Littoral Cell Angioma Research Articles

Primary Liposarcoma of the Spleen: Case Report With Review of the Literature.

We present a 55-year-old woman with a palpable mass in the right upper abdomen. Contrast-enhanced computed tomography showed a highly vascularized and primary resectable tumor of the spleen. We performed an open splenectomy with locoregional lymphadenectomy. Histopathological and immunohistochemical evaluation did not reveal classic markers of angiosarcoma, littoral cell angioma, or follicular dendritic cell sarcoma, but showed MDM2-amplification by FISH in all tumor cells as a marker for liposarcoma. The tumor showed growth limited to the spleen tissue. No MDM2-amplification was detectable in the perisplenic adipose tissue, so that infiltration of the spleen by retroperitoneal liposarcoma could be excluded. In summary, well-differentiated liposarcoma confined to the spleen was diagnosed. To the best of our knowledge, this is the first description of a primary liposarcoma of the spleen reported in the literature. Due to the local recurrence risk of liposarcomas, even after R0 resection, we recommended long-term periodic follow-up.

Littoral cell angioma: An incidental finding in a traumatic spleen

Littoral cell angioma (LCA) of the spleen, a rare vascular tumor, is usually asymptomatic, often discovered incidentally and a majority of them are benign in nature. A 20-year-old boy with a history of blunt abdominal trauma, who underwent splenectomy showed the presence of a capsular tear and variegated outer surface on gross examination of the spleen. On the cut section, multiple nodular areas with areas of hemorrhage were noted. Microscopical examination revealed the proliferation of anastomosing, irregular, tortuous blood-filled channels with plenty of histiocytes in the splenic tissue and extensive areas of hemorrhage, inflammatory cell infiltration, and fibrinoid deposits. Immunohistochemistry revealed positivity for CD31 (endothelial differentiation), CD68, and lysozyme (histiocytic differentiation) but was negative for CD34. The final diagnosis was LCA in a case of ruptured spleen after ruling out angiosarcoma. The possibility of this benign entity to have contributed to the potentially fatal splenic injury cannot be overlooked.

Surgical treatment of littoral cell angioma of the spleen

Littoral cell angioma is a very rare benign splenic neoplasm with typical morphological features. It is difficult to differentiate this angioma from other benign or malignant splenic tumors. It is a unique vascular tumor followed by abdominal pain or detected incidentally during examination. Incidence of this tumor is small, as only few appropriate cases were described in the literature. We present successful surgical treatment of littoral cell angioma of the spleen. The issues of clinical course, diagnosis, therapeutic tactics, morphologic and histochemical features are discussed.

Sclerosing Angiomatoid Nodular Transformation of The Spleen: A report of a rare entity

Abstract Introduction/Objective Sclerosing angiomatoid nodular transformation (SANT) is a benign vascular lesion of spleen with uncertain etiology. It predominately affects women between 30 to 60 years of age. Clinically, it is asymptomatic or can cause abdominal pain, but usually discovered incidentally on imaging, which can identify a mass but may not provide a definitive diagnosis. In uncertain vascular lesions, there is always a risk of spontaneous rupture of large vessels and potential for spreading malignancy. Hence, the final diagnosis is rendered on microscopy after splenectomy Methods/Case Report A middle age female came to clinic complaining of abdominal pain. Radiology showed solid splenic mass and the patient underwent splenectomy. Gross examination showed a 3 cm white firm mass with focal hemorrhage. Microscopy revealed multiple nodules of variable sizes surrounded by fibrosclerotic stroma. The nodules showed round to slit-like vascular spaces with numerous red blood cells. The internodular stroma consisted of dense fibrous tissue with scattered plump myofibroblasts and lymphoplasmacytic cells. These distinctive features lead to the diagnosis of SANT Results (if a Case Study enter NA) SANT possessed characteristic histologic features with distinctive immunohistochemistry (IHC). IHC reveals three different types of vessels within the nodules: 1. Small veins (CD34- CD31+ CD8-); 2. Sinusoids (CD34- CD31+ CD8+); and 3. Capillaries (CD34+ CD31+ CD8-). All 3 vessels are negative for CD21/CD35 and CD68. Hemangioma and littoral cell angioma are two frequent vascular tumors in the spleen that should be differentiated. Both lesions lack microscopic features of SANT and have only a single type of vessels. The vessels in hemangioma are (CD31+ CD34+ CD8-); while in littoral cell angioma they are (CD31+ CD34- CD8- CD21+ CD68+) Conclusion There are no specific clinical or radiologic findings for SANT. It is important to recognize these characteristic features and to differentiate it from other benign and malignant lesions, such as angiosarcoma. A thorough histopathologic examination and IHC are helpful to make the correct diagnosis

S3385 Diagnosis of Littoral Cell Angioma of the Spleen With Endoscopic Ultrasound-Guided Fine Needle Aspiration Biopsy

S3385 Diagnosis of Littoral Cell Angioma of the Spleen With Endoscopic Ultrasound-Guided Fine Needle Aspiration Biopsy

Case Report: Incidental Littoral Cell Angioma of The Spleen [Benign Splenic Tumor

littoral cell angioma is an unusual benign tumor of the spleen and is primary splenic vascular tumor arises from the normal littoral cells lining the sinus channels of the splenic red pulp we are report a case of 17 years male boy have history of abdomen pain for 2day with fever and he was managed with laparotomy and splenectomy and Postoperative pathology biopsy examination confirmed the final diagnosis of LCA of spleen.

Multimodality imaging findings of splenic littoral cell angioma: a case description and analysis of literature.

Multimodality imaging findings of splenic littoral cell angioma: a case description and analysis of literature.

Case Report: Littoral Cell Angioma in a Patient with Rectal Carcinoma

Case Report: Littoral Cell Angioma in a Patient with Rectal Carcinoma

Laparoscopic management of benign splenic tumors in children

BackgroundThe splenic tumor is relatively rare in children. However, diagnosing and managing this disease remain controversial. This study aimed to evaluate the clinical characteristics and pathological features of benign splenic...

Algorithmic Approach to the Splenic Lesion Based on Radiologic-Pathologic Correlation.

Splenic lesions are commonly discovered incidentally at imaging, without clinical signs or symptoms that may aid in diagnosis. As such, the differential diagnosis and subsequent management are based primarily on imaging characteristics. Much has been written about the myriad pathologic conditions that can occur in the spleen; however, there is little guidance on the approach to an incidental splenic mass. Applying an approach frequently used in imaging to the splenic mass-based on the number and consistency of lesions and refined by supplementary imaging features-allows formulation of a useful differential diagnosis. Solitary cystic masses include true cysts, pseudocysts, and parasitic cysts. When multiple cystic lesions are present, the differential diagnosis expands to include infectious lesions (abscess or microabscesses) and lymphangioma (a benign cystic neoplasm). Hemangioma is the most common solitary solid mass, although other vascular lesions (hamartoma, sclerosing angiomatoid nodular transformation) and nonvascular lesions (inflammatory pseudotumor, lymphoma) manifest as solitary and solid. When multiple solid masses are present, diffuse inflammatory disease (sarcoidosis), littoral cell angioma, and lymphoma should be considered. Malignancies, such as angiosarcoma or metastasis, can manifest as solitary or multiple and solid or cystic masses but are typically associated with symptoms or widespread primary malignancy. Careful assessment of the multimodality imaging characteristics of splenic lesions based on this approach aids the radiologist faced with the incidental splenic lesion. Online supplemental material is available for this article. Work of the U.S. Government published under an exclusive license with the RSNA.

Real-time CEUS-guided biopsy revealing a littoral cell angioma of the spleen after inconclusive CT and MRI findings.

The aim of this case report is to present real-time CEUS-guided biopsy for diagnosing rare benign splenic pathologies after inconclusive findings on cross-sectional imaging. We present the case of a 50-year-old male patient who received a contrast-enhanced computed tomography scan of the thorax during the evaluation for lung transplant due to lung fibrosis, with incidental finding of disseminated hypodense splenic lesions. During follow-up imaging, the patient did not tolerate a complete MRI examination, and two acquired pulse sequences did not confirm the final diagnosis. While CT-guided biopsy revealed no results, CEUS-guided target biopsy with repeated contrast injections showed a benign littoral cell angioma of the spleen. The use of real-time CEUS-guided target biopsy during lesion washout may be a useful tool to improve the accuracy of biopsy and accelerate the diagnosis in patients with parenchymal lesions after inconclusive cross-sectional imaging findings which may pose a challenge for CT-guided biopsy.

Case report: Infrequent littoral cell angioma of the spleen.

Introduction and importanceLittoral cell angioma is a rare solid spleen tumor with uncertain malignant potential. It is usually asymptomatic; therefore, its diagnosis is usually incidental. There are approximately 150 cases reported in the medical literature, but none of them in the Hispanic population.Case presentationWe present a case of a 54-year-old woman who presented to our clinic with nonspecific abdominal pain. Imaging studies show a splenic mass with littoral cell angioma characteristics. The patient underwent an open splenectomy with subsequent histopathologic and immunohistochemical studies that confirmed the presence of a littoral cell angioma of a diameter of 8 × 4.5 × 3.5 cm. The patient was discharged after an uneventful postoperative recovery and was referred to the outpatient clinic for follow up.Clinical discussionThis case report highlights the close relationship between the littoral cell angioma, neoplasias, and autoimmune diseases. Even though LCA has a good prognosis, there is still the possibility of malignant transformation, especially when the spleen weighs 1500 g; our patient's sample pointed towards a benign pathology. LCA has a positive IHC for endothelial and histiocyte tissues. The IHC results of our patient were positive for CD34+ and CD68+, confirming the LCA diagnosis.ConclusionWithin red pulp spleen tumors, LCA should be highly considered as a differential diagnosis in all types of populations. In the case of a confirmed LCA, routine screening for neoplasias and autoimmune diseases should be performed.

Littoral cell angioma of the spleen in a child with Cornelia De Lange syndrome

Littoral Cell Angioma (LCA) is a benign and rare vascular tumor of the spleen. It is uncommon in children, and a comprehensive literature review has yielded only 12 published pediatric cases of LCA. Here we present a seven year old, female who was a known case of Cornelia De Lange syndrome who presented with splenic cysts and underwent splenectomy. Histopathological review revealed LCA.

Littoral cell angioma of the spleen: A case report

Littoral cell angioma of the spleen: A case report

Laparoscopic Central Splenectomy for Littoral Cell Angioma

BackgroundSplenic littoral cell angioma (LCA) is a rare vascular tumor with malignant potential originating from the red pulp of the spleen.1,2 Although laparoscopic splenectomy is the treatment choice for LCA,3 patients suffer from long-term complications of sepsis, thrombosis, and tumor.4,5 Laparoscopic central splenectomy (LCS) for LCA is a technically challenging operation mainly due to the risk of intraoperative hemorrhage. However, it can be a valuable alternative particularly for adolescent patients in whom maximum preservation of splenic function outweighs the risks associated with organ preservation. MethodsA 15-year-old young man was admitted with left upper abdominal dull pain for 1 month. Laboratory tests and enhanced abdominal CT scans were performed. Laboratory test results were all normal, as follows: complete blood count showed normal WBC: 5.74 × 109/l, platelets: 173 × 109/l, Hb: 151 g/l, tumor markers (CEA: 1.62 ng/ml, CA19–9: 10.30 U/ml, AFP: 1.13 ng/ml) which were within normal limits. CT scan showed that a 5 × 5.9-cm solid tumor was discovered in the middle part of the spleen during diagnosis, and the density of this tumor was high in the early artery phase and low in the late portal venous phase of contrast-enhanced CT. LCS was performed where the trunk of the splenic artery was intermittently occluded and several branches of the vessels were freed from the tumor which was then resected. The splenic parenchyma was dissected by following a line 0.5 cm from demarcation line. The tumor was completely removed with the activity of the splenic parenchyma being preserved to the maximum extent. Total operation time was 175 min, with an estimated blood loss of 200 ml; thus, no blood transfusion was required. Intraoperative frozen section suggested vascular tumor-like lesions, and then the tumor was completely removed with the resection margin being free of tumor involvement. ResultsThe patient was fully recovered after a 7-day hospital stay. The reason for the 7-day hospital stay is that the patient maintains normal ambulating independent assistance on POD-3 and can eat without intravenous fluids on POD-4. Meanwhile, the patient needs to remove the drainage tube after the abdominal CT scan on POD-6, rather than postoperative complications. Diagnosis of LCA was confirmed using histology, and the symptoms disappearing after the operation was done. A total of 86·laparoscopic partial splenectomy (LPS) procedures were performed at the Department of Pancreatic Surgery, West China Hospital and Department of Hepatopancreatobiliary Minimal Invasive Surgery, Chengdu ShangJin NanFu Hospital from May 2011 to May 2020. Five patients among the total number of patients were diagnosed with LCA of which LPS was performed in four patients and LCS performed for one patient. The mean duration of surgery for LPS was 100 min (range of 80-120 min) while the mean estimated blood loss was 125 ml (range of 100-200 ml). On the other hand, the mean length of hospital stay was 7.5 days (range of 5-11 days) and the mean tumor diameter was 5.1 cm (range of 3.2-7 cm). All patients recovered without complications. In addition, there were no signs of immune insufficiency, thrombosis, and tumor recurrence during the follow-up which had a mean of 31.2 months (range from 10 to 53 months). ConclusionLCS is a technically feasible technique for a select group of patients with centrally located LCA. The technique helps to maximize preservation of splenic function and maintains the advantages of minimally invasive surgery.

Multi-Focal Splenic Tumour in a Belgian Patient and a Brief Review of the Literature on Littoral Cell Angioma.

Littoral cell tumour, although a very rare neoplasm, must be included in the differential diagnosis of splenic lesions observed by imaging.As imaging cannot differentiate between benign and malignant lesions, a definitive diagnosis is made only by histopathology and immunohistochemistry.Individuals diagnosed with littoral cell angioma must be carefully evaluated to exclude associated primary, secondary and synchronous malignancies as well as accompanying inflammatory/autoimmune disease.

Vascular neoplasms and non-neoplastic vascular lesions of the spleen.

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.

Littoral cell angioma of the spleen in a patient with cerebral palsy and sigmoid volvulus: a rare case report

Background: Littoral cell angioma (LCA) is a rare vascular tumour of the spleen with unique histologic and immunophenotypic features. LCA is generally diagnosed incidentally and has a benign clinical course. However, it has rarely been reported to behave as a malign entity. Case presentation: A 49-year-old woman with cerebral palsy admitted to the hospital suffering from abdominal pain and weight loss. Laboratory results revealed iron deficiency anaemia and mild thrombocytopenia. Computed tomography scan revealed splenomegaly with multiple hypoattenuating lesions. A diagnostic and therapeutic open splenectomy and sigmoid colectomy were performed to exclude possible lymphoproliferative disorders or malignant lesions. The histologic and immunohistochemical studies confirmed the diagnosis of LCA and sigmoid volvulus. The post-operative period was uneventful with a significant increase in haemoglobin and platelet levels. Unfortunately, the patient died due to sepsis 36 days after surgery. Conclusion: This is the first case of LCA and sigmoid volvulus. Although this association is likely to be entirely coincidental, clinicians should be aware of this rare clinicopathologic entity whilst evaluating the differential diagnosis of splenomegaly with solitary or multiple nodules.

Sclerosing angiomatoid nodular transformation of the spleen: an unusual cause of abdominal pain

Vascular neoplasms are the most common primary nonhematopoietic tumors of the spleen. They include hemangiomas, littoral cell angiomas, splenic hamartomas, lymphangiomas, hemangioendotheliomas, angiosarcomas, and Sclerosing Angiomatoid Nodular Transformation (SANT)1. SANT is a rare and benign lesion arising from the red pulp of the spleen 2.Martel et al first described the disease entity in 2004 in middle aged- adults with slight female preponderance , the etiology and pathogenesis are still not clear3, SANT poses diagnostic clinical and radiological challenges4, we present this case report with review of the literature.

Practical Applications in Immunohistochemistry: An Immunophenotypic Approach to the Spleen.

Even though immunohistochemistry is routinely used by pathologists, evaluation of immunohistochemistry in splenic lesions remains difficult for many. Classification of benign and splenic lesions often requires a combination of hematoxylin-eosin evaluation, immunophenotyping, and sometimes molecular testing. Immunohistochemical staining is essential in evaluating many splenic lesions, and requires an understanding of the normal compartments of the spleen. To address different immunohistochemical features used for identification and subclassification of different lesions of the spleen, as well as in the normal compartments of the spleen. The information outlined in this review article is based on our experiences with a variety of spleen cases, on the current World Health Organization classification of hematopoietic and lymphoid tumors, and on a review of English-language articles published during 2018. Features for phenotyping normal spleen as well as a variety of splenic lesions, including littoral cell angioma and splenic marginal zone lymphoma, are discussed. Suggested immunopanels are provided to assist in the diagnosis of different lesions of the spleen.