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BackgroundLittoral cell angioma (LCA) is an extremely uncommon benign vascular tumor of the spleen. Cases of LCA in infants are rarely reported, and due to the rarity of the tumor and non-specific symptoms, the diagnosis of LCA is often overlooked in clinical practice.Case reportWe present a 3-year-old girl with pulmonary inflammation who was admitted to the hospital due to the discovery of a space-occupying lesion in the spleen. Pathology after splenectomy confirmed LCA, and there was no recurrence observed at the 5-month follow-up examination.ConclusionLCA should be considered when a child shows asymptomatic splenomegaly, with antigen expression indicating dual positivity of endothelial and histiocytic markers. Laparoscopic splenectomy remains the primary method of treating LCA.

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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Littoral cell angiomas are uncommon primary splenic haemangiomas with rare malignant potential. We report a case of a 76-year-old male with an incidental solitary littoral cell angioma found within an accessory spleen. We provide an overview of the literature of littoral cell angiomas and highlight the diagnostic challenge and treatment of this important differential for general surgeons caring for patients with splenic masses. This is the first case to describe primary resection of a littoral cell angioma with splenic preservation. [ABSTRACT FROM AUTHOR]

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.

Littoral cell angioma (LCA) is a rare benign tumor originating exclusively from the venous sinus lining cells of the splenic red pulp. These cells are unique in having a distinctive hybrid endothelial/histiocytic phenotype. Also, there are reports of the association of LCA with internal malignancies. We present a case report highlighting an unusual association of LCA with conventional renal cell carcinoma (RCC), masquerading as a metastatic lesion. Knowledge of such an association is necessary to avoid misdiagnosis and prevent potential overtreatment. [ABSTRACT FROM AUTHOR]

Objective: Littoral cell angioma (LCA) is currently considered to be a rare splenic tumor with malignant potential. As the epidemiology, pathogenesis, clinical manifestation, treatment, and prognosis remain unclear, the clinical diagnosis and treatment of LCA have not been standardized. Hence, we performed a comprehensive analysis of 189 observational studies comprising 435 patients to improve the current status of diagnosis and treatment. Methods: PubMed, Embase, WanFang and CNKI were searched from inception to May 2021 to identify LCA studies that were published in English and Chinese. The clinical information of LCA patients were extracted and analyzed. Results: The LCA has a male-to-female ratio of 0.90 and a solitary-to-multiple ratio of 0.31. In terms of clinical features, 69.7% of the patients showed splenomegaly, 49.7% were asymptomatic, and 39.2% experienced epigastric discomfort. As the imaging findings of patients with LCA were nonspecific, an image-guided biopsy (10/12) was a safe and effective method for diagnosing in this condition. Notably, results of the prognostic analysis indicated that LCA has a lower risk of recurrence and metastasis. The patient may develop a stable disease or the tumor will grow but will not metastasize. Besides, the novel immunohistochemical pattern of LCA was described as CD31+/ERG+/FVIII Antigen+/CD68+/CD163+/lysozyme+/CD8−/WT1−. Conclusion: LCA should be reconsidered as a benign primary splenic vascular neoplasm, which is more like an intra-splenic manifestation of abnormal body function. Image-guided biopsy with follow-up might be a beneficial choice for LCA patients. For LCA patients with abdominal discomfort, pathological uncertainty or continuous tumor enlargement, splenectomy remains the preferred treatment. [ABSTRACT FROM AUTHOR]

ObjectiveLittoral cell angioma (LCA) is currently considered to be a rare splenic tumor with malignant potential. As the epidemiology, pathogenesis, clinical manifestation, treatment, and prognosis remain unclear, the clinical diagnosis and treatment of LCA have not been standardized. Hence, we performed a comprehensive analysis of 189 observational studies comprising 435 patients to improve the current status of diagnosis and treatment.MethodsPubMed, Embase, WanFang and CNKI were searched from inception to May 2021 to identify LCA studies that were published in English and Chinese. The clinical information of LCA patients were extracted and analyzed.ResultsThe LCA has a male-to-female ratio of 0.90 and a solitary-to-multiple ratio of 0.31. In terms of clinical features, 69.7% of the patients showed splenomegaly, 49.7% were asymptomatic, and 39.2% experienced epigastric discomfort. As the imaging findings of patients with LCA were nonspecific, an image-guided biopsy (10/12) was a safe and effective method for diagnosing in this condition. Notably, results of the prognostic analysis indicated that LCA has a lower risk of recurrence and metastasis. The patient may develop a stable disease or the tumor will grow but will not metastasize. Besides, the novel immunohistochemical pattern of LCA was described as CD31+/ERG+/FVIII Antigen+/CD68+/CD163+/lysozyme+/CD8−/WT1−.ConclusionLCA should be reconsidered as a benign primary splenic vascular neoplasm, which is more like an intra-splenic manifestation of abnormal body function. Image-guided biopsy with follow-up might be a beneficial choice for LCA patients. For LCA patients with abdominal discomfort, pathological uncertainty or continuous tumor enlargement, splenectomy remains the preferred treatment.

Littoral cell angioma (LCA) is a rare vascular tumor of the spleen that often requires histopathological analysis for diagnosis due to non-specific imaging features. The current approach is either splenectomy or image-guided percutaneous biopsy which carries notable procedure-associated morbidity and limited accuracy. We present a novel case of LCA successfully diagnosed with endoscopic ultrasound fine-needle aspiration biopsy (EUS-FNAB), demonstrating its potential to reduce the morbidity associated with traditional percutaneous biopsy methods. This case highlights EUS-FNAB's advantage in minimizing complications and its effectiveness in diagnosing vascular tumors of the spleen, supporting its inclusion in the diagnostic algorithm for splenic lesions. Further cases are encouraged to explore EUS-FNAB's role in diagnosing rare vascular tumors such as LCA to establish its efficacy and safety profile., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Aleem et al.)

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 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.

Littoral cell angioma (LCA), a rare vascular neoplasm that occurs in the spleen, is difficult to definitively diagnose preoperatively because histological examination is the only accurate means of diagnosing this condition. Thus, the preoperative diagnosis is often incorrect. Splenectomy is the appropriate treatment and can be performed laparoscopically or by open surgery depending on the size of the tumor. Here we present a case of a 38-year-old man who presented with a history of slight non-specific abdominal distension for 2 months. An abdominal CT scan showed a huge spleen measuring 35x18 cm and containing multiple lesions, the largest being 11.3x9.2 cm. The patient underwent open splenectomy and recovered well. Histological examination showed LCA of the spleen. To our knowledge, this is the largest reported LCA thus far and may advance our understanding of this condition. [ABSTRACT FROM AUTHOR]

Introduction Littoral- cell angioma (LCA) is a rare benign vascular tumour of the spleen. There have been less then 80 cases reported in the literature. Recent reports have described it to be a malignant lesion with congenital and immunologic associations. We report a case of LCA of the spleen. Presentation of case A 52 -year-old male patient was admitted to hospital with a three month duration of intermittent upper abdominal pain and nausea. Imaging studies, including computer tomography (CT) and magnetic resonance imaging (MRI), showed multiple lesions in the spleen as well as in the accessory spleens. An open splenectomy was performed and his post-operative recovery was uneventful. Discussion Littoral cell angioma of the spleen is a benign vascular tumour that has been infrequently reported in the English literature. While it does have malignant potential, the vast majority are benign. Diagnosis depends on the expression of endothelial markers like CD31 and histiocytic markers like CD68.Malignant potential is enhanced by the presence of splenomegaly as well. Conclusion This rare condition is made even more rare by the presence of the tumour in the two accessory spleens as well. [ABSTRACT FROM AUTHOR]

Littoral cell angioma is a rare primary, vascular tumor thought to originate from the endothelial cells lining the sinuses of the splenic red pulp (the “littoral cells”). It is a benign, usually asymptomatic lesion diagnosed incidentally. Ultrasound and tomography appearance is not characteristic and histopathological examination is required. This work provides a case-study of littoral cell angioma which was seen in a 55-year-old female who complained of non-specific upper abdominal pain. Computed tomography revealed multiple hypo-attenuated splenic lesions suggestive for metastasis. A splenectomy was performed and routine microscopic examination supported by immunohistochemistry reactions with CD68, CD34 and CD31 showed littoral cell angioma.

Littoral cell angioma (LCA) is a rare, benign primary vascular neoplasm of the spleen. The tumor originates from the littoral cells lining the sinuses of the red pulp of the spleen. Preoperative distinction of this tumor from other benign or malign splenic lesions is difficult. Radiologically most cases present as multiple nodules. Definitive diagnosis can only be made histopathologically and immunohistochemically following splenectomy. This clinical situation can coexist with various malignancies and autoimmune disorders. Even though, it is mostly benign, since it has the potential to become malignant after splenectomy, long-term follow-up is required. We present an LCA case, which appeared as a solitary mass in the spleen of an 11-year-old girl with abdominal pain admitted to our hospital.

Littoral cell angioma is a rare primary splenic vascular tumor, originating from the littoral cells lining the red pulp sinuses of the spleen, with variable presentation. Here, we present the case of a 2-year-old male child presenting with chronic thrombocytopenia which resolved after splenectomy.

Introduction: Kasabach-Merrit syndrome (KMS) is characterized by the association of a vascular tumor, thrombocytopenia and potentially life-threatening coagulopathy. Observation: A 22-month-old child was referred to our center for incidental discovery of thrombocytopenia in a context of recurrent throat infections. Clinical examination showed petechial purpura, subicterus, no tumoral syndrome and good overall condition. His biological parameters showed regenerative hemolytic anemia (10.2 g/dL), thrombocytopenia (32 G/L), no schistocytes, moderate fibrinopenia and positive D-dimer test. The results of bone marrow analysis and autoimmune tests were normal. No treatment was undertaken given the absence of diagnosis and complication. The child was regularly followed-up in consultation. Complementary investigations ruled out Wilson's disease, thrombotic thrombocytopenic purpura and constitutional red blood cell disorders. The main hypothesis remained a thrombotic microangiopathy despite absence of schistocytes on repeated blood tests. Two months later, he was rehospitalized for acute clinical and biological worsening with splenomegaly and severe consumption coagulopathy leading to suspect a myeloproliferative syndrome, finally refuted. Steroids were started due to worsening hemorrhagic symptoms, daily transfusion requirements and increasing splenomegaly. Due to worsening health status and risk of threatening rupture, splenectomy was performed and histopathological analysis revealed a vascular lesion infiltrating the whole spleen, compatible with a littoral cell angioma. The child showed rapid clinical improvement and complete hematological normalization within 48 hours post-splenectomy. Conclusion: Littoral cell angioma is a very rare and benign vascular lesion which may be complicated by a lifethreatening KMS. This diagnosis should be suspected in patients presenting with splenomegaly and features of thrombotic microangiopathy. [ABSTRACT FROM AUTHOR]

Littoral cell angioma (LCA) is a unique lesion of the spleen that arises from the cells lining the venous sinuses of the splenic red pulp and shows the features of combined endothelial and histiocytic differentiation. Several cases of LCA have been reported in the literature; however, the cytological findings have been described for only a few cases. We report the case of an 11-year-old boy with anemia, epigastric abdominal pain, and splenomegaly. The splenic lesions showed anastomosing vascular channels with cyst-like spaces filled by many sloughed endothelial cells, which were positive for CD68 and CD31 and negative for CD34. Scraping cytology revealed isolated and clusters of three-dimensional bland looking, epithelioid foamy tumoral cells with low nuclear cytoplasmic ratio, which mostly contained intracytoplasmic hemosiderin pigment. Although the fine needle aspiration cytology of splenic lesions is uncommon and LCA is a rare splenic lesion, it must be noted in the differential diagnosis of any splenic vascular neoplasm. [ABSTRACT FROM AUTHOR]

The spleen is an enigmatic organ that for a long time was thought to be useless, it is anything but. Made up of two main functional units, the spleen plays an important role in the immune system (white pulp) and in blood filtration and removal of unwanted material (red pulp). Neoplasms of the spleen are either primary or secondary. Primary tumours can be lymphoid or non-lymphoid. This review is focused on the most common non-lymphoid tumours namely, the vascular neoplasm, which includes benign, malignant and intermediate grade tumours. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Littoral cell angioma (LCA) is a rare splenic vascular neoplasm that arises from the cells lining the red pulp sinuses. Many recent reports have described this as a malignant tumour with congenital and immunological associations. Majority of patients remain asymptomatic though few present with variable symptoms. We present the case of a 65-year-old male with multiple splenic space occupying lesions associated with recurrent fever and weight loss. Computed tomography (CT) scans revealed splenomegaly with multiple hypoattenuating lesions. Further investigative workup failed to give the definitive diagnosis. Patient underwent laparoscopic splenectomy with an intent of establishing diagnosis and potential cure. Postoperative histological and immunohistochemical studies confirmed the diagnosis of LCA. Only a few case reports have been published as inconsistent results. Based on our experience of this case, we reviewed the available literature about the clinical, radiological and pathological features of this uncommon disease. [ABSTRACT FROM AUTHOR]

The patient is a 54 year old, presenting with a 4 month history of worsening lower back pain, radiating to both lower limbs. CT Thorax Abdomen and Pelvis was carried out which showed a 6.2cm dense fluid density lesion in the spleen. The case was discussed at the multidisciplinary team meeting and open splenectomy was carried out. Histological diagnosis was consistent with an infarcted littoral cell angioma. [ABSTRACT FROM AUTHOR]

Littoral cell angioma (LCA) is a rare primary benign vascular neoplasm of the spleen. The case of a 52-year-old female with LCA is reported. The patient did not have any symptoms or a significant past medical history. Computed tomography (CT) and ultrasound (US) imaging studies showed multiple lesions in the spleen and gallbladder stones. The tumour was removed successfully by laparoscopic splenectomy and simultaneously cholecystectomy was conducted for gallbladder stones. The patient recovered uneventfully. The tumour lining cells were positive for CD31/CD68 markers, and negative for CD34. This is the first report of a LCA combined with gallbladder stones.

Splenic rupture secondary to blunt trauma is a common condition. Non-traumatic, also known as spontaneous or pathological splenic rupture is an uncommon, but potentially life-threatening condition. Spontaneous splenic rupture caused by a primary splenic tumor is rare. In this case study, we present a special, benign tumor causing splenic rupture. Our 78-year-old female patient was hospitalized due to left shoulder pain and chest discomfort. Her blood pressure was low, the laboratory tests showed anemia, and the chest CT scan involving also the upper abdomen raised the suspicion of a splenic rupture. During the emergency splenectomy, there was a large amount of blood in the abdominal cavity. Macroscopic pathological examination of the removed spleen showed multifocal cystic lesions that led to splenic rupture. Immunhistochemical analyses revealed a littoral cell angioma. Littoral cell angioma is a rare, benign vascular tumor of the spleen, which is thought to originate from the red pulp sinuses lined with littoral cells. The aim of our report is to describe an unusual cause of sudden splenic rupture without traumatic history, the histologically benign littoral cell angioma that has not been published in Hungary. Orv Hetil. 2023; 164(10): 393-397.

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. [ABSTRACT FROM AUTHOR]

Littoral cell angioma is a rare primary vascular neoplasm of the spleen, composed of littoral cells that line the splenic sinuses of the red pulp. It was thought to be a benign, incidental lesion. However, many recent reports have described it to be a malignant lesion with congenital and immunological associations. The definitive diagnosis can only be made after histology and immunohistochemistry studies.

Littoral cell angioma (LCA) is a rare, benign primary vascular neoplasm of the spleen. The tumor originates from the littoral cells lining the sinuses of the red pulp of the spleen. Preoperative distinction of this tumor from other benign or malign splenic lesions is difficult. Radiologically most cases present as multiple nodules. Definitive diagnosis can only be made histopathologically and immunohistochemically following splenectomy. This clinical situation can coexist with various malignancies and autoimmune disorders. Even though, it is mostly benign, since it has the potential to become malignant after splenectomy, long-term follow-up is required. We present an LCA case, which appeared as a solitary mass in the spleen of an 11-year-old girl with abdominal pain admitted to our hospital. [ABSTRACT FROM AUTHOR]

The behavior of littoral cell neoplasms ranges from benign (littoral cell angioma, LCA) to highly malignant (angiosarcoma). Two unusual cases of low-grade metastatic littoral cell angiosarcoma (LCAS) have been reported with late recurrence and bulky metastases. We present the third case of this rare neoplasm in a 38-year-old man with cirrhosis and a large splenic artery aneurysm, without extrasplenic masses. The spleen showed nodules resembling LCA, immunoreactive for CD31, factor VIII, CD68, and CD163 but not CD8 or CD34. Also present were solid areas of immunophenotypically identical bland spindle cells, although lighter CD31 immunostaining distinguished them from LCA-like angiomatous channels. Similar cells diffusely infiltrated the cirrhotic liver. After splenectomy, pancytopenia resolved, and he is asymptomatic 19 months later. Low-grade LCAS is a previously unreported cause of cirrhosis and may metastasize without forming masses. In cases of LCA, CD31 immunohistochemistry may facilitate detection of LCAS and indicate metastatic potential. [ABSTRACT FROM PUBLISHER]