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

ABSTRACT 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.

CT features of inflammatory pseudotumor like follicular dendritic cell sarcoma of the spleen / 中华肝胆外科杂志

Objective:To explore the CT features of inflammatory pseudotumor like follicular dendritic cell sarcoma (FDCS) of the spleen.Methods:The clinical data of 12 patients with splenic inflammatory pseudotumor like FDCS admitted to 3 central hospitals including Yongjia People's Hospital in Zhejiang Province from January 2015 to December 2022 were retrospectively analyzed, including 4 males and 8 females, with a median age of 60 years old. The number, shape, size and CT features of the lesions were analyzed based on patient's CT image data.Results:CT scans of 12 patients showed 15 lesions, including 10 single lesions and 2 multiple lesions. The lesions were circular in 5 cases, elliptical in 4 cases, and irregular in 3 cases. The median maximum diameter of the mass is 6.5 cm. On plain scan, all 12 tumors showed low density or slightly low density. The CT value is (41.3±7.2) HU; 8 cases had uneven density and 4 cases had uniform density. There were 8 cases with clear tumor boundaries and 4 cases with unclear boundaries. There were 8 cases with tumor necrosis and cystic transformation, and 5 cases showed patchy bleeding lesions in the center of the tumor. Enhancement: the arterial phase shows small patches or flocculent enhancement at the edges or parenchymal parts of the tumor, with CT value of (56.0±3.8) HU. Among them, there were 7 cases of mild enhancement, 4 cases of moderate enhancement, and 1 case of significant enhancement. During the portal phase, there was mild to moderate persistent small patchy uneven enhancement, with CT value of (62.0±4.3) HU. Among them, there were 8 cases of mild enhancement and 4 cases of moderate enhancement. The delayed phase showed a slow withdrawal of enhancement, with CT value of (45.0±8.2) HU. All 12 cases underwent complete resection and were diagnosed with FDCS through pathological examination.Conclusion:FDCS plain scan shows circular or elliptical uneven low-density masses, with small patches or flocculent light to moderate uneven enhancement in the arterial phase, continuous enhancement in the portal phase, and slow withdrawal in the delayed phase as the main characteristics.

Angioma de células litorales esplénico. Presentación de caso clínico / Littoral cell angioma of the spleen. Report on a clinical case / Angioma de células litoraneas do baço: apresentação de um caso clínico

El angioma de células litorales del bazo es una neoplasia vascular benigna poco frecuente, de presentación clínica, paraclínica e imagenológica inespecífica. Se realizó en nuestro paciente la esplenectomía, el tratamiento indicado y curativo, y se logró el diagnóstico a través de la histología e inmunohistoquímica de la pieza de anatomía patológica.

Littoral cell angioma of the spleen is rather an unusual benign vascular neoplasm with non-specific clinical, paraclinical and imaging presentation. Our patient underwent splenectomy, performed as the appropriate and remedial treatment. Diagnosis was made by means of histology and immunohistochemistry techniques of the pathologic anatomy of the surgical piece.

O angioma de células litoraneas do baço é uma neoplasia vascular benigna rara com apresentação clínica, laboratorial e de imagem inespecíficas. A esplenectomia foi realizada em nosso paciente, sendo esse o tratamento indicado e curativo, obtendo-se o diagnóstico através da histologia e imunohistoquímica da peça anatomopatológica.

Surgical treatment and prognosis of 51 cases of splenic marginal lymphoma / 中华普通外科杂志

Objective:To explore the clinical characteristics, treatment and prognosis of splenic marginal zone lymphoma.Methods:The clinical data of 51 patients with splenic marginal zone lymphoma who underwent surgical treatment at Department of Hepatobiliary in Peking University People's Hosipital from Dec 2009 to Oct 2022 were retrospectively analyzed.Results:The patients 's average age was 60.5±11.8 years old, and the ratio of male to female rate was about 1:1. As of the last follow-up date of Feb 28, 2023, 8 patients died of the primary lymphoma and 14 patients suffered from disease progression. The 5-year progression free survival rate was 68%, and the overall survival rate was 79%. Univariate analysis showed that age ≥65 years old, decreased preoperative albumin, increased bilirubin level, and increased lactate dehydrogenase bode ill for overall survival time, but none of them were independent risk factors; Age ≥65 years old and diffuse large B cell transformation were independent risk factors.Conclusions:Surgery combined with comprehensive treatment which contained rituximab is currently the main treatment method. Splenectomy remains an important treatment and diagnostic method for patients with obvious symptoms or persistent unrelieved blood count abnormalities and unexplained splenomegaly. Advanced age, elevated bilirubin and lactate dehydrogenase, decreased albumin, and diffuse large B cell transformation, it may indicate poor prognosis.

Linfangiomatosis esplénica en un paciente adulto: Reporte de caso

Los linfangiomas son malformaciones benignas del sistema linfático que afectan con mayor frecuencia a los niños y en raras ocasiones a adultos. Presentamos el caso de una mujer de 36 años, con cuadro clínico de 6 meses de dolor abdominal difuso sin irritación peritoneal, y asociado a adinamia. Como parte de los estudios, se solicitó una tomografía axial computarizada con contraste de abdomen, que reveló múltiples lesiones esplénicas hipodensas hipovasculares, con sospecha diagnóstica de lesiones linfoproliferativas. Por tal motivo se solicitó una esplenectomía diagnóstica, que finalmente mostró hallazgos macroscópicos, microscópicos y de inmunohistoquímica (positividad de las lesiones quísticas para CD31 y D2-40) compatibles con linfagiomatosis esplénica difusa.

Lymphangiomas are benign malformations of the lymphatic system. They most often affect children and rarely adults. We present the case of a 36-year-old woman with a 6-month clinical picture of diffuse abdominal pain without peritoneal irritation associated with adynamia. As part of the studies, a computerized axial tomography with abdominal contrast was requested, which revealed multiple hypovascular hypodense splenic lesions with suspected diagnosis of lymphoproliferative lesions. For this reason, a diagnostic splenectomy was requested, which showed macroscopic, microscopic and immunohistochemical findings (positivity of cystic lesions for CD31 and D2-40) compatible with diffuse splenic lymphagiomatosis

Angioma de células litorales del bazo: un diagnóstico de lesión focal esplénica: caso clínico / Littoral cell angioma of the spleen: report of one case

Splenic vascular neoplasms are the most common form of spleen tumors. Among them, littoral cell angioma is rare and it is frequently an incidental finding in imaging studies. It has no specific clinical, laboratory or imaging findings. Splenectomy allows definitive diagnosis throughout a histopathological examination. We report a 52-year-old man presenting with asthenia and abdominal distension. Computed tomography with intravenous contrast showed multiple splenic hypodense masses and a prostatic enlargement. Presuming a lymphoma, a laparoscopic splenectomy was performed. Histopathologic examination diagnosed littoral cell angioma. During urological follow-up, a prostate adenocarcinoma was diagnosed.

Diagnosis of splenic marginal zone lymphoma / 白血病·淋巴瘤

Objective:To explore the clinical features, diagnosis and differential diagnosis of splenic marginal zone lymphoma.Methods:The clinical diagnosis and differential diagnosis processes of 3 cases of CD5 - CD10 - B cell non-Hodgkin lymphoma with splenomegaly and cytopenia who were admitted to Tongji Hospital of Tongji Medical College of Huazhong University of Science and Technology in 2019 were retrospectively analyzed, and the relevant literature was reviewed. Results:The 3 cases were all elderly patients with varying degrees of splenomegaly and cytopenia. CD5 - CD10 - monoclonal B lymphocytes were found in the bone marrow or lymph nodes. Based on the patient's clinical characteristics, peripheral blood and bone marrow morphology, immunophenotype and genetic characteristics, 2 patients were diagnosed as splenic marginal zone lymphoma, and 1 patient was diagnosed as diffuse large B-cell lymphoma. Conclusions:The diagnosis of splenic marginal zone lymphoma requires comprehensive analysis of clinical characteristics, peripheral blood and bone marrow morphology, immunophenotype and genetic characteristics. Careful differentiation from other CD5 - CD10 - small B-cell lymphomas is also needed. The next-generation gene mutation high-throughput sequencing and mutational spectrum analysis will help the accurate diagnosis of atypical and difficult cases.

Hemangioma esplénico / Splenic Hemangioma

RESUMEN Los tumores primitivos del bazo son relativamente raros, y entre ellos, los hemangiomas constituyen la neoplasia benigna más común en los adultos. Se presenta un paciente de 22 años de edad, piel blanca, con antecedentes de hipertensión arterial. El paciente refirió molestia en el hipocondrio izquierdo y en el ultrasonido abdominal se detectó esplenomegalia y litiasis renal. Ante el planteamiento de hiperesplenismo se indicó esplenectomía diagnóstica y terapéutica. La principal y más importante complicación que puede originar esta afección es la rotura espontánea del bazo en la cuarta parte de los casos informados, habitualmente en los de gran tamaño. El tratamiento de elección es la esplenectomía, principalmente para evitar el riesgo de rotura esplénica.

ABSTRACT Primitive tumours of the spleen are relatively uncommon, and among them, hemangiomas are the most common benign neoplasm in adults. We present a 22-year-old white-skinned patient with a history of high blood pressure. The patient reported discomfort in the left hypochondrium and abdominal ultrasound detected splenomegaly and renal lithiasis. Diagnostic and therapeutic splenectomy was indicated given the medical approach of hypersplenism. The main and most important complication that can originate this condition is the spontaneous rupture of the spleen in a quarter of the reported cases, usually in the large ones. The treatment of choice is splenectomy, mainly to avoid the risk of splenic rupture.

CT findings and differential diagnosis of benign tumors of spleen / 中国医学影像技术

Objective: To explore CT manifestations of 4 kinds of benign tumors of spleen and the key points of differential diagnosis. Methods CT findings of 4 kinds of benign tumors of spleen were analyzed retrospectively, including 18 cases of hemangioma, 3 cases of lymphangioma, 19 cases of hemolymphangioma and 1 case of harmatoma. CT characteristics of the above 4 kinds of benign tumors of spleen were observed. Results All hemangiomas were solid lesions, including single lesion in 11 cases, multiple lesions in 7 cases of spleen; 16 cases were with typical enhancement pattern. Lymphangioma was cystic lesion without enhancement. Hemolymphangioma could be solid, cystic, solid-cystic or mixed lesions. Most of the hemolymphangiomas were multiple (n=15) and solid (n=9) lesions, mild enhancement in the solid component and without enhancement in the cystic component. The appearance of harmatoma was a hypodense tumor with enhancement pattern similar to hemangioma. Conclusion All 4 kinds of benign tumors of spleen have certain CT characteristics, which are helpful to the diagnosis and differential diagnosis.

Radiological features of follicular dendritic cell tumor of spleen / 中华放射学杂志

Objective To summarize the radiological features of follicular dendritic cell tumor of spleen (FDCS).Methods The clinical, radiological and pathological data of 8 patients from November 2011 to November 2017 in 5 hospitals with FDCS confirmed by pathology were retrospectively analyzed. All patients underwent CT examinations including plan and enhanced CT. Three patients underwent additional MRI and two patients underwent PET‐CT examinations simultaneously. The imaging features such as location, number, shape, boundary, size, internal structure, density (or signal, 18F‐fluorodeoxyglucose uptake), enhancement model and the relationship with surrounding structures were observed and compared with pathological results. Results Of the 8 patients with FDCS, 7 were located in the spleen and 1 was located in the spleen of the ectopic spleen of the pancreas. Seven patients with splenic FDCS underwent splenectomy and 1 patient with pancreatic ectopic spleen FDCS underwent resection of the pancreas. Multiple lesions were detected in 1 case, while single in the others. Tumor was round or oval. The tumors were well‐circumscribed and presented as expansive growth. On unenhanced CT, the tumors showed a slightly lower density, and hemorrhage and necrosis could be detected in 6 lesions. Calcification was seen in 1 case, significant necrosis, and cystic change was presented in the pancreatic ectopic spleen FDCS. The solid part presented isointensity or slightly hypointensity on T1WI, and hyperointensity on T2WI. Cystic necrosis areas were hypointensitive on T1WI, and hyperointensitive on T2WI. Spoke‐like areas with hypointensity on T1WI and hyperointensity on T2WI were detected in the center of the solid part with the distribution among the substantial degenerative and necrotic regions. PET‐CT showed that the 18F‐fluorodeoxyglucose was uptaked obviously. The enhancement CT showed that at the arterial phase, the tumors were markedly enhanced and continuously enhanced at portal vein phase and balance phase. Multiple liver metastases were detected in 1 case with huge FDCS. One patient was followed up for 6 years, and gastric lymphoma was detected. The others were followed up for 6 to 53 months, there remained no transfer or recurrence.Conclusions The features of FDCS of spleen mainly manifest as solid or cystic mass with clear solitary sphenoma accompanied by scarring, calcification and hemorrhage. The enhancement mode is persistent enhancement. MRI and PET‐CT help to further reflect the tumor pathological basis and biological characteristics.

Hemangioendotelioma esplénico en paciente pediátrico. Presentación de caso / Splenic Hemangioendothelioma in Pediatric Patient. Case Report

Los hemangioendoteliomas esplénicos son neoplasias de origen vascular poco frecuentes, se presentan en pacientes entre 3 y 54 años. En edad pediátrica se han descrito pocos casos. Se caracteriza por presentar lesiones bien circunscritas, debidamente separadas del parénquima esplénico sano, moderada atipia celular, poca actividad mitótica y una apariencia histológica intermedia entre el hemangioma y el angiosarcoma.

Splenic hemangioendothelioma are rare vascular neoplasms that occur in patients between the ages of 3 and 54. Few pediatric cases have been described. It is characterized by well-circumscribed lesions, properly separated from the healthy splenic parenchyma, moderate cellular atypia, little mitotic activity, and an intermediate histological appearance between hemangioma and angiosarcoma.

Clinicopathologic features and prognosis of inflammatory pseudotumor-like follicular dendritic cell sarcomas in liver and spleen: an analysis of seven cases / 中华病理学杂志

Objective@#To investigate the clinicopathological features and prognostic parameters of the inflammatory pseudotumor-like follicular dendritic cell sarcoma (IPT-like FDCS) of liver and spleen.@*Methods@#Ninteen cases of inflammatory pseudotumor (IPT) and 5 cases of IPT-like FDCS of the liver and spleen were collected at the First Affiliated Hospital, Army Medical University from 2006 to 2016. HE sections, immunohistochemical staining, and Epstein-Barr virus encoded nuclear RNA (EBER) in situ hybridization were reviewed along with a summary of the literature.@*Results@#Among the previously diagnosed 19 cases of IPT of the liver and spleen, 2 cases were misdiagnosed (the ratio of 2/19). Among 7 new cases including 3 males and 4 females, 3 cases involved the liver and 4 cases involved the spleen. The age range was 37-64 years (mean 53 years). The maximum tumor diameter ranged from 3.0 to 11.0 cm (mean 6.5 cm). Surgical resections were performed in all patients with follow-up time ranging from 3 to 84 months.All patients were disease-free.7 new cases were all positive for EBER, and showed the expression of at least one of the FDC markers, including CD21, CD23, and CD35. The rest of 17 cases of IPT were all negative for EBER and essentially negative for FDC markers, but were all positive for SMA.@*Conclusions@#IPT-like FDCS of the liver and spleen is a rare low-grade malignant tumor morphologically mimicking inflammatory pseudotumor, and is easy to be misdiagnosis due to under-recognition. EBER in situ hybridization and FDC markers are indispensable for confirming the diagnosis.

Application value of secondary splenic pedicle separation technology through superior posterior approach of the pancreatic tail in laparoscopic partial splenectomy / 中华消化外科杂志

Objective To investigate the application value of secondary splenic pedicle separation technology through superior posterior approach of the pancreatic tail in laparoscopic partial splenectomy.Methods The retrospective cross-sectional study was conducted.The clinicopathological data of 13 patients who underwent laparoscopic partial splenectomy in the Ningbo First Hospital from March 2016 to October 2017 were collected.After preoperative assessment using computed tomography(CT) angiography,13 patients underwent laparoscopic partial splenectomy using secondary splenic pedicle separation technology through superior posterior approach of the pancrcatic tail.Observation indicators:(1) intra-and post-operative recovery situations;(2) follow-up situations.Follow-up using outpatient examination was performed to detect postoperative changes of peripheral blood platelet (PLT),thrombosis of splenic vein,lesions residual or recurrence up to November 2017.Measurement data were represented as average (range).Results (1) Intra-and post-operative recovery situations:13 patients underwent successful laparoscopic partial splenectomy using secondary splenic pedicle separation technology through superior posterior approach of the pancreatic tail,without conversion to open surgery,including 6 with laparoscopic partial splenectomy of inferior pole of the spleen and 7 with laparoscopic partial splenectomy of upper pole of the spleen.Operation time was 42-93 minutes,with an average of 61 minutes;volume of intraoperative blood loss was 30-260 mL,with an average of 92 mL;postoperative gastrointestinal function recovery time was 22-47 hours,with an average of 34 hours;postoperative drainage tube removal time was 3.0-6.0 days,with an average of 4.2 days.The postoperative pathological examination of 13 patients:7,2,2,1 and 1 patients were respectively confirmed with splenic cysts,splenic hemangiomas,vascular hemangiomas,splenic hamartoma and splenic lymphangioma.Of 13 patients,1 was complicated with splenic recess effusion and fever,and was improved with B ultrasound-guided percutaneous catheter drainage.Duration of hospital stay of 13 patients was 7.0-16.0 days,with an average of 9.6 days.(2) Follow-up situations:13 patients were followed up for 1.0-19.5 months,with a median time of 8.5 months.During the follow-up,PLT level of 13 patients was normal.Color Doppler ultrasound examination showed no venous embolism,and CT angiography showed good vascular perfusion.There was no recurrence of splenic cysts in 7 patients and no tumor residual or recurrence in 6 patients.Conclusion Laparoscopic partial splenectomy using secondary splenic pedicle separation technology through superior posterior approach of the pancreatic tail is safe and effective,and it can precisely dissect splenic hilum,preserve blood supply and function of the remnant spleen,and reduce surgical injury.

Laparoscopic partial splenectomy for splenic solid lesions / 中华普通外科杂志

Objective To analyze the safety and clinical effects of laparoscopic partial splenectomy for splenic solid benign lesions.Methods Retrospective analysis was made on patients with splenic solid benign tumor admitted from Jan 2015 to Feb 2017.Results 6 patients (4 males,2 females) underwent successful partial splenectomy for splenic tumors.Mean patient age was 44.7 years (range,28-58 years).5 patients were diagnosed by wellness examinations,1 patient had abdominal discomfort.The diameter of tumors ranged from 5.0 to 8.3 cm.Tumors were located in the superior lobes in 2 cases and the others were located in the inferior lobes.The operation times were 120-240 min and intraoperative blood loss was 50-1 400 ml (mean,375 ml).Laparoscopic procedure was successful in all patients without major complications.Postoperative pathology showed hemangioma in 5 cases and hemangioendothelioma in one patient.After 3 to 28 months follow-up no patients experienced recurrence.Conclusions Laparoscopic partial splenectomy is safe and effective in patients with focal benign splenic lesion that was located at the edge of the spleen or in the upper or lower pole of the spleen.

Imaging manifestations and pathologic features of splenic lymphangioma / 中华普通外科杂志

Objective To explore CT,MRI and 18F-FDG PET/CT features of splenic lymphangioma.Methods A retrospective analysis of 18 pathology proved splenic lymphangioma cases was made,including their clinical,imaging characteristics and pathological findings.15 cases with plain and enhanced CT scan,6 cases with plain and enhanced MRI scan and 1 case with 18F-FDG PET/CT examination.Results (1) 13 cases were single lesion,4 cases were multiple lesions and 1 case was lymphangiomatosis.The largest diameter was 15.8 cm,minimum diameter was less than 1.0 cm.(2) On CT imaging:cystic watery density and equal density interval was showed in 12 cases,in 3 cases as solid and slightly were showed lower density.Calcification was showed in 3 cases.On enhanced CT,internal septa was slightly enhanced and progressive inhomogeneous enhancement was shown in 3 cases.(3) On MRI imaging:cystic content was manifested as low signal on T1WI and high or highlight signal on T2WI,and there was a difference between the lesion and the splenic tissue on DWI.(4) One case underwent PET/CT imaging:18F-FDG uptake was not increased.Conclusions Preoperative CT,MRI and 18F-FDG PET/CT help establish a tentative diagnosis of splenic lymphangioma while final diagnosis relies on pathological examination.

MRI features and pathologic manifestations of splenic sclerosing angiomatoid nodular transformation / 中华普通外科杂志

Objective To investigate and summarize the MRI characteristics of splenic sclerosing angiomatoid nodular transformation (SANT).Methods A retrospective analysis of 5 SANT cases were analyzed,in terms of their MRI characteristics and pathological findings.Results MRI findings of SANT included:T1WI presents iso-signal or slightly low signal,all displayed lesions were detected as low signal compared with spleen,but higher than muscle signal on T2 WI,and with speck dots or starlike low signal in the central area,without necrosis and cystic change.The signal was significantly differentiated compared with the spleen on DWI.On chemical shift imaging,2 cases were showed slightly higher signal on out phase,the others without signal change.On enhanced scan,4 cases had edge obvious enhancement on arterial phase,inward filling enhancement,and the signal was higher than the spleen,1 case without arterial phase enhancement,but with mild concentric delayed enhancement.All of the speck dots and starlike areas decreased with time delay,with certain degree enhancement on delayed phase.Conclusions There were some MRI features of SANT,preoperative MRI can prompt diagnosis,but final diagnosis depends on pathology.

Laparoscopic total splenectomy vs partial splenectomy for splenic benign tumors / 中华普通外科杂志

Objective To evaluate laparoscopic partial splenectomy (LPS) for benign splenic tumors.Method Data of 55 patients undergoing laparoscopic partial splenectomy (20 cases) vs total splenectomy (LTS in 35 cases) at Peking University Third Hospital from August 2008 to July 2016 were collected and retrospectively analyzed.Results There was no difference in sex,BMI,preoperative H GB,preoperative PLT,operation time,operative blood loss and hospital stay between two groups.Age in LPS cases was younger than LTS group,while the tumor size was larger.On the 4th day postoperatively,PLT level was significnatly higher in LTP group.More patients in LTS group suffered from thrombocytosis.Conclusions Laprtoscopic partial splenectomy is a safe and effective procedure for the management of splenic benign tumors.

Tumors of lymphoid and hematopoietic tissue of spleen: a clinicopathologic analysis of 53 cases / 中华病理学杂志

Objective@#To study the clinicopathologic features, diagnosis and differential diagnosis of the tumors of lymphoidand hematopoietic tissue of the spleen(TLTS).@*Methods@#Fifty-three cases of TLTS were selected from the pathologic files from Peking University People′s Hospital from April 2002 to April 2017. According to WHO classification of tumors of hematopoietic and lymphoid tissues (2008) and its updated classification (2016), the cases were studied by microscopy, immunohistochemistry and in situ hybridization, combined with the bone marrow biopsy and clinical examination.@*Results@#In 53 cases of TLTS, the male to female ratio was 3.4∶1.0; the mean age was 55.4 years (range 21-76 years), and all patients presented with variable degree of splenomegaly. Laboratory examination showed increased percentage of lymphocyte in peripheral blood in 22 cases, and elevated serum LDH level in 24 cases. Abnormal blood counts were seen in 26 cases pre-operatively, in which 22 cases showed complete or partial correction of these abnormalities post-operatively (84.6%, 22/26). The clinical symptoms included abdominal pain or distension, fatigue, fever, and weight loss, etc. Seventeen cases presented with lymphadenopathy of abdomen or other sites. Bone marrow biopsy was performed in 30 cases, and 19 cases were involved by tumor (63.3%). Of all 53 cases, 43 were diagnosed as primary splenic lymphoma (PSL), and the remaining 10 cases as secondary TLTS. According to Ann Arbor staging, 14 cases were stages Ⅰ or Ⅱ, 6 were stage Ⅲ and 28 were stage Ⅳ. By histopathologic classification, 43 cases of PSL were splenic B-cell marginal zone lymphoma (SMZL; 48.8%, 21/43), diffuse large B cell lymphoma (DLBCL; 23.3%, 10/43), splenic diffuse red pulp small B-cell lymphoma (11.6%, 5/43), mantle cell lymphoma (9.3%, 4/43), follicular lymphoma (4.7%, 2/43), and composite lymphoma (CL, DLBCL and classical Hodgkin lymphoma; 2.3%, 1/43). The remaining 10 cases were chronic lymphocytic leukaemia/small lymphocytic lymphoma (4 cases), hairy cell leukaemia (1 case), hepatosplenic T-cell lymphoma (HSTL; 5 cases), with lesions in other sites. Of the 53 cases of TLTS, 47 were B cell neoplasm (88.7%, 47/53), and the T cell neoplasms were all HSTL(5 cases, 9.4%, 5/53), 1 case was composite lymphoma. In 11 cases of TLTS, EBER in situ hybridization was performed and all cases were negative. Forty eight cases had follow-up data, and the median survival period was 17.0 months(range: 1-96 months). The survival of patients with SMZL and DLBCL were 25.7 and 18.6 months respectively. Thirteen patients died (27.1%, 13/48). The prognosis of those with elevated LDH level, high clinical stage, B symptoms and older than 60 years of age was worse. And the prognosis of DLBCL was worse than that of SMZL. There was no statistically significant difference between these factors and prognosis (P>0.05).@*Conclusions@#Most TLTS cases present with splenomegaly and abnormal blood counts, and complete or partial remission of blood counts isseen after splenectomy. The most common pathologic types of TLTS are SMZL and DLBCL. Definite diagnosis of TLTS could be made by combining clinical features, histopathology, immunophenotype, genetics, bone marrow biopsy and laboratory examination.

A Case of Sclerosing Angiomatoid Nodular Transformation of the Spleen: Spoke Wheel Pattern on Computed Tomography / 대한내과학회지

Sclerosing angiomatoid nodular transformation (SANT) is a rare, benign vascular neoplasm. Most patients have no clinical symptoms, and the tumors are usually discovered incidentally on abdominal computed tomography or ultrasonography. Some studies have reported the clinical features and imaging findings of SANT, but the diagnosis is based on histopathologic examination of a tissue specimen obtained at splenectomy. We report herein an incidentally discovered case of SANT and review the related literature.