Littoral cell angioma of the spleen: A study of 10 cases case series and literature review.

BACKGROUND: Current study aimed to investigate the clinical characterization, differential diagnosis, and treatment of splenic littoral cell angioma (LCA). METHODS: A retrospective analysis was performed for 10 LCA cases admitted to Huzhou Central Hospital from 2007 to 2023, for clinical manifestations, hematological tests, imaging features, pathological features, treatment methods, and prognosis along with the relevant literature was also reviewed. RESULTS: During examinations, no specific clinical manifestations and hematological abnormalities were seen in all 10 cases of LCA. Imaging observations depicted single or even multiple spherical lesions in the spleen. Plains shown by computed tomography (CT) were found somewhat equal or slightly lower in density. On the other hand, magnetic resonance imaging (MRI) plain scans viz. T1 weighted image showed equal low and mixed signals while T2-weighted showed high and low mixed signals. Moreover, punctate low signals could be seen in high signals named "freckle sign" in MRI scans. On contrast-enhanced CT scans, the enhancement of the lesions was not obvious in the arterial phase, and some of the lesions showed edged ring-like enhancements and "filling lake" progressive enhancement during the venous phase and delayed phase. In multiple lesions, the number of enhanced scan lesions showed a variable changing pattern "less-more-less." MRI-enhanced scan showed the characteristics of "fast in and slow out." Microscopic examinations identified tumor tissue actually composed of sinus-like lacunae that anastomosed with each other in the form of a network. Furthermore, cystic expansion and pseudopapillary protrusions were also seen in the dilated sinus cavity which was lined with single-layer endothelial cells having conspicuous cytoplasmic hemosiderin. High immunophenotypic expressions of vascular endothelial cell phenotype (CD31, CD34, FVIII) and tissue cell phenotype (CD68) were also seen. Total and partial splenectomy were performed in 8 and 2 patients, respectively, and follow-up examinations showed survival in all patients with no recurrence. CONCLUSION: LCA is a rare splenic benign lesion with atypical clinical manifestations. CT and MRI imaging are important tools in preoperative diagnosis based on pathomorphological and immunohistochemical examinations. Splenectomy is a superior therapeutic choice with significant impacts and prognosis.

Littoral cell angioma of the spleen: case report and literature review.

Littoral cell angioma is an extremely rare splenic vascular tumor originating from the cells lining the splenic red pulp sinuses. Approximately 150 cases of littoral cell angioma have been reported since 1991. Its clinical manifestation is usually asymptomatic and is mostly diagnosed as an incidental finding through abdominal imaging. Herein, we present a case of littoral cell angioma in a 41-year-old woman with no previous comorbidities, which initially presented as a nonspecific splenic lesion diagnosed on imaging in the emergency room. The patient was treated through laparoscopic intervention.

Clinicopathological analysis of sclerosing angiomatoid nodular transformation in the spleen.

Background: Splenic sclerosing angiomatoid nodular transformation (SANT) is a rare benign nodular lesion in the red medulla of the spleen. In the past, SANT has not been consistently recognized as the name for this condition and was often misdiagnosed for other conditions. In recent years, SANT has been acknowledged by most scholars as multiple reports have been published. Aim: To assess the clinicopathological features of SANT to identify the histological characteristics of SANT to improve diagnosis and clinical treatment. Materials and Methods: We assessed 25 cases of SANT diagnosed at Zhongshan Hospital affiliated with Fudan University from September 2014 to October 2021, including 14 men and 11 women, aged 24-62 years old. Results: Fourteen cases were complicated with benign tumors of the liver, pancreas, kidney, uterus, and prostate. One case was complicated with renal clear cell carcinoma, and one was complicated with hepatocellular carcinoma. The gross neoplasm is multinodular and well defined. Histologically, angiomatoid nodules are composed of fattened, round, or irregular blood vessels, with or without red blood cells in the lumen, with unequal red blood cell extravasation, and fibrocytes around the nodules. The hemangiomatous nodules were positive for CD31 and CD34, while the vascular wall smooth muscle cells and fibrocytes around the nodules were positive for SMA. Conclusion: The diagnosis of SANT requires a combination of immunohistochemical and histological features, and early splenectomy is crucial for treatment.

A case of multifocal sclerosis angiomatoid nodular transformations of the spleen occurring after partial splenic infarction with transcatheter arterial embolization for splenic artery aneurysm.

A 48-year-old woman underwent transcatheter arterial embolization (TAE) for a splenic artery aneurysm, which resulted in a partial splenic infarction in the middle lobe. Five years after TAE, a 20-mm diameter mass in the noninfarcted area of the spleen was detected on imaging, which grew to 25 mm in diameter after 6 months. MRI after gadolinium administration showed a 35 × 34 mm mass within the superior pole and 15 × 12 mm mass within the inferior pole. The patient underwent laparoscopic splenectomy and had an uneventful postoperative recovery. No evidence of recurrence was observed during the 2-year follow-up period after surgery. The mass was pathologically confirmed to be sclerosing angiomatoid nodular transformation (SANT) of the spleen. While some studies hypothesize that SANT is a response to vascular injury or trauma, to the best of our knowledge, there have been no previous reports of SANT occurring after procedures directly affecting splenic blood flow. Additionally, multifocal SANTs are reported to be very rare, accounting for only 4.7% of all reported SANTs of the spleen. We highlight a rare course of SANT of the spleen and discuss the possible relationship between blood flow abnormalities and the appearance of SANT.

Laparoscopic partial splenectomy in a patient with splenic hemangioma after distal gastrectomy for a neuroendocrine tumor.

Herein, we report the case of a patient with splenic hemangioma after distal gastrectomy who was treated with laparoscopic partial splenectomy. A 64-year-old woman previously underwent laparoscopic distal gastrectomy with regional lymph-node dissection for a gastric neuroendocrine tumor (G3) with venous infiltration and no lymph-node metastases. Periodic follow-up abdominal computed tomography revealed a well-defined, heterogeneous mass in the lower pole of the spleen 5 years after the operation, which grew from 12 to 19 mm 1 year later. A laparoscopic partial splenectomy was planned. During surgery, a smooth-surfaced mass with a lighter color than that of the surrounding area was observed at the lower pole of the spleen. The inferior polar branch of the splenic artery was transected, and the ischemic area of the lower pole of the spleen, where the tumor was present, was confirmed. First, the line used to perform splenic transection was determined using soft coagulation. The splenic parenchyma was then gradually transected using a vessel-sealing device system, and partial splenectomy was possible with almost no bleeding. The patient was discharged on postoperative day 8 without any complications. Pathological examination revealed a hemangioma without any malignant findings. Laparoscopic partial splenectomy is a safe and useful procedure that can be performed, considering the tumor size and location.

Robot-assisted resection of benign splenic tumors in children.

PURPOSE: Robotic surgery is becoming increasingly widely used in the field of pediatric surgery. The present study aimed to evaluate the safety and feasibility of robot-assisted resection of benign pediatric splenic tumors and to discuss the technical points. METHODS: A total of 32 patients who were diagnosed with benign splenic tumors and underwent minimally invasive surgery from January 2017 to September 2023 were included in the study. The clinical data including demographic criteria, operative details, and postoperative outcomes were analyzed retrospectively. RESULTS: Thirteen patients underwent robot-assisted surgery, and 19 patients underwent laparoscopic surgery. The median operation time was 150 min, with an interquartile range (IQR) of 120 to 200 min for the robot-assisted group and 140 min with an IQR of 105 to 180 min in the laparoscopic group (P = 0.318). Despite four cases in the laparoscopic group (21%) being converted to laparotomy because of intraoperative bleeding, compared with none in the robot-assisted group, there was no significant difference between two groups (P = 0.128). The intraoperative volume of blood loss was significantly less (P = 0.041), and the hospitalization expense was significantly higher (P = 0.000) in the robot-assisted group than for the laparoscopic group. There was no significant difference in patients' age, tumor size, postoperative feeding time, and the postoperative hospitalization time between two groups (P > 0.05). CONCLUSION: Robot-assisted benign splenic tumor resection was safe and feasible, and it reduced surgical trauma for the pediatric patient.

[Surgery for primary splenic tumors and metastases of the spleen]. / Chirurgie bei primären Milztumoren und Metastasen der Milz.

The correct indications for surgical treatment of primary splenic tumors as well as metastases of the spleen are challenging due to the rarity of the various entities. Primary solid splenic tumors include benign lesions, such as hemangiomas, hamartomas and sclerosing angiomatous nodular transformation (SANT) of the spleen. In these cases, surgical treatment is indicated only in the case of inconclusive imaging and after careful consideration of the risk-benefit ratio, even in the case of pronounced symptoms. In contrast, primary angiosarcoma or undifferentiated pleomorphic sarcoma as highly malignant tumors represent an urgent indication for surgery. Although more frequent than primary splenic malignancies, secondary splenic tumors are also not that frequent. Solitary splenic metastases are rare; however, from an oncological point of view they can be treated by resection. In the case of oligometastasis with splenic involvement, splenectomy is used only as part of a palliative concept in cases of pronounced symptoms or in the context of cytoreductive surgery. In general, the laparoscopic approach is to be preferred when the operation is technically feasible as it is associated with fewer pulmonary and infectious complications and a shorter hospital stay. In addition, to reduce the risk of severe infections after splenectomy, the option of partial splenectomy should be considered, especially for benign lesions. A thorough informing of the patient regarding both intraoperative and perioperative risks as well as potential long-term sequelae, especially severe infectious diseases, is an essential component of informed consent before surgery.

Time Trend Analysis of Splenectomy for Splenic Marginal Zone Lymphoma: Declining Surgery, Promising Survival.

BACKGROUND: The purpose of current study was to examine the incidence, characteristics, treatment, and survival of splenic marginal zone lymphoma (SMZL). METHODS: Using SEER-18 database, patients diagnosed with SMZL between 2000 and 2018 were included. Effect of splenectomy on survival was evaluated after balancing the confounding factors by propensity score matching. Rates of splenectomy and 1-year relative survival were calculated for each year. A logistic regression model identified factors related to splenectomy, and a Cox regression model assessed factors linked to overall survival (OS). RESULTS: A total of 2790 patients with SMZL were analyzed. The majority were older than 60 years, female, and white. The age-adjusted incidence of SMZL was 0.17/100,000 person-years, with higher incidence in males. Incidence increased by 0.68%/year and peaked at 80-84 years for both genders. The SMZL-specific survival rates at 3 and 5 years were 89.6% and 85.3%, respectively. Meanwhile, the relative survival rates for the same periods were 88.6% and 85.9%, respectively. Splenectomy patients were more likely to be younger, male, and diagnosed with early-stage disease. Despite the decreasing utilization rate of splenectomy from 59.4% in 2000 to 16.2% in 2018, the 1-year relative survival rate remained relatively stable with minor fluctuations over time. Whether or not the patient underwent splenectomy was not found to be a significant prognostic indicator for OS. CONCLUSIONS: Our study demonstrated a decreasing use of splenectomy but a relatively stable survival in patients with SMZL, highlighting the urgency to better understand the role of splenectomy and its associated outcomes.

Littoral cell angioma of the spleen associated with two primary cancers.

Littoral cell angioma is a benign vascular tumour of the spleen, and malign transformation is seldom. The angioma is associated with a high risk of simultaneous occurrence of other primary cancers, and it is of utmost importance to perform extensive diagnostic investigations to detect other cancers. Definitive treatment of littoral cell angioma is surgical resection of the spleen. This is a unique case report about a 73-year-old woman who had a simultaneous adenocarcinoma of the colon and a gastrointestinal stromal tumour. She underwent simultaneous splenectomy with colonic and gastric resection.

Surgical management of splenic flexure cancer: is there an optimal technique? A bi-national registry analysis.

BACKGROUND: Splenic flexure tumours (SFC) are uncommon and present at more advanced disease stages. The optimal surgical technique for SFC remains controversial. We sought to compare the short-term outcomes of a left hemicolectomy (LHC) versus an extended resection (subtotal colectomy, STC) for SFCs. METHODS: A retrospective analysis using the Binational Colorectal Cancer Audit (BCCA) registry was performed. All patients with SFC who underwent elective or emergency surgery for a SFC between 2010 and 2021 were included. Primary outcomes included short-term inpatient complications. Secondary outcomes included survival outcomes. RESULTS: Six hundred and ninety-nine patients underwent resections for SFCs. A LHC was more common, performed in 64.1%. Patients having a LHC were significantly older, with proportionally more LHCs done laparoscopically. Overall grade III/IV complications were similar between both operations. Prolonged ileus and return to theatre were significantly higher in patients undergoing a STC. On multivariate analysis, anastomotic leak and overall grade III/IV complications were not independently associated with the type of operation. There was no difference in medial survival based on type of operation. Higher tumour stage (Stage III/IV) were independently associated with worse survival. CONCLUSION: Segmental and extended resections are both oncologically sound procedures for SFCs. Segmental resections are associated with lower rates of prolonged ileus.

The role of splenectomy in management of splenic B-cell lymphomas.

INTRODUCTION: Splenic B-cell lymphomas are rare and understudied entities. Splenectomy is frequently required for specific pathological diagnosis in patients with splenic B-cell lymphomas other than classical hairy cell leukemia (cHCL), and can be effective and durable therapy. Our study investigated the diagnostic and therapeutic role of splenectomy for non-cHCL indolent splenic B-cell lymphomas. METHODS: Observational study of patients with non-cHCL splenic B-cell lymphoma undergoing splenectomy between 1 August 2011 and 1 August 2021 at the University of Rochester Medical Center. The comparison cohort was patients categorized as having non-cHCL splenic B-cell lymphoma who did not undergo splenectomy. RESULTS: Forty-nine patients (median age 68 years) had splenectomy (SMZL n = 33, HCLv n = 9, SDRPL n = 7) with median follow up of 3.9 years post splenectomy. One patient had fatal post-operative complications. Post-operative hospitalization was ≤ 4 days for 61% and ≤ 10 days for 94% of patients. Splenectomy was initial therapy for 30 patients. Of the 19 patients who had previous medical therapy, splenectomy changed their lymphoma diagnosis in 5 (26%). Twenty-one patients without splenectomy were clinically categorized as having non-cHCL splenic B-cell lymphoma. Nine required medical treatment for progressive lymphoma and of these 3 (33%) required re-treatment for lymphoma progression compared to 16% of patients following first line splenectomy. CONCLUSION: Splenectomy is useful for the diagnosis of non-cHCL splenic B-cell lymphomas with comparable risk/benefit profile and remission duration to medical therapy. Patients with suspected non-cHCL splenic lymphomas should be considered for referral to a high-volume center with experience in performing splenectomies for definitive diagnosis and treatment.

Splenic flexure cancer survival: a 25-year experience and implications for complete mesocolic excision (CME) and central vascular ligation (CVL).

BACKGROUND: The management of splenic flexure cancers (SFCs) in the era of complete mesocolic excision (CME) and central vascular ligation (CVL) is challenging because of its variable lymphatic drainage. This study aimed to compare survival outcomes for SFCs and non-SFCs, and better understand the clinicopathological characteristics which may define a distinct SFC phenotype. METHODS: An observational cohort study at Concord Hospital, Sydney was conducted with patients who underwent resection for colon adenocarcinoma (1995-2019). Clinicopathological data were extracted from a prospective database. Overall survival (OS) and disease-free survival (DFS) estimates and their associations to clinicopathological variables were investigated with Kaplan-Meier and Cox regression analyses. RESULTS: Of 2149 patients with colon cancer, 129 (6%) had an SFC. The overall 5-year OS and DFS rates were 63.6% (95% CI 62.5-64.7) and 59.4% (95% CI 58.3-60.5), respectively. SFCs were not associated with OS (P = 0.6) or DFS (P = 0.5). SFCs were more likely to present urgently (P < 0.001) with obstruction (P < 0.001) or perforation (P = 0.03), and more likely to require an open operation (P < 0.001). These characteristics were associated with poorer survival outcomes. No differences were noted between SFCs and non-SFCs with respect to tumour stage (P = 0.3). CONCLUSION: SFCs have a distinct phenotype, the individual characteristics of which are associated with poorer survival. However, the survivals of SFCs and non-SFCs are similar, possibly because the most important determinant of outcome, tumour stage, is no different between the groups. This may have implications for the surgical approach to SFCs with respect to standardization of CME and CVL surgery for these cancers.

SAGES masters program: the top 10 seminal articles for the laparoscopic left and sigmoid colectomy pathway for complex disease.

BACKGROUND: The SAGES University Colorectal Masters Program is a structured educational curriculum that is designed to aid practicing surgeons develop and maintain knowledge and technical skills for laparoscopic colorectal surgery. The Colorectal Pathway is based on three anchoring procedures (laparoscopic right colectomy, laparoscopic left and sigmoid colectomy for uncomplicated and complex disease, and intracorporeal anastomosis for minimally invasive right colectomy) corresponding to three levels of performance (competency, proficiency and mastery). This manuscript presents focused summaries of the top 10 seminal articles selected for laparoscopic left and sigmoid colectomy for complex benign and malignant disease. METHODS: A systematic literature search of Web of Science for the most cited articles on the topic of laparoscopic complex left/sigmoid colectomy yielded 30 citations. These articles were reviewed and ranked by the SAGES Colorectal Task Force and invited subject experts according to their citation index. The top 10 ranked articles were then reviewed and summarized, with emphasis on relevance and impact in the field, study findings, strength and limitations and conclusions. RESULTS: The top 10 seminal articles selected for the laparoscopic left/sigmoid colectomy for complex disease anchoring procedure include advanced procedures such as minimally invasive splenic flexure mobilization techniques, laparoscopic surgery for complicated and/or diverticulitis, splenic flexure tumors, complete mesocolic excision, and other techniques (e.g., Deloyers or colonic transposition in cases with limited colonic reach after extended left-sided resection). CONCLUSIONS: The SAGES Colorectal Masters Program top 10 seminal articles selected for laparoscopic left and sigmoid colectomy for complex benign and malignant disease anchoring procedure are presented. These procedures were the most essential in the armamentarium of practicing surgeons that perform minimally invasive surgery for complex left and sigmoid colon pathology.

Non-Small Cell Lung Cancer Causing Atraumatic Splenic Rupture Without Splenic Metastasis.

Atraumatic splenic rupture (ASR) is a rare occurrence but an important clinical entity. Although trauma is the most common cause of splenic rupture, there is limited literature on ASR. This case report discusses a 59-year-old woman presenting with tension hydrothorax and ASR in the setting of non-small cell lung carcinoma requiring emergent chest tube insertion and emergent splenectomy. Her hospital course was complicated by pulmonary embolism and thrombosis of the inferior vena cava. The patient expired three months after her initial presentation. This patient's presentation represents only the second documented case of atraumatic splenic rupture secondary to metastatic lung carcinoma without pathological evidence of splenic metastasis. Atraumatic splenic rupture secondary to metastatic NSCLC is a rare occurrence; though failure to detect, it may be fatal. Pathologic ASR may be an occult presentation of lung malignancy and in the presence of confirmed NSCLC may portend a poor prognosis.

Littoral cell angioma discovered after splenectomy in a patient with non-Hodgkin lymphoma and melanoma. Case report and review.

BACKGROUND: Littoral cell angioma (LCA) is a rare vascular primary tumor of the spleen with no more than 440 cases described so far. Although often seen as benign, it is described to have malignant potential and to be associated with other immunologic disorders or malignancies. CASE PRESENTATION: We present the case of LCA in a 75-year old man with a concomitant non-Hodgkin lymphoma and medical history of malign melanoma. The tumor was discovered incidentally after splenectomy for splenomegaly and refractory thrombocytopenia. The post-operative period was uneventful. CONCLUSION: Our case is the first to report an association of LCA with both lymphoma and melanoma thus far. It emphasizes the need for a thorough total body examination for synchronous diseases and close follow-up to reveal associated malignancies or immunologic disorders. Further research is required to identify etiologic and pathogenetic mechanisms behind this tumor and a common basis between the three diseases. KEY WORDS: Littoral Cell Angioma, Neoplasm, Splenectomy, Solid Spleen Tumor.

Splenic surprise in a case of renal cell carcinoma: Unusual case or association?

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.

[Isolated splenic lymphangioma]. / Laparoskopicheskaya rezektsiya pri izolirovannoi limfangiome selezenki.

Splenic lymphangioma is a rare malformation of splenic lymphatic channels characterized by cysts resulting from increased number of enlarged thin-walled lymphatic vessels. In our case, there were no clinical manifestations. Lymphangioma was congenital and diagnosed by ultrasound as an accidental finding. Surgery is the only method of radical treatment of splenic lymphangioma. We describe an extremely rare case of pediatric isolated splenic lymphangioma and laparoscopic resection of spleen as the most advantageous variant of surgical treatment.