 15744827 PMCID: PMC2823050 DOI: 10.3349/ymj.2005.46.1.184] [Citation(s) in RCA: 12] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/22/2023] Open
Abstract
A littoral cell angioma (LCA) is a rare benign vascular tumor of the spleen. A 60-year-old man, with multiple nodules in imaging study and liver cirrhosis graded as Child-Pugh classification class A, was transferred for splenomegaly. A thrombocytopenia was found on hematological evaluation. Because there was no evidence of hematological and visceral malignancy, a splenectomy was performed for a definitive diagnosis. The histological and immunohistochemical features of the splenic specimens were consistent with a LCA. After the splenectomy, the thrombocytopenia recovered to the normal platelet count. There has been no previous report of a LCA combined with liver cirrhosis. Herein, the first case of a LCA in Korea, diagnosed and treated by a splenectomy, is reported.
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Affiliation(s)
- Hi-Gu Kim
- Department of Internal Medicine, Inha University, Incheon, Korea
| | - In-Suh Park
- Department of Pathology, Inha University, Incheon, Korea
| | - Jung-Il Lee
- Department of Internal Medicine, Inha University, Incheon, Korea
| | - Seok Jeong
- Department of Internal Medicine, Inha University, Incheon, Korea
| | - Jin-Woo Lee
- Department of Internal Medicine, Inha University, Incheon, Korea
| | - Kye-Suk Kwon
- Department of Internal Medicine, Inha University, Incheon, Korea
| | - Don-Haeng Lee
- Department of Internal Medicine, Inha University, Incheon, Korea
| | - Pum-Soo Kim
- Department of Internal Medicine, Inha University, Incheon, Korea
| | - Hyung-Gil Kim
- Department of Internal Medicine, Inha University, Incheon, Korea
| | - Yong-Woon Shin
- Department of Internal Medicine, Inha University, Incheon, Korea
| | - Young-Soo Kim
- Department of Internal Medicine, Inha University, Incheon, Korea
| | - In-Sun Ahn
- Department of Internal Medicine, Inha University, Incheon, Korea
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Abstract
Primary vascular neoplasms of the spleen constitute the majority of nonhematolymphoid splenic tumors. The benign primary vascular tumors include hemangioma, hamartoma, and lymphangioma, whereas those of variable or uncertain biologic behavior include littoral cell angioma, hemangioendothelioma, and hemangiopericytoma. The primary malignant vascular neoplasm of the spleen is angiosarcoma. Peliosis is a rare lesion of unknown cause that is usually found incidentally in asymptomatic patients but may be associated with hematologic or metastatic disease. Although these vascular neoplasms of the spleen are uncommon, their importance lies in that they must be differentiated from the more common neoplastic disorders of the spleen, such as lymphoma and metastasis. The most common echogenic solid or complex cystic mass in an asymptomatic patient is splenic hemangioma. However, the imaging appearance of splenic hemangiomas may be complex, and differentiation of these lesions from malignant disease may not be possible. The diagnosis of splenic hamartoma may be suggested when findings of increased blood flow on color Doppler images are seen in association with a homogeneous solid echogenic mass. A large subcapsular solitary cystic abnormality discovered incidentally in a child in association with internal septations and tiny mural nodules favors the diagnosis of lymphangioma. Any invasion of the surrounding splenic parenchyma by a splenic lesion should indicate a more aggressive or malignant process. Evaluation of a focal splenic abnormality identified on sonograms should be followed up with computed tomography or magnetic resonance imaging with and without contrast material enhancement. Splenectomy may be required for definitive evaluation of a splenic mass with atypical features.
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Pulford K, Morris SW, Turturro F. Anaplastic lymphoma kinase proteins in growth control and cancer. J Cell Physiol 2004; 199:330-58. [PMID: 15095281 DOI: 10.1002/jcp.10472] [Citation(s) in RCA: 182] [Impact Index Per Article: 8.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/04/2023]
Abstract
The normal functions of full-length anaplastic lymphoma kinase (ALK) remain to be completely elucidated. Although considered to be important in neural development, recent studies in Drosophila also highlight a role for ALK in gut muscle differentiation. Indeed, the Drosophila model offers a future arena for the study of ALK, its ligands and signalling cascades. The discovery of activated fusion forms of the ALK tyrosine kinase in anaplastic large cell lymphoma (ALCL) has dramatically improved our understanding of the pathogenesis of these lymphomas and enhanced the pathological diagnosis of this subtype of non-Hodgkin's lymphoma (NHL). Likewise, the realisation that a high percentage of inflammatory myofibroblastic tumours express activated-ALK fusion proteins has clarified the causation of these mesenchymal neoplasms and provided for their easier discrimination from other mesenchymal-derived inflammatory myofibroblastic tumour (IMT) mimics. Recent reports of ALK expression in a range of carcinoma-derived cell lines together with its apparent role as a receptor for PTN and MK, both of which have been implicated in tumourigenesis, raise the possibility that ALK-mediated signalling could play a role in the development and/or progression of a number of common solid tumours. The therapeutic targeting of ALK may prove to have efficacy in the treatment of many of these neoplasms.
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Affiliation(s)
- K Pulford
- Leukaemia Research Fund Immunodiagnostics Unit, Nuffield Department of Clinical Laboratory Sciences, University of Oxford, John Radcliffe Hospital, Oxford, United Kingdom.
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Abstract
Splenic vascular tumors are uncommon and are more typically encountered as benign incidental findings. By contrast, splenic angiosarcoma may present acutely and dramatically and typically pursues a very aggressive clinical course. Vascular tumors in the spleen may show conventional endothelial, specialized endothelial (sinusoidal/littoral cell) or lymphatic differentiation and there is morphologic overlap between some of the currently defined diagnostic categories, within which benign, intermediate, and malignant subsets are recognized. The greatest problem in trying to better define and analyze these tumors is the availability of only relatively small case numbers. This overview describes the diagnosis and differential diagnosis of splenic vascular tumors as presently understood.
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Affiliation(s)
- Jeffery L Kutok
- Department of Pathology, Brigham and Women's Hospital and Harvard Medical School, Boston, MA 02115, USA
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Goldfeld M, Cohen I, Loberant N, Mugrabi A, Katz I, Papura S, Noi I. Littoral cell angioma of the spleen: appearance on sonography and CT. JOURNAL OF CLINICAL ULTRASOUND : JCU 2002; 30:510-513. [PMID: 12242742 DOI: 10.1002/jcu.10101] [Citation(s) in RCA: 17] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 05/23/2023]
Abstract
Littoral cell angioma (LCA) is a rare benign vascular tumor of the spleen with characteristic histomorphologic features. Only a few descriptions of the radiologic appearance of this tumor have been published, and those descriptions are variable. We report a case of LCA in a 37-year-old man with psoriasis and nonspecific symptoms of weakness, pain and fatigue, normocytic anemia, and thrombocytopenia. The results of abdominal sonography and contrast-enhanced CT correlated: the 2 modalities revealed hepatosplenomegaly and multiple round splenic lesions of similar appearance and size (on sonograms, ill-defined echogenic lesions up to 3.2 cm without acoustic enhancement; on CT scans, hypodense, nonenhancing lesions up to 3.5 cm). Because making a differential diagnosis was difficult and our presumptive diagnosis was hemangioma or lymphoma, splenectomy was performed. Postoperative pathologic examinations confirmed a final diagnosis of LCA. The patient's recovery was uneventful. LCA should be considered when making a differential diagnosis of splenic lesions, and sonography may be more helpful than CT in reaching a diagnosis of LCA.
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Affiliation(s)
- Moshe Goldfeld
- Department of Radiology, Western Galilee Hospital, POB 21, Nahariya, Israel
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